My sister had a condition called “aromatic L-amino acid decarboxylase deficiency”.
Mouthful, right? It’s also exceedingly rare: something like seven other children had been diagnosed with it back when she finally got the diagnosis at age 12. The condition is so unusual that no pharmaceutical company would ever invest in studying it.
It’s a pediatric neurotransmitter disorder, but from when she was born until her death at age 17, it was easier to just say she had cerebral palsy – a blanket diagnosis given for many neurological disabilities caused by damage to the brain around the time of birth.
Cerebral palsy can look like anything, from a bum hand to full paralysis. My sister was unable to walk or talk, was incontinent, and could not hold her own body up, even her head – a perpetual infant.
We always knew she was not long for this life. Doctors had told my parents she would not live past seven. But I guess the power of an Arab mom’s love and resilience gave her 10 extra years.
They were not easy years. Growing in this way, lengthening without building muscle tone, resulted in a deformed skeleton. She had extreme scoliosis and her esophagus (where food goes in) and trachea (where air goes in) were completely misaligned. The strain to get air caused her lungs to weaken. By her teenage years, she was on a suite of respiratory medications and a regular flow of oxygen; she almost constantly needed to have mucus manually suctioned from her throat, and my mom had become adept at administering chest PT at least twice a day (which could maybe be compared with a slightly more violent version of a Thai massage). Eventually, when the choking hazard became too prominent to ignore, a feeding tube was implanted in her belly.
Every step in this journey was a fight with our health insurance. I remember my parents changing insurance companies four times in as many years in a desperate attempt to get my sister’s needs approved. They had to upgrade their plans to the most comprehensive options, going beyond the plan provided by my father’s work to include full coverage for durable medical equipment – from an oxygen generator and oxygen tanks to nebulizers and suction machines, to name just a few. They were paying hundreds of dollars a week in premiums.
But even with the best insurance, each item took about six months to a year to get approved. Insurance would cover seemingly random things, and inconsistently. It covered the cost of her oxygen monitor, but only so many tanks. My mother became adept at the appeals process, all the while pleading with each new letter or phone call: “How is it not a medical necessity for my daughter to breathe?” This isn’t even mentioning things like adult diapers, nasal cannulas, that gel used to keep people’s mouths hydrated when they can’t drink water – much of which my parents paid for out-of-pocket, something they were blessed to be able to handle financially, though only barely.
While my sister’s existence surpassed her doctors’ expectations, we still knew this couldn’t last for ever. The dystonic spells were in check thanks to a perfected alchemy of seizure medications, though they still popped up occasionally, threatening the entire delicate, house-of-cards ecosystem. We were sure she would eventually die of respiratory failure.
Except that’s not what happened.
My sister required a custom-made wheelchair, one designed to safely secure and support her torso, hold up her head in a way her neck could not, and keep her breathing, while being gentle on her skin to prevent sores. It also needed to hold two tanks of oxygen, her suction machine, her pulse oximeter and a decent reservoir of other supplies.
It was a behemoth of a chair, considering it wasn’t even motorized. It didn’t fit in a normal-sized van, though thankfully, state resources helped a bit with that. My parents bought a used conversion van out-of-pocket for about $16,000, and the New Jersey division of developmental disabilities provided the extra $8,000 to install a lift and brackets.
This chair was a beast that prevented us for years from using an airplane (accessibility in air travel is a rant for another essay), but it gave her, and us, a life. We took her to Disney World, to Niagara Falls, to Virginia Beach and Wildwood thanks to that chair. We even took that thing on a cruise, though I think my parents would say in hindsight they wouldn’t really recommend it (accessibility in boat travel is also a rant for another essay).
Regardless of her limitations, we do know one thing: she laughed. She smiled. She knew how to express her joy.
Nearly 20 years after her death, I like to speculate that she was a fully formed mind behind that underdeveloped tongue muscle. That she heard and understood everything that was said around her, that she wished she could contribute but maybe appreciated being included – such as when my grandfather would sing to her, when we would take her hands and dance with her, when we would make funny faces and noises at her.
We ultimately decided the threshold of her life by the measure of her laugh. When it became clear to us that her breathing was unstable enough to consider a trach tube, we decided as a family that that was our limit. We signed a DNR (do not resuscitate) form and left the rest to God and the tireless care of my mother’s strong hands and stronger heart.
Every few years, my sister’s chair needed to be upgraded. She was a growing girl, after all. A chair that’s required to fit like a glove out of medical necessity must continuously evolve.
