Moving

Hello, friends!

We've moved! About six months into our move to Pittsburgh, Peter and I started discussing longer term housing options. At every appointment, my doctors in Cleveland and Pittsburgh reiterated their belief that my wait for "Edwina-sized" lungs would likely be significant. Our apartment was perfectly adequate, spacious enough, comfortable enough, accessible enough. But it was nothing like we'd imagined our home would feel, regardless of our city of residence. Maybe we could feel differently in a neighborhood we loved and embraced. 

Given the prospect of an extended Pittsburgh stay, relatively inexpensive local real estate, and the positive ratio of mortgage to rental prices, buying seemed an obvious choice. The Pittsburgh real estate market was an eye opener. We quickly recognized that the highlight of Pittsburgh's landscape, all of its incredible hills, had created an architectural roadblock to success. Very few people live in condos. Almost everyone lives in a house, and as in Boston, the housing stock is old. Due to the hilly landscape and Pittsburgh tradition, houses are constructed with three stories and a basement. (Technically, the third story is a finished attic.) In addition to the many stairs required to get from the typical basement laundry to attic office space, external access is a problem as well. Even in pricier neighborhoods, there are often a minimum of five or six steps to enter a home on the flat side of the street, fifteen or twenty steps on the hillier side. In steeper, less prosperous neighborhoods, fifty steps are possible to reach a house from the street. Additionally, many homes are without a first floor bathroom; a first floor bedroom is even rarer. Old houses often have narrow staircases, too narrow to install a chair lift for second floor access. Newly renovated homes with wider staircases blurred together in my mind, one cookie cutter, HGTV clone with an open concept and crown molding running into the next. The only rancher we viewed had "updated" bathrooms in 1970's baby pink and blue. The housing market moved quickly, even in the dead of winter. Houses we asked to tour with our realtor often had several offers before we could tour them, just days after listing. Exceedingly frustrated, we started looking into building an accessible home, but had even less success. We went back to Zillow stalking, unsure of what to do. Eventually, we decided to revisit a house we'd already toured. Accepting that the brick rowhouse ticked all the requisite boxes, we filled out the thirty page document required to make an offer, hoping it was for the last time. And we got the house! 

Kitchen

We moved in a few weeks ago with generous assistance from friends and family, midwives and church members. My brother and sister-in-law passed through on their way back to Atlanta and helped me pack up the apartment kitchen. Peter's parents worked all weekend, packing, cleaning, installing electrical outlets, and otherwise making themselves useful. Friends came in shifts all day Saturday, emptying our apartment, then filling our house, almost every box in its proper room. Two hired movers did the heavy lifting, toting mattresses up the stairs and reconstructing our glass-topped table. Sitting on the couch at the end of the day, our house looked more like we'd had a boisterous party than that we'd moved in at noon. With a stairlift to assist my travels between the first and second floors, I can independently reach all the rooms except the third floor office. With an outdoor space to design, I can finally get the hammock I've been clamoring for since Peter and I got married almost eight years ago!

Dining room

For Peter, a bonus of our move is proximity to three of his favorite coffee shops. For both of us, living a block away from La Gourmandine Bakery is both a constant temptation and frequent reward. Butler Street, the main street through the Lawrenceville neighborhood, is just two blocks away and lined with restaurants, boutiques, tattoo shops, and an independent movie theater. Almost every local we inform that we've moved to Lawrenceville tells us the neighborhood is "the up and coming spot." Regardless of the neighborhood's relative popularity (or our shame in encouraging gentrification), its greatest perk is proximity to friends. Our backyard is steps away from that of a midwife friend and her husband. We joke that we won't even have to get dressed to borrow that proverbial cup of sugar. Our decorating is far from finished, but we can share a few photos of our progress so far. As always, we miss you all and would be delighted to see you any time you're passing through Pittsburgh.

Back yard

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

Right heart cath update

Hello, all!

