Cystic Fibrosis (CF) is a serious genetic disease that affects the lungs and other organs. Currently, around 30,000 people in the United States have the disease and there is no known cure. However, modern genetics and public awareness, have made it possible for doctors to diagnose CF earlier and new technology can help improve the quality of life for those affected.
What causes Cystic Fibrosis?
Your body was designed to fight infection. The epithelial cells in your organs produce mucus which traps germs. People with cystic fibrosis have trouble with their epithelial cells. Due to a mutation in their cystic fibrosis transmembrane conductance regulator (CFTR), their cells produce especially thick and sticky mucus. This mucus clogs their lungs and makes it difficult for them to breathe and interferes with other bodily functions.
Symptoms of CF in the respiratory system can include the following:
- Coughing accompanied by mucus discharge
- Wheezing
- Repeated lung infections like pneumonia and bronchitis
- Chronic sinus infections
- Trouble breathing during physical activity
CF affects the digestive system as well because of the excess mucus blockages in these organs. These symptoms include:
- Trouble gaining weight
- Difficult bowel movement
- Abnormal stool texture (greasy, bulky)
- Intestinal blockage in infants (also known as meconium ileus)
- Excessively salty skin
Because these symptoms are common for several illnesses, it is important to have testing done and let a doctor diagnose whether or not you have Cystic Fibrosis.
How Is It Diagnosed?
Genetic defects cause cystic fibrosis. This means that it has to be inherited. Those with CF inherit a copy of the defective gene from both of their parents. However, even if both parents have the gene, their children only has a 25 percent chance of developing CF.
CF is often diagnosed soon after a baby’s birth because of the newborn screening that the United States hospitals mandate. This screening involves taking some of the baby’s blood, sending it to a lab, and looking for the genetic defects. If the test comes back positive, doctors may order a sweat test. This tests the amount of salt in your baby’s sweat. Sweat test reliability outweighs all others.
Those with milder cases of CF may not discover they have it until they are teens or even young adults. A sweat test is often used to diagnose CF in this more mature crowd. Blood tests and genetic testing may also help diagnoses.
Treating Cystic Fibrosis
Although medical science has come a long way in CF studies, there is no known cure for CF. However, if you or your child are diagnosed with CF there are ways to manage the disease.
- Certain medications help with digestion since the body struggles to properly digest food. Working with a nutritionist helps establish a diet high in vitamins so patients get the nutrition they need.
- Several medications help CF sufferers from the complicated lung problems they face. These medications reduce the amount of mucus produced. This helps them to breathe easier.
- Doctors can monitor lung function with portable spirometers. These devices can help identify respiratory issues before they become more serious.
- Certain breathing techniques clear the lungs. This improves the quality of life and increases the function of the lungs. Inhaled medicines open up the lung passages and supply antibiotics to prevent infection in the lungs.
- Keeping active is a must. By moving around, it clears the lungs naturally and increases the quality of life.
Living With Cystic Fibrosis
If you or your child has been diagnosed with cystic fibrosis, what steps should you take next? The first step is to follow the plan that your doctor has made for you. CF varies for each person, so it is important to follow your own treatment plan. This plan helps your quality of life and ensures that you stay as healthy as possible.
You should also make sure to follow a nutritious lifestyle. Because of the nature of CF, it makes it difficult to absorb the nutrients your body needs. Part of your doctor’s plan should include what enzymes and foods you need to help your body absorb needed nutrients. This will also help you to experience less of the digestive issues caused by CF.
At times it may be difficult to stay active, but it is important for you to stay as active as possible. It helps your lungs to gain strength, balances the fluids in your body, and helps your heart stay healthy.
Joining a support group for those with CF helps connect you with others who are facing similar issues. This helps you to see you are not alone while facing the challenges that CF brings.
There is no known cure for cystic fibrosis, but researchers have made many advances. CF sufferers now enjoy longer lives with the many treatment options available to them.
