Cystic Fibrosis and Autism Spectrum Disorder: Unique challenges of this
Background Cystic Fibrosis (CF) and autism spectrum disorder
(ASD) are life-long conditions with intense treatment burdens for
patients and families. Patients with a concurrent diagnosis (CF-ASD)
experience unique challenges to CF care. This study describes the
experiences of our multidisciplinary CF team in caring for patients with
CF-ASD and provides insight into provider and parental perspectives on
clinical management. Methods This is a three-part IRB-approved
study involving 1) retrospective chart review of patients with CF-ASD,
2) qualitative interviews with multi-disciplinary care teams, and 3)
qualitative interviews with caregivers of patients with CF-ASD.
Challenges in clinical management of this specific cohort were compiled
using data from chart review and care team interviews. Strategies to
address these challenges were identified and rated by individual
families based on relevance and