I was diagnosed with cystic fibrosis-related liver cirrhosis in 2004 at the age of 9. Growing up, my disease wasn’t something to be pitied or seen as a weakness. It was a strength. I had the mentality of, “I can beat this.” I don’t remember much from that initial diagnosis, but this is what my 9-year-old brain could gather: I had to take two new medications, adding to my list of multiple other life-sustaining medications; I had to see a new doctor very frequently who weirdly resembled Elton John; I got to go to Bertucci’s Italian Restaurant in Boston after every appointment as a treat (I was and still am very food motivated); and I had to get a liver biopsy to confirm said diagnosis. I will never forget that experience.
Even though I was quite the veteran of hospital procedures and visits, nothing could have prepared me for a liver biopsy. They inserted a large needle into my abdomen to grab a piece of my liver to test it for disease. I was so glad I was put to sleep for that procedure, but boy did I hate it when I woke up. I was so confused, disoriented, angry, and in the most pain my little body could’ve ever comprehended. It felt as though I had been shot in the stomach and nothing helped it. The nurse had to examine my scared and hurt self, and in my pain-induced haze, I punched her. I had never hit anyone in my life. I don’t consider myself an aggressive person, so this was extremely surprising behavior. Luckily, she was compassionate and understood that it was the pain doing that, not me. I was 9 and probably 60 pounds soaking wet. I was small. Even though it was a totally unintentional violent reaction, to this day, I still feel awful about it.
When the results came back and confirmed cirrhosis of the liver due to my CF, I was promptly listed with a low Model for End-Stage Liver Disease (MELD) score. My mom worked very hard to get me healthy and back to baseline. After being on prescribed liver medications (and adding an herbal supplement my mother gave me), I became healthy enough to no longer need a liver transplant. If it wasn’t for my doctors and mother, I would probably have needed one sooner than I ever anticipated. I am so grateful for them.
Years flew by and I became better at taking care of myself with the help of my mother. She helped me develop a routine. She eventually started to transition me into being my own advocate and taking care of my medical needs without her help. She was preparing me for the future — adulthood. I became very good at care management, and it became a habit (not without some ups and downs). Thanks to support from my mom and my doctors, my liver thrived, and I was able to put off needing a transplant. That was, until I turned 23 and things got bad again.
After many months of severe stomach pain and not being able to tolerate drinking alcohol without my liver screaming, I got referred to a liver doctor in Boston. My life had halted in its tracks, and all my energy got put toward seeing this liver doctor once — sometimes even twice — a week. I was being evaluated for a liver transplant. My parents, siblings, and friends took turns taking me to all these appointments because I was so exhausted all the time. It was a very emotionally intense time for myself and those close to me. I was faced with the possibility of a major surgery and transplantation yet again. This time, I understood it all. We searched for a live donor for a year or so. A few candidates came close to being able to donate. They were ultimately not an option due to confidential medical and social reasons. But I will never forget their selflessness.
I organized a few fundraisers and gathered resources for all the costs associated with my liver transplant journey with the help of family, friends, and even strangers. We flew out of state to Jacksonville, Florida with help from an organization called Angel Flights. My mom and I stayed at The Gabriel House for almost a month while I was being evaluated at the Mayo Clinic. For four weeks, I was going into the Clinic for 10 hours every weekday for tests, doctors’ visits, counseling, procedures, scans, bloodwork, and group presentations discussing all the aspects of a transplant. The schedule was intense and physically and emotionally draining. I had hope at first, but by the end of it, my MELD score was too low for them to do anything. I wasn’t sick enough. Although I learned a lot about my body and liver disease — and I was given more medication that did help — I felt so frustrated and defeated. We went through all of this heartache and stress, not to mention the countless medical procedures and tests I had to endure, for what felt like nothing.
For a while, I remained on the transplant list in Boston for my liver and pancreas until I was ultimately removed entirely. The doctors’ visits became less frequent, my liver hurt less and less, I had fewer issues, and my MELD score never went up. I still see a doctor, Dr. Bonder, to keep up with my liver numbers and now GI issues, but only every six months instead of every week. Dr. Bonder is by far one of my favorite liver doctors I’ve ever had. He listened to me when no one else would, he understood me and the emotional toll this took on me. I didn’t feel like just a patient, I was a person with a disease he wanted to treat and possibly cure. He just wanted me to be OK. I credit him, among others, with making sure I got the care I needed, and he helped me get better.
After all the ups and downs of being listed and removed from the transplant list twice, the biggest downside was the news that if I wanted to get pregnant, it would be extremely harmful to myself and the unborn child. It would even be extremely risky to do IVF or surrogacy, as the hormones in those shots could undo all of the hard work I have put into stabilizing my liver. I was completely and utterly devastated and blindsided by this development. I was just seeing a high-risk OB to learn about my chances of pregnancy and how to safely go about my family journey with my husband. I was always told that even though I have CF, with Trikafta and my fairly stable baseline, I could safely conceive and carry a child, like countless others in the CF community. I always wanted to be a mother, and now yet another dream I had was slipping through my fingers due to my illness. As far as I was concerned, my disease had robbed me of the one thing I wanted most in life — to have a family.
After much discussion and grieving about what could have been, we decided on adoption. I now can’t think of a better way to start a family with the man I love. I am and I think I always will be, grieving what pregnancy would’ve looked like for my husband and me, however we made the right choice for us. I know this journey won’t be easy, but the end result will be well worth it. And it’s not like I haven’t survived difficult journeys before — in fact, I’ve thrived in them.
Interested in sharing your story? The CF Community Blog wants to hear from you.
