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!

Slow and steady

Hello, everybody!

I've heard murmurings through the grapevine that folks would love an update on my health and general status. Thank you so much for your concern and for checking on me so faithfully. I'm doing moderately well. I tire easily, of course, but on my pulmonary rehab six-minute walk this week, I walked one hundred feet farther than I did for my intake last fall. I'll definitely be at my strongest for the transplant! On the other hand, activities from showering to walking to putting away dishes require twice as much oxygen now as they did when we arrived in Pittsburgh last August. My oxygen level while resting and without supplemental oxygen has drifted down from the high 80's to the high 70's. (Normal would be 96% or greater without extra oxygen.) I feel this change acutely. Although friends say I seem the same in conversation, my thoughts feel fuzzier. I search for the most precise word more often than in the past. A transplant social worker told me that this is a common complaint: the higher one's oxygen needs, the stronger the perception of foggy mental processing. 

Although writing is a bit more laborious, my time with friends here has grown richer and more frequent. I cherish our pedicures, sci-fi/fantasy lectures, book club discussions, and burger nights. My Pittsburgh friends are the gift I couldn't have imagined after leaving our beloved community in Boston. Sustained by kindness and affection from near and far, I keep writing, little by little. I'm almost always working on a blog post, usually two or three, but time slips past me faster than before. Things take longer. Sometimes I struggle to decide whether to give up on a post about an event a month past or to continue revising to incorporate more recent happenings. I promise (tentatively) to have another post up in the next week. I've also decided to take a few more photos of daily life so you can "see" me more often. Most importantly, I'll keep having adventures, so I have something to write about.     

Flowers at 4121 Main