The photo above shows my last two CBCs. I was at 94,000 platelets when I had my first Rituxan infusion on 7/1, and before my second infusion this past Monday (7/8), I went up to 100,000! I was killing off around 12,000 platelets a day before treatment, so this was extremely impressive.
I am feeling pretty good despite the mild headaches, constipation, fatigue, and lack of appetite. Those things really don’t bother me when I know my B-cells are being suppressed!
My next infusion is Monday, and I’ll have another CBC before treatment. I am curious to see where my platelets will be at the half-way mark. I’ve noticed my purpura fading, I haven’t developed any new bruises and I have no brain fog. So far, a night and day difference.
Some other good news: my nurse Jen was able to tap my wrist for the infusion, which saved me from having to hold my arm straight for a few hours. I also was administered 100mg of IV steroids in addition to Benadryl and Tylenol as pre-meds. I infused pretty quickly, from 10 am to 1:15pm, with no reaction. The only thing I don’t like about my wrist is the burning in my arm after Benadryl. It hurts for about 15 minutes and then subsides. I also have to be careful that I don’t let anything touch the area for 24 hours after, because it is tender. I usually wear my Apple Watch on my left wrist, but have to keep it off while the area heals.
That’s the update for now, I am happy with how things are progressing and ready for infusion three. #PlateletsUp
I’m home from my first Rituxan infusion, only three left to go!
While I’m smiling, I need to be brutally honest. Today was hard, the smile is put on. I’m tired, stressed, and I had another massive emotional rollercoaster caused by our broken healthcare system. I almost didn’t have my infusion today.
First, upon my arrival to the infusion center my authorization was not in the system. Instead of calling upstairs to get the information, they made me go upstairs to find the employee responsible. Why it couldn’t have been handled by a phone call I’ll never know. Just another part of the process where the systems and staff don’t seem to have an open line of communication. Insurance issues are handled in an entirely separate department and no one knows how to navigate it outside of a few people. Thankfully, the problem was fixed with a phone call down to the infusion center from the authorization staff.
When signing all the paperwork to do Rituxan last Tuesday, I had to take a pregnancy test to process the orders. It took my doctor a good ten minutes of navigating the EHR system to determine which of the tests was needed for Rituxan. At no point was a Hepatitis B panel shown, but apparently I needed that too. Unfortunately when I went to infuse today, my treatment was almost cancelled because I didn’t have the panel done. It wasn’t discovered until the Pharmacy was prepping my infusion. My amazing nurse got my doctor on the phone (he’s out on vacation) to override the testing requirement so we could get the prescription processing. She ended up drawing blood and everything came back fine.
At the time this all went down, I finally broke down and cried. This disease has been a constant source of unrelenting stress since March. I honestly never anticipated having this much trouble when ITP came back. Pair that with not sleeping and feeling generally miserable because of crashing platelets, I couldn’t hold in my frustration any longer.
While I tend to handle my disease with sarcasm, humor, and witty banter, I am still human with raw emotions. The system is not designed for rare disease patients, especially ones who are using off label therapies. We are forced to micromanage care, and anytime you look away, you risk someone else dropping the ball. Most of the time, those people are not the ones directly caring for you (like doctors or nurses), so it makes advocating for yourself much more difficult.
I absolutely hate having to be assertive, direct, and at times downright demanding to get what I need to battle ITP. I want to find creative solutions to the problems all of us rare disease patients face. Every time I’ve shared these bumps in the road, patients tell me their horror stories. I know we are not alone, but it seems like the system temporarily corrects itself for one of us, then goes right back to operating under broken processes. At the end of the day, this helps no one, because the cycle continues.
I hope that after all my infusions are finished and I am back in remission, I can work with payers, hospital systems, EHR companies, legislators and rare disease groups to fix a lot of these issues. There’s no sense in dismissing them once my treatments are over, because I will eventually be back in the system when I come out of remission again. I also want to turn negatives into positives. I want my challenges, problems, and pain points to help inspire change.
Now on to some interesting things about today’s infusion:
I had a reaction an hour in, complete with itchy eyes, throat and ears. I became flushed while my nose got stuffy. They had to stop the infusion and administer more Benadryl plus IV steroids. I had pre-meds of Benadryl and Tylenol, but no steroids. That’s likely what caused my allergic reaction (it was similar to what I experience with cats). For the next three infusions, I am going to ask for the steroids as pre-meds too.
