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.

Pulmonary rehab update

Mes amis!

With the exception of a bitterly cold day with a high of 19 degrees, and Thanksgiving Day, I have faithfully attended pulmonary rehabilitation every Tuesday and Thursday for a dozen weeks now. I still guard my energy stores avidly, but on my good days, I can squeeze in a bit more excitement. For example, last week I drove myself to and from pulmonary rehab, put in my full hour plus of exercise, hosted three midwives and a husband for our weekly get together, and still won at Scrabble after they'd gone home! On the other hand, I definitely overdid it. Showering was my most strenuous activity the following day. Yet despite my fatigue, I couldn’t suppress my satisfaction that I’m at least a little stronger than before I began this program.  

My rehab sessions have begun to follow a predictable pattern with some hard-won modifications. I always begin and end each session with five minutes of stretching to warm up and cool down. I do a circuit of "Arm-R-Cise," ten upper body exercises in a row, beginning with boxing jabs and ending with reverse speed bag (the air version of the small punching bag). Thirty seconds of each exercise adds up to five minutes of burning arms and then huge relief upon sitting down again. The eventual goal is to reach the maximum ninety seconds per exercise. "Leg-R-Cise" is based on a similar premise and ranges from hamstring curls to chair stands, repeatedly alternating sitting and standing with arms crossed x-style on the chest. I also use free weights for additional arm strengthening. During my initial sessions, I progressed from six to eight to ten to twelve minutes on the treadmill. After pleading for a bit more of a challenge, I finally received permission to advance my pace from 1.5 to a blazing 1.6 miles an hour. Unfortunately, I've maxed out my upright exercise strenuousness. Pulmonary hypertension sometimes causes a paradoxical drop in blood pressure during exercise, putting patients at risk of sudden fainting with excessive exertion. Clearly, this is suboptimal on a moving treadmill, however slowly I'm walking. Due to this limitation, my most strenuous exercise is on the Nu-Step, a recumbent elliptical machine. This decreases the concern for complications following a drop in blood pressure, since the machine is human-powered and stops when I stop. If I feel lightheaded, I'm already seated and don't risk a fall. The Nu-Step lets me feel as if I'm getting a "real" workout, even if I'm only up to level four out of ten after twenty-four rehab sessions. Since I started at level two, this feels like an accomplishment.

Repeating the same set of exercises twice weekly, however varied their order, can grow monotonous very quickly. However, Deb and Shawn, our faithful team of respiratory therapist and exercise physiologist, constantly think of new ways to make the sessions more enjoyable. Distraction is a recurrent element of their schemes. From the weekly trivia question, I’ve learned both that Jonathan Priestly discovered oxygen in 1774, and that the heaviest recorded pumpkin weighed 2,323 pounds this October. Also, you disperse 39,000 organisms when you sneeze! Their patter of light conversation keeps us energized as they come to check oxygenation levels at the halfway point of each exercise. The team’s joking banter has built camaraderie within my cohort of rehab participants, bridging the gap between me at 36 and the oldest participants in their 70s. We now inquire solicitously after each other’s pets and grandchildren and spouses like old friends, and worry about each other if someone is absent for a session. There’s nothing like a stressful experience to unify a group quickly. The double stresses of enforced exercise and transplant preparation have served quite adequately. Even if I'm not feeling my best, I always leave an exercise session feeling supported by my rehab buddies and gratified that I'm strong enough to bear the stress of transplant surgery. Officially, a course of pulmonary rehab ends after thirty-six sessions. In my case, I'll continue to attend until my transplant surgery in order to sustain the benefits of increased activity tolerance. Maybe by my next update, I'll be up to level five on the Nu-Step...

This is a special nasal cannula called an oxymizer; it conserves oxygen in the moustache to give a slightly larger dose.
My fingers are too cold to give accurate oxygen readings, so the headband keeps my forehead probe in place. Except that I have way too much hair for a measly headband. It's a good look, right?

Where are you on "The List"?

Hello, dear ones!

Last week marked two milestones for me. First, seven years of marriage with my wonderful husband, Peter, have been such a gift. In the Canticles, Solomon extols the virtues of love. "Many waters cannot quench love, neither can floods drown it." (SoS 8:7) Peter and I have faced many floods together in the past two years, both related to my declining health and the ordinary struggles of daily life. But doing the work of loving another imperfect person as well as I endeavor to love myself is a source of renewed joy and gratitude as we walk through life as partners.

Second, I came through my first set of major pulmonary testing since our move to Pittsburgh, scoring with respectable, if not flying, colors. Since our return from Cleveland Clinic, lots of you have kindly inquired about my status and wondered about my placement on "The List". For solid organ transplant candidates, "The List" both is and is not a document ranking patients with end-stage organ failure. The transplant waiting list is actually a pool of eligible recipients, all deemed sufficiently ill to require a replacement organ, but sufficiently healthy to rebound from the stresses of surgery with renewed vigor. Lung transplant candidates are assigned a Lung Allocation Score (LAS) based on multiple factors including age, blood and antibody type, disease diagnosis, relative lung function (disease severity), BMI, distance walked in six minutes, and minimum oxygen dosage at rest. The LAS attempts to balance the likelihood that a person will survive another year without a transplant with the likelihood that the person will thrive post-transplant. Scores range from zero to one hundred, with an LAS in the mid-thirties as the minimum for transplant listing. While sicker patients have higher scores, this alone is insufficient for a match. When an organ becomes available, an algorithm eliminates obvious incompatibility: candidates with the wrong blood or antibody type, height, and other medical factors. Remaining candidates are ranked based on still more factors, acuity and possibly proximity to the transplant center among them. For lung transplants, time on the waiting list rarely affects candidate selection; it's considered only as a tie breaker for two patients in the same geographic zone with identical scores.

