Amber was diagnosed with CF when she was seven months old and has been living with this disease all of her life. Get educated:
What is Cystic Fibrosis?
In people with CF, a defective gene causes a thick, buildup of mucus in the lungs, pancreas, and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.
Cystic Fibrosis Facts:
~More than 30,000 people are living with cystic fibrosis (more than 70,000 worldwide).
~Approximately 1,000 new cases of CF are diagnosed each year.
~More than 75 percent of people with CF are diagnosed by age 2.
~More than half of the CF population is age 18 or older. The median predicted survival age is about 40.
Learn more about Cystic Fibrosis and how you can help.
Consider making a donation to the Cystic Fibrosis Foundation. Your help will aid with research and development of new drugs to fight the disease, improve the quality of life of those with CF and the ultimate goal: finding a cure we desperately need. Together we can make CF stand for Cure Found!
Download Amber’s rose vine lungs digital coloring page (printer required) inspired by those living with CF and lung disease.
My Life Pre-Transplant
I lived a relatively normal early childhood with cystic fibrosis, taking dance lessons every year, playing soccer for one season, rode bikes, and played outside games along with my friends. I missed half a day of school only during my routine doctor’s appointments. I lived a life like all the other little girls, attending sleepovers, dressing my Barbie’s in the latest trends, and gently caring for my precious doll babies. I set up a hospital in my bedroom, to tend to my sick American Girl Dolls. I wanted to be a nurse, just like the ones that cared for me.
My health began to slowly decline, just as I was entering fifth grade, at ten years old. During that time, I went through a few surgical procedures to help keep me stabilized. My parents decided that it was best for me to get a Port-A-Cath for long-term IV antibiotics and a feeding tube (MIC-KEY button) for extra nutrition. Unfortunately, I also started showing signs of Cystic Fibrosis related diabetes. I depended on shots for insulin. Along the way, I developed progressive hearing loss from extended use of an ototoxic intravenous medication called Amikacin, which eventually lead to complete hearing loss and deafness over the course of one year. I was able to learn sign language and became quite skilled with lip reading. At the age of thirteen, my small, fragile lungs had become damaged beyond repair, from years of respiratory infections. No amount of medication was going to keep me alive. I was in ultimate respiratory failure. I was placed on the double lung transplant waiting list at Pittsburgh Children’s Hospital in March 2005. I struggled to breathe, despite wearing oxygen all day, every day. I couldn’t leave the house for long periods of time for many reasons; my oxygen tank only lasted a few hours, I had IV medication that needed to be kept refrigerated and I would become easily exhausted with very little movement. I couldn’t walk across a small room without gasping for air. I simply couldn’t enjoy a moment of laughter, without physically getting sick from laughing too much. My parents had to haul my heavy wheelchair everywhere. In most situations, the only thing I could do, was sit there and watch everyone else have fun. Even at home, my freedom only went as far as my oxygen tubing would let me go. I was not prepared to die at such a young age. There was too much I had not experienced yet. I had so much left to live for.
Receiving the Call for a Life-Saving Double Lung Transplant
It was early in the morning, when I finally received the call on March 13, 2007. The waiting was almost unbearable. It felt like the longest two years of my life! I awoke in the ICU and quickly realized, that because I was deaf (at the time), I couldn’t communicate with my family, while still connected to the breathing tube. Their little knowledge of sign language wasn’t enough. They couldn’t read my sloppy handwriting, due to the effects of the sedation medication. It was extremely frustrating for everyone involved. Recovery wasn’t easy. I was walking the hallways three times a day, with several chest tubes dangling from my body. I endured daily physical, and occupational therapy, and learned how to breathe again. I’m proud I was able to get through it, one day at a time. I received tons of greeting cards, gifts, visitor’s, and encouragement during my extended hospital stay. The therapy dogs lifted my spirits a few days a week. I had to learn all about transplant living and my new medications, which was a lot! I became too dependent on oxygen for three years, and because of that, in my mind, I still needed it to breathe. I thought for sure I would be gasping for air without it. I wore the tubing on my face for a few days, before I realized they had turned it off! It was my security for so long, I had to tell myself, I can breathe. I don’t need it anymore. There came a day, when I sat in that uncomfortable lounge chair and thought, God, you’ve given me more than I can handle, and I can’t go on. I give up. I looked across the room at my parents with watery eyes, swallowing the lump that formed in my throat. They had been cleaning up the small area, which seemed like I had been living in for more than a few weeks. A few hours later, another batch of cards were delivered to my room. I opened one from my aunt, Tammy. The front of card read, Many impossible things have been accomplished by those who refuse to quit. I slowly opened the card, almost too afraid to read the inside greeting. To my surprise, the words I silently voiced to myself only a few hours before, were right in front of me, in bold letters. I fiercely held back my strong emotions, as I read the words, Don’t Give Up. Was this a coincidence? I don’t think so. It’s true when they say, people don’t realize how close to success they were when they gave up. I have God to thank for that. (1 Corinthians 10:13)
I wasn’t out of the woods just yet. I suffered mild kidney damage, caused before transplant, another strong side effect of Amikacin. The anti-rejection medication I was taking for my lungs, ultimately put me in renal failure. My mother donated her own kidney to save my life only 4 months later, in July. There was also one more, minor bump in the road, during the first year of my transplants. My doctor noticed a spot on a routine chest x-ray, which was diagnosed as “Post Transplant Lymphoma Disorder” (also known as, PTLD) He lowered the dose briefly on my anti-rejection medicine to resolve the issue. Thankfully, the Lymphoma disappeared on its own and I didn’t have to go through any more major procedures, such as, chemotherapy. I haven’t had a lung biopsy in 8 years. I no longer wear oxygen or spend hours doing breathing treatments and most importantly, I can breathe easily. I’m finally free!
Thank you for reading my story! Read more.