I bet you are wondering what the title implies.
What in the world does recherche mean? It’s an adjective meaning carefully chosen, rare, or exotic.
Why is that important? I’m glad you asked.
It’s because I am going to paint you a picture with my words about someone that has a very special place in my life and in my heart. His life is recherche. His story deserves to be known. My hope is that by writing this post, no matter how long it ends up being, that you all will help me share this around, spread the word – and maybe, just maybe, the answers that we have been searching for since 2006 can finally be answered or at least some light can be guided our way.
This is Mike and this is his medical story because it is complex, chaotic, frustrating and ultimately chronic. He has ITP (Idiopathic [Immune] Thrombocytopenic Purpura) and his journey with this rare disease is unlike any other ITP suffers known to his specialists and with further research on our own time.
So, let’s start at the beginning.
Not even a year into our relationship, we went to a carnival in the city, three hours away from our hometown. It seemed as if we were having a great time until Mike became overcome with a debilitating headache – the sun, the wind, any noise would set off a painful trigger and he almost collapsed going back to the hotel. What we thought was heatstroke turned into something more, never would we have guessed that a small trip to the doctor’s office would turn into 12 years of doctors appointments, specialists appointments, surgeries, bone marrow aspirations, weekly blood work, specialist testing, genetic testing – the list goes on.
Throughout his life he had suffered from headaches that would sometimes turn into migraines that would make him vomit, laying in pain not knowing when they would end and no amount of Tylenol would help. We didn’t think this had any correlation to what was going on now – we still don’t really have definitive answers if it does. But after that headache, I made him go to the doctor because I was worried about the small little purple dots that had developed around his eyes, down his neck, on his arms and legs. Having come from a home that cancer took a life away, I didn’t want to take any chances – I knew how serious something so small could be.
Looks harmless right?
The abnormal blood work took us to repeat the testing to make sure that the lab did not have any errors. This led to the doctor, once again, scratching his neck and sending the results to a specialist. This is when we met the specialist that we have to this day, she is wonderful and informative. She ordered more specialist blood tests and a full body scan. She told us to go home, wait for the results. So, that’s what we did – back to life as usual.
At this point, we are well into the Winter and his hockey season was beginning. It was his last year in the minors, in the last year of his high school education. It should have been a time for celebration, for a 18-year-old kid to get in trouble and just live. That wasn’t in his cards.
He was notified that from the results he had to remove himself from all contact sports. He was told that he had to take it easy and go for blood work monthly to monitor his abnormal count and until further testing, a diagnosis couldn’t be definitively made.
And then, ITP was thrown into our sight.
To have your dreams taken away in 15 minutes – I can’t imagine what was going on in his mind. He was mad, of course. He thought that it was bogus, that just because of some little purple dots he couldn’t play hockey anymore. But in that 15 minute conversation, his entire life changed.
He could count on one hand the amount of platelets he had in his body.
Those purple little dots turned into something BIG.
But the thing about ITP is the lack of awareness of the condition and not knowing the symptoms to look out for. We didn’t even know what it was when we were notified that his blood work came back with abnormalities. What made it worse was that the doctor that we had seen, a doctor that he had seen previously couldn’t give us any answers. There was no groundwork laid down, there was no information packet that we could go home with. There was nothing. His life could have been in serious danger and if we had never gone to the doctor something far worse could have happened.
If you haven’t already stopped reading to Google ITP, here is a very brief summary of the rare disorder: (*Information from the Mayo Clinic)
- It can lead to easy or excessive bruising and bleeding. The bleeding results from unusually low levels of platelets – the cells that help blood clot. In adults, the disorder is often long-term. If you don’t have signs of bleeding and your count isn’t too low, no treatment is needed. In rare cases, the number of platelets may be so low that dangerous internal bleeding occurs.
- Symptoms include: purpura or petechiae, bleeding from the gums or nose, blood in urine or stool, fatigue
A normal platelet count is anywhere from 150 to 450 per microliter of circulating blood. People with ITP have below 20.
Remember how I said he could count the number of his platelets on one hand? He has seen numbers from 0 – 400. Today, March 28 2018, his platelets are at 4.
What happened initially after the diagnosis?
