Bodies of Knowledge

<div class="book"> <H2>[[Embodying]] [[My Mother's|Memories]] [[Bones|X-rays]]</H2> <h3>A cultural osteobiography</h3> ''Felice Larsen: Multiple Hereditary Exostoses'' <img src="./img/title.jpg" alt="a brown and white sepia picture of a young woman overlaid with a shoulder bone with an obvious tumor".> [[Skeleton key: Felice's skeleton and memories|Skeleton key]] [[Multiple Hereditary Exostoses (MHE)|MHE and HSPG]] [[Family album|Memories]] [[Behind this book|How this book came about]] //[[Bodies of Knowledge: Vol. 1|Bodies of Knowledge]]: [[Forensic Investigation Research Station|About FIRS]] Colorado Mesa University, Grand Junction, Colorado.// ''Volume Authors: '' Deena Larsen Melissa Connor Christiane Baigent Series Editor: Melissa Connor 2024 ''Acknowledgements'' X-Rays courtesy of Dean Havlik, MD, Community Hospital, Grand Junction, Colorado All of this work is licensed under Attribution -Non-Commercia 4.0 Creative Commons, 2024.CC-By-NC 4.0 DEED //Please note this is a work in progress. I welcome comments on it (deena.larsen AT wsu.edu). I am keeping this up as an example of working on a complex hypertext... as well as for convenience. Feel free to read what we have so far--knowing that like everything else, this will change!// +++++++++++++++++++++++++WORKING NOTES HERE+++++++++++++++ ''New Pages to look at'' [[Navigation ideas]] [[Knees and lower leg: Mr Silent]] ''Dr. Connor's punch list'' Send Deena Christi's text with a paragraph header and one sentence lay introdcution References--send Deena references and keep sending them as you get them Send Deena photos numbers 846 [[Ribs|Ribs:Bad Guy]], 553 [[Osteoarthritis]], 407 [[Spine|Spine: Sitting Fused]] Need detail on the T thorasic and the L Lumbar STRATEGY 1. Hip picture (wikipedia) shows the proximal edge for replacement to show bone overgrowth 2. Do the spine for Lexie (pendunculated projection) 3. Get Christi's osteological report to Deena with the headings and easy to understand summary for each point to make 4. Work with existing photos Need images and descriptions for * Ask art intern for a picture of ribs from the front and then the side to show that exos toses do not show up on a living x-ray... * any spine that shows impingement of the [[spinal cord|Spinal Coordination]] (I have T7--check to see if this is ok) * [[Wrists: Writing]] * [[Hands: Holding]] * [[Ankles: Hiking Boots]] * [[Feet: Warn Me First!]] * On [[Continued Growth]] can we get a better shot of the growth on the actual metal ball joint? Need short clinical descriptions on images for * [[Jaw: Eating issues]] * [[Spinal Coordination]] This is spine5.jpg, which I think is T7 showing a compression point at the spinal cord? References * Need the reference to Back Pain Authority 2020 [[Spine: Sitting Fused]] * Check all references. I did add Ahn et al. 2021 to [[Hips]] discussion I am putting specific references on the individual nodes and referring to the external <A HREF="http://www.tinyurl.com/MHETalk">MHE Talk Google doc</A> where I keep all MHE references. This will allow me to keep up with the literature in one place. ''Graphics to do'' Separate the post-mortem x-rays. Get family photos with crops clean up the skeleton comparisons we have ''Deena To Do'' Integrate * [[C1-C7 neck|Neck: Stiff upper]] * [[Broken Neck|Cervical Fracture]] * [[Rib with a callus likely the result of a healed break|Broken Rib]] * Decide on a better navigational structure. This is a bit fluid right now as we gather info... * html code for tables in [[Limb Length Discrepancies]] (Should we split this into medical/memories?) * Try sometime again to remember where Felice had her surgeries and ask for xrays? Massive project. </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2> The solitary exostoses, called an osteochondroma, is a common bone tumor in children. But in [[MHE|MHE and Bone Formation]] a single individual can have tens, or a hundred of these tumors. These tumors can [[grow|Femur:Gowing bones]] (Stiever, and Dormans 2005). Manifestations of hereditary multiple exostoses. Journal of the American Academy of Orthopaedic Surgeons, 13: 110-120.) and decrease throughout life (Wengrowicz et al. 2011). Almost every one of Felice’s bones included exostoses, some more significantly prominant than the others. Felice named some of the tumors that most impacted her life. Discover some of [[Deena’s recollections|Memories]] of [[Felice|Her name]] living with these exostoses with photographs of the tumors that are most likely the ones Felice is talking about. [[Jaw: Eating issues]] [[Neck: Stiff upper]] [[Spine: Sitting Fused]] [[Ribs:Bad Guy]] [[Shoulder: Mr Crunchy]] [[Arms: Sweetums]] [[Elbow: Polished]] [[Wrists: Writing]] [[Hands: Holding]] [[Pelvis: Woman Parts]] [[Hips]] [[Hip Replacements: Super Hips]] [[Femur: Growing bones]] [[Knees and lower leg: Mr Silent]] [[Ankles: Hiking Boots]] [[Feet: Warn Me First!]] <img src="./img/skeleton.jpg" alt="a layout of Felice's skeleton."> [[X-rays]] [[Memories]] [[References]] Stiever, JR., and J.P. Dormans, 2005. Manifestations of hereditary multiple exostoses. Journal of the American Academy of Orthopaedic Surgeons, 13: 110-120. https://pubmed.ncbi.nlm.nih.gov/15850368/ Wengrowicz, M.L., J Pretell-Mazzini, J.P. Dormans, R.S. Davidson, 2011.. Regression of a Sessile Osteochondroma: A Case Study and Review of the Literature. https://www.researchgate.net/publication/267426996_Regression_of_a_Sessile_Osteochondroma_A_Case_Study_and_Review_of_the_Literature </div><!--this closes the book div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/ribs.jpg" alt=a normal rib bone compared to those from Felice. AT the end of her rib bone is a large wedge-shape bone.> <img src="./img/rib3-right-distall.jpg" alt=a normal rib bone compared to those from Felice. AT the end of her rib bone is a large wedge-shape bone.> //Ribs. A.C. Ribs with exostoses. B, D, plastic skeleton ribs for comparison. The exostosis on A would be on the right side of the back, approximately mid-back. The exostosis on C would be the lower right side near the end of the rib cage, this is the most obvious candidate for “Bad guy”. The tumors seen in Fig x would probably catch on the adjacent soft tissue when Felice had to take deep breaths. // Third rib, right side. Right Rib 3 presents a small, sessile deposition (11.17 x 5.75 mm) of bone superior to the tubercle. The sternal end presents a pedunculated growth along the caudal border and displaced visceral growth. The architecture of this defect appears to be related to MHE but the presence of well healed antemortem trauma (oblique fracture with well healed callous) overprinted by MHE following cartilage deposition during callous formation and subsequent MHE derived disruption to the healing cascade likely. This may be a suite of change related to both MHE and antemortem trauma ''Deena's Memories late 1970s'' A few years later, we are driving in the mountains to see the fall leaves (my sister is living with my father, so it is just the two of us). We stop suddenly at the side of the road, where there are only dark pines and no pretty yellow and red aspen trees shimmering in the sun. I start to ask why we stopped here and then I see the lines of my mother’s mouth tighten in pain. I sympathize. Both of us have long since become inured to ibuprofens or aspirins and we don’t take other pain pills because a doctor once explained patiently that we could be addicted and that would be much worse than the minor pain we might feel from a bone. (I will go through my life with an insane fear of drugs and addictions.) My mother goes through [[her breathing|Lungs]] and calming exercises. “Bad Guy” she tells me when she can breathe and she can drive again. I nod, knowing exactly what she means. Bad Guy was the bone she’d had since childhood, the one lower down in her ribs that catches the edge of her lungs. She cannot feel it on the outside of her ribs, and when she went in for X-rays once, they said there was nothing there but an odd shadow. So, they told her it was nothing to worry about. [[Deena's ribs|Deenas Ribs]] [[Spine: Sitting Fused]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="deena"> <H2>(print: (passage:)'s name)</H2>] ''//Embody.//'' verb (used with object),em·bod·ied, em·bod·y·ing. * to give a concrete form to; express, personify, or exemplify in concrete form: * to embody a spirit. * to collect into or include in a body; organize; incorporate. * to embrace or comprise. <A HREF="https://www.dictionary.com/browse/embody">Dictionary.com</A> [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the deena div>

<div class="book"> <H2>(print: (passage:)'s name)</H2>] <img src="./img/firs.jpg" alt=a logo in reddish brown showing a fingerprint, skeleton hand, magnifying glass with a chemical compound, dna strand, and crime scene.> This volume is the first of what the authors hope to be a series. Over a hundred people, each incredible in their own way, have donated their bodies to Colorado Mesa University’s [Forensic Investigation Research Station (FIRS)|About FIRS]]. Another hundred-plus have become living [[donors|Donations], signing up to come to the FIRS when they die. Each of these are individuals with stories to tell, and often those stories are reflected in their bones. Telling the stories from the bones is what forensic anthropologists and bio archaeologists are trained to do. But almost never does the scientist interact with the decedent or the families of the descendent the way that we do at the FIRS. The skeletal collection at the FIRS is growing, and every one, as stated above, has a story. In addition to this rare disease, the collection includes suicides, those with substance abuse, other rare diseases, cancer patients, and amputees. FIRS’ researchers and staff write scholarly articles and publish for the academic community. This series is a way to get some of this information to the public that supports the FIRS. We would like the public to see what we see in these skeletons and understand why they are so very valuable to the academic and medical community. Also, we hope the reader understands why the FIRS staff feels such deep respect for and community with our donors. [[How this book came about]] [[Dedication]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2> To Felice Larsen: Let’s turn this crazy mixed-up, upside down world around Create our world where humor, joy, love, and laughter abound. The Laughing Lady, [[My Dance of Joy]]: [[Felice's Poetry|Felices poetry]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2> This volume in the Bodies of Knowledge series became an idea as Connor, Baigent, and Larsen converged on the authorship of this volume. All of us knew that due to the rarity of Hereditary Multiple Exostoses (MHE) that Felice’s skeleton would be extraordinary. From the day she first left her mother at FIRS, Larsen, who suffers from the disease herself, brought up the idea of using the skeleton to show the effects of the disease in a manner that could not be done in any other manner. Baigent came up with the idea of a socio-osteo-biography that included how Felice lived with the disease integrated with the formal osteological analysis. Once Felice’s skeleton was clean, Deena came to visit and discuss a conference for MHE sufferers that center on the skeleton and coping mechanisms. Connor broached the idea of a book that would use the anecdotes that Deena told about her mother. Connor realized, at that point, that Larsen was a talented poet and writer and the perfect co-author for this volume. The images in this volume are all used with Larson’s permission, along with using Felice’s name. There are multiple goals to this volume. This first is to highlight this disease, show the effects on the body, and tell the story of a remarkable person who lived with that disease. Felice was, even without MHE, unique. Deena has given us permission to reproduce Felice’s poetry and has added her own stories of life with MHE. This material is included in the hopes in provides a deeper, non-clinical understanding of the condition. Many of us who do not live with this condition have no idea that it exists, what it is, and its impact on people’s lives. The Larsens’ story shows that the reverse is also somewhat true. The continual pain, the daily subluxations (bones going “out”), the perpetual potential for broken bones, the fear of post-surgery bone overgrowth, all becomes normalized when constant. This condition is also rare enough that sufferers can feel isolated. We hope the reader without MHE takes from this text an understanding of, and heightened sensitivity to, this condition. We hope the reader with MHE feels that their story, along with Felice’s, is reflected in this volume. [[Dedication]] --- [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the book div>

<div class="felice"> <H2>(print: (passage:)'s name)</H2> My Dance of Joy: Body Beautiful Felice Larsen Body beautiful not for me My inheritance from my family tree Multiple Hereditary Exostoses or MHE a particular sugar lack put me on the wrong track My body branches bone spurs grew On joints old and new Extra calcium deposits on bones emerged at times painful they surged Movement impeded lost flexibility forward body movement impossibility From childhood my love of dance I followed In mountains I loved hiking and skiing My body defects did not stop me Throughout my life I practiced much activity My walker helped make walking a wonderful possibility Other physical gadgets I used to increase--motion I did not lose </div><!--this closes the felice div>

<div class="book"> <H2>(print: (passage:)'s name)</H2> The Forensic Investigation Research Station (FIRS), operated by Colorado Mesa University (CMU), is one of a handful of facilities in the world, nicknamed “body farms” that use human remains to study how bodies decay. While that is the primary purpose, they also clean and curate the donor skeletons, providing a sample of skeletons from modern populations for research and education. The FIRS is in western Colorado. At approximately 4600 feet above sea level, it is the second highest such facility in altitude in the world; the highest being TB40 in Park County Colorado, also operated by Colorado Mesa University. Both facilities are in an arid climate, and bodies tend to mummify. The FIRS is a research hub, and associated professionals and students have publications on decomposition, scavenging, microbiology of death, and other subjects. The FIRS personnel hold classes and training for CMU students, but also train professionals, law enforcement, and death investigators in how bodies decay, osteology, and telling the difference between human and non-human bone (the location of deer skeletons have been known to be held as crime scenes for an hour or two until law enforcement can reach an “expert”). In addition, the FIRS holds annual cadaver dog training, helping dog handlers who work with their animals to locate human remains. None of this could be possible without individuals [[donating their bodies|Decisions to donate]]. [[Decisions to donate]] [[Legalities for donations]]:[[Organ donation]], [[Whole body donation]], [[Body farm donation]] [[Donating to FIRS]] Felice: [[Felice's decision|Felices Decision]] [[Finding FIRS]] [[Transporting Felice's body|Transporting Felices Body]] [[Delivering Felice's body|Delivering Felice]] [[Accepting Felice's body|Accepting Felice]] [[Understanding Felice|Understanding Felices body]] </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2>]  None of this could be possible without individuals donating their bodies. Donors come to the FIRS and TB40 by two means. Individuals can donate themselves while they are still alive, in much the same way that individuals can designate their organs can be donated. Relatives can donate deceased individuals when they are the legal next-of-kin. The FIRS currently has over 100 individuals that have signed up to come to the FIRS on their death, but many of the donations are calls from relatives near or at the time of death of the donor. Why do people donate their bodies or their relatives? One reason is fiscal; dying is an expensive business. A cremation in 2020 is likely to cost about $1,000, and that comes with an inexpensive plastic box as an “urn”. Needless to say, nicer urns exist, and a family can spend as much money as they wish on an urn. Extras such as a memorial service, obituary, and copies of the death certificates add to the cost. An unexpected cost of $1,000-plus is significant for many. Others respect and support the educational goals of the facility. The younger donations are in their 30s, generally the result of drug overdose or suicide. Families have donated to help make our students more aware of the issues of addiction and suicide, and as a way to open the door for those conversations. [[Legalities for donations]] [[Felice's decision|Felices Decision]] </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2>] Human remains can be donated to science by the person themselves before they die or by the legal next of kin after death. Even if a person wishes to donate their own body, they need to ensure that the next of kin, or their personal agent, will carry out their wishes. Few donation agencies will take a body over the strident objections of the next of kin, even if they have the legal right to do so. In the United States, the Federal Anatomical Donation Act lays out guidance for states in the donation of human remains. Most states have enacted laws or regulations that reflect a regional version of the federal act. The regulations usually outline the entities that can set up a donation program and for what purposes. Also, they define who is next of kin. Generally, a person designated as a legal representative heads the list, spouses are next, then adult children, then parents, and the list goes on. The FIRS prefers that all relatives are in favor of the donation, but we follow Colorado law and the wishes of the legal next of kin. [[Whole body donation]] [[Organ donation]] [[Body farm donation]] </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2>] Whole body donation is different than the donation of organs. Many whole bodies go to medical schools or universities. For this type of donation, there are restrictions on how long the individual has been dead, how tall they are and how much they weigh. For biology classes, the remains need to be relatively fresh. The height and weight restrictions are so that the embalmed body can fit in a container of a standard size, which classrooms are set up to work with. After about two years, the embalming gets a little funky, the remains are cremated, and the cremains (ashes) are sent back to the family. Organ donation prior to anatomical donation is not an option. </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2>] Organ donation, the type of donation people sign up for by checking a box on the driver’s license, is different. Generally, organ donors die relatively suddenly in a manner where their organs are still usable. Advanced cancer patients, for instance, will probably not be able to donate many organs. Many elderly people can still donate parts of their eyes. In other cases, skin is taken from the back of an individual for skin grafts. Marrow donations are made by taking the actual long bones from the arms and/ or legs. These are replaced by dowels and batting, so that the body can still be clothed and view-able in a funeral. Generally, the body is sent back to the family after the organ donation, which takes place quickly after death, and a funeral or cremation is still possible in a normal timeline. </div><!--this closes the book div>

