Familiarity with the disease and its problems
What is CF?
Cystic fibrosis, or CF, is an inherited genetic disease that mainly affects the respiratory and digestive systems. Genetic defects in these patients cause the body's secretions, including those in the airways and intestines, to become thick and sticky instead of thin and slippery.
Following these discharge problems, two unfortunate events occur:
- Dust and germs that enter the lungs are not well cleaned and cause chronic, prolonged and sometimes dangerous infections in the lungs.
- The outlet of pancreatic secretions is closed and the foods that the child eats are not well digested and absorbed.
In the past, many children with cystic fibrosis died in the early years of life, but today, with countless advances in the care and treatment of these patients, not only has their life expectancy increased and many of them have aged. They reach adulthood, but the quality of life of these patients has also improved significantly.
Patients with cystic fibrosis can have a variety of symptoms, such as:
- Salty skin due to high salt in their sweat.
- Sputum and refractory cough.
- Recurrent lung infections.
- Wheezing and shortness of breath.
- Poor weight gain despite excellent appetite.
- Excretion of greasy, bulky, smelly stools many times.
Definitive diagnosis of CF is usually confirmed by sweat testing and genetic testing.
- Sweat test: Cystic fibrosis is usually determined by calculating the amount of salt in a person's sweat. Sweat glands are one of the types of endocrine glands that are disrupted during CF. Sweat is normal in these patients, but it has more salt and is saltier than normal people.
The sweat test is a simple, inexpensive, painless and accurate test. There are no needles in this test. The test is performed by first placing a colorless, odorless chemical and a thin electrode that conducts very weak currents on a small area of the hand or foot. These two stimulate sweating in the desired area. Then, to collect sweat from the skin of that area, a special gas pad or a piece of paper is placed on the skin and the desired area is wrapped in plastic for 30 minutes to sweat. After 30 minutes, the obtained sweat sample is sent to the laboratory to measure the amount of salt in it.
The baby should be sweaty enough to trust the test result. Babies born on time must be at least two weeks old before they can be tested for sweat. If the child does not sweat enough the first time, the test should be repeated. A cold or mild illness in a child is not a barrier to testing. There is no need for any special activity or nutrition restrictions for the child before the test. The amount and timing of breastfeeding as well as the medication regimen can be continued as usual; Just keep in mind that the cream or lotion should not be rubbed on the baby's skin for 24 hours before the test.
- Genetic testing: This test is performed on a blood sample taken from a patient and is one of the most definitive methods of diagnosing the disease. This test helps to identify the infected fetus during pregnancy (if the child is already in the family) as well as to identify the carriers in the patient's family. Genetic testing is mainly used when sweat test results are reported inconclusively.
Treatment of the disease: Ways to treat cystic fibrosis have made significant progress compared to previous years. Today, we are able to use new methods of physiotherapy to remove concentrated secretions from the airways of these patients and minimize inflammation in their lungs with the help of inhaled antibiotics. Replace pancreatic enzymes and with the help of dietary supplements and improve nutrition, bring growth and maturity to normal in these patients and ultimately improve their quality of life and longevity.
The treatment of cystic fibrosis mainly serves three main purposes:
- Replacement of pancreatic enzymes
- Cleans the airways of concentrated secretions
- Improve nutrition
Pancreatic enzyme replacement: The main treatment for pancreatic enzyme replacement is the "crown" capsule, which contains pancreatic enzymes (amylase and lipase). This capsule contains granules, which are counted according to the age and weight of the child, the number of granules and are given to him with each meal. In older children whose dose is one full capsule or more, they can swallow the capsule.
There is no standard way to replace pancreatic enzymes. Suitable dose is to improve steatorrhea and abdominal pain as well as reduce the frequency and volume of stool. In cases such as consumption of high-fat food, frequent defecation and loose, bulky, pale and oily stools, higher doses of alternative enzymes may be needed; However, stool shape and defecation pattern should not be expected to return to normal in all patients.
Airway clearance from thick discharge: Airway clearance in patients with cystic fibrosis is a general and very necessary principle. This is where the main prevention of lung infections, followed by hospitalization and ultimately the quality of life and general condition of the child, begins. Daily physiotherapy with nebulizers and inhalers are used to clear the airways and dilute the secretions. It is important to note that cleansing should be done every day, not just when the child is coughing or shortness of breath.
Antibiotics are very important as an important treatment in preventing the onset and progression of lung infections and can be used in three ways: oral, injectable and inhaled according to the patient's needs.
Improved nutrition: Patients with cystic fibrosis suffer from indigestion and malabsorption of nutrients due to obstruction in the outlets of pancreatic enzymes, and despite their high appetite, their growth curve is often in the lower percentiles of the growth chart. Nutritionists design a unique diet for each patient according to age, sex, disease progression and many other areas. The main point about the nutrition of these patients and their need for high-fat and high-calorie foods to compensate for the dysfunction of pancreatic enzymes as much as possible. Dietary supplements such as fat-soluble vitamins and minerals are also included in the daily diet of these patients.