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Treatments for cystic fibrosis

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When a loved one has cystic fibrosis, it is comforting to know that several treatments exist to make daily life easier and to maintain a state of optimal health. A long time ago, children died at a young age from this illness; today, there are ways to help them live a happy, active life.

When a child is diagnosed with cystic fibrosis (CF), his or her parents are faced with the unknown and are often completely devastated. Thankfully, a team of competent health professionals will be there to guide them through each of the steps that will lead to optimal management of the illness. This teamwork will help the child develop and grow while enjoying a satisfying life.

This health file presents the medical treatments that are available for CF. For more information about cystic fibrosis, see our health file entitled “Cystic fibrosis: how to recognize this little-known illness”.

An overview of cystic fibrosis

CF is a hereditary illness that is most often diagnosed in young children; it is possible, however, for it to manifest itself only in adolescence or young adulthood. It mainly affects the lungs, the digestive system–especially the pancreas, liver and intestines–and the sweat glands. In people who are affected by this disorder, a genetic anomaly disrupts the proper functioning of certain glands, which then produce abnormally thick, sticky mucus (mucus is a usually fluid substance that coats and moistens the inside of certain ducts in the body; the production of mucus is a normal occurrence in human beings).

Excessive secretion of mucus leads to various health problems, such as:

  • respiratory issues;
  • frequent respiratory infections;
  • persisting phlegmy cough;
  • inability to absorb certain nutrients, such as vitamins;
  • reduced secretion of certain enzymes in the pancreas, which are needed for digestion;
  • digestive issues;
  • production of abnormally salty sweat.

Some of these problems can lead to complications, such as:

  • irreversible lung damage;
  • poor growth and slower weight gain;
  • dietary deficiencies;
  • diabetes;
  • cirrhosis of the liver;
  • infertility;
  • death.

Improved treatments have managed to reduce the consequences of problems caused by malnutrition, which means that most CF-related deaths are now attributable to lung problems.

Treatment goals

To this day, there is no cure for CF; treatment options are only for symptoms. They require vigilance and a long-term commitment from the patient (if he or she is old enough to actively take part in the treatment), his or her family and care team. These treatments aim at, among other things:

  • managing symptoms;
  • ensuring adequate nutritional intake;
  • preventing or eliminating infections;
  • reducing hospitalizations;
  • delaying complications;
  • improving quality and expectancy of life.

For optimal management of the illness, patients must seek the expertise of a multidisciplinary team of professionals: medical specialists, nurses, nutritionists, dieticians, pharmacists, physiotherapists, inhalation therapists, etc. Regular medical visits are essential.

Here is an overview of some of the most common treatment options:

Antibiotic therapy

Antibiotic therapy is commonly used for individuals who suffer from CF. Antibiotics can be administered orally, intravenously or in aerosol form. This treatment aims to prevent or eliminate bacterial respiratory infections, and partly slows down the progression of lung damage. For optimal treatment, it is important to follow the doctor and pharmacist’s recommendations.

Respiratory therapy

To be completely effective, inhaled medications must reach the bronchial tubes or the lungs. They are administered with a specially designed device (vaporizer or aerosol). Two main classes of inhaled medications are prescribed to CF patients: mucolytic drugs, which liquefy respiratory secretions to facilitate their elimination, and bronchodilator drugs, which expand the airways to make breathing easier and reduce respiratory symptoms.

Supplemental vitamins and nutrients

In people who have CF, the digestive system does not properly absorb certain essential nutrients, such as fat-soluble vitamins (vitamins A, D, E and K). Diet only is therefore not enough to get the necessary daily fat-soluble vitamins, and taking high doses of supplements is often suggested. The doctor might also determine that other nutrients (calcium, for example) should be taken each day in supplement form.

Supplemental digestive enzymes

One of the characteristics of CF is an insufficient secretion of certain pancreatic enzymes that are essential for proper digestion. This phenomenon prevents the absorption of fatty matter, protein and carbohydrates by the body. To fix this problem, enzyme supplement are taken with each meal and snack.

Special diet

CF patients need a diet that is adapted to their condition. Despite having a normal appetite, they lose weight or have a hard time gaining weight, so their diet should be high in calories and various nutrients, such as protein. In addition, a high-sodium diet is also often required to replace the high amounts of salt lost in sweat. To ensure they have the best possible diet, it is essential for CF patients to be closely followed by a nutrition specialist.

Vaccination

Because they are highly susceptible to infections, individuals affected by CF must make vaccination a top priority. First, they need to make sure that their routine vaccination schedule is up to date. Every year, they need to take the flu vaccine, as should their loved ones. Complementary vaccines are also recommended, such as the pneumococcus shot. See a competent health professional to learn more about vaccination.

Chest physiotherapy

The goal of this important component of treatment is to improve respiratory functions. It promotes the elimination of excess secretions through coughing and thus helps restore the airways’ permeability. The typical depiction of chest physiotherapy is percussion (clapping the patient’s chest or back to dislodge mucus). A multitude of effective exercises and techniques can be taught to patients, depending on their age and state. Practicing them regularly improves their efficiency.

Lung transplant

Considered a last-resort solution, a lung transplant consists of replacing sick lungs with healthy ones, taken from a deceased person. Sometimes, the transplant must also include a new heart with the lungs, if the heart is too damaged. A lung transplant is literally a fresh of breath air for the patient, allowing him or her to go back to a more active and comfortable life. This intervention is not without risk, however, which is why it is only for the most serious cases, i.e. adults who meet very specific criteria.

Although the life expectancy of people who suffer from CF is diminished, there is still reason to hope. Many years of medical research have had a positive outcome; indeed, by better understanding the illness, researchers have been able to produce medications and medical interventions that significantly improve the lives of CF sufferers. Let’s hope that the world’s great minds will continue to dedicate their talents and efforts to research so that one day, cystic fibrosis will become a curable illness.

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