The doctor told us that ITP was very rare, it was even rarer that a platelet level would ever be under the 20 range. But they were reassuring, almost confident that things would be fine. They checked that there was no bleeding and asked numerous times if when he had a cut would it bleed long, etc. We had no idea why his platelets were disappearing so quickly – was Houdini pulling some crazed trick from beyond the grave?
He was sent home with Dexamethasone, a type of corticosteriod. The nice print out stating the common side effects was overwhelming – vision changes, swelling, rapid weight gain, sleeping problems, mood changes, headaches, dizziness, nausea, and so on. At one point, Mike was taking 16-20 little white pills a day to keep his platelet counts in the 50s. When that wasn’t enough, he was up to 30 pills to get them into the 100s. When they stabilized he was pulled off of the steroid with a weight gain of almost 80 pounds. He was told to continue to monitor his counts.
And so we did.
Until we got that one phone call that told us he had to go back on the pills because his count had plummeted. He was also told that he had to go for another scan to see is his spleen was enlarged and that could be causing this, that if we got the defective organ out, he would be back to normal in no time.
So, that’s what happened. Summer 2007, his spleen was removed.
Everything seemed fine after the surgery. His recovery went well. His counts stabilized.
Impromptu Side Note:
A very important thing that I had learned about ITP was that as soon as you make a commitment to doing anything at all at a future date, then that is inevitably the time when things go wrong. That is the moment the platelet count falls, you notice a random bruise, you feel nauseous, you get a thumping headache, tummy upset, or something completely random. It’s inevitably related to ITP or the drugs being used to treat it. It’s as if a purple ghost decides to haunt you when you put any firm commitments in your diary.
But anyone who suffers from ITP knows that just when you start to think positively, the ground tends to shake. The carpet gets pulled out from under you and you flail about trying to catch yourself. And this is where the darkest times came. But it also when we found one of the most incredible family doctors in Edmonton where we moved to so I could go to University.
In 2010, Mike caught a bug that he just couldn’t kick. He was short of breath, his head was throbbing, his vision blurred. He looked absolutely terrible. So, we found the nearest doctor. After an hour of physical exams with him, he called the ambulance and we were both transported to the University of Alberta hospital where Mike (and myself) was quarantined due to the doctor suspecting that he had contracted mono. He was in the emergency quarantine for close to 30 hours before the all-clear came. We were sent on our way with a follow-up appointment the next day. With that came FULL disclosure of his entire medical history, routine blood work to do and another follow-up for the following week. The doctor also was in contact with the hematology department at the University where we were put in touch with another specialist because we couldn’t get into see our previous specialist.
This specialist was one of the tops in the department and we were reassured that Mike would be in good hands. Little did we know that because of that, this man was so overworked that he would ask us the same questions each time we met and would ultimately come to the conclusion that Mike must have been on some form of drug to make his platelets be all over the place.
But he wasn’t. His body was just eating through his platelets and building them up just as quickly and eating them again. So, this specialist put him on Revolade (Eltrombopag) which is a thrombopoietin receptor agonist. It is on the front-line of treatments for ITP and is supposed to be monitored closely. This medication is also EXTREMELY expensive. We blew through our medical prescription budget in three months.
And Mike wasn’t responding properly.
In fact, he was responding too well. His platelets were on a roller-coaster that was only going up (800 at one point – counts this high could cause a LOT of damage) and then when they shot back down it was hell. The ride was not fun. He was getting massive headaches again, a sign that his platelets were going too high (we found this out later), and he was sweating faster than I could change the sheets on our bed.
And the doctor who was overworked stopped receiving our phone calls.
So, we continued to do routine blood work that was monitored by our family doctor. Mike was taken off the Revolade completely. Everything after that seemed to be going great… until it wasn’t.
Flash forward to 2014
Many ITP patients, like Mike, will have no outward signs of the illness. The only symptoms that he has ever had is petechiae, mouth blood blisters, and random bruising along with a headache. To all intents and purposes, nobody would ever have suspected him to have an illness, let along a rare and very serious autoimmune disorder. So, it is hard to tell when that downward spiral is going to take place. And sometimes by the time that you think something is going wrong, it’s too late.
Months went by. More needles were poked into his skin weekly to monitor his blood. He was permanently side-lined from work until something happened. This is when we got back into contact with his original hematologist. It took another few weeks but with the blood work constantly coming back with abnormal counts, Rituximab was on the doctors radar. But not before other specialized tests determined that his spleen had not regrown.
