In and Out: November 2013

A Letter
This is the letter I wrote last February to the Lung Transplant Committee at the University of Pittsburgh, where Colleen and I went for her week-long evaluation. For five days, I carried copies of the letter with us to every appointment and handed them out to each of the key doctors, who would ultimately be making the decision whether or not to put Colleen on the transplant list, doctors in Rheumatology, Cardiology, Gastroenterology, and Pulmonology. We also handed a copy to the lung transplant surgeon, one of only a select few, who would have been Colleen's surgeon. We learned that, in spite of its length, they all read the letter, to which I attached photographs of Colleen, lest they forget the face attached to the name.

I'm including it here because it fills in a bit more of Colleen's scleroderma story. I'm also attaching the pictures.

and recur, pass and recur. And thus began the physical, emotional, and spiritual journey that now—a long, 22-years later—brings her to UPMC and to you. We, her family and friends, have born witness to the hundreds upon hundreds of physical assaults, losses, medical emergencies, doctors’ appointments, fear and feelings of aloneness that scleroderma has thrown at Colleen unceasingly for all this time. We’ve watched her live with this disease of attrition, day by day, and prepare, more than once, to die from it—with indomitable courage, honesty, strength, and always that smile of hers. Remarkably, I can’t recall ever hearing Colleen complain or exhibit a shred of self-pity—tears, yes, self-pity, never.

The first doctors Colleen consulted were stymied by her symptoms. Both Lyme’s disease and lupus were considered, but ultimately ruled out. The University of Pennsylvania diagnosed Undefined Collagen Vascular Disease, which later came to be called Mixed Connective Tissue Disease, a diagnosis we understood to be an umbrella for autoimmune collagen diseases. Searching for a more specific diagnosis was prolonged and taxing, as was, and still is, the repeated trial-and-error of seeking the right medications. From the time of its arrival in her life, up until this very moment, scleroderma has required nothing less than herculean patience of Colleen—and heroic acceptance.

In Year 3, Raynaud’s came to stay. Year 4 brought a diagnosis of Adult Still’s. Year 8 hospitalized Colleen with acute pericarditis. Commonplace were mouth sores, high fevers, night sweats, swelling, pain, backache, and bronchitis.

Colleen continued teaching a full-course load and raising her children, never stinting on family vacations or fun of any kind, never missing her daughter’s school plays or her son’s karate competitions, never forgetting one of her many nieces’ or nephews’ birthdays. If you asked her how she was doing, invariably she’d say, “There are good days and bad days.”

In Year 12, Systemic Scleroderma was diagnosed and Colleen was given a 5-year prognosis. That was 2002—ten years ago.

A few years prior to that prognosis, Colleen began doing yoga, which she fell in love with immediately, in spite of the daunting contraction and stiffness she faced from years of scleroderma. Feeling her body unfold, millimeter by sometimes excruciating millimeter, was a joy beyond measure for Colleen. Her mantra: “I want to touch my toes, I want to touch my toes.” My sisters and I began envying the muscle definition developing in her upper arms. She practiced yoga daily, both at home and in the studio. Her example inspired me to find my own yoga practice. And I watched it inspire other students when the time came that Colleen had to start bringing her oxygen tank with her to class.

During Years 13-15, heart complications meant hospital stays and eventually a pacemaker. Colleen joined a clinical drug trial at Robert Wood Johnson Scleroderma Program in New Brunswick, a two-hour drive from Colleen’s home at the South Jersey shore. The program’s Dr. Vivien Hsu becoming her primary scleroderma doctor at that time.

Year 16 marked a watershed. Colleen began oxygen therapy for routine housecleaning, for exercise—yoga, outdoor walking, treadmill—and for sleep. By the next year, Year 17, she was on oxygen 24/7. The good news was that she had made it past her dire 5-year prognosis. The bad news was that she was now permanently tethered to oxygen equipment.

