I'm Aware That I'm Rare: Kathleen Sheffer

For former PH patient, Kathleen Sheffer, writing has become a tool for her to process her heart-lung transplant and the events leading up to it. Kathleen candidly shares her experience post transplant. You can also follow her journey through her blog “Rose Colored Mask” featured on the phaware and The Mighty website.

Transcript:

My name is Kathleen Sheffer and I’m from San Francisco, California.

I was diagnosed with pulmonary hypertension when I was six years old. On July 1st, I received a heart-lung transplant. We talk about transplantation as the only cure for pulmonary hypertension, and while I no longer have pulmonary hypertension now, it’s really more like trading one chronic illness for another. Transplant comes with a lot of risks, just the procedure itself and then there’s also the risk of rejection, which never goes away. In addition to that, there’s risk of infection with lifelong immunosuppression. You’re really balancing rejection and infection for the rest of your life.

My goal was always to beat PH and to live long enough for them to find a cure, and maybe a medication that would reverse the effects of the disease. For 16 years I sought the most aggressive therapies. I saw multiple specialists. I exercised as much as I could and just tried to live a normal life while I waited for the cure. Some people have asked how I made the decision to go forward with a transplant. I had questions myself. I didn’t know when the right time would be. I worried that I wouldn’t be able to have a transplant in the window where I was healthy enough to have the surgery, but sick enough to qualify to be eligible for a transplant.

When it came down to it, it didn’t feel like much of a choice. On May 19, 2016, I woke up with a cough. I started wheezing and eventually spit something up. I went to the bathroom and discovered that I was holding blood in my hand. I was visiting a friend in Seattle, and as I sprayed blood all over her toilet seat she called 911. Two ambulance rides and three hospital rooms later, I learned that this event, what they called massive hemoptysis, was a serious progression of my PH. I was lucky to have survived it, and they said I was unlikely to survive another event.

I’d been dreading transplant for years. It was always the “when all else fails” option, but when my doctor called my mom, who then relayed his message to me, saying that it was time for me to be listed for transplant, I felt surprisingly calm. I was lucky that they recognized the window, and I was listed for a transplant 14 days after my hemoptysis. 28 days later, I received a call offering me a new heart and lungs. They came just in time. I wish this were always the case. I’ve watched too many friends lose their battle with pulmonary hypertension while waiting for a transplant. In the United States, an average of 22 people die each day waiting for a transplant.

I’ve heard a lot of patients who are considering transplant worrying about the statistics. Centers are required to provide patient survival data, so for me, looking at spreadsheets that only list outcomes as far as 10 years post-transplant made it a pretty unappealing option, especially at 23 years old. But PH patients know that life expectancy numbers are meaningless. Doctors predicted that I wouldn’t live to be nine years old. Even after I proved that wrong, they predicted that I would not live to graduate high school. My parents set up a college savings account for me anyway, and I graduated UC-Berkeley in May 2015. I plan on continuing to beat the odds, now with a new heart and lungs.

Even though this experience has been filled with a lot of pain, irritability, confusion, and anxiety, I’m really grateful to know what it’s like to live with a healthy heart and lungs. The first time that I became breathless with exercise, stopped, and then caught my breath, I cried happy tears of joy. With PH I would have to stop after walking a block or two, and then when I eventually continued on I would still be breathless and stop even more frequently. Now, every time I get off the train I take the stairs instead of the escalator, and it feels great.

What’s been really helpful for me has been reading memoirs of other patients who have gone through similar procedures. I read a book by the first heart-lung transplant recipient. Her description of her experience helped me be able to communicate mine and feel okay about the feelings that I was having about the confusion and the guilt. In multiple memoirs that I’ve read, patients will express that they feel like they were unworthy of the transplant, and that they don’t have the appropriate amount of gratitude.

When I’m sitting on my couch at home and I’m debating going out for a hike, I’ll usually motivate myself by thinking someone died so that I could go on this hike. I’m laughing about it now but it’s the truth. You live every day knowing that someone else gave their heart and lungs so that I could be here today. You still are in a lot of pain, and it’s still really difficult. You are living with chronic immunosuppression. I have multiple appointments a week still. Being able to read that other patients go through the same set of emotions where you’re following like you need to be the poster child of organ transplantation and encourage people to register to be donors … because of course you want that, but at the same time I feel like I need to speak for others and show the bad with the good.

When I talk to people on the street and they hear that I had a heart-lung transplant, they’ll say, “Congratulations.” One woman called me a walking miracle over and over again. It’s a lot of pressure. Some days I just want to be 23 and be upset about my appearance or whatever I’m worried about at that time.

Reading these accounts by other patients who have gone before me has been really helpful, so I started writing partly as a way to process it and give a timeline to what’s happened. Writing it down makes it make more sense in my head. At the same time, it’s been really rewarding to be able to describe what I’m going through and have other patients tell me that they felt the same way, or thank me for sharing, writing about this experience, because often they don’t feel like they can talk about it.

