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!

New Year's Thoughts

December 21 marked Sunreturn, the winter solstice. This is the day we cherish for indicating, in all its brevity, a reversal of course. Now, undergirding colder and still colder days crowned with mounds of unmelted snow, is the slumbering promise of spring. Scarce hours of winter sunlight creep forward as the planet spins round the Sun, propelled toward warmth and light and verdancy. We wait in hope for crocuses and groundhogs and jacket weather.

One year, seven months, have passed since I was first listed for transplant at Cleveland Clinic. The waiting wears on me more now than it did at the beginning. Initially, I could mostly forget the reason I’d transformed my occupation from nurse-midwife to lady-who-lunches. My faithful friends made the wait seem self-imposed, a sabbatical staycation of sorts. In a new city with fewer lunching companions, my major weekly activities focus on caring for my health: doctor’s appointments, pulmonary rehab sessions, nurse coordinator check-in’s. There are fewer distractions from the reasons I’ve interrupted my life in Boston to move to Pittsburgh.

It’s been a hard year, one of repeated accommodation and mental readjustment down the continuum of my changing physical abilities. It’s uncomfortable to acknowledge the steady progression of new “normals,” each slightly less robust than the old. I often feel stuck, suspended between a past and a future when my body obeys my mind, to live in a present when the response is intermittent. This sense of liminality also applies when I attempt to plan for the future. It’s much easier to fill the day with tasks to pass the time than to prepare a timeline with a fixed endpoint.

And yet each new normal is just that. Some days, I'm walking out the front door before I remember that the fifty foot length of tubing attached to my stationary oxygen tanks is poorly adapted to riding the elevator. I can almost forget my fifth limb. Likewise, my weekly routine of pulmonary rehab and mentoring feels like my “job” for now, as distant as it may seem from twelve-hour labor shifts and prenatal visits.

Still, I glean moments of transcendence wherever I may find them: in morning meditation, in the four part harmony of Sunday morning hymns, in words of love from true blue friends. In comparison with those of the planet, my troubles are small, and I know that my blessings are many. I am fed, clothed, and sheltered. I am loved by my husband, my family, my friends. I live in peace and security, safeguarded by a building with a doorman and excellent health insurance.

This seemingly ceaseless wait will end soon. One day I’ll write to you of dancing with Peter, running along the Allegheny or the Charles, sitting through a movie marathon, all with no regard for oxygen tanks or shrinking energy stores. What are you allowed to ask for when you pray for a transplant, knowing that more life for you inevitably means another life’s termination? I ask for peace, and patience, and perseverance, and for the richest, fullest life imaginable for my donor before his or her lungs become mine. While I wait, I receive the love that each of you sends: kind words, positive intentions, lovingkindness meditations, intercessory prayers, love vibes. I send it back with gratitude for your compassion and empathy.

Thank you for waiting with me. Spring is coming. 

Happy New Year!

Phipps Conservatory Botanical Gardens

Saturday in Sewickley and the disappearing spoons - a mystery

Hello, friends!

On a brilliantly sunny Saturday not long after arriving in Pittsburgh, Peter and I were up for a little local exploration. Still slightly drained from an active week, I woke up with just enough energy for one activity. We took a picturesque fifteen minute drive to Sewickley, the closest local equivalent of a New England village. Bookstore visit, village stroll, and lunch were completed by one of Peter's favorite pastimes, sporty car window-shopping. Total time expenditure: 2.5 hours. Total energy expenditure:100%. The five minute walk from the car to our apartment was misery. Peter eventually snaked my oxygen tubing out our front door and down the hall to connect me with a higher dose of oxygen for the last few steps. Three hours after being tucked into bed, I had just enough energy to prop myself on the couch for the rest of the evening. I had used all my spoons.

Christine Miserandino (www.butyoudontlooksick.com) is a lupus advocate and blogger who created the Spoon Theory to explain the daily experience of lupus to a friend. She wanted to illustrate the necessity of making conscious choices about every energy expenditure every day for individuals living with a chronic illness. She gave her friend a dozen spoons, her energy quotient for the day and her constant reminder that she had a chronic illness. Christine took them away one by one as her friend weighed clothing choices (zipper vs buttons), transportation options (driving vs subway), and food selection (utensils vs hand held), the activities of a typical day. In theory, the subway requires fewer spoons than driving, but not while standing in the middle of a jostling, constantly shifting rush hour crowd. Cutting up food with a lupus-inflamed elbow is no fun, but neither is only finishing half your sandwich due to jaw pain from chewy bread. And not enough fuel means another spoon gets docked.

