Cystic Fibrosis and Physiotherapy

What Is Cystic Fibrosis?

Cystic fibrosis is an inherited condition that causes damage to several organs of the body, including the lungs, digestive system, genitourinary system, musculoskeletal system, etc. The cells that make mucus, sweat, and digestive fluids are affected by cystic fibrosis. Normally, these produced fluids are slick and thin. However, a faulty gene in cystic fibrosis patients makes the secretions thick and sticky. The secretions clog up tubes, ducts, and passages (especially in the lungs, pancreas, and intestine) instead of lubricating them. Until quite recently, not much was known about cystic fibrosis. In 1949, Lowe et al. proposed that the autosomal recessive pattern of inheritance of cystic fibrosis must be due to a genetic abnormality.

For white Americans, the prevalence of cystic fibrosis is one case per 3200 people. In comparison, it is one case per 15000 for black Americans, one case per 9200 for Hispanic Americans, and one case per 31000 for Asian Americans. Cystic fibrosis is reported in 40 percent of individuals who are 18 years or older.

What Causes Cystic Fibrosis?

Cystic fibrosis is a hereditary condition due to variations or defects in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. A hereditary condition is genetically transferred from parents to their children. Two CF (cystic fibrosis) genes, one from the mother and one from the father, must be inherited for cystic fibrosis to occur. The result of this faulty gene is that the pancreas, sweat glands, GI (gastrointestinal) tract, and respiratory systems generate an excessively thick and sticky fluid. There are different genetic mutations that are connected to various levels of disease severity.

How Is Cystic Fibrosis Treated Medically?

There is no definitive cure for cystic fibrosis. Most cystic fibrosis patients pass away between the ages of 20 and 30 due to lung failure. However, a thorough treatment plan and early diagnosis can increase a cystic fibrosis patient's chances of survival and quality of life.

Cystic fibrosis is medically managed in the following ways:

• Recurrent respiratory tract infections can be treated with the help of antibiotics.

• Multiple vitamins and supplements.

• Bronchodilators are used to widen the airways and improve respiratory symptoms.

• The use of DNAse (deoxyribonuclease) enzyme replacement treatment thins the mucus and facilitates expectoration.

• Pain killers.

• Children with CF must have the standard childhood immunizations, including those for diphtheria, tetanus, and whooping cough (DTP) and measles, mumps, and rubella (MMR). To help avoid chest infections, a child with CF should also receive vaccinations against the flu and pneumococcus.

How Is Cystic Fibrosis Managed With Physiotherapy?

Although airway clearing remains a crucial component of treatment, physical activity, postural care, and the requirement to address the particular difficulties that arise due to longevity have fundamentally altered the character of physiotherapy in cystic fibrosis. The primary objectives of physiotherapy are to maintain or enhance lung function, prevent secondary problems, and improve quality of life. When necessary, they also assist with musculoskeletal therapy.

What Is Chest Physiotherapy?

An individual with cystic fibrosis requires extensive chest physiotherapy; these include:

• Massage: A vigorous massage can assist in breaking up the thick mucous.

• Postural Drainage: Postural drainage involves gravity-assisted positions that aid in secretion drainage and improve airflow or ventilation to various lung areas.

• Percussion: This method, sometimes called chest clapping, is employed to aid in loosening secretions. This technique uses a cupped hand to firmly and rhythmically clap the chest.

• Shaking and Vibrations: This method involves repeatedly giving the chest a quick, rhythmic squeeze while exhaling to get the secretions moving.

• Regular Monitoring: During physical therapy, it is important to examine and monitor the patient frequently since they could need extra oxygen, particularly if they have severe cystic fibrosis.

What Is Airway Clearance and Why Is It Important?

The main goal of airway clearance is to postpone or stop the development of bronchiectasis (damage to the lung’s airways) and lung damage. Because it helps prevent the buildup of thick mucus and its subsequent obstruction of the lungs, airway clearance is crucial. This reduces lung damage and helps prevent infection.

What Are the Different Types of Airway Clearance Techniques?

Everybody has a different set of lungs; thus, each person will require a different quantity of airway clearance. Each approach has yet to be proven superior to another, which may be because everyone's chests behave considerably differently. Additionally, individuals may prefer different methods at different points in their lives.

Some airway treatments, which concentrate on breathing exercises to clear mucus, do not require special tools. These consist of the following:

• Active Cycle of Breathing Techniques: The Active Cycle of Breathing Techniques involves cycles of deep breathing, puffing, coughing, and relaxed breathing to move mucus.

• Autogenic Drainage: Autogenic Drainage uses controlled breathing at various levels to transfer mucus from tiny to larger airways. While the patient exhales, autogenic drainage uses rapid flow rates, ensuring the airways remain open.

Some methods employ a tool to aid in mucus clearance. Some retain the airways open with positive pressure, while others additionally cause vibrations inside the airways. These consist of the following:

• Positive Expiratory Pressure: To create pressure behind the mucus and move it, the patient uses a mask or mouthpiece and exhales against resistance.

• Oscillating Positive Expiratory Pressure: The patient uses the oscillating positive expiratory pressure technique to exhale against resistance, producing positive respiratory pressure and vibrating the airways to move mucus.

• High-Frequency Chest Wall Oscillation: This technique uses an inflatable vest and an electric air pulse generator to vibrate the chest.

What Are the Benefits of Exercise in Cystic Fibrosis Patients?

Regular physical activity has been shown to have both short and long-term advantages, and it is well-acknowledged that it is vital to the course of physiotherapy. The benefits of exercise in cystic fibrosis patients include:

• Improved fitness.

• Improved endurance.

• Improved clearance of sputum.

• Delays dyspnea (shortness of breath).

• Delays or prevents decreased bone density.

• Improves cellular immune response.

• Improves quality of life in patients.

• Improves mental health and gives confidence.

• According to some research, exercise may even reverse some of the disease-related changes in ion transport, which would reduce mucus buildup and enhance pulmonary function.

Conclusion:

Depending on the symptoms, physiotherapy can be begun very early in the cystic fibrosis treatment process. It covers a wide range of issues, such as airway clearing, to aid in loosening and to remove accumulated mucus, improving posture, and preventing back problems that might impair lung function. Each person will require a distinct kind of physical therapy, which can be tailored to their specific symptoms and requirements.