The Toll of Traveling Hundreds of Miles for Treatment Because I'm Rare
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 and
call 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.
This story originally appeared on Muddy Paw Therapy
I am an instructional technologist and former search and rescue volunteer / wilderness first responder living in the Pacific Northwest. I was first diagnosed with ITP at two years of age. After decades of physical and mental health challenges, I want to reach out to others to normalize the experience of being sick, which is after all the experience of being human.
colinnotjustin