Tag Archives: Surgery

Can We Understand What It Is Like To Hear Sound for the First Time?

17 Jan

listen(Image by Jay Morrison used under CC license via)
 
In the 1990s, Cristina Hartmann was one of the first of a few hundred deaf and hearing impaired children in the United States to undergo surgery for a cochlear implant. She has written extensively about the experience of hearing sound for the first time after the implant in her right ear was activated, most recently this month on Quora.com:

My mother was the one who told me, “Raise your hand when you hear something.” That statement left me baffled. What was I looking for? It was a bit like searching for Waldo when you didn’t know what he looked like.

In that tiny, windowless room deep in the large Manhattan hospital, the audiologist began tapping away at her keyboard. Everyone stared at me, even a woman standing in the doorway whom I had never seen before. I felt the heavy weight of expectations on my shoulders. I had to do something. I concentrated very hard, searching for the mysterious, indefinite Waldo. Whenever I felt anything, an itch or a breeze, I raised my hand slowly, searching everyone’s expressions for whether I had gotten it right or wrong. Nobody gave me any confirmation, so I went on guessing. Twenty-five years later, I realize the whole thing was a show that I performed. I knew this was a momentous event, and I didn’t want to disappoint….

As a congenitally deaf child (who was a bit long in the tooth at 6), I had never formed the neural pathways for my brain to even begin processing auditory stimulation. In the fashion of the ostrich, my brain ignored the strange stuff, and I remained as deaf as I had been an hour prior…

It took months and plenty of therapy for her brain to adapt. Thirteen years later, the activation of a second implant, this time in her left ear, proved a more harrowing experience than the first:

As the audiologist began the beep sequence, I burst into tears and involuntarily clenched the left side of my face. She looked up, puzzled. “Why are you crying? You’ve had this before!” she said. The pain was like sparklers going off on the left side of my head. The stimulation, as little as it was, completely overwhelmed me.

Even though I had already laid the neural pathways for auditory stimuli for my right ear, my brain was unprepared for the stimuli coming from the left side. Since my brain had already experienced this type of stimuli, it could process it, but it was still sensory overload. That stuff hurts. It took me months to acclimate myself to the new implant, but in the meantime, I cringed every time I turned it on. As I said, laying new neural pathways takes work.

Hartmann was later told by the mother of another patient, “Once they started with the beeps, [my daughter] screamed and cried.”

Such narratives exist in stark contrast to the YouTube videos of newly activated implant users laughing and smiling—and, in one case, crying for joy—that have been bouncing around the Internet with far greater frequency. While both narratives provide important information for those considering cochlear implants for themselves or their children, they are also an important contribution for the greater public in our understanding of what it means to be deaf.

It makes sense that crossing out of the world of silence into the world of sound is just as disorienting as its opposite. A hearing person with a middle ear infection strains to perceive the sound of speech, and a deaf person with a new cochlear implant strains to tune out noise pollution: the knocks of a radiator in another room, car doors slamming on the street, wind, footsteps, not to mention the countless background beeps and clicks of the Digital Age. After all, when a baby leaves the womb, she does not instantly adapt to her new home. She comes out crying. There’s too much light and not enough warmth. And, if she is not deaf, there is too much sound.

Speech is no less difficult to learn than Sign language, just as English is no less difficult than Chinese. The ease with which we learn one form of communication or the other depends entirely upon our personal experience and place in the world. For those of us who have grown up hearing speech, the viral videos communicate something very different than for those who grew up in Deaf culture.

While the experiences of utter delight portrayed in the videos are valid, their popularity contributes to an oversimplification of the issue. Watching a toddler smile upon finally hearing his mother’s voice for the first time sends a very strong subliminal message: Being deaf must be worse than not being deaf, and therefore anyone would want to join the world of the hearing. But the general public as an audience is already biased toward the hearing world’s standards of happiness. We are moved by the sound of loved ones uttering our names but not at the image of them signing our names because our culture does not rely on—and therefore does not highly value—Sign language.

