Infusion fail

Hello, all!

Take One of the post-infusion prednisone taper halted a few weeks ago. After the initial challenge of the first infusion, the second infusion two weeks later was problem free. After an additional two week wait, I began the steroid taper my rheumatologist intended. The rationale my doctors offered for tapering my prednisone dose was perfectly reasonable: reducing risks of surgical complications, osteoporosis, and infection. However, every intervention, whether additive or reductive, has its risks and benefits. The taper schedule was simple: decrease my dose by one-half milligram every two weeks to transition from nine down to five milligrams per day. Two weeks at 8.5 milligrams went smoothly. The few initial minor joint aches I experienced resolved in several days. However, the further a medication taper progresses, the greater a percentage of the previous dose half a milligram represents. 8.5 milligrams represented a 6% reduction from my original 9 milligram dose. 8 milligrams was a 12% reduction. For my body, ever hopeful that I would call off the joke and stop making it work so hard to create its own corticosteroids, this was a step too far.  One and a half weeks at eight milligrams was my undoing. Between increased shortness of breath and increased oxygen needs for all my activities, I was feeling miserable. Saddled with the dreaded fankles, swollen ankles that merged with my feet, my vanity was wounded as well. When I informed my doctor of these symptoms at a routine visit, she took immediate action. Dr Crespo increased my steroid dose to ten millligrams daily both to improve my respiratory struggles and in anticipation of other medication tinkering. While the 25% dose increase did cause a transient rash, I felt better within days.

When I reviewed my right heart catheterization results with the physician who performed the procedure, he confirmed that I have severe exercise-induced pulmonary hypertension. Of my two pulmonary hypertension (PH) medications, only one is at its maximum dose. The other can be prescribed at five or ten milligrams, and I've only ever taken five. Dr Risbano suggested that the higher dose might improve my activity tolerance, lessening my shortness of breath. My transplant doctor and nurse practitioner decided that given his specialty as a pulmonary vascular doctor, Dr Risbano should manage all my PH medicines. Unfortunately, when I started Letairis a few years ago at a five milligram dose, I tolerated it very poorly. It took more than two weeks for the initial shortness of breath and flu-like symptoms to abate, making for a rather miserable vacation in Paris. The Musee Marmottan, with its extensive collection of Monets, was wonderful; searching for elevators to avoid panting up the many steps in an old European museum was less so. Anticipating a similar adjustment period when my dose increases, but without the beneficial distraction of international travel, my pulmonary team deferred re-initiating the prednisone taper until after I adapt. 

I receive Letairis once a month from a specialty mail order pharmacy. I'm currently awaiting the arrival of the ten milligram dose for a three month trial. If all goes well, I should have lower pulmonary pressures, resulting in less shortness of breath with daily activities. An echocardiogram (cardiac ultrasound) in three months will check those pressures non-invasively. If I tolerate the higher dose poorly, the team has already agreed that I can return to my original five milligram dose. Five milligrams has already proven a sufficient dose to provide some benefit. I'm reassured that we've already set an endpoint for initial evaluation, and the trial of the higher dose won't drag on interminably without evidence of additional benefit. 

After the transplant, I'll no longer need my PH medications. Many of the lupus drugs will be stopped as well, since transplant immunosuppressants will take their place. For now, I'll maintain my medication regimen faithfully, good practice for a more elaborate one in the future. Medication changes always come with a degree of trepidation; even wonder drugs have side effects. This time, though, it's clear to all that if I have to take a step backward, it isn't a failure, only a recalculation of my body's needs. I'm encouraged that during the wait for a transplant, it's possible for me to see some improvement. I'm still nervous that I could have a rough few weeks ahead, but I'm blessed by the luxury of sharing my journey with you, supported by your friendship. Happy Sunday!


Shout outs are in order: Thank you to Yumi and Oeimae for your whirlwind weekend visit from Boston, and for doing some of my favorite Pittsburgh things with me. Thank you to Allison for sending some delightfully thoughful gifts that will be displayed prominently. Thank you to Laura for commissioning Whitney to bake two batches of amazing cookies. We put half in the freezer, so we wouldn't eat them all at once. Oeimae, your cookies just came out of the freezer... We need to start walking more. Highrock Brookline, we love you!