By this time, Colleen had moved from classroom teaching, which had become too difficult physically, to becoming head of the school library, a role she loved. All those books. All those potential young readers. Outside of work, she began travelling, singlehandedly making all the necessary O₂ arrangements with airlines and with her many destination points, including a most ambitious journey to Ireland, our ancestral home. She also began co-teaching a hatha yoga class at at her local Gilda’s Club and, looking ahead, trained to teach Chair Yoga as well.

Year 18. Colleen was diagnosed with Hodgkin’s lymphoma. She made it through six months of chemotherapy, lost her hair, and wore cute hats. I never heard her lament aloud about her lost hair nor, before and after cancer, about her premature aging from scleroderma—beauty blows that would take most women down.

The cancer went into remission and Colleen resumed travelling—until she no longer could. Two years ago, in Spring 2011, she came to visit me here in Santa Monica. We went to the theater a few nights in a row and during the day made our final preparations to leave for Yosemite, a six-hour road trip we were over-the-moon excited about, Colleen’s O₂ needs already arranged and taken care of by her. But Colleen suddenly got very sick with some kind of bronchitis and immediately had to fly back to New Jersey from California.

For the entire 22 years she has lived with this disease, Colleen has pushed the outside of its envelope. I have watched her need to test the edge each and every day in order to know her limits so she could exceed them, to live her life without pulling back one second too soon. This has been her modus vivendi even during this past year when her world took a steep nose-dive. Dr. Fredrick Wigley, head of the Johns Hopkins Scleroderma Center, in conjunction with Dr. Stephen Mathai of JH’s Pulmonary Division—two remarkable and dedicated physicians—tried everything to level off the precipitous drop in Colleen’s function in order to get things trending back upward for her. No dice.

Colleen’s world has narrowed pretty much to her sweet yellow house with the massive white oak in the backyard. Her life includes a wheelchair now and Colleen has to ask for help a whole lot more than she wants to. And the O₂ can’t be cranked up any higher. Is every day for Colleen like being on Mt. Everest, in the thinnest of air, with no oxygen mask? Probably. We try to comprehend what it’s like not to have been able to take a deep breath—for years. We can’t comprehend this.

Hardest for Colleen to accept in the past 20 months, though, might be the loss of yoga, which was integral to her daily life and sustained her physically, emotionally, and spiritually for more than a decade. She talked to me last summer about knowing that as each day passes without being able to “get on the mat,” her hard-won elasticity and ability to touch her toes was slipping away.

The heartbreak was plain.

In recent weeks, since Colleen was approved for a transplant evaluation, I’ve dared envision the very best—a new pair of lungs for her, lungs that stick around, soft air-filled lungs that Colleen can fly with for however long—a year, two years, five years, ten? I’ve dared envision her completely untethered from the oxygen machine, miraculously walking with a lightness in her step again—her yoga bag slung over her shoulder perhaps—and her blissfully taking a series of slow, down-deep breaths. I also envision other outcomes.

My family and I know that Colleen could be in no better hands than yours. Godspeed in your evaluation.

Deep gratitude,

Mignon McCarthy

FAMILY SUPPORT TEAM	Proximity to Colleen’s Home
Suzanne McCarthy, mother, Ocean City NJ	10 min
Camille McCarthy Nickles, sister, Ocean City NJ	10 min
Leslie McCarthy Phillips, sister, Ocean City NJ	10 min
Kirsten Wenzel, niece, Ocean City, NJ	10 min
Dan McCarthy, brother, Ventnor, NJ	20 min
Sean McCarthy, brother, Mays Landing, NJ	20 min
Chelsea Di Pilla, daughter, Margate City, NJ	20 min
Ben DiPilla, son, Atlantic City, NJ	25 min
Kim McCarthy, sister, Boston, MA	6-hr drive, 2-hr flight
Myself, Mignon McCarthy, sister, Santa Monica, CA	5½ hr flight