Other considerations

Microbiology & cross infection control

More than 41 % adults with CF have chronic pseudomonas infection.

Nearly 9% have Aspergillus infection and around 7% of CF patients suffer from ABPA which is an immune response to Aspergillus.

Non tuberculous mycobacterium is found in 7% of CF patients.

CF patients with transmissible or chronic infections with Pseudomonas aeruginosa or Burkholderia cepacia may need isolation whilst in healthcare facilities specially to prevent cross infection to other CF patients or immunodeficient patients. CF patients needing inpatient care would need individual rooms with separate toilets.

Pharmacy & therapeutics

Inhaled antibiotics: CF patients might be on inhaled antibiotics like colistimethate sodium, aztreonam, tobramycin etc for respiratory infections.

Mucoactive agents: Mucus thinners like hypertonic saline, mannitol dry powder and DNase (recombinant human deoxyribonuclease which are first line agents; dornase alpha) are prescribed to around 80% of CF patients to help clearing respiratory secretions.

Immunomodulators: CF patients might be on long term immunomodulation dose of Azithromycin or oral steroids might

CFTR modifiers: A proportion of CF patients may be on CFTR modifiers like Ivacaftor, Lumacaftor etc. A knowledge of this may be necessary to evaluate ED presentations with possible side effects like abdominal pain, diarrhea dizziness, headache, respiratory reactions (oropharyngeal pain, nasal/ sinus congestion etc.), etc.

Transplant

Number of CF patients are double lung recipients or are on the waiting list. It may be important to address transplant related issues like immunosuppression, specific infections etc. while assessing them.