Did you know that over 30,000 Americans have Cystic Fibrosis; mostly children. Over 10 million Americans are symptomless carriers of the disease; most don't know they are carriers (my wife and I didn't know). The mean life expectancy just went up to 36.8 years; in the 1970's the mean was about 17.
I have two daughters, ages 7 and 5. Both have CF. No one in either of our extended families ever had the disease as far as anyone knew. But both my wife and I were carriers. Each baby had a 25% chance of getting the disease and both did get it. (I know, I should play the lottery). When we found out our first child had CF (before she was born), we were initially devastated. We spent a week meeting with specialists and the Cystic Fibrosis Foundation. Our thoughts ranged from abortion to fighting. We chose to fight and have become somewhat experts ourselves in this area.
More below...
People with CF have a variety of symptoms including: very salty-tasting skin; persistent coughing, at times with phlegm; wheezing or shortness of breath; an excessive appetite but poor weight gain; and greasy, bulky stools. Symptoms vary from person to person due, in part, to the more than 1,000 mutations of the CF gene.
Most CF kids look `normal' except that they are undersized. Most people think my 5 year old is really 3 years old. She is only 39 inches tall and weighs only 32 pounds. My 7 year old was very small until last fall. She had a gastronomy tube (G-tube) inserted into her stomach wall. We feed her over 700 calories of high-nutrient formula each night while she sleeps by hooking her up to a pump that pumps the formula directly into her stomach.
Kids with CF have problems with digestion and with lung function. A defective gene causes the body to produce abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food. The mucus also can block the bile duct in the liver, eventually causing permanent liver damage in approximately six percent of people with CF.
My girls take 3-5 enzymes before each meal and snack. They also take about 6-7 other oral medications each day. They must do lung therapy each day too. They take nebulized medicine to help open up their airways and prevent lung infections. They also spend two twenty minute sessions a day on the Vest. They wear a vest that is hooked to a specialized air pump (cost - $20,000) that sends air pulses and beats against their chest wall. This helps loosen the mucus so it can be coughed up.
Kids with CF also must spend time in the hospital getting a CF Tune-Up. My kids have been lucky in that each one has only had 1 tune-up. They get a PICC line (an IV that goes in your arm and delivers medicine directly to your heart). Then they get intravenous antibiotics several times a day for 2 weeks. Many kids go in at least once a year.
I am writing this diary to raise awareness of CF. You or someone you know is probably an unknowing carrier. It is a simple blood test to get tested if you are thinking of having children. If children are diagnosed early, the disease can usually be managed, as in our case. It is a lot of work, but it can be managed. The disease is progressive, so if a child isn't diagnosed for a few years, much damage can be done to the lungs.
Sunday, May 21st is the yearly Great Strides Walk to Cure CF in Chicago. This month there are walks all over the country. The various Chicago walks raise over 1 million dollars a year. Our team (Bradley Bunch) raised $23,000 last year and hopes to raise more this year. This is all non-corporate money that our team raises. It is all through friends and family donations. The CF Foundation is one of the best charities in the country. Over 91 cents of every dollar donated goes to research and care programs.
If you know someone who is walking, please sponsor them. I know Kossacks give a lot of money to various political candidates and that is very important, but this is very important too and I hope you will consider it. My kids and many others health depend upon it!
Thank you!