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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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Zenpep, the New Enzyme on the Block

Since I’ve been battling gaining weight for so long, I mentioned my desire to fatten up every time I go to clinic. I’ve had some really rough bouts with intestinal blockages this year. I had two in just 3 months, and one put me in the hospital overnight after spending all night and all day in the ER because regular medical staff can’t wrap their heads around the fact that I had two feet of my small intestine removed at birth. I did some calculations and came up with figures that call that 25-40% of my small intestine. There is scar tissue and some bottleneck in there that binds from time to time.

After my last episode, I was messed up for weeks. I was in pain for over a week and my normal body rhythm was all out of whack. There was bloating, gas, discomfort, chronic bathroom breaks, etc. One evening with family over, I went to the bathroom 10 times after dinner. Something had to change! Eventually, things returned to normal, but I was still deadlocked at 108-111lbs.

I went to clinic on February 19th and mentioned to my coordinator that things were still “not right” and told her my 3 main complaints were still bothering me. She said that a new enzyme just got approved and she had a sample bottle available for me. It’s called Zenpep. If I liked its results, my aerosol supplier ships the enzymes, too.

I went home and tried it that afternoon. That evening, no excessive bloating or bathroom trips. The next day brought the same good news, plus some added bonus of gaining 1.2lbs. The next time I weighed myself, I’d gained 2.5lbs. Things were on the move in the direction I wanted. During the time between running out of the samples and my new shipment arriving today, my old enzymes were their usual mess, shall we say. Today, I’m all better again.

Enzymes can really make a difference in your life.

Here is my theory on why Zenpep is working so much better for me than Ultrase MT20 and Creon MT20 have been: the amounts of enzymes within each capsule is vastly different. For example:

Ultrase MT 20:

lipase 20,000 USP units
amylase 65,000 USP units
protease 65,000 USP units
lipase 20,000 USP units
amylase 109,000 USP units
protease 68,000 USP units

You’ll notice a nearly two-fold increase in the amount of amylase in Zenpep. Now, I haven’t done any research on why they use this formulation, but here is what my research about those enzymes came up with:

amylase – Amylase is an enzyme which helps digest glycogen and starch. It is produced mainly in the pancreas and salivary glands.

lipase – Lipase; an enzyme secreted in the digestive tract that catalyzes the breakdown of fats into individual fatty acids that can be absorbed into the bloodstream.

protease – Enzymes acting on peptide bonds. EC 3.4.-

Given the nature of much of the nutrition we eat trying to gain massive amounts of calories (complex carbohydrates), it stands to reason in my head, dangerously educated by 2 semesters of chemistry and a growing understanding of my body chemistry with the advent of diabetes risk, that my diet requires higher concentrations of amylase to process those complex carbs.

I would highly recommend asking your doctor or clinic coordinator about Zenpep. They even offer up to a $50 rebate on your co-pays through December, so long as you have private insurance, not gov’t insurance. I’ll be getting these for free!