Cystic fibrosis medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chetan Lokhande, M.B.B.S [2]

Cystic fibrosis Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Cystic fibrosis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
Chest X Ray
Echocardiography or Ultrasound
CT
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Cystic fibrosis medical therapy On the Web
Most recent articles
cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Cystic fibrosis medical therapy All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Cystic fibrosis medical therapy
CDC on Cystic fibrosis medical therapy
Cystic fibrosis medical therapy in the news
Blogs on Cystic fibrosis medical therapy
Directions to Hospitals Treating Cystic fibrosis
Risk calculators and risk factors for Cystic fibrosis medical therapy

Overview

A typical breathing treatment for cystic fibrosis, using a mask nebuliser and the ThAIRapy Vest

The cornerstones of management for cystic fibrosis are treatment of airway infection, maximizing organ function, and encouragement of good nutrition and an active lifestyle. The treatment for cystic fibrosis continues throughout a patient's life and aims to maintain quality of life. At best, current treatments delay the decline in organ function. Targets of therapy include the lungs, gastrointestinal tract (including Insulin treatment), the reproductive organs (including Assisted Reproductive Technology (ART)) and psychological support. Intravenous, inhaled, and oral antimicrobial agents are used to treat acute and chronic infections. Mechanical devices and inhaled medications are used to alter and clear the thickened mucus. In addition, therapies such as gene therapy and [[Organ transplantation may also be used in the treatment of cystic fibrosis.

Medical Therapy

Antibiotics are given whenever pneumonia is suspected or there has been a decline in lung function. Antibiotics are often chosen based on information about prior infections. Many bacteria common in cystic fibrosis are resistant to multiple antibiotics and require weeks of treatment with intravenous antibiotics such as vancomycin, tobramycin, meropenem, ciprofloxacin and piperacillin. This prolonged therapy often necessitates hospitalization and insertion of a more permanent IV such as a PICC line or Port-a-Cath. Inhaled therapy with antibiotics such as tobramycin and colistin is often given for months at a time in order to improve lung function by impeding the growth of colonized bacteria. Oral antibiotics such as ciprofloxacin or azithromycin are sometimes given to help prevent infection or to control ongoing infection. Some individuals spend years between hospitalizations for antibiotics, whereas others require several antibiotic treatments each year.

Several common antibiotics such as tobramycin and vancomycin can cause hearing loss or kidney problems with long-term use. In order to prevent these side-effects, the amount of antibiotics in the blood are routinely measured and adjusted accordingly.

Antibiotics

• 1.Pathogen-directed antimicrobial therapy

• 1.1 Pseudomonas aeruginosa

• 1.1.1 Adults

• Preferred regimen (1): Tobramycin 3.3 mg/kg PO tid OR Tobramycin 12 mg/kg IV q24h AND (Piperacillin 100 mg/kg PO qid
• Preferred regimen (2): Ticarcillin 100 mg/kg PO qid
• Preferred regimen (3): Ceftazidime 50 mg/kg IV q8h (to maximum of 6 g/day)
• Alternative regimen (1): (Tobramycin 3.3 mg/kg PO tid OR Tobramycin 12 mg/kg IV q24h) AND Aztreonam 50 mg/kg IV q8h
• Alternative regimen (2): (Tobramycin 3.3 mg/kg PO tid OR Tobramycin 12 mg/kg IV q24h) AND Imipenem 15-25 mg/kg IV q6h
• Note: If Tobramycin resistant add Ciprofloxacin 500-750 mg PO bid for 7-14 days OR Ciprofloxacin 400 mg IV q12h for 7-14 days OR Levofloxacin 750 mg PO qd for 7-14 days

• 1.1.2 Children

• Preferred regimen: (Ciprofloxacin 500-750 mg PO bid for 7-14 days OR Ciprofloxacin 400 mg IV q12h for 7-14 days) AND Ceftazidime 0.5-1 g IV q8h

• 1.2 Staphylococcus aureus

• 1.2.1 Adults

• 1.2.1.1 Methicillin sensitive staphylococcus aureus

• Preferred regimen: Nafcillin 2 g IV q4h OR Oxacillin 2 g IV q4h

• 1.2.1.2 Methicillin resistant staphylococcus aureus

• Preferred regimen: Vancomycin 15-20 mg/kg IV q8-12h OR Linezolid 600 mg PO/IV q12h

