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What, Exactly, Is Cystic Fibrosis?

How does cystic fibrosis do what it does?

I’ll admit it, we either have to stop and think of the logic of what the biological process is or go to Wikipedia and look it up to refresh our memory. I’d rather have the information here at my fingertips, so here goes a scientific/layman combination attempt at explaining what is wrong with CFers’ bodies.

CF LungsCystic fibrosis transmembrane conductance regulator – CFTR

What the heck, Fatboy? I know, I’ll explain this – don’t worry. Simply stated, this is a protein on the a gene in chromosome 7 in people that regulates the balance and flow of water and sodium chloride between cell membranes to keep a consistent ratio of water in one’s cells. In cystic fibrosis, particularly in the mutation referred to as ΔF508, it just doesn’t work. I happen to have a double ΔF508 mutation (one gene from each parent) that makes my form of CF the “most common” by percentages of mutations known of the CFTR gene.

That means thick, sticky mucus and other bodily fluids

From an early age, even inutero, this gene can wreak havoc on the body, depending on the mutation. Some CFers go decades without diagnosis because they generally function just fine with their mutation. Others are on the lung transplant list by the age of 5. I happened to be born with exploded intestines, which is a common, obvious diagnosis of CF in infants called meconium ileus and have a huge scar running from side to side across my gut.

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Supplements and Vitamins – My New Life

PillsI used to hate taking my vitamins, and I still do. I also hated that powder my mom would mix into the milk that I’d pour onto my cereal every morning. I still hate it, but I married a super helper. She puts my pills into one of those old person pill containers on the end table for me every day of the week, and I’ve been doing much better taking everything.

What does Fatboy take?

Good question! My mom asked when they were over and I had to say,  “I don’t know. Beautiful puts everything in there and I take what she gives me.” Well, I’ve found out, and we’ll see if I know why I take everything, too. Keep in mind, this doesn’t include any of my enzymes (8 bottles of pills per month) or any of my aerosols or injectables.

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