Treatment Overview

There is currently no cure for cystic fibrosis. Treatment for cystic fibrosis is focused on easing symptoms, slowing the progression of the disease, and preventing and treating complications.

Types of Treatments

Treatments for cystic fibrosis include:

  • Airway clearance
  • Medications
  • Surgery
  • Long-term pulmonary rehabilitation
  • Other therapies

Airway clearance – Patients with cystic fibrosis are taught airway clearance techniques that are used several times a day.

Airway clearance techniques used for cystic fibrosis include:

  • Chest physical therapy
  • Cupping with hands on the front and back of chest
  • Breathing and coughing techniques
  • The use of machines, such as a vibrating vest, that pulse air into the lungs

Medications – Medications used to treat cystic fibrosis may include:

  • Antibiotics
  • Anti-inflammatory drugs
  • Mucus-thinning drugs
  • Bronchodilators (to keep airways open)
  • Oral pancreatic enzymes (to improve nutrient absorption)
  • Stool softeners
  • Acid-reduction medications

Antibiotics – Inhaled antibiotics, such as tobramycin and aztreonam, may be used to treat lung infections in patients with cystic fibrosis.

Anti-inflammatory drugs, such as ibuprofen, are used to lessen swelling associated with cystic fibrosis

Mucus-thinning medication – The drug, dornase alfa, and hypertonic saline, may be used to thin the thick mucus associated with cystic fibrosis.

Bronchodilators – Inhaled bronchodilators, such as albuterol, are used to open the airways in patients with cystic fibrosis.

Oral pancreatic enzymes – Oral pancreatic enzymes, such as lipase, protease, and amylase, are taken before meals by patients with cystic fibrosis to help break down food.

Stool softeners – Stool softeners, such as polyethylene glycol, are used to prevent intestinal obstructions in patients with cystic fibrosis.

Gene-targeted therapy – Patients with cystic fibrosis who have certain gene mutations may be given gene-target therapy to improve lung function, weight, and to reduce the amount of sodium in sweat.

Gene-targeted therapy for the treatment of cystic fibrosis may include:

  • Elexacaftor
  • Ivacaftor
  • Tezacaftor
  • Combination of tezacaftor and invacaftor
  • Combination of lumacaftor and ivacaftor

Acid-reducing drugs – Acid-reducing drugs for the treatment of cystic fibrosis may include the proton pump inhibitors:

  • Ecomeprazole
  • Lansoprazole
  • Omeprazole

Surgery – Some patients with cystic fibrosis may need surgery to remove nasal polyps, to treat chronic sinusitis, or to repair an intestinal obstruction.

In some selected patients with severe lung damage due to cystic fibrosis, lung transplantation may be an option.

For patients with cystic-fibrosis liver disease, such as scarring of the liver, also called cirrhosis, liver transplantation may be an option. Liver transplantation can be combined with lung or pancreas transplantation.

Other therapies – Additional therapies for cystic fibrosis may include:

  • Oxygen therapy to prevent pulmonary hypertension, which is high blood pressure in the main lung artery
  • Noninvasive ventilation, also called CPAP, which uses a machine and mask to assist breathing while sleeping
  • Placement of a feeding tube to counteract or prevent malnutrition
  • Vitamin therapy with vitamins A, D, E, and K
  • Diet therapy

Long-term pulmonary rehabilitation – Pulmonary rehabilitation for cystic fibrosis includes:

  • Exercise
  • Nutritional counseling
  • Supportive counseling
  • Cystic fibrosis education

New, Experimental Treatments

The existing antifungal drug, amphotericin, has recently been shown to help improve lung function in patients with cystic fibrosis. Amphotericin can also help to fight off the chronic bacterial infections that are a hallmark of the disease.

Long-Term Follow Up

Long-term follow-up is necessary for patients with cystic fibrosis to prevent and treat infections and complications and monitor treatment effectiveness.

Sources

This content was written by the MediFind Medical Team. Last updated: 6/9/2022

Medications for Cystic Fibrosis

These are drugs that have been approved by the US Food and Drug Administration (FDA), meaning they have been determined to be safe and effective for use in Cystic Fibrosis.

