I intern at a children’s hospital, so it’s sometimes difficult to focus on one disease over another. Since I also intern at the Cystic Fibrosis Foundation, CF has been one disease that stays with me every day. Today I had the opportunity to interview someone in the office who happens to have two cousins (they’re brothers) who have CF.
It did not really occur to me how terrible it is to learn that your child, sibling, cousin has CF. In fact, the two men I learned about today were diagnosed when the younger brother was born and the older brother was only 3. At that point in time, the parents were told their sons had about 15 years to live.
Cystic Fibrosis affects around 30,000 people in the United States. The disease is carried by a recessive gene—when a man and woman have the CF gene, their children have a 1 in 4 chance of developing CF. Around 3% of Americans are carriers for the CF gene—they exhibit no symptoms and may not find out they are carriers until they have a child diagnosed with CF.
Cystic Fibrosis is a disease in which the body produces thick mucous that coats and clogs vital organs—especially the lungs. The mucous also makes it difficult for the digestive system to function properly as the digestive enzymes cannot work effectively—leaving many individuals with CF underweight. In order to maintain a healthy weight, people with CF have to take enzyme supplements with every meal.
So what’s the day-to-day like for someone with CF? Today, children go through hours of respiratory treatments every day and take dozens and dozens of medications (years ago the only way to lift the extra mucous from the lungs was through PT—which really consisted of beating the person with CF on the back). If individuals with CF come into contact with someone with a cold or flu, they can become critically ill. To make matters worse, there are certain bacteria that live in the lungs of people afflicted with CF and if these people come into contact with the bacteria of other people with CF, this can also cause extreme decreases in lung function.
Exercise is very important for children and adults with CF, because it helps loosen the mucous in the lungs. It is also important to cough when possible, as this is the body’s way of eliminated the extra fluid. People with CF become dehydrated more easily than typically developing individuals, and they also have a higher caloric need. That said, it is important to have high calorie, high sodium snacks and drinks after exercise to maintain proper nutritional levels.
So while the average age of survival for someone with CF is now 37—this is currently a set number. That means no matter how well someone with CF follows his/her treatments and how good he/she is as a person—he/she is going to die young.
The Cystic Fibrosis Foundation donates around 90 cents for every dollar raised to fund research, education and care. It is amazing to me how wonderful the “CF Family” is—I have learned so much from working with the people at the Cystic Fibrosis Foundation, and I am committed to fight to cure CF.
*Much of the statistical information found on the CFF website.*