My platelets went from 169,000 to 94,000 in 6 days. I am destroying them rapidly, so I am anxious to see how quickly Rituxan will work.
Food already tastes a little bland, but I’m hoping that will help me lose the 10lbs I gained from steroids!
Now that infusion one is finished, I am anxiously awaiting next Monday. Keeping my fingers crossed that the rest of the process is smooth from here on out. I appreciate the compassion shown by my nurse today. She went above and beyond to advocate for me when I felt defeated. She made it happen, and for that I am eternally grateful.
On Monday 7/1, I’ll be repurposing Rituxan for Immune Thrombocytopenia for a second time. The goal is to put me back into remission for an extended period of time. When I used Rituxan at the end of 2014, I had a remission period over four years. I’d like to think we will be able to achieve similar results this time around.
Rituxan is a chimeric monoclonal antibody targeted against the pan-B-cell marker CD20. In plain English, it targets B-cells by going after CD20 which is expressed on the surface. Ultimately, after multiple blood tests and positive results from immunosuppression by steroids, we were able to figure out my B-cells were responsible for destroying my platelets. By shutting them down using Rituxan, I am able to maintain a normal platelet count.
The first test I received is Anti-Nuclear Antibody (ANA) which came back positive and indicates autoimmune activity. We also tested for Lupus, Rheumatoid Arthritis, and Sjögren’s Syndrome among others. I ended up also testing positive for SS-B antibodies, further confirming autoimmune activity. Another great marker: I respond very well to immune suppression with Prednisone. All of these factors collectively helped my doctor come to the conclusion that I had chronic Immune Thrombocytopenia and I was destroying platelets via my B-cells. I never had a bone marrow biopsy because when I am on high doses of Prednisone, my platelet count returns to normal. There is nothing to indicate that I have any issues with platelet production in my bone marrow.
Because of this, N-Plate and Promacta would not be the right treatment for me. I could stimulate platelet production, but my B-cells would destroy those platelets as well. Ultimately, the idea is to stop the process of platelet destruction. That’s how I came to the decision to use Rituxan in 2014, and why I advocated so hard to use it again this time.
So now I begin the process of making myself an N-of-1 example of how Rituxan can help ITP patients with B-cell led destruction of platelets. We will collect a CBC before each of the four infusions to track progress. I am currently on no medication for my platelets after I finished my Dexamethasone pulse last Monday (one week before my first infusion).
Besides chasing remission for a second time, I had an inordinate amount of trouble getting insurance to approve this treatment. We must see to it that Rituxan become an on-label therapy for Immune Thrombocytopenia, especially since patients with B-cell led destruction of their platelets do not have an FDA-approved treatment.
Acknowledging the clinical diversity of ITP is a must, especially with multiple drugs on the market and more in development. There is no one size fits all treatment for ITP, and what works for me may not work for another patient. Gathering treatment data and comparing it with ANA tests will be a great way to dissect some potential remission patterns in patients.
Last week, I was forced to reflect on the last five years of my life. The diagnosis, the treatments, the risks, and reward of remission for over four years. The heartbreaks that came from losing people (and my dog) to things I could not save them from, no matter how hard I tried. It was the first time I told my story from start to finish. It was also the beginning of my self realization that I am a completely different person from who I was on April 30th, 2014, the day before my D-day (diagnosis day).
Before ITP, I was a tough chick with a no nonsense attitude. But I had no calluses. That came to an abrupt halt on May 1st, 2014. Baptism by fire, taking on a dysfunctional body in a broken system that does little to help patients like me. There was no soft introduction into the world of rare diseases, I was simply dropped into the pit and expected to sink or swim.
I was forced to become my own advocate, to research, absorb, digest, and become the expert on ITP. My view of physicians changed from putting my implicit trust in them, to questioning everything. I no longer wore rose colored glasses when it came to my views on healthcare. I was being raked over hot coals, and that’s how I developed my calluses.
There is a certain level of pressure to be gentle, warm, and kind as a patient. I’d like to think I have retained those qualities over the last five years, but they’re not as apparent now. It’s hidden underneath a hardened exterior, one I had no control over developing because I needed it for survival. It builds through every bump in the road, side effects from treatments, disappointing lab results, and just the overall way life can seem to come down on you during difficult times.
To every rare disease patient who takes time to reflect on their patient odyssey, don’t beat yourself up over a hardened exterior. While we do our best to navigate a system that constantly throws us curveballs, a roughed up exterior is part of the package. Wear your calluses with pride, you earned them like your zebra stripes.
