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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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I Say Megase, You Say Megace. I Gain Weight.

Megase / MegaceNo matter how you spell it, Megase (megestrol acetate) is really making an impact this week, so I looked up some information on how/why it works. Here is what I already knew going into my research:

  • It’s often prescribed to anorexia, cancer, and AIDS patients who have a severe loss of appetite.
  • Whenever I take it, it’s not that I become hungrier, my brain doesn’t receive the signal to stop eating.

Going along perfectly with one of my most popular articles: Eating With Cystic Fibrosis: It’s a Full-time Job, this is just another attempt at creating a caloric income greater than caloric outgo. This week, I’ve decided to try to take it as prescribed on the bottle instead of just as a little booster for meals to make sure I eat “enough.” Let’s see if I can express the results of said action accurately.”

“Oh my!”

I’ve been eating to the point where I actually have to lay down because sitting up is uncomfortable. My weight is back up over 120 at all times of the day, and it almost seems like I’m thinking about food more. Maybe it does have an appetite thing that I wasn’t getting the effects of when just taking a bit at a time for a certain meal.

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Monthly Maintenance: My Trip to the ENT

Smiling before...I’ve lost track, but I believe I’ve had 7 sinus surgeries over the years. It’s between 6 and 8, for sure. I had my first one around the 4th or 5th grade, but my worst one was my second one when the surgeon realized during surgery that I had broken my nose over the summer and he BROKE it again, shoved 4″ plastics shin guards up there and stitched them through my septum! I was not a happy camper when I woke up. The shortest duration between operations was 9 months and the longest is my current streak: going on since 2004.

Nasal polyps are teardrop shaped, noncancerous growths on the lining of your nasal passages or sinuses. Small nasal polyps may cause no problems and go unnoticed. Larger nasal polyps can block your nasal passages or sinuses and cause breathing difficulties, a loss of your sense of smell, frequent sinus infections and other problems. – Mayo Clinic

I’ve got chronic sinusitis and had some really bad situations that were discovered during surgeries. One time, the doctor said that I had an infection in my frontal sinus (above the eyes) on the right side that had eaten through my skull and was in danger of infecting my brain. Every surgery has been called for because of sinus polyps and needing to open things up, which they call “scraping and windows.” I’ve only been operated on by two different surgeons, but never needed what they call “packing:” yards of gauze shoved into my sinuses for a week or two, which is very painful to remove. “Yay!?”

My Florida ENT retired last year after fighting and winning a round with cancer. I miss him a lot (only a CFer would say something like that), but I’m getting used to and liking his referral ENT now. She’s in the same office, so really the person shoving instruments up my nose is the only thing that’s changed in the last 12 years of ENT visits in Tampa. After my last surgery, we decided to do monthly flushes with antibiotics into the frontal sinus (through a very, very small opening) and the main maxillary sinuses.

So far, so good. No surgeries since and only a handful of sinus infections that required antibiotics.

Today, I have a treat for you: photos. I asked my new ENT, Dr. Janet Seper if she could have someone take photos with my phone so I could post them for the site for the benefit of others.

Warning: If you don’t want to see me looking uncomfortable, don’t continue. The last image is for the benefit of those who want to see what ails me and lives in my nose – as she finally got it out at the last moment with a special vacuum attachment, which you will have to click to view.
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Eating With Cystic Fibrosis: It’s a Full-time Job

I realized this morning how much of my day is spent contemplating my next meal or two, snacks, and whether or not I think I will meet my daily caloric goal. Today, Beautiful suggested that I explain how much I need to eat just to maintain my weight, so here it goes.

Requirements

According to a nutritional calculator at Mayo Clinic’s website, with my lightly active lifestyle, I should only need 1,900 Calories per day to maintain my weight as a 31 yr-old, 5′ 4″ male weighing ~120lbs. That would be nice! I also calculated Beautiful’s daily requirement as being 1,750 Calories. You’ll see the problem with that in a second.

If I go through a normal day of eating (pre-Fatboy) to just get through life without paying attention to eating enough, I’ll probably encounter 2,000-2,500 Calories and lose 2-3lbs by the next morning. If I make an effort to be sure I get enough to eat to get in the neighborhood of 4,000 Calories, I’ll break even. It’s not unless I intake 5,000-6,000 Calories that I can have any expectation of seeing the scale budge upward.
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