May is traditionally 'National Cystic Fibrosis' Month. I am not sure if Congress will get around to making it official this year as they have the last several years (but that is ok with me because they are rather busy with much more pressing issues).
I hope no one in this great community of ours minds if I use a diary to spread a bit of information about this disease. It affects me personally as my two young (8 and 6) daughters have CF.
Mackenzie and Anika were diagnosed before they were even born. Although no one in either my wife's or my family can ever remember a relative having CF, we both were carriers of the genetic disease. This was determined during routine blood tests we each took when trying to get pregnant.
We knew nothing of the disease but were not worried when we found we were carriers as we would only have a 25% chance of having a baby with CF. We got 'lucky' in both cases.
Having found out 'in uetero' that we had a baby with CF, we agonized over whether or not to keep it. When we were told it was a girl, we quickly decided that we would keep her and work to find a cure. (I don't know why that mattered but it seemed to make the decision easier).
Cystic Fibrosis is a genetic disease; the largest genetic killer of children in America. 30,000 Americans have it with 1000 new cases every year. In the 1950's almost no CF children lived to go to Kindergarten. Last year, the mean life expectancy rose to 37.
CF is a progressive disease that affects the lungs and digestive system. It causes a think mucus to form in the lungs and pancreas. This leads to poor digestion and constant lung infection. Eventually the lungs are so damaged that a lung transplant is the only recourse.
CF patients have salty tasting skin, persistent coughing, poor weight gain, and frequent lung infections. Many have a 1-2 week hospital stay (called a tune-up) every year. This is to receive intravenous antibiotic treatments.
For more information on CF, visit the CF Foundation at: http://www.cff.org
Each of my daughters takes 6-8 different oral prescription drugs a day, including 3 – 6 enzyme capsules before every meal and snack. They also have to go on antibiotics several times a year. They each do therapy twice a day which takes about 25-40 minutes a session. The therapy includes them wearing a ‘Vest’ that is hooked up to an air pumping machine. It beats air against their back for 20 minutes in an attempt to loosen mucus in their lungs. They also breathe medicine using a nebulizer.
Last August, Anika went into the hospital for a G-Tube. This is a ‘port’ they put in her stomach wall. At night we hook her up to a pump and feed her high-caloric, high-nutrient formula. It looks like a little orifice on her tummy and is about 1 inch across. It sticks out about half an inch. This has helped her gain both weight and height and has really improved her overall health. Mackenzie had the same procedure done in August 2005.
Because there is such a relatively small population of CF patients, most drug companies don't make drugs for CF patients. They can't sell enough of them to make their profits. The CF Foundation raises money to give to drug companies to defray the cost of developing new drugs.
In the last 10 years or so, many new drugs have been developed through this model which will likely extend the life and quality of life of my two daughters. The CF Foundation is really solely responsible for this increase in life expectancy.
It has been a hard 8 years of CF care; something neither of us was really prepared for. It is with us all the time. In fact, just this past weekend, I dropped my youngest daughter off at a birthday party, but I forgot to give her enzymes. This meant that she was unable to have any pizza, cake or ice cream to eat with the other kids. If she doesn't take these 3 large capsules before eating fatty foods, she will get horrible tummy aches and bad bowel movements for a day or so.
My wife and I are committed to making sure our daughters are more than just 'CF Kids' though. We try to treat them the same as other kids and let them do things that other kids do. But we are strict about CF care. We never miss a treatment or therapy. This often means they have to quit playing before other kids do so they can go in to do their therapy.
Every May, the CFF hold Great Strides Walk for a Cure around the country. This year, in Chicago, our walk is on May 20. Our team will be out there walking as we have every year for the last seven. As a team over these past years, we have raised over $110,000.
I would never use a diary to ask for money, but if you feel the inclination to make a donation or just read more about CF (or see a picture of my girls), my web page for the walk is:
If you donate, please email me with your address (snail or email) so we can properly thank you.
Really though, this diary is just about CF awareness. I hope you consider what these kids go through and how things like stem cell research and universal health care can help their quality of life.
Thank you for reading!