Chronic Immune Thrombocytopenia

Of all the tools I’ve learned in cognitive behavioral therapy (CBT), “reframing” might be the one most valuable to me as someone with chronic illness . There are so many labels ascribed to disabled and chronically ill people that imply a fusion of destiny with diagnosis. Many of these labels, while they may be useful to medical professionals for diagnosis and treatment, reduce the lived experiences of ill people to black-and-white binaries. In CBT terms, what’s a black-and-white binary? That’s right: it’s a cognitive distortion! Labels can be useful, but sometimes hearing them repeated over and over only serves as a barrier toward fulfilled, meaningful living. I want to “talk back” to these words that are plastered all over my body and life. I want to reframe the intractable, and be the impossible. 1. Incurable. The disease I live with, immune thrombocytopenia , is technically incurable. It is a relapsing and remitting disease. I have a chronic, severe, treatment-resistant form. If people with autoimmune disease experience permanent remission, it’s because their bodies righted themselves, not because they were “cured.” It is difficult to think of being saddled permanently with this thing you don’t want, this burden, like you’ve been branded with a hot iron against your will. Being told you have an incurable disease, whether common or rare, minor or life-threatening, involves a shift in the way you see yourself. But maybe there’s another way of looking at it. What is it I need to be cured of, really? Perhaps there is a difference between a cure and healing. Perhaps internal healing is actually possible without a cure. Maybe there are ways I can heal myself, and even the world, that are open to me regardless of whether medicine ever finds a “cure” or not. Maybe there are relationships I can have despite chronic illness that touch deeper parts of me, that make any pain or sickness I feel pale in comparison to what I can transform through care and commitment. 2. Impaired. Before I got sick, I felt like I could do anything. I was extremely high-functioning, active and successful at my job. I was planning on hiking the 220-mile John Muir Trail the summer after I got sick. I say this not to boast, but to underscore how much of a change I have seen in myself since being under the influence of medications that mess with how my brain works, and the sinking feeling that long-term psychiatric problems are affecting my ability to think clearly. I have a verifiable cognitive impairment, as well as physical tiredness and reduced stamina that is readily apparent to anyone who knows what the “old” me was like. I went from being able to hike 12 miles a day to having trouble with two. It’s difficult not to look at myself as damaged goods sometimes. I miss having the drive and ability to accomplish things. Impairments can feel like chains, holding you back from what you ought to be able to achieve. But, on the other hand, I have no way of knowing that a life without physical impairments is actually a freer life. True, I would have fewer doctors’ appointments, fewer bruises, less pain, more travel, more career advancement. But I would also have more obligations to continue advancing in those directions. The inertia created by increased ability would bring with it its own limitations. There is no alternate reality in which I will not experience difficulty and pain of some sort. I would have kept doing many unhealthy things in my life out of a sense of momentum and responsibility that, in the end, probably weren’t actually worth it. In some ways, being sick has set me free. I am free to contemplate how I am valuable as a living being without the expectations of society determining my value based on what I can accomplish. 3. Disordered. I hate this word, disordered. It feels dirty, like greasy hair sticking to your eyes, or stumbling down a road you don’t recognize in the dark. How I would hate to be disordered … and yet I have so many of these diagnoses ascribed to me. Generalized anxiety disorder (GAD) . Persistent depressive disorder (PDD). Bipolar disorder. Unspecified neurocognitive disorder. Other specific trauma -related disorder. Autoimmune disorder. Blood disorder. The list goes on. One begins to question whether there is any part of my body and mind that is functioning correctly. Disorder means pain. Disorder means chaos. Disorder means out of balance, swinging back and forth outside the bell curve and smacking your face against the bell tower. When I see the litany of disorders, I need to remind myself of the ways in which I am very ordered. When I feel pain or nausea or tingling in some part of my body, it helps to focus on a part of me that does not have that sensation, and live in it, like a comfortable nest in a stormy winter. There are also ways in which I am extremely ordered. The way in which I cope with my illness, for example. I tackle that like it’s a full-time job. That’s no small feat. Organizing illness and chaos into mindful, manageable living is creating a beautiful, elegant order all on its own. My love of language and animals, the ability to taste what is sweet and bitter, the feeling of soft cotton on my skin, all of this speaks to an orderly, functional dance of desire, experience and sensation. 4. Unresponsive. I am not completely unresponsive to treatment. Most treatments, I am “partially responsive,” but I know many others who will be hit with the “unresponsive” label. For autoimmune diseases, there are often a plethora of treatment options available, none of which are guaranteed to work. The only way to find the right one is to try them, one by one, living through the side effects and negative reactions, waiting to see if relief comes. I have yet to find that relief. I’ve found partial, temporary relief many times, but at a cost. Treating your own body like a guinea pig in a medical experiment goes against your self-care instincts. It feels like every time I take a risk, only to hear that disappointing result, “unresponsive,” that I’ve just injected myself with poison for no reason. This is hard to reframe. But it helps me to realize that actually, my body does respond each and every time. The reason certain treatments might not work is because my organs are filtering out the foreign chemicals too well for them to work. My liver and kidneys are champions, my body responds to damaging substances by fighting against the intrusion. So, even if the desired result isn’t what I had hoped for, I’m hardly lying there passively. There’s a war going on, one my body is actively engaged in fighting, responding to changing circumstances every day. I am not unresponsive, just unpredictably responsive. This just means I am alive. 