Tag Archives: Achondroplasia

It’s Dwarfism Awareness Month and I’m Aware that Most of Us Don’t Understand Genetics and Medicine

8 Oct

Bones(Image by Gema used under CC 2.0 via)
 
 
“Hey, I had a test question about achondroplasia today!” an old roommate of mine reported to me a few years back. He was a medical student and had been studying for his licensing exam.

“Oh, yeah?” I asked. “What was the question?”

“Whether or not achondroplasia affects intelligence.”

“And the answer was…?” I smiled.

“The answer was no,” he replied, returning the smile.

That this was a test question implies a necessity for teaching this fact. Indeed, for a good part of modern history, children with achondroplasia and other types of dwarfism were too often assumed to be intellectually disabled and placed in institutions or special ed classes for life. Hence a meme from Little People of America that’s been floating around the Internet in honor of Dwarfism Awareness Month: “A common misconception about people with dwarfism is that they are cognitively delayed or mentally impaired. This is NOT true.” Activism will remain crucial until this misconception is no longer common.

What are the chances that it ever will be? Equality and empathy are the heart of every human rights movement, but these ideas alone did not disprove the idea that lower than average height is indicative of lower than average intelligence. Science did that. The scientific method uncovers the facts. Political activism spreads the word.

Despite what some extremist conservatives claim, science doesn’t have a liberal bias. It often reveals facts upsetting to many. For example, contrary to some Dwarfism Awareness campaigns, people with achondroplasia cannot be anything they want to be. They cannot be construction workers, gymnasts, military combatants, weight lifters, or participate in most contact sports because the achondroplastic spinal column is compressed, rendering these activities more life-threatening for us than for most people. That’s a scientific fact.

It need not be a cause for regret. I don’t consider a life without the ability to participate in contact sports or construction work any less enriching than a life without the ability to sing on key or identify bird calls or cut hair or write without spellcheck. (When I’m feeling snarky, I steal a line from this movie: “Dear White People on Instagram: You own an iPhone and you go on hikes. We get it.”) Competitive and aggressive feats of strength can be a way to be a stronger person, but they are not the only way. You can tell me a million times that I cannot safely lift anything heavier than a small child over my head and I will never take it as an insult.

What is insulting is to tout broad assumptions about conditions and bodies as facts when they have not been corroborated by several studies. Someone with dyslexia is unlikely to master a word scramble, but that does not mean she cannot be a skilled writer. Someone with Down Syndrome cannot practice medicine, but that does not mean he cannot get a job. If you hear that people with dwarfism cannot have a high IQ, teach, drive, play tennis, perform surgery, give birth, or take care of children, these are not facts. They are assumptions. Yet they have been dispersed far and wide, terrifying far too many people with dwarfism and their parents.

Having a rare genetic mutation has taught me that teaching science to the masses is hard. Most of us who are not scientists develop our understanding of medicine based on doctor’s visits, pop science news articles, and hearsay, as opposed to peer-reviewed research published in medical journals or textbooks. This is to be expected—when was the last time I read a medical journal straight through?—but it results in all sorts of inaccurate and potentially dangerous assumptions.

When I recently tried to explain to some new friends that the gene for achondroplasia is dominant, one insisted, “Achondroplasia can’t be dominant because then most people would be dwarfs!” Wrong.

When my parents visited one of their first Little People of America meetings shortly after I was born back in the early Eighties, one volunteer said, “Dwarfs don’t live as long as average-sized people do because they have to walk twice as many steps in their lifetime.” Also wrong. The most common cause of lower life expectancy among dwarfs throughout history has been a lack of access to appropriate health care due to social marginalization.

When a journalist asked the owner of a Hollywood freak show last year why one of his main performers had died at the age of 32, he replied, “A lot of them don’t have long life spans. Little hearts and the whole thing.” The reporter revealed in his excellent exposé of the depressing freak show business that the performer in question died of alcoholism.

My career as a writer has helped me see how much we love stories that are both out of the ordinary and easy to understand. My dwarfism has caused me to be confronted with the ubiquity of scientific misinformation in these stories and has helped me see how xenophobia facilitates the lazy thinking perpetuating scientific myths about minorities. Black Americans can’t swim? More like they were barred from learning how. Half of gay male teens have AIDS? File that one next to the Victorian belief that masturbation causes blindness. Women don’t have the skills to be Silicon Valley programmers? In Western cultures where men are expected to be bread-winners, women have been dissuaded from pursuing the highest-earning jobs, whether we’re talking about doctors and nurses, professors and school teachers, or milk men and milk maids.

This is why I approach most scientific and medical “facts” uttered to me with a heavy dose of skepticism. This can be draining. Some days I would like to simply trust Google or a Facebook Group for dwarfs instead of having to track down out-of-print medical textbooks or wait months for my orthopedist to have a free appointment in order to find it out if I should be concerned about osteoporosis or fibroids. But doubt is the fuel of innovation and vigorous research ultimately harms no one.

And when facing complex disabilities and learning about what certain bodies absolutely can and cannot do, we should not confuse being talented with being good. Just as it is hard for us to resist a fascinating story, it is hard for us to resist the idea that strength of body and mind also indicates strength of character. But acing any sort of competition says little about your ability to be brave, honest, generous or humble. Need proof? Celebrity scandals are but a Google search away.
 
 

Advertisement

Tags: , , , , ,

“Sometimes It’s Better to Deal with a Terminal Illness Than to Live with a Dwarf for the Rest of Your Life”

19 Mar

body(Image by Anthony Easton used under CC 2.0 via)

 

A Sydney woman has been declared fit to stand trial after being charged with murder for the 2010 death of her infant daughter. The judge has concluded that before the child died, the mother was “obsessed with perfection,” and was panicked that her daughter had achondroplasia, the most common type of dwarfism. She insisted that skin tags, a flat nose, and the shape of the baby’s forehead were proof of the condition, and subjected her to rigorous x-rays and genetic tests, which all came back negative. The Sydney Morning Herald reports: “When one friend got ‘fed up’ and told her she wasn’t dealing with something like cancer, the mother replied: ‘Sometimes it’s better to deal with a terminal illness than to live with a dwarf for the rest of your life.’ ”

That her daughter did not have achondroplasia is wholly irrelevant. Neglecting or harming a child on the basis of a bodily deformity she did or did not have is tragic no matter how you cut it. It sends two extra shivers down my back stemming from the fact that I have achondroplasia and would have a 50% chance of passing achondroplasia on to any children I were to have biologically. In several previous articles, I’ve examined the complicated issue of children with rare conditions and parents who lack the skills to give them the support they deserve. I am equally preoccupied with what it means for the child and what it means for the parent.

I’m not interested, however, in judging the accused woman personally because we can draw few accurate conclusions from the reports of her case. Many will argue that her schizophrenic disorder was the sole catalyst of her actions, while many experts on mental illness have tried to convince the hard-to-convince public that having schizophrenia does not make someone more likely to commit murder or manslaughter, and bigotry against achondroplasia is certainly not a symptom of the illness. Schizophrenic disorders are complex, and armchair diagnosis is a dangerous game far too many of us like to play. The temptation is best left resisted.

But it is safe to say that the likelihood of incidents like these would dramatically decline if our society saw nothing wrong with looking like a dwarf. Humans have a long history of parents abandoning or murdering deformed or disabled children. It goes as far back as Ancient Sparta and was codified into law here in Germany under the Nazi regime. And even in cultures where disabled or deformed citizens have generally not had to fear a death sentence, being humiliated or abandoned for having a certain body type is horrid enough. Firm belief in bodily hierarchy can be found in countless corners of modern society, from the glossy pages of lifestyle magazines, to Nobel Prize winner James Watson’s lectures on inherent attractiveness, to capitalist icon Ayn Rand’s arguments about who should be considered subnormal. 

Yet while the long history of ableism and lookism may be a daunting fact, it is also a fact that fashion is constantly in flux. Humanity’s habit of relentlessly coming up with new ideas for how bodies should look is a cause for hope. Not because a woman with achondroplasia winning a beauty pageant could ensure our universal acceptance once and for all. It couldn’t. But by understanding how utterly diverse beauty standards, athletic standards, and intelligence standards really are throughout time and space, and by facing the very real dangers of xenophobia in extremis like the horror in Sydney, we should be able to agree that we’re all better off never being “obsessed with perfection” when it comes to bodies.

 

 

Tags: , , , , ,

It’s Dwarfism Awareness Month!

2 Oct

x-ray of a dwarf(Image by Lefteris used under CC 2.0 via)

It’s October, so you know what that means. Here’s one of the most popular pieces from the Archives:

 

October is Dwarfism Awareness Month. This means you should be aware of the facts and experiences regarding people with dwarfism for the next 25 days. Then you can stop and go back to life as usual.

We have picked this month because it has Halloween in it. This way we can ensure that no one will offend us through drunkenness or choice of costume. This plan is foolproof.

We realize that October is also Breast Cancer Awareness Month, LGBT History Month, and Polish-American Heritage Month. Given that a month can handle only one minority at a time, we urge you to side with us. I myself picked my dwarfism over my Polish heritage and I promise you, the choice was easy. Anyone who chooses otherwise is a self-hating dwarf and the reason why we haven’t had a dwarf president yet.