The last chair she sat in was given to her when she was 13. Within a year, she was starting to grow out of it; she began developing pressure sores where her ribs, not sufficiently coated with muscle or fat, rubbed raw against the foam paddings. Knowing how expensive these chairs run, her doctors would recommend seat upgrades, but even those were a fight. My mother ultimately had to appear in court to get a new seat cushion, and even that was not exactly what my sister’s care team had deemed sufficient.
When she was 15, her doctors determined that a new wheelchair was medically necessary. They wrote the prescription. Unfortunately, her insurance company did not agree. I mean, why would they? This thing’s the price of a car, a whole ten to twenty thousand dollars. That would utterly ruin the corporation, and we wouldn’t want that.
At the age of 17, she finally got approved for a new chair. And my goodness was this thing a beaut! The black fabric wrapped around the fresh, plump foam and gel molds was shining. The metal frame was a cool marbled electric blue. It made the old one look like some junkyard scrap metal creation out of Mad Max.
She never sat in it, because it was too late.
A few weeks before this chair arrived, my sister developed a sore on the small of her back, right under the line of her diaper. By that point in her life, all antibiotics had stopped working, as she had developed a complete resistance to them. The feeding tube also created a messy, loose situation with her bowels, which made it hard to keep the wound clean.
As diligent as my mother was, the sore just got bigger and bigger, deeper and deeper, more and more unruly, until one day – I was home with my mother that day, having just quit my previous job and awaiting the start of my next one – I went in with her to help change my sister’s diaper. As always, my sister was propped up on her side to assist her breathing, her legs resting one on top of the other. When I lifted one leg, I saw the tell-tale signs of lividity, something I learned about in my days working an ambulance in college. Lividity is when blood starts to pool under the skin, gravity taking over, and it’s a sign of death. I knew, in that moment, this was the end.
I told my mom to call an ambulance. When we got to the hospital, we were told her kidneys were failing. She was dying of sepsis.
I asked to take her home. My parents weren’t really in their right mind any more, so the healthcare worker I never became took over. The version of me who compartmentalizes in order to solve crises took the wheel. I coordinated with the doctors and nurses to set up home hospice and stayed with my mom and sister overnight while the staff sorted it out, demanding that they stop food, which I recognized would only cause her further pain at this point – food meant she’d need to defecate, risking painful contamination of a dreadfully open wound – and torturously delay the inevitable.
The nurses were very uncomfortable with this, because they needed to justify an overnight stay to insurance companies. They needed to show they had done something. Even if that something was counterproductive. Even though my sister had a DNR and was entering the hospice system, I still had to sign a form explicitly saying that we were refusing treatment.
The next couple days were surprisingly quite beautiful. In Muslim tradition, when a loved one dies, people gather en masse around the family for three days, an aaza. It’s a little like a Jewish shiva, though perhaps more chaotic. People show up at all hours, bring hoards of food, and most importantly, provide emotional support. My sister got a pre-aaza, which might sound bad but it wasn’t. Set up in her hospice bed in her room with all the equipment and weird stains on the walls from years of medical flotsam, we gathered around her, and the community gathered around us. Tens of us, all saying goodbye together.
In private moments, I whispered my penance in her ears, for sins of resentment I will never know if she’d witnessed or understood. I made promises I don’t think I kept. But there was a hint of a smile on her lips when I said her name.
The morning we brought her home from the hospital, while hospice workers were setting up my sister’s room for her last days, my mom received a call from a delivery worker saying that my sister’s new wheelchair was being delivered that day. My mom asked if they could take it back, but they couldn’t. Wouldn’t you know it, it was already paid for. Mama didn’t let it in the house. She refused to even look as she directed the drivers to leave it in the garage, tucked into a back corner of our home and our minds, until we eventually donated it back to my sister’s school.
When I stumbled upon it a couple of weeks later, meandering through the now too-quiet house – no more beeps from her pulse-ox monitor, or steady clicks from her feeder, no more low hum from the oxygen generator, or ever-unsteady rumble of her breaths coming through a baby monitor – I felt like a bowling ball had landed on my chest. There it was, all shiny and new. It took all this time, all this fighting to get it, and now it was useless. It didn’t come in time to prevent what doctors warned, insisted, would happen, because an insurance company didn’t think she needed it.
We’d been so sure her lungs would one day give out, but instead a simple wound felled her. A wound that was in fact caused by negligent insurance practices. God only knows how long she would have lasted had she not developed that stupid little sore. She’d already lasted longer than anyone expected.
We only know Sarah – for that was her name, Sarah – loved to laugh, and that a faceless insurance company decided it was OK to go against a doctor’s order in order to line their pockets and increase their bottom line.
Playing God, they decided her fate.
