There is a light at the end of the tunnel… hopefully it’s not a freight train!”Mariah Carey
Seems like it is time to share the next chapter of my journey with Scleroderma, that time when I got sick with optimism… In a previous posts I have discussed my first bad Scleroderma flare/crisis (in case you missed them, you can check out Sick With What? or I Will Tell You When to Panic ). By the summer of 2018 I was beginning to stabilize and by the end of the season I was adapting well to treatment. By that time I was on twice weekly plasma exchange, nightly peritoneal dialysis at home, and a variety of drugs, including Aranesp injections to help stimulate red blood cell production and an immunosuppressant, Mycophenolic Acid at a dose of 360mg twice per day.
By September, through gradually increasing doses of Aranesp to 80mcg weekly I was beginning to see a normal Hemoglobin (Hb) level which of course gave me more energy and general ‘zest’ for life that I had been lacking for more than a year. Unfortunately, because I was on dialysis normal hemoglobin levels are considered too high as it can pose a risk of stroke. The solution was to pause the injections.
At the same time, my kidneys were showing some signs of recovery. The Aranesp injections had been replacing the hormone my kidneys should have been creating to produce red blood cells in the bone marrow. I was optimistic that the sudden rise in Hb meant that my kidneys were now producing the hormone on their own. By October, my nephrologist was beginning to reduce dialysis to see how my kidneys would function without the support of 8-hours per night dialysis. I was filled with joy and a certain amount of vindication that my optimism was actually being rewarded!
While my anemia seemed to be under control, the skin tightening from Scleroderma was still marching on. I could no longer make a fist or lay my hand flat. My feet still tingled and I could not bend my toes or feet, the texture of my skin across my face, my arms, feet and half up my legs appeared waxy and hard. The advancing swelling, hardening, tightness and pain was a disappointment as my rheumatologist anticipated that after renal failure these symptoms would relax. Together we decided that with my improved Hb levels I could support an increased dose of the immunosuppressant (the drug tends to cause anemia so I could not take the maximum dose when my Hb was low). Over thee next two weeks I gradually doubled my dose to 720mg twice daily. I was excited to see what improvements could be achieved!
What is Hoped For versus What is Real
I excitedly embarked on the next part of my journey! Less and less dialysis, no Aranesp and double the Mycophenolic Acid, this was it! I was getting better! My brain was so full I could barely settle it down. My body of course was weak and could not keep up with my optimistic outlook, but I determined to work on that too! I spoke to my boss about beginning to come back to work part-time once I was sure I was stable. I obtained a lovely exercise bike from a generous neighbour who was getting rid of theirs. Things were really looking up… for a couple of weeks anyway!
Unfortunately, my situation quickly deteriorated… my Hb began dropping quite rapidly. I felt as though a tonne of bricks had fallen on me and I couldn’t get out from under them. Eventually we began with the Aranesp injections again, but I was not responding well. The only good news story was my kidney function continued to improve to the point where we were experimenting with taking a break from PD. Within a week of this pause in dialysis (during December) I was throwing-up and having other G.I. issues constantly. I had a cold from early November that would not go away, even after a round of antibiotics. My Raynaud’s became painful and out of control. I ended up needing a blood transfusion as my Hb hit an all time-low on Christmas Eve, and another at the beginning of the year. I was back on dialysis. I was coughing constantly and had to sleep sitting up on the couch as best I could for over a month. I was, once again, a Christmas zombie, too sick for my usual baking and shopping for a second year in a row.
I know you must be thinking, how can I write a blog called Sick With Optimism if I believe that I actually got sicker from being too optimistic? The fact is, I was overly eager. Upon reflection, I realized that making so many changes all at once did more harm than good. In future I determined to be more realistic in my expectations of what drugs and therapies can do and what my body can withstand.