In order to become Aware Of Dwarfism (A.O.D.), you can read up on it under the FAQ’s, read about some of my dwarfish opinions here or here or here, or consider any one of the following facts:

To begin with, Peter Dinklage is the best dwarf. Everyone agrees on this.

We love being photographed on the street. (Thank god for camera phones!) It is every dwarf’s dream to end up on Tumblr or Instagram someday. Either that or in a Peter Jackson film.

It is true that all dwarfs are magical. But especially homosexuals with achondroplasia. They are dwarf fairies.

We love it when you ask about Lord of the Rings. Please keep asking us about Lord of the Rings. We’re currently in fierce competition with New Zealanders over who gets the most LOTR questions.

Mini-Me is even better. It is the height of originality. We can’t get enough of it.

Please keep telling us that we must be really good at hide-and-seek. We don’t quite believe it yet, so your pointing it out is helpful.

Tossing a dwarf will make you more of a man. This has been scientifically proven by evolutionary biologists.

Adding a dwarf or two to your fantasy/cabaret/oddity story will make you a sophisticated artist. In his little known essay “L’art mystérieux du nain,” Toulouse-Lautrec wrote that World War II would have been prevented had there been more dwarf figures in 1930s song and film. (Terry Gilliam and Amanda Palmer are currently in a bidding war over the rights to the essay.)

In China and Russia and other evil countries, limb-lengthening is a cruel form of torture. In America, limb-lengthening is a miracle.

Liberals say a dwarf who has had limb-lengthening is destroying the dwarf community. Conservatives say a dwarf who has not had limb-lengthening is destroying their own future. Realists point out that dwarfs are destructive by nature.

Indeed, there are three dwarf personality types: belligerent, cute or nefarious. That is all. If you have met a dwarf who is contemplative or sarcastic or boring, that person is a not a dwarf. He is a fraud. I mean frawd.

Garden gnomes are frawds.

Midgets are left-handed dwarfs. Munchkins are elves in disguise. Oompa-Loompas are related to Pygmies, but less racist, so when in doubt, say Oompa-Loompas.

Dwarfs are happy to answer any of your questions about their sex lives. Just remember that if you don’t laugh at some point, we will be offended.

Dwarfs cannot have normal children. Like our great-great-great grandfather Rumplestiltskin, we are always on the lookout for normal children to kidnap. If you see a dwarf with normal children, contact the local authorities immediately.

Remember these facts and you will be officially A.O.D., which means no one has the right to accuse you of being insensitive from here on in. Better yet, you can recite these facts at dinner parties and lecture your friends with your newfound expertise. It is very important to be the expert on a subject at a dinner party. It proves you are a grown-up.

It is also important to spend as much time as possible this month making up height puns. Unfortunately, this is a bit of a challenge as many of the best puns have already been taken: Thinking Big; Don’t Sell Yourself Short; Even Dwarfs Started Off Small; Little People Big World; In Our Hearts We Were Giants. I suggest aiming for slightly more abstract sayings like, “All dwarfs have high voices. Ironic, isn’t it?” But make sure you say “high” emphatically or it will be lost on people. (Oh, and I’ve found that saying, “Achondro -paper or -plastic?” confuses most supermarket cashiers.)

And finally, sometimes it’s spelled “dwarfs” and sometimes it’s spelled “dwarves.” We get to decide. It’s the best part about being a dwarf.

 

 

Originally posted in October 2013

Tags: , , , , , , , , , , ,

Frozen Sperm & the Slippery Idea of Increased Risk

21 Aug

Hanging bodies(Image by Gillie Rhodes used under CC 2.0 via)
 
As long as people do not want to have children, or certain types of children, they will search for a way to avoid it. In a recent CNN report on reasons why a small but growing number young men have been freezing their sperm, achondroplasia was listed as one of the conditions the risk for which is associated with advanced paternal age. While the report cites single studies finding an increased risk for various conditions and disorders, many studies over the past two decades have linked achondroplasia to advanced paternal age. (Perhaps Mick Jagger should educate himself, if he hasn’t already.)

Most people with achondroplastic dwarfism are born to non-achondroplastic parents like mine. (“Are you parents little, too?” is one of the most common questions I get from strangers.) Most of us grew up told that our genetic mutation could not be traced to any known source. That is now changing, as news networks repeat the link to advanced paternal age.

My father was 28-years-old when I was born, hardly what we in the West think of when we envision advanced paternal age. All but one of my friends with achondroplasia have parents that were roughly the same age as my own when they were born: that is, late 20s or early 30s. And the majority of my friends with achondroplasia are first-borns.

Anecdotal evidence is often rife with bias, so I cannot officially dispute the researchers’ findings. Perhaps my friends and I are simply exceptions to the rule just like every smoker can name someone who beat the odds and puffed their way to age 95. But my personal experience easily invalidates any argument that men who freeze their sperm in their 20s or 30s are on a clear path to avoiding fathering a child with achondroplasia.

Bioethicists are divided on whether or not to advise men to freeze their sperm to avoid various conditions. Regardless of the answer, men should hear that the statistics on achondroplasia and age risk imbuing them with an inflated sense of control. One could say most forms of genetic counseling do.    

 

 

Tags: , , , ,

Art That Explores Dwarfism with Uniforms & Anti-Selfies

20 Mar

Laura Swanson(Image ©Laura Swanson, used with permission)
 
Laura Swanson is a rising artist on the New York scene. Born with achondroplasia, her work zeroes in on bodily difference and human perception. Her latest show, “Resistance,” opened recently at the JCC in Manhattan. The first part of the exhibit features uniforms and cultural apparel—that of a beekeeper, a welder, a plague doctor—altered to her size.

“I started thinking about if you see a person of average adult height wearing a uniform they wouldn’t judge or question…,” she told DNAinfo.  “[However,] when something is made smaller in scale, does it change the meaning?”

The second part of the exhibit features what Swanson calls “anti-selfies”: portraits in which her face is obscured. Does this draw attention to her extraordinary body? Or would the average viewer’s attention already be distracted by it?

In an interview at the Center for Art and Thought, she explained:

Anti-Self-Portraits examines longing for agency and privacy. I wanted to depict a naïve, comic desperation: that this person is so tired of being looked at, she is grabbing whatever is close to camouflage her body, but not doing a very good job at it, kind of like an ostrich sticking its head in the sand. At the same time, I wanted the photos to have a paradoxical feel. With the frontal, theatrical staging of the body, I wanted to convey that this person might not be such a desperate person, but actually a knowing person who is in control of how she is seen. 

A previous show, titled “Display,” featured an average-sized coat next to one that would fit the body of someone with achondroplasia. Here again Swanson invited her viewers to consider what exactly they were staring at. And why. Is an average-sized coat utilitarian, but a dwarf’s coat something you would pay to look at? Freak show attendees certainly did—and continue to do—throughout time and around the world.

Another planned project would explore notions of privacy. She described it in an interview at Haverford College in 2013:

My acute awareness of this desire stems not only from personal experience, but also from the history of photography, which is riddled with images of the Other…

I am working on a multi-part project that deals with an issue that is becoming increasingly unavoidable – the experience of having unwanted photographs taken of me and other people with physical differences while we go about our lives in public. It is funny because there is a lot of coverage and creative projects being made about government surveillance due to the recent PRISM/NSA spying controversy, but my project is actually looking at the ways ‘citizens’ use their phones to document others (ranging from people with physical differences and disabilities to depictions of homelessness) and share those photos on social media to amuse their friends. One part of the project will be … to design and fabricate devices for those who want to avoid having their image taken in public without consent. So not only am I getting further away from the camera, I am trying to prevent its usage!

As a professional photographer and sculptor, Swanson is thrusting tough questions upon the art world – a community renowned for having both broad and narrow definitions of beauty. As a person with dwarfism in the public eye, she is elevating the social issues of disability and physical difference to a more contemplative plane. That these issues tend to come in packaging that is either simplistically cute-and-cuddly or outright freak-show voyeuristic makes Swanson’s approach all the more refreshing.

 

 

Tags: , , , ,

And the Name of the Drug That Might End Dwarfism Is Vosoritide

28 Jun

Medicine 3(Image by Marosh used under CC license via)

 

Pharmaceuticals company BioMarin announced last week the first results of their clinical trials for the drug BMN-111, now named vosoritide by the World Health Organization. Researchers have been developing vosoritide in hopes of one day curing achondroplasia, the most common type of dwarfism. Vice-President Dr. Wolfgang Dummer reported:

In children receiving the highest dose of 15 micrograms per kilogram daily, we observed a 50% increase in mean annualized growth velocity compared to their own natural history control growth velocity. This increase in growth velocity, if maintained, could allow children with achondroplasia to resume a normalized growth rate. More importantly, vosoritide was well tolerated in all dose cohorts and we have observed no major safety concerns to date.

Whether or not vosoritide could reduce an achondroplastic person’s increased risk for chronic joint pain, bowed legs, spinal stenosis, sleep apnea, or hydrocephalus remains to be seen.

Since many of my readers are new to the blog, I’m re-posting my article “Will We Live to See the End of Dwarfism?” about how some of us with achondroplasia feel about all of this.

