Cystic Fibrosis: How do we treat children?

Cystic Fibrosis (CF) is a genetic disorder which mainly affects the lungs, pancreas and liver. Unfortunately, at present there is no cure for cystic fibrosis (CF), but regular care can help control symptoms, prevent or reduce complications, and improve the quality of life. Children and adults with cystic fibrosis (CF) need to be under regular care of a cystic fibrosis (CF) team usually led by a pediatric pulmonologist along with a physiotherapist, nutritionist and gastroenterologist. Close monitoring and early, aggressive intervention is recommended to slow the progression of CF , which can lead to a longer and a better quality of life.

Broadly the treatments of cystic fibrosis (CF) includes antibiotics to prevent and treat chest infections, medicines to make the mucus in the lungs thinner and easier to cough up, chest physiotherapy to help clear mucus from the lungs and medicines that help the person absorb food better.

Antibiotics to prevent and treat chest infections for children with cystic fibrosis (CF)?
Antibiotics are another mainstay of treatment for children with cystic fibrosis (CF). Children with cystic fibrosis (CF) have a thick and sticky mucus which gets frequently infected. Bacterial infections are common especially with pseudomonas, staphylococcus etc. Children with cystic fibrosis (CF) also get infected with various atypical organisms including fungi and mycobacteria. Some infections happen for short periods of time (known as exacerbations) while some can last for many years. Cough swabs or sputum tests are usually done every 3-4 months to keep a track on the infections.

Children with cystic fibrosis (CF) having acute infections or exacerbations need oral or sometimes intravenous (IV) antibiotics. Children with chronic or long lasting infections need inhaled antibiotics like tobramycin and colistin.

Mucus thinning medicines for children with cystic fibrosis (CF)?
Mucus thinners or also known as mucolytics, are medications that help the mucus to get thinner and less sticky in patients with cystic fibrosis (CF). Thin mucus can be coughed out more easily which lets the lungs be free of impacted mucus and infection free. The three main types of mucus thinners are hypertonic saline, mannitol and dornase alfa or Pulmozyme. Of these hypertonic saline is the cheapest, easily available and most commonly used in India in patients with cystic fibrosis (CF).

Hypertonic saline is a sterile salt saline solution and is available as 3 percent and 7 percent. Hypertonic saline increases the amount of salt or sodium in the airways. Salt attracts more water into the airways which in turn makes the mucus thinner and less sticky. All children and adults with cystic fibrosis (CF). Chest physiotherapy should be done in all patients with cystic fibrosis (CF) after hypertonic saline to bring out the mucus from the lungs.

Cystic Fibrosis: How do we treat children?

Pancreatic Enzyme Replacement Therapy for children with cystic fibrosis (CF)?
More than 90 percent of children with cystic fibrosis (CF) have a very sticky mucus which blocks the ducts in the pancreas. This prevents the digestive enzymes from reaching the intestines hence reducing absorption of food. This on one hand leads to malnutrition and vitamin deficiency, on the other hand the undigested food causes crampy abdominal pain and loose, greesy stool. Pancreatic Enzyme Replacement Therapy (PERT) is essential for all children with cystic fibrosis (CF) who have pancreatic enzyme deficiency. Enteric-coated microencapsulated enzymes are the most effective treatment for pancreatic insufficiency in children with cystic fibrosis (CF). The most commonly available in India is CREON Caps (10,000 U). Pancreatic enzymes should be taken with all meals and snacks.

If your child is having cystic fibrosis you need to be under the care of a Cystic Fibrosis team led by a pediatric pulmonologist for optimal care of your child.

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