At one clinic appointment my doctor asked me, “Who is in charge of you now?” meaning, which doctor was coordinating and spearheading my treatment. I was a little taken aback, I figured she should actually know that — the trouble was, I didn’t know myself… In fairness, Scleroderma is a confusing illness, potentially involving doctors from a variety of specialties; I have had three nephrologists from three different clinics, two hematologists that work as a team, a rheumatologist in my community as well as another in the Scleroderma Clinic, there is my GP and a cardiologist in my community as well as so many interns and inpatient doctors and fellows, I can’t even count! My case seemed to be passed around to various specialists depending upon what my greatest need was at any given time.
My ‘perfect storm’ was a result of a complicated illness, involving a number of specialty areas across two different hospitals. Don’t get me wrong, my doctors do communicate amazingly well, considering, but I now understand that I am the one in charge of me. It is up to me to make sure that my care is coordinated and makes sense. Just because my symptoms may indicate a certain change in medication or treatment is required, I need to be doing an overall reasonability check. For example, recently my blood pressure increased dramatically and I needed to start on a new medication and subsequently increase the dose to be effective. At the same time a doctor from a different clinic wanted me to increase the dose of my immunosuppressant again, but this time I held off. I told the doctor I wanted to adjust to one medication before increasing another. It can be difficult to bring yourself to respectfully dispute a doctor’s recommendation, but I found that he was extremely understanding and supportive of my position when I explained my reasoning.
Mine is indeed a cautionary tale. I confused my hopes with what was realistically possible. I was so eager to see improvements that I agreed to multiple treatment changes at the same time, making it almost impossible when things went wrong to determine what was causing my problem or if it was the confluence of changes that resulted in a recipe for disaster. HOWEVER, I CONTINUE TO BE OPTIMISTIC. Throughout that crash of 2018, I understood that if the doctors had gotten me on the right track in the summer, eventually they would get me back on that track again. The complete trust in my doctors never waned, but I realized they had been missing an integral player – I had been a spectator instead of a member of the team so far and that needed to change.
Who Is In Charge of You?
For years I was that stereotype — a minivan-driving soccer mom. I know that even an excellent team will struggle when they are playing short a player, even if that player is not a star. Sure, I could beat myself up for not realizing this sooner, but I won’t, for two reasons: First, regret is a negative emotion and I try to stay as positive as I can, and second, I was really sick… like an injured player I was sidelined by my disease, leaving my team playing 10 v 11. Proud as I was, I didn’t admit to myself or anyone else that it was more than I could handle alone. I went to almost all appointments on my own. At first I would just listen and try to remember, then I started taking a few notes, and finally, I pretty much just got lost in the fog of it all.
If you are lost in the fog of your own perfect storm, do say something. If you can’t sort out the details or understand why you are taking certain medications, speak up. Try to find an advocate or perhaps a few people who are close to you who can be called off the bench to substitute for you if it all becomes too much to handle on your own. It might be a good idea to keep one notebook that you or whomever you ask to help you can keep chronological notes in. Keep the notebook with you, that way when you think of a question you can write it down rather than relying solely on your memory. I was using my smartphone for notes but in the end, they weren’t easy to organize and if you have an advocate helping you out, it might be difficult for them to understand and use the applications on your phone. (That being said, the phone has been a great place to calendarize appointments and lab tests with advance notification reminders — It can get very confusing if you don’t keep careful track! I don’t know how many times I’ve heard in a waiting room of patients being late, missing appointments and/or showing up on the wrong day…)
Add a Little Optimism to Your Reality
I believe that being optimistic is a choice, and a powerful weapon in battling any disease, but it can be made stronger when tempered by reality. Your notebook is the reality… test results, diagnoses, appointment dates, questions. Now do one more thing to help yourself… write down things that inspire you towards optimism in your notebook: quotes, stickers, positive affirmations you think up or are shared with you, a good deed you did or plan to do, the title of a book or blog that is recommended to you, a website or Youtube channel you want to explore later, and so on.
So that is the chapter of my journey when I got sick from optimism. Am I sorry I was too optimistic? Nope. That hope was a two-year high for me and it also served to teach me a few important lessons on the importance of: being optimistic in a realistic way, taking more responsibility for my healthcare, and asking for help when I need it. I’m still struggling with that last one… but that’s part of my journey and I am optimistic that I will do better in the future.