To ensure that people with cystic fibrosis continue to experience steady gains in length and quality of life, the Cystic Fibrosis Foundation develops clinical practice guidelines to help guide the care of individuals with CF. The guidelines are based on available medical evidence and consultation with experts on best practices. The following are updates from the clinical practice guidelines committees. These committees consist of subject matter experts who write guidelines on topics related to CF care and include doctors, nurses, respiratory therapists, dietitians, social workers, and people with CF and their families.
Colorectal Cancer Screening Consensus Statements
Drs. Albert Lowenfels and Alexander Khoruts are chairing the colorectal cancer screening consensus statements. The committee is preparing the manuscript for submission to Gastroenterology.
Diagnosis Consensus Conference
Drs. Philip Farrell, Patrick Sosnay and Clement Ren are chairing the diagnosis consensus conference. The committee submitted six manuscripts for a supplement that has been published in The Journal of Pediatrics. These manuscripts comprise the consensus guidelines; a historical perspective; genetics to facilitate diagnosis; diagnosis in screened populations; CFTR-related metabolic syndrome (CRMS) and cystic fibrosis screen positive, inconclusive diagnosis (CFSPID); and diagnosis in non-screened populations.
Clinical Practice Guidelines: Executive Summaries
Drs. Chris Oermann and Manu Jain are leading an initiative to make the guideline recommendations more accessible to the CF community and beyond. Pairs of junior and senior authors have been reviewing the guidelines and developing concise executive summaries to be made available on the CFF.org guidelines page. In October 2016, the first two executive summaries were posted to CFF.org:
The next set of executive summaries will include guideline recommendations on enzymes, bone health, eradication of initial Pseudomonas aeruginosa, care for preschool-aged children and non-tuberculosis mycobacteria. They are expected to be posted on CFF.org in early 2017.
Palliative Care
A workshop led by Dr. Elisabeth Dellon met in July and again at the 2016 North American CF Conference (NACFC) to discuss the current state of research in cystic fibrosis palliative care. This group is considering its next steps, which may include the development of consensus guidelines.
CFTR Modulators
Drs. Clement Ren and Ted Naureckas and a non-conflicted committee are developing guidelines on the use of cystic fibrosis transmembrane conductance regulator (CFTR) modulators.
For more information about the clinical practice guidelines, contact Sarah Hempstead at shempstead@cff.org.