While people often imagine jets zipping across the country, carrying organs in coolers to and fro, eighty percent of donor organs are transplanted to local recipients. The US comprises eleven geographic regions. Generally, donor organs are first offered within their own region, then to adjacent regions, and finally to more distant regions, in hopes of rapid transplantation and minimized complication. Removed from an active circulatory system, an organ's limited shelf life requires prompt transfer to another living host. While timing varies, lungs need to find a new home in approximately six hours, not enough time for zipping from Seattle to Orlando with an organ still intact. 

When I saw my pulmonologist in Cleveland last Thursday, she mentioned that she'd recently had a near match for me, an almost good enough set of lungs. She received an offer for the lungs of a six year old that were a suitable size, except that the trachea was too small an airway for an adult. Pediatric candidates receive priority for pediatric organs, but adults can receive these organs if they match donor criteria. While I felt encouraged that my transplant is truly possible, I wasn't sure how to feel about the potential donor. It's disheartening to think that a six year old's lungs are adequate to meet my body's oxygenation needs, and also to consider that a family had to contemplate donating the organs of their six year old child. So far I've been listed at Cleveland Clinic for one year and five months, and at UPMC for six months. Some days, it's disheartening to consider how long I've waited and how much longer the wait could be, but this is the only legal organ transplantation system we have. And yet I see the blessings of my life very clearly. According to the Organ Procurement and Transplantation Network (HHS), an average of twenty-one people in the United States dies each day while awaiting transplant. In the last two weeks, two of my pulmonary rehab cohorts have been taken to the ED during our sessions due to acute respiratory illness. For all my struggles, I enjoy my life in relative health. Though the journey is long, I'm still satisfied to be me.

Oxygen for Cleveland: squeezing R2D2 in X-wing fashion

Pittsburgh Symphony Association "Flaunting the Flutes" fundraiser

Pulmonary rehab

Hello, wonderful ones!

UPMC pulmonary rehabilitation is now in session! Every Tuesday and Thursday until the end of December, lunchtime will find me with a group of fellow pre-lung transplant patients, hoping to mitigate the effects of our disease process. In twelve weeks the program attempts to improve a patient's quality of life by enhancing overall functional status and ability to perform activities of daily living. Chronic lung disease patients, those with illnesses such as COPD, cystic fibrosis, and pulmonary hypertension, quickly learn to dread shortness of breath and the accompanying panic that "I can't breathe!" We'd do almost anything to avoid it: skip stairs, avoid even slight inclines, skip beach trips to avoid the extra effort of walking on sand. But curtailing activities only worsens endurance and muscle strength, making each action even more energy consuming. A primary goal of pulmonary rehab is improved activity tolerance and resulting shortness of breath. Each ninety minute session includes education and exercise, stretching, weight and resistance training, treadmill, and seated elliptical, under the supervision and guidance of an exercise physiologist and a respiratory therapist. Multiple patients complete the exercise program during their scheduled time slot, each with instructions tailored to his or her disease process and physical fitness level. 

I was a little nervous to start an intensive exercise program, even one as individually tailored as this one. My first day included a reverse speed bag exercise that risked knocking myself out with uncoordinated punches. Fortunately my worst complaint was mild shoulder soreness. The next few sessions have been similar. Adequate tolerance of each rehab appointment means slightly longer or more intense sessions on the treadmill and elliptical and more repetitions of the other exercises at the next one. I'm always ready for a lengthy nap afterward, and my energy level for the remainder of the day ranges from slightly less peppy to exhausted. Progress is tricky, following the tradition of the tortoise, not the hare. Years of twelve hour labor and delivery shifts left me with good enough muscle tone to jump right in after nine months of physical activity confined to daily walks. However pulmonary hypertension can have the paradoxical effect of lowering blood pressure with physical exertion; my blood pressure can even continue to drop after I stop to catch my breath. I'm now up to twelve minutes on the treadmill but might have to switch to hallway walks, since moving treadmills and potential fainting spells are a risky combination. Even so, I'm enjoying the sessions. The repetitive actions are soothing, even meditative, a reminder that most of my body works pretty well. And I'm slowly adapting to the extra energy expenditure. Last night, Peter and I finally were able to reinstute date night, our beloved weekly tradition. The dinner at Piccolo Forno was satisfying. Even better was enjoying an evening conversation with my husband in a beautiful public space without fighting waves of drowsiness. 

A double lung transplant is a risky and potentially life-threatening procedure. If my diligence at pulmonary rehab facilitates a rapid and strong recovery from the surgery, then it's well worth the effort. The health benefits and eventual extra energy I reap now are a welcome bonus. 
    PSA: Flu season is upon us and lasts through spring. If you are able, please get your flu shot! Even if you're healthy enough to fight off the virus with no ill effects, others you may expose, especially those medically unable to receive the vaccine, may not fare so well. Please care for your health! It's an irreplaceable gift.

Pittsburgh evening clouds

Looking homeward from the 16th Street Bridge

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