I made a Fanhouse page so that I could have a safe corner of the internet to share intimate details of my life I wouldn’t typically share on other platforms like Instagram, Facebook, and Twitter; safely hidden behind a $5 paywall.
It donned on me the other day after getting sick with what I thought originally was a Cystic Fibrosis exacerbation that while I share photos of my pets, what I had for lunch, dating drama, and everything else I share on there, I have been leaving my followers out of possibly the biggest thing about my life: That I have Cystic Fibrosis.
I realized that if I have a hospital admission, which is something that typically happens every six months or so, that everyone who follows me on there might be confused and worried—so this is to kind of pre-emptively let my followers know that when that inevitably happens, it’s not that deep.
Cystic Fibrosis is a progressive genetic disease so as you get older, it gets worse. It mostly affects the lungs and over time, limits the ability to breathe. It is the most common genetic disease affecting more than 30,000 children and adults in the United States. (70,000 worldwide.) You inherit one CF gene from your mother and one CF gene from your father. Approximately 1 in 35 Americans are a carrier of the CFTR gene mutation, which means that 10 million Americans are Cystic Fibrosis carriers. If two people are carriers, there is a 25% chance that your child will have Cystic Fibrosis. In 1982 hospitals started implementing tests for CF as part of the newborn screening process but as new genes were discovered throughout the years, a lot of babies slipped (and continue to slip) under the radar. Because of this, I was not diagnosed until I was two years old.
I was very frail as a child and was diagnosed because I was going through something called failure to thrive. At diagnosis, it was discovered that I needed a feeding tube to help intake more calories than I was able to take by mouth. I had a g-tube from age 2-15. When I was 15, I was able to demonstrate that I had my calorie intake by mouth under control and was able to maintain a healthy enough weight for my CF team to feel comfortable with me removing the feeding tube. I actually took it out myself and it was supposed to close up on it’s own but did not so for an entire month every time I drank anything, it came out of my stomach. (Which made me very popular with the guys—just kidding!)
People with Cystic Fibrosis are born with a protein in their cells that doesn’t work right. That protein is called CFTR. CFTR controls the flow of water and certain salts in and out of the body’s cells. As the movement of salt and water in and out of the cells changes, mucus becomes thickened.
In the digestive system, CF mainly affects the pancreas. The thickened secretions can block the ducts in the pancreas. This blockage causes a drop in the amount of digestive enzymes the pancreas puts out, which makes it difficult to digest food. Because of this, CF patients have to take digestive enzymes before consuming any calories.
In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, avoiding germs is is a top concern for people with CF. An increase in infections can cause the lungs to deteriorate over time which results in a loss in lung function. My lungs currently function at about 60% which means that eventually I will have to receive a lung transplant. Most people do not realize that transplanted lungs only last for about 5 years but can typically last for a shorter amount of time or longer. The longest documented single-lung transplant patient survived 30 years post-transplant.
When I was diagnosed, I was not expected to live past 8. Today, because of improved medical treatments and care, more than half of people with CF are age 18 or older. Many people with CF can expect to live healthy, fulfilling lives into their 30s, 40s, and beyond. This is thanks to medications like Kalydeco, Orkambi, Trikafta, and Symdeko—all of which I am ineligible for due to my genes. I am in the 10% of CF patients that CF modulator therapies will not help, but pharmaceutical companies like Vertex are constantly working on new drugs and it’s just a matter of time before something I can take is on the horizon.
Hospital admissions usually come in the form of a two week stay where a tune up is done. During a hospital admission, a PICC line is placed in one of my arms. A PICC line is a long, thin tube that’s inserted through a vein in your arm and passed through to the larger veins near your heart. It is generally used to give medications or liquid nutrition. A PICC line can help avoid the pain of frequent needle sticks and reduce the risk of smaller veins in your arm being damaged. One of the risks of a PICC line is blood clots and infections. PICC line infections aren’t fun and can cause sepsis if not caught early enough. Sepsis is life-threatening.
I am actually allergic to one of the I.V medications I have to receive through my PICC line (Vancomycin) to treat my lung infections so before each dose I have to take I.V Benadryl through my PICC line which basically makes me feel like an extra on the Walking Dead.
Being hospitalized in general is pretty risky for a CF patient because hospitals are filled with germs, so most CF hospitals will allow a patient to do I.Vs at home through a home health agency. I typically do 7 days in and then continue the IVs at home, along with other parts of my CF regime like oral medications and breathing treatments alongside chest physiotherapy using a VEST—mine is pretty and pink, of course.
I’m due soon for a tune up so stay tuned for that and as always, thank you for reading. If you’d like to follow my journey with CF and receive health updates as well as updates on all the happier parts of my life, go here.
If you’d like to donate to the Cystic Fibrosis foundation you can do so here. :)