Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It mainly affects the:
- Lungs
- Pancreas
- Liver
- Intestines
- Sinuses
- Sex organs.
In the United States, cystic fibrosis is a common genetic disease that mainly affects Caucasians (whites). It also affects other ethnic groups, but this is much less common.
Cystic fibrosis occurs in:
- About 1 in 3,000 Caucasian births
- About 1 in 15,000 African-American births
- About 1 in 31,000 Asian-American births.
Understanding Mucus and Cystic Fibrosis
Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, in cystic fibrosis, an abnormal gene, called the cystic fibrosis transmembrane conductance regulator (CFTR) gene, causes mucus to become thick and sticky. The thick, sticky mucus builds up in your lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. Over time, these infections can cause serious damage to your lungs.
The thick, sticky mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These digestive enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully.
As a result of cystic fibrosis:
- Nutrients leave your body unused, and you can become malnourished
- Your stools become bulky
- You may not get enough vitamins A, D, E, and K
- You may have intestinal gas, a swollen belly, and pain or discomfort.
Understanding Sweat and Cystic Fibrosis
The abnormal
cystic fibrosis gene also causes your sweat to become extremely salty. As a result, when you perspire, your body loses a large amount of salt. The loss of salt can upset the balance of minerals in your blood, which may cause you to have a heat emergency.
The symptoms and severity of cystic fibrosis vary from person to person. People with severe cystic fibrosis symptoms can have serious lung and digestive problems, while people with mild cystic fibrosis may not have any symptoms until they are adolescents or young adults.
The most common symptoms of cystic fibrosis are:
- Frequent coughing with phlegm
- Frequent bouts of bronchitis and pneumonia
- Salty-tasting skin
- Dehydration
- Diarrhea
- Malnutrition
- Poor growth
- Infertility, mostly in men.
Over time, mucus buildup and infections will lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs.
Some babies with cystic fibrosis have meconium ileus, a blockage of the intestine that occurs shortly after birth.
Until the 1980s, most deaths from cystic fibrosis occurred in children and teenagers. Today, with improved treatments, people with cystic fibrosis live, on average, to be more than 35 years old. However, respiratory failure is the most common cause of death among people with this condition.
The sweat test is the most common diagnostic test for cystic fibrosis. The sweat test measures the amount of salt in your sweat.
Other tests that can be used to help diagnose this condition include:
- A chest x-ray
- A sinus x-ray
- Lung function tests
- Analysis of sputum cultures and/or stool samples
- Genetic testing of a blood sample.
Current Treatment for Cystic Fibrosis
Antibiotics are the primary treatment for lung problems due to cystic fibrosis.
Other treatment options include:
- Chest physical therapy
- Exercise
- Mucus-thinning drugs
- Other medications to reduce inflammation in your airways and help to open them up.
Lung transplantation is an option for some people with the condition.
The digestive problems in people with cystic fibrosis can be managed with:
- Nutritional therapy
- Enemas
- Mucus-thinning drugs
- Medications to reduce stomach acid.
Ongoing medical care from a team of healthcare providers who specialize in cystic fibrosis is important.
Good cystic fibrosis self-management includes:
- Eating a healthy diet
- Avoiding tobacco smoke
- Exercising frequently
- Doing chest physical therapy every day
- Drinking lots of fluids
- Washing your hands often to reduce your chances of infection.
Other Names for Cystic Fibrosis
Other names for cystic fibrosis include:
- CF
- Cystic fibrosis of the pancreas
- Fibrocystic disease of the pancreas
- Mucoviscidosis
- Mucoviscidosis of the pancreas
- Pancreas fibrocystic disease
- Pancreatic cystic fibrosis.
Summary of Cystic Fibrosis
Key information about this disease includes the following:
- Cystic fibrosis (CF) is an inherited disease of your mucus and sweat glands. CF affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs.
- In cystic fibrosis, an abnormal gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene causes mucus to become thick and sticky. This mucus builds up in the lungs and blocks the airways, creating an environment that makes it easy for bacteria to grow. This leads to repeated serious lung infections that can damage your lungs.
- The mucus can also block tubes, or ducts, in your pancreas so that the digestive enzymes it produces cannot reach the intestines where they are needed to break down food.
- When you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood, which can cause a heat emergency.
