People with CF may take antibiotics only when needed, or all the time. Doses are often higher than normal. Inhaled medicines to help open the airways. Other medicines that are given by a breathing treatment to thin mucus and make it easier to cough up are DNAse enzyme therapy and highly concentrated salt solutions hypertonic saline.
Flu vaccine and pneumococcal polysaccharide vaccine PPV yearly ask your health care provider. Lung transplant is an option in some cases. Oxygen therapy may be needed as lung disease gets worse. These methods include: Activity or exercise that causes you to breathe deeply Devices that are used during the day to help clear the airways of too much mucus Manual chest percussion or chest physiotherapy , in which a family member or a therapist lightly claps the person's chest, back, and area under the arms Treatment for bowel and nutritional problems may include: A special diet high in protein and calories for older children and adults Pancreatic enzymes to help absorb fats and protein, which are taken with every meal Vitamin supplements, especially vitamins A, D, E, and K Your provider can advise other treatments if you have very hard stools Ivacaftor, lumacaftor, tezacaftor, and elexacaftor are medicines that treat certain types of CF.
They improve the function of one of the defective genes that causes CF. As a result, there is less buildup of thick mucus in the lungs. Other CF symptoms are improved as well. Care and monitoring at home should include: Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew.
Giving plenty of fluids, especially to infants and children in hot weather, when there is diarrhea or loose stools, or during extra physical activity. Exercising 2 or 3 times each week. Swimming, jogging, and cycling are good options. Clearing or bringing up mucus or secretions from the airways. This must be done 1 to 4 times each day.
Patients, families, and caregivers must learn about doing chest percussion and postural drainage to help keep the airways clear. No contact with other people with CF is recommended as they can exchange infections does not apply to family members. Outlook Prognosis. Death is most often caused by lung complications. Possible Complications.
By Editorial Team. While there is no cure yet for cystic fibrosis CF , people with CF are living longer, healthier lives than ever before. In fact, babies born with CF today are expected to live into their mids and beyond. Life expectancy has improved so dramatically that there are now more adults with cystic fibrosis than children.
The Foundation began a patient registry in the s that tracks the care each patient with CF receives. This data is then used to create improve the quality of care and drive related research and drug discovery. Prior to the s, children with the most common and most serious forms of cystic fibrosis rarely lived past age 5. In the s and s pancreatic enzymes , airway clearance techniques , and antistaphylococcal antibiotics were introduced as treatments and CF life expectancy began to increase.
In the s, average life expectancy grew to age 15 as antipseudomonal antibiotics were added to the treatment arsenal. A person with cystic fibrosis is born with the condition. It's not possible to "catch" cystic fibrosis from someone else who has it.
To be born with cystic fibrosis, a child has to inherit a copy of the faulty gene from both of their parents. This can happen if the parents are "carriers" of the faulty gene, which means they don't have cystic fibrosis themselves. Cystic fibrosis tends to get worse over time and can be fatal if it leads to a serious infection or the lungs stop working properly. Currently, about half of people with cystic fibrosis will live past the age of Children born with the condition nowadays are likely to live longer than this.
Support is available to help people with cystic fibrosis live as independently as they can and have the best possible quality of life. If you or your child has cystic fibrosis, your clinical team will ask you if you consent to being on the UK Cystic Fibrosis Registry. This is a secure anonymous registry sponsored by the Cystic Fibrosis Trust that records health information on people with cystic fibrosis. Each person with cystic fibrosis is different, so the severity of the disease will vary from person to person.
Patients are advised to stay as active as possible to improve lung function and overall health. Because cystic fibrosis affects the pancreas and its ability to produce digestive enzymes, many people with CF will need to eat extra calories in order to stay nourished. Find out more about living with cystic fibrosis. Cleanliness is also an issue for those living with cystic fibrosis. In order to avoid infections, patients must be vigilant about hand-washing and wary of shared items and surfaces to avoid germs.
As patients age, they are more likely to experience complications of the disease. Lower lung function can often lead to infections which may require hospital treatment. Damage to the airways can cause other lung diseases such as bronchiectasis.
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