What is Cystic Fibrosis?
Cystic Fibrosis is a progressive, genetic disease of the secretory glands. The glands making mucus and sweat are a part of these secretory glands. The disease is known to affect cells producing sweat, mucus, and digestive enzymes. Ordinarily, these cells are very thin and slippery designed to ensure proper working of the body system. But, when a person has Cystic Fibrosis, the defective genes turn it thick and glue like. This resultantly leads to the blocking of tubes and ducts in the body. After some time, the air tracts are filled up with mucus, which renders breathing a difficult task. Not only this but also this mucus becomes a breeding ground for various germs. This, in turn, leads to infections, cysts, and fibrosis. That is the reason this disease is termed as cystic fibrosis.
Causes of Cystic Fibrosis
Well, the disease is caused by a defect in cystic fibrosis transmembrane conductance regulator gene or more simply, CFTR gene. This gene is responsible for making a protein known as CFTR. The main task of CFTR is controlling the movement of salt and water in and out of the cells of the body. In people suffering from Cystic Fibrosis, this gene makes proteins that do not work efficiently. This inefficient working might result in thick, sticky mucus in the respiratory, digestive and reproductive system.
This disease is considered genetic as:
Every person inherits two copies of the CFTR gene, one from each parent. The child who inherits both the faulty genes is said to have cystic fibrosis. If only one faulty gene is inherited then the child does not suffer from cystic fibrosis, but he acquires CF carriers in his body. These CF carriers do not lead to the disease in that person, but there are chances that the child born to these persons can have this disease.
Symptoms of Cystic Fibrosis
The symptoms vary according to the age of the patient and severity of Cystic Fibrosis. Generally, the infant does not show much symptoms except his skin becomes salty. The parents feel this when they kiss the baby. However, the prominent signs of the disease start reflecting as the child begins to attain puberty.
The various symptoms include persistent coughing at times with phlegm, frequent lung infections that might cause pneumonia or bronchitis, wheezing, shortness of breath, improper height growth and weight gain according to the age, male infertility, nasal polyps etc. The disease mainly affects the lungs, pancreas, liver and other glandular organs.
Diagnosis of Cystic Fibrosis
The doctors generally diagnose cystic fibrosis through the help of various tests. These are:
- Newborn Screening-Usually, the newborns undergo screening using a genetic test or blood test. The genetic test for examining the presence of faulty CFTR genes in the body, and blood test for checking the working of the pancreas of the newborn.
- Sweat test– After the first test; the diagnosis is confirmed using the sweat test. As the name suggests, sweat tests calculate the amount of salt in sweat. This test is done twice. The presence of a high level of salt means the occurrence of cystic fibrosis.
- Chest x-ray test– This test works on the principle of capturing structures of the chest by taking pictures. This can easily determine inflamed or scarred lungs, trapped air etc.
- Lung function test– Lung function test involves the measuring of the amount of air that can be breathed in and out, the rate of this breathing and the efficiency with which the lungs deliver oxygen to the blood.
- Sputum culture test– The doctor collects the sample of sputum to examine the growth of bacteria.
There are many other tests too, that involves the diagnosis of cystic fibrosis.
Treatment of Cystic Fibrosis
Well, the disease is not curable until date but its treatment might actually reduce the effects of the disease. In fact, the symptoms of the disease vary from person to person and so the treatment. Let us discuss the various methods of treatment in detail.
- Airway clearance– This is the technique adopted to get rid of the thick mucus which is collected in the lungs and that leads to the various respiratory problems. These techniques might include drainage and percussion. It involves clapping the patient’s chest and back to remove the mucus from the lungs.
- Inhaled medicines– Inhaled medicines are used in liquid form to be inhaled through a nebulizer. These medicines contain in them various antibiotics to protect the body against various infections and diseases and keep the passage clean.
- Implanted devices– These devices ensure a healthy pathway to the bloodstream. This is helpful in facilitating an efficient administration of drugs.
- Home remedies– Pieces of evidence suggest that the persons having cystic fibrosis require a high amount of calorie. A balanced diet can solve the problem largely. Moreover, proper exercising is another significant tool to combat the disease.
Well, having a good diet is the best therapy rendered to a human being, especially to a patient. The same is the case with cystic fibrosis. In this connection, let us talk about some essential diet requirements for these patients.
Protein– Protein is one of the most essential nutrients that fulfills the calorie requirement of the patient. These proteins can be provided from foods like meat, eggs, fish, nuts etc.
Iron– The deficiency of iron is the root cause of anemia. Anemia, in turn, leads to fatigue, and the body has reduced strength to deal with infections and diseases. Some of the best sources of iron are cereal, meats, dry fruits, green vegetables etc.
Calcium-Calcium is the nutrient, the deficiency of which leads to weak and brittle bones. Patients suffering from cystic fibrosis are at high risk of developing these diseases. Therefore, it is best to consume dairy products, juice etc. in order to avoid the deficiency of calcium.
Salt– It is obvious that patients having cystic fibrosis lose a significant part of the salt consumed in the form of sweat. Since a large part of the salt is lost, the body is not able to carry out its proper functioning. Thus, in order to overcome this problem, the quantity of salt intake in the body should be enhanced.
Apart from these, calorie intake must be increased. It is to be remembered that the balanced diet is effective only when supplemented with proper exercising and medication.