* * *

Medicine has been transforming the fate of human society since the first moment someone bandaged a wound. Bearing this in mind, along with the more recent advances in genetics, I have realized for the past decade or so that there is a future, however near or distant, that promises a world without dwarfism. But what if this world arrives as soon as the next generation?

Pharmaceuticals company BioMarin reported earlier this year the start of clinical trials for a drug called BMN-111. If it ends up doing what it promises, repeated injections could transform the bone and cartilage growth of children born with achondroplasia, essentially curing them of the condition. Could this mean that I might someday belong to the last of the dwarfs?

To be clear, BMN-111 could cure only achondroplasia, the most common type of dwarfism, not the other 200+ types. (So the attention-grabbing name of this article is a tad misleading.) Dwarfism caused by growth hormone deficiency—which affected circus performer General Tom Thumb and most of the actors playing the Munchkins in The Wizard of Oz—has already been cured by hormone injections invented at the end of the last century. But 70% of all dwarfs have achondroplasia. Without us, the small number of people identifiable as dwarfs would become much smaller.

Because I’m a fully grown adult, I can’t ever cure my achondroplasia. But would I have chosen to do so if I could? Were my doctor to offer me a pill that would transform my joints and my muscle tone, allowing me to walk and stand around for longer than an hour without my feet swelling with pain, I would take it in an instant. The same goes for a pill that would endow me with more normal fine motor strength, so that I could open jars and push down sticky buttons and do all those tasks that leave me swearing and/or asking someone else for help. I would gladly have taken a pill that would broaden my Eustachian tubes so that I would stop getting ear infections every year. And I would have embraced any sort of medicine that would have widened my spinal column so that I would never have had to have a laminectomy, and so that I could cook and clean my house without back pain. All of the discomfort and inconvenience I just listed are part and parcel of achondroplasia – parts that limb-lengthening could never alter.  

But when I consider a pill that, in ridding me of all that pain, would also rid me of every physical marker of achondroplasia, I suddenly hesitate. My wrists, my feet, my skull, my face would look significantly different from the one I have. The idea of never having had to learn how best to react to being the most physically remarkable person in school, of never having undergone limb-lengthening, of never having lived in an institution with children with all sorts of serious conditions, of never having had to explain my unique history to others – it makes me have a hard time imagining an Emily Sullivan Sanford that is anything like the one I know today. My dwarfism is only part of who I am, but it has been a significant part of who I am. This is why I understand the Little People of America members who balk at BMN-111, put their fingers in their ears and chant, “Go away, go away, go away!”

We must approach the future rationally because our emotional attachment to life as we know it can lead us to delude ourselves with an unrealistic sense of control. History after all demonstrates that future generations will never know all kinds of things we treasure today. Give or take a few centuries, people in our part of the world will most certainly not face the same illnesses, speak the same language, wear the same clothes, eat the same foods, or observe the same traditions we do. Whether we’re debating the politics of Hawaiian Pidgin or that punk’s not dead, we do not get the final say on what future generations will know and what will be lost to the ages.

Identity is a construct, but a construct that is as powerful as any other. As Andrew Solomon writes, “I don’t wish for anyone in particular to be gay, but the idea of no one’s being gay makes me miss myself already.”

Granted achondroplasia is not merely a difference like a dialect or homosexuality. It is a medical condition that causes very real physical pain and health risks. Like diabetes. I can write with certainty that the vast majority of people with diabetes, while rightfully proud of the obstacles they’ve overcome, would happily rid themselves of the disease. They would celebrate never having to check their blood sugar, inject themselves with insulin, or worry about developing dangerous complications. We can safely make the same assumption for people who have to deal with migraine headaches or deep-vein thrombosis.

But let’s consider a condition that, like achondroplasia, has as many social ramifications as medical ones. I bet most people who wear glasses would gladly take a pill that guaranteed perfect vision. No more headaches, no more pressure sores on the bridge of your nose, no more wondering where you set them down, no more worrying if they break, no more bills! But would they so easily let go of their bespectacled appearance? Although he no longer needs glasses since his laser surgery, comedian Drew Carey wears non-prescription glasses to maintain his look.

I surveyed a handful of friends in Europe and the U.S., and most answered that they would indeed take a pill guaranteed to improve their vision, and also that they would never wear anything but sunglasses again. If this scenario ever becomes reality, the movement of the past 100 years to broaden beauty standards to include the bespectacled will begin to fade. The 20% of my respondents that answered, “I would wear non-prescription glasses because it’s a part of my identity,” will belong to a shrinking minority left to fend for itself. They will likely start counting the minutes until they hear something marginalizing like: “Isn’t it great you won’t have to look like a nerd anymore?”    

Once again, people with achondroplasia must admit that our distinguishing condition involves far more innate physical complications than simply needing glasses or being gay. Activist Harry Wieder bemoaned the reticence among people with dwarfism to even admit that we are disabled, and he was right to be so critical. Downplaying the pain and surgical risks everyone with achondroplasia faces is a matter of denial. But such denial is often rooted in the worry that others will overemphasize our pain, distancing themselves from us in a way all too similar to the fear and pity that fuels ableism. Such distance imposed by other minorities can break solidarity and lead to hierarchical thinking along the lines of, “At least I’m not like that!”

Anyone who reacts to the idea of BMN-111 ridding humanity of the achondroplastic appearance with a sigh of relief has a problem. It’s a problem we can never afford to ignore. The lessons of diversity awareness and inclusion are priceless. If dermatologists some day offer a cure for vitiligo, Winnie Harlow’s recent successes in the world of modeling will still have only been a good thing.

My attachment to my starfish hands, my achondroplastic nose, and my scars is not rational. But the human experience is never purely rational. And self-acceptance is an achievement like no other. Almost every person with achondroplasia has a jarring moment when they see themselves in photos or on film and are reminded that their hands are not at all slender, like most of the hands they see in photos or on film. Or that their hips sway when they walk. Or that their skulls are larger. Learning to live with the shock is a difficult but worthwhile experience. When a mother of a girl with achondroplasia wrote to me, asking about her four-year-old daughter’s future, my family awwwwwed at the photos she sent us. “I remember having an adorable little girl with a forehead like that!” my dad grinned.

I was not nearly so moved by the recently published images of celebrities photoshopped to “reimagine them with dwarfism” next to an image of Peter Dinklage photoshopped to “reimagine him without” because only their legs were modified.

The project itself is thought-provoking, but Daniel Radcliffe simply wouldn’t get into the achondroplasia club with those ridiculously long arms. And Peter Dinklage—whom GQ declared a “stud” in its 2011 Men of the Year list—would have a dramatically different forehead, cheekbones, jaw, and nose.

One of the respondents to my survey who said he would keep his glasses explained, “Not really for aesthetic reasons, exactly, though that’s part of it (and it is fun to buy glasses). But because they’re a part of my face! I’ve never considered contacts, either, come to think of it. They serve some other function, beyond utility and style, I guess.”

Similar feelings have been expressed by people who underwent surgery to remove the sixth finger on their right hand for convenience, while opting against the removal of the sixth finger on their left: “Why would I cut it off? It’s a part of me.”

Syndactyly runs in two sides of my family. One relative remarked about her child, “I was so happy when she was born to see she didn’t have those fused toes!”

To which another relative with fused toes later said, “Why? It hurts a bit more when you stub them, but otherwise, what’s the big deal?”

Replace the word “fused toes” with red hair or monolids or pale skin or dark skin or freckles or whatever intrinsic part of you might somewhere be considered unfashionable and you’ll know a little how dwarfs feel about BMN-111. As with limb-lengthening, BMN-111 threatens to out the uglier feelings some people have about our appearance. We must remember that it’s the feelings that are ugly, not the body.    

Talking out my endlessly complex thoughts about a world without dwarfism feels like moving through a labyrinth that is partly my own making. During one such recent talk, a close friend said to me, “If we could look at a version of you that never had achondroplasia, I understand that you would miss yourself and I would miss you, too.  But you would be awesome in a different way that would still be your own way, and it would be without all the pain and complications and danger.”

This is what people with achondroplasia need to hear from those who truly accept them.

 

 

Tags: , , , , , , ,

Interview on Berlin Television

6 Jun

©Ines Barwig(Image ©Ines Barwig)

 

Berlin’s public broadcasting station rbb has just aired a report on Painting On Scars, which you can read about and watch here.

For those of you not fluent in German, I advise you against using GoogleTranslate. As a professional translator, I’ve always considered the service a bit of a rival, but now we’re talking full-blown war. Because while any half-educated human Germanist could tell you that the rbb report translates into English as “Short-Statured – Getting Taller Through Operations,” Google says:

 

GoogleTranslate

 
 

Tags: , , , , , ,

When It Comes To Health, Who Should Minorities Trust?

12 Apr

Medication diet squircle(Image by Barry used under CC.20 via)

 

At the beginning of this year, I underwent orthopedic surgery and rare complications immediately arose from it, causing me to take three months of sick leave. In that time, both my country of origin and my country of residence experienced outbreaks of measles that have set the Internet ablaze with raging arguments about medicine, personal choice and the greater good. While the critics of Big Pharma have plenty of good points, recent studies of Big Herba—which is unregulated in the U.S.—have debunked an array of flaws that can be deadly. Glossing over the vitriol, at the crux of the matter lies a very reasonable question: When it comes to health, who should you trust?

