Treatment for cystic fibrosis is a combination of medicine, diet, oxygen therapy and respiratory physiotherapy, to alleviate symptoms and prevent complications, such as lung infections or digestive problems.
Cystic fibrosis is a genetic disease that alters the function of the glands, resulting in more viscous and thick secretions, mainly in the lungs and digestive tract, causing symptoms such as difficulty breathing or bulky, greasy and foul-smelling stools. See the main symptoms of cystic fibrosis.
The treatment of cystic fibrosis is carried out by a multidisciplinary team, including a pulmonologist, gastroenterologist, nutritionist and physiotherapist, and should begin as early as possible to prevent the disease from worsening quickly, alleviate symptoms and improve quality of life.
13 treatments for cystic fibrosis
The main treatments for cystic fibrosis are:
1. Pancreatic enzymes
Pancreatic enzymes, such as lipase, protease and amylase, may be recommended by the gastroenterologist to help with the digestion of food, since in cystic fibrosis the secretions produced by the pancreas become thicker, making their elimination difficult and may result in fatty stools or obstruction of the pancreatic ducts.
Additionally, the use of acid production reducers in the stomach may be indicated to help pancreatic enzymes function better and improve food digestion.
Using pancreatic enzymes also helps prevent pancreatitis and pancreatic insufficiency.
2. Food supplements
Food supplements, such as vitamins A, D, E and K, may be recommended by the gastroenterologist to prevent or treat deficiency of these vitamins, which may have their absorption impaired in cystic fibrosis.
In addition, the doctor may recommend other dietary supplements, such as folic acid, biotin, niacin or B vitamins, according to nutritional needs.
3. Laxatives
Emollient laxatives, such as docusate, may be indicated for cystic fibrosis to prevent constipation, as it leaves the stool more hydrated, facilitating its elimination.
Furthermore, treatment with emollient laxatives also helps prevent intestinal obstruction. See other complications of cystic fibrosis.
4. Bronchodilators
Inhaled bronchodilators, such as salbutamol or ipratropium bromide, may be recommended by the pulmonologist to relax the muscles around the bronchi and help keep the airways open, relieving shortness of breath or difficulty breathing.
Read too: Bronchodilators: what they are, what they are for (and how to use them)
5. Mucolytics
Mucolytic medications, such as acetylcysteine, may be recommended by a pulmonologist for cystic fibrosis, as they make the phlegm more liquid, facilitating its elimination, and preventing airway obstruction.
Furthermore, another mucolytic that may be recommended is inhaled dornase alfa, which makes phlegm less thick and is recommended together with respiratory physiotherapy to reduce the frequency of respiratory infections and improve lung function.
The doctor may also recommend inhaling hypertonic saline to help promote the release of respiratory secretions.
6. Corticosteroids
Corticosteroids, such as prednisone or methylprednisolone, may also be recommended by the pulmonologist to reduce swelling in the airways and prevent obstruction.
7. Antibiotics
Antibiotics may be prescribed by a pulmonologist for the treatment or prevention of respiratory infections or acute pulmonary exacerbations of cystic fibrosis.
Some antibiotics that may be recommended by the doctor are ciprofloxacin, sulfamethoxazole + trimethoprim, azithromycin, chloramphenicol or gentamicin, for example.
8. Gene therapy
Gene therapy with CFTR modulating drugs (from English, Cystic Fibrosis Transmembrane Conductance Regulator) or cystic fibrosis transmembrane conductance regulators, may be prescribed by the doctor to help regulate defective genes specific to cystic fibrosis.
These drugs, such as ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor, or elexacaftor/tezacaftor/ivacaftor, improved the production, intracellular processing, or function of the CFTR protein, correcting the changes caused by the altered cystic fibrosis gene.
9. Oxygen therapy
Oxygen therapy is a treatment for pulmonary fibrosis that increases oxygen levels in the blood, reduces respiratory effort, helps unblock the airways and prevents complications such as pulmonary hypertension.
This treatment can be recommended by a pulmonologist and carried out using a nasal catheter and pure oxygen, for a few hours a day or continuously, depending on each case.
Read too: Oxygen therapy: what it is, types, what it is for and care
In addition, non-invasive ventilation may also be indicated, such as the use of an oxygen mask, called BiPAP, and in cases of risk of respiratory failure, intubation with mechanical ventilation may be recommended. Understand how non-invasive ventilation and intubation works.
10. Respiratory physiotherapy
Respiratory physiotherapy for cystic fibrosis helps to complement treatment with bronchodilators, mucolytics and corticosteroids, and is carried out by the physiotherapist with breathing exercises.
These exercises help to release secretions, improve oxygenation throughout the body, unblock the airways and make breathing easier.
Read too: Respiratory physiotherapy: what it is for and how to do it (with exercises)
Respiratory physiotherapy also helps to alleviate symptoms and improve quality of life, allowing you to carry out activities of daily living with greater ease.
Watch the following video with physiotherapist Mirca Orcanha with breathing exercise tips:
11. Diet for cystic fibrosis
Diet is very important for the treatment of cystic fibrosis as thick mucus and poor functioning of the pancreas can make it difficult to digest food and absorb nutrients.
Therefore, the nutritionist may recommend a diet rich in calories, but low in inflammatory substances, to avoid malnutrition and worsening of the disease, in addition to recommending vitamin and mineral supplements to help with digestion.
In some cases, especially when the person has severe malnutrition, the nutritionist may recommend enteral nutrition, through a nasogastric tube that is inserted into the nose and reaches the stomach, or gastrostomy or jejunostomy, for example.
Read too: Enteral diet: what it is for, types and how to feed
12. Bowel surgery
Bowel surgery for cystic fibrosis is indicated in cases of intestinal obstruction or intussusception, which occurs when one segment of the intestine slides over the other, blocking the intestine and intestinal blood flow.
Read too: Intussusception: what it is, symptoms, causes and treatment
Furthermore, other types of surgeries may be indicated for cystic fibrosis to treat complications, such as pneumothorax, nasal polyps or chronic sinusitis, for example.
13. Lung transplant
Lung transplantation may be indicated in cases of pulmonary cystic fibrosis when it causes severe respiratory problems, as it helps the person breathe more easily and improves quality and life expectancy. See how a lung transplant is performed.
In addition to lung transplantation, liver transplantation may also be indicated when the person has cirrhosis, or other organs, such as the pancreas, for example.
Read too: Transplant: when it is indicated, how it is done, recovery (and other questions)