It’s been really helpful for me to get positive feedback from parents or other patients who have gone through transplantation, and to hear that my story resonates with them. It has helped me build community and connect with people who are across the country. Probably the most meaningful to me has been the mother of another PH patient who had a transplant. She thanked me for sharing how I felt.

I count things by week now. I have on my calendar 18 weeks post-transplant. I also have 200 days and 100 days, and I’m constantly keeping track of how far post-transplant I am. It really was striking, the difference between, say, 20 days post-transplant and then 40 days post-transplant, or one month post-transplant and two months post-transplant. Now I’m four months post-transplant, and the changes have been just mind-boggling: the fact that I can run today, and four months ago I couldn’t walk around a block.

Time works really differently now. To know that 23 days post-transplant I was finally released from the hospital and I weighed 30 pounds heavier than I should have in water weight. I felt like I would never be feeling this great. To look back and see how good I’m feeling today and all that I’m able to do, and then think back to it was really just three months ago when I was feeling miserable and confused.

I know that when I was in the hospital, I tried to remember how bad I felt then. Because I knew I would feel better later, but I wanted to … people kept saying, “Think big picture,” and that this was the worst of it. But when I was there in the worst of it I really wanted to remember just how terrible it was. Because it seemed like I had to fight through every single minute and every hour. It just took so long to recover. Now it’s literally only been four months, and feeling like this, it was totally worth it just to go through that. Looking back, it was just one bad month, really. It felt like a year, but I’m glad that I kept fighting through it. There was a lot of times when I just had to think of something else or put on my headphones and listen to really loud music. I would listen to Twenty One Pilots because it was the angriest band that I could think of.

Being able to exercise has always been one of the things that I wanted most in life. I come from a family that loves to exercise, and I would always be standing there waiting while they went up and down stairs. My sister and my mom would run up and down stairs, and just love the workout. Now I’m able to, and stairs actually suck. It’s still hard to exercise, but I’m really grateful to be able to do this. I don’t know. It’s just pretty cool.

Yesterday I went to Sutro Baths in San Francisco. You have to go down tons and tons of stairs and then walk back up them. I was carrying a backpack of camera equipment and a light stand. I had done it months ago before my transplant, and I had to stop along the way over and over again. It was one of the hardest things I’d ever done, so I swore I’d never go back to that spot. But it’s a beautiful spot, so every wedding photographer ends up taking their bride there usually. We went there yesterday and I just did all of the stairs, walked up all of them without stopping. When I got to the top I was just thrilled. I had to tell someone, like, “Four months ago I couldn’t walk around a block and I just did all those stairs.” That’s pretty cool.

It’s been interesting, my sister is two years younger than I am, and when I was diagnosed at age 6, she was 4. Mostly, my experience with PH was my parents and I would go to appointments. I was gradually learning about the disease and how to care for my own medications. For the most part, she wasn’t a huge part of it. She was younger, so most of what she knew was that my parents and I would have to leave and go to hospitals, and she would stay at home with a friend. She knew that I couldn’t keep up with her if she went on a run. It’s been interesting now because she’s having a hard time adjusting, actually, to us being an exercising family and me being able to keep up with her. Because for so long she had to slow down and make sure that she wasn’t going too fast for me. Now we can exercise together, which is fun, but a bit of an adjustment for her.

It was really nice having the transplant at 23 with her being 21. She was there throughout the procedure and my hospitalization afterward, and was really an important part of my care team. To be able to have her, in addition to my parents and friends, was really nice. You spend a lot of time with your caregivers post-transplant. You get pretty close to them. Being able to have my sister, who’s one of my best friends and around my age, to be there, it’s nice to have someone younger to talk to, and nice for my parents as well to have her as sort of relief.

It’s a lot of work for one person to do, so I’ve been lucky to have friends. Friends have spent the night with me when my parents wanted to take one night off. They won’t let you get a transplant unless you prove that you have a support system. It’s really a lot of work for anyone.

I don’t know if I conveyed it enough. A lot of people have said, “Oh, I should wait until I go into, like, a crisis before being listed for a transplant.” Should they wait till they have something like I had? I had hemoptysis. I didn’t really think that I was going to survive that, so for me, it was good that I had doctors that I trusted. When they said it was time for my transplant, then I just believed them and did it. It is a really scary thing. When I was in the darkest part of it, I was convinced that no one should go through this and no one should try to have a transplant, but my recovery was, in retrospect, really fast. I feel great today and I’m just really grateful that I did take the plunge and had doctors who advocated for me to be listed at priority, and to recognize that window.

I’m Kathleen Sheffer, and I’m aware that I’m rare!

Listen to “I’m Aware That I’m Rare: the phaware™ podcast” at phaware.global/podcast. Learn more about pulmonary hypertension at phaware.global. #phaware