I felt compelled to share my CF story because even those who know me, and know I have CF, often don’t understand what that means. So, I’ll offer it out to the world so that it can hopefully raise some awareness about this disease.
This is NOT meant to make anyone feel sorry for me – please don’t. I love my life and enjoy every second of it I am blessed enough to have!
First, what is CF or Cystic Fibrosis??
“Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
- clogs the lungs and leads to life-threatening lung infections; and
- obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.”
Text taken from http://www.cff.org
In other words, CFers have to take medications in order to be able to digest foods, and we have to do daily nebulized medications and airway clearance to keep our lungs as clear as possible and free from infection. There is no cure for this disease, and repeated lung infections typically lead to decreased lung function, which in turn usually leads to a double lung transplant (hopefully).
Next, what does it mean for me? Here is my story and a typical day’s schedule:
At birth, I had a few complications which led the doctors to test and ultimately diagnose me with cystic fibrosis. After 2 intestinal surgeries, a feeding tube, and 6 weeks of being in the NICU, I was finally home with my parents. I am so thankful for this immediate diagnosis because it meant I was on medications and being watched from day one.
In order to have cystic fibrosis, each parent needs to be a carrier for the disease. You can be a carrier without even realizing it, and if your spouse is also a carrier, then you have a 25% chance of having a child with CF. There are hundreds of gene mutations which a CFer can inherit.
When I was first diagnosed, we were only aware of one mutation Delta508. The other, at that point, was unknown and therefore I was re-genotyped last year. We found out that my second mutation is also Delta508. Why it didn’t pick it up the first time, we don’t know, but ultimately what that means is that I have the most common gene mutation out there.
I was hospitalized once at age 5 with a sinus infection but nothing lung related. Other than this minor hospitalization, I was a pretty normal kid for years. I ran outside and played endlessly, loved school, loved reading, loved learning (Ok, so maybe I was never normal).
I did my nebulized treatments with few complaints until I reached the age of 14. Suddenly, high school hit and I didn’t want to be “abnormal.” So, the treatments were done sporadically – only when I felt I needed it, or more realistically, when my parents made me do them. I firmly believe my lack of compliance with my treatments is why, at 17, I found myself hospitalized with pneumonia, bronchitis, MRSA, and pseudomonas aeruginosa (The latter two being very bad bugs for CF lungs). I ended up in the hospital for weeks and came home on a PICC (peripherally inserted central catheter…basically an IV line that goes into the upper arm and allows for continuous antibiotics) line for 2 more weeks.
You would think this would have been a huge wake-up call for me, right? Well, in some ways it was and I did start doing my treatments more often, but due to my desired invincibility, I still was not 100% compliant with them. At this time in my life, being 100% compliant meant doing my vest (a machine that helps loosen the mucous out of CF lungs by vibrating them) two times a day, nebulizing albuterol sulfate two times a day, and nebulizing pulmozyme one time a day. Total treatment time was an hour a day. I felt, at this young age, that was simply too much to do. And when my post-PICC line PFTs (pulmonary function tests) were back up over 100%, I saw no reason to be completely compliant.
By now, it should be clear that I’ve been blessed and God has been taking care of me day in and day out because I certainly wasn’t taking care of myself. This struggle continued into college where I still just wanted to feel normal. My sophomore year of college, however, my relationship began with my now-husband, and suddenly everything changed. We had been friends from freshman year on, but as soon as we started dating, I felt as if there was a purpose to my life – a reason to live, if you will.
Instantly, I became more compliant. I wasn’t 100% compliant from the first date, but it wasn’t long after that. Now, I only miss a treatment if there is no choice (for example, if we are on the road for 12 hours at a time). In fact, now, I keep trying to add more treatments or additional exercise to keep myself as healthy as possible. It’s a total shift from the way I was just six or seven years ago! Unfortunately, my treatments aren’t just an hour-long anymore, but it’s worth it in the end.
Since meeting my husband, I have had two additional surgeries and one more PICC line. The first surgery was the removal of my gall bladder in early 2006, and then in August of 2008, I had sinus surgery to clear out my 100% blocked sinus cavities. The mucous in my sinuses cultured MRSA and pseudomonas aeruginosa which led to a 2 week PICC line.
I also was diagnosed with CFRD (cystic fibrosis related diabetes) around 2006. I recently have started on insulin to try to put weight on me (a very hard thing to do). My current FEV1 (the indicator number of how well us CFers are doing) is 81-84% It’s far from the 100% I used to have when I was younger, but it is still really good. The only way to keep it up this high is to continue with all my treatments and exercise. With that being said, here is my typical day’s schedule:
6:30 AM: Check sugars and eat high-calorie, high-fat breakfast (Can’t just pour a bowl of cereal anymore…)
7:00 AM: Albuterol Sulfate nebulized, Hypertonic Saline nebulized, vest for 20 minutes
8:30 AM: Check sugars again and eat morning snack (which actually looks more like a lunch…)
9:00 AM: Do nasal irrigation rinse (These help tremendously with sinus pressure)
11:00 AM: Check sugars again and eat lunch
12:00 PM: Second treatment. This time, vest by itself for 20 minutes
2:00 PM: Albuterol inhaler or albuterol nebulizer (depending on how I’m feeling)
2:05 PM: Checking sugars and eat if sugars are low
2:30 PM: Exercise for 30 minutes
5:30 PM: Check sugars, take insulin, eat large dinner (usually 1000-1200 calories)
7:00 PM: Second nasal irrigation rinse
8:00 PM: Third treatment. This includes albuterol sulfate nebulized, pulmozyme nebulized, and vest for 30 minutes.
I’m not including the 4-5 pills I have to take prior to each meal and the other antibiotics/vitamins I take throughout the day because they hardly interrupt my schedule.
In addition, every other month, I am on Cayston which is a nebulized antibiotic treatment three times daily which increases treatment time only by a few minutes (it’s an amazing nebulizer system).
It’s a lot to get in to a single day and if I get a cold or any illness, I add in a few more vest treatments. While it sounds like I do a lot of treatments, many other CFers have an even tighter schedule than I do. I am truly blessed!
I know this has been extremely long but hopefully it will give someone out there additional insight as to what cystic fibrosis is and how it affects my life. Feel free to ask any questions you may have. I’ll be glad to answer them!