CFTR modulator therapies. CFTR cystic fibrosis transmembrane regulator is the protein that is not formed correctly in people with CF. There are new CFTR modulator therapies that are designed to correct the function of the defective protein made by the CF gene. These proteins are only appropriate for certain people with CF. They only help people with certain genetic abnormalities. Chest physical therapy. This helps loosen and clear lung secretions.
Medications, vaccinations, enzymes, and airway clearance techniques only play one part in keeping someone with CF as healthy as possible. Lifestyle also matters, with nutrition, exercise, sleep habits, stress management, and organizational ability all playing equally important roles.
It varies by individual, but the average amount of time an adult with CF spends on their treatments medications, enzymes, and airway clearance is minutes per day, regardless of the severity of their disease.
Caregivers of children with CF report 74 minutes per day spent on treatment tasks. To maintain health, chunks of time also should be set aside daily for exercise, nutritious meals and snacks, and adequate sleep. These activities must be built into a day filled with school, work, hobbies, taking care of family, and at least quarterly visits to the CF care center.
The medications needed to manage cystic fibrosis are wide-ranging and vary dramatically from person to person. Some people with a mild form of the disease may only need the occasional antibiotic to fight off a lung or sinus infection and daily pancreatic enzymes see below. Those with more advanced cases may need frequent to daily doses of inhaled and oral antibiotics , bronchodilators , mucus thinners mucolytics , and CFTR modulators.
Additional medications are often needed to treat other conditions that often coincide with cystic fibrosis, such as acid reflux, CF-related diabetes, and liver disease.
Exacerbations , which are a very serious lung complication, usually require IV antibiotics, along with extra sessions of airway clearance and supplemental oxygen and nutrition. This is because most people with CF have a condition known as pancreatic insufficiency. Enzymes come in capsule form as soon as the person with CF gets old enough to swallow pills. Enzyme capsules are opened and sprinkled onto food for babies and young children too small to swallow the pills.
Someone with CF may swallow as many as 20 pills a day before and during meals to help them absorb nutrition. People with cystic fibrosis may need to take different medicines to treat and prevent lung problems. These may be swallowed, inhaled or injected. It's also important that people with cystic fibrosis are up-to-date with all routine vaccinations and have the flu jab each year once they're old enough.
Any kind of physical activity, like running, swimming or football, can help clear mucus from the lungs and improve physical strength and overall health. The Cystic Fibrosis Trust has more information on airway clearance techniques and physiotherapy.
Eating well is important for people with cystic fibrosis because the mucus can make it difficult to digest food and absorb nutrients. A dietitian will advise on how to take in extra calories and nutrients to avoid malnutrition. The type of defect is associated with the severity of cystic fibrosis. The damaged gene is passed on to the child from their parents. In order to have cystic fibrosis, a child must inherit one copy of the gene from each parent. Cystic fibrosis is most common among people of Northern European descent.
The diagnosis of CF requires clinical symptoms consistent with CF in at least one organ system and evidence of CFTR dysfunction usually based on an abnormal sweat chloride test or the presence of mutations in the CFTR gene. The immunoreactive trypsinogen IRT test is a standard newborn screening test that checks for abnormal levels of the protein called IRT in the blood. A high level of IRT may be a sign of cystic fibrosis. However, further testing is required to confirm the diagnosis.
The sweat chloride test is the most commonly used test for diagnosing cystic fibrosis. It checks for increased levels of salt in the sweat. The test is performed by using a chemical that makes the skin sweat when triggered by a weak electric current. Sweat is collected on a pad or paper and then analyzed. A diagnosis of cystic fibrosis is made if the sweat is saltier than normal.
During a sputum test, the doctor takes a sample of mucus. The sample can confirm the presence of a lung infection. It can also show the types of germs that are present and determine which antibiotics work best to treat them. A chest X-ray is useful in revealing swelling in the lungs due to blockages in the respiratory passageways.
A CT scan creates detailed images of the body by using a combination of X-rays taken from many different directions. These images allows your doctor to view internal structures, such as the liver and pancreas, making it easier to assess the extent of organ damage caused by cystic fibrosis.
Pulmonary function tests PFTs determine whether your lungs are working properly.
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