Friday, September 24, 2010


Okay so I have noticed that a lot of CF patients use a variety of different nebulizer systems and nebulizer cups.

I was just wondering what nebulizer system does everyone use. What cups do you use. And do you use different systems/cups with different medications?

For example I am on cayston - so I use the Altera nebulizer system and cups JUST for Cayston.
I use the Pari LC cups for the rest of my meds and I use the Pari ProNeb Ultra 2 compressor with those.

Thursday, September 23, 2010


Taken from the website
"Vertex Pharmaceuticals is developing two oral compounds designed to treat the basic defect in Cystic Fibrosis - a faulty gene and its protein product, called CFTR.
VX-770, the most advanced of the two compounds, is known as a potentiator and is designed to allow CFTR located at the cell surface to function correctly.
Vertex is also developing VX-809, known as a corrector, which is designed to move defective CFTR to its proper place in the cell."

In January of this year my doctor's office called me and asked me if I wanted to participate in a new drug trial. It was for Vertex 770. They wanted a group of CF patients who were homozygous (carried two of the same genetic mutations) for DeltaF508. I was one of the few patients at my CF Center that was eiligble for this. I jumped at the chance! In order to qualify for the study you had to have a FEV1 of 40%. Luckily at enrollment date my FEV1 was 50%. I began the 16 week study. My first PFT which is the one that counts was only 38%. But since I had already enrolled in the study at 50%, I was still able to partake. It was a double blind study. That means that both myself and my doctors did not know whether I was receceving the actual drug or the placebo. The study entailed me keeping in touch with the doctors every two weeks. I either had to go into the office for a sweat test, multiple blood draws, pulmonary function tests and EKG's or follow up with a phone interview. The pill was a large blue pill that I had to take twice a day after consuming a high calorie meal. To this day I still am not sure whether I was on the placebo or actual drug but I am glad that I participated! At the end of the 16 weeks you were able to continue on to the second part of the trial if you had either a 10% increase in your FEV1 and/or a decrease in your sodium chloride level from the baseline sweat test that was drawn. I am not sure what my last sweat test result was but, I did have an increase in my PFTs. Day One (FEV1 - 38%). Week 16 (FEV1 - 43%) which calculated to be over a 10% increase.

In June I began the open label part of the Vertex 770 study. The study is going to go over a 96 week period. I am now officially getting the real drug. I still have to go to the doctors for visits but not as often as the first part. When I do go I still get blood draws, EKG's, PFTs, and sometimes a sweat test. I also have to go for liver function tests every two weeks. I got hospitalized at the end of June and my doctors made me stop taking Vertex for a little while because I was running fevers and they wanted to make sure that it didn't have anything to do with the drug. I began taking it again continuously at the end of August. It's been almost a month now of taking it on a regular basis and I really do feel like it is making my mucus thinner.

I am excited for the future of CF drugs. Vertex has two different compounds VX-770 and VX-809 and from what I have been reading - eventually they will be tested together. The two compounds together are supposed to have the best result. One is a corrector and one is a potentiator - so each compound has different effects based on your mutations and the effect it has on the CFTR protein. Research has actually shown that VX-809 has a better result in my genetic mutation (DDF508) - so I am really hoping that my CF Center will soon be testing this compound as well.

Wednesday, September 22, 2010

Health Renovation!

First let me start out by saying that life has been CRAZY since I last posted! I am still working as a NICU nurse and absolutely loving it! I have now been at my job for 6 months! The time has been flying by. It's really amazing how you don't mind working when it's doing something that you love.

This update is basically about getting myself as healthy as I possibly can be. I know that I have said this all before but after the last few months that I have had - I am more motivated and determined than ever to NOT let CF get in the way of my everyday life.

The past few months have been spent in and out of the hospital. I was in the ICU. Had a systemic infection from another PORT infection. I had fevers up to 108! I required oxygen 24/7 for almost two months straight. My oxygen levels went down to the low 80's with even the slightlest movement. After two back to back hospitalizations, being on oxygen 24/7, missing TONS of work, six long weeks of IV antibiotics, getting a THIRD port and losing a tremendous amount of weight - I want to physically do everything in my power to never get to that point again. Granted - the infection was probably something that I couldn't have prevented - but I wasn't in the best shape physically before the infection took place, so when it hit - my body had no idea what to do. It really took a toll out on everything. When I got home from the hospital I couldn't even make it up a flight of stairs without literally pulling myself up the banister. I was so scared, I honestly felt like I was starting to die. While I was in the hospital I had set up a meeting with my doctor, my mother and my sister because I wanted to ask her point blank if she thought I was getting progressively worse. My doctor, whom I trust very much, stressed that she really didn't have all the answers, but that she really felt I was just going through a rough patch and that I would start to turn around.

It's now almost a month and a half since I was discharged from the hospital and I do believe my doctor was right. I no longer feel that I am losing control of my very existence. My PFT's are stable. My FVC on 9/14/10 was 63% and my FEV1 was 42%. In January my FEV1 was in the 60's. I KNOW that I can do better. I have been following a blog on here written by a fellow CF patient, Ronnie Sharpe. He has an amazing outlook on life and is extremely beneficial to anybody that has CF and is in a funk. His blogs, plus guest blogs that he features on his site have officially motivated me to really give my all to my health. Through exercise, many CF patients have turned their health around and increased their lung functions.

From all of the data that I have seen and written proof that exercise is the key to CF management - it's time I get serious about fitness! I have always been compliant with my medications. Unfortunately for someone with CF, that isn't always enough. I am going to start today - exercising seriously. Getting my body in the best possible shape will help me to be able to work at my job for a very, very long time. It will hopefully enable me to feel well enough to be able to realistically think about becoming a mother when the time comes. Most importantly, it will show CF who's boss!