Found 6 Approved Drugs for Cystic Fibrosis

Tobramycin

Brand Names
TOBI Podhaler, TOBI, Bethkis, TobraDex, Tobrex

Tobramycin

Brand Names
TOBI Podhaler, TOBI, Bethkis, TobraDex, Tobrex
Form: Injection, Ointment, Inhalant, Suspension, Capsule, Solution
Method of administration: Respiratory (inhalation), Oral, Intravenous, Intramuscular, Ophthalmic
FDA approval date: June 28, 1981
Classification: Corticosteroid
Tobramycin injection is indicated for the treatment of serious bacterial infections caused by susceptible strains of the designated microorganisms in the diseases listed below: Septicemia in the neonate, child, and adult caused by P. aeruginosa, E. coli, and Klebsiella sp Lower respiratory tract infections caused by P. aeruginosa, Klebsiella sp, Enterobacter sp, Serratia sp, E. coli, and S. aureus (penicillinase- and non-penicillinase-producing strains) Serious central-nervous-system infections (meningitis) caused by susceptible organisms Intra-abdominal infections, including peritonitis, caused by E. coli, Klebsiella sp, and Enterobacter sp Skin, bone, and skin structure infections caused by P. aeruginosa, Proteus sp, E. coli, Klebsiella sp, Enterobacter sp and S. aureus Complicated and recurrent urinary tract infections caused by P. aeruginosa, Proteus sp, (indole-positive and indole- negative), E. coli, Klebsiella sp, Enterobacter sp, Serratia sp, S. aureus, Providencia sp, and Citrobacte r sp. Aminoglycosides, including tobramycin, are not indicated in uncomplicated initial episodes of urinary tract infections unless the causative organisms are not susceptible to antibiotics having less potential toxicity. Tobramycin may be considered in serious staphylococcal infections when penicillin or other potentially less toxic drugs are contraindicated and when bacterial susceptibility testing and clinical judgment indicate its use. Bacterial cultures should be obtained prior to and during treatment to isolate and identify etiologic organisms and to test their susceptibility to tobramycin. If susceptibility tests show that the causative organisms are resistant to tobramycin, other appropriate therapy should be instituted. In patients in whom a serious life-threatening gram- negative infection is suspected, including those in whom concurrent therapy with a penicillin or cephalosporin and an aminoglycoside may be indicated, treatment with tobramycin may be initiated before the results of susceptibility studies are obtained. The decision to continue therapy with tobramycin should be based on the results of susceptibility studies, the severity of the infection, and the important additional concepts discussed in the WARNINGS box above. To reduce the development of drug-resistant bacteria and maintain the effectiveness of Tobramycin Injection and other antimicrobial drugs, Tobramycin Injection should be used only to treat or prevent infections that are proven or strongly suspected to be caused by susceptible bacteria. When culture and susceptibility information are available, they should be considered in selecting or modifying antimicrobial therapy. In the absence of such data, local epidemiology and susceptibility patterns may contribute to the empiric selection of therapy.

Ivacaftor

Brand Names
Trikafta, Kalydeco, Symdeko, Orkambi

Ivacaftor

Brand Names
Trikafta, Kalydeco, Symdeko, Orkambi
Form: Tablet, Kit, Granule
Method of administration: Oral
FDA approval date: January 31, 2012
Classification: Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
KALYDECO is indicated for the treatment of cystic fibrosis (CF) in patients age 4 months and older who have one mutation in the CFTR gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data [see Clinical Pharmacology (1.

Azithromycin

Brand Names
Azasite, Zithromax

Azithromycin

Brand Names
Azasite, Zithromax
Form: Injection, Tablet, Powder, Suspension, Solution
Method of administration: Oral, Intravenous, Parenteral, Ophthalmic
FDA approval date: September 28, 1994
Classification: Macrolide Antimicrobial
Azithromycin for oral suspension USP is a macrolide antibacterial drug indicated for the treatment of patients with mild to moderate infections caused by susceptible strains of the designated microorganisms in the specific conditions listed below. Recommended dosages and durations of therapy in adult and pediatric patient populations vary in these indications.

