Clare Dowling and Smokey,
one year after her transplant.
Just before her 19th birthday, Clare Dowling got a terrifying surprise. The college freshman and competitive equestrian learned she had an extremely rare, dangerous and poorly understood lung condition, called pulmonary veno-occlusive disease (PVOD). Characterized by high blood pressure in the lung area, PVOD has no treatment other than a lung transplant.
Dowling's PVOD progressed quickly. Three months after the diagnosis, she was living in the hospital, fighting to stay alive until suitable donor lungs became available. She received a double lung transplant in August of 2010, but recovery has been challenging. After her transplant, she spent nearly three months in the Intensive Care Unit, and many more recovering from nerve damage that initially put her in a wheelchair.
A year of rehabilitation work later, Dowling is now getting back to the life she wants – carrying a full class load at school and riding horses. Dowling talks about her out-of-the-blue diagnosis, transplant and recovery.
What were the first signs that something was wrong with your lungs?
In my first semester at college, I had to leave early because I got really bad pneumonia. The second semester I was fine, but I noticed I was out of breath walking anywhere, so out of breath that I had to sit down.
People told me, "You had really bad pneumonia — maybe you're just out of shape and need to recover your strength." But it didn't get better, and working out in the gym didn't help. I started having to take the elevator at school.
I had this intuition something was wrong. I felt that before, when I was diagnosed with lupus when I was about 12. I had joint pain and everyone said, "Oh, it's just growing pains," but I knew in my gut that something was wrong. I had that same feeling again. So during spring break, I went to a pulmonologist and had a lung biopsy. I was diagnosed and put on oxygen.
I didn't go back to school after spring break. The pulmonologist referred me to UCSF, and I was put on the lung transplant waiting list almost immediately.
How did the disease progress from there?
It was tough because I went from being a completely normal kid to being extremely ill. They didn't know how quickly the disease would progress, because they don’t know much about it in general.
I started to pass out a lot, so on July 4, I entered the hospital. I was on a life support machine called ECMO (extracorporeal membrane oxygenation) which took the blood out of my body and put oxygen into it, then another tube put the blood back into my leg. I couldn't sit upright because it would kink the tubes — I had to be flat on my back for 31 days.
Even though ECMO kept me alive, the residual effects of having to be in bed that long made for a long and difficult recovery. I know a girl who was also on ECMO before her transplant, but the tube went in through her heart instead of her leg. She was able to walk around and keep her body in shape a little bit. My heart was enlarged, so I had to have the tube in my leg.
I was on pain meds for those 31 days and don't really remember a lot of it, which my parents say is a good thing. Memories here and there are coming back. I was in the hospital July 4 until Nov. 22.
How did you stay sane, being stuck in the hospital and being so sick?
It was a scary time, but having the support of my family and loved ones was my rock. We tried to focus not on ifs, but when — when I get the transplant.
I thought of the transplant as a hurdle and once I was over it, I would get back to doing what I want to do.
Was it hard to learn to breathe on your own again?
After the transplant I was taken off ECMO, but I did need help breathing. I was on a ventilator at first, then they weaned me off of it.
I had a lot of trouble getting used to breathing on my own, due in large part to being stressed. I would often feel like I couldn’t breathe when I was actually good. It's hard to explain, but breathing on my own felt foreign.
After the transplant you initially couldn't walk because of nerve damage. What happened?
The nerve damage was not something the doctors expected and it's the part that's difficult for people to understand, because what do your legs have to do with your lungs?
I had three different cardiac events while I was in the hospital, both before and after the transplant. During one of these, my heart got to about 280 beats per minute, because all of the sudden I had these new lungs that worked perfectly and my heart was overwhelmed with all the oxygen it was now getting. The doctors had told my parents this could happen.
In total between those three events, oxygen was cut off to my brain and legs for 18 minutes. I had nerve damage from those 18 minutes, and also from being on my back for 31 days straight.
My surgeon wanted me to get up and walk the day after the transplant. But when they put me in a chair and I didn't even have the muscle to hold myself upright, they realized the damage was more than they realized.
I also had memory loss from the lack of oxygen. For about two weeks after a cardiac event, I woke up every morning not knowing where I was or why. It was like "Groundhog Day" every morning. My friends tell me that I would call them all the time and say, "Hello! Did you know I was in the hospital? I haven't talked to you in forever..." when I had talked to them the previous day.
Some of those memories are tough, because I have never been more scared, confused or in that much pain.
It must have been hard to accept yet another unforeseen challenge.
