Cystic fibrosis is a very dangerous condition that affects 30,000 people in the United States. Because of how serious and life-threatening the condition can be, it is usually screened for in American infants within the first few months of life. If your child is diagnosed with cystic fibrosis, you should not panic; the prognosis for sufferers of the disease is getting better every day.
What Is Cystic Fibrosis?
Mucus can be very annoying when you have a cold and it makes your nose stuffy, but the thin liquid serves a very important purpose in your body; it keeps the airways, intestines, and other tubes in your body lubricated. However, cystic fibrosis causes the body to produce mucus that is thick and sticky, leading to dangerous blockages. The condition can cause sufferers to have extreme trouble breathing or to have difficulty absorbing important nutrients from the food they eat.
What Causes Cystic Fibrosis?
Cystic fibrosis is caused by a mutation of the CFTR gene. This gene is responsible for making the the CFTR (cystic fibrosis transmembrane conductance regulator) protein, which produces mucus and other bodily fluids. Because it is a genetic disorder, it runs in families; both parents need to be carriers of the recessive defective gene in order to pass on the condition to their children.
What Are the Symptoms of Cystic Fibrosis?
Some of the most common symptoms of cystic fibrosis are:
- Salty-tasting skin
- Cough (possibly producing phlegm)
- Stuffy nose
- Shortness of breath
- Frequent lung infections
- Greasy stools
- Lack of growth
How is Cystic Fibrosis Diagnosed?
In the United States, infants are usually screened early for cystic fibrosis. A blood test is the first diagnostic tool. Doctors screen for high levels of chemicals that indicate stress in the pancreas. If these results come back positive, a more comprehensive DNA test is done to confirm the diagnosis of cystic fibrosis.
How Is Cystic Fibrosis Treated?
Cystic fibrosis cannot be cured, but there are many treatment options available to help patients carefully manage the condition. Some of these treatments include:
Various medications can be used to treat infections, thin mucus, and keep airways open.
- Physical Therapy
Physical therapy of the chest can help to loosen mucus in the lungs and prevent blockages. This therapy can be done with the help of another person or by using a machine designed for cystic fibrosis patients.
- Feeding Tubes
If a cystic fibrosis patient has severe problems with digestion due to intestinal blockages, a feeding tube may be used to help the patient receive proper nutrition.
In extreme cases, surgery may be required. Possible surgeries can range from outpatient ones such as endoscopy to remove mucus in the throat to more serious ones like bowel surgery to remove blockages.
How Can Cystic Fibrosis Be Prevented?
Because cystic fibrosis is caused by a recessive genetic mutation, the only way to prevent it is to avoid having children if you are a carrier. If you do not know whether you carry the gene that causes cystic fibrosis, you and your partner can have genetic blood testing done that will tell you the genetic disorders that your children will be at risk for. If you and your partner both carry the cystic fibrosis gene and you still decide to have children, make sure that they are tested for cystic fibrosis as soon after birth as possible.
Even just 50 years ago, children with cystic fibrosis were lucky to see their fifth birthday. While the disease is still very serious, the prognosis is much better; more than half of cystic fibrosis sufferers are over the age of 18. Hopefully, medical advancements will continue and cystic fibrosis will one day see a cure. For more information or for emergency care with cystic fibrosis symptoms, contact an urgent care clinic.