By Karen Couture
Part One: July 11, 2002
Click here to read Real Heroes of Transplantation
Part Two: January 17, 1996
Listed For Life, How I came to be on the lung transplant waiting list
On December 15, 1991, I was hospitalized with my first lung collapse. Three weeks and three lung collapses later, I was diagnosed with Lymphangioleiomyomatosis (LAM), a rare lung disease that affects only women. While in the hospital, doctors told me some people could live for 20 years with this disease — I figured it might take me that long just to learn how to pronounce it. Anyway, they said they were going to try hormone therapy first, and if that didn’t work, I’d be listed for a lung transplant.
“Lung transplant!!??” I asked, “Isn’t there something less drastic we could try?” Heck, I couldn’t even believe I had the disease, let alone, consider the idea of a transplant. Why me? I was always so healthy. I couldn’t even say the words “lung disease” for a long time. I used to tell people I had a “lung condition.” But the fact that I needed a lung transplant seemed too difficult to accept. I refused to believe it — in the beginning anyway. I told myself I would be an exceptional patient. I would do everything my doctors prescribed. I’d exercise, eat well and keep a positive attitude. It wouldn’t happen to me — Right?
But here I am, four years later, waiting for a lung transplant. My path down this road has many hills and valleys, and turns taken due to unforeseen consequences and coincidences. The decisions I made were the best I could given the information I had at hand. Here is my story in hope that it will help others in a similar situation. Remembering that medicine is an art, not a pure science.
In the first year of my diagnosis, my breathing tests began drifting slowly downward. The doctors weren’t that concerned because I never lost more than 10% at any one time. After three months of physical therapy, I increased my pulmonary function tests (PFTs) to an all-time high of 56% of predicted value for a person of my age and size. I received monthly Depo-Provera injections at 200 mg; much lower than the recommended dosage of 800mg. The doctors hoped that the lower dose would minimize the drug’s effects on me, which were identical to menopause. Here I was, at the tender age of 32, experiencing my first hot flashes.
In 1993, I started to notice blood when I coughed. This upset and surprised me, but the doctor told me that it indicated that the disease was progressive in nature and not a static process. I decided to do whatever I could to make this go away. Despite six months of exploring Chinese medicine and acupuncture, I was still getting sicker.
I started visiting Boston’s medical libraries about this time and read anything I could get my hands on by searching Medline, a computer index put together by the National Library of Medicine. Even though my doctors optimistically reported that some LAM patients lived for 20 years with the disease, my research lead me to a startling prognosis: a 5 – 10 year survival rate after diagnosis was generally expected.(1) That first year, I experienced a 15% drop in lung function.
The following year, I caught colds that would linger and fester in my lungs for a month at a time. My lower right leg began to swell, causing a great deal of pain. At this point, the doctors sent me to rehab to try to build my self up. They also doubled my dosage of Depo-Provera to 400 mg. They never considered removing my ovaries, as some have recommended, even though the downward trend indicated that the medication wasn’t working. They felt the chemically-induced menopause was the best option because of my age. I tended to agree.
At this point, I was getting very short of breath merely attempting to walk around. Small inclines began to look like Mt. Everest. And stairs? They were out of the question. Then I learned about Lupron, which had been used for fibroid tumors and endometriosis. I began taking injections of 7.5 mgs monthly to see if it would have any positive effects. In hindsight, I realize I was grasping at straws.
By July of 1994, my PFTs continued to drop. I actually plotted my test scores over the years on a chart, so I could see how fast and how far I was declining. It wasn’t a pretty picture. The two breathing test figures that we had been watching very closely were the FEV.1 (forced expiratory value in one second) which was now at 38% of predicted and the MVV (maximum voluntary ventilation) which was at 25% of predicted.
Eventually I was referred to Mass. General Hospital for a lung transplant evaluation. It was November by then, and I had stopped taking Lupron and went back on an elevated dosage of the Depo-Provera at 800 mg. The inpatient evaluation, which took five days, consisted of a battery of tests and consultations; including blood tests, cat scans, EKGs, electrocardiograms, exercise studies, PFTs and ultrasounds.
Basically, the doctors were studying my case to determine if I was sick enough to warrant the transplant and, ironically, if I was well enough to survive it. They looked for signs of an active infection or systemic disease such as cancer or hepatitis — any thing that might rule me out as a candidate for a successful lung transplant. The evaluation also consisted of numerous consultations with transplant team specialists; including social workers and psychiatrists. These individuals determined whether I could be compliant with doctors’ orders and had a reliable support system and necessary coping skills to survive the wait and recovery period. It was during the transplant evaluation, doctors discovered that I needed to be on oxygen full-time. This news was a big blow; it didn’t fit in with my image of who I was. (So much for being the exception to the rule.)