<div class="book"> <H2>(print: (passage:)'s name)</H2>] Donation to a taphonomic facility, or “body farm” has some major differences. The body can be dead for longer than in any of the other donation types and size restrictions are mainly for safety in handling the remains and not restricted by a container. Most important for many people is that nothing is returned to the family. Facilities like the FIRS keep the skeleton and use the bones for research and education. It is this unique aspect that made the present study possible. </div><!--this closes the book div>

<div class="deena"> <H2>Felice's Decision</H2> Deciding to Donate Felice’s Body Deena’s Recollections 2001 It is one of my rare visits up to Salida to see my Felice, my mother, and we have been to the hot springs and are now relaxing at her favorite local hangout. She is sipping her signature drink, hot chocolate laced with coffee. Our conversation is continually interrupted by people passing by our table: “Hey, I saw you in that last play, it was wonderful”… “ Are you going on our hike next week?”.. “Wanted to just tell you…” But we manage a serious conversation anyway. I tell her about [[donating her body|Decisions to donate]] to research—that with [[our condition|MHE and Bone Formation]] research could help save lives or save some kid from the pain we have been through. She agrees enthusiastically, and then someone starts playing music and she just has to dance. With every single person there. Well, in her 70s, she can be a little eccentric, I think. [[Donating to FIRS]] [[Transporting Felices Body]] </div><!--this closes the deena div>

<div class="mhe"> <h3>(print: (passage:)'s name)</h3> This rare disease has multiple names: Multiple Hereditary Exostoses (MHE), Hereditary Multiple Exostoses (HME), Multiple Osteochondromeda (MO) and other titles. The key word here is multiple--there isn’t just one bone or one single area as every cell is affected. Felice and Deena grew up with the term "MHE", so that term is used consistently throughout this volume. Patients with MHE have a genetic mutation in the EXT1 and EXT2 genes, so their bodies do not produce heparan sulfate proteoglycan (HSPG). In MHE, the lack of HSPG causes patients to develop [[exostoses|Skeleton key]], benign tumors in multiple locations throughout the body (Brown 2008, Thompson 2011, and Mansouri et al. 2017). In MHE, benign bone tumors grow out of the bones throughout the body. Some patients have only a few tumors, some may have hundreds. Initially, the tumors tend to grow out of the ends of the long bone shafts but may being to form on most of the skeleton. The tumors may be attached to the bone at the base (sessile like Felice's [[arm|Arms: Sweetums]]) or by a stalk (pedunculated like Felice's [[shoulder|Shoulder: Mr Crunchy]] ). The pedunculated tumors look a little like a nuclear explosion or a broccoli floret with a stalk that comes out and branches out into a lumpy bowl shape. The sessile tumors broadly attach to the bone at the base of the tumor. [[Limb Length Discrepancies]] [[Osteoarthritis]] [[Osteoporosis]] [[Trauma]] [[Continued Growth]] [[References]] --- [[Skeleton key]] [[Memories]] [[Begin again|Title page]] </div><!--this closes the mhe div>

<div class="felice"> <H2>(print: (passage:)'s name)</H2> [[Dedication]] My Dance of Joy: Body Beautiful [[My Dance of Joy]] [[Creative Dance]] [[Look for the Silver Lining]] [[I'm growig old!|I'm Growing Old]] [[Chosen]] </div><!--this closes the felice div>

<div class="felice"> <H2>(print: (passage:)'s name)</H2> Creative Dance Felice Larsen From stillness Breath born Impulse arising Imperceptible movements My chest rolls Movement grows Travels--arms, shoulders, head Soon my whole body dances Ready now, I join others Form duos, trios, quartess Finally, whole group Feeling together Spontaneously improvising Our culminating dance Brought to fruition fulfilment felt! </div><!--this closes the felice div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/jawall.jpg" alt="a close up of a jaw where the bone is flat instead of rounded. Image captions Figure x. Right. Felice’s mandible with insert showing flattening of head of the mandibular ramus, or the part of the jaw that articulates with the skull. Left. Comparison skeleton. A. Felice’s skull. B. plastic skeleton. Insets showing where the mandible articulates and the flattening in Felice’s surface to match the articulating surface on the jaw."> ''Deena's Memories '' ''2017'' I am visiting the nursing home at dinner to see why my mother has lost weight so quickly. I see a dedicated nurse feeding her, and her dinner is mostly spewed in multi-colored confetti all over the floor. I grin ironically, as our meal times have always been chaotic and messy. And I recall her dental stories. And her bridge work. I always thought it a character flaw in us that we had rotten teeth and mouths. I, too, could never eat neatly and politely (my American sushi parties, where the point is to eat as messily as possible are always so much fun, but in public…ahh well not so much). Now, I wonder. Could my mother’s jaw have been malformed as well? Would [[MHE|MHE and Bone Formation]] be the real culprit behind our dental issues? Can I have an excuse for not eating in public or for constantly having a crumb-strewn desk? [[Neck: Stiff upper]] [[Shoulder: Mr Crunchy]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] Image captions </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> 1973 A different hospital memory. This time we are here for my wrist, which is growing a new bone on my wrist at an awkward angle, and I can’t hold a pen. We will decide against surgery, as I am only 9 and the bones could grow back. Surgery only in life-threatening circumstances is our rule. (50 years later, I will read about multiple surgeries with other kids and I will cry inside as sometimes it works and sometimes it doesn’t). My mother shows the doctor her own misshapen wrist and says it hurts like hell and what are you going to do for my daughter? The doctor shrugs. The doctor feels my wrist, grabbing it hard and I try not to flinch from the lightning burst of pain. He says it can’t be too painful because I can still flex my wrist and make a fist. We leave knowing nothing will be done. I will later learn to hold a pen differently and to type around the pain. [[Hands: Holding]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/pelvis.jpg" alt=a normal pelvis compared to the one from Felice. At the front of the pelvis are barnacle shape growns and a fused sacrum and ox coxae. The two plastic hip impants have a sheath of bone over them.> //Fig. x. The front of the pelvis. A. Felice’s pelvis, photo by Curtis Martin. The normal pelvis (B) is in three bones (sacrum, and two os coxae) separated by cartilage. In Felice’s pelvis, the cartilage has turned to bone, fusing the three pieces into one. The plastic caps are where the femur articulates and are part of the hip replacements that she had. It is apparent that the bone grew over those replacement parts and, as with the femur head in Fig. x, the healing bone did not grow back in the normal template.// While the photos are of Felice’s hips, not Deena’s, it is fairly easy to understand how the exostoses could make menstrual cramps worse as the muscles try to cramp around the tumors. Probably even more to the point, the deficiency in heparin sulfate proteolycans that causes the tumors may also be linked to production of the chemicals that control the menstrual cycle (syndecans) and the production of the endometrium, part of the layer that is shed during menstruation (Germeyer et al. 2006). ''Deena's Memories'' ''1977'' I’m 13, lying on an air mattress in a dark room in Penny’s house in upstate New York. We are on our Trip Back East. I am moaning softly in pain, doubled and tripled up with cramps. These pains are swift and severe, unrelenting as they squeeze my belly like an anaconda, grabbing more and more of my body with each labored breath. Sharp pokes in my pelvis echo this pain and I cannot breathe without these shards of pain. My mother is sitting uncomfortably on the carpet floor beside me, soothing my brow, trying to calm me quietly so we won’t wake Penny. We have already been to a gynecologist about my severe menstrual cramps a few months ago. He said that my period pain was just stress, dear, and hardly anything to worry about. Every girl got this, and I obviously just had to get used to the pain. I will have these episodes throughout my life (where I can not even stand up against the onslaught of pain) and find out 25 years later that they are a combination of burst ovarian cysts and one of the most severe cases of endometriosis that my new gynecologist says she has ever seen. She will give me birth control pills and I will have violent reactions to them. So I will thank the gynecologist for her understanding and just go on grimly coping with these episodes without meds. My mother tells me first about her episodes, that every other month or so she is doubled in pain, and I know this. I have seen it and waited on her when she is in bed like this and she is in so much pain that she can not sit or stand. It is normal, she insists. Every girl goes through this, and they just don’t say anything about it, because this is a secret, private thing. She certainly would not complain, and I shouldn’t either. To shush me and distract me, my mother tells me about her adventures, mostly with Penny, from her college and career years before she had me. Penny and the Bear, Penny and the Bike that Ran Away, The Lost Canoe with the Sleeping Bags and Food, the Mountain Cabin with the Roof that Fell Down, [[the Dance|My Dance of Joy]] under the Waterfall, the Stone Path in the Moonlight. I still sob quietly under the onslaught of pain on the pointy air mattress. My mother tells me that our vaginas and cervices are not like [[our lungs|Lungs]], as they are trapped in a bony pelvis and we have no way to shift them. She lays down on the floor and shows me how she shifts blankets and pillows and extra sweaters and socks stuffed with underwear and other socks to soothe her own “woman parts.” She tells me about how she did this when she was pregnant with me and how she vowed never to go through that again. “You have to shift your entire body,” she says, to accommodate those woman parts. [[Hip Replacements: Super Hips]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/hip-replacement.jpg" alt=a close up of bone that has formed a sheath over the plastic hip replacement.> <img src="./img/hip-replacement2.jpg" alt=a close up of bone that has formed a thick layer of bone over a hip replacement.> ''Deena's Memories 2000'' I am having dinner with my mother at [[Taki’s|Spine: Sitting Fused]]. She has come into Denver from Salida to see her hip specialist about a hip and knee replacement. This is years after my only surgery, which had failed spectacularly. I tell her not to do it. I remind her again about my knee surgery when I was 18 to reconstruct my knee. My bones had grown back in sharp planes along the incision points on my knee. And I tell her stories back to her about her knee surgery in the 1930s and how that grew into [[Mr. Silent|Knees and lower leg: Mr Silent]]. Her bone grew back in a large lump on top of her knee. Her hips continually goes out these days, and last week when was on a hike with her second husband, Jerry, her hip went right out and she could not walk. Jerry had been a bit ahead and had not waited for her and she had had to shove her hip into place and get a couple of branches for canes and come down off the trail on her own. She hates that the hip might go out again on a trail (note that not hiking was certainly not an option) and she wants the hip replacement. I try to dissuade her: “The bone will just grow back. Having surgery is just hurting yourself in the long run.” but she is having none of it. She replies that Helen, a good friend she had met in grade school, was having her hip and knee done at the same time. They would share a room together in the rehab. It would be an adventure, an in-one-place cruise with meals and activities right there. Fine I say. Don’t listen to me. Do whatever you want. The bones will grow back. “No they won’t” she snaps. “The surgeon said that can never happen and bones don’t grow back. Who knows more--the surgeon or you?” I stop arguing. I visit her in the rehab and she complains that Helen got to go home after three weeks but she was there for six weeks. Helen only did the hip, I tell her. You did both the knee and hip. “Yeah,” she says. “And I had to use a wheelchair for two whole weeks.” Undeterred, they will both go through this again in about two years, and my mother will once again do both her hip and knee at the same time, crowing this time about only staying in rehab 10 days longer than Helen and only using the wheelchair for a single week. She’ll refer to them as her super hips from then on, crowing about the miracles of modern surgery. ''2024'' Almost 25 years later, I see the bone has just oozed over her plastic hip sockets and is creeping inside it. The bones spill over like old snow piled on the side of the road, encasing the metal and plastic in swirling drifts of bone. Her skeleton is now a mute testimony that I cannot help myself from crowing in triumph over the long-gone surgeon. I know our [[MHE|MHE and HSPG]] bodies. I live in one, I told you the bones grow back. If a surgeon ever tells me that is a fantasy, I’ll whip out these pictures and prove that reality is far more complicated than they can possibly imagine. [[Hips]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> Figure x. A. Felice’s right lower leg compared to B. a plastic skeleton. The thinner bone on the left is the fibula, the thicker bone on the right is the tibia or shin bone. By the time of her death, her two lower leg bones were fused, which undoubtedly allowed her less movement in her lower leg. Mr. Silent may be the protruding portion of the tibia in the upper (proximal) portion of the bone. 1981 In Children's Hospital waiting for my own [[knee surgery|Deenas knees]], Felice settles into her chair and feels her shin. She tells me about the knee operation she had when she was 17, oddly enough almost exactly my age to the month. Her tibia had sported a row of huge bones, and her knee had fused and locked so that she could not walk. The doctors had shaved her tibia and reconstructed it, she said. Then she lifts her pant leg up and I see her huge knot on her leg. I had named this knot Mr. Silent when I was a child, as the strictest rule in our house growing up was never ever to touch her leg. If anything touched it, she would yell out. She tells me Mr. Silent grew back in a few months after her leg surgery, and just keeps growing every year. She tells me she has RSD because of the surgery, and that means that if anyone touches her leg anywhere around the spot, she feels like someone has torched her whole body with lightning. Later, in the nursing home, I will tell every nurse not to touch her leg. They often do not listen, and then have to endure Felice’s screams and sobs. Put images on a gallery page <img src="./img/knee.jpg" alt=a normal tibia and fibula compared to Felice, where the bones are bumpy and pitted with a protrusion on the tibia.> <img src="./img/knee-replacement2.jpg" alt=a normal tibia and fibula compared to Felice, where the bones are bumpy and pitted with a protrusion on the tibia.> [[Ankles: Hiking Boots]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> ''Deena 1970s ''You never dared touch my mother's feet. She wore only the softest of socks and would shoo anyone away from grabbing her. Shoe stores were an ordeal for her (as they are still for me). The only shoes she could tolerate were soft leather ones or sandals that were cut at a particular place to avoid the extra bones on her feet. She would tell any shoe sales man that they could not go within a foot of her foot, and she'd say it was because any shoe she bought she needed to put on herself. But it was really that she was terrified of anyone touching her foot. '' 2017'' Felice could not reach her feet as [[her arms|Arms: Sweetums]] would not reach and she did not have the mobility. So she had always used a stocking puller. If anyone had to help her, she would patiently explained that her feet hurt and she needed to be warned first. If they warned her, she would take a huge breath and then slowly exhale, forcing her body to relax into the pain of the touch. As her dementia increased, her ability to warn someone to warn her first decreased. And the nurses got tired of waiting for an hour or so for her to pull her socks on, so they grabbed her feet without warning her first. This was always a HUGE mistake. I explained to the nusrses each and every time that Felice's bones in her foot stuck through nerve and tissue. Finally, I stuffed a sock with stones and cotton. The stones stuck out, and I said, Look, if you had stones like this on your foot, it would hurt as well. This was probably not the best metaphor, because the nurses kept grabbing my mother's foot and Feice would howl every single time. [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> (text-colour:purple)[The front of the right shoulder blade. Right scapula, anterior. Left, plastic skeleton. Right, Larsen’s scapula. The large bone growth on the top is a pedunculated exostosis, named by Felice “Mr. Crunchy”.] <img src="./img/scapula.jpg" alt=Two views of a scapula. One is normal and the other is from Felice showing a large broccolli shape bone growing from the tip of her scapula.> //The front of the right shoulder blade. Right scapula, anterior. Left, plastic skeleton. Right, Larsen’s scapula. The large bone growth on the top is a pedunculated exostosis, called “Mr. Crunchy”.// Mr. Crunchy is the lump that sticks out on her shoulder, the one she babies more than anything else. ''Deena’s Recollections 1969 '' We are in Glenwood Springs, Colorado and I am in the bubble chairs, just looking at the patterns on the stones, years of polished accretions of minerals, with smooth bumps and odd rough ledges that feel like my arm bones. I love the bubble chair and I beg shamelessly for quarters for it--unlike most kids who begged for quarters to play in the arcades. I never play the arcade games, for my coordination does not permit the effortless moving of joysticks and screens. In fact, I am crying a bit because a swimming instructor in Denver has just informed my mother and me that I am hopeless at swimming, that I will never master the crawl through the water and I should be watched at all times, lest I drown. I am 5 maybe 6, and I love the soothing feel of the warm water, so this is a heavy blow. My mother comes towards me and pries me out of the bubble chairs in the Hot Pool and into the Big Pool. “Time you learned to swim for real,” she says. She says she never mastered the crawl either, and she had wanted to be a lifeguard in the Reform Jewish summer camps she went to as a child. “But they never let me. I told my father once and he said I should not give up, that I should practice and make the lifeguard team next year” she starts the story. “but I never did get my arm above my head the way they wanted me to. She hesitates here for more than a minute in the story, looking away at the pale red and bone white hill above us, her mind clearly reliving something in other far away hills that she is not willing to confide to her young child. “I went dancing instead,” she says finally. She shows me how she lies on the one side and then reaches her arm under the water and then out a little in front, almost up to her head but not quite. Then she shows how her one knee bends one way in the water against the other one, and then they kick sideways through the water.” It’s a modified side stroke,” she says. “Don’t show this to a real swimming instructor. They like to have their little fits.” She holds me on my side under my waist, away from my hip and spine bones as I first move my arms and legs one way, then another, experimenting. “You have to find a way,” she says. And I do. late 1960s through early 1980s We pile in the car and wait impatiently for Felice to get settled. We are late going to school, or we are heading for the hills or to the zoo or the park or wherever--this action is repeated every day, many times a day--so often that my memory’s edges are well beyond fuzzy. My mother carefully eases herself into the driver’s seat, avoiding the seat until she leans forward to adjust her shoulder in the pillow behind her, the seat belt pad in front. “Oh come on!” I think to myself, and sometimes I say it out loud. “Why does Mr. Crunchy always get so much attention!” 2017 45 or so years later, I am at the nursing home, ready to take her to her beloved swimming at Salida Hot Springs just up the block. But we still drive as it is too far for her to walk and swim with her weak lungs after her pneumonia. I have safety-pinned a baby towel with a soft duck’s head to the passenger seat that is just right to cradle Mr. Crunchy, and she adjusts it carefully. She tells me that the nurses lost her special pillow, that they don’t think it is really important for Mr. Crunchy to have a good night’s sleep. I decide to fight the battle to keep her on oxygen rather than the battle with the nurses to make Mr. Crunchy happy. [[Arms: Sweetums]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> (text-colour:red)[Top right – the front of an upper arm bone (anterior humerus) from a comparative skeleton. Bottom. Felice’s right humerus in the same position.] <img src="./img/humerus.jpg" alt="Two views of an upper arm bone. The one from Felice is twisted oddly and is bumpy, and her joints are polished and worn smooth."> //Top right – the front of an upper arm bone (anterior humerus) from a comparative skeleton. Bottom. Felice’s right humerus in the same position.// ''Deena's Memories'' 1996 I am visiting Felice when she lived in Santa Fe, and we are in her favorite consignment dress shop. My mother is looking at something with soft sleeves, a bit loose, in a bright fabric. She loves to shop at thrift stores because they are good deals, but more than that, the clothes are soft and not stiff as they travel up and down her arms. The sales lady bustles over with a tight-fitting dress in long tight sleeves with close fitting cuffs and crows that my mother has the trim figure to fit it. Felice recoils in horror from the constrictive garment and picks out a flowing Mexican tunic with a bright pattern picked out in citron yellows and oranges. I too, carefully choose clothing on how soft it feels against my bumpy arms. I have to admit, though, that my clothing choices are not as wild as Felice’s. (text-colour:red)[Felice’s right upper arm bone turned inward (medially) slightly to best see the exostoses that is probably the one named “Sweetums”. ] <img src="./img/humerus-medial.jpg" alt=Upper arm bone turned toward the body to show a large hill-like bump.> 2000 It’s late June, a bit before my mother’s 75th birthday, but she doesn’t want to miss the Class IV rapids down the Arkansas River. We arrive at the rafting company an hour or so early as I did not want to explain this birthday present over the phone. “And I’m going in the lead raft!” she chirps. And tells me again about how she and Penny, her best friend from college, canoed all the way down all the rivers in the Adirondacks. It took them three summers. I look over at her. She is 80, 85 pounds tops if you count her hiking boots. She’d been in a severe depression that winter again, and I hadn’t checked on her. She says she might have eaten on most days. Probably. Well, maybe not every day. But she did not attempt suicide that winter (we have a pact, she and I. We know that we both have severe Seasonal Affective Disorder. So, we don’t commit suicide between October and April. Any other time, well, that may be because the pain is too much and we want to be able to leave the party. Knowing we have an out makes it that much easier to stay—and she did enjoy the party for her 92 years of it.) She flexes her arms to prove that she can still raft with the best of them. She points to the muscle mass a bit down from her shoulder. I know this bump on her arm is not actually muscle—its Sweetums, her arm bone, as there is no equivalent bump on her other side. She named this bone because she says she can rub arms the same way most people rub shoulders. And it gives people a place to hug--above the bone, above Sweetums. The rafting guide turns out to be wonderfully patient as he takes Felice in stride. “Let’s just go out and test this first,” he says, leading her into a raft on dry land. She happily tells him she cannot do the overstroke, but she has developed an understroke that will give the same power against the current. He asks her to put her special moves against the paddle as he pushes against it like an undertow. She grunts with the effort and the paddle moves an entire inch against his hand. I’m shocked at her strength, but it is definitely not enough for the river. “Let’s put you in as Queen of the Nile,” the guide says. “You can sit up high and get the best views.” She prances up to the oar boat, lead rafting dream forgotten now. “I am Queen of the Nile,” she crows to the gathering rafters as we all suit up. “Whoohoo!” and down the river we go. [[Elbow: Polished]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> Glockenberg et al (1997) suggest that there is ankle deformity in 50% of the cases they examined. Of course, their sample consisted of two individuals, so perhaps that should be taken with a grain of salt. ** ''Deena’s Recollections 1997'' Even though the literature says the [[bones do not grow back|Bones Grow]], now I can prove--they really do (often from an [[injury|Trauma]], but also when they just feel like it for no discernable reason). I fell through a plate glass table and badly slashed my wrist. The bone and keloid now form a noticeable bump. I show every doctor this and that alone usually convinces them that something odd is going on here. I show my mother my healed-bone wrist when she is in town, and she tells me the story of her ankle when she and Penny had been climbing a hill. She can show the part on her ankle to doctors, but it is not as in your face as my wrist is. So, my injury was much luckier than hers. ''Felice’s story 1950s'' Felice had scrambled up a switchback trail and was right above Penny on the trail--and Penny wanted Felice to toss down a candy bar, as Felice had the food on her back and Penny had the shelter and bags on hers. So she tried to and caught her foot and twisted her ankle so badly that “technically it was a break, but not really worth a cast” as the doctors would say later. Her ankle grew a new bump, and from that point on, she had to stretch any hiking boot to fit over the bump. However, because she had not cut the surface, she did not have a scar to prove that the bump was from that mountain trip. [[Feet: Warn Me First!]]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="felice"> <H2>(print: (passage:)'s name)</H2> ''Look for the Silver Lining'' Felice Larsen Look for the silver lining It’s the light shining Sustaining me through adversity That spark of joy of love of humor that sheds surrounding shadows that climb to fresh horizons that silver lining buried deep midst coal mine blackness Look for the silver lining You’ll find it in my poetry “Silvered silver sun’s arrow strikes Dispersing fears” You’ll find it in my life’s message You’ll find it in the care home I visit daily as I go among Blank faces glazed eyes Lost use of legs or arms, Dulled senses fading minds. </div><!--this closes the felice div>