“Trust to your doctor” sounds simple enough until we consider the many instances throughout history when medical professionals have abused this trust, particularly in regard to minorities. Health organizations around the world classified gay people as mentally ill as late as 2001. A panelist on Larry Wilmore’s The Nightly Show last month cited the Tuskegee syphilis experiment, which treated African-American men like lab rates from 1932 to 1972, as the basis for his overarching distrust of government health organizations. Investigations recently revealed that the U.S. Public Health Service committed similar crimes against mental patients and inmates in Guatemala in the 1940s. The polio vaccine, which has saved millions of lives globally, was first tested on physically and mentally disabled children living in asylums and orphanages. Researchers advocated the forced sterilization of trans people and ethnic minorities as recently as 2012. And of course there were the Nazis and the many, many scientists before them who passionately promoted eugenics. ITV recently rebroadcast a documentary hosted by Warwick Davis detailing Dr. Mengele’s horrific experiments on dwarfs at Auschwitz.

In other words, minorities don’t have to dig too deep to come up with plenty of reasons to be wary of scientists and doctors. Regulation, transparency and a never-ending, highly public debate on bio-ethics and human rights are necessary to prevent such crimes from happening again.

But an ideological opposition to all doctors based on such abuses ignores the myriad successes. A Slate article appearing last fall, “Why Are You Not Dead Yet?” catalogs the thousands of reasons so many of us are living so much longer than our ancestors did—from appendectomies to EpiPens to everyday medications—which we so often overlook because we have come to take the enormous medical advances of the past 200 years for granted.

And yet, as so many scientists are only too ready to admit, science does not know everything. Almost no medical procedure can be guaranteed to be risk-free, and many people base their distrust of doctors on this fact. My current post-surgical complications were just cited to me by an acquaintance as reason enough for why I never should have had the operation at all and instead gone to a TCM healer.  

In my 33 years I have undergone 14 surgeries, physical therapy, hydrotherapy, occupational therapy, electro-muscular stimulation therapy, and the list of medications I’ve taken undoubtedly exceeds a hundred. I have also been treated with reiki, shiatsu, osteopathy, acupuncture, massage, prayer, and herbal remedies based on macrobiotic, homeopathic and detox theories. Some of these treatments I chose as an adult, and some of them were chosen for me by adults when I was a child and a teen. Some of the medical treatments worked, some didn’t, and some caused new problems. Some of the alternative treatments rid me of lingering pain, and some were a complete waste of time, money and energy as my condition worsened. I won’t ever advocate any specific treatment on this blog because my readership is undoubtedly diverse and the risk of making inaccurate generalizations is too great.

Indeed, a grave problem in the public debate on health is the frequent failure to acknowledge human diversity. Most health advice found online, in the media, at the gym or a healing center is geared not at minorities but physiotypical people, who are seeking the best way to lower their risk for heart disease, fit into their old jeans, to train for a marathon, or to simply feel better. They are not seeking the best way to be able to walk to the corner or have enough strength to shop for more than half an hour. Those in the health industry who endorse one-size-fits-all solutions—“We just need to jog/Start tai-chi/Eat beans, and all our troubles will go away!”—rarely address minority cases that prove to be the exception to their rule. But atypical bodies have just as much to teach us about our health as typical bodies, and leaving them out of the conversation benefits no one but those seeking to profit off easy answers.

When it comes to seeking treatment for my condition, I follow a simple rubric: I don’t want to be the smartest person in the room. I have no professional training in medicine or anatomy. As this physician explains so well, self-diagnosis is a very dangerous game. Yet I sometimes am the expert on my body thanks to the relative scarcity of people with achondroplasia—there are only 250,000 of us on earth, or 0.00004% of the world population—compounded with the scarcity of people with achondroplasia who have undergone limb-lengthening and sustained bilateral injuries to the anterior tibialis tendons. A visit to a healing center or a hospital often entails conversations like these:

Shiatsu Healer: You’re walking with a sway-back. Your wood energy is obviously misaligned because you are stressed.

Me: My hips sway when I walk because the ball-and-socket joint in the hip is shaped instead like an egg-and-socket in people with achondroplasia.

***

Physical Therapist: Your hips sway when you walk because one leg is obviously longer than the other.

Me: No, I have my orthopedist’s report documenting that my legs are precisely the same length. My hips sway when I walk because the ball-and-socket joint in the hip is shaped instead like an egg-and-socket in people with achondroplasia.

 ***

Nurse: Your temperature is pretty high. I’m a bit worried.

Me: These anesthesiology guidelines I got from the Federal Association for Short-Statured People say that hyperthermia is to be expected post-op in patients with achondroplasia.

Sometimes the information I offer goes unheeded. In both the U.S. and in Germany, I have found arrogance is equally common among doctors and healers. Some of them are delightfully approachable, and others are so socially off-putting that they make you want to throw your wheelchair at them. The same arrogance, however, can take different forms. I have documented before the particular brand of pomposity so endemic to doctors, and it is safe to say that holistic healers are less likely to treat their patients like products on an assembly line because, by definition, they are more likely to take psychological well-being into account. But they are also more likely to endorse a one-size-fits-all solution for health, which invariably marginalizes minorities like me.

Those of us with extremely rare conditions are far more likely to find specialists among those licensed in medicine than among alternative healers. Living Naturally, the only website on alternative treatments I could find that even mentions achondroplasia, emphasizes that none of the therapies they suggest for achondroplasia have ever been tested on patients who have it. To be fair, rare conditions by definition are not well-known to your average GP either. But physicians more often know how to work with the facts, embracing the medical literature on achondroplasia I hand to them. Some alternative healers also embrace such literature, while others dismiss anything written by anyone in a white coat.

Even when a visceral hatred of hospitals and their hosts is irrational, it is understandable. My most recent stay involved some of the kindest medical professionals I have ever encountered but nevertheless left me waiting for two and a half hours on a metal bench with no back support in a hallway glaring with fluorescent lights and echoing with the cries of patients in pain. I respect everyone’s right to opt against surgery, or any medical treatment, as long as their condition does not cause others harm. But no matter how much modern medicine has abused minorities’ trust, disabled people are the only minority that cannot afford to forgo it.

A worldwide study presented to Little People of America found that, at this point in history, dwarfs have a higher quality of life—i.e., access to effective health care, employment opportunities, acceptance in society—in Northern Europe than anywhere else on earth. Reductive arguments that demonize all of Western medicine because the Nazis! can be canceled out by reductive arguments that dismiss anything developed outside the West because Asia’s terrible disabled rights record!  

Broad generalizations like “Natural is better” can only be upheld by those ensconced in the privileges of a non-disabled body. In 2011, the parenting website Offbeat Families banned the term “natural birth”—urging writers to instead refer to “medicated” and “unmedicated” birth—because “natural” had so often been used to imply “healthier.” An unmedicated birth is wonderful for anyone who can and wants to experience it, but it is important to remember that it is a privilege. A privilege, like a disability, is neither your fault nor your achievement.      

“Healthy” is a relative idea. Our choices about our bodies will always be limited. This is a sometimes terrifying fact to face. But in the public debate, we must remember that it is a fact those among us with rare disabilities and conditions can never avoid. In failing to remember it, we fail to make decisions about human health that are truly informed.

 


Tags: , , , , , , ,

Will We Live To See The End of Dwarfism?

29 Mar

Hands

 

Prologue: My three-month long hiatus from blogging was due to tendon surgery I underwent in January and rare complications that arose from it. I am now gradually returning to work from sick leave and thrilled to be back.

* * *

Medicine has been transforming the fate of human society since the first moment someone bandaged a wound. Bearing this in mind, along with the more recent advances in genetics, I have realized for the past decade or so that there is a future, however near or distant, that promises a world without dwarfism. But what if this world arrives as soon as the next generation?

Pharmaceuticals company BioMarin reported earlier this year the start of clinical trials for a drug called BMN-111. If it ends up doing what it promises, repeated injections could transform the bone and cartilage growth of children born with achondroplasia, essentially curing them of the condition. Could this mean that I might someday belong to the last of the dwarfs?

To be clear, BMN-111 could cure only achondroplasia, the most common type of dwarfism, not the other 200+ types. (So the attention-grabbing name of this article is a tad misleading.) Dwarfism caused by growth hormone deficiency—which affected circus performer General Tom Thumb and most of the actors playing the Munchkins in The Wizard of Oz—has already been cured by hormone injections invented at the end of the last century. But 70% of all dwarfs have achondroplasia. Without us, the small number of people identifiable as dwarfs would become much smaller.

Because I’m a fully grown adult, I can’t ever cure my achondroplasia. But would I have chosen to do so if I could? Were my doctor to offer me a pill that would transform my joints and my muscle tone, allowing me to walk and stand around for longer than an hour without my feet swelling with pain, I would take it in an instant. The same goes for a pill that would endow me with more normal fine motor strength, so that I could open jars and push down sticky buttons and do all those tasks that leave me swearing and/or asking someone else for help. I would gladly have taken a pill that would broaden my Eustachian tubes so that I would stop getting ear infections every year. And I would have embraced any sort of medicine that would have widened my spinal column so that I would never have had to have a laminectomy, and so that I could cook and clean my house without back pain. All of the discomfort and inconvenience I just listed are part and parcel of achondroplasia – parts that limb-lengthening could never alter.  