Pulmozyme

Generic Name
Dornase

Pulmozyme

Generic Name
Dornase
Form: Solution
Method of administration: Respiratory (inhalation)
FDA approval date: December 30, 1993
Classification: Recombinant Human Deoxyribonuclease 1
PULMOZYME ® is indicated, in conjunction with standard therapies, for the management of pediatric and adult patients with cystic fibrosis (CF) to improve pulmonary function. In CF patients with an FVC ≥ 40% of predicted, daily administration of PULMOZYME has also been shown to reduce the risk of respiratory tract infections requiring parenteral antibiotics. PULMOZYME is a recombinant DNase enzyme indicated in conjunction with standard therapies for the management of cystic fibrosis (CF) patients to improve pulmonary function. ( 1 )

Aztreonam

Brand Names
Cayston, Azactam, Emblaveo

Aztreonam

Brand Names
Cayston, Azactam, Emblaveo
Form: Injection, Kit, Powder
Method of administration: Intravenous, Intramuscular
FDA approval date: November 05, 2009
Classification: Monobactam Antibacterial
To reduce the development of drug-resistant bacteria and maintain the effectiveness of aztreonam for injection, USP and other antibacterial drugs, aztreonam for injection should be used only to treat or prevent infections that are proven or strongly suspected to be caused by susceptible bacteria. When culture and susceptibility information are available, they should be considered in selecting or modifying antibacterial therapy. In the absence of such data, local epidemiology and susceptibility patterns may contribute to the empiric selection of therapy. Aztreonam for Injection is indicated for the treatment of the following infections caused by susceptible Gram-negative microorganisms: Urinary Tract Infections (complicated and uncomplicated), including pyelonephritis and cystitis (initial and recurrent) caused by Escherichia coli, Klebsiella pneumoniae, Proteus mirabilis, Pseudomonas aeruginosa, Enterobacter cloacae, Klebsiella oxytoca *, Citrobacter species*, and Serratia marcescens *. Lower Respiratory Tract Infections, including pneumonia and bronchitis caused by Escherichia coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, Haemophilus influenzae, Proteus mirabilis, Enterobacter species, and Serratia marcescens *. Septicemia caused by Escherichia coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, Proteus mirabilis *, Serratia marcescens *, and Enterobacter species. Skin and Skin-Structure Infections, including those associated with postoperative wounds, ulcers, and burns, caused by Escherichia coli, Proteus mirabilis, Serratia marcescens, Enterobacter species, Pseudomonas aeruginosa, Klebsiella pneumoniae, and Citrobacter species*. *Efficacy for this organism in this organ system was studied in fewer than 10 infections. Intra-abdominal Infections, including peritonitis caused by Escherichia coli, Klebsiella species including K. pneumoniae, Enterobacter species including E. cloacae *, Pseudomonas aeruginosa, Citrobacter species* including C. freundii *, and Serratia species* including S. marcescens *. Gynecologic Infections, including endometritis and pelvic cellulitis caused by Escherichia coli, Klebsiella pneumoniae *, Enterobacter species* including E. cloacae *, and Proteus mirabilis *. Aztreonam for injection is indicated for adjunctive therapy to surgery in the management of infections caused by susceptible organisms, including abscesses, infections complicating hollow viscus perforations, cutaneous infections, and infections of serous surfaces. Aztreonam for injection is effective against most of the commonly encountered Gram-negative aerobic pathogens seen in general surgery. Concurrent Therapy Concurrent initial therapy with other antimicrobial agents and aztreonam for injection is recommended before the causative organism(s) is known in seriously ill patients who are also at risk of having an infection due to Gram-positive aerobic pathogens. If anaerobic organisms are also suspected as etiologic agents, therapy should be initiated using an anti-anaerobic agent concurrently with aztreonam for injection. Certain antibiotics (e.g., cefoxitin, imipenem) may induce high levels of beta-lactamase in vitro in some Gram-negative aerobes such as Enterobacter and Pseudomonas species, resulting in antagonism to many beta-lactam antibiotics including aztreonam. These in vitro findings suggest that such beta-lactamase-inducing antibiotics not be used concurrently with aztreonam. Following identification and susceptibility testing of the causative organism(s), appropriate antibiotic therapy should be continued.
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