FYI – it didn’t.
It is important to state that all specialists and medical advisors are different in their opinions about what is a safe level of Platelet count. Each individual case is so different and we all have different responses. The only thing to say here is that there is no one size fits all with ITP and there never will be. What is appropriate for one person will not necessarily be appropriate for another.
Rituximab is an IV drug that is a chimeric anti-CD20 monoclonal antibody that depletes B lymphocytes. It is given weekly for 4 weeks and response isn’t guaranteed. It could put ITP into remission for up to 5 years. So, this would mean that Mike would continue to be side-lined from work until the treatment has officially taken its action and it was apparent that his platelets would stabilize. What we didn’t know is that he would be denied long-term disability leave based on a fault in the wording of their policy. A medical appointment to get sensitive test results (that up until that point HAD to be received in person at the clinic) was considered a treatment and therefore, since he had too many of those appointments the policy was denied. This put him, and us into such a debt it still has been a struggle to get out of.
But, back to the point at hand.
<– That was us when we were given the news that the treatment worked. His system allowed the drug to take action, his platelets had stabilized!
And everything could get back to our form of normal…
His ITP was in remission from the beginning of 2015 until the end of 2017. We got two years. But now…
There is more going on.
Up until this point it had been glossed over that Mike’s neutrophil (type of white blood cell) and lymphocyte (made up of B and T cells) counts were a little wonky. We just thought it was another piece of the ITP puzzle that we would never have true answers for. However, this year we have been notified that this is simply not something you see in ITP sufferers.
In 2015 Mike had gotten sick again, his platelets didn’t take a huge hit (thank you Rituximab) but soon after a lymphnode in his neck became enlarged. It protruded out and caused a considerable amount of pain. After a few tests to see if there was any other underlying cause, the specialist contacted an oncology surgeon to remove the node and perform a biopsy on it to rule out Lymphoma.
That hit me hard because I saw first hand what cancer does to a person. But at the same time, I wanted answers for him – his well-being, his sanity. He needed answers and the treatment that would allow him to live his life to the fullest. I could see the panic behind his eyes, I watched as they danced back and forth. He just wanted something definitive.
A few months went by and the results came back negative. Just an overactive node that wasn’t doing him any good.
Keep doing what you’re doing. Heal. Check your blood regularly. We will be in contact.
But the main problem that all of us were finding is that with ITP you can can never really forget about it completely. It is always in the back of your mind, always nagging away. A constant itch, an attention seeker.
And it wanted to be heard again in November 2017 – a month after a special trip.
Another headache. More tiny purple petechiae. A cold that just won’t go away.
Back to the doctor. Back to blood work. Another leave of absence from work.
I know he had hoped that the Rituximab would last longer, hell, we all thought it would. But it ran its course, I guess.
So, what do we do now?
The question that looms on us every appointment.
Today, Mike has been off work and is still continuing to stuggle with this latest episode of ITP flare up. We have seen more doctors and specialists because his bloodwork is no longer coming up point blank ITP. His platelets were going up on their own for a month or so but his neuts and lymphs were very low. And then his platelets stayed low and continue to drop. When I said up there that they were at 4? Our latest result from this afternoon – down to 2.
Sitting and waiting.
But we have to wait because two more of his lymphnodes need to be resected and biopsy’d before he can even begin the Rituximab. We have to wait because there are more genetic results that are waiting to come in. We have to wait because every other test of other autoimmune disorders comes back negative and they only give us more questions. We have to wait because there are just no answers to the questions that his blood work poses.
And I guess, I am writing this because awareness is good. Everyone deserves to have insight into something. Knowledge is power – right? But I am mostly writing this for him because maybe someone will read this and can get in touch with me. Maybe a doctor will find this and have insight. Maybe it will start something great to happen in terms of finding out the right answers.
So, please, pass this around. Help me share his story, get into the right hands. I would be more than glad to share more indepth knowledge, ie more insight into his blood work results and give you an inkling of how that works (another day thought because this is already 3 hours in the making and longer than I ever anticipated).
I hope that this finds the right eyes. I hope that something comes from sharing this.
Thanks for reading.