But when I consider a pill that, in ridding me of all that pain, would also rid me of every physical marker of achondroplasia, I suddenly hesitate. My wrists, my feet, my skull, my face would look significantly different from the one I have. The idea of never having had to learn how best to react to being the most physically remarkable person in school, of never having undergone limb-lengthening, of never having lived in an institution with children with all sorts of serious conditions, of never having had to explain my unique history to others – it makes me have a hard time imagining an Emily Sullivan Sanford that is anything like the one I know today. My dwarfism is only part of who I am, but it has been a significant part of who I am. This is why I understand the Little People of America members who balk at BMN-111, put their fingers in their ears and chant, “Go away, go away, go away!”

We must approach the future rationally because our emotional attachment to life as we know it can lead us to delude ourselves with an unrealistic sense of control. History after all demonstrates that future generations will never know all kinds of things we treasure today. Give or take a few centuries, people in our part of the world will most certainly not face the same illnesses, speak the same language, wear the same clothes, eat the same foods, or observe the same traditions we do. Whether we’re debating the politics of Hawaiian Pidgin or that punk’s not dead, we do not get the final say on what future generations will know and what will be lost to the ages.

Identity is a construct, but a construct that is as powerful as any other. As Andrew Solomon writes, “I don’t wish for anyone in particular to be gay, but the idea of no one’s being gay makes me miss myself already.”

Granted achondroplasia is not merely a difference like a dialect or homosexuality. It is a medical condition that causes very real physical pain and health risks. Like diabetes. I can write with certainty that the vast majority of people with diabetes, while rightfully proud of the obstacles they’ve overcome, would happily rid themselves of the disease. They would celebrate never having to check their blood sugar, inject themselves with insulin, or worry about developing dangerous complications. We can safely make the same assumption for people who have to deal with migraine headaches or deep-vein thrombosis.

But let’s consider a condition that, like achondroplasia, has as many social ramifications as medical ones. I bet most people who wear glasses would gladly take a pill that guaranteed perfect vision. No more headaches, no more pressure sores on the bridge of your nose, no more wondering where you set them down, no more worrying if they break, no more bills! But would they so easily let go of their bespectacled appearance? Although he no longer needs glasses since his laser surgery, comedian Drew Carey wears non-prescription glasses to maintain his look.

I surveyed a handful of friends in Europe and the U.S., and most answered that they would indeed take a pill guaranteed to improve their vision, and also that they would never wear anything but sunglasses again. If this scenario ever becomes reality, the movement of the past 100 years to broaden beauty standards to include the bespectacled will begin to fade. The 20% of my respondents that answered, “I would wear non-prescription glasses because it’s a part of my identity,” will belong to a shrinking minority left to fend for itself. They will likely start counting the minutes until they hear something marginalizing like: “Isn’t it great you won’t have to look like a nerd anymore?”    

Once again, people with achondroplasia must admit that our distinguishing condition involves far more innate physical complications than simply needing glasses or being gay. Activist Harry Wieder bemoaned the reticence among people with dwarfism to even admit that we are disabled, and he was right to be so critical. Downplaying the pain and surgical risks everyone with achondroplasia faces is a matter of denial. But such denial is often rooted in the worry that others will overemphasize our pain, distancing themselves from us in a way all too similar to the fear and pity that fuels ableism. Such distance imposed by other minorities can break solidarity and lead to hierarchical thinking along the lines of, “At least I’m not like that!”

Anyone who reacts to the idea of BMN-111 ridding humanity of the achondroplastic appearance with a sigh of relief has a problem. It’s a problem we can never afford to ignore. The lessons of diversity awareness and inclusion are priceless. If dermatologists some day offer a cure for vitiligo, Winnie Harlow’s recent successes in the world of modeling will still have only been a good thing.

My attachment to my starfish hands, my achondroplastic nose, and my scars is not rational. But the human experience is never purely rational. And self-acceptance is an achievement like no other. Almost every person with achondroplasia has a jarring moment when they see themselves in photos or on film and are reminded that their hands are not at all slender, like most of the hands they see in photos or on film. Or that their hips sway when they walk. Or that their skulls are larger. Learning to live with the shock is a difficult but worthwhile experience. When a mother of a girl with achondroplasia wrote to me, asking about her four-year-old daughter’s future, my family awwwwwed at the photos she sent us. “I remember having an adorable little girl with a forehead like that!” my dad grinned.

I was not nearly so moved by the recently published images of celebrities photoshopped to “reimagine them with dwarfism” next to an image of Peter Dinklage photoshopped to “reimagine him without” because only their legs were modified.

The project itself is thought-provoking, but Daniel Radcliffe simply wouldn’t get into the achondroplasia club with those ridiculously long arms. And Peter Dinklage—whom GQ declared a “stud” in its 2011 Men of the Year list—would have a dramatically different forehead, cheekbones, jaw, and nose.

One of the respondents to my survey who said he would keep his glasses explained, “Not really for aesthetic reasons, exactly, though that’s part of it (and it is fun to buy glasses). But because they’re a part of my face! I’ve never considered contacts, either, come to think of it. They serve some other function, beyond utility and style, I guess.”

Similar feelings have been expressed by people who underwent surgery to remove the sixth finger on their right hand for convenience, while opting against the removal of the sixth finger on their left: “Why would I cut it off? It’s a part of me.”

Syndactyly runs in two sides of my family. One relative remarked about her child, “I was so happy when she was born to see she didn’t have those fused toes!”

To which another relative with fused toes later said, “Why? It hurts a bit more when you stub them, but otherwise, what’s the big deal?”

Replace the word “fused toes” with red hair or monolids or pale skin or dark skin or freckles or whatever intrinsic part of you might somewhere be considered unfashionable and you’ll know a little how dwarfs feel about BMN-111. As with limb-lengthening, BMN-111 threatens to out the uglier feelings some people have about our appearance. We must remember that it’s the feelings that are ugly, not the body.    

Talking out my endlessly complex thoughts about a world without dwarfism feels like moving through a labyrinth that is partly my own making. During one such recent talk, a close friend said to me, “If we could look at a version of you that never had achondroplasia, I understand that you would miss yourself and I would miss you, too.  But you would be awesome in a different way that would still be your own way, and it would be without all the pain and complications and danger.”

This is what people with achondroplasia need to hear from those who truly accept them.  

 

 

 

Tags: , , , , , ,

Does It Matter If It’s Genetic?

16 Feb

Photo 02-07-14 12 29 21(Image by Eduardo Unda-Sanzana used under Creative Commons license via)

 

There is an argument gradually gaining momentum in the LGBT movement: “So what if being gay is a choice?” Rather than lecturing social conservatives that homosexuality is an inborn trait and not a chosen lifestyle, we should ask them what’s so bad about two consenting adults loving each other. With bisexual, pansexual, and genderfluid identities becoming more visible, and all sorts of people becoming more open to experimenting, who really cares if any of it is a choice?

It’s an important question in the broader debate about sex and gender. And it forces me to question the parameters of this blog.

Painting On Scars is founded on the rights of people who are viewed as minorities based on qualities they have no choice about: gender identity, sexuality, ethnicity, nationality, class background, physical traits, and mental abilities. This foundation is built on my own minority status being indisputably determined by factors beyond my control. There is no doubt whatsoever that I was, as Lady Gaga hollers, born this way.

And when it comes to confronting bigotry, there is something particularly painful about being belittled for something you have no choice about. All of us can feel insecure about the decisions we make, but being told that you’re seeking work in the wrong field or that you talk too loud on the phone is still far less harrowing than being told that your natural appearance is universally repulsive or that your gender makes you intellectually or emotionally inferior. Every one of us wants to be accepted for the way we were born because a rejection of it feels like a rejection of our very lives. As autism activist Jim Sinclair explains:

When parents say, “I wish my child did not have autism,” what they’re really saying is, “I wish the child I have did not exist and that I had a different, non-autistic child instead.” Read that again. This is what we hear when you mourn over our existence. This is what we hear when you pray for a cure. That your fondest wish for us is that someday we will cease to be, and strangers you can love will move in behind our faces.

For this reason, Painting On Scars examines the existence of minorities who are born this way and the myriad reasons why any of us still struggle to accept them. (More on the complexities of parenting disabled children here.)

However, the born-this-way rubric is not always helpful. What about the explicit decision to not conform? What about the human right to the pursuit of happiness? It seems only natural—for lack of a better word—to defend alternative traits and behaviors that are very much a choice but do no harm. Women who don’t wear makeup. Filmmakers who dare to feature minority accents. People who want to preserve their parents’ cultural traditions rather than assimilate for assimilation’s sake.  Men who don’t identify as transgender but still very much like wearing dresses. Objection to these choices usually stems from a rigid belief in homogeneity or simply a difference in taste. Such objections make it clear to me as a blogger that as long as a difference doesn’t cause real harm, it is worth protecting from harm.

