Cystic fibrosis

Treatment

Is there treatment for cystic fibrosis?

Although there is no cure for cystic fibrosis (CF), there are FDA approved treatments for different health problems seen in cystic fibrosis. Some treatments attempt to loosen up the mucus from the lungs and thus decrease the frequency of lung infections. Treatment is broken up into medications, respiratory therapies, nutritional support, and exercise. Medications for cystic fibrosis include constant antibiotics, which can prevent and reduce the frequency of lung infections. Airway clearance or respiratory therapies are aimed at loosening up the thick mucus from the lungs. Typically, high frequency chest wall oscillations are provided through a vest. The oscillations shake up and loosen the mucus. Manual coughing techniques can help loosen up the mucus from the lungs also. Additionally, exercise is beneficial in clearing out mucus. Surgeries for sinus complications may be necessary. As the disease becomes worse, lung transplant may be necessary for some individuals with CF. Newer treatments are targeted for individuals with certain gene changes (mutations) and help correct the underlying defect in the CFTR gene.

Pancreatic enzymes before meals can help digestion. These are dosed by a dietician, and taken at specific times before each meal to help digestion. Diet supplements may also be necessary to help people with CF get proper nutrition. A feeding gastric tube (G-tube) may help some people with this. Liver complications may need treatment with medication also. Speak with your care team to find out what specialists you may need to see.

If someone with CF develops cystic fibrosis related diabetes mellitus, treatment would depend upon the severity of the diabetes. An endocrinologist can help manage diabetes mellitus in an individual with CF.

Males who want to have children may need to consult with an infertility specialist to be able to father a child.

Treatment guidelines have been developed and are available on the Cystic Fibrosis Foundation website.

References
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Are there other organ specific treatments for cystic fibrosis?

What is the infectious bacteria Pseudomonas aeruginosa seen in Cystic fibrosis?

What antibiotics are available to treat lung infections in people with Cystic Fibrosis?

Are there other organ specific treatments for cystic fibrosis?

Most of the organ specific treatments of cystic fibrosis are focused on the lungs, the pancreas, and the liver. Medications for lung problems include routine antibiotics, which can prevent and reduce the frequency of lung infections. Airway clearance or respiratory therapies are aimed at loosening up thick mucus from the lungs. Typically, high frequency chest wall oscillations are provided through a vest. The oscillations shake up and loosen the mucus. Manual coughing techniques can help loosen up the mucus from the lungs. Exercise is also beneficial in clearing out mucus. Pancreatic enzymes before meals can aid in digestion. These are dosed by a dietician, and taken at specific times before each meal to ensure improved digestion. Medication is available to treat liver disease also. Your care team can help you find out what specialists you need to see based on the symptoms you are showing.

References
What is the infectious bacteria Pseudomonas aeruginosa seen in Cystic fibrosis?

Pseudomonas aeruginosa or P. aeruginosa is the most common type of bacteria that can cause infections in the lungs and other organs in cystic fibrosis. The P. aeruginosa infections lead to chronic (constant) airway infections that over time make the lungs less able to work. In addition to treatments for cystic fibrosis focused on reducing the thickness and amount of mucus in the airways, preventing blockage of the intestines, and ensuring the proper intake of vitamins and nutrients, early treatment to prevent and clear up infections caused by P. aeruginosa is an important part of treating cystic fibrosis. Different antibiotics may be used to treat lung infections. The antibiotic tobramycin comes in forms that can be inhaled (TOBI®, manufactured by Novartis) and aztreonam for inhalation (Cayston®, manufactured by Gilead) have been approved by the FDA for the treatment of bronchopulmonary (tubes leading to and in the lungs) infections of Pseudomonas aeruginosa (the most common source of chronic lung infection) in individuals with cystic fibrosis. Other companies have or are working on other antibiotics to treat chronic lung infections. For example, as of May 2016, an inhaled fluoroquinolone antibiotic (Quinsair®, manufactured by Raptor) is being studied in the United States for use. This antibiotic is already approved for the management of chronic pulmonary infections due to Pseudomonas aeruginosa in adults with cystic fibrosis in Europe and Canada. Talk to your doctor about whether treatment for this bacteria is needed for you.

References
  • National Organization of Rare Disorders. "Cystic Fibrosis." 2014. Web. NORD - CF
  • Raptor Pharmaceuticals. "MP-376 For Cystic Fibrosis." Web. Raptor Pharmaceuticals
  • Lenney W. "Pseudomonas aeruginosa in cystic fibrosis is potentially serious, and more than merely a marker for disease severity." Paediatr Respir Rev. 2015;16(Suppl 1):35-6.
What antibiotics are available to treat lung infections in people with Cystic Fibrosis?

Although many types of bacteria can cause infections in the lungs and other organs of people with cystic fibrosis, Pseudomonas aeruginosa or P. aeruginosa is the most common type of bacteria that can cause infections. The P. aeruginosa infections lead to chronic (constant) airway infections that over time make the lungs less able to work. In addition to treatments for cystic fibrosis focused on reducing the thickness and amount of mucus in the airways, preventing blockage of the intestines, and ensuring the proper intake of vitamins and nutrients, early treatment to prevent and clear up infections caused by P. aeruginosa is an important part of treating cystic fibrosis. Different antibiotics may be used to treat lung infections. The antibiotic tobramycin comes in forms that can be inhaled (TOBI®, manufactured by Novartis) and aztreonam for inhalation (Cayston®, manufactured by Gilead) that have been approved by the FDA for the treatment of bronchopulmonary (tubes leading to and in the lungs) infections of P. aeruginosa in individuals with cystic fibrosis. Other companies have or are working on other antibiotics to treat chronic lung infections. For example, as of May 2016, an inhaled fluoroquinolone antibiotic (Quinsair®, manufactured by Raptor) is being studied in the United States for use in children. This antibiotic is already approved for the management of chronic pulmonary infections due to Pseudomonas aeruginosa in adults with cystic fibrosis in Europe and Canada. Oral and intravenous antibiotics are also used to treat infection in cystic fibrosis. For more information about how to best treat lung infections in CF, speak to your doctor.

References
  • National Organization of Rare Disorders. "Cystic Fibrosis." 2014. Web. NORD - CF
  • Raptor Pharmaceuticals. "MP-376 For Cystic Fibrosis." Web. Raptor Pharmaceuticals
  • Lenney W. "Pseudomonas aeruginosa in cystic fibrosis is potentially serious, and more than merely a marker for disease severity." Paediatr Respir Rev. 2015;16(Suppl 1):35-6.

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