Table 1.
Proposed treatable traits of NTM lung disease in people with cystic fibrosis and their clinical management
| Treatable Traits | Clinical figures | 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 |
CF cystic fibrosis, LABA long acting beta-2 agonists, LAMA long acting anti-muscarinic agents, ICS inhaled corticosteroids, GERD gastro-esophageal reflux disease, NTM-DR drug resistant non-tuberculous mycobacteria, ACT Airway clearance technique