Wednesday, June 25, 2014

Another CF drug breakthrough...and what it means to me

It's an exciting time to have cystic fibrosis (CF)!

Who would ever think to hear those words? That sentence alone probably makes me sound certifiably insane.

I mean do I look like a guy with issues? Important note: I'm on the right in this picture.

Cystic fibrosis is one of the toughest, nastiest diseases that the world has ever seen. It doesn't just affect the lungs. It affects the digestive system and the reproductive system as well. It also makes patients more apt to have other diseases and conditions including certain cancers and CF-related Diabetes. Moreover, it continues to be one of the most life-threatening genetic diseases that the world has ever seen.

As cruel as this disease is, there is now some hope. There have been a lot of new drugs hitting the market as well as some great news from drug studies. Here is just one example:

Now if you read this article, the results state that improvement averaged only 2 to 3% and some patients got off the drug due to side effects. There are many that say these numbers indicated that this isn't much of a breakthrough. The truth is though that any type of a breakthrough is still a breakthrough and I as a patient am very excited not only about this new treatment but about the potential of having even more of them.

As a kid, I would have given anything to hear the kind of news we're hearing today. There were no breakthroughs when I was growing up. There were no adult CF clinics. Very few people lived that long. Most people who knew me had never even heard of cystic fibrosis. The common questions then were "Is it contagious?", "Can you just have it removed" and "Is that bad?". Some even asked if it contributed to my poor sense of humor. Due to all the notoriety these new treatments and studies are getting, those questions are becoming fewer and fewer...well except the last one.

Whenever I would go in to see my doctor as a kid and even as an adolescent, the typical answer in regards to when a potential breakthrough would hit was "7 to 10 years" which sounded more like a prison sentence rather than a hope for a cure. I felt defeated because those numbers never changed. I'd heard "7 to 10" when I was 10 and I heard that same timeframe when I was 20.

I acted tough back then but in reality I was scared to death of my prognosis.

Vertex and the CF Foundation continue to work to find a cure and while there is no cure as of yet, there are several new drugs in the pipeline (like the one in the article above) that are either now available or will soon be available to those who badly need them. The combination drug for my genotype is not available yet but I know they are now working on it both in Israel and the United States.

I remember the days when I was in my twenties and had to go to a children's hospital to see my CF doctor. When I went for X-Rays, they asked where my son was as they assumed I was the father since a) very few people lived to adulthood with CF and b) I was in a children's hospital. Or maybe they were taking a stab at my maturity level. They would not have been the first.

Cystic fibrosis works harder than any person in the world. It never takes a second off. Even when we as patients sleep, mucous tends to fill our lungs and we have to cough up several cups of phlegm when we wake up.

CF has changed throughout my life but it has never been removed from my Most Wanted List. As a young kid, it annoyed me because I had to lie down for 30 minutes a day while my parents hit my back, sides and front - a procedure called postural drainage. As a preteen, it aggravated me because I couldn't do the things that a lot of kids could - overnight camp, trips without my parents and being around anyone with even a sniffle. As a teenager until my early twenties, I felt alienated because of my CF. I hated traveling with a 50-pound machine even though it beat postural drainage. The irony was that CF patients are mostly skinny and lack energy yet the machines we used were remarkably heavy for even weight-lifters to carry. For the last 20 years, CF has affected me in trying to have a family, dealing with IV treatments and trying to live life without interruption as an adult.

It didn't matter whether I was a youngster...

or an adult. Cystic fibrosis got the better of me for a long time.

I hate this disease and a few decades ago I would take that hate and use it to hurt myself. I would give in to depression. I would stop doing my meds. I would refuse to read anything regarding research or things I could do to better myself.

Today I have reversed that sense. I now comply with my treatments everyday - now twice a day and sometimes three times a day. Over the last 20 years, I've rarely missed a med or a treatment. I make sure to go to the doctor at least once every 3 months and I am a workout fanatic. I know that somewhere there is someone who thinks that he or she is working harder than me. I refuse to let that be the case. I still deal with depression but I also see a therapist and psychiatrist and take antidepressants. I don't believe that admitting that I have a problem is a shortcoming. I believe, in fact, that it is one of my biggest strengths. I now read countless articles regarding research and listen to other patients to see what they're doing that has helped them be successful in the fight against CF.

Running the Peachtree Road Race, a 6.2 mile run in Atlanta, every year (this year marks #18) is one way I fight against CF.

As tough as CF has been, I'm very fortunate. I have a loving wife. Andrea has helped me with my IV's and been by my side through everything. She knows when to give me encouragement and when I need tough love. Anyone with CF knows that our partners play a huge role as to how well we do.

I have been fortunate in other ways too. To be successful with In Vitro Fertilization once is a miracle in itself. Andrea and I have been fortunate twice. I don't just give my all for me anymore. It's about Andrea, Avery and Ethan too.

I have a lot of people in my corner.

Each day I do 2 hours of treatments, take 30+ pills and make sure to exercise for at least 30 minutes. I used to be bitter about the regimen I had but now I'm proud that I can still do these things and be a CF fundraiser, a full-time employee and a loving family man as well.

New treatments or no new treatments, I'm beating this disease!

My pulmonary function has diminished over the years which is common for people with or even without cystic fibrosis. Still I'm in really good shape for someone with the disease. Maybe in 7 to 10 years I won't have this disease anymore.

Now more than ever that seems possible.

Like I was saying, "It's an exciting time to have cystic fibrosis!"

And now you know why.

Live your dreams and love your life!

Best Wishes,


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