So in the last post I mentioned I have Cystic Fibrosis, also known as CF, and other associated health problems. I'm not planning for this blog to be hugely about my health but a) it's a large part of my life, I can't ignore it and b) my health has affected me and my mentality on life and also causes me to make decisions and form opinions I don't believe I would if I didn't have CF.
Firstly lets start with what is CF? CF is often referred to as a lung disease, this isn't strictly true, CF effects every cell in the body. Mutations in the genetic material prevents the movement of salt and water in and out of the cells within the body. Now I don't know about you but I hear mutations and I'm thinking x-men and superpowers but until it becomes possible to save the world by coughing up wallpaper paste and having the ability to shit oil (enzymes are important) then these are not cool mutations to have! Basically this lack of movement with the salt and water makes bodily secretions thick and sticky, this causes the most problems in the lungs and digestive system. Bacteria grow in the sticky gunk in the lungs causing bad chest infections, lung scarring, eventually leading to the need for a lung transplant. Most people with CF have, due to secretions blocking the pancreas, insufficient pancreatic function and require enzymes to digest their food, missing enzymes with fatty foods tends to be, erm... explosive (see above reference to shitting oil). Some people also have diabetes due to these secretions not allowing insulin to leave the pancreas properly/at all. I'm one of those lucky people! Another problem people can have with CF is cirrhosis of the liver, unlike alcoholic cirrhosis 'CF cirrhosis' is nothing to do with vodka, cider or tennets super strength but caused by thick secretions (yep, those again) scarring the liver. I got cirrhosis fairly young and eventually had a liver transplant aged 14. Other problems/illness's associated with CF are constipation or bowel blockages/DIOS, osteopenia/osteoporosis, gastro-oesophageal reflux disease, arthritis, tachycardia, infertility, vitamin deficiency, etc... So, yeah, as you can see, there isn't much in the body that CF can't royally fuck up!
"How do you get CF?" I hear you say. Well it's genetic illness, you can't catch it, you're born with it. Our genes come in pairs, one from your mother and one from your father, then when you reproduce you pass on one of your pair as does the other person you reproduce with. A person with CF will have a CF mutation (one of over 1500) on both of their genetic pair and a carrier has the 'CF gene' by having a CF mutation on one of their genetic pair, approximately 1 in every 22 people have this CF gene, passed on to them from one of their parents. So if two people who carry the CF gene decide to have a child (or have an unprotected night of passion) there will be a 25% change neither of them will pass on their mutated half of the pair, a 50% chance that one mutation will be passed on to make another carrier or another 25% that both parents pass on their mutation, resulting in CF.
Lastly, in this rather long post, there is no cure for CF. For most people with CF medication only deals with treating symptoms, although a new drug, Kalydeco, has been developed which for people with the genetic mutation G551D, which accounts for approx. 4-5% of the CF population, that treats the cause of CF by allowing salt and water to move round cells as normal. New drugs are in development for other mutations which, whilst not a cure, will lead to much better management of the illness.
Well I think I've waffled on for long enough here. To those who already know all about CF I am sorry for wasting your time with this post but to those who were clueless about it I hope I've given you a better understanding of it all.