On December 12, 1994, my name was officially added to the national lung transplant waiting list, along with 1,254 others.(2) This computerized list is maintained by the United Network of Organ Sharing (UNOS). While most people have heard of the list, few understand how organs are allocated among the more than 275 transplant centers in this country.
All organs are allocated according to a series of point systems. Transplant candidates get “x” number of points for the right blood match, the right size, the individual’s geographic location in relation to where the organ is recovered, and how long they’ve been waiting. For kidney, bone marrow, and some heart transplants, a tissue match must be made. But for lungs, tissue typing isn’t practical due to the need to retransplant the organs as soon as possible. Lungs are only viable without a blood supply for up to six to eight hours, which limits the range organs can be shared among the different regions within the country.
Two weeks after being added to the waiting list, I ended up in the hospital again with more lung collapses, making a grand total of six. Now I had surgeries (sclerotherapy and pleurodesis) on both lungs. As a result, I lost even more lung function and went down to about 23% which meant that I couldn’t return to work and had to go on disability.
Once I got out of the hospital, I started attending a support group for patients waiting for lung transplants at Mass. General Hospital. There I was exposed to patients who were both pre- and post-transplant. Most of the post-transplant patients were doing pretty well, which was very encouraging. However, I did start to notice some trends. It seemed there were an awful lot of patients who had been waiting for more than two and three years. I began to worry. Not only did I fear having a rare disease; needing a transplant; but I feared I would never get the chance to have the operation in the first place.
At the time I was listed, there were about 120 people waiting for lungs in my area; 55 of them were the same blood type as I was, and were ahead of me on the list.(3) New England, which is in UNOS region one, was only doing about 20 a year at that point. I could do the math, and it just didn’t seem realistic that I could get my transplant in time.
Finally my fear turned into action after I had a conversation with a mother whose daughter had had LAM and died while on the waiting list. I immediately obtained opinions from my primary care physician, my lung transplant doctor, my pulmonologist and an outside lung transplant specialist. I needed a reality check. Was I being too emotional and not seeing things clearly? All of them agreed that it was medically necessary for me to leave New England and go to a region with a shorter wait. With the help of my primary care doctor, we petitioned my HMO to allow me to go outside of the region; my request was approved in May 1995.
Armed with a list of all 76 lung transplant centers in the country, I began the selection process to narrow the list down. I chose centers that had been established for a long time, had performed quite a number of transplants and had good survival statistics. In addition to that criteria, I also wanted to know which centers had transplanted LAM patients and their results. It turned out the one center I could find with the most experience, had only done about four.
So, I compared survival statistics, estimated waiting times and asked whether the center felt a single or a double lung would be considered in my case. Unfortunately, there is neither a consensus within the medical community on how to treat LAM patients, nor agreement on whether a single or a double is the best course. However, in my research, I learned that people who received double lung transplants (referred to as bilateral single lung transplants) had infectious diseases like cystic fibrosis and bronchiectasis. Everyone else received single lung transplants.(4) If doctors can save two lives, instead of one, isn’t that better given the shortage of donor organs? While it is true that a person can live on one lung, he or she would be lucky to regain 60% of prior lung function; whereas, a person with a double lung regains nearly 90 – 100% function.(5) People getting double lungs also have a better long-term survival rate over the singles.(6) This is probably because the remaining diseased lung creates problems that the new one can’t overcome.(7) I also found a recent case report that confirmed a recurrence of the beginning stages of LAM in the donor lung when a single lung transplant was performed.(8)
After spending a great deal of time conducting research, which wasn’t exactly scientific, I determined that the two most important things for me were to get into a center willing to give me a double lung and one with a much shorter waiting time than New England. I had determined that Loyola University, which was in Chicago, would be my top choice. They had a wait of approximately six months for a double lung, compared to the more than two year wait in New England. In addition, they typically give people under 45 years old a double lung as a matter of policy.
One of the most frustrating realizations of this whole process was how hard it was to get into a program outside of my region. Loyola was interested in having me as a patient, but they would not transfer my accrued waiting time from New England because it would give me an unfair advantage over their local patients. Therefore, if I wanted to go to Chicago, I was going to have to start from zero, which I didn’t think I could do. Plus, without family there and my health deteriorating I really needed somebody to help take care of me.
I then considered going to a new center at Shands Hospital in Gainesville, Florida. They had already done more lung transplants in the year and one-half since they opened in April 1994, than New England had in the past five years. Equally important, they had a short wait, were close to family, and it would be warm in the winter.