<div class="felice"> <H2>(print: (passage:)'s name)</H2> ''I’m Growing Old!'' //Every maternal matriarch in my mother's line adapted and recited this poemon their 80th and 90th birthdays. This is Felice’s adaptation that she sang on her 90th birthday on July 19, 2015// Yeah Boy! Feels like I’m 16 inside-- Ready to hike those mountains and Hit those trails once again! Dancing to my pleasure--what a treasure! Go out and play on this sunny day! Some people as they age complain of aches and pains and cloudy rains-- They want to range at each passing wrinkle, Each new gray hair But let them go along the way And let us go out to play. As for me, I’m ready to play again and again. So let us dance and color and create For all of us can re-create a world we can celebrate! So trot out your appendicitis, your hip surgery, your plitis. I’m ready to take them all on so they no longer plight us. So let us laugh with joy and make new friends and treasure old friends And let us play and dance today! </div><!--this closes the felice div> [[Felices poetry]]

<div class="bone"> <H2>(print: (passage:)'s name)</H2> { (set: $slideshow to (a: "<img src=./img/C1-400px.png> The first cervical (neck) vertebrae was broken prior to death directly left of the midline. The fracture did not heal and formed a false joint.", "<img src=./img/C2-400px.png> The second neck (cervical) vertebrae has a projection (dens) that fits into the first and helps in rotation of the skull. Here the projection reacted to the break on the first neck vertebrae and moved backward slightly (a posterior displacement).", "<img src=./img/C3-400px.png> The third neck vertebrae shows compression fractures between the superior and inferior articular facets. Osteoarthritic changes to the joint surfaces are characterized by scoring, macroporosity, eburnation, and peripheral deposition of bone.", "<img src=./img/C4-400px.png> The 4th neck vertebrae shows changes to the superior (top) articular facets to accommodate the proliferative deposition observed in C3. Dramatic deposition on the edges resulted in the ventral (forward) extension of the joint contour. The dorsal (back) aspect of the joint surface is characterized by expansion of the joint surface and a ridge of new bone formation to accommodate the posterior tilt of the overlying vertebrae. The inferior articular facets are characterized by marginal expansion of both joint surfaces with densification and microporosity. The right inferior facet presents dramatic poster-lateral and lateral overgrowth with concomitant formation of pseudo arthrosis.", "<img src=./img/C5_6-400px.png> The bodies and the right articular facets the fifth and sixth (C5 and C6) neck vertebra are fused. The top of the articular facets of C5 changed to accommodate the changes in C4. The bodies and the right articular facets of C5 and C6 are fused. Fusion of the right facet surfaces of C5 and C6 is characterized by reactive bone that extends to the pedicle. The edges of the bodies of C5 and C6 have grown bone that resulted in bridging the space between the two bones.", "<img src=./img/C7-400px.png> The top surface of C7s body presents a diffuse formation of relatively large pores (macroporousity) and bone growth around the edges. Bone growth bridging the gap between C6 and C7 was in a state of active formation but incomplete. Both superior articular facets present new bone deposition on the joint surface and overall changes to the joint contour. The left superior facet presents ventral expansion that resulted in the reorientation of the joint over the process on the side. The left inferior articular facet presents an expansion of the joint surface, resulting in the extension of the joint along the length of lamina, terminating at the root of the spinous process. The joint surface is characterized by small pores (microporosities); a plague of new bone deposition is present along the length of the joint surface. The right inferior articular facet presents posterior and lateral widening of the joint surface that is less dramatic than the changes presented on the left but still pronounced.")) (link-repeat: "[(print: $slideshow's 1st)]<slideshow|") [(replace: ?slideshow)[(set: $slideshow to (rotated: -1, ...$slideshow))(print: $slideshow's 1st)]] } This would be in the middle of the upper back. The discs between the upper three vertebrae have disappeared and the bone fused. The space between the bodies of the lower two vertebrae show where the disc would be. The lipping on the right side of the picture (anterior or front of the bone) is typical of severe osteoarthritis. Generally, the vertebrae in the backbone are separated from each other by a soft disc, that keep the bones apart and cushions them. They add height to the spine and facilitate movement. It is common in older people for the discs to deteriorate and this one factor in why people often get shorter as they age. If the disc deteriorates until there is nothing there, the bones fuse together. This is more often the case with severe OA. Fusions of multiple vertebrae, such as in Fig x below, are likely to reshape the spine and decrease flexibility. In people with porous bone, these may areas may break repeatedly causing pain and instability (The Back Pain Authority 2020).// Tipping on the left shows a more normal range of osteoarthritis for 92, exostoses outside of the range of normal osteoarthritis. Collapsed vertebra may be a function of trauma or failure from lack of bone density or age. Unusual fusion on her spinal column. [[Osteology: Spine]] ''Deena’s Recollections 1974'' I’m 10 and my mother has found a special gym class for me which we go to every Thursday. They teach me how to bounce a ball, jump rope, and tie my shoes, as I have poor coordination. They also spend hours and hours with me to walk straight, with a book on my head and a glass of water in my hand for balance. This proves to be about as impossible as climbing Mount Olympus and having tea with the gods there. The PT asks my mother to do the same thing, and then mimics how she slumps a bit, her spine not quite straight, ostensibly to show us both our bad habits. “This is as straight as I get,” she informs him after some frustrating moments with the book sliding down into the floor. “Unless you want me in a straight jacket.” The PT produces a brittle laugh, and suddenly, I don’t have to go to that gym class anymore. ''1990s-2016'' After Felice moved to Salida, she occasionally came down to Denver where I lived. For about 25 years, I always made the same arrangements to meet her and one of my friends at Taki’s, a local restaurant she and I can both reach by bus. (She no longer drove at this point as her macular degeneration had progressed and this is both before and after I had my Lasik surgery, which allowed me to combat the severe astigmatism that had rendered me legally blind since childhood). The food is cheap, nutritious, and good, so she has no complaints. We talk about the weather, politics, her poetry, the new play she is in, the latest concert she has seen, or my latest work. I strive to keep things friendly, but afterwards my friend helps me validate what I heard from my mother’s remarks--did she really say I looked three months pregnant after I’d gained weight on Lyrica or that I made the right decision in not having kids because they would have a horrible life, or that my accomplishments were all due to her doing a good job raising me? Yes, she did. But it is ok. I never tell my mother the real reason why I chose Taki’s as our meeting place--the chairs. These chairs are curved in such a way that her coccyx hurts after about an hour, and she finds excuses to cut the evening short as she still has to have the spoons to get the bus to Helen’s, her friend’s apartment where she usually stays. I have a similar problem, but I always strategically place two socks in my underwear before meeting with her. That way, I feel fine on the way home. [[Spinal Coordination]] [[Pelvis: Woman Parts]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> 1972 I am just learning to sew and I am sewing clothes for my one rag doll, Charlotte, named after Laura’s doll in the Little House series. I don’t like dolls in general, as they remind me too much of people. But Charlotte is special as she is soft and her body just exactly cradles my neck as I lay in soft summer grass and tell her secrets. I pick up the needle but I cannot thread it--the thread refuses to go where I want it. I run to my mother, holding the tiny silver sliver and the even smaller thread. She tries to show me how to hold the needle between thumb and forefinger, and then her thumb slips out of joint and at an odd angle. She tells me to go to Mrs. Darling, a neighbor, to thread needles. I look at her dislocated thumb and ask if she is alright. We did not name the bones in our hands, as there were so many, and she and I are used to hands that suddenly go on strike and yet refuse to negotiate about their working conditions, leaving us in a stalemate. My mother assures me she is fine, and Mrs. Darling takes an entire pack of needles and threads them all for me, each nestled in its own place on an old playing card. 1985 We emerge out of the forest trail into a small meadow with pockets of columbines and bright strokes of Indian paintbrush, rimmed in light blue spruce and dark shadowed pines. We break out the rations and talk about politics, my college classes, her new pottery obsession, and more as we lay in some of the softer grasses. At some point, I remind my mother of the way we used to compare our bones. We compare hands only because our hiking boots and her thick jeans and my thick hiking skirt preclude anything else. She tells me about a new bone in her finger. I feel it as it hooks under and through her skin. She tells me a story about when she was seven or so. or just starting to write. She had the worst penmanship in school, and a teacher had twisted her wrist and held it down, right over one of her bumps. She just bit her lip and wrote. Her arm was bruised from the pressure. Her mother visited the school, and she was moved to a different class, never seeing that teacher again. 1994, Years later, I travel for work frequently. Each trip is a barrage of noise nad worry about missing a plane if my leg goes out and I can't walk or making it to the gate on time. This is before 9/11 so security is pretty lax. I carry a small embrodiery hoop with patterns of celtic knots and I use thick embroidery threads and a few patterns as a way to calmly occupy my hands while I am waiting for a plane or a call and cannot lose myself in a book. I ask my neighbors to thread the needles before I go, as I havel never really mastered the fine coordination needed to thread my own needles. My fingers or wrist usually cuts out or refuses to work for a while, so I pack brain teasers and logic puzzles for when my hands are out to lunch. 2017 At the nursing home, I watch my mother’s hands shake a bit as she grips her walker, and I realize that now over the years they have slowly and imperceptibly carved themselves into a fantastically gnarled mess, like the roots of an old pine. Her hands move glacially, like a crab’s knobby pincers encrusted in barnacles and swollen from seeping seawater, as she tries and fails to grasp a crayon, a spoon, a cup. Fig. x hands [[Pelvis: Woman Parts]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