It took 14 days for my platelets to plummet back to a level low enough where I was beginning to bleed again. I had my first IVIg infusion at the beginning of the month, starting off at 20,000 platelets. My vein blew during the CBC beforehand. My insurance played games with approval, causing a four hour delay that ultimately rushed my treatment. 48 hours later, I was suffering with the classic symptoms of aseptic meningitis (don’t worry, an adverse event report has already been filed and I am okay).
My platelets reached 98,000 three days after the infusion, then 78,000 and 14 days later, back down to 21,000. Given all I went through, this was a disappointment. However, notably absent is my Sjögren’s related pain, so I won’t say this is a total loss. I declined doing one more infusion because I don’t believe a 14 day window is worth it. Ironically, Dr. Ahn predicted back in 2014 that an IVIg infusion would last no more than two weeks, again showing how absolutely brilliant he is!
There are a few questions surrounding IVIg and how it will impact ITP patients with B-cell led destruction of platelets. I wonder if the prevalence of other autoimmune activity shortened the effectiveness of the infusion, or perhaps if I did two back to back, would it have lasted longer? I don’t feel the need to put myself in a lab rat position for this, simply because the scientific evidence is not compelling enough for a second look right now. I have my eyes set on the long term goal.
I ended up doing a Dex pulse for four days, and today is my last dose of 10 pills. This is not as bad as Prednisone in that it is quick, but it still comes with major side effects. Heartburn, insomnia, irritability, sweating, etc. Pretty much the same things I experienced before. Tomorrow morning I will have my platelets checked again, and I am curious to see how much they popped up.
Which brings me to the big news: we are trying Rituxan again. I have been waiting to hear those words since March, and I have dealt with a lot of miserable experiences to get here. This time is much different, I know what to expect, but the stakes are higher. I want to see Rituxan become an on-label treatment for patients with Chronic Immune Thrombocytopenia that have B-cell led destruction of platelets. That means it is not for people with T-cell issues, platelet production issues (who respond well to N-Plate and/or Promacta), and those on Tavalisse.
I launched the ITP Patient Driven Research Initiative last month to tackle this issue, and ultimately work toward better targeted therapies for patients with my rare disease. The research and drug development currently underway is a start, but we can do better. A solid data platform led by patients inputting their results based on a variety of treatments over extended periods of time will help us develop data sets needed for better treatment protocols. From there, we can acknowledge the clinical diversity of ITP and get pharmaceutical companies on board to run better clinical trials.
That’s the update for now. I have an early day at the hospital tomorrow with bloodwork and planning. I’m hoping to start infusing ASAP, especially since I have been through the platelet olympics these past few months.
Thursday’s doctor visit was a rollercoaster. I ended up at 31,000 platelets and needing treatment, so he suggested we try IVIge. Cue major nerves, I have spent the last five years reading about the brutal side effects other ITP patients experienced, so I am worried about how I will feel after the infusion.
I’m curious to see how long my platelets will remain at a normal level after treatment. At the height of my platelet destruction in 2014, Dr. Ahn believed it wouldn’t hold for more than 10-14 days. However, this time around I caught it early, so the outcome may be entirely different. I am happy it is only one infusion, instead of four, and will hopefully have me back on my feet after the weekend.
So how am I preparing for it? First- I asked fellow patients. The hospital told me to hydrate, but I wanted to hear from people who have been in my position. Thankfully, everyone told me to load up on electrolytes, so I bought a ton of Powerade (Seminoles don’t drink that gator-garbage), Smart Water and Coconut water. I’m also planning to eat soft foods the day before, during, and a few days after the infusion in case I have vomiting. Because my teeth were weakened by past prednisone usage, I want to minimize any potential damage that can be caused by throwing up. Infusion clinics really should be giving out this advice when you schedule your appointment, but that is a fight for another day. I want no surprises, so I’m glad I asked because just chugging water the day before was not going to cut it.
The most difficult part about this infusion is the unknown. I did not experience this level of apprehension when I used Rituxan, but I was confident it was going to work. This is a sort of a shot in the dark, I expect for the treatment to fail after a few weeks and my platelet count to drop. However, we need the data and we need to see how I tolerate it. So I’ll play along, for science.
It’s no secret this go-around with ITP has been stressful. I thought by now I would be close to finishing another round of Rituxan, but since Dr. Ahn retired, no such luck.
