When I ask people what’s cool about me (I don’t just do that for fun, btw), this is the first thing they say: “Your lungs aren’t yours.”
Sigh. While I sort of wish something more awesomely heroic was what they said, it is indeed true that my Big Claim to Fame is twofold: Having lungs that aren’t mine, and being the first double lung transplant at my center.
So, here's the story:
I was diagnosed with cystic fibrosis (CF) when I was 11 years old, which is somewhat late for a CF diagnosis. CF is an genetic disease which affects the respiratory and digestive systems.
CF is an autosomal recessive trait; that means that both my parents had to be carriers of the recessive gene. And even then, they had a 25% chance of having a child with CF. So…I have CF, my brother and sister do not. They may be carriers. A lot of people are carriers and don’t know. Since I have a large extended family, and my diagnosis was late, my cousins all had sweat tests done. Today, CF testing at birth is mandated by most states.
I began testing for a double lung transplant in the Fall of 2003 at the Cleveland Clinic (during my senior year in college). At the time, my CF center at Nationwide Children’s Hospital was working to create their own transplant center. In the Fall of 2004, the center was created,and I was one of their first patients. I had another workup done in March of 2005 during a period of hospitalization. The center was accredited by UNOS (United Network for Organ Sharing, who handles organ transplants in the U.S.) that spring, and I was placed on the national waiting list in May of 2005.
June of 2005 was spent mostly in the hospital. I was sleeping most of the day, and the simplest things, like washing my hair and brushing my teeth, took tremendous effort. Upon discharge, I was on supplemental oxygen at night.
On Sunday, July 10, 2005, at 8:45 PM I received the call that lungs were available in Minnesota, from a 50 year old woman. Around 1 a.m. on July 11, my family and I drove to Children’s ER, where the registration process began. I was on the CF/Pulmonary floor (4AE) for the last time that night.
The surgery took 12 hours, and I was in the CICU for a few days, then down to the new transplant floor, 6T (now it’s the fourth floor of the new hospital building–the Floor of the Squirrel!). I was in the hospital for a month, undergoing med changes, rehab, and evaluation for the burn I received on my right arm during surgery. (For more on the burn, see below) Finally, I was discharged in early August.
Since then my PFTs are around 54%, compared to 19% (or thereabouts) before transplant. In 2006, I was interviewed as part of the Children’s Fundraising Drive that happens every June. I’ve also become a volunteer for Lifeline of Ohio, which promotes organ and tissue donation throughout central and southeastern Ohio.
I celebrated my 13th transplant anniversary on July 11, 2018.
For information on the Catholic perspective on organ donation, click here.
After being interviewed by Hallie Lord and Jennifer Fulwiler on Jennifer's Sirius XM Catholic Radio show. The interview talked about my 10th transplant anniversary and how it has impacted my faith.
Do your new lungs have CF?
No, they don’t. These are a complete clean set of lungs, without the CF genetic issues, so they are CF free, as it were. The rest of my body still has CF. I am “pancreatically sufficient”, meaning I don’t take enzymes to help digest my food, like almost all CF patients do (I did, for awhile after diagnosis, but a very low dose). I don’t have liver problems, and I do not have CF related diabetes (CFRD). I did have trouble with my blood sugars post-transplant but as my predinose level (a steroid) decreased, those settled down.
How often do you see your doctors?
I see my transplant team about every three months. This is much more regularly than almost all people post transplant do. It’s not because I have a lot of issues, but it’s my normal routine to see my doctors here about every 2-3 months, and so I just haven’t broken it. :) Several times we’ve discussed lengthening my visits, but as such, that hasn’t happened yet. I also only live twelve minutes from my center, which is a lot closer than most people. It was actually on the way to my office, when I worked downtown.
Wait a minute…your arm was burned during surgery?! How did that happen?!
