Hi
I have occasionally reviewed this site on and off since my ITP diagnosis some 7 years ago at the age of 45 . A month and a half ago, my ITP flared and my platelet numbers bottomed out. I’m doing pretty well now and thought I would introduce myself and tell my story.
That original diagnosis , years ago, occurred during a routine work checkup and found my platelets around 120, if I remember correctly. With just a reading in an assay and no symptoms, it all seemed very theoretical and not a big concern - although I did follow-up.
So within a year or so of my diagnosis, my numbers dropped one week but not to a dangerous level (I cannot remember the numbers but above 20, probably above 30). High prednisone restored them to around 100. Upon tapering the prednisone, my numbers would drop as the dose dropped. I tolerated the prednisone very well, and used it (around 20 mgs and I am a very tall big guy, 270 lbs) to maintain my numbers above 20, often above 30/40 for the next 5 years or so. I had few symptoms during this time. Purpura appeared on my shins and I would have the occasional impressive bruise on my forearms – sometimes two or three. My forearms seem to be a sensitive area for such bruises.
Aware that long-term pred is not a good thing to do, my Dr and I tried several regimens to get me off the drug. Two sessions of retuxan - no effect. We also tried three immunospressants, individually and at same time: imuran, cyclosprorine, and mycophenolate mofetil- all to no effect. So I remained taking 20 mg of pred each day and my weekly numbers fluctuated from 25 – 40.
At the end of this past September, on the last morning of a vacation, I awoke in the hotel room with three or four slightly bleeding mouth sores. Knowing this as a marker of low numbers, my wife and I visited a local emergency room : my platelets were at 11. So, for the first time I received IVIG over the next 48 hours. This brought my numbers to 68 and we traveled home on that Monday. The following Friday my numbers were at 2: another day long IVIG had no effect and I received platelet transfusion that evening for the first time. After the weekend on the next Monday: again around 2. That week I had daily IVIG that had no effect: my numbers fluctuated from 2- 6 each day. We also bumped my prednisone from 50 to 150 mg/day. Impressive bruises started to bloom, mostly on my forearms, that were orange sized, but also quarter-sized bruises along my back and arms.
We started talking about a splenectomy: my hematologist recommended a surgeon in Baltimore with great experience with this procedure under low platelet conditions. I had a bone marrow biopsy, which indicated a moderately higher than normal platelet production (no sense in taking out the potential site of destruction – the spleen- if the platelets aren’t being produced). I had the three vaccinations.
Other changes: I started taking Amicar (aminocaproic acid), which helps maintain clotting; this seemed to prevent bleeding mouth sores, although bruises still bloomed along my body. I stopped driving myself (I have a 45 minute drive to the clinic near my workplace) and I started wearing a good helmet while a car passenger in case of a fender bender. And that wed of the daily ineffective IVIG I got my first Nplate injection at I ug/kg.
The following Monday I checked into the hospital. The plan, with bottoming out platelets, was to perform a spleen embolism, by placing coils to block blood to the spleen to effectively “choke” it, then hope platelets rise over a few days and then take the spleen out under higher platelet numbers with less of a bleeding risk. However, if the numbers don’t rise, we would be locked in, and the splenectomy must occur. Under low counts, the surgeon preferred open surgery, not laproscopy because he can better see everything and control bleeding: with laproscopy, a bleed could be invisible to the cameras. Made sense to me.
It was difficult for me to think about all this clearly: stress, sleeplessness and steroids had me pretty strung out. Fortunately, my wonderful wife is a great patient advocate: she is a calm logical person, a working Harvard-trained epidemiologist and an expert in medical device epidemiology. So she was concerned about snaking things up my femoral artery under nearly zero counts (required for the embolism) and had discussions with the surgery team about the relative risks of this vs straight open surgery. Thankfully the Drs there were very welcoming of her input recognizing her training and background. (I’ll add here that I am also a working scientist. I head a lab of researchers who study embryonic development and mouse genetics. This helps a little with reading the ITP research literature, but it is my impression that most ITP patients are rather well informed regarding their options regardless of their professional background.)
We also had a very knowledgeable Hem Dr at the hosptial, and my wife and I started cramming, reading the Nplate scientific literature and discussing it with her (mostly my wife did this ; again I did some , but I wasn’t thinking well. At one point I watched a capsule- sized bump appear on my arm as I sat in the chair, tethered to heart montitors and IV. A bump? Didn’t hurt…. Over the 48 hours it spread into a grapefruit sized purple blotch. 30% of my arms were now dark shades of purple. I was working on not freaking out. Each bruise seemed a metaphor for a potential aneurysm)
We decided to wait to see if Nplate would raise my numbers and put off the embolism procedure, with the idea that we would use it as a bridge to a safer splenectomy. So another injection at 2 ug/ml. After 9 long days of hospital observation and my numbers never rising above 8 and often at 2, one morning my value was 15 and we determined this value was statistically meaningful. After three weeks I was finally responding to Nplate injections. I was sent home, away from potential hospital infections, to see if the numbers would rise. Indeed they did each day: 36, 91, 128, 300s, 600s. I returned to the hospital for laproscopic splenectomy. This trip was quite different: instead of working on reducing fear, I was calm and ready for the surgery. Three days after the surgery I went home, sore but optimistic: the surgery itself occurred four and half weeks ago as I write. (Also, they didn’t find any extra spleens, but these are harder to see laproscopically. Did you know that 15% of ITP patients have extra small spleens? After the main spleen is removed these can sometimes grow and resumed splenic function).
My number right after the operation was in the 600s and with each week steadily dropped by 100 or more. Last week it finally plateaued with a value of 230, which was about the same as the prior week. So now we wait and see- living with uncertainty is part of this syndrome, isn’t it? My best hope is to be drug free. We’ve been tapering the prednisone and I am now down to 20 mgs. During the past 3 weeks or so an amazingly shattering insomnia mad me in its grip – I’ve never experienced anything like it- each day I would have periods of shuffling incapacity and mental incoherence. Instead of a real nap I my attempts would produce only 10 minute of half awake hallucinations that would render me more or less functional for the next 5-6 hours. At night I would only sleep 2-3 hours. But thankfully this has cracked during the last week and I have been able to sleep throught the night and nap during the day. Now I am only exhausted but I feel human again.
I know we cannot know, but I also cannot help some speculation regarding the flareup that initiated all this. There are two possibilities. A few weeks prior to my platelet drop I went through one of the more stressful events in my life. At work my research program is reviewed every four years by a team of the leading academic scientist from universities around the country. This requires months of preparation for an ultimate all day event of talks and meeting This review process largely determines our funding and support for the next four years. It is always stressful but is even more so these days when funds for Amercian scientific and health-related research is at an all time historical low. So I ended up doing exceedingly well, but it was also one of the more stressful events in my life. The second possibility is that three days before those mouth blood sores in the hotel room, I started taking a drug, called rapamycin (or Sirolimus) or to see if I could wean myself rom pred. Rapamycin is an immunosuppressant used mostly by kidney transplant patients but we were trying it out with a researcher looking into its used for ITP. I stopped taking it immediately.
So a long posting here! Thanks if you made it this far! I wish a high (enough) platelet number to all for the holidays-