5. Dependent. It sucks to think I am dependent on treatment to live. Whether it’s a synthetic peptide or donated plasma products, I’m dependent on regular monitoring and some kind of medication injected into my blood on a regular basis to stay alive. This is sobering. It means I’m dependent not just on modern medicine, but on health insurance, and the whole societal system that keeps such expensive intervention possible for an individual like myself. It certainly makes me feel fragile and childlike, as if I have an umbilical cord to the womb of society I cannot cut. This one is probably the hardest for me to reframe. Ironic, isn’t it, because I do live alone and have a job, albeit with accommodations — two criteria our society believe are necessary for one to be “independent.” By all objective measures, I am actually doing quite well for myself. But I just can’t shake the feeling one mishap could ruin it all. I feel dependent on the good graces of Fate herself some days. I guess the only thing that really helps with this one is to remind myself we are all dependent. There is no one alive who does not depend on other human beings for communication, food, protection and a myriad of other needs. I may have unique needs, but I am hardly the only human being alive who has needs. I am certainly not the only one who is dependent on modern society to stay alive. In many ways, I have independence in the form of knowing advanced first aid and having very good research skills. So perhaps, even though my dependence is physically quite striking, rare, dramatic and very financially apparent, there are other ways in which I am more independent than others. Being medically dependent also does not preclude a healthy interdependency with others. Our culture values autonomy to such an extent we sometimes forget “ nemo est insula ” (no one is an island). Seeking relationships of kindness for the exchange of care, goods, services and healing is not only necessary, it can be deeply satisfying. As human beings, our connectedness to the communal need is what propels us forward to new discoveries and insights. For that transformation to occur, each and every one of us is needed.

Today, I found out I have a third spleen. This, in and of itself, is not all that odd. Apparently, accessory spleens are found in 10-30 percent of people and are not necessarily a problem. What’s odd about it is that I had already had two of my spleens removed to treat a rare bleeding disorder that I was first diagnosed with as a toddler. The splenectomy was a last-ditch effort to try and trigger a remission. Since I already knew from my previous surgery that I had one accessory spleen, it seemed possible there could be others lurking in my belly fat or in the crevices of my surgical scar tissue. When my hematologist walked in carrying my CT scan results, his face told me all I needed to know. The only question was how many extra spleens I had. Thankfully, there was just the one, a little grape-sized thing hiding near my surgical clips. I do know of a patient who ended up with six. I asked my hematologist if I needed another surgery, if my abrupt relapse after the first was due to the accessory spleen. “Well, that’s the hard part. The literature is mostly tracking people who had a relapse after more than a year of remission post-splenectomy, and yours…” My remission lasted all of four weeks, if you can even call it a remission at all. It was just long enough to give me hope, just long enough for me to find love and start envisioning a future without a bleeding disorder and dream of a life I thought I could never have. And then shortly after, it came crashing down in a pile of CBCs and empty bottles of Gammagard. We don’t even know if the splenectomy was the cause of my “remission” because it was so brief it could have simply been a result of the infusions I received in order to make the surgery safe in the first place. I declined my hematologist’s offer to refer me back to the surgeon for another consult. The surgeon had missed a spleen; why would I go back to him a second time? Now my next surgery, if it made sense to have one, would be even more difficult due to the scar tissue from the first. I asked for a referral to a larger teaching hospital 244 miles away. But this was not the first time I have needed to be sent there. I live in a town of fewer than 35,000 people with only two aged hematologists about to retire, five general surgeons, and one hospital. My splenectomy was my surgeon’s third. Before surgery, we tried just about every chemical therapy it was reasonable to try. I was refractory to them all. And yet, still, I had to ask to be sent 244 miles away for a referral to another hematologist because the two in my small town were not up-to-date on my disease and had the mistaken belief that there was a high risk of bone marrow fibrosis with the latest medications. This research is more than 10 years old. But for most people with my diagnosis, it’s easier to just throw some prednisone at them andcall it a day. I got up before dawn to drive to the airport because I was concerned that with my level of fatigue, driving alone across the Columbia Gorge with ice still on the highway would be too dangerous for me. I wandered the unfamiliar streets of downtown Portland in a daze trying to navigate public transit, getting off at the wrong stop and having to walk three miles to the hospital because I couldn’t figure out how to get back on going the other direction. After traveling all day long and waiting more than an hour to see him, the director of hematology spent 15 minutes with me before writing a letter to my hematologist at home recommending I try the standard treatment that I had been denied. I am a fraction of a fraction of a fraction. I was one of the 15 percent of children who go on to develop chronic immune thrombocytopenia (ITP), rather than the self-limiting form. My first episode lasted six years. My rare disease is diagnosed in 3.3 per 100,000 adults per year. Of these, only about 12 percent have bleeding problems and 20 percent relapse after splenectomy within the first year, if they choose to have splenectomy. I’ve never met anyone with my disease in person. Even if I do meet someone, someday, the likelihood is that they will have a mild, asymptomatic form of my disease and be unable to relate to my treatment experiences. My wish is for doctors in rural towns to start listening to their rare patients. Many of us research the latest treatments for our illnesses like it’s a full-time job. Be open to the possibility that there is more knowledge to be gained from listening, from taking a moment to consider that maybe some diseases are more heterogeneous than you think. Be open to the possibility that maybe treatments change faster than you knew, and maybe some zebras do reside in horse pastures.

