To provide diagnostic and treatment guidance for clinicians dealing with cystic fibrosis, the Cystic Fibrosis Foundation recently published updated guidelines for physicians. The document, titled ‘Cystic Fibrosis Pulmonary Guidelines, Chronic Medications for Maintenance of Lung Health,’ was recently published in the American Journal of Respiratory and Critical Care Medicine.
Cystic fibrosis (CF) is a multiorgan disease with lung disease accounting for nearly 85% of the mortality. CF causes obstruction of the lungs’ airways due to dehydrated, thickened secretions, resultant endobronchial infection, and an exaggerated inflammatory response leading to development of bronchiectasis and progressive obstructive airways disease.
In order to establish the guidelines, a panel of Cystic Fibrosis specialists assessed current evidence from published clinical Cystic Fibrosis related literature and deliberated the guidelines. Based on the newly published guidelines, the Cystic Fibrosis Foundation recommends that clinicians should make a CF diagnosis if:
- There is history of CF in a sibling or if following a screening test, a newborn tests positive. Furthermore, there must be evidence of abnormal activity in the cystic fibrosis transmembrane conductance regulator gene or protein.
- Test sweat chloride and examine if levels are normal (≤39 mmol/L), intermediate (40 to 59 mmol/L) or abnormal (≥60 mmol/L).
- In newborns that tested positive in the screening test and present sweat chloride levels ≥60 mmol/L.
- Diagnosis should also be made if at any age, the patients have family history of CF and sweat chloride levels ≥60 mmol/L. However, the foundation recommends that these patients take a second chloride test, unless there is the presence of two cystic fibrosis-causing mutations after laboratory tests.
In terms of recommended treatments for CF, the Cystic Fibrosis Foundation established a consensus in the recommendations, further noting that for patients aged 6 years and over the prescription of 1) aerosolized tobramycin for either asymptomatic or with mild lung disease that have Pseudomonas aeruginosa; and 2) recombinant human DNase (dornase alfa) to improve lung function and reduce exacerbations.
“The introduction and use of specific medications will depend on the individual patient, their social situation and parental or patient preferences,” members of the guideline committee reported. “We are hopeful that clinicians will find these recommendations to be useful in their care of patients with cystic fibrosis.”