I complete a spoon count every morning. Lupus and related lung disease have made it essential. A good or bad day yesterday means more or fewer spoons today, and they have to last all day. Using all my spoons usually means my day is finished. A nap might replenish a few; a long nap might even provide an afternoon second wind, with the caveat that it will now take less activity to use up the remaining spoons. Peter often helps with my morning ablutions, not because I'm incapable of bathing independently, but because doing so usually means that after shower, clothing, and breakfast comes preferred activity, not nap. Excitement-induced distraction from "spoon management" on a less energetic day could result in that Sewickley Saturday's problem: lots of day left but no more spoons. For a really worthwhile and special activity, a friend's wedding, an evening at the symphony, I might push myself, borrowing against the next day's spoons. Such debts must always be repaid with interest, though; the next day might not bring me beyond my front door.

In some ways, the worsening severity of my illness has made coping a bit easier. With an oxygen tank in tow, at least there's conspicuous external evidence, requiring slightly fewer explanations. Even with the extra effort it takes to get to pulmonary rehabilitation sessions, the physical conditioning has gradually increased my spoon supply, not in entire spoons but a quarter-spoon here, a third there. I'm grateful for the bonus spurts of energy. I'm hopeful they'll be sufficient to tide me over until a transplant resolves the effects of advanced respiratory disease.

As always, a good day is in the framing. A good day is one when I'm still breathing, energetic enough to fuss about never having enough spoons. Every morning, I wake up knowing that I'm not alone, that love surrounds me, flowing from heaven, from friends and family, from kind strangers, from the compassion I try to grant myself. As Roethke says in "The Waking," I learn by going where I have to go. This may not be the path I would have chosen, and I certainly would have stockpiled a few more spoons, but I can't deny a tinge of elation that I'm still moving forward.    

Waiting for snow

Coffee shop tour: Part one

Hello, friends!

This morning, Peter dragged me out of bed to accompany him to one of his favorite coffee shops, 21st Street Coffee and Tea (www.21ststreetcoffee.com). Last night I (foolishly) agreed to tag along so that I could both get out of the apartment for a bit and get some editing done. But the day dawned foggy, with a hazy mist threading the surrounding foothills, the perfect sort of day for rolling over to steal another hour's rest. I felt it was best to comply with the day's obvious hints, but apparently a deal is a deal. Peter nudged me into the shower with promises of tea and pastries; the best of us is not immune to a bribe...

21st Street CaT sits near the corner of Smallman and 21st Streets in the Strip District. Historically, the area housed many mills and factories that gradually gave way to wholesale purveyors of fish, pasta, produce, and other foodstuff. However with the hipster invasion, it now additionally hosts trendy eateries, boutiques, and converted lofts, as well as a market with the most expensive bag of King Arthur flour I've ever encountered. (In its defense, Marty's Market also sells delectable chocolate covered gelato pops.) This is a great little coffe shop, comfortably spacious. The work of local artists lines walls of exposed brick. A lofted work space for telecommuters overlooks the coffee bar and multiple tables and chairs. There's a quiet buzz of background sound, enough to mask a conversation or phone call, but not so loud as to distract mental focus.

21st Street CaT brings in fresh pastries daily from two local bakeries, my newfound favorite, La Gourmandine Bakery, and Bella Christie Sweet Boutique. La Gourmandine is run by two French transplants, who make a nearly perfect traditional croissant. Light, flaky, buttery, beautifully layered, this is one of the top three croissants I've had in North America. Peter's apple walnut pastry from Bella Christie was almost as delicious with flaky crust surrounding cinnamony filling. And let's not forget 21st Street's beverages. Peter was lured here by their Intelligentsia beans, a longtime favorite, and ensnared by their coffee orthodoxy. The hardcore baristas reluctantly permit milk and sugar in their coffee but insist on dispensing it themselves in precise quantities. Efficiently friendly, they serve drinks and snacks with care. The shop carries Paragon tea in several varieties, including my favorite oolong Ti Kwan Yin. While bribery and coercion played some role in my visit to 21st Street Coffee and Tea, I'd gladly be bribed again.

Birthday musings

Hello, wonderful people!