<div class="deena"> <H2>(print: (passage:)'s name)</H2> I, too have a few bones that catch on my ribs as well, and they have grown and shifted since I was a child. My story may help illuminate [[Felice's ribs|Ribs:Bad Guy]]. ''Deena’s Recollections 1971, 1977'' I am rolling down the tiny hill in the park in front of our house--over and over and over again, laughing into my dizziness. I’m 7 and this is my favorite summer activity; I feel like I’ll never have to stop. Then, whomp! The pain slams into my chest like an express freight train through an insignificant tiny station. I can’t expand my lungs or my chest and I struggle to draw air into my body. I feel trapped under the train wheels of pain and I start to panic inside as I cannot breathe enough to move at all. My mother sees me motionless at the bottom of the hill and comes running out. “Breathe in slow” she commands me. “Slowly. Slowly. No panic. Calm. You can do this.” I stay still as the never-ending train uses my ribs for train tracks, finding pockets where I can breathe under the pain. “Explore the inside of your chest,” my mother instructs me. I know what she means. I cannot remember the first time we have had this conversation, and this time will only be one of a countless many that I have with myself as bumps on the inside of my sternum and ribs catches my lung or my heart. I calm down, go into my chest in my mind and fill my lungs oh so very slowly and carefully, like a creaky hot air balloon caught in a cave full of stalactites and mites. She tells me once that [[her father taught|Felices father]] her this same breathing exercise for her for her own rib pain, that she had named “Bad Guy” as a child. But I see the pain in her eyes from telling me this, so I do not press for details. Once I have caught the rhythm of breathing through the pain again, I tell my mother that I think I am growing a new bone, and I point to the epicenter of the pain. I will name this one Mr. Meany, I say. (The Mr. Men series has just come out and my mother has bought me the first book, so every bone name from that era stems from that obsession.) I will name the next one to bother my lungs Mr. Nasty, and the one by my aorta Mr. Grumpy and I will carry them in my chest, never knowing when they might catch on tissue or just act cranky. We name our bones as a shorthand map of our pain. My mother must have named [[Mr. Crunchy on her shoulder|Shoulder: Mr Crunchy]] about this time, as this is about the era that I recall her shoulder bone really bothering her. ''2024'' I am shifting my breathing and body to get more comfortable around a new one growing on the inner side of my ribs. I have the same pain symptoms as a heart attack, and I've gone to the ER to prove it was not a heart attack. The chest x-ray show no problems (although they do want to remove the tumor on my upper shoulder, the one I've been calling my [[massively strong|Arms: Sweetums]] deltoid). I show my physician assistant the page with my [[mother's rib|Ribs:Bad Guy]] and the tiny pendiculated hook on the inside of her rib cage, hiding from any x rays. We agree that this is probably the same for me and that I could do a CT scan nowadays to fully delineate the problem. I refuse. I just don't want to go through the fight that would ensue about going in and removing it. </div><!--this closes the deena div>

1930s Felice had lain down on the bed beside her father and had gently touched what he termed his knobbly bones on his knees and ankles, his arm and elbow. What are those? She had asked him, pointing to his arm, which had spikes midway down and points sticking up like a second hand trying to emerge from his wrist. “My door knobs,” he said. “Gives me an extra way to knock on the doors of opportunity.” I have those too! She had giggled and shouted, sitting up and pointing to her ankle. He had sighed heavily and said,” I know. And I am so sorry.” 2024 I wish now, writing this, I’d asked my mother more about her childhood and how she grew up with her father, who also had MHE. I had asked general questions, and she had responded with memories of her school, PPS 99, swimming at the beach, feeding people at the back door, saving every scrap of anything--the general stories of a child growing up in the Depression. I wish I’d asked about the MHE more often, but every time I did, she would tell that same story about giggling with her father and nothing more. Her mother was an epileptic, and her father was always exhausted (out of spoons, we would say) so she had to be extremely quiet at home or she’d be blamed for making one or the other of them ill. I wish I had asked her more what her father had said about the bones. About how he coped. But I think she did not know that much more about his story, either. An accountant and financier who had pulled his money out of stocks in 1928 before the crash, he hid his disability, wearing shoes that corrected his posture under fashionable wingtip covers, having long jackets with a high shoulder pad on one side, but not the other. We never really talked about it—vestiges of shame in being thought in-valid, crippled, diseased still trickle down through the silence of generations. The family lore is that his mother did not want to appear pregnant, and so she tightened her corsets in the 1880s—thus disfiguring her child. More likely, my mother said, is that her paternal grandmother hid her condition with fashion even before and after her pregnancy with my grandfather. My mother mentions that her father always carried a handsome gentleman’s walking cane with a head carved like a parrot’s beak that he once said his mother had used until her death, and he’d use it until his. I finally persuade my mother to [[use a walker|In Praise Of My Walker]] in her early 80s, and she just named it Roll-on-da and went on hiking. My own knees were no longer supporting me then, so I had learned to hike trails in a wheelchair. And thus I taught her how to use the walker on mountain trails we both loved as well.

<div class="mhe"> <H2>(print: (passage:)'s name)</H2> Research is also showing that heparan sulfate significantly impacts lung structure and regulates cellular signaling. The EXT1 and EXT2 gene code for the elongation of the heparan sulfate and may have something to do with endoderm development in the lung. Heparan sulfate may also impact healing during any lung injury (Haeger et al. 2016). The impact of the heparan sulfate deficiency on the lungs of MHE patients does not appear to have been studied, but this may well be a part of the pain that Felice and Deena report. [[Skeleton key]] [[Memories]] [[MHE|MHE and HSPG]] [[Begin again|Title page]] </div><!--this closes the mhe div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/pelvis-posterior.jpg" alt=a normal pelvis compared to the one from Felice. At the front of the pelvis are barnacle shape growns and a fused sacrum and ox coxae. The two plastic hip impants have a sheath of bone over them.> The back side of the pelvis (the posterior of the posterior). The large tumors that hang attach to the top of the hip bone (along the iliac crest) can be seen in A, as well as the tumors in the left side of the junction between the sacrum and iliac. The medical term for a hip socket that doesn’t fully cover the ball portion of the upper thighbone is hip dysplasia. This condition allows the hip to frequently dislocate. Since Felice has [[hip replacements|Hip Replacements: Super Hips]] on both sides, she would not have suffered this at death. Research, however, suggests that approximately 25% of patients with MHE have one or more tumors in the ball of the femur that keeps it from articulating with the socket in the hip bone and causes hip dysplasia (Malagón 2001). Ahn et al. 2021 adds: "The results suggest that the characteristic deformities represented by coxa valga in the MHE hip act as an offset for FAI symptoms, on the contrary, act as a trigger for ischiofemoral impingement (IFI) symptoms." *** ''Deena's memories'' ''1960s'' My mother is swinging to some classical music, something sprightly like Bach's Goldberg Variations or some mysticalasian tune. She'll sway and move sinuously, slowly, then quickly. And then suddenly stop, well before the music ends. She hardly evern crashes to the ground--but stops in mid sway, frozen. I know what happens next, as I have done it myself in my own body, weekly, daily.. There is a precipice of anticipation as her (Sacroilliac) SI joint has just "gone out" and the next motion will create a crashing crescendo of pain. She breathes deeply in and slowly moves into the pain. Her leg is oddly distended, her back a bit bowed. She won't walk far for a while and will continue to contort herself to ease her hip back in. She looks over at me, swaying with her. Stopping with her. "I hope this doesn't happen for you," she whispers. I hear her anyway. And it does happen to me. And, like her, I take a deep breath, steel myself to what is next, and keep moving. ''2000s'' As this happens more and more, Felice goes for [[hip surgery|Hip Replacements: Super Hips]] and after that, I don't hear about the hip going out as much. [[Knees and lower leg: Mr Silent]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] References Ahn, YS., Kim, S., Kim, WJ. et al. Characteristics of hip impingement syndrome in patients with multiple hereditary exostoses. BMC Musculoskelet Disord 22, 153 (2021). https://doi.org/10.1186/s12891-021-04021-1 Malagón, V., 2001. Development of Hip Dysplasia in Hereditary Multiple Exostosis. Journal of Pediatric Orthopaedics, 21(2): 205-211. https://journals.lww.com/pedorthopaedics/Abstract/2001/03000/Development_of_Hip_Dysplasia_in_Hereditary.14.aspx. (Malagón, V., 2001. Development of Hip Dysplasia in Hereditary Multiple Exostosis. Journal of Pediatric Orthopaedics, 21(2): 205-211. https://journals.lww.com/pedorthopaedics/Abstract/2001/03000/Development_of_Hip_Dysplasia_in_Hereditary.14.aspx. In approximately 25% of patients with hereditary multiple exostosis, there is an abnormal osteochondral formation localized in the femoral proximal metaphysis. This formation often causes a mechanically progressive insufficiency of the acetabular cavity, a true developmental hip dysplasia, that together with a coxa valga deformity, which is also present, causes a gradual deterioration in the relations of this joint. This malformation has a poor prognosis and is difficult to manage. Although this entity is rather frequent and quite severe, it is rarely found in the medical literature. The author describes six private cases, taken from a total of 24,000 patients (0.25/1000) as examples of this entity, and provides a review of the literature.) [[Femur: Growing bones]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the bone div>

Tibia – Deena’s Recollections 1981, 2004, 1938 I’m settling into a hospital room in the cold of January, this time for a few days. My three knee bones on my right leg have finally colluded with each other and gotten to the point where I cannot walk at all. I joke about my leg at school--when God handed out knees, I went back for thirds and fourths even! Although I have been out on my own for a couple of years, I’m not 18 yet, and so someone has to sign for my care. (My father and I were not on speaking terms from when I was 13 through my third year of college, which is a completely different story.) I have been going to Children’s Hospital in their charity care program, and they have convinced me that I could walk again if only I take these three big bone tumors off of the inside and outside of my knee and reconstruct my knee. Being crippled and “housing insecure” is just not the greatest combination. Since the surgeons here have promised me a warm breakfast, packed lunch, and PT and transportation to high school every day until I can walk again I have agreed. I hate the fact that everyone just stares at my leg, that gaggles of doctors and students keep goggling at me, but I just don’t see any other choice. Spoiler alert: the surgery will go well and it will take me well through the summer to learn to walk again . thanks to Peggy, a wonderful nurse there and Susie and Steve, the PTs who came in before their shifts each day and so many dedicated people there. (Lift your leg. Lift now with a sock on your knee. On your ankle. Over your hip. Now the sock has two beans. Now it has twenty. Try this weight). The surgeons will cut a nerve during surgery and I get RSD, so I will wear long soft skirts for the rest of my life because anything touching the scar will set off an electric socket of tingles and pain. I will ignore the constant pain in each step so that I can hike and explore odd pockets of the world, climb mountains, and have a wonderful time doing it--until my next collapse in 2004, when we will find that the bones have grown daintily back precisely along the surgical cuts and broken off, and that I have ground my meniscus and cartilage into long-forgotten memories. My mother comes to meet me as agreed in the entrance hall of Children’s Hospital, bringing me some clean clothes I’d left at her house, a few books I loved to reread when I was in pain, and a new nightgown. She accompanies me to the room and signs the paperwork. We haven’t talked in a while, so it is a bit awkward as she sips her powdered hot chocolate laced with coffee from the ages-old visitor coffee pot. We talk about my schoolwork, how I am doing in the accelerated honors classes and will be able to test out of maybe even an entire year of college. We talk about her poetry, her new dance class, and dwell on the fact that a few Iranian students could hold the U.S. hostage for years and force a new turn to the right. “I feel like my spirit is a never-ending hostage to my body,” I tell her. “I know,” she says. “My father felt the same way. But most of the time, I just have to imagine my spirit is free.” [[Knees and lower leg: Mr Silent]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]]