This what inspired Lalit Marcus, the daughter of deaf parents and an active promoter of Deaf culture, to pen an article for The Wire titled, “Why You Shouldn’t Share Those Emotional ‘Deaf Person Hears for the First Time’ Videos”:

I want to make it clear that I don’t have a problem with people who choose to get cochlear implants. Medical decisions are painfully personal… I’m all for people making the health choices they think are best for them. What bothers me are the maudlin videos produced out of someone’s intense, private moment that are then taken out of context and broadcast around the world. What bothers me is how the viewer never learns how the individual came to the decision about their implant, which factors they took into account, whether their medical insurance covered it. Sometimes we don’t even learn their names.

This gives me pause. I consider the clip of me removing my casts to look at my newly lengthened legs, which featured 15 years ago in the HBO documentary Dwarfs: Not A Fairy Tale and last year on Berlin’s public station. The moment was simply joyous—as was the moment I stood up, let go of my friend’s hands and took my first steps—but the story behind it was abundantly complex. Which hopefully both documentaries portray.

I have endeavored to communicate that through this blog and all the media work I have done for the past 20 years.

Limb-lengthening and cochlear implant procedures are markedly different in several ways. Limb-lengthening, for example, does not threaten to endanger another language. But it does threaten to break ranks in the dwarf community through the controversy of altering versus accepting extraordinary bodies. Both procedures have proven to evoke vitriol among their proponents and detractors.

Hartmann reveals:

Most of my deaf friends were good about my CI. They didn’t mind it, except for the fact that my speech therapy cut into play time. That being said, people in the Deaf community felt free to make pointed and derisive comments about my CI. I still get these comments, even almost 24 years after my surgery. To some, I’ll always be a CI-wearer and a turncoat.

The CI advocates aren’t any better, if not worse.

I have very pleasant relationships with many parents of implanted children and CI users. I, however, have also been called a failure because I still use [American Sign Language] and don’t speak perfectly. I’ve also seen a mother run across a room to prevent her child from signing to another deaf child. I’ve been scolded for making gestures and looking too “deaf.”

The debate, of course, is ongoing.

But for those of us not faced with opting for or against a cochlear implant, we are faced with the challenge of overcoming our bias and remembering that Deaf culture is no less valid than the hearing culture we inhabit. Especially when those admittedly tantalizing videos wind up in our Facebook feeds.

 

 

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.

 


New Rights for Intersex Newborns in Germany

25 Aug

Germany has become the first country in Europe to allow parents to check one of three boxes for gender on their child’s birth certificate: “male,” “female,” or “blank.” The new option is intended to accommodate the parents of intersex newborns; i.e., those whose reproductive or sexual anatomy does not appear to fit the traditional definitions of male or female. The children will be allowed to choose “male” or “female” later in life, but they will not be required to. This will all go into effect November 1st.

While the law says nothing about gender ID in passports, equality activists are celebrating it as a tremendous step forward. According to Silvan Agius of the International Lesbian, Gay, Bisexual, Trans and Intersex Association, the European Union has been slow to act on issues of gender identity. “Germany’s move will put more pressure on Brussels,” Agius told Der Spiegel. “That can only be a good thing.”

However, not everyone in the intersex community is celebrating the idea of a third gender box. Creating a new category, they argue, is to give in to the idea of narrowly defined categories. Instead of turning the gender binary into a triad, why not loosen the definitions of “male” and “female” to include those with all sorts of bodies? Many people with intersex conditions have a perfect sense of belonging when it comes to gender – they only feel alienated when others insist they don’t belong.

And while they often cooperate politically, intersex people should never be confused with transgender, transsexual, or genderqueer people. The Intersex Society of North America states, “Most people with intersex conditions come to medical attention because doctors or parents notice something unusual about their bodies. In contrast, people who are transgender have an internal experience of gender identity that is different from most people.” The ISNA’s history of intersex offers much information about the long medical tradition, and resulting problems, of conflating and confusing the two.