And conversely, I tend to defer to others when it comes to minority traits that people have little choice about but that do cause a good deal of harm: personality disorders, psychosis, sexual attraction to children, paranoia, trauma, suicide, or anything that precipitates emotionally abusive tendencies. I research these issues voraciously, not only because I have personal experience with many of them, but because they raise questions about human rights and individual freedom, as well as the greater good and personal safety. (The pro-mia and pro-ana movements, for example, argue that any attempt to treat or cure people with eating disorders qualifies as oppression rooted in narrow-mindedness.) Yet I refrain from opining about these issues publicly because my knowledge of them is as simplistic as they are complex.

Whether to change society or change oneself is a persistent predicament that accompanies every stage of life. When exploring the answer as it applies to minority issues, I keep coming back to the same question: Who suffers more in the change?  Humans have repeatedly proven to cause less suffering when we accept body diversity, intellectual disabilities, LGBT identities, and gender equality than when we oppress them.

Of course, what constitutes oppression and what constitutes acceptance is sometimes disputable. Alex Andreou argues this week in The Guardian that the current search for the gene for homosexuality is quite harmful. While LGBT activists have traditionally opposed the idea of homosexuality as a choice to combat those who argue for a cure through therapy, LGBT critics of the genetic research fear that discovery of a gene for homosexuality will lead to its elimination. Those of us whose conditions are genetically determined and socially marginalized have been acutely aware of this problem not just since the advent of the Human Genome Project 30 years ago, but since eugenicists began sterilizing all sorts of peoples over 100 years ago. In democratic societies where governments no longer dare to be too vocal about medical decisions regarding minorities, everyone still fears the coming of the day when insurance companies inform expecting parents that they will not cover children who will cost more. Because the existence of minorities precludes the efficiency of a one-size-fits-all system, we will always cost more.

In the spring of 1994, I was headed into the operating room to have my Ilizarov fixators removed. While prepping for surgery, one of the members of the surgical team excitedly told my mother, “Have you heard the news about achondroplasia? They found the gene! We can test Emily for it!”

My mother signed a release allowing for them to perform the test during the operation. Several weeks later I received a letter confirming that my fibroblast growth receptor gene 3 had the achondroplasia mutation. My first reaction was, “No shit. Who cares?”

I had been officially diagnosed with achondroplasia on my third day after birth, though admittedly, such an early diagnosis back in the 1980s was a stroke of luck. A girl with achondroplasia who later became my best friend had been born at the same hospital six months earlier, so the doctors recognized our similarities and ordered x-rays on my limbs. My achondroplasia was obviously a result of nature, not nurture.

Then again, in olden days dwarfism was often thought to be caused by mothers with loose morals. I myself had once asked my mother if perhaps I got achondroplasia because of the decisions she had made about the birthing process. (I had just watched Look Who’s Talking and had learned a lot about the pop culture understanding of what goes into having a baby.) The gene for achondroplasia explained how I got it, how I could pass it on, and lay rest to any modern blame-it-on the-mother mindset that might suspect it was because of aspirin or salami or cinnamon. Such information can—but does not have to—affect your sense of self.

A few years ago a woman living in the U.S. contacted me because her two-year-old daughter had been diagnosed with achondroplasia. The girl’s grandparents lived in another country and had steadfastly dismissed the diagnosis. “Americans are famous for over-diagnosing every little thing,” they shrugged. “She’ll grow out of it!” (Pun intended?)

A friend from the same country explained to me that disabled people there generally have few support networks and even fewer opportunities for independence. Perhaps the grandparents’ refusal to believe in achondroplasia stemmed from their fierce desire to remain hopeful about her future.

Would running a genetic test finally convince them to accept reality? When I was born, my parents and I benefited greatly from the dwarf rights movement of the 1970s and 80s, which had emerged due to the egalitarian spirit of the times that indulged in civil rights and celebrating diversity. As with the gay rights movement, millions of supporters showed that they did not need to see the results of genetic testing in order to justify and defend a minority’s right to exist and be accepted. If they could do it, so can we.

 

 

Tags: , , , , , , , , , ,

Is Dwarfism A Disability?

27 Oct

(Image by Ron Riccio used under Creative Commons license via)

 

A more sober ending to Dwarfism Awareness Month…

I remember being around 10 years-old when I began taking care to never refer to my dwarfism as an “illness” or a “disease.” An illness is something that tries to destroy you. It demands you go into battle. Even if you end up grateful for its having made you stronger, you’re glad when it’s gone. My dwarfism has always been around and I’ve never tried to conquer it. It’s a condition, a word as neutral as it is fitting. But is it a disability?

Many in the dwarf community insist that it is not. The thinking goes that being extraordinarily short is no more serious than being left-handed. We don’t think of left-handedness as a disability. It’s merely a difference, one of many physical features that can shape someone’s identity, like hazel eyes or an outie belly button. Being left-handed is only an inconvenience insofar as the world is built for those who are right-handed, and populated by some who still cultivate fear and hatred of those who don’t conform to the majority. Needing left-handed scissors and mouse buttons is not really thought to be an issue of disabled access – it’s more akin to needing glasses or extra-moisturizing shampoo. Diversity awareness over the last 50 years has led the vast majority of Westerners to shrug at the idea of left-handedness.

And such a neutral shrug is what dwarf activists seem to be coveting when they insist that dwarfism is not a disability. In the words of Andrew Solomon, “Neutrality, which appears to lie halfway between shame and rejoicing, is in fact the endgame, reached only when activism becomes unnecessary.” But is dwarfism only an inconvenience insofar as the world is built for those who are taller? It’s a compelling thought experiment, but it ignores all the medical complications I’ve had to deal with. And it raises the question: What is dwarfism?

The official definition, which lumps hundreds of skeletal dysplasias and growth hormone deficiencies into one category, is in fact only concerned with height. Little People of America defines a dwarf as anyone who stands fully grown below 4’10” (1.47 m). But height is relative. Women in Indonesia and Guatemala are 4’10” on average, which means that the LPA definition is based on a certain culture, and cultures are always changing as we move through time and around the world. As a pre-teen, I always got a kick out of seeing my towering parents become the minority at LPA meetings, while as an adult, I got a kick out of seeing my German-Swedish partner tower over my parents.

Physically, Warwick Davis and Peter Dinklage have no more in common than a black-haired Korean does with a black-haired Irishman. But they share many experiences rooted in society’s reaction to their short stature. They were both cast as dwarfs in the second Chronicles of Narnia film because the fantasy tradition cares first and foremost about looks, making up its convoluted ideas about heritage and separate races as it goes along. Most forms of dwarfism are caused by genetic mutations, but others result from chromosomal abnormalities, malnutrition, or even child abuse. Thus, because it encompasses all sorts of conditions with a tremendous variety of causes and complications, dwarfism is a social construct. Can a social construct be a disability? What is a disability?

This blog recognizes disability as a medical condition that causes you to experience more pain and/or limitations than the average person in your peer group, and therein attracts inordinate attention from society. And the attention has traditionally been negative. Disabled people carry a burden most other minorities do not in that we must argue that our lives and identities are no less valuable than anyone else’s, while at the same time admitting that we will always experience a good deal of pain no matter how accepting or accommodating society is. (Poor people are the only other minority that shares this burden.) This idea of inherent pain is what causes many activists in the autistic community and the transgender community to buck the disabled classification.

But when pain is indisputably inherent to a condition, it is frequently relativized in the hopes that this will reduce ableist attitudes. When I was born, the doctor pointed out to my parents that “everyone has something different about their bodies. One person has bad knees, another has a chronic skin rash. Emily’s difference is just a lot more noticeable than other people’s.” But does this mean that bad knees and skin rashes and seasonal allergies are all disabilities? There’s more to it than that.

If a medical condition is only minimally limiting and can be treated with standard procedures, we don’t really consider it a disability and rightfully so. While there is value in relativizing everyone’s struggles in order to calm our fears of the Other, it carries the risk of our failing to recognize differences that have much to teach us. The regular migraines I inherited from my mother don’t make me disabled. The pain can be intense and it’s infuriatingly inconvenient to feel one coming on at a dinner party while also feeling the hollow echo of an empty pill box in my bag. But the migraines are treatable—and not exorbitantly expensive to treat—and easily understood by others because plenty of people get them. Having to explain to people what my back and joints can and cannot endure is a more complex task.  Alleviating or avoiding the pain is even harder.

I interviewed friends and acquaintances with achondroplasia about the physical difficulties they regularly face. Some described always needing to lie down for at least half an hour whenever they vacuum for 10 minutes or more, and needing to get up earlier than everyone else on weekdays in order to afford themselves more time for walking to work or class. Everyone has trouble finding comfortable shoes that fit—women’s business shoes and sandals pose the biggest challenge—and many need to wear orthotics. Camilla, a college student who has not undergone limb-lengthening, told me:

I definitely believe I feel fatigue more easily than people my age. I went out dancing with friends last night and I had to stop and just stand for a while because my legs were starting to hurt. Also, when I walk places with my average height friends, my joints start to hurt while they feel almost no effects of fatigue at all… I would say that the hardest physical aspect of having dwarfism would not be the height difference but the extreme muscle and joint pain that seems to be more and more easily triggered as I get older.