<div class="mhe"> <h3>(print: (passage:)'s name)</h3> ''Disparity in paired bones'' Generally, MHE's clinical manifestations include short stature, discrepancies in limb length and frequently a misalignment of the wrist joint (Waldron 2009:202). Most of the literature on MHE talks about the disparity in limb length as a cause of fatigue and pain. Felice had two hip replacements and a knee replacement on the left side, making the comparison of sides slightly unnatural. On the right side, the tibia and fibula (Fig. x) were fused, so she would have had little ability to rotate that lower leg. For most people, that alone would cause significant pain and fatigue. Table x. Maximum lengths of paired limb bones. Bone Left (mm) Right (mm) Arms Upper arm/ humerus 260 268 Lower arm/ radius 184 192 Lower arm/ ulna 194 195 Approx. length of shoulder to hand (humerus+ radius) 444 460 Thighbone/ femur Shinbone/ tibia 335 321 Lower leg/ fibula 301 301 Heel/ Calcaneus Approx. leg length (femur + tibia+ calcaneus) ''Deena’s Recollections 2019'' Felice always put an extra padding in her shoe on her right side. We both always wore the sturdiest shoes, usually hiking boots, to compensate for an uneven stride. Photos of my grandfather also show a marked higher leg. Felice's arms were very short, and she had a reacher in practically every room in the house so she could just “reach over and reach” whenever she needed something. [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div><!--this closes the mhe div>

I have split open my wrist on a plate glass coffee table in a stupid accident. The wrist has healed slowly and I now have a beautiful keloid covered scar just barely covering a new bone that provides a nice resting place for my palm [[Hip Replacements: Super Hips]]

<div class="mhe"> <H2>(print: (passage:)'s name)</H2> Osteoarthritis **Put picture 553 in and The extent of the OA Osteoarthritis (OA) is one of the most common bone diseases. Osteoarthritis is the inflammation or loss of cartilage in the joints. The cartilage is a slippery tissue that allows the joint to move with less friction. Generally, the protective cartilage that cushions the ends of the joints wears down over time until there is less cartilage, and sometimes the bones are rubbing together with no protective cartilage between them. Symptoms can include pain, swelling, warm and tender joints, and limited movement of the joint. Sometimes sufferers feel a grating sensation when they use the joint or hear a popping sound. OA can be managed, but not reversed, and generally worsens over time. OA can be caused by any number of reasons and the prevalence increases with age, so that in extreme old age, few individuals do not have some signs of osteoarthritis. It is also more common in females. As a 92-year-old female, it is expected that Felice would have some signs of this disease. However, in her case, the disease is extreme and likely made significantly worse by the same genetic mutations that cause the exostoses which also impact bone formation. It is almost impossible in bone to determine the cause of OA (Waldron 2009: 29). In bone, OA may result in production of new bone (hypertrophic) or very little bone (atrophic). Signs of OA in bone include polishing (eburnation)m marginal osteophytes, new bone on the joint surface, pitting on the joint surface, and/ or alteration in the joint contour (Buikstra 2019; Waldron 2009: 34; White et al. 2012). The hypertrophic bone, or formation of new bone, generally has a different presentation than the tumors caused by MHE, but they could overlap and would occasionally be difficult to separate. As the two diseases are working together, it is not possible to separate out the presentation on the bone in all cases. Felice's OA can be seen in [[her jaw|Jaw: Eating issues]] [[MHE and Bone Formation]] [[MHE and HSPG]] </div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/spine5.jpg" alt=a .> The tumors, as they grow in the vertebrae and the ends of the ribs that reside there, have the potential to compress the spinal cord, which can cause loss of control in legs, feet, arms, and hands, depending on where the compression lies. Documentation of this is rare, as is the disease, but one case of a 14- year old girl with MHE documents this compression (Vinstein and Franken 1971). Another study discusses three children with bone tumors (one with MHE) and discusses the surgery used to address the compression (Twersky et al 1975). ''Deena's Memories '' The doctors have told my mother that without operating, she will continue to experience her foot drop and still have trouble moving her feet and legs. But this has been true for all of our lives. We commiserate, as we both have always had trouble and pain walking up and down stairs. Often, our feet or hands just don’t seem to pick up the message to move--like they are out of town and their voicemail is full and we’d have to try back later. I’ve long since picked up my mother’s habit of pitching my hands under my knee and yanking a foot up the stairs when it doesn’t want to go. We always had suddenly dropped whatever we were carrying. Ahh the crashes of life. Neither one of us ever had decent coordination, and we have always fought rebel body parts, as my mother terms them. She mentions that her father told her once that she would have to put down the rebellion in her own body every single day. Now she tells me again about her yoga, her dancing, her swimming, and how I should do more of that to regain a range of motion and improve my coordination. Fine, I tell her. Fine. I’ll do that. </div>

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/humerus-right-distal-end.jpg" alt="End joint of elbow polished smooth.">Image 207 Distal end of right humerus, showing polishing (eburnation) of the capitulum which articulates with the head of the radius (the lower arm bone on the thumb side). Eburnation indicates the loss of the articular cartilage and the rubbing of bare bone on bare bone, creating a polishing of the surface. Also note the lipping around the margins of the joint. Both indicate osteoarthritis; eburnation suggests extreme osteoarthritis (Waldron 2009). ''Deena’s Recollections 2024'' The FIRS researchers keep coming back to the polished state of her elbow, which indicates severe arthritis and pain with every motion. Dr. Connors says this would be the main issue for most people. However, I honestly think that this one just got lost in the crowd. I cast my memory back over our lives and can not find a time when she complained of elbow pain. ''Deena's overlay 2024'' My own elbow will pop in and out of joint on its own, rendering that arm temporarily useless. I'll casually create a sling for a while out of a long-sleeve shirt (I have long since taught myself how to do this with just one functioning arm). And I'll shift to using just one hand for a while. I unconsciously plan my life around the potential for a sudden lack of mobility or use--just as people living near a flash flood area or fire area unconcsiously know exactly what they will do and where they will climb to safety at any given time. I have been working with a PT to learn how to gently persuade my arm muscles to relax a bit and regain their former shape around the protruding bone fragment or popped elbow or whatever it is. I can press in with a towel or softly press the muscles and tendons back over the bone with my hand. I tend to be too rough, so I have to learn to be a patient patient. But try as I might, I do not recall my mother ever accommodating her elbow. No shifting of weights or what she was carrying; no casual mention of her elbow going out. I surmise that her elbow osteoarthritis did not suddenly cut off her functioning. If this were a gradual process over her lifetime, I suspect she simply learned over the years to live with the pain. [[Wrists: Writing]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] Page references: Waldron, T., 2009. Paleopathology. Cambridge: Cambridge University Press. </div><!--this closes the bone div>

<div class="mhe"> <h3>(print: (passage:)'s name)</h3> Osteoporosis is common, characterized by reduced bone mass and an increasingly fragile skeleton. Healthy bone is constantly being re-absorbed and re-modeled, with the full skeleton being recreated about every seven years. When there is an imbalance between the re-absorption and re-modeling, osteoporosis is the result. The most common result is increasing bone fragility and fracture. In the section below on trauma, the overt broken bones seen in the skeletal analysis are presented. For the bone remodeling to go well, the re-absorption and re-modeling have to be coupled in time and space (Nozawa et al 2018). Otherwise, this the skeleton becomes like a house remodel, where the owner takes out the kitchen, but then paints the bedroom and puts in a new toilet, while the kitchen sits there, not functional. If the reader has been paying attention so far, it will come as no surprise that [[heparin sulfate proteoglycans|MHE and HSPG]] are integral molecules in the biochemistry of this process (Nozawa et al. 2018). Nozawa and collagues (2018) outline the process by which the EXT1 mutations associated with MHE also lower bone density. [[MHE and Bone Formation]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] ''Page references:'' Nozawa S, Inubushi T, Irie F, et al. Osteoblastic heparan sulfate regulates osteoprotegerin function and bone mass. JCI Insight. 2018;3(3):e89624. Published 2018 Feb 8. doi:10.1172/jci.insight.89624.

Trauma Trauma implies an external force that impacts the body. It includes broken bones, dislocations, and injuries such as knife wounds or gun shots (there are no knife wounds or gun shots on Felice’s remains, that is just used as an example of trauma). MHE can cause thinning of the bone, as does osteoporosis. Both then contribute to what is known as pathological fractures (Auderheide and Rodriguez-Martin 1998:361), or fractures that happen during normal use of the body, but the bone has thinned to the point that it cannot take the stress. This section discusses antemortem trauma, trauma that happened while Felice was alive and there is a clear healing response in the bone (Buikstra 2019). As with the other conditions discussed here, this is not an exhaustive list of the trauma on Felice’s remains (see Baigent, Appendix A for a complete list). It is also not unlikely, given Felice’s bones response to insult, that there is trauma hidden under bone growth that presents as OA or MHE tumors. [[Cervical Fracture]] [[Broken Rib]] ---[[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]]

Cervical fracture. Non-union Fracture At some point in Felice’s life, she broke the first bone in her neck, directly below the skull. The fracture is through both arches, both the anterior and posterior, with some displacement of both sides of the vertebrae. This is a burst, or Jefferson’s fracture (Waldron 2009: 140). The fracture healed but left the bone in two pieces (Fig x) which each healed separately. This type of fracture is often caused by an impact to the head so the skull is driven into the vertebrae, such as diving into shallow water, falling, or hitting the head on the roof of a vehicle. Fig. x. The first vertebrae below the skull, the atlas or cervical 1 (C1). Left plastic skeleton, Right Felice’s C1. <img src="./img/cervical-fracture.jpg" alt=a .> Generally, a cervical fracture is diagnosed through pain, not being able to twist the neck freely, numbness at the base of your head, and/ or loss of feeling or pinprick pain in your arms or legs. A Jefferson fracture, like this may include pain in the upper neck, but no neurological signs. As with most broken bones, one diagnosed, the bone would be immobilized to allow it to heal. It is possible, given Felice’s generalized pain, she did not realize that she had broken her neck, or decided that the collar that would be used to immobilize it would be more painful than the fracture. ''Deena’s Recollections 1998 - 1999'' Someone does an X-ray of my mother’s spine (was it for a minor car accident? a fall down a mountain? A slipped trail when cross-country skiing? I can’t remember now.) She calls me at my work, distraught. I grab a cup of tea from the breakroom and go into a conference room and call her back so we can talk. She says that they found a bone spur in her spinal column (I forget which C or T it was) that could impinge on her spinal cord, and if she fell again she’d be paralyzed. They want to operate right away. I tell her no way, that the bones would grow back, that the certainty of damage from the surgery would be worse than the uncertainty she has obviously lived with up to now. She agrees. She doesn’t go bck for any follow up, as she feels fine. Ironically, I am in a car accident the next year. The ambulance driver feels my spine at the back of my neck and I stupidly say that that hurts. So, they put a rigid collar on me and strap me to a baseboard. I explain about the extra bones in the X-ray lab, but they will not let me off the rigid carrying board. It is a Friday night, and it takes forever to find someone to read the X-ray results. I explain that I already know about the exostoses and would they please let me off the damn board. The doctor says he is worried about the C7 impingement. If you don’t take care of this now, we cannot guarantee how long you will be able to walk. So, look, we can do a more thorough MRI next week to determine any spinal cord damage and then operate. I ask if there is an immediate danger from the car accident. The doctor hesitates, well, no not as such… I leave the ER with a promise to follow up. I call my mother. We discuss the pros and cons for both of us and the risks we face. Is it better to undergo the surgeon’s scalpel and risk them cutting a nerve, impinging our spinal cords? The bones will probably grow back, as they do after any injury, and then who knows what danger those new bones would pose. Or is it better to just trust to luck and chance and deal with any fallout after an accident that severs or impinges the spinal cord? We end up pledging to each other to be careful and not to fall down. Anything more seems to be a bit silly to our minds. When the nurse calls back to set the dates for my exam, I tell her what they can do with their operations. False joint, Cervical vertebrae. [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]]

Broken Rib Often around a broken bone a callus forms, bridging sections of the break (Waldron 2009; White et al 2012). Fig x. The bottom side of a right rib. The arrow points to a callus likely the result of a healed break. [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] <img src="./img/broken-rib.jpg" alt=a .>

<div class="mhe"> ''Migraines and dementia'' Migraines and dementia don’t show up on the skeleton, but they were a part of Felice’s life and are included here as part of Deena’s recollections. It may be worth it here to repeat that the gene that causes MHE, causes defects in the substance heparin sulfate that the body produced. In addition, to impacting bone formation, heparin sulfate also seems to have a role in the development of the nervous system. There is research that suggests that heparin and pentosan poly sulfate may help to prevent migraine headaches (Russell and McCarty 1999; Theoharides et al. 2009); that might suggest that poor heparin sulfate could make someone at greater risk for migraines. It also appears that heparin sulfate plays a role in the brain function. A study that inactivated EXT1 totally, produced morphological defects in the brains of mice and in mice, a range of autistic symptoms (Fumitoshi et al 2012). The researchers suggest that a heparan sulfate deficiency may impact socio-communicative deficits and play a role in a person having autistic characteristics. ''Deena’s Recollections 1973 - Migraines'' My mother’s door is firmly closed and has been for a day or so. My mother is having one of her sick migraines again and will come out for the cans of soup we store for times like this. It is early spring, March, so we can’t bother my father, even if he were home, which he often is not at this point--this is tax season and he is an accountant. My parents are now in the process of a loud and messy divorce, so my sister and I maintain as low a profile as possible. I quietly tiptoe into my sister’s room and get her ready for school. My sister is in first grade now, and I am in third, so unlike last year, she won’t have to wait for me after school because we are on the same schedule. We know how to run the toaster on silent, and we pour milk into our bowl first to quiet the crunchy fall of cereal. We shut the front door ever so softly and then walk the three blocks to school. I make plans to meet her at her classroom door and walk us both home. I have the headaches too, but I try not to miss school. I dim the lights and hide under the covers until the pain that fractures my head goes away. I pretend the pain is a spider, its legs penetrating my skull, and I tell the spider silly stories to make it go away. I will be plagued by these sick headaches all my life, and I will learn to go immediately into a quiet dark room when I see the little silver flashes that presage the attack. There I meditate and calm my breathing. I think I learned some of this from my mother, and some on my own. I honestly can’t remember. 46 years later, I stare at the smooth contours of my mother’s skull, wondering whether the migraines that plagued her left any stamps on her skull. I make a note to ask, but I forget to. [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div>

