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The following medications are some of the most commonly used in the care of cystic fibrosis.
Digestive Tract Medications
Enzymes: Pancreatic enzymes are lacking in most children with CF, so these are taken to help digest protein and fat from food. The amount prescribed is based on a child’s weight and the dose will be adjusted often as a child grows. Brand name enzymes include:
Each brand enzyme comes in multiple strengths. Generic enzymes should NOT be used for children with CF.
Certain vitamins are poorly absorbed in children with CF, and extra vitamins are needed to keep children healthy. They include:
AquADEK® - A multi-vitamin with extra vitamin A, D, E and K
Vitamax ® - A multi-vitamin with extra vitamin A, D, E and K
Complete Formulation (MVW) - A multivitamin with extra vitamin A, D, E and K
Vitamin D - Ergocalciferol (Vitamin D2) or cholecalciferol (Vitamin D3)
Vitamin K - Phytonadione (Mephyton®)
These medications are needed to treat stomach reflux when it occurs. They are also prescribed to some children to help enzymes work better.
Proton pump inhibitor (Prevacid®, Prilosec® and others)
Enulose (Lactulose®) - A liquid syrup medicine given to young infants with constipation
Polyethylene glycol (MiraLax®) - A powder mixed in liquid for older infants with constipation
Ursodiol (Actigall®) - A medication prescribed to treat liver disease in CF
Inhaled or Nebulized Medications
Pulmozyme® - A preventive medication that helps break down mucous in the lungs making it easier to cough out
Bronchodilators (albuterol, Xopenex®) - These help open the airways to improve clearance of mucous
Hypertonic saline - A strong salt solution that helps restore a normal environment in the lungs and encourages a healthy cough
TOBI® - An antibiotic (tobramycin) that keeps certain bacteria (pseudomonas) from growing in the lungs
These medications can lessen the inflammation or swelling in the airways. Although steroids are used less frequently, they may be prescribed for children who are having asthma-type symptoms in addition to CF. They can be given for a short time by mouth, or can be taken long-term in an inhaled or nebulized form.
Oral Antibiotics for Respiratory Illnesses
These are medications used to treat bacteria in the lungs or sinuses that can cause an infection. Common antibiotics used in CF include:
Amoxicillin and clavulanic acid (Augmentin®)
Sulfamethaxazole and trimethoprim (Bactrim®), sometimes in combination with rifampicin (Rifampin®)
Antibiotics are chosen based on what bacteria are found on respiratory cultures, the symptoms during an illness, any allergies and a child’s previous response to antibiotics.