Tomorrow I see my new hematologist again, he’s taking blood and after the results come back (within 10-15 minutes), a plan will be crafted. Why the nerves? I’m afraid of once again being told I need to “wait” for treatment.
Because my platelets can drop to zero pretty quickly, there is no sense in waiting around for it to happen. Unfortunately, ITP treatment varies by doctor and some are willing to wait until I get very low. I know my body well enough to know that will come quick, so acting now is best. After all, we wouldn’t wait until cancer reached a more advanced stage before issuing chemo. ITP should be treated no differently.
This is a short blog tonight, I need to try and get some rest, but I wanted to document my nervousness because I know there are other patients out there like me. I guess this is “normal”, but it shouldn’t be. I should be able to access the treatment that saved my life once before. I’m chasing that “new normal” I started blogging about almost five years ago. I want to go back to being myself, not living week to week between lab appointments.
Cue the long groan, I am out of remission and my platelets hit a startling 53,000 this week. This triggered swift action by my new hematology team, so I am taking 40mg of prednisone daily.
Before I get into that- let me tell you how much I love my new hematologist. Dr. Ahn retired February 2018, so I was nervous about seeing a new doctor. Friends of mine recommended Dr. Harrington at UM (who’s father happened to discover how ITP works). He’s brilliant, funny, and practical in his treatment of ITP, especially when it comes to steroids. I was so glad to hear him say he won’t keep me on prednisone for a long period of time.
I had blocked out most of the negative experiences I had almost 5 years ago with steroids. After all the moon face, acne, weight gain, constant hunger, anger, poor temperature regulation, and insomnia were horrible. Well, that’s all back and I have been expressing my frustration about it. I was finally able to sleep last night, thanks to medical marijuana (more on that in an upcoming post). I wish I had that stuff back in 2014 when this journey started. I woke up this morning a bit more refreshed and less hungry. My goal right now is to not gain any weight while moving through this part of the treatment process.
I’m documenting bruises again and getting weekly CBCs. Obvious goal: using Rituxan again. But I run into the same problem I had in 2014, safety data is scarce. I’m leaning on my team at UM to trust that since it worked once, it will work again. Since I am traveling a lot this month, I am hoping by mid-May we will be able to get those infusions going.
If you have used Rituxan more than once for ITP and are willing to share your experiences with me (and your CBC data), please email me at RareCandace@gmail.com. I’m planning on doing something similar to what I did in 2014, creating a mini data set to show the efficacy of Rituxan for immune destruction of platelets.
There’s more to this story, I am still hard at work behind the scenes to get ITP patients access to a CD 20 inhibitor ON LABEL. Stay tuned.
I have not blogged in a significant amount of time. For those of you who followed Kona’s story on Twitter, Instagram, and Facebook, you know the magnitude of her death. In 13 months I lost Kona and Shaun, which was too much to handle. I felt the need to take a break from this blog and focus on myself for a while. I now finally feel ready to come back and start fresh.
Kona’s death signified the end of what was left of my old life before I got sick. She was my final connection to the old me, and my partner in crime. I fought with everything I had to save her, and still came up short. I am still haunted by the fact that I could not save her, even though I was able to figure out how to save myself. My hope is that Kona will live on in a variety of ways. The first being donating her tumor to research.
Kona’s tumor was divided into three segments: one for pathology, one donated to work on a treatment for Glioblastoma, and another is being stored at Purdue University for a later project. I wake up every morning and the first thing I think about is this. I comb Google Alerts on Glioblastoma, hoping that something will pop up with canines. Perhaps one day, that missing link will be discovered.
Kona’s story has grabbed global attention, from animal lovers to the rare disease community. As her Mom, it is therapeutic to have people approach me months after her death to tell me they followed her story. Now, Kona is being discussed on Capitol Hill, and I hope to introduce the KONA Act to the 116th Congress in her honor. Additionally, I will be requesting funding for the Purdue University College of Veterinary Medicine. Everyone there from Dr. Bentley, Dr. Embersics, and the staff did everything they could to save my girl. They deserve to have their research funded to the fullest extent possible.
I now have a Kona angel that comes with me to all my meetings. It’s a reminder of her and the long road ahead to make sure we find effective treatments for glioma-based tumors. Her pathology report came back: oligodendroglioma. I promised her the night she passed that I would fight until my last breath to make sure they’re wiped off the face of the earth.