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Treatment of Cystic Fibrosis

The treatment of cystic fibrosis is different for each patient, according to how severe symptoms are and which organs are affected by the disease. The treatment can be complex, demanding discipline, dedication, and time from the patients and their family members, who are also key participants in the treatment over the years.

The first step to successfully treat cystic fibrosis is to understand the disease, the changes it causes on the body, and the importance of correctly following medical recommendations. A good relationship of the patients and their families with the physicians and other health professionals involved in the treatment of cystic fibrosis is essential. There must be space for conversation and trust so there is adherence to the treatment.

Another key step is keeping in mind that, because of advances in care and treatment, people with cystic fibrosis have been able to live better and longer. This is possible because they are followed-up by a multidisciplinary team involving professionals such as pediatricians, general practitioners, pulmonologists, gastroenterologists, radiologists, geneticists, nutritionists, physical therapists, psychologists, and nurses.

The follow-up of cystic fibrosis treatment is generally done in specialized clinics and care centers for cystic fibrosis. The treatment of cystic fibrosis goes beyond administering medications – it requires, for example, regular physical activity and respiratory physical therapy.

Furthermore, special attention should be given to the diet of the patient with cystic fibrosis. It should be hypercaloric and high-protein to supply the body's high demands for energy and to reduce the deficiencies in the absorption of nutrients and vitamins caused by the disease. This key measure avoids malnutrition and allows a healthy growth from childhood into adult life.

The medications used to treat cystic fibrosis include inhaled medications (bronchodilators, antibiotic solutions or powder, mucoactive agents such as dornase alfa and saline solutions); oral medications (antibiotics, anti-inflammatory drugs, pancreatic enzymes, nutritional supplements, medications to lower stomach acidity and to increase bile fluidity in the liver); and intravenous antibiotics used during hospitalizations.

An active lifestyle including physical activity should be encouraged, and respiratory physical therapy is a key component of the treatment of cystic fibrosis, with the use of respiratory devices and exercises to help remove mucus from the lungs. In more severe cases or at advanced stages, the possibility of lung transplant should be considered.


The importance of pulmonary rehabilitation in the treatment of cystic fibrosis

Pulmonary rehabilitation is a type of physical therapy program that can be recommended for people with cystic fibrosis. It consists of a series of exercises adapted to each patient's abilities and needs and seeking to help them improve control over their breath and have a better quality of life.

Overall, people with cystic fibrosis should do pulmonary rehabilitation daily. It is worth noting that pulmonary rehabilitation does not substitute adherence to drug treatment, since it is an additional therapy.

Diet and cystic fibrosis

Patients with cystic fibrosis need to be extra careful with their diet. The intake of hypercaloric and high-protein foods (that is, foods containing many calories and proteins) is recommended to compensate for the loss of nutrients that are not absorbed in the intestine. Increased fluid intake is also recommended.

To maintain a healthy nutritional status, the patient should ideally eat three meals a day and have additional snacks between meals. The list of recommended foods includes:

  • high-fat: butter, margarine, ice cream, and chocolate;
  • high-sugar: jams, puddings, and sweets in general; 
  • milk and its products: cheeses and yogurts; 
  • high-starch: pasta, rice, and bread;
  • high-protein: fish, eggs, and meat; 
  • high-mineral: fruits and vegetables.