It’s no secret that I have CF. My least favorite question is “when did you catch CF?” – never. I never caught CF. CF, ahem, Cystic Fibrosis is a genetic disorder where you inherit the disease courtesy of two mutated strains of DNA.
I wasn’t diagnosed, however, until 4 years old. So what is CF? Well, mainly it’s an annoyance and a source of anxiety with a side helping of frustration. Medically speaking, it’s a genetic disorder in which the chloride channels in my body aren’t functioning correctly. Our body relies on specific balance of salt to help keep things fluid and moving. Think of how you spray a saline solution up your nose when it’s stuffy, and then magic happens and you can blow your nose and get relief again. It’s kind of like that. Our bodies rely on this salt balance to keep mucus moving throughout our bodies – it helps keep our noses, lungs, digestive tracts, and even our cervix’s clear. When these salt channels fail to work properly the body fails to move out the healthy mucus and it becomes stuck; in our noses, sinuses, lungs, pancreas, GI tract, and yes, even in our cervix (again that’s just mostly for us ladies, the fellas have different problems downstairs mainly in terms of a disconnected vas deferens). Doesn’t sound too bad right?
If you ever have had a sinus infection, think of it as such, but for your whole body. All this stagnant, sticky mucus is just hanging out clogging up important things we need to function… mainly our lungs and airways. Sure we don’t secrete enzyme to digest food but luckily we have quite a few good replacements for those, replacement airways? notsomuch.
Due to all this sticky lung mucus gunk, we tend to cough, a lot! It’s the body’s way to try to clear out that junk. Since we lack the proper salt balance it’s very hard to move so we cough forcefully. Since it tends to hang out for quite a long time it attracts certain strains of infections. Normally CFers culture a least one “bug” in their sputum.<side note, grosses word ever?>
Psuedomonas is a very common bug, and now MRSA is becoming a very common bug. There are some not so common ones, and some are more antibiotic resistant than others.
We take a ton of daily meds to try to alleviate symptoms and avoid really bad lung infections. We do phsyiotherapy where we try to breathe, shake, or pound the mucus out of our lungs. We take enzymes since we are unable to produce our own. This makes gaining weight very hard as we are unable to digest fat naturally. Us CFers usually tend to be very thin.And no, we’re not “so lucky!”
Our lung function is tested quarterly, unless sick (or pregnant in my case!) and then it’s tested more frequently. We take oral antibiotics to fight off infections and when they just aren’t cutting it, we head into the hospital for what I like to call a “tune up.” We get a rigorous round of IV medication, physiotherapy and loads of hospital food (are you jealous yet?) for anywhere from 7-21 days. Some clinics will let you go home after a certain number of days to finish off your IVS at home and some won’t.
We struggle with getting winded doing daily things, climbing stairs, carrying groceries, being on top during sex (okay okay so this may not be a daily thing… sorry Dad!). All kidding aside, sometimes when we’re sick, we’re pretty much useless.
This leads me to another important point about CF. Everyone is different. This disease is so fascinating because two people can have the EXACT same mutations, but manifest two completely different diseases. Even if they live in the same house, with the same parents. While I may get winded when I’m not feeling the best, doesn’t mean someone else doesn’t. I can walk up a flight or two of stairs no problem these days, but for others even just walking is tiresome.
As the disease progresses, and more mucus blocks off more airways, the airways eventually die and you’ve permanently lost lung function. Yikes! It takes a lot of hard, dedicated work to try to get your lung function numbers up and sometimes it just isn’t possible. As the disease progresses and more and more lung function is lost, an oxygen supplement can be used. An oxygen tank and cannula is a tres cute accessory for any outfit. But seriously, those things stink. I’ve personally have never had to have oxygen but watching my sister lug hers around for two years sucked. Those things are heavy!
And finally as you lose most of the ability to breathe on your own you can opt for a lung transplant. This is another very important part. This part doesn’t solely rely on yourself. You rely on a selfless family to donate the gift of life in a heartbreaking moment of grief, sadness, anger and confusion. I know what you’re saying, “Well, I’m an organ donor, my card says so!” That’s great and all, but did you know they still need your family’s permission?? Please let your family know your wishes, be firm with them so they understand what’s at stake here.
Lastly, CF is emotionally trying. There are days where I wish I could say it’s easy because I’ve had it all my life, but I constantly go through a grieving cycle where my life isn’t what I envisioned due to CF. It can feel limiting, isolating, unfair and scary. But hey, at least it gets me out of being on top.