However, because I wasn’t a Florida resident, they weren’t sure they should allow me into their program. Eventually, they decided that I was an excellent candidate because of my overall health and age, and the fact that I would have a good support system there. They agreed to an evaluation and, eventually, accepted me into their program. I moved to Florida to live with my father this past fall. Where I have been waiting, ever since.
Currently in this country there is a severe shortage of organ donors. While about 43,000 patients wait for transplants, only about 8,000 people become donors each year — that’s both living-related and cadaveric.(9) Unless the supply increases, people will continue to die on the waiting list at the rate of 8 – 10 people a day.
If you are considering whether or not you need an organ transplant, I would first suggest that you talk to your doctor to make sure all other avenues have been pursued, including treatment options that might have been overlooked or ones you may need to reconsider. Also, I would suggest you start looking into which centers are closest to you. Remember, no center will be the best for everyone. Consider whether you feel comfortable with the program, whether you agree with their assessment of your condition and whether you can live with their average wait. Also the criteria for being put on the waiting list, varies from hospital to hospital. There are no standardized guidelines that need to be met in terms of your diagnosis, your condition, your medical history, method of payment, etc. If one center says no, look at another one. I, myself, have been turned down by two different centers because the pleurodesis I had on my lungs was considered a problem.
I’d also like to mention even though transplantation is a recognized therapy for end-stage organ failure, transplants are not considered a cure. As one doctor told me, “It’s like trading one set of problems for another set of problems…hopefully, for ones that aren’t life-threatening.” Some of the long-term responsibilities of having a transplant include taking immune-suppressive therapy for the rest of your life, which involves large quantities of expensive medications that have numerous side effects, some of which can be potentially serious. Cyclosporine, one of the major immune-suppressive drugs, can contribute to renal disease.(10) And the fact that the immune system has been suppressed to keep the body from rejecting your new organ, also increases one’s susceptibility to other diseases. There is a documented increase in the number of tumors in transplant patients, particularly cancers involving the lymph systems.(11)
I think people need to be aware of some of the problems associated with transplants; however, most transplant recipients tell me it’s worth the wait and the changes they see in their lives are often for the better. Successful lung transplantation has come a long way since 1983. In the last 10 years, great strides have been made to improve the surgical technique, new medications have been released and detection of rejection is more sophisticated. The national survival statistics for all types of lung transplant patients are at 71% in the first year — more like 80 – 90% for younger, healthier patients. This figure reduces about 5 – 10% a year. Currently, five years out, the survival rate is about 50%.(12) But we can expect these figures to improve as centers gain more experience with the procedure.
So I have a 50% chance of survival after five years. And as my father tells me, “you can put a lot of good livin’ in five years!” This is exactly what I hope to do. But for now, I must wait.
For information on how to become an organ donor call 1-800-24-DONOR
REFERENCES: (1) Taylor J, Ryu J, Colby T, Raffin T. Lymphangioleiomyomatosis: clincial course in 32 patients. N Engl J Med 1990;323:1254-60. (2) HRSA (Health Resources and Services Administration) fact sheet, February, 1994. (3) New England Organ Bank (4) Davis Jr. R, Pasque M. Pulmonary Transplantation. Annuals of Surgery 1995, 221:14-28. (5) Davis Jr. R, Pasque M. Pulmonary Transplantation. Annuals of Surgery 1995, 221:14-28. (6) Montoya A, Mawulawde K, Houck J, Sullivan H, Lonchyna V, Blakeman B, Hinkamp T, Garrity E, Pifarre R, and the Loyola Lung Transplant Team. Survival and functional outcome after single and bilateral lung transplantation. Surgery 1994;116:712-8. (7) Chaparro C, Maurer J, Chamberlain D, De Hoyos A, Winton T, Westney G, Kesten S. Causes of Death in Lung Transplant Recipients. J Heart Lung Transplant 1994;13:758-66. (8) Nine J, Yousem S, Paradis I, Keenan R, Bartley G, Griffith B. Lymphangioleiomyomatosis: Recurrence after Lung Transplantation. J Heart Lung Transplant 1994;13:714-9. (9) UNOS Update, Nov. 1995, p34. (10) Davis Jr. R, Pasque M. Pulmonary Transplantation. Annals of Surgery 1995,221:14-28. (11) Chaparro C, Maurer J, Chamberlain D, De Hoyos A, Winton T, Westney G, Kesten S. Causes of Death in Lung Transplant Recipients. J Heart Lung Transplant 1994;13:758-66. (12) Washington University School of Medicine, St. Louis International Lung Transplant Registry, September 1994 report.
Update February 1999 Karen received a double lung transplant from Shands Hospital on January 21, 1996. She is doing great and won two bronze medals at the 1998 US Transplant Games. Karen also published a book, Information You Should Know about Transplantation: Before, During and After.