And by “older” she means approaching her mid-twenties. These physical limitations would sound less surprising coming from senior citizens, which is why, as an advisor explained to me, your eligibility for disability status decreases as you age and aching joints become more common to your peer group.

A friend who had limb-lengthening at the same time I did told me, “I know if I’ve been on my feet all day, my ankles get really stiff and I’m limping around at home at the end of the night… as compared to my friends who work all day and still manage to hit yoga class, the gym, or cycling class afterwards.” Those of us who have undergone limb-lengthening can test whether achondroplasia is a disability because we control for the socially-constructed advantages of height. Yet in my interviews, I noticed that many who have had limb-lengthening are often reticent to talk about their current physical hardships lest someone conclude that all that time spent breaking and healing and growing bones was for naught.

Indeed, pride complicates our perceptions of pain. While hypochondriacs rejoice when they qualify as “disabled,” those who have regularly been reminded by peers and institutions of the supposedly pitiful nature of their condition are often less willing to revel in it. Those who reject the idea of calling dwarfism a disability are often motivated by the desire to de-stigmatize dwarfism. I of course understand this desire, but I don’t see how we can make the argument without stigmatizing disability. And I am suspicious of any mindset that supports a hierarchy by essentially saying, “At least I’m not like them.”

Like people of color, people with dwarfism are united only by society’s reaction to them, not by any medical traits. This is why I do not believe dwarfism itself is a disability. However, most types of dwarfism are. The way in which the physical pain brought on by achondroplasia intersects with social limitations is explained very well by Spoon Theory, an idea invented by Christine Miserandino, who has lupus. It bears repeating that I can only begin to imagine what living with lupus is like.  In the presence of someone needing to vent about the pain, I hope to be as wonderfully deferential as so many non-disabled friends have been to me. But the fact that lupus is an illness while achondroplasia is a not is no reason to ignore the fact that Spoon Theory perfectly illustrates the broader concept of chronic pain and fatigue experienced by people with all kinds of disabilities. Emily Brand described it eloquently in The Guardian last year:

The basic idea is that you have a limited number of spoons available for the day and each action will cost a given number of them – the more demanding the task, the more spoons would be required. The phrase “running low on spoons” can be a useful way of communicating the need for rest to fellow “spoonies” who also use this system and to friends and family who are in the know. Reading up on this is one of the best things anyone could do to help with providing day-to-day support to someone with a chronic health condition, as it’s a powerful analogy that can help people to empathise with how much of an impact even an invisible symptom like chronic pain can make.

I love the idea of “running low on spoons.” I used it just last week in explaining to a friend that I couldn’t peer with her into a store window because my swollen feet were begging me to keep off the cobblestones. But at the risk of sounding, well, confused, I’m not entirely comfortable calling myself a “spoonie” because experiences in college have left me averse to glamorizing conditions with labels that sound like club memberships. And between dwarf and has dwarfism and midget and little person and LP and short-statured and disabled and physically challenged and differently-abled, I’ve got enough labels to sort through.

 

 

Tags: , , , , , , , , ,

Doctor Tries to Be Hip And Misses

21 Jul

spine(Image by Katie Cowden used under CC license via)

 

Fifty-five year-old Terry Ragland of Tennessee recently sought medical attention for lower back pain at her local orthopedic center. She was introduced to Dr. Timothy Sweo, who ordered x-rays. After analyzing the results, Sweo concluded that the pain was caused by a curve in the spine called lumbar lordosis. He delivered the diagnosis to the patient by saying plainly, “You have ghetto booty.”

Lumbar lordosis is a severe curvature of the lower spine most visible from the side and it can be caused by a variety of a factors. “Ghetto booty” is, according to the most popular Urban dictionary definition, “a term used when you see a girl with a firm, big, tight packed ass. {Most black girls have ghetto booties}.” In other words, it’s slang for simply having a big butt.

For a medical professional to use the term is fantastically patronizing at best. For a white male medical professional to use racially-charged sex slang with a black female patient he has only met once before is jaw-droppingly gauche. His attempted apology to Ragland via letter does not help his case: “I was trying to take a technical conversation regarding your lower back and make it less technical.”

Presuming orthopedic patients are unable to comprehend medical terms like “lumbar lordosis” is ludicrous. After a month into my first limb-lengthening procedure at age 11, I could explain the difference between lordosis and scoliosis, a corticotomy and an osteotomy, and I could name every bone in the human body. I wasn’t exceptional – I just wanted to understand the world I was living in, like every one of my fellow pediatric patients. Priscilla Alderson’s excellent book Children’s Consent to Surgery presents overwhelming evidence that child patients are far more aware than adults tend to give them credit for. And Ragland is not a child.

“It says to me that he doubts what type of intellect I have, how intelligent I am to be able to understand what he conveys to me in a medical term,” Ragland told reporters.

While Sweo’s condescension comprises a particularly stunning mix of nasty prejudices, he is hardly the first doctor to speak disrespectfully to a patient. Medical specialists are renowned for being scientifically brilliant but socially inept. After making you sit in the waiting room, sometimes for several hours, they swoop in, keep their eyes on your body or the floor, bark a few questions at you, rattle off some orders for the nurse to take down, and swoop out again.  The patient is supposed take solace in the fact that it is all a sign of how important the doctor is.

Since this stereotype has become so pervasive, some medical professionals do make earnest attempts to shatter it, but their success varies. Some try through their body language and demeanor to give you the sense that they are genuinely listening and care about your all-around well-being. Others try by jamming a few blunt jokes into your narrow time slot. It gives you the sense that they’ve just watched Patch Adams and decided that being a clown is the perfect defense against being accused of coldness, so let ’er rip! Your body, your condition and your diseases are hilarious!

Years ago I attended a conference where an orthopedic specialist did a presentation on achondroplasia and said with a smile, “The short bones cause the average-length muscles on achondroplastic people to bunch up so that they look like the Michelin Man!”  He clicked forward to a slide featuring a list of achondroplastic symptoms with “Michelin Man look” featured at the top.  He was obviously very proud of having come up with this description.

I was the only person in the room with achondroplasia, and I had to kick my friend sitting next to me because he couldn’t stop giggling at the surgeon’s cluelessness. The Michelin Man?

Indeed, the most exasperating aspect of the Dr. Sweo case is that he appears to genuinely believe that his comments might have been helpful. Usually it is easier to engage in productive discourse with someone whose intentions are good than with someone who aims to hurt. But in light of his oblivious apology, it seems Ragland has a better chance of getting through to other, more perceptive doctors via the media than to Sweo via complaint.

I have lumbar lordosis.  It’s one of the primary symptoms of achondroplasia and it’s why I had to undergo spinal surgery last year.  I could have crashed this site with a list of all the off-putting doctors and healers I encountered, as well as the sarcastic jokes my closest friends came up with to keep me sane.  As Ragland files a formal complaint with the Tennessee Department of Health, there will inevitably be some backlash about PC culture gone mad and minorities being too sensitive and humorless.  But more power to her for sticking up for herself, and for patients everywhere.

 

 

Tags: , , , , , , , , , , , , , , , ,

What To Do When I Go FWOMP!

23 Jun

(Image by Stephen Alcorn © 2003 http://www.alcorngallery.com)

 

“HEEEEEY!” Friends were at the door, back fresh from a vacation that had seemed far too long for me to endure. At the sound of their dulcet voices calling me in unison, I jumped from my chair, rounded the corner, darted down the hallway toward their open arms, and FWOMP! Iwassuddenlyhorizontal.

My friends gasped, “OMIGOD, ARE YOU OKAY?!” Apparently this time I was, from what I could tell of the pain, and I bounced up before they finished asking, throwing my arms around them both at once and laughing, “How’s that for a dramatic hello?”

“You’ve been drinking again, haven’t you?” one of them smiled.

“Yes!” I beamed. “You know exactly what to say! How was your vacation?”

We chatted for about five minutes, made plans for the next day, and said hasty goodbyes because their toddler was itching to get home. As I shut the door, I rubbed my knee, looked at my partner, and shook my head. “I’m gonna have a new bruise on the left to match the right.”

Two weeks before I’d gone flying down the same hallway, but that time it had really knocked the wind out of me and left a cut needing a bandage. I had reacted a little less wryly – diaphragm spasms are never pleasant and they forced me to let out a yell that sent my partner running from the kitchen. But after my initial roar, I switched to hollering, “I’M FINE! I’M FINE! DON’T PANIC! I’M FINE!” Our guests came peeking out of different rooms, everyone asking me how I was.

I was fine, but I was mad. Mad at gravity, mad at the pain, mad at my useless tendons and weak muscles that cause me to stumble on average about every ten days. But I wasn’t that mad. I’ve gotten used to it, after all.

Because my anterior tibilias tendons on both legs were severed some time during my first limb-lengthening procedure, I use different tendons to lift my feet when I walk. They compensate relatively well, but since they cause my feet to point slightly outwards rather than straight ahead, I’m a walking accident waiting to happen. It’s compounded by the fact that my muscles fatigue more quickly than others’ due to my dwarfism. It’s been this way since I was twelve and changes only in that the bigger I get, the harder I fall.

