Traveling with Cystic Fibrosis

December 15, 2011 By 6 Comments

Remembering everything you need to bring when traveling can be challenging for anyone. As you can imagine, it gets even more difficult when you have an arsenal of Cystic Fibrosis medications and equipment to bring along. For years Jesse chose to ignore CF and skip most (or all) treatments when traveling so we’re just now getting the hang of how to pack everything he needs. After a couple of successful trips this year, I thought I’d share some tips we’ve learned for our CF friends who are traveling for Christmas…

  • We try to pack all medications and related supplies in one suitcase. It just makes sense that this is the best way to make sure we have everything and it makes it easier to unpack when we get to our destination. When we travel by air we keep this as our carry-on bag so that there is no risk of it not arriving when we do. When we travel by car we want to make sure that none of the meds get left in the car if we don’t bring everything into the hotel room, and risk them overheating.

Meds bag

  • Also when traveling by air… we like to get a letter from clinic stating that all of the medications, supplies, and equipment are necessary for Jesse to travel with. The meds bag usually gets flagged and checked out pretty carefully (understandable) and we like to make sure we will get through with the needles and all the liquid vials. We also like to make sure we will be allowed to keep the Vest machine and CPAP machine with us as extra carry-ons since they are too expensive to replace if they were to get lost somehow.
  • Since the last two trips were road trips, I just brought the entire container of weekly vitamins. We were gone for more than two weeks both times and it was much easier to fill the pills container like usual instead of trying to count them all out ahead of time. And of course… it never hurts to bring extra Zenpep!

Vitamin container

  • We like to have a separate cooler, other than our food cooler, for the refrigerated meds so that it isn’t getting opened and closed all day. When get to our hotel I empty the meds into the fridge and refreeze the ice packs for the next day.

Cold meds

  • Lastly, Jesse has gotten used to finding a treatment chair to unload all of his stuff when we get where we’re going. The most important part is making sure the treatments actually get done. It’s hard when you’re busy being on vacation, but he feels so much better during the activities of the day when he has done them.

Treatment chair

That’s what works for us. Anyone else have some helpful tips to share?

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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: 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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