After three weeks in Pittsburgh, I celebrated my thirty-sixth birthday last Wednesday. It was a bittersweet day, filled with reminders of how much I love my profession and with the joy of celebrating with Peter, but also with a deepening sense that I'm far from home and out of my element. While I appreciated Boston's beauty as a city of abundant greenspace, I grew to love it deeply as a place of emotional, spiritual, and occupational wholeness and community, as my home. The nearly seven years I've spent there so far are the longest I've lived anywhere as an adult, close to the longest I've lived any place ever. The transition from a place of familiarity coupled with the passing of another year is a liminal space for self-assessment as I enter a new phase of life.

I'm not thrilled about moving away from the wonderful security of my Boston support network to a city I associate with the Rust Belt and repeated blood draws. However, moving to Pittsburgh is the safest, most prudent way to care for my health at this time. So I move on, not suppressing or ignoring unpleasant feelings, but letting them run their course as I keep moving forward.

Despite any sadness I have due to leaving Boston, I'm grateful that my status on the transplant list has advanced enough to make this move a logical and appropriate one. I'm grateful that my husband's job allows him the flexibility to work from home, wherever that home might be. I'm grateful that between the Affordable Care Act and Peter's excellent insurance, there's no cap on coverage for transplant medical bills that could top $800,000. I'm grateful that we found a tenant for our Boston apartment before we left for Pittsburgh. I'm grateful for all the love still flowing from Boston, the cards and texts and phone calls and emails and Facebook posts, all rephrasing the kindness and generosity I've received in abundance over the past year and a half. I'm grateful for the gracious Pittsburgh hospitality of Peter's high school friend and his wife, who have become my friends as well. Despite the stress of so many changes, I'm blessed with a really good life.

My birthday prayers are for a more peaceful world, greater compassion, and deeper connection with others. The transplant will happen when it's time. Well, I'm off to the symphony to hear Sibelius, Smetana, Prokofiev, and Bruch as the final gift of my birthday week. It's going to be a good year!

13/Pittsburgh Symphony Orchestra 9

Settling in

Hello, wonderful people!

Today completes two weeks of residence in our new city. Pittsburgh is beautiful green hills, three big rivers, and a ridiculous number of bright yellow bridges. It is gritty in ways that Boston is urbane, a city of doers to Boston's thinkers. Friendly to a fault, the city slightly unnerved me as a place where every passing stranger says, "Hi!" But for all my homesickness for Boston's standoffish intelligentsia, Pittsburgh is growing on me. There's no shortage of hipster coffee shops, tasty bakeries or avant garde art. Plus affordable housing with indoor parking connected to my apartment building! We have a three bedroom, three bathroom apartment, big enough to comfortably host visitors and still give Peter a home office. Settling in will take some time, but it's looking promising.

Now that we're ten minutes' drive from University of Pittsburgh Medical Center (UPMC), the transplant team has interpreted our presence as an invitation to monopolize our time. So far we've spent half a day at the hospital each week, and next week promises two days of appointments. After seeing the Pulmonology and transplant surgery teams so far, I'll get set up with a primary care doctor and start pulmonary rehab. The biggest medical development to date is having to give up Darla, my portable oxygen concentrator (POC). Despite our love-hate relationship, I have her to thank for the level of independence I've maintained so far. Having a machine that will keep puffing oxygen as long as the battery is charged has afforded me the freedom to set my own schedule, as long as I stayed reasonably close to an electrical outlet. Unfortunately I need to transition from intermittent to continuous oxygen, and no POC is equipped to do that at a rate higher than 3 liters per minute. My current oxygen dose is 6 liters per minute at rest, more when I'm up and moving around, so an oxygen nurse and transplant nurse practitioner are hashing out my new oxygen doses. They will also decide whether liquid or gas oxygen will better serve my needs. They've already warned me that I'll be more limited in my activities due to the finite quantity of oxygen each tank can hold. While it's disappointing to have my movements limited even further, paring down to the essentials of a few good friends and really good chocolate is always worthwhile! There aren't any updates on how soon the surgery could be. Apparently there are fewer organ donors this year, so everyone is waiting a bit longer. Prayers, love vibes, and positive thoughts for patience and readiness are all appreciated!

Finally, sending a shout out to our families, Peter and Denise, and Julius, Linda, and Ada! We couldn't have moved in without your considerable help. No more trips to Ikea, though, right? A second shout out to Jen and Sam! Thanks for bringing Highrock to Pittsburgh for us. All other potential visitors, just let us know you're coming.

Heinz 57

Heinz Lofts

Allegheny River and Pittsburgh Hills