It has been a rough couple of months for the Cystic Fibrosis Community. So many have lost their battle, their lives to this awful disease. Not one of the 11 I knew from Facebook, were over the age of 23. One of those 11...an innocent one week old baby boy, Baden. This disease shows no mercy. Just last night a 20 year old woman got her angel's wings and can finally breathe easy. She and those others no longer need to fight to breathe, chained to an oxygen tank, drowning in their own lungs. Hannah's death has hit me a little harder tonight. I am not entirely sure why, but she was just starting her life, engaged to be married. She couldn't even go into a bar and order herself a drink yet. I desperately want to see Mason graduate High school, then college. I want to see Mason get married and have children. I want him to live a long happy life. I want him to outlive us. Some days it's just hard to dream past these 24 hours because I am too scared to. I don't want to be blind sided with CF. It is scary because we know someday it very well could be our child people are posting their sadness about. There is a statistic that states 40% of people with cystic fibrosis don't even live beyond 17...makes me cherish every moment that much more with Mason.
He is my very best medicine and Hero!
With sadness there also comes hope. Hope that there is a drug, Kalydeco, only 6 months away that could stop this disease in it's tracks. Without going into much detail..because I would be bring you back to high school science and then some, there is great things happening in science these days. There has been a drug that is going through the FDA process as you read this. It is a first of it's kind. It is a drug that corrects one of the DNA defects which causes CF. Now, there are 5 different ways the CF gene is defective (mutated) and this only target's one way. They are divided into "classes." This drug has been researched on the mutation G551D (only 4% of the CF population have his mutation), which is a class 3 mutation. Mason does not have this one, but one of his mutations is either a 2 or 3...it is rare so they are not certain yet. His Pulmonologist had said to us that she would like to possible have him try it out in a few years to see if it would help! My heart about stopped right there. You see this drug, has increased the CF patient's lung ability (FEV1 scores), lowered a person's sweat test to normal ranges, and has increased weight (which is hard to do with CF even though they are eating about 50% more calories a day as me and you). There are also two other drugs that possibly do similar things and are still in the trial process, but have promising results so far! She also thinks it is a possibility that these would help Mason as well. So, we need to keep Mason's lungs in as best of shape as we can so when he can try these new AMAZING drugs they will be of most benefit. They won't reverse any lung damage. What I just shared with you is a VERY VERY condensed can of info. Please don't hesitate to ask questions! This video is from the CBS nightly news. It does a good job of explaining what I attempted above.
We also learned another little tid bit of info. A question was asked why all these drugs are targeted at the lungs. I assumed it was because it is the bigger concern and what people with CF die from...respiratory failure. Why not target the pancreas as well? Our pulmonologist explained, because the mucus started thickening even before birth, the pancreas ducts are blocked and the pancreas is shot by the age of 6 months. That floored me. Hence, he take his digestive enzymes with every meal and will for the rest of his life.
Most people (and us initially) thought Cystic Fibrosis was a lung disease, but it affects sooo many more parts/organs/systems in the body. The picture below does a great job of explaining...click on it to enlarge.
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