Since I was my surgeon’s only known case of tendons severing during limb-lengthening, most people with dwarfism do not face this problem. Some do, however, when their greater susceptibility to fatigue combines with their having to carry an average-sized trunk around on exceptionally short legs. In other words, had my tendons not severed, I may or may not have had this habit of losing my balance. It’s exasperating and inconvenient, but what can I do about it?

Laugh, for one thing. Over the years, I’ve decided a woman falling down is both hilarious and revolutionary—what with the delicate ballerinas we’re supposed to be—and drinking too much is just one of many lovely excuses to offer for it. Years ago I fell while carrying an armload of water glasses and promptly ended up in the emergency room with stitches and a black eye. From the physician named Dr. Goebbels to the nurses insisting my partner leave the room so that I could be free to explain what had happened, the opportunity for sick jokes was everywhere.

Friends have kept records of my losses in the battle against gravity. Some are critical, sighing, “EMILY, that’s the second time today!” while others are cheerleaders: “It doesn’t count this time because the ground is uneven.” (And can I just point out that the German word for gravity—Schwerkraft—literally translates as “heavy force”? I love German.)

Of course, I’m not always at my best when it happens. Often I fall because I’m particularly tired and this results in my being particularly bad-tempered about it. That I kvetch the most to those I know and love the best is logical, but not entirely fair.

When my peers witness me falling for the first time, many of them don’t know what to do. I’m trying to get better at telling them. If I’m not badly hurt, but still somewhat hurt, I try to shout that I’m okay to curtail their apprehension. Taking a minute to help me up and, depending how close we are, offering me an arm until I’m steady on my feet is almost always appreciated. Breaking into a panic and giving me the sense that it’s my job to calm them down is less helpful.

Most people who have to deal with pain caused by disabilities don’t want any more sympathy or attention beyond what we would give someone with a light headache. (In fact, many of us want a tad less sympathy than what some with mundane headaches go fishing for.) If I’m not hurt, anything you say to keep the mood light as a Screw-you! to my heavy fall will be invaluable. If I am hurt, any offers to help before I have to ask will be worth even more. And if your gentle-yet-practical manner demonstrates particularly good caregiving skills, I’m going to tell you so. Experience has made me a particularly good judge.

And I’m not embarrassed when I fall, so please don’t be embarrassed for me. At best, it’s as disruptive as a mighty sneeze. At worst, it’s a mood-killer.

The one fall that still makes me cringe to this day happened as I was stepping off a stage after delivering a poem to thunderous applause. I spent the summer before my senior year of high school at a young writers’ workshop in the Berkshires, where I found all the beauty, intellect and acceptance I been dreaming of ever since I first put pen to paper. Reciting one of my pieces to giggles and cheers made me feel as great as anyone on any podium has ever felt. The moment had been just perfect. And then, I slipped. The handsome emcee looked sincerely concerned: “Are you okay? Are you okay?” He had to keep asking because I was mumbling my answer, mortified to even acknowledge what had just happened. In my head I was begging everyone in that room, Please remember my poem and not my fall. Please.

Then again, “And Emily came tumbling after” is a poem in itself. It doesn’t work as well in Germany, what with no one having grown up with Mother Goose, so I’ll have to settle for the joke about being drunk. That one’s an international success.

 

 

 

Tags: , , , , , , ,

Lessons Learned From A Laminectomy

2 Dec

Sippy Cup Forgotten

(Image by Randy Robertson used under CC license via)

 

Five weeks ago I had a spinal surgery to relieve compression brought on by my achondroplasia.  I took a break from blogging because, first of all, I’ve only recently been allowed to sit for longer than an hour or two, and secondly, major life interruptions are almost always best discussed from hindsight.  (Even though the personal usually ends up being political, this blog is not and never will be a tell-all of how high my temperature is or how my incision looks today.) 

I will confess that the hardest aspect was the lack of community.  No one at home or in the hospital had the same condition I did.  Since several of my readers have achondroplasia or children with achondroplasia, and I myself was ravenous for any sort of information I could get my hands on, here’s a synopsis of the past five months:

One night in July, I noticed I couldn’t sleep on my stomach without the muscles in my left thigh and hip burning with pain.  I took some Ibuprofen and applied a hot pack but to no avail.  Within a few days, the burning sensation expanded up into my lower back and deep in my backside.  It came whenever I lay on my stomach, lay on my back, or walked more than a few yards.  Strangely, it disappeared when I was sitting up straight.  I had to sleep propped up on pillows to keep the pain at bay and woke up during the night whenever I curled into a new position.  I described it as sciatica – which is, apparently, just a name for a set of symptoms and has various causes.  Maybe sleeping for five nights straight on a friend’s uncomfortable couch had done it?  My doctor gave me a prescription for physical therapy and stronger pain killers, but the medication had no effect and, after three weeks of physical therapy, the symptoms only got worse.    

By the time I met with an orthopedist, the burning began to be replaced with a pins-and-needles sensation that ran all up and down my left leg and worsened with walking.  Once again, it disappeared whenever I flexed my hips.  While the therapists tossed out the usual suspects for usual patients—disc herniation or degeneration, etc.—my family and I had begun to suspect achondroplastic lumbar spinal stenosis.  People with achondroplasia are at high risk for this because our spinal columns are exceptionally narrow and become acutely so with age.  The symptoms described in the medical literature on achondroplasia exactly matched mine.  Between one-quarter and one-third of all people with achondroplasia develop stenosis, usually in their 20s or 30s, and I was a perfect candidate.  Average-sized patients with stenosis are usually encouraged to turn to surgery only as a last resort, but achondroplastic patients almost always require a laminectomy.  And, according to most specialists I’ve spoken with, the sooner the better.  

I hate having surgery.  Talking with the anesthesiologist about all the medications I’m allergic to brought back all sorts of unpleasant memories.  But I eventually got in contact with an excellent team of neurosurgeons who were very informed and reassuringly confident that a laminectomy (without spinal fusion) would be the best defense against permanent paralysis.  And with my 13th operation now behind me, I know several things I didn’t before.

I learned that, unlike orthopedists, neurosurgeons cannot tell you at what time your surgery will take place until the day of, if at all, because emergency cases such as strokes and spinal cord injuries take priority.  Your surgery could be postponed by such cases more than once, as mine was.  It is surreal to find out you just spent a whole day without food or water for nothing, while also finding out the people who knocked you to the back of line are probably fighting for their lives.  

I learned that, contrary to what I had assumed, you wake up after back surgery lying on your back.  I was especially grateful for this after my partner pointed out that I had a black-and-blue mark on my cheek from lying on my face for the two and a half hour procedure.

I learned that the day of surgery is one of the easiest.  Waking up in the recovery room and discovering I could cope with the pain and seeing myself wiggle my feet sent waves of relief everywhere.  Seeing my husband waiting for me in my hospital room was thrilling.  And the drugs took care of the rest.

After that, however, each day threw a new curveball, whether it was the pain of moving, the vomiting that came after moving (typical for spinal patients), or the dilemma of never wanting to go to the bathroom because it destroyed whatever comfort I had finally found.  Unlike the patients whose stenosis had been caused by disc herniation, I could not walk without a walker after surgery and managed no more than baby-steps.  As with limb-lengthening, I learned to take it week by week in order to see that progress was happening, however slowly.  By the third week, the worst pain was gone and I could walk short distances without any assistance.  (After five weeks, I can now manage a few blocks, though it takes me twice as long as it used to and my balance remains fragile, so I like to avoid crowds.)

I learned that after spinal surgery, walking and lying down are good for you.  Sitting and standing are bad for you.  I can’t remember the last time I watched so many films in such a short time.

I learned that sippy cups are perfect for drinking when you have to lie flat on your back.  They make you look ridiculous/adorable.

I learned nurses are among the hardest working, strongest, most fearless people in the world.  No one whose work is free of analyzing other people’s vomit and urine can say otherwise. 

I learned (once again) that there is always someone at the hospital about to go through something a lot worse than what you’ve endured.  Hospitals have a bizarre way of inundating you with more self-pity than you’ve ever felt before and, at the same time, more sympathy for others than you’ve ever known before.

I learned that as an adult I could see how much skill and patience goes in to being a great caregiver.  When you’re a child, you expect—and should be able to expect—your parents and relatives providing unconditional support and tolerance for your needs and your bad moods.  When you’re an adult, you’re more likely surrounded by friends and partners; people who choose to check in on you and listen to you and soothe you for three hours straight and accompany you to the doctor and run errands for you and reach things you can’t out of their own free will.  You begin to understand the sacrifices your family made and those your true friends are making.  Just because you don’t deserve the raw deal you’ve been given doesn’t mean you deserve to take their patience or attention for granted.  No matter how bad you think you have it, always, always say thank you to whoever is being kind to you.  (And take a break from whoever isn’t.)

So now I have a new scar and hopefully I’ve helped flood the web so that googlers can find information about “achondroplasia spinal stenosis” more easily.  In my experience, seeing what you’ve learned, what you’ve been humbled by, is the whole point of having scars.

 

 

Tags: , , , , , , ,