Professor Alice Dreger explains that cases wherein intersex individuals also qualify as transgender because they elect to transition from the gender assigned to them at birth—this is essentially the plot of the 2002 Pulitzer Prize-winning novel Middlesex—are quite rare. Dreger notes, “Far more often, the concerns of intersex and transgender people represent opposite sides of the same coin: intersex people get surgeries they don’t want, and transgender people can’t get the surgeries they do want.” The surgeries many intersex people regret having undergone in childhood are primarily cosmetic, removing or adding tissue only for the sake of “normalizing” the appearance of their genitals, and at the expense of sensation and function.

Another all too common problem is the concealment of the patient’s intersex condition by her parents and doctors, leaving her unaware for as long as possible and discouraged from asking the questions she might begin to have about her anatomy. In her essay “Twisted Lies,” Sherri G. Morris writes that not finding out until college that you are without a uterus can be rather upsetting, to say the least.

As for the well-known term “hermaphrodite,” it is inaccurate at best and defamatory at worst. The word represents the idea of one person being anatomically both male and female, and this idea is a purely mythological one. Because it is physiologically impossible. As Dreger points out:

…the only way you could be born with “both sets of genitals” is if you had two bottoms. The clitoris and the penis are homologues—they are the same organ developmentally—so you get one or the other, or one in-between organ. Similarly, the labia majora and the scrotum are homologues—so you get either a set of labia majora, a scrotum, or something in between. But you can’t have all the female parts (clitoris, labia majora, etc.) and all the male parts (penis, scrotum, etc.) on one person…

What people mean when they say a person “was born with both sets of genitals” is that a child may be born with a phallus that looks a lot like a penis plus a vagina (the tubular organ that goes from the outside of the body towards the uterus, if there is a uterus). This can happen because of hormones, in conditions like congenital adrenal hyperplasia and partial androgen insensitivity syndrome. But to say that gives you “both sets of genitals” is to pretend that somehow all that matters to males is their penises and all that matters to females in their vaginas. In fact, many of us women also care about our clitorises. (For that matter, many men care about their scrotums.)

Unfortunately, sick fascination with the hermaphrodite is utterly pervasive today. Comedians of all stripes, from South Park to Flight of the Conchords, have yuk-yukked over the idea of a person with both sets of genitals being able to have intercourse on their own, while artists have done their fair share of poking at and playing with the myth. (See here for an intersex woman’s take on Middlesex.)

On this issue the ISNA is emphatic: “The terms [‘hermaphrodite’ and ‘hermaphroditism’] attract people with sexual fetishes and fantasies that, frankly, we as a patient advocacy organization are not interested in hearing from.” They therefore advocate expunging any terms related to “hermaphrodite” from all medical literature:

We think it is much better for everyone involved when specific condition names are used in medical research and practice… While some intersex people seek to reclaim the word “hermaphrodite” with pride to reference themselves (much like the words “dyke” and “queer” have been reclaimed by LBGT people), we’ve learned over the years it is best generally avoided, since the political subtlety is lost on a lot of people.

Meanwhile, in an Op-Ed piece appearing yesterday in Spiegel International, Agius argued, “…real progress for intersex people is not measured through the number of available labels but through an end to the human rights breaches currently being inflicted.”

Indeed, the new German law is just the tip of the iceberg. Considering that one in every 2,000 infants is born with an intersex condition, shame-induced secrecy continues to be an abysmal problem. The rights and concerns of those with intersex conditions receive far too little attention. (I was completely uninformed until I met Dreger ten years ago at the conference Surgically Shaping Children.) Whatever the legal specifics, Germany’s new law will hopefully promote awareness above all else, and in more ways than one.

 

 

 

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.