• 1.2.2 Children

• 1.2.2.1 Methicillin sensitive staphylococcus aureus

• Preferred regimen: Nafcillin 5 mg/kg IV q6h (age > 28 days) OR Oxacillin 75 mg/kg IV q6h (age > 28 days)

• 1.2.2.2 Methicillin resistant staphylococcus aureus

• Preferred regimen: Vancomycin 40 mg/kg IV divided q6-8h (age >28 days) OR Linezolid 10 mg/kg PO/IV q8h (up to age 12)

• 1.3 Burkholderia cepacia

• Preferred regimen: Trimethoprim-sulfamethoxazole 5/25 mg/kg IV q6h
• Alternative regimen: Chloramphenicol 15–20 mg/kg PO/IV q6h

Other methods to treat lung disease

Several mechanical techniques are used to dislodge sputum and encourage its expectoration. In the hospital setting, physical therapy is utilized; a therapist pounds an individual's chest with his or her hands several times a day. Devices that recreate this percussive therapy include the ThAIRapy Vest and the intrapulmonary percussive ventilator (IPV). Newer methods such as Biphasic Cuirass Ventilation, and associated clearance mode available in such devices, now integrate a cough assistance phase, as well as a vibration phase for dislodging secretions. Biphasic Cuirass Ventilation is also shown to provide a bridge to transplantation. These are portable and adapted for home use. Aerobic exercise is of great benefit to people with cystic fibrosis. Not only does exercise increase sputum clearance but it also improves cardiovascular and overall health.

Aerosolized medications that help loosen secretions include dornase alfa and hypertonic saline. Dornase is a recombinant human deoxyribonuclease, which breaks down DNA in the sputum, thus decreasing its viscosity. N-Acetylcysteine may also decrease sputum viscosity, but research and experience have shown its benefits to be minimal. Albuterol and ipratropium bromide are inhaled to increase the size of the small airways by relaxing the surrounding muscles.

As lung disease worsens, breathing support from machines may become necessary. Individuals with CF may need to wear special masks at night that help push air into their lungs. These machines, known as bilevel positive airway pressure (BiPAP) ventilators, help prevent low blood oxygen levels during sleep. BiPAP may also be used during physical therapy to improve sputum clearance. During severe illness, people with CF may need to have a tube placed in their throats and their breathing supported by a ventilator.

Treatment of other aspects of CF

Intracytoplasmic sperm injection is used to provide fertility for men with cystic fibrosis.

Newborns with meconium ileus typically require surgery, whereas adults with distal intestinal obstruction syndrome typically do not. Treatment of pancreatic insufficiency by replacement of missing digestive enzymes allows the duodenum to properly absorb nutrients and vitamins that would otherwise be lost in the faeces. Even so, most individuals with CF take additional amounts of vitamins A, D, E, and K and eat high-calorie meals. It should be noted, however, that nutritional advice given to patients is, at best, mixed: Often, literature encourages the eating of high-fat foods without differentiating between saturated and unsaturated fats/trans-fats; this lack of clear information runs counter to health advice given to the general population, and creates the risk of further serious health problems for people with cystic fibrosis as they grow older. So far, no large-scale research involving the incidence of atherosclerosis and coronary heart disease in adults with cystic fibrosis has been conducted. This is likely due to the fact that the vast majority of people with cystic fibrosis do not live long enough to develop clinically significant atherosclerosis or coronary heart disease.

The diabetes common to many CF patients is typically treated with insulin injections or an insulin pump. Development of osteoporosis can be prevented by increased intake of vitamin D and calcium, and can be treated by bisphosphonates. Poor growth may be avoided by insertion of a feeding tube for increasing calories through supplemental feeds or by administration of injected growth hormone.

Sinus infections are treated by prolonged courses of antibiotics. The development of nasal polyps or other chronic changes within the nasal passages may severely limit airflow through the nose. Sinus surgery is often used to alleviate nasal obstruction and to limit further infections. Nasal steroids such as fluticasone are used to decrease nasal inflammation. Female infertility may be overcome by assisted reproduction technology, particularly embryo transfer techniques. Male infertility may be overcome with intracytoplasmic sperm injection. Third party reproduction is also a possibility for women with CF.