Hands up who thinks CF affects only the lungs? Who thinks it only affects the lungs and digestive system? Who has no idea?
CF affects a lot! When I’m describing what CF is with my patients at work I usually only mention the effects in the lungs and digestive system. These are the two most reliably affected organs and it’s simpler to focus on the big two issues. Other effects are not as widely known or have a tendency to be glossed over… even by me! Time and time again this blog will deal with the lungs and the digestive system. So, let’s give special attention to some of the forgotten body sites.
Since they belong to the upper respiratory tract the sinuses can be affected. I’m glad to report that my sinuses are perfect! Dave’s have been pretty troublesome though. He’s had 11 surgeries to clean out polyps from his nostrils. He is a frequent flyer with the ENT’s.
People with CF (PWCF) have a tendency to develop osteoporosis mega early. Vitamin D is essential for everyone’s bone health but because it is a fat soluble vitamin we generally have low vitamin D. This is because we absorb and store less fat and therefore absorb and have less vitamin D available. Coupled with prolonged steroid use our bones leech calcium big time. If bone density reaches a critical low point we can have infusions that help to protect and harden the bones. The infusions can be quite painful – kind of like Skele-Gro in Harry Potter.
Liver, gall bladder and bile ducts
This zone of the body is generally unhappy with its working conditions in PWCF. The bile is thicker which apparently makes producing it, transporting it and storing it a bit of an ordeal. My liver likes to complain about this which manifests in persistently and slightly abnormal liver function tests. I take a drug called ursodeoxycholic acid (“urso” for short) to help the bile achieve a normal-ish consistency and fend off gall stones and liver damage. For years now my gall bladder has been shrunken and useless. Whenever I have an abdominal ultrasound the sonographer spends a ridiculous amount of time simply looking for my gallbladder. It’s quite funny watching their stress levels rise as they search for my elusive gall bladder.
Congenital bilateral absence of the vas deferens (CBAVD)
WARNING: We’re about to consider sperm. So if this grosses you out then feel free to skip the next paragraph.
Ok, so here’s something unexpectedly weird. 99% of men with CF have a blockage in their vas deferens tubes which means their sperm have no way out of their testicles – the sperm simply meet a dead-end. Men with CF still produce sperm but it’s exactly like they’ve had a vasectomy. A natural vasectomy… for free… and without their consent. For the sake of the male ego I must stress that CBAVD does NOT equal impotence. Also, I have it on good authority that a number of young men with CF love the fact that they are unable to conceive children naturally! However, if men actually want to have kids it requires a large needle to their private parts and many thousands of dollars spent on IVF. The alternatives are a sperm donor, adoption, fostering or no kids – each with their own financial/emotional costs.
Women with CF can experience reduced fertility too (obviously for different reasons).
I’ve talked about CF related diabetes (CFRD) in a previous post entitled Sugar. Kidney issues can occur in some PWCF due to an increased susceptibility to kidney stone formation. CF associated arthritis is a potential threat too. PWCF often experience gastric reflux and can have low hormone levels. Check out the John Hopkins Cystic Fibrosis Center for more information about all of these issues.
Treating CF requires a multi pronged attack. It’s impossible to predict in any individual which organs of the body will rebel and to what extent. Science is still trying to figure out what contributes to different manifestations between people, even between twins or siblings with CF.
Meanwhile we are monitored for potential problems so they can be caught and treated early so we stay in tip-top condition!