Cystic Fibrosis: Treatments

May 23, 2011 By 4 Comments

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.

Most CFers, including Jesse, have a pretty long list of medications that are necessary to stay healthy. We are very fortunate to have excellent medical insurance for Jesse that makes it possible for him to get all the latest treatments available. Many of these prescriptions would cost thousands each per month if it were not for insurance, but we only have to pay the co-pays!

The first treatments Jesse does each morning is a series of aerosols. These are the best type of treatments for cystic fibrosis because the medication is delivered right to the airways and lungs, which is where the problems are. The liquid medicine is poured into the cup on the aerosol head and then forced out through the mouthpiece as a vapor. Each morning he does Albuterol, Colistin, Pulmozyme, Cayston, and HyperSal. Luckily the technology in the new aerosol machine makes each of these only take a few minutes to complete.

Aerosols

The next treatment is the Vest. The vest machine is used to get rid of any gunky stuff that has become attached to the lungs. It straps on like a life jacket and then tubes attach to the front of jacket forcing air through. The percussion effect shakes and clears the lungs. Both aerosols and Vest are done twice each day.

Lastly, Jesse takes a variety of pills that help him get the necessary nutrients that are not absorbed properly from food. Jesse takes calcium, ADEKs (specially formulated vitamins for CFers), fish oil, bisacodyl, mephyton (vitamin K), vitamin D, acidophilus, omeprazole, and azithromycin. This is where I am able to help him with treatments. I actually kinda enjoy portioning out all the vitamins for the week on Saturdays. Plus I do my allergy pills at the same time, only my list is much shorter.

Vitamins

Thanks for taking the time to read and learn about cystic fibrosis this month! Don’t forget that we still have awareness bands available for purchase. If you haven’t bought yours yet it’s not too late… and they’re only $1!

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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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