The CF.


Cystic fibrosis (CF) is a genetic disease which mainly affects the lungs and digestive system, but can affect other parts of the body too, for example, the liver and kidneys due to the amount of drugs we have to take daily, and weak bones sometimes resulting in arthritis. I have lower back pain which is possibly due to bone thinning, which can't have been helped by constantly being on prednisolone for years. Due to the toxic levels of the drugs, I've lost my hearing over the past 10 years. It's a side effect that does happen. Although rarely on this sort of level - most people experience a loss of hearing in certain pitches, rather than all around hearing loss and nerve damage resulting in severe hearing loss. And tinnitus, also caused by the drugs. This is more common, although the severity does vary. For me, it's 24/7, and really really loud ringing in my ears. Which doesn't really help the tiny amount of hearing I have left.

People with CF struggle to gain weight due to the poor digestive system - we usually have to eat twice as many calories than a healthy adult is required - most of these calories are used up fighting off infections and through general daily energy, so eating like a horse is a requirement if you want to gain weight - it slips off so easily and poor weight just fuels infections.

So because of this, a lot of people with CF are on the small side and usually underweight, or just managing to maintain an okay weight. Because of the deterioration of our lungs, lung function is measured at every clinic appointment (I attend my clinic every 4 - 6 weeks). They do lots of tests, including a lung function test which measures FEV1, which is the amount of air your lungs can blow out in one second, and FVC, which is how much air your lungs can hold altogether. The percentage they work out is that of which is typical for someone else your age/height/weight can hold.

Creon 10,000   - Probably up to 20 or 25 a day (5-6 for meals and various for snacks). To help me digest food.

Omeprazole   - 20mg twice a day. For acid reflux

Ursodeoxycholic acid   - 250mg twice a day. For my liver, I think?

Flucloxacillin   - four 500mg twice a day. General daily antibiotic

Azithromycin   - 250g mon, wed, fri. Another antibiotic

Ferrous Sulphate -  200mg tablets twice a day - To treat/prevent anaemia

Vit A+D   - once a day general vitamin as we don't tend to absorb everything from food

Vit E   - once a day ^^

Vit D3 -  For my bones to avoid any further bone damage

Cetirizine - antihistamine to be taken once a day when on IVs as I usually have mild itchy reactions from Ceftazadime

Sumatriptan - one tablet for migranes, usually as a result from colomycin IVs

Metoclopramide - antisickness, when needed

Paracetamol/ibuprofen/codeine/diclofenac - pain relief. When needed

Calogen Extra shots - supplements, 40mls with 160 calories and 16g fat. Probably highly responsible for finally getting my weight on track.

Symbicort inhaler  - 2 puffs twice a day

Spiriva inhaler - 18 micrograms, once a day

Salbutamol - 2 puffs when needed - 100 micrograms per dose

Tobipod inhaler - Twice a day, replaces tobi neb. Antibiotic

Colomycin nebs   - 1mu twice a day alternate months. Antibiotic (hopefully changing to the inhaler for this too)

Hypertonic saline   - twice a day or extra if needed. Makes you cough a lot and helps shift the gross chest gremlins

Dnase - once a day. Helps loosen the lung gremlins

Sodium Chloride 600mg slow release tablets - usually up to 8 a day depending on the heat - you sweat, you lose salt, so these replace it. Lack of salt makes you feel pretty shit.

Orkambi - Lumacaftor and Ivorcaftor (kalydeco) for DDF508, which is 2 pink tablets a day, with 20g fat at each dose. I don't think its done much for me, personally, but I take it because I do what I'm told.

Current personal best for downing meds with one sip of water: 19
(Refusing to try more b/c that'll result in lots of coughing, choking, and other unattractive things)

Clumsy adventures

You'd think most of us with CF would have already had our fair share of hospitals, and would generally avoid the place like the plague o...