Deena’s Recollections December 2017 - Dementia We are in the foyer of the nursing home, with the winter sun streaming in. Felice is beaming up at me from the wheelchair they have restrained her to, leaning forward for a kiss. She warbles on about the cold weather but as the sun is shining now we can go outside. For the first time in over a decade, she has not greeted me with the drama that she wants my address, and my blood shivers with the realization that her dementia has finally gotten truly out of control. More than a decade before this, I met and married MaJe, who was appalled at my mother’s way of saying “I am sorry you were ever born” every time we got together (ostensibly apologizing for the MHE) and her habit of threatening to come over to “truly make our house a home.” MaJe, my wife, had determined that not giving her our address to Felice was a good and sane boundary with my mother, and I had agreed. Although MaJe had passed away 2010, I kept this boundary, as I have learned that once you ease anything around my mother, the whole carefully kept relationship cracks. I had been mindfully putting up healthy boundaries ever since I had left home at 15 in an “I quit/you’re fired” fight over who should try to corral my then wayward sister. However, after my mother’s pneumonia and sudden relocation to the nursing home about 2 years ago, most of my boundaries had flown out the window. This single one I still kept in memory of my wife. Before her dementia, my mother had total control over her pain. You would not ever have had an inkling that her body creaked and moaned like an old haunted house, as she always put it. Now, however, she shrieks when anyone touches her shoulder to put her shirt on her, or puts her arm in a tight sleeve, or grabs her ankle or shin to put socks on or off, or moves her hip to get underwear on or to clean her. Each time I come, I show Felice’s bony bumps to the nurses, “see how this one would hurt? And this one, oh and that one, and this one?” Like picking up shells on an abandoned beach, I find more and more, not willing to stop once I’ve started the catalogue, overfilling the limited storage space in the nurses’ patience. The nurses would nod, and then forget. My mother would continue to shriek her pain. I record all the songs we sang when I was a child onto a fit clip, and the nurses put that in her ear to calm her. She croaks out “look for the silver lining, and then “happy trails to you,” whenever prompted by my voice in her ear. These words are the only words she truly remembers, and this crooning works to calm her--most of the time. And as my mother loses her ability to handle or communicate her pain, I invent a dress the nurses could put on over her shoulders and belt in place, which helps a bit at the end. I insist on sandals that went over just the few places in her foot and ankles without the bones, and that helps. I make marks on her knee and foot where the Reflex Sympathetic Dystrophy (RSD) is, noting not to ever touch her there if you do not want to be violently kicked. These marks wash off, and nurses just learn. Salida’s mountain town nursing home could not deal with full-blown dementia, of course. And they were afraid they’d have to relocate her. I showed the doctor the notes on another MHE patient and fronto-temporal dementia (FTD), so we now use this as a practical diagnosis, as a guide. FTD is treated much differently than regular Alzheimer dementia, as there is an emotional component and breakdown associated with FTD. I wonder though now, how much the pain colored these outbursts--as if a pain beast emerged from its concealed cave and took over after her conscious guarding took a break from reality. Research on frontotemporal dementia suggests that the mutation in EXT2 associated with MHE plays a role in the patter of neurodegeneration in this type of dementia (Narvid et al., 2009). This was based on a genetic, pathological, neuroimaging and cognitive study of a single individual, but symptoms seemed to be quite similar to Felice’s. The authors do not suggest that there may be an age component, but they do emphasize the degeneration and atrophy in the parts of the brain, which often are age-related. [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]]

<div class="felice"> <H2>(print: (passage:)'s name)</H2> Chosen Birth family choice not ours Neither Helen nor I sister-blessed The omission, to heal, we adopted each other Naturally following our long-lived history Sunday school mates Brooklyn NY Few decades later destiny drawn Denver CO party Helen discovered her long-lost school mate What an unplanned mystery Budding blooms flowered fast friendship bloomed Our seemingly infinite similarities continually emerging Attracting the same people as friends Our way of thinking Our lives positive open-minded philosophy Different viewpoints accepted appreciated Our differences negotiated and accepted Now Denver-bound Helen’s 90th Birthday Celebrations/ Years you’ve worn so well Accomplishments aplenty Mind, body, spirit strong Your age difficult to tell Helen always eager to know In adventure mode The road not taken Compassionate care-- a trait much to rare My elder sister’s lead I follow Working together in joy, peace, and love. </div><!--this closes the felice div> Felice Larsen [[Felices poetry]]

[[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] References on specific pages are listed at the bottom of that page. Deena keeps a more up to date reference list at <a href="http://www.tinyurl.com/MHETalk"; target="_blank">http://www.tinyurl.com/MHETalk</a> References Alvarez, C., S. Tredwell, M. DeVera, and M. Hayden. (2006) The genotype-phenotype correlation of hereditary multiple exostoses. Clinical Genetics, 70: 122-130. Aufderheide, A. C., C. Rodriguez-Martin (1998). The Cambridge encyclopedia of human paleopathology. Cambridge: Cambridge University Press. Bathin, T., S. Fredwell, U. Steen, and E. B. Svendby (2019). <A HREF="https://doi.org/10.1016/j.ijotn.2019.02.001">Fatigue and pain in children and adults with multiple osteochondromas in Norway</A>, a cross-sectional study. International Journal of Orthopaedic and Trauma Nursing Volume 34, August 2019, Pages 28-35. Benoist-Lasselina, C., E. de Margerieb, L. Gibbsa, S. Cormierc, C. Silve, G. Nicolas, M. LeMerrera, J. Mallete, A. Munnicha, J. Bonaventurea, L. Zylberbergb, L. Legeai-Malleta (2005). <A HREF="http://www.thebonejournal.com/article/S8756-3282(05)00540-5/fulltext">Defective chondrocyte proliferation and differentiation in osteochondromas of MHE patients</A>. Bone, 39 (1): 17–26. Bovée JV (2008). <A HREF="https://www.ncbi.nlm.nih.gov/pubmed/18271966">Multiple osteochondromas</A>. Orphanet J Rare Dis. 13;3:3. doi: 10.1186/1750-1172-3-3. Brown, Anissa Joy. Function of heparan sulfate proteoglycans (HSPGs) and heparanase (HPSE) in endochondral bone formation University of Delaware, ProQuest Dissertations Publishing, 2008. 3324491. Buikstra JE. (2019). Ortner’s identification of pathological conditions in human skeletal remains, 3rd edn. Amsterdam, Netherlands: Elsevier Inc., Academic Press. Fumitoshi I., H. Badie-Mahdavi, Y. Yamaguchi (2012). <A HREF="http://www.pnas.org/content/109/13/5052.short.">Autism-like socio-communicative deficits and stereotypies in mice lacking heparan sulfate.</A> PNAS 2012 109 (13) 5052-5056 Germeyer, A. M.S. Klinkert, A-G Huppertz, S. Clausemeyer, R.M. Popvici, T. Strowitzki, M. von Wolff, (2007). <A HREF="https://www.fertstert.org/article/S0015-0282(06)04007-6/fulltext">Expression of syndecans, cell–cell interaction regulating heparan sulfate proteoglycans, within the human endometrium and their regulation throughout the menstrual cycle.</A>. Fertility and Sterility, 87, (3):657 - 663. Glockenberg, A., E Sobel, and SJ Levitz (1997) . <A HREF="https://doi.org/10.7547/87507315-87-2-70.">Multiple hereditary osteochondromatosis. Two cases with ankle and knee deformities</A>. Journal of the American Podiatric Medical Association 87 (2) :70-73 Haeger, S.M., Y. Yang, and E.P. Schmid (2016). <A HREF="https://doi.org/10.1165/rcmb.2016-0043TR">Heparan Sulfate in the Developing, Healthy, and Injured Lung. American Journal of Respiratory Cell and Molecular Biology 55 (1) Malagón, V., 2001. <A HREF="https://journals.lww.com/pedorthopaedics/Abstract/2001/03000/Development_of_Hip_Dysplasia_in_Hereditary.14.aspx.">Development of Hip Dysplasia in Hereditary Multiple Exostosis</A>. Journal of Pediatric Orthopaedics, 21(2): 205-211.</A> Mansouri, R., Jouan, Y., Hay, E. et al. <A HREF="https://doi.org/10.1038/cddis.2017.287">Osteoblastic heparan sulfate glycosaminoglycans control bone remodeling by regulating Wnt signaling and the crosstalk between bone surface and marrow cells</A>. Cell Death Dis 8, e2902 (2017). McKensize, C., 2008. An overview of the paleopathological analyses of the medieval human remains from Ballyhanna, County, Donegal. In Sullivan, J. and M. Stanley (eds), Roads, Rediscovery, and Research: Proceeding of a Public Seminar on Archaeological Discoveries on National Road Scemes, August 2007 – Archaeology and the National Roads Authority Monograph No. 5. Waterstones: London. Mohammedi M., S.K. Olsen, and R. Goetz, 2005. A protein canyon in the FGF–FGF receptor dimer selects from an à la carte menu of heparan sulfate motifs. Current Opinion in Structural Biology Volume 15, Issue 5, October 2005, Pages 506-516. <A HREF="https://doi.org/10.1016/j.sbi.2005.09.002 DID not use. Multiple Hereditary Exostoses Research Foundation. 2020. About MHE. Retrieved from <A HREF="https://www.mherf.org/about-mhe. Narvid, J, ML Gorno-Tempini, A Slavotinek, SJ DeArmond, YH Cha, BL Miller and K. Rankin (2009). Of brain and bone: The unusual case of Dr. A. Neurocase, 15(3), 190-205, DOI: 10.1080/13554790802632967. Nozawa, S. T. Inubushi, F. Irie, et al. 2018. Osteoblastic heparan sulfate regulates osteoprotegerin function and bone mass. JCI Insight. Feb 8; 3(3): e89624. <A HREF="doi: 10.1172/jci.insight.89624. Park, P.J. and Shukla, D., 2013. Role of heparan sulfate in ocular diseases. Experimental Eye Research, Volume 110, May 2013, Pages 1 - 9. <A HREF="https://www.sciencedirect.com/science/article/pii/S0014483513000274 Russell AL, and MF McCarty (2000) Glucosamine for migraine prophylaxis? Medical Hypotheses 55 (3): 195-198. <A HREF="http://www.sciencedirect.com/science/article/pii/S0306987799910125. Sarrazin S, WC Lamanna, and JD Esko, 2011. Heparan sulfate proteoglycans. Cold Spring Harb Perspect Biol. 2011;3(7): a004952. Published 2011 Jul 1. doi:10.1101/cshperspect.a004952 <A HREF="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3119907/ Stitzman Wengrowicz, M.L., J Pretell-Mazzini, J.P. Dormans, R.S. Davidson, 2011.. Regression of a Sessile Osteochondroma: A Case Study and Review of the Literature. Staal HM, Witlox AMA, Mooij DT, Emans PJ, Ham JSJ, et al. (2014) Current Knowledge on Exostoses Formation in Hereditary Multiple Exostoses: Where do Exostoses Originate and in What Way is their Growth Regulated? Hereditary Genet 3: 134. <A HREF="Doi: 10.4172/2161-1041.1000134. Stiever, JR., and J.P. Dormans (2005). Manifestations of hereditary multiple exostoses. Journal of the American Academy of Orthopaedic Surgeons, 13: 110-120. The Back Pain Authority. (2020). Naturally Fused Vertebrae. Retrieved from <A HREF="https://www.cure-back-pain.org/naturally-fused-vertebrae.html. Theoharides TC, R Letourneau, P Patra, L. Hesse, X Pang, W Boucher, C Mompoint, and B Harrington. (1999) Stress-induced rat intestinal mast cell intragranular activation and inhibitory effect of sulfated proteoglycans. Digestive Diseases and Sciences: 44(8 Suppl):87S-93S]. <A HREF="http://europepmc.org/abstract/med/10490045 Thompson, W.R. 2011. Perlecan modulates the function of the osteocyte lacuno-canalicular system. University of Delaware, ProQuest Dissertations Publishing, 2011. 3443246 Twersky, J, EG Kassner, MS Tenner, and A Cameram (1975). Vertebral and Costal Osteochondromas Causing Spinal Cord Compression. American Journal of Roentgenology: 124: 124-128. 10.2214/ajr.124.1.124. U.S. National Library of Medicine. 2020. EXT1 Gene. Genetics Home Reference, U.S. National Library of Medicine. Retrieved from <A HREF="https://ghr.nlm.nih.gov/gene/EXT1#:~:text=The%20EXT1%20gene%20provides%20instructions%20for%20producing%20a,to%20form%20a%20complex%20that%20modifies%20heparan%20sulfate. Vinstein, AL and FA Franken, 1971. Hereditary Multiple Exostoses: A Report of a Case with Spinal Cord Compression. American Journal of Roentgenology, American Roentgen Ray Society: 112 (2) <A HREF=" https://www.ajronline.org/doi/pdf/10.2214/ajr.112.2.405. Waldron, T. 2009. Paleopathology. Cambridge Manuals in Archaeology. Cambridge University Press: Cambridge. White TD, Black MT, Folkens PA. 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We are climbing down the stairs after the appointment now. My mother says elevators are only for people who want to end up in wheelchairs, a fate worse than death in her eyes. My mother’s knee and hip refuse to move on some stairs and she presses her lips together and hooks her arm under her leg, moving it down as she holds the banister with her other hand. We rest on the second-floor landing. I want my skeleton right here, my mother says. Or in a medical school staircase. I want a big sign on it staying I told you it hurt. This becomes my life-long dream too.

Donating The Forensic Investigation Research Station (FIRS), operated by Colorado Mesa University (CMU), is one of a handful of facilities in the world, nicknamed “body farms” that use human remains to study how bodies decay. While that is the primary purpose, they also clean and curate the donor skeletons, providing a sample of skeletons from modern populations for research and education. The FIRS is in western Colorado. At approximately 4600 feet above sea level, it is the second highest such facility in altitude in the world; the highest being TB40 in Park County Colorado, also operated by Colorado Mesa University. Both facilities are in an arid climate, and bodies tend to mummify. The FIRS is a research hub, and associated professionals and students have publications on decomposition, scavenging, microbiology of death, and other subjects. The FIRS personnel hold classes and training for CMU students, but also train professionals, law enforcement, and death investigators in how bodies decay, osteology, and telling the difference between human and non-human bone (the location of deer skeletons have been known to be held as crime scenes for an hour or two until law enforcement can reach an “expert”). In addition, the FIRS holds annual cadaver dog training, helping dog handlers who work with their animals to locate human remains. None of this could be possible without individuals donating their bodies. Donors come to the FIRS and TB40 by two means. Individuals can donate themselves while they are still alive, in much the same way that individuals can designate their organs can be donated. Relatives can donate deceased individuals when they are the legal next-of-kin. The FIRS currently has over 100 individuals that have signed up to come to the FIRS on their death, but many of the donations are calls from relatives near or at the time of death of the donor. Why do people donate their bodies or their relatives? One reason is fiscal; dying is an expensive business. A cremation in 2020 is likely to cost about $1,000, and that comes with an inexpensive plastic box as an “urn”. Needless to say, nicer urns exist, and a family can spend as much money as they wish on an urn. Extras such as a memorial service, obituary, and copies of the death certificates add to the cost. An unexpected cost of $1,000-plus is significant for many. Others respect and support the educational goals of the facility. The younger donations are in their 30s, generally the result of drug overdose or suicide. Families have donated to help make our students more aware of the issues of addiction and suicide, and as a way to open the door for those conversations. Skeletons tell the life history of an individual in ways that sometimes even the individuals are unaware of. Each is unique and reflect the individual’s activities, injuries, and aging processes. The students consider the unveiling of a skeleton almost like opening presents - you never know what it will be like until you see it. While donors are asked for an outline of their medical histories, it is not unusual to find healed fractures that individuals either did not know existed—or didn’t think were significant to report.

