Cystic fibrosis is a genetic disease that affects the lungs and digestive system. The body produces thick, sticky mucus that can obstruct the lungs and prevent the pancreas from functioning correctly. Its name, cystic fibrosis, is derived from the characteristic fibrous growths (cysts) in the pancreas and other organs.

In addition, it is an autosomal recessive disease, which means that the patient must have two copies of the abnormal gene before symptoms develop. Children who inherit one abnormal gene from their parents are not affected. However, they can still pass the gene on to their children.

Symptoms

1. Frequent Lung Infection

The mucus that is produced in cystic fibrosis causes frequent lung infections. The mucus blocks the airways in the lungs, which makes it hard to breathe. The mucus also gives bacteria and viruses a place to live. 

2. Salt Loss

The thick mucus you expel also stops the sweat glands in the skin from working. This leads to a loss of salt (sodium), which the body needs to help maintain the right balance of water in the body.

3. Abdominal Distension

The sticky mucus is also hard to get rid of, which builds up in the digestive system. This causes the stomach and intestines to work overtime and makes it harder for food to move through the digestive tract.

4. Dehydration

Dehydration is caused by salt loss and occurs when the body loses water faster than it can be replaced.

Diagnosis

The two most often used tests to identify cystic fibrosis (CF) are a sweat test that analyzes the chloride content of sweat and a genetic test that looks for chromosomal abnormalities associated with the disease. Due to the severity of cystic fibrosis and the critical nature of early therapy, neonates are routinely checked. 

While this approach is used for most diagnoses, some are not confirmed until later in life or during childhood.

Common Complications

1. Lung Damage

Lung damage in cystic fibrosis is caused when mucus blocks the tiny airways in the lungs and becomes filled with mucus. These airways become damaged, making it hard for the body to get oxygen.

2. Respiratory Failure

Lung damage can lead to pulmonary fibrosis, which is irreversible and fatal. Once the condition has progressed, the only treatment is a lung transplant.

Dietary Management

1. Low-Salt Diet

People with cystic fibrosis should reduce their salt intake as much as possible. This means they should not use salt at the table or in cooking, and they should not add salt to prepared foods.

2. Low-Fat Diet

A low-fat diet is necessary to prevent malnutrition and loss of body fat, making it harder for the body to produce energy. A healthy diet contains enough calories and nutrients to meet the needs of a person’s body.

3. High-Calcium Diet

Calcium is essential to the growth and maintenance of bones. Many experts recommend that people with cystic fibrosis have a diet high in calcium and vitamin D. This might help prevent bone loss (osteoporosis).

Conclusion

Cystic fibrosis is a rare genetic disorder that can be fatal. People with cystic fibrosis pass a CFTR gene protein to the next generation of children. The protein is responsible for the movement of salt and water in the lung and digestive system.

Additionally, it causes the body to make thick mucus, which clogs the lungs, leading to various health problems. The disease results from a faulty gene that makes a protein necessary for transporting chloride ions.

If you need a diagnostic service from the best physicians, Garden State Group is here at your service! Our board-certified physicians and specialists practice multidisciplinary integrative and functional medicine focusing on prevention, management, and education. We offer services in North Bergen and Secaucus, New Jersey. Contact us now!

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