Table 1 Proposed treatable traits of NTM lung disease in people with cystic fibrosis and their clinical management

Mucus plugging

Respiratory physiotherapist

Airway clearance techniques; mucoactive adjuncts; pulmonary rehabilitation

CF pathogen chronic respiratory infection

CF specialist

Infectious disease specialist

Systemic antibiotics (if acute phase); inhaled antibiotics (for either eradication of new pathogens or chronic suppression)

Airflow obstruction

CF specialist

Pulmonologist

Long-acting inhaled bronchodilators (both LABA and LAMA); ICS (if hyper-responsiveness demonstrated)

CF-related diabetes

CF specialist

Diabetologist

Referral to diabetology service

Osteoporosis

CF specialist

Endocrinologist

Vitamin D/cholecalciferol supplementation; referral to bone health service

Anxiety and depression

Psychiatrist

Psychologist

Psychological support; referral to the mental health service

GERD

CF Dietician

Gastroenterologist

Dietary restrictions; proton pump inhibitors; antacids; prokinetics; referral to the gastroenterology service

Environmental exposure

CF specialist

Respiratory physiotherapist

Avoid risky activities (fishing, gardening, hot springs); segregation at CF center; disinfection of devices

Undernutrition and performance status

CF dietician

Nutritional screening at each clinical encounter; assessment of energy and nutrient requirements; individual dietary counseling to maintain optimal nutritional status and avoid undernutrition and excessive weight gain; Physical training

Adherence to treatments

CF specialist

Tele-monitoring; easy access to CF center; psychological support; directly observed therapy; three times weekly therapy

Risk of NTM-DR during long-term azithromycin

CF specialist

Pulmonologist

Infectious disease specialist

Rule out NTM before starting azithromycin; evaluate azithromycin discontinuation in case of NTM occurrence; optimize ACT and chronic treatment

Risk of NTM-DR during inhaled aminoglycosides

CF specialist

Pulmonologist

Infectious disease specialist

Rule out NTM before starting inhaled treatment; evaluate aminoglycoside discontinuation in case of NTM occurrence (shift to other inhaled antibiotics); optimize ACT and chronic treatment

Interactions with CFTR modulators

CF specialist

Pulmonologist

Infectious disease specialist

Evaluate rifabutin instead of rifampicin; check drugs interactions; CFTR modulator dose adjustment

Avoiding inhaled corticosteroids

CF specialist

Respiratory physiotherapist

Test for bronchial hyper-responsiveness; evaluate safe ICS withdrawal