An IV infiltrated–that means that the IV skewed out of my vein and began leaking the solution/med that was in the IV line into the surrounding tissues. While I did have a port-a-cath in place for the surgery, (and still have my port, under my collarbone on my left side) you need more than just one line when you're having such a huge surgery. My arm is fine now, if lacking a tiny bit in flexibilty around the wrist area, and it’s cosmetically not quite as “pretty” as my left arm. But I still have it, which was actually a concern immediately post-transplant. Fortunately I was too drugged up to worry about losing it!
Are you on a lot of meds?
Actually, fewer than pre-transplant! There are two immunosuppression drugs, and one steroid, a few prophylactic antibiotics (to keep bad things for taking hold in my body), and some vitamins and dietary supplements, like magnesium and vitamin D, to make up for what the drugs leech from my system. I find it a much easier regimen!
To compare: before transplant, I took a lot more drugs: a special vitamin, powerful antibiotics, dietary supplements, a med to help me sleep and also to reduce anxiety, as well as IV medications in 2-3 drug cocktails from time to time, and respiratory therapy twice a day, which included 2-3 inhaled medications and postural drainage therapy. (I started out having my parents do it manually, then we moved to various machines, with the Vest being my final iteration.) As I got older, the "therapy" combo ( inhaled meds and the Vest) took about an hour every time, twice a day. If I was in the hospital, then these treatments were done four times a day.
So, yes--taking some pills twice a day? Sign me up!
How do your doctors check up on your lungs, to make sure they’re OK?
Every time I go in to see my docs, there is blood work (to check drug levels and other things, like my CBC [complete blood count], cholesterol, triglycerides, vitamins, etc.) and a chest X-ray. Then I have Pulmonary Function Tests(PFTs) which show how well my lungs are working. I meet with the nurse coordinator and the doctor, and we talk about things that need discussed, or just go over test results. It depends on the day.
Once a year–usually in June or July–I have my yearly testing. This involves more complex blood work, PFTs, a more detailed clinical visit (the social worker and the dietician will come in), a CT scan of my chest, and a bone density scan (also called a DEXA), to check for osteopenia or osteoporosis. I have gained bone mass since my transplant, so go me!
If any of these tests show things that may be a concern regarding rejection of my lungs, we have a few choices: first we do a CT scan, to get a better look at the lungs (I can’t have MRIs because of the magnet in my head!*). Sometimes we do a bronchoscopy/biopsy, which is the best way of seeing what’s really happening down there, and to see if there is an infection, versus some sort of rejection. (There are two types of rejection, but that's a different story.)
*OK, the magnet thing–that’s from the cochlear implant.
As a result of the many ototoxic (meaning, they damage your hearing) drugs I was on, both pre and post transplant, I lost a lot of my hearing. This became evident right around the time I was transplanted, and I was fitted for hearing aids soon after the surgery. However, hearing aids just magnify sound. They don't help you understand and decipher sound. That's why, in 2008, I received a cochlear implant in my left ear.
The implant is two parts: internal and external. The internal part is implanted in my skull (yes, it’s very Borg), covered by skin and my hair. The cochlear array actually is in my ear and that’s what allows me to hear, since the hair cells that produce normal hearing are destroyed. The outside part sort of looks like a regular behind-the-ear hearing aid. The two parts are attached via the magnet.
The cochlear array takes the place of the dead hair cells that, in normal ears, are what transmit sound. The implant bypasses the ear's hearing mechanism itself and conducts sound directly to the brain. The surgery destroys whatever residual hearing you have left, so I am completely deaf in my left ear when the implant is removed. I have about 20% hearing in my right ear. I do not have bilateral implants, because if I did I'd be completely deaf when swimming, sleeping, or if my cochlear implant battery died (which has happened to me) unexpectedly.
So, since the magnet cannot be removed (in my skull, remember), I can't have MRIs or any sort of magnetic imaging tests.
For more detail about this, as well as a look at the implant itself, this is a post I wrote several years ago.