The Summit Foundation for Cystic Fibrosis

What Hand are you Playing?


What hand are you playing?
-Nicki Perkins, 44-year-old Cystic Fibrosis warrior and CrossFitter

I was contemplating life as I usually do when another year ends and I am lucky enough to still be alive. I was also reminded about that quote about making do with the hand you are dealt. The hand I was dealt requires me to spend more than half my life fighting a disease that is vociferously trying to kill me. This time I was thinking about how much time I spend doing, taking, inhaling, injecting medications that keep me alive along with the sanitizing, ordering, stocking, and organizing said meds. I figured that in one month I spend 120 hours on the pipe (machine called a nebulizer that allows me to inhale mediation) and about 3 hours cleaning and sanitizing the hand-held pieces that I breathe the medication through. This is very important to keep highly sanitized as it is the tool I use to inhale directly to my lungs.

Side note: a lung transplant is the most difficult organ to transplant and the most difficult to keep from being damaged post transplant. Why? Because the lungs are the only organ that is exposed to the outside environment and most significantly deadly bugs that can turn new lungs into dead lungs.

On any given day, I swallow over 30 capsules of various medications, supplements, and antibiotics. Every time I eat I must take enzymes with it. Why? Because due to my CF complications my pancreas is blocked with an abnormal amount of mucous and thus is unable to send digestive enzymes to my stomach to digest the food.

Side note: Lung function and nutritional status are directly correlated. This means if I am not at a proper BMI my lungs will struggle to keep the infection in them under control. I need 4000 calories a day to stay ahead of the game. In 1998 I had a g-tube placed directly into my stomach to help get me those 4000 calories. When you are chronically ill an appetite is elusive. Before I got the feeding tube I was down to 74lbs and circling the drain. The beauty of this g-tube is that while I sleep I deliver over 2000 calories of density rich nutrition, with the use of an IV pump like machine. I wake up with some energy and can get the other 2000 calories eating normally throughout the day.

As an older person with CF I have what’s called CF related diabetes. Its unlike both type 1 and type 2 but does require monitoring and insulin injections. I average 3-5 injections per day. The difference between CFRD and normal diabetes she that exercise and diet are not limited. In fact, caloric intake does not change. We just adjust our insulin to help. So yes, I still get to indulge in that can of coke once in awhile.

Again, as an older person with CF I have bone density issues. Instead of having to supplement with harsh and often side effect laden meds I choose to weight train to strengthen my bones. 1 less med!

Lastly, I must exercise/do physiotherapy everyday of my life. Because the underlying issue with CF is the abnormal amount of mucous in the body I have to clear my airways. The exercise is meant to bring up the mucous trapped in my lungs to prevent it from staying their and blocking the smaller airways permanently. I do anywhere from 1-3 hours daily. CrossFit being apart of my therapy. Now because my lung function teeters between awful and bad at 30% I need oxygen while I sleep and when I workout. This has been the case since 2014 and I can tell you that the first night I put those nasal cannulas under my nose I was a mess. I thought it was the start of the end. I was sure it was going to make me 100% dependent on it and all the independence I have worked so hard for, all my life, would be gone in an instance. Thankfully I proved the docs wrong and still only require it when doing arduous activity and sleeping.

Side note: The chronic infections that are trapped in my lungs permanently are there because the mucous creates a biofilm. I must inhale antibiotics to keep the infection from turning into a full blown exacerbation (aka need for intravenous). Its impossible to get all the mucous out on a daily basis and this biofilm is impossible to break up. It is like those sticky fly strips you see hanging in porches to catch flies.

As I age with CF I wade into uncharted territory. We haven’t seen patients live this long and with it comes new complications. Kidney and liver disease due to the medications, loss of hearing from the meds, brittle bones, and the all-important resistance to any of the limited antibiotics we use. There comes a point in our lives where the meds stop working and we start our journey to lung transplantation or palliative care.

Side note: I lost my sense of smell after my 7th polypectomy in 1992. The polypectomies were surgeries to remove polyps blocking my nasal passages again due to CF. Can you imagine NEVER getting to smell a home cooked turkey dinner ever again? I can. Although I am your gal when your dog gets skunked!

So, in one month I spend 120 hours inhaling meds, 12 hours cleaning and sanitizing the devices needed to inhale these meds. I do 120 hours of exercise/physio. I ingest over 360-400 pills for various reasons. I inject 120 needles into my body with over 540 units of insulin. I pump in over 60,000 calories of food through my g-tube while I sleep. I use oxygen for about 350 hours. When all this is done, I add the rest of my life into it. I crossfit, run my CF charitable foundation, love on my dog, spend time with family and friends and then if I have time left over I indulge in some guilty pleasures such as binge watching Vanderpump Rules.

Other things that I celebrate with ageing is grey hairs, the need for reading glasses, occasional bout of arthritis, slow to recover from a hangover, and the inability to understand the younger generations lingo! When one complains about such luxuries I just chuckle and say “its better than the alternative”. You are only as old as you feel. I have the body of a 90-year-old lifetime smoker but the heart and soul of a teenager and that I am grateful for. Despite the hand I was dealt, I choose to live as if I have a royal flush! What hand are you playing?

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