Chapter Five: Ouroboros
Chronic conditions do not necessarily manifest as pain 100 percent of the time, or even a majority of the time. The uncertainty of one’s body being fully functional one day to completely shutting down the next can be just as debilitating.
My friend Emma suffers from an autoimmune disease called idiopathic thrombocytopenic purpura (ITP).
With autoimmune diseases, it is as if the body betrays itself. ITP is a blood disorder that occurs when one’s immune system mistakenly attacks one’s own blood platelets, resulting in extremely low platelet levels.
ITP can be either acute, as in resolving on its own within a few months, or chronic, lasting longer than six months. The former is more common and mainly occurs in young children, while the latter is more prevalent in adults and generally requires long-term treatment.
For Emma, ITP has manifested as severe bruising and nosebleeds. She has experienced a lot of joint pain as a result of not the disorder itself, rather, the treatments meant to address it.
“I have taken steroids for it,” she said. “I was on this bone marrow stimulant which caused a lot of joint pain. Then this past summer, I did a round of immunotherapy infusions which were pretty painful, just a lot of joint pains, nausea. The symptoms lasted for a couple of months post-infusions.”
In addition to those side effects, she also experienced a full-body allergic reaction to the first infusion.
At the time of our conversation, she had been off of any ITP-related medications for several months. “As of right now, I'm not on any medication for the disorder, so I've been feeling a lot better. But when I was taking daily medication, I had horrible joint pain. I was always tired.”
Emma first started noticing symptoms of what she would come to learn was ITP relatively recently. Similarly to Madi, this all happened within the time that I have known her.
“I had just moved into my apartment my sophomore year, and I just started to get bruises literally everywhere, and I wouldn't be bumping into anything,” she said.
Suspecting an iron deficiency, she ended up getting her blood taken at urgent care that August. After seeing the results, the technician urged her to visit the ER as soon as possible.
The normal range for blood platelets is between 150,000 and 400,000 per microliter of blood. Emma’s first blood draw returned just 10,000.
She received the ITP diagnosis right away and was put on steroids, to which she responded well. Things improved for a few months, but, due to the nature of the autoimmune disease, getting sick made her platelet count drop again.
“I got a cold, and my levels dropped again. And so then I did a round of separate infusions, which didn't work,” she recalled.
What followed from that round of infusions was a frustrating, drawn-out process of her doctors testing for, and ruling out other illnesses, only for the doctors to settle on chronic ITP.
“They thought I had Lupus for a little bit. They thought I had leukemia, so I got testing for all of that. And then once they ruled other things out, they were like, ‘Okay, this is definitely ITP, and probably chronic.’”
“I kind of had an issue with my doctor, because they didn't rule out leukemia as quickly as they should have,” she said. Over the course of four months, Emma repeatedly asked if leukemia was a possibility. The doctor said it was unlikely, but held off on testing for it for months.
“I ended up switching doctors; I was in the pediatric section, and then I switched to adult hematology,” she recalled.
The adult hematologist informed her that she should have been tested for leukemia immediately. It was not until December, four months after her first hospital visit, that Emma was able to get a bone marrow biopsy.
Her previous doctor had also dropped the ball on genetic testing, Emma discovered, which, after switching doctors, revealed that her platelets also had a protein deficiency.
“The platelets I do have, they're not even at full power. Even when I have a normal amount of platelets, I still have bleeding and bruising symptoms.”
Emma has since undergone additional rounds of steroids. That medication is a quick fix to get her levels back up, but comes with severe side effects: “I can't sleep, I get very hungry and angry. I just hate them.”
After more trial and error with infusions, she ended up on a bone marrow stimulant that caused her most severe joint pain symptoms.
“I worked as a catering waitress at MDining, and I would not be able to hold trays because my hands would just hurt,” she recalled. “I was on that medication for six months at the highest dosage. And then I switched doctors, and they said, ‘This medication is not going to work long-term.’”
Not only does she have to live with the uncertainty of a chronic autoimmune disease, all of the treatment options available to her come with financial, physical, and emotional costs even more severe.
“I think the infusions I got this past summer were $16k per infusion, and I think I paid probably $200. I'm very grateful, but it definitely opened my eyes to how expensive it is without insurance to get health care.”
Those infusions were successful, and, save for monthly blood draws, she has not yet needed to undergo another treatment.
Still, there remains a great deal of uncertainty surrounding her condition. “I could be in remission from anywhere between a year to my whole life. It just depends on the person. If I were to ever get pregnant, that would cause a flare-up.”
“When my levels are low, there's just a lot I can't do normally. I can't take any sort of blood thinners or ibuprofen, which sucks, because Tylenol doesn't really do much,” she said. “When my levels are low, I can't really fly on a plane for too long because it might cause a brain bleed.”
“I'm kind of nervous to travel to countries where English isn't the primary language. My doctor told me about this one friend that she had that went to a foreign country, and he had what I have, and he got hit by a motorcycle or something, and he died on the street because he couldn't communicate what was happening.”
Besides those cases, anxiety and paranoia over the “what-ifs” in her day-to-day life pervades.
“Now that I'm not experiencing the extreme joint pain, I feel much better,” Emma said. “I definitely feel more relaxed, day-to-day now, but I'm definitely more aware of, ‘What if something happens?’”
“With crossing the street, when my levels are low, I'll think, ‘Oh my God, if I get hit by a car, I could just bleed out and die.’”