You can overcome many of the obstacles posed by this hereditary respiratory ailment and have a healthier life with supportive therapy, which includes drugs to help heal infections and open airways.
A dangerous hereditary condition that damages the lungs and other organs is called cystic fibrosis. It involves the mutation of a mucous production-related gene. The lungs, pancreas, and other organs produce a thicker, stickier kind of mucus rather than a thin, slick kind.
One of the primary objectives of treatment is airway clearance, which is achieved by using a mechanical device that resembles a vest or physical therapy to break up mucus in the lungs.
Another crucial component of care is supportive therapy, which includes drugs for a variety of conditions, including infections, mucus thinning, and enzyme replacement.
What is cystic fibrosis supportive therapy?
The phrase "supportive therapy" refers to the range of treatments offered for cystic fibrosis. The particular forms of support therapy you might require will depend on your general health and the severity of your cystic fibrosis.
You could require less support therapy than someone who is more sedentary and has other medical issues if you can exercise frequently and take care of other aspects of your health.
Mucus accumulation in the pancreas, for instance, prevents the pancreas from releasing enzymes that are necessary for proper digestion and nutrient absorption from food. Up to 80–90% of those with cystic fibrosis may need pancreatic enzyme therapy, according to a 2020 estimate.
In addition, supportive therapy may involve taking specific vitamin and mineral supplements, specifically:
- calcium
- sodium chloride (salt)
- zinc
- iron
A range of drugs are frequently used in supportive therapy for cystic fibrosis. Among the three most popular categories of drugs are:
- antimicrobials to treat bacterial illnesses
- bronchodilators to aid in mucus clearance and airway relaxation
- corticosteroids to stop respiratory inflammatory responses
If issues related to cystic fibrosis arise in the liver, pancreas, or other parts of the body, medication therapy could also be required.
Cystic fibrosis care team
A care team for someone with cystic fibrosis may consist of different kinds of doctors, such as:
- gastroenterologists
- OB-GYNs
- urologists
- endocrinologists
- otolaryngologists (ENTs)
Treatment and supportive care may be provided by additional medical specialists, including:
- pharmacists
- respiratory therapists
- dietitians
- genetic counsellors
- nurses
- social workers
Why do you need cystic fibrosis supportive therapy?
Since there is no treatment for cystic fibrosis, maintaining the condition requires constant care. Every type of supportive therapy plays a vital part in maintaining your well-being.
To aid in the digestion of carbohydrates, lipids, and sugars and to guard against malnutrition, pancreatic enzyme replacement treatment may be required. A lot of people who have cystic fibrosis also lack certain vitamins and minerals, so taking supplements is essential to staying healthy.
Although they aren't always necessary, antibiotics can play a vital role in treating or preventing bacterial infections that frequently develop in the lungs of cystic fibrosis patients.
The accumulation of mucus caused by triggers for cystic fibrosis can induce wheezing, coughing, and dyspnea. Reducing mucus and opening up airways are two more important goals of supportive treatment.
Although clearing the airways by shaking off mucus is crucial, supportive therapy can help reduce bothersome symptoms even more.
What happens during cystic fibrosis supportive therapy?
Oral administration is used for digestive enzymes and vitamin/mineral supplements. This also applies to the majority of antibiotics, while some require intravenous administration, requiring a visit to a clinic or doctor's office.
You might need a nebulizer to inhale some corticosteroids or bronchodilators. It's a mechanical apparatus that turns liquid drugs into an inhaled mist.
A flexible plastic hose is connected to the nebulizer to transfer the mist. To prevent the medication from escaping, there is a facemask at the other end of the hose that goes over your mouth and nose. All you have to do is inhale the full dosage of medication while wearing the facemask, then exhale.
How do you prepare for cystic fibrosis supportive therapy?
When, how often, and how much preparation is required for supportive therapy depends on the particular kind. For example, pancreatic enzyme supplements need to be taken before meals. Discuss the scheduling of additional nutrients and drugs with a medical team.
To help your inhaled therapy become a regular, scheduled part of your day, you might want to use it at roughly the same time every day. Maintaining a routine can also assist in preventing you from taking your medication too early in the day or from skipping doses.
How effective is cystic fibrosis supportive therapy?
According to the Cystic Fibrosis Foundation, individuals with cystic fibrosis are living longer and have higher quality of life. Developments in supportive therapy and other drugs aimed at lowering pathological mucus accumulation and other aspects of the illness have allowed for these gains.
Takeaway
Taking care of someone with cystic fibrosis requires daily attention to bodily needs, such as releasing thick mucus, as well as supportive therapy involving vitamins, drugs, and supplements. To reduce the strain on the lungs, nebulizers are frequently used to breathe in prescription drugs.
People with cystic fibrosis and their families must prioritise healthy habits including a balanced diet, regular exercise, restful sleep, and stress reduction to maximise the effectiveness of these medicines.
Having friends and a caring medical team at your side can help you cope with the various obstacles that come with having cystic fibrosis.
FAQs
What is the latest treatment for cystic fibrosis?
Trikafta (elexacaftor/ivacaftor/tezacaftor)
Can cystic fibrosis be fully cured?
There's no cure for cystic fibrosis
Is cystic fibrosis a lifetime disease?
The average life span for people with CF who live to adulthood is about 44 years
Can a child with cystic fibrosis have a normal life?
Most people with cystic fibrosis live a normal daily life
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