In the last few weeks, I’ve seen both of my transplant pulmonologists at UPMC and Cleveland Clinic. I was somewhat alarmed to be informed at a routine visit that I’d become the subject of their Sunday night conversation, two medical directors of transplant pulmonology commiserating about a mutual patient. Both physicians were concerned about a recent near-fainting episode I had that resulted in an overnight hospitalization. The surrounding circumstances, fatigue, slight dehydration, and a recent medication change, were certainly sufficient to account for the event. On the other hand, nothing like this ever happened before. The doctors assumed this was a symptom of worsening underlying disease but wanted more data. Their plan was a right heart catheterization, the gold standard for diagnosis and evaluation of pulmonary hypertension (RHC). This test evaluates the health of the heart muscle and valves, and measures pressures within the heart and lungs. First, a physician inserts an introducer sheath, similar to the elongated part of an IV that stays inside the vein. Then, a pulmonary artery catheter passes from the internal jugular in the neck to the superior vena cava to the right atrium and right ventricle of the heart to the pulmonary artery, measuring pressures and blood flow as it progresses from one chamber to the next. Typically the test is performed while the patient lies supine on an operating table, relaxed but motionless. However a simulation of the exertional dyspnea (shortness of breath with activity) that I experience when mobile can document more accurately how much resistance the heart is working against when pumping blood to the lungs. To assess the effects of physical activity on pulmonary hypertension, the patient additionally pedals a cycle ergometer while a physician continues to take measurements.

In preparation for the catheterization, I juggled three sets of pre-procedure bloodwork orders, two on paper and one sent electronically. When I left the lab last Tuesday, I assumed all appropriate tests were drawn. Unfortunately, the cardiac nurse practitioner found no record that clotting studies were drawn, so I needed a second set to be redrawn on the day of my RHC. After following instructions to fast from all food and liquids after midnight, I didn’t have much to offer in the way of veins or blood. The first vein, one in the bend of my left arm, looked promising initially but the IV wouldn’t thread. The second one vanished as soon as the needle went in and never reappeared. For the third attempt, I made an executive decision to direct the medical assistant to utilize the basilic vein on the back of my forearm; it’s in an odd position but a really easy IV insertion.  

In the cardiac catheterization lab, despite initial palpitations (PVCs) from the curve of the catheter, the first part of the procedure was straightforward, all pressures and volumes measured and recorded. Then, the pulmonary vascular nurse scooted me down the procedure table to strap my feet into the cycle ergomer pedals. The timer started, accompanied by a beep to keep my cycling rate at sixty rotations per minute. The cycle resistance gradually intensified to a maximum of 3.2 mets after almost eight minutes. Huffing and puffing with pulse flying, I was relieved to slump against the table while my heart slowly, slowly quieted to one hundred beats per minute, just above my typical heart rate.

Ironically, the testing results will have little impact on my daily life. My doctors will tweak a few medications. I’ll start seeing the doctor who did the catheterization for closer pulmonary hypertension monitoring. The results will be entered into the algorithm that calculates my lung allocation score, and my LAS might even rise a few points. But overall things will stay about the same, and I’m content with that. So much has changed in the last half year: the friends I grab for a spontaneous pedicure, the doctors who manage my health most closely, my go-to hot chocolate and tea shops, the distance I can walk in six minutes, the place I call home. The rhythm of daily life has changed as well, now structured by Tuesday/Thursday pulmonary rehab and once weekly mentoring. Most of these changes have been beneficial, contributing greatly to my happiness in Pittsburgh, but all change, even good change, can be stressful. I’m gratefully relieved to recognize my treasured constants: my community of family and friends, and my faith. Most days, my body repeatedly lets me down, failing to deliver enough oxygen or energy to accomplish all the tasks I’ve proposed, and progressively destroying my remaining healthy lung tissue. Yet aside from that immense, inescapably disruptive change, most everything else remains reassuringly monotonous. The IV that took three attempts to place never got used after a ten cc flush. The range of motion of my neck is back to normal. I finally replaced my “flesh tone” 4”x3” bandage with a Doc McStuffins band-aid. My weekly routine can continue with minimal interruption. After facing down all the other changes, I’m grateful for every serenely uneventful day that brings me closer to my transplant. 

In the recovery room with Peter post-procedure

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.

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