Cystic Fibrosis: Diet

May 18, 2011 By Leave a Comment

May is Cystic Fibrosis Awareness month! Since I didn’t know anything about CF before I met Jesse, I’m taking some time to learn and share some information about CF.

Proper diet and nutrition is important for everybody, and having CF makes paying attention to diet even more important.  Just as mucus forms in the lungs making breathing difficult, thick mucus also forms in the pancreas preventing normal digestion and absorption of food.  To combat this problem most CFers require a high-calorie diet that is also high-fat.  Most of you who know Jesse also know the he really likes to eat!  I know that the idea of being able to eat as much as you want may sound ideal (because I used to think so too), but there are times when eating so much is not so enjoyable for Jesse.  Like when we get home late and we’re tired and I just grab a little snack from the pantry.  A small snack like that is not sufficient when his daily intake needs to be at least 5,000 calories.

Jesse eating spaghetti

The reason that calorie intake in so important is because of all the extra energy that is needed for breathing.  It’s also important to have the extra pounds so that when an infection hits it doesn’t completely knock him down.

The tricky part of a high-calorie diet is making sure that all these calories come from a healthy source.  Because the pancreas is already not functioning well, CFers run the risk of developing cystic fibrosis related diabetes.  So while soda and sugary snacks may be an excellent source of extra calories, they aren’t the best idea in trying to avoid adding diabetes to our list of things to worry about.  Instead, Jesse tries to get most of the extra calories from protein shakes, whole fat milk, and adding extra sauce to almost everything.  Probably our most used trick is always adding lots of extra cheese to Jesse’s portion of the meals that I make.

Cheesy TexMex

Lastly, the most important part of Jesse’s diet: ENZYMES!  Most all CFers require digestive enzymes to digest foods.  Jesse needs to take about 8 enzymes with meals, maybe extra if there’s lots of cheese.  Forgetting to take enzymes means that the food is not digested which can lead to an intestinal blockage, often resulting in a very unpleasant trip to the hospital.

Zenpep

 

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Cystic Fibrosis: Germs

May 13, 2011 By 1 Comment

May is Cystic Fibrosis Awareness month! Since I didn’t know anything about CF before I met Jesse, I’m taking some time to learn and share some information about CF.

Ordinary germs can be particularly dangerous to people with cystic fibrosis. When I catch a cold I typically feel crappy for a day or so and then I get better. However Jesse usually isn’t so quick to recover. Even after he gets over the cold, his lungs have to fight the effects of getting sick for a longer time. He’s tried to explain to me how his lungs feel after being sick or overly exhausted. He says they feel like plastic. Hard to imagine how that really feels. I think of trying to breathe in and out of a two liter bottle, having to inflate and deflate it with each breath.

Of course (like everyone else) we try our best to avoid people who are sick, but it’s really impossible to avoid all situations. Everywhere you go there are people coughing, sniffling, and sneezing. I tend to not get sick very easily, but it doesn’t take much exposure at all for Jesse to catch a bug. In the end you can’t think about it too much or you would drive yourself crazy.

Germs

The infection that Jesse is always working to keep under control is called pseudomonas. Once a CFer colonizes pseudomonas it’s pretty much impossible to get rid of it. CFer lungs are just too perfect an environment (like a nice warm and gunky greenhouse) for the bacteria to ever want to leave. Eventually pseudomonas takes over the lungs and becomes resistant to many antibiotics. That’s where Jesse is at now, and that’s why he often needs IVs when he does get sick because oral antibiotics are no match for his bugs. Luckily, the last couple of clinic cultures have shown only light growth of pseudomonas, which is pretty much his best case scenario.

Back in the day, Jesse used to go to CF camp to be with and get to know other CFers. Then they realized that all the campers were sharing all of their super bugs with each other, and camps stopped. Then they said CFers could have no contact with each other because it was too easy to get sick. That’s why the nurses make all the CF patients wear a mask at clinic, you never know what kind of germs are floating around those halls. Now we’ve been given permission to meet other CFers as long as we’re careful… three foot rule and no sharing drinks and such.

Hopefully I didn’t gross you out about germs too much, but now you know!

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Cystic Fibrosis: History

May 6, 2011 By 1 Comment

May is Cystic Fibrosis Awareness month! Since I didn’t know anything about CF before I met Jesse, I’m taking some time to learn and share some information about CF.

Even though cystic fibrosis wasn’t officially recognized until the late 1930s, people recognized signs of it all the way back in the 18th century. Literature warned “Woe is the child who tastes salty from a kiss on the brow, for he is cursed, and soon must die.” I find it amazing that even though they didn’t know why, they still made the connection that something was wrong. It makes me wonder how many undiagnosed CFers there are in Jesse’s family history.

Fortunately, Jesse was diagnosed at birth so he has had the benefits of treatments his whole life. There are over 1,000 different mutations of CF that cause variations in symptoms and severity of symptoms. Jesse has the most common mutation, ΔF508/ΔF508. Since about 70% of CFers have this particular mutation, hopefully a cure will soon be found to correct this mutation.

It’s pretty scary to think about how “new” this disease is:

Dorothy

National Library of Medicine photo of Dorothy Hansine Andersen. Andersen first described cystic fibrosis in 1938

  • 1938 – Dorothy Andersen, M.D. writes the first comprehensive medical report on cystic fibrosis (CF).
  • 1953 – During a heat wave in New York City, Dr. Paul di Sant’Agnese and others connect the extra loss of salt by people with CF to the disease’s underlying problem.
  • 1962 – The CF predicted median survival age is 10 years.
  • 1989 – A team of Cystic Fibrosis Foundation-supported scientists discover the defective CF gene and its protein product (CFTR) thus opening the door to understanding the disease at its most basic level.
  • 1990 – CF researchers achieve “proof of concept” that gene therapy (in the lab dish) is possible.
  • 1993 – Landmark gene therapy trial begins in people with CF.
  • 2000 – Scientists supported by the Cystic Fibrosis Foundation map the entire genetic structure of the most common cause of CF lung infections-the Pseudomonas aeruginosa bacterium. Researchers can identify the function of specific genes and find ways (drugs) to turn off the bad ones.
  • 2008 – The Foundation and Vertex Pharmaceuticals achieve a “proof of concept,” showing that it is possible to treat the root cause of CF. During Phase 2 studies of VX-770, trial participants, all of whom carry the G551D mutation of CF, show unprecedented improvements in key signs of the disease.
  • 2010 – Approximately 30 potential therapies are in the Foundation’s drug discovery and development pipeline. The more drugs in the pipeline, the greater the odds of producing successful therapies and a cure for CF.
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