We had a big conference — all the doctors, my speech therapists, physical therapists, my parents and me — and one of the doctors said I was making progress but she didn't know if I'd walk again, or if I did, how well I'd be able to walk. She said I would probably need large plastic braces on my legs.
At that point I started to tear up and had to leave the room. I came back and said, "I appreciate your input but I just can't accept that. I'm going back to school, and I'm going back to riding horses."
Maybe that was denial, but now I'm walking with just one tiny brace on my left calf, and hopefully I'll be rid of it soon. I've been really lucky in getting the return that I've gotten. It's a combination of luck and really not wanting to be stuck in a wheelchair.
It's always difficult when the doctors don't know, but on the other hand, if the doctors don't know there's the possibility that the progress will be exponential!
How did you recover from the nerve damage?
Once I was out of the ICU I began working on rehabilitation from 9 a.m. until 3 in the afternoon. I did a lot of physical therapy, of course, but also cognitive therapy and occupational therapy. I was so weak — I'd lost a third of my body weight and weighed 88 pounds.
The people on the rehabilitation team weren't that much older than me, so I felt like I could have fun with them and talk to them about anything. They kept me sane for sure, and it gave me something to do instead of moping around the halls. They were amazing, to say the least.
I also had neuropathy in my feet from the nerve damage, which was the most painful part I remember from the whole experience. It was a constant pins-and-needles feeling in the most extreme way you can imagine. If you got anywhere near my feet it would make me scream.
The occupational therapist helped me desensitize my legs and feet and figure out how to calm those nerves down. They helped me do tasks that had become extremely difficult and exhausting, like getting dressed or in and out of the wheelchair. And, after almost three months, showering!
The cognitive therapy was my least favorite because it made me feel dumb. I was always an A student, and could cram 20 minutes before a test and do fine. But even though the exercises they gave me seemed so easy, I had lots of trouble with them.
When I went home I had an in-home nurse for awhile, and I worked with physical, occupational and speech therapists who came to my home.
Are you still doing rehabilitation?
I go to physical therapy every other week and to the gym at least four times a week. I'm also doing equine therapy at Hoofprints on the Heart in Livermore. Last week I got to canter for the first time since the transplant, which was exciting. It's been good to get back to what I love. It's good for my legs, but it's also good for the soul.
When I first visited UCSF, my surgeon asked what I like to do and I told him I love riding horses. He promised I'd be able to ride again after the transplant. All in all, even though it took a little longer than expected, my surgeon kept all his promises.
What's your advice for patients preparing for a transplant?
I know some of my story may not sound very comforting, but it's important to know that I can't compare my personal experience to any other transplant patient, because everyone's story is unique. You might have a tougher time before transplant and smooth sailing after, or vice versa.
I was hoping I would get a phone call, drive to UCSF for the transplant, be out in a week and continue on with my life. That did happen with another transplant patient I know. It's hard to say I had a good experience because of how urgent the situation was, and the aftermath of the nerve damage which made recovery very difficult. I really do love all the doctors and nurses at UCSF and feel thankful every day that they helped guide me through this experience.
The most comforting thing I was told when I was sick was a message of honesty and positivity. A friend/mentor/fellow transplant patient, Natalie Szabados, would basically say just this: "I've been there and what you're going through right now, pardon my language, really sucks (an understatement of course!). But you will get through this."
How did this experience change you?
What I've learned most is, it's not about things. If I were to die I wouldn't remember anything about what I have. It's the people in your life and the experiences you have. I live in Danville, and it's easy to get wrapped up in the car you drive and the clothes you wear.
It's going to sound dumb and cliché, but I'm excited about life. I'm excited to go to school, about what I'm going to do this weekend.
When I first came out of the transplant I said, "I can't walk, I can't do anything." And my doctor said, "You may not be able to walk right now, but you're alive and it's a huge deal that you even made it." You can't take the people in your life for granted, or your health for sure.
Before I was kind of passive — if something bothered me I didn't really speak up about it. Now I'm a lot more assertive. It was a crash course in life lessons.
My fear of blood and needles has also gotten a lot better!
Have you talked all your friends and family into signing up to be organ donors?
My father works for Safeway and we made a video about organ donation for the company. He tells me that people at work will come up to him and say they signed up to be organ donors, which I think is awesome.
People think the concept is kind of strange, but I ask them, "If you had a son or daughter wouldn't you do anything to keep them alive?" If I were in heaven I'd feel really good, knowing I prolonged someone's life and made a family so happy.
Photos by Eric Desch Photography.
Lung Transplant Program
400 Parnassus Ave., Sixth Floor
San Francisco, CA 94143
Phone: (415) 353-4145
Fax: (415) 353-4166