I was first diagnosed with immune thrombocytopenia (ITP) at 18 months of age. ITP is a bleeding disorder where the immune system destroys platelets, the cells needed to form blood clots. As a toddler, my parents had to bring me home from the hospital in a big white helmet to protect my brain from bleeding. I was lucky to have many years of total remission, but unfortunately became sick again. Chronic ITP that persists into adulthood is rare, so I often run into misconceptions about what my illness is like. 1. “Oh, I know about ITP. My 6-year-old niece had that.” Most people do not realize that ITP in children is different from ITP in adults. [1] ITP in children is usually short-term and self-limiting, whereas in adults it is more often chronic and hard to treat. My primary care doctor was optimistic that I would be one of the 20 percent of lucky adults who would go into remission, but so far this hasn’t happened. Adult patients are also more likely to get some of the really heavy-weight treatments like chemotherapy drugs and surgery, which may still not work. I have yet to meet another adult who has the chronic, intractable form of ITP that I have. 2. “I have a cold. Do I need to stay away from you?” Another common misconception I run into is that I have some sort of immune deficiency. This is a totally understandable leap to make, though! ITP is caused by the immune system destroying cells it should leave alone, not by having an immune deficiency. It is important to understand, however, that many patients with ITP do have to take steroids, immunosuppressive drugs or IV antibodies that can weaken the immune system. I actually hardly ever get sick from external viruses or bacteria. I do still prefer to avoid people who have a contagious illness like the common cold, because being sick when you’re already sick is no fun. 3. “You don’t have any bruises! You’re on the mend!” There’s more to ITP than bleeding and bruises. Like most autoimmune diseases, ITP has an inflammatory component [2]  that affects the body, whether or not platelet counts are severely low. For me, this sort of feels like I am recovering from a viral illness even though I don’t have one. I often feel foggy, cold, and exhausted for no reason. Patient surveys show that ITP leads to severe fatigue [3]  as well as decreased work productivity and lower quality of life. [4]  I also deal with a lot of drug side effects. More on that below. 4. “Your medicine is working so you must be feeling better, right?” Here’s the annoying thing about chronic illness: the treatments are often worse than the disease itself. It would be nice if I were one of the many people with milder ITP who don’t need treatment, but my platelets regularly get so low that I’m in the range where increased risk of intracranial hemorrhage (ICH) occurs. [5]   This is a rather frightening term which means bleeding in the brain. ICH is super rare, but it’s also serious enough that most doctors want to treat to raise the platelet count just to be sure it doesn’t happen. This leaves me stuck dealing with unpleasant side effects of immunoglobulin infusions like headaches, fatigue, neuroinflammation, and flu-like symptoms. [6] Finding the right treatment with the fewest side effects is tough, because all of them have their downsides and any of the available treatments can stop working at any time. 5. “You should get some kitchen gloves, then you wouldn’t cut yourself.” Oddly, I’ve never cut myself with a kitchen knife since having ITP, maybe because I’m extra careful around knives now. I have, however, invented a seemingly endless number of ways to make myself bleed, some of them hilarious. I’ve bled from fingernail clippers, from falling in the snow, running into a door post, having a basket fall on my face, getting my hand caught in a cabinet drawer, playing with my dog, stubbing my toe, and sometimes I don’t even remember what started it. I’m just a walking accident waiting to happen some days. Once, I fell down and braced myself with my hand, and the bruising was so terrible my doctor thought I had broken my wrist. I want to stay as active as possible, so I just keep lots of first aid supplies handy wherever I go. I have learned through trial and error what I can handle myself and what requires a visit to the doctor. It’s amazing what our bodies can adjust to.