Some of the beautiful midwives of The Midwife Center at their annual Let Them Eat Cake fundraiser!

Infusion confusion

Hello, friends!

Based on recent evaluations, I started a new medication last week. Rituximab is a monoclonal antibody, an immunosuppressant that targets a protein on the surface of B lymphocytes, a type of white blood cell. In addition to organ transplant rejection, many diseases characterized by abnormal B cells can be treated with the drug, including leukemias, lymphomas, and autoimmune disorders. By destroying B cells, which sensitize the immune system to invaders, real or imagined, the medication should quiet my immune system, making me less prone to lupus flares. Theoretically, it should also slow the progress of my respiratory disease, which is essentially lung scarring due to chronic inflammation. However, disrupting an overactive immune system is a bit like taming a vicious guard dog. It no longer indiscriminately attacks all passersby, but it might let in the burglar it used to scare away. While the tradeoff for less inflammation includes greater infection susceptibility, both my pulmonologist and rheumatologist are attempting to keep the underlying cause of my lung condition stable during my possibly lengthy pre-transplant wait. My rheumatologist also initiated rituximab as a step toward reducing my steroid dose. Although my prednisone dose isn't especially high, higher doses are associated with surgical complications, poor wound healing, and osteoporosis. In anticipation of transplant surgery and long-term steroids post-operatively, reducing my overall steroid exposure now is almost guaranteed to be of benefit. Since rituximab can also be used as an immunosuppressant after transplant, it may be a head start on anti-rejection medications as well. 

Because rituximab is only administered intravenously, I spent last Monday at the UPMC outpatient infusion center. Six open cubicles form a “J” along the edge of small room, and a stream of patients flows through as treatments end and begin. A rituximab infusion often causes a mild reaction during and for a few days after the treatment, including headache, skin flushing, and fatigue. To mitigate those effects, the initial dose is typically administered over five to six hours. Most patients are premedicated with oral Tylenol as well as IV steroids and Benadryl. However, a steroid dose above twenty milligrams per day requires inactive status on the transplant list, so I only received Tylenol and Benadryl. Fifty milligrams of intravenous Benadryl is a lot for someone who typically takes half a pill for occasional allergies. Halfway through the two minute dose, the room began wavering. The nurse recommended that I close my eyes, but that only induced rapid swirling. After alternating between waving and swirling a few times, I stopped resisting the wooziness and relaxed in my recliner. Then the real medication started. The infusion burned a little, a thread of discomfort wending up my hand, up my arm, then dissipating. After twenty minutes or so, I stopped noticing the rhythmic zing of slight pain and fell asleep, awakening for Peter's occasional visits to keep me company.

The session finished in mid-afternoon. As a treat for getting through my first dose, Peter took me to the Jacques Torres pop-up shop for hot chocolate. Then, we went to the grocery store to shop for dinner. I was in charge of vegetables; Peter was responsible for protein. I managed to select and bag broccoli and Japanese sweet potatoes before realizing that I was no longer wearing my wedding band. All the fluid from the IV infusion made my left hand swell, so I took off my ring afterward and put it on my other hand, incorrectly thinking it would stay put. I was totally panicked but still loopy enough from the Benadryl that I could only stand in front of the potatoes in bewilderment. Fortunately, Peter's detective skills were sharper than mine. After spending five minutes removing and replacing all the potatoes in the bin, he quickly tracked my ring to the floor in front of the broccoli display. He put my ring on his pinky for safekeeping, and shuffled me through the checkout to the car before I could lose any more valuables, insured or otherwise. Of course the Benadryl wore off around midnight, when I should have been ready for bed. I felt inspiration stirring as I one-finger pecked blog ideas into my phone in the dark. The next day, I had some mild skin reddening and warmth, but once they wore off, and after a few days of sluggishness, I'm back to my usual self. After my second dose next Monday, I'll wait an additional two weeks before attempting to reduce my prednisone dose. Then we’ll see if all the Benadryl befuddlement was worth it.