Deena February 14, 2018 So after Dr. Connor agrees to take Felice drive to Salida and then drive my mother on her final journey over the mountain passes she loved from Salida to Grand Junction, Colorado, singing all the songs she loved along the way. One of my mother’s friends, Wayne, drives a white van in front of me as a convoy just in case I am stopped with a body in the back of my car. The windows are wide open in the cold winter heights of the passes over Leadville, and my hands are stiff beyond numbness in the frigid air, my lungs dimly understanding what wives in the covered wagons went through. We stop for hot soup in Minturn, and we watch breathlessly as a policeman casually looks at my car and moves on. My car is decked out in rubber ducks, as it is the only model of car that fits my often-dislocated shoulder, hip, knee, or arm perfectly--so I cannot risk it being stolen. Pirate and party ducks are strategically arranged so that I can grab on and move through my life even when one or the other of my limbs suddenly decides to take a few minutes or a few days off. But more than that, the ducks remind me and everyone else to laugh a bit, to enjoy what life we have. I get a kick out of making kids smile and adults waving, knowing I have lifted their day.

We agree, and I drive to Salida and then drive my mother on her final journey over the mountain passes she loved from Salida to Grand Junction, Colorado, singing all the songs she loved along the way. One of my mother’s friends, Wayne, drives a white van in front of me as a convoy just in case I am stopped with a body in the back of my car. The windows are wide open in the cold winter heights of the passes over Leadville, and my hands are stiff beyond numbness in the frigid air, my lungs dimly understanding what wives in the covered wagons went through. We stop for hot soup in Minturn, and we watch breathlessly as a policeman casually looks at my car and moves on. My car is decked out in rubber ducks, as it is the only model of car that fits my often-dislocated shoulder, hip, knee, or arm perfectly--so I cannot risk it being stolen. Pirate and party ducks are strategically arranged so that I can grab on and move through my life even when one or the other of my limbs suddenly decides to take a few minutes or a few days off. But more than that, the ducks remind me and everyone else to laugh a bit, to enjoy what life we have. I get a kick out of making kids smile and adults waving, knowing I have lifted their day. The staff and I all sing “Happy Trails to You” when we arrive. My mother’s songs were always full of joy—"Oh, What A Beautiful Morning”, “Look for the Silver Lining”, and of course “Happy Trails” …It’s the way you ride the trail that counts, here is a happy one for you, and just sing a song and we will bring the happy weather….” Keep smiling, truly feel the smiles down into your bones. I grew up thinking that every family sang” Happy Trails to You” when they said their goodbyes. I leave, knowing she is in good hands, and that this is precisely what she wanted.

Deena 1February 13, 2018 I’ve been at my mother’s death bed in Salida, Colorado for a couple of weeks, watching her light flicker, sometimes bursting into brilliant lucidity when she calls my sister and we reminisce about past adventures, and sometimes dimming in the howling winds of pain, when I soothe her as best I can. As she loses her spirit to dementia over the past few years, her iron-clad control over her life-long pain has slowly diminished. I begin to see how much control she has had all her life over her pain. I am grateful that she taught me how to love life, despite my own pain from this same disease. I have to go back to Denver to work and I anxiously await the news over the next few days. I get the call the morning after she has passed away in the night. Her first choice for donating her body to research is not taking body donations at that time. It is 8 am and I have until 10 am to find a new solution that would benefit from my mother’s body, or the lawyers will start the cremation process. I search frantically through the internet, and FIRS catches my eye: “The skeletal remains are a very important component to our research and teaching program.” Well, I whoop! That is just the place for us! My mother was proud of her odd bones, and she made her bumps her crowning feature. So I call up Dr. Connor: “So how would you like to be the first research station to work on a modern skeleton with multiple hereditary exostoses (MHE)?”

Deena Larsen called CMU in February 2018 to ask if she might donate her mother’s body in accordance with her mother’s last wishes. Her mother had passed away that morning, and previous arrangements could not be followed. Dr. Connor had never heard of MHE and was googling the condition while also calling the deputy coroner to ensure that this was not considered a coroner case and that Felice’s body could be released. The State of Colorado allows transport of human remains within the state in a “safe and sanitary” manner, generally considered a body bag or sheet. Remains must also be refrigerated or embalmed within 72 hours. Generally, the FIRS does not encourage families to transport their own relatives, but it has happened occasionally; always when the Director has touched base with the coroner or hospice nurse to ensure they are aware of the death and to discuss who will fill out the medical side of the death certificate. Once the coroner cleared the paperwork, the Director talked to Deena about transport. An arrival time was arranged, and two interns were on hand to assist in moving the body from the car to the gurney to the cooler. A hatchback pulled into the driveway at the FIRS and as the Director waved it into the delivery area, they noted the yellow rubber ducks attached around the edge of the top of the car. Deena Larsen got out of the driver’s side and, using the rubber ducks as support, worked her way to the back of the car and opened the back. Inside was a blue body bag and Deena’s wheelchair. The bag was light and the interns, Alex and Rachel, are easily able to move it onto the gurney and strap it in. The FIRS interns work with dead bodies, that’s what they expect to do when they come to the FIRS. Working with recently bereaved families is not within the usual scope or work for interns; the Director usually does that. Alex and Rachel are both obviously nervous about acting “wrongly” in front of family. Neither have ever had a death in their immediate family and their experience in grief is limited. The rubber ducks also seem to have them puzzled. Once the body was on the gurney, Deena said that one of her mother’s favorite songs was “Happy Trails to You”’. She passed out sheet music and asked the FIRS staff to sing along with her. The Director couldn’t carry a tune in a bucket, and neither Rachel nor Alex had ever heard of the song (Rachel was an exchange student from England not familiar with American country music; Alex had just never heard of it). But they make what must have been a passable rendition. After Deena leaves, Connor explained who Roy Rogers and Dale Evans were. Alex and Rachel take the donor to the cooler room and complete the check-in process. Deena gets the wheelchair out and comes into the lab with Connor. Deena already has a vision for what this volume will be and wants to use the remains to educate those that have the disease and medical practitioners about the impact of the condition on those that have it.

A year later, I am again at the FIRS in Grand Junction, looking out at the dry mesquite, the bone-bare beige cliffs in the distance as the shadows move slowly across them. As we enter, I tell Dr. Connor that I only want one thing—to validate my whole existence. Doctors have told my mother and me that there was probably no pain as bone tissue does not have nerve endings, so any tiny twinges we felt were probably just stress. I need to know: would my mother have been in pain for a reason—or was our pain simply imaginary? Dr. Connor assures me that no, indeed, every bone in my mother’s body played host to large bumps, too big to be called bone spurs. These bony structures that continued to grow after adulthood pushed aside tendons and pressed on nerves, so yes, there would definitely be pain. We decide to relate these FIRS findings—not in the usual dry scholastic fashion—but to tell the stories to underlie our main message: to believe the patient, to be open and creative about finding solutions, and to forge links between the patient and the research, the dry bones of the academics and the true flesh of the story. I, too, have the bumps, so I can take up the reins of our story—having lived my life as a continuation of this dance between pain and frustration, joy and living, fatigue and determination.

A life goes far beyond the calcium deposits, the bone fragments. And in medicine, in life, it is important to see the whole picture. [[Deenas History with Felice]] [[Felice's Father|Oscar Winkler]] [[Down the Stairs]] [[Felice's poetry|Felices poetry]] [[Felice's memories of her father|Felices father]] Deena's memories fused with [[Felice's skeleton|Skeleton key]]

EXT1 is involved in the manufacture of a complex of sugar molecules, heparan sulfate, which is involved in regulating a variety of processes including blood clotting and the formation of blood vessels. Heparan sulfate is a fairly ubiquitous substance throughout the body and plays a role in many disorders, but its role in each varies (Park and Shulka 2013) and only with recent research is there a beginning of an understanding of the widespread impact of a heparan sulfate defect on the body, and mind. Overall, heparan sulfate changes can have profound effects at the cellular, tissue and organismal level (Sarrazin et al. 2011), dentition (Wisweger et al 2012), and breast and ovarian cancer (Yoneda et al. 2011). The difference in heparan-sulfate synthesis with the mutations in the EXT1 and EXT2 genes changes how the bones are formed significantly enough to promotes oddly formed bones, usually through extra growth on the bone (Benoist-Lasselina et al. 2005; Staal et al. 2014). The result being bones that are functional, but not as strong as in individuals without this mutation and are shaped in the same fashion as bones without this, but with extra growths on them. Heparan-sulphate appears to help regulate bone mass (Nozawa 2018), and the result is reduced bone mass in individuals with this mutation.

<div class="mhe"> <h3>(print: (passage:)'s name)</h3> MHE results from a mutation in the EXT1 and EXT2 genes (cf Busse et al. 2014). MHE patients have defective Heparan Sulfate ProteoGlycan (HSPG) biosynthesis--their bodies do not produce HSPG. In addition to causing abnormal [[bone growths|MHE and Bone Formation]], the HSPG lack causes other problems. Deena has been collecting abstracts from scholarly articles dealing with the implications of the lack of HSPG as a form of medical defense. (link:"MHE list")[(gotoURL:"https://docs.google.com/document/d/1XLaJ84ClR6CH9A70alF00ttxrELogm2o20nKyETlzPI/edit";)] [[Biochemistry]] [[Continued Growth]] [[Migraines]] [[Dementia]] [[Autism Spectrum]] </div>

"//All happy families are alike; each unhappy family is unhappy in its own way."// Tolstoy, Anna Karenina Yes, I nod. And at least my family is a little easier to follow than Anna's there. I hope. I sit here, screens blinking at me, waiting for my explanations. Wondering where to start, as usual; what to include, what to slide over. Because ultimately, this is a story about my mother's bones and not her psyche, her spirit that inhabited them for not quite a century. But not saying anything about that life, that spirit, lifts this story away from any context. And is no more possible than lifting each molecule of calcium out of [[my mother's bones|Skeleton key]]. I have a few fragments of her writing, and I as editor can pick and choose. The story belongs to those who tell it, so do remember that you are not seeing her side of this any more. Only mine. My mother married my father in Spring 1963 for what might have been a "shotgun wedding" or might have simply been her caving to the societal pressure. She had a miscarraige in the fall of that year, and I was born in the Spring of 1964. Before her marraige, Felice had graduated with a BS. from the University of Wisconsin, a Masters Degree, and worked with patients at Pueblo State Hospital. The marriage was an unhappy one (which of course, does translate into my childhood memories of hiding with my little sister in a cubbyhole we had created in the living room couch while they yelled, screamed, threw things, etc.). The family split: me with Felice and my sister, Shanna, with Don, our father, and his new wife and family. We swapped places occasionally and finally, Shanna also stayed with Felice starting in 1978 after Don had kicked her out of his house for various offenses. Felice married Jerry in the late 1970s. There was rising animosity that culminated in an "I quit/You are fired!" situation where I left home in my sophomore year in the winter of 1979 at 15. This sounds clinical--if not cynical--now. But it had been for the best, as I was able to get myself through the rest of high school and college on my own. I still kept in touch with Felice and we would meet for awkward dinners at [[cheap restaurant|Spine: Sitting Fused]]--not really mentioning our respective health, not really avoiding the subject, either. From then on, I would meet with Felice on neutral territory. We would still go [[hiking|Hands: Holding]], still see the leaves together in the fall. For the rest of her life, I would continue to counsel her on what to do with her life, her finances, her decisions. As with many relationships, we were much closer when we were apart from each other. I graduated from the University of Northern Colorado in 1986, went to San Francisco and then Japan, then settled back into the University of Colorado in 1990 and got my MA in English. I stayed in Denver, working a stable government job as a technical writer and writing electronic literature on the side. (Health insurance and a sedentary job were the big motivating factors in my career choice). In the early 80s, Felice had moved from Denver to live in Santa Fe with Jerry for a decade or so, and then they both settled in Salida (in the Colorado Rockies, a 3 hour drive from Denver if you are not behind a truck, and a 4 or 5 hour drive if you are). Jerry passed away in 2008, and Felice stayed in Salida for the rest of her life. I rarely visited. We did talk about [[bones|Trauma]], as we were each other's only confidantes, the only ones we knew with this disease. In late 2014, I got a call from one of Felice's group of friends, the ones that banded together as "Felice's Angels." Felice had gotten pneumonia and her whole house of cards fell apart. She was sent home on a Friday in a small town with instructions to get oxygen--but the weekend wait (as the oxygen place was closed) permanently damaged her, and she could no longer function in her home alone. I came up, got her into the only nursing home in Salida and helped transition her to a new life there. I would come up as often as I could--usually monthly--to advocate for her and fight for her comfort. Gradually, she grew more and more into her [[senility|Dementia]] and less and less into herself. She [[passed|Delivering Felice]] in January 2018.

My sister, Shanna, had wondered as well why I always called my mother by her first name, Felice. Well, first of all, it was the 60s. It was a fad then to instruct your children to address you by your first name--to show you were hip parents that understood that children were simply little adults longing to be free. Shanna never did use those terms. It was mostly a darker mutter of "Her" or a nod. Or occasionally, when there had been a rapproachment, a truce between us all, Shanna would say "Mother." I very rarely said that. [[Memories]]

NEED THE NECK XRAY ** ''Deena’s Recollections Multiple Years'' Felice would always stretch as far as she could go, moving her neck from side to side. But this was never very far. She would turn her entire body to see over her shoulder. She had always had extra mirrors in the car so that she could see cars on either side without turning her neck. I have always done the same. Her range of motion was always truncated--never nearly as far as any other dancer could go. When Felice taught me to swim, she used a side stroke. Neither of us can manage the arm overhead, the turning of the neck needed for a breast stroke. But, my mother said, a side stroke will get you where you need to be . ''1990s'' After our respective [[car accidents|Trauma]] a few months apart, Felice and I would joke with each other--a ritualistic, private knock-knock joke: "Hey doc, it hurts when I go like this:" and we'd tilt our heads or bob chins slightly forward or back or try to look side to side. But of course this hurt like hell for both of us and we did not get very far. "So don't go like that!" the other person would answer, and we would both laugh wryly, [[Ribs:Bad Guy]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]]

<img src="./img/knee.tif" alt=a normal knee contrasted with Felice, where there is bone groth on the actual ball.> <img src="./img/knee2.png" alt=a normal knee contrasted with Felice, where there is bone groth on the actual ball.> An individual may have surgery to remove one of the tumors, but additional growths may occur. The bone responds in a variety of ways to this insult, but Fig xa shows a typical response with overgrowth and macroporousity, rather than healing within the normal template of the bone shape and texture. Figure xb shows some of the areas where growths occurred after Felice had joint replacements. <img src="./img/hip1.png" alt=a pelvis showing bone overtaking a plastic insert.> Fig. xa. The top portion of Felice’s right thighbone (proximal femur), showing hip replacement. B. Comparative plastic skeleton showing shape without the hip replacement. Comparing, the texture of the bone around the replacement with the texture of the shaft, the portion of the bone most insulted by the surgery for the replacement appears to have responded by overgrowing the normal bone template and becoming very porous. Some researchers suggest that the number of exostoses increase until about 12 or puberty (cf Waldron 2009: 202), and then lessen or stop. Bovee (2008) suggests the mean number of tumor locations is 15-18 and are asymptomatic. This is in contrast to the study by Bathen and others (2019) showing the vast number of people with MHE suffer from chronic pain. Here, Deena’s recollections strongly side with Bathen and others. Bovee (2008) also states that the tumors cease to grow when the growth plates close at puberty. However, with Felice, they appear to continue to increase, particularly when the bone is insulted, as during a joint replacement (See Fig. xc). [[MHE and Bone Formation]]

Oscar Winkler, Felice's father, had MHE. I never knew him, as he passed away suddenly on his way home from a doctor's visit. I do not know if it was the same visit as this well preserved note. <img src="./img/oscar.jpg" alt=a collage of photos of Oscar Winkler from the early 1900s withclose ups showing deformed writs and knees.> ''Biographical information'' Oscar Winkler was born in 1884 and passed away in the late 1940s. ''Family Legends'' Whenever Felice spoke of her father, she got a distant look in her eye. Her father was an accountant, a financier who lead an active social life and very involved in his Jewish community. She would usually talk about him when we were both hurtnig or when I complained of pain. Her father firmly believed that pain was a sign of weakness and should be hidden from the world. There are three stories I remember most. //''1904''// My grandmother lived until 1982 and told me this story when I was a child. My grandmother was born in 1899 and was five when she first met Oscar, my grandfather, who was 15 at the time. She had a sister, Ella, who was older by 13 years, and Oscar was tagging along with his older brother who was courting Ella, along with a troupe of other boys. Mae snuck under the table and tied everyone's shoes together. Oscar saw the gleam of mischief and started to send Mae books. I have some of these, like the copy of Alice in Wonderland, inscribed "To Mae on her seventh birthday from her Ocar, who looks forward to discussing these foibles together." //''1926''// Oscar was a financier, who was a bit ahead of his time. He had written a piece for an affluent magazine like the Atlantic (I have never been able to find the article, but then I have not really tried). In this, he argued against buying stocks on credit and predicted the fall of teh stock market. He took his money out of stocks that year, completely missing the exuberent highs of the next 3 years, but also missing the crash. Felice recalls those years where she was thrilled to live in a brownstone in Brooklyn. Her mother kept a pot of stew and sandwiches, and would offer food to anyone who showed up on their doorstep. //''1930s''// Both my grandparents' parents were from a small shtetl near Pécs, Hungary, and both my grandparents were born in the U.S. However, they remained close in this community, speaking a Hungarian yiddish. He had read Mein Kampf and kept up with articles in European presses. As he had not lost his fortune when most others around him had, Oscar had the wealth needed to bring over many of the people in this Jewish community in Pécs. However, he did so in the late 1920s and early 1930s, when the dangers of World War II were still far distant. So many people went back to the land they knew and loved. Of the //''1930s''// I was stomping through the house or making noise, and my mother snapped at me saying that children need to learn to be quiet. Her mother, Mae, had had grand mal epilepsy in a time when no one knew what to do for seizures. And her father, Felice said, would always caution her not to make a sound, for fear of causing one of those seizures. And so my mother would be as silent as possible, and would desperately comb through her memories and actions when her mother had a seizure, as she was convinced that every seizure was something she had done. ''Medical information'' A doctor's note from October 11, 1946 notes several health problems: To Whom It May Concern: Mr. Oscar Winkler has been under my observation for almost a year. When I first met him, he was in a state of marked congestive failure and was treated with mercurials and digitalis. He is still on a maintenance dose of Digitalin Natiel, 0.2. mgm od five days o, two days off. He is also on a salt-free diet. Mr. Winkler is a diabetic of old standing. He is taking Protamine Zinc and regular insulin. The dosage is known to himm. He is to modify the dosage in accordance with the urinary findings. About five months ago, he underwent a two-stage opeation for a tumor of the descending colon. About six weeks ago, he had a carbuncle on the anterior surface of the upper abdomen and also osetomyelitis of the left thumb. Both of these conditions are at present under control. He is taking Thera-Vita (Warner) once daily. Mr. Winkler has a tendency to a slight tachycardia. The blood pressure readings lately have been in the neighborhood of 130/70. Fluoroscopic examination shows only a plus, minus enlargement of the heart. Ther is also a moderate anemia (hemoglobin 11.0 grams). Very Truly Yours THEODORE T. FOX M.D.

Rollanda Ramber Felice Larsen January 2, 2009. (align:"=><=")+(box:"X=")[ My vehicle my faithful companion correct ecology no fuel required foot powered my two feet and your four wheels ... Down Salida's path smoth sailing we race your wheels purring hum like hummingbirds' wings . . . . My walker and I in tandem a pair Our unique attributes in unity together in harmonic synchrony problem solvers obstacles overcome]

This is a snippet of a letter my mother wrote me in 2006 or so, two years after I had collapsed into a wheelchair, I had written to Felice detailing my recent medical horrors and explaining that ignoring problems as I had ignored my leg pains until I ended up in a wheelchair is not a good idea. I was still trying to convince her to use a [[walker|In Praise Of My Walker]] I had not written accusingly. But my mother took it as an accusation that I blamed her for being born, for passing on this disease. We had had this talk many times, but still, each time she saw me, she would beg my forgiveness and say that she was sorry I had been born. I, myself, had no children, so I do not know if I would have felt the same amount of guilt. I think not, but then, you never know. "I realize and relate to yur anger at me, as I have experienced my anger toward my fahter. I know he did not know his condition was hereditary. However, I did know. I did not marry until my late 40s. Don did want his child so we decided to take the 50-50 chance. We lost, but with you won in so many other ways. I have already told you this. I do hope you will find forgiveness as I have for my father. You are my daughter who I love greatly. I love you, Felice.

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/c1a.jpg" alt=""> <img src="./img/c1b.jpg" alt=""> ''C1: Broken neck''//At some point in her life, Felice [[broke her C1|Trauma]] and it never healed correctly.// The figure above shows an antemortem anterior-posterior non-union fracture of C1 directly left of the midline. Remodeling of the fracture interfaces is complete. Eburnation is present on the inferior surface of lamina where a pseudoarthrosis has formed with the dorsal spinous process of C2. The anterodorsal surface (including the articular facet for dens) is structurally compromised, resulting in complete loss of the joint surface and underlying trabeculae, with subsequent deposition of woven bone mechanically smoothed by dens. The inferior articular facets present circumferential deposition of bone, scoring, pitting, and densification consistent with osteoarthritis. ''C2: Modified''//The bone modified to accommodate the break in C1.// C2 presents posterior displacement of dens with subsequent pseudoarthrosis formation on the superoanterior surface. A pseudoarthrosis has formed on the superior surface of the dorsal spinous process, osseous deposition has resulted in generalized expansion of the dorsal spinous process presenting a distinct joint surface characterized by densification, pitting, and new bone deposition. The superior articular facets present densification, scoring, eburnation, and pitting. The posterolateral aspect of the right superior articular facet has resulted in loss of the lateral border of the transverse foramen. A pseudoarthrosis has formed posterior to the anatomical joint surface on lamina. The inferior articular facets are posteriorly displaced by pseudoarthrotic joint formation. Osteoarthritic changes to the joint surfaces is characterized by scoring, macroporosity, eburnation, and peripheral deposition of bone. ''C3: Compression fracture''//The bone compressed due to weakness// C3 presents compression fractures between the superior and inferior articular facets. Osteoarthritic changes to the joint surfaces are characterized by scoring, macroporosity, eburnation, and peripheral deposition of bone. ''C4: Modified''//The bone modified to accommodate the break in C1.// C4 presents complementary changes to the superior articular facets to accommodate the proliferative deposition observed in C3. Dramatic peripheral deposition resulting in the ventral extension of the joint contour with underlying fortification of the pedicle. The dorsal aspect of the joint surface is characterized by expansion of the joint surface and a ridge of new bone formation to accommodate the posterior tilt of the overlying vertebrae. The inferior articular facets are characterized by marginal expansion of both joint surfaces with densification and microporosity. The right inferior facet presents dramatic poster-lateral and lateral overgrowth with concomitant formation of pseudo arthrosis. Complimentary osseous change is presented in the superior articular facets of C5. The bodies and the right articular facets of C5 and C6 are fused. The superior facets of C5 present peripheral circumferential deposition of new bone to accommodate the pseudoarthrosis presented by C4. Fusion of the right facet surfaces of C5 and C6 is characterized by reactive bone that extends to the pedicle. Circumferential osseous buttressing is present on the bodies of C5 and C6 resulting in complete paravertebral bridging. The inferior centrum of C6 presents macroporosities and marginal osteophyte formation that is more pronounced along the anterior border. The inferior articular facets present changes to the joint contour, scoring, and microporosity formation consistent with osteoarthritis. The superior surface of C7’s centrum presents diffuse macroporosity formation and circumferential osteophyte formation. Paravertebral bridging between C6 and C7 was in a state of active formation but incomplete. Both superior articular facets present new bone deposition on the joint surface and overall changes to the joint contour. The left superior facet presents ventral expansion that has resulted in the reorientation of the joint over the transverse process. The left inferior articular facet presents pseudoarthrotic expansion of the joint surface, resulting in the dorsal extension of the joint along the length of lamina, terminating at the root of the dorsal spinous process. The joint surface is characterized by microporosities; a plague of new bone deposition is present along the length of the joint surface. The right inferior articular facet presents posterior and lateral widening of the joint surface that is less dramatic than the changes presented on the left but pronounced.

We would have four major sections [[Felice's skeleton and memories|Skeleton key]] [[Multiple Hereditary Exostoses (MHE)|MHE and HSPG]] [[Family album|Memories]] [[Behind this book|How this book came about]] Each section can have a corresponding look and feel <img src="./img/Slide3.jpg"> would show the entrance for each bone part, giving a brief overview and then linking to memories/pathology report. We would have a gallery page that would show the bones and photographs (if any) corresponding to that bone part. <img src="./img/gallery-navigation.jpg">

<div class="bone"> <H2>(print: (passage:)'s name)</H2> <img src="./img/femur-left-right-anterior.jpg" alt="left and right femurs with views of exostoses".> Left and right femurs. Anterior (front) view. <img src="./img/femur-left-right-posterior.jpg" alt="left and right femurs with views of exostoses".> Left and right femurs. Posterior (back) view. <img src="./img/femur-left-implant.jpg" alt="left and right femurs with views of exostoses".> Close-up of the posterior of the proximal end of left femur (the rear of the thighbone where it meets the hip). Notice the amount of bone growth since the implant was placed. <img src="./img/right-implant.jpg" alt="left and right femurs with views of exostoses".> Close-up of the posterior of the proximal end of right femur (the rear of the thighbone where it meets the hip). Notice the amount of bone growth since the implant was placed. [[The bones grow back|Continued Growth]]. We keep saying this over and over again to every doctor. Of course the bones don't grow back, is their inevitable reply. Bones don't grow after childhood. But in our experience, this automatic response fails to recognize that the bone tumor growth response is faster when the bone is disturbed, such as in surgery. ''late 1930s'' My mother's [[knees|Knees and lower leg: Mr Silent]] were operated on when she was in her late teens, and she claimed that the bones grew back three fold. I had roughly the same experience [[Deenas knees]] with my own knee surgery when I was 18. ''2000s'' This bone growth had been one of her major concerns about getting the hip surgery in the first place. Her knee replacement a decade prior had gone relatively well, with just about as much pain as she had had before the surgery as after the surgery--but she could once again hike and waltz through Santa Fe and then Salida, those small artsy towns she loved so much. Her beloved surgeon told her the bones would definitely not grow back, that this was such a silly notion she should immediately dismiss it from her mind. Alien invasions would be more likely, she informed me smugly. I pursed my lips and said nothing. It was her decision. As for me, after [[my surgery|Deenas knees]], I vowed never to have surgery again. Every doctor, every PT, every single medical person that I manage to see twice knows that the S word is forbidden and I will walk out if I hear it. So my medical team and I avoid even saying the S word. ''2019'' I share these images with the facebook groups that have come together over this disease. Of course, growing up we thought we were completely unique and no one could possibly have this condition. The internet brought us all together as we shared stories and symptoms, like the odd social and nuerologicaltendencies we all had that later resulted in a genetic confirmation that yes, [[MHE has an autism-like aspect|Autism Spectrum]] as well as bone growth. . And now our tiny band of MHErs had a rallying point, some actual physical proof that we were not crazy. The bones grow back, we cry to the medical professionals. No, they don't, the medicos say. Yes, they do! And now we shove this picture in their face. Oh, they say. Hmmm.... [[Knees and lower leg: Mr Silent]] [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div>

<div class="mhe"> <h3>(print: (passage:)'s name)</h3> ''Deena's memories 2004'' After decades of microfracturing my legs, I finally collapsed into a wheelchair in 2004 and could no longer walk. Then I started to reach out on facebook and discovered a group of us--there were more MHErs than my mother and me. So we began to compare notes. And nearly all of us were called Aspies or Auties, that feeling of being a stranger in a strange land, unable to understand and bridge the gap of social interactions. Dr. Yu Yamaguchi studied this and developed lab rats with the genetic defect of MHE. I watched his mice in fascination: That is me!!! (https://www.youtube.com/watch?v=6-EXRt_YL6A&t=546s Minute 6:24) <img src="./img/mice-social.jpg" alt="two cages of mice, one normal with interaction and one with the EXT1-KO where the mouse hides in an atypical nest."> A screenshot of a 2012 lecture from Dr. Yu Yamaguchi showing autistic behavior in EXT knockout mice. ''References'' Fumitoshi Irie, Hedieh Badie-Mahdavi, and Yu Yamaguchi, 2012. Autism-like socio-communicative deficits and stereotypies in mice lacking heparan sulfate. PNAS 2012 109 (13) 5052-5056; March 27, 2012 vol. 109 no. 13 http://www.pnas.org/content/109/13/5052.short Irie, F., H. Badie-Mahdavi, Y. Yamaguchi, 2012. Autism-like socio-communicative deficits and stereotypies in mice lacking heparan sulfate Proc. Natl. Acad. Sci. U. S. A., 109 (2012), pp. 5052-5056. Kambouris , M., Fadda A, Al-Arraj, Y, et al., 2016. Putative Relation Between Autism Spectrum Disease & Hereditary Multiple Exostosis Investigated by Whole Genome Sequencing & Comparative Genome Analyses in a Family with ASD and HME with EXT-1 Mutations Kim, Min Jeong, Yunjin Lee, Sang Ook Nam, Young Mi Kim, 2021. An 8q24.11q24.13 Microdeletion Encompassing EXT1 in a Boy with Autistic Spectrum Disorder, Intellectual Disability, and Multiple Hereditary Exostoses. Annals of Child Neurology. Letter to the Editor, 11/9/2021. Li H, Yamagata T, Mori M, Momoi MY. 2002.Association of autism in two patients with hereditary multiple exostoses caused by novel deletion mutations of EXT1. J Hum Genet 2002;47:262-5. Pérez, C., Sawmiller, D. & Tan, J. The role of heparan sulfate deficiency in autistic phenotype: potential involvement of Slit/Robo/srGAPs-mediated dendritic spine formation. Neural Dev 11, 11 (2016). https://doi.org/10.1186/s13064-016-0066-x. Yamaguchi, Yu. Research on rare bone disorder reveals new insights into autism. https://www.eurekalert.org/news-releases/857331 March 2012, [[Skeleton key]] [[Memories]] [[MHE|MHE and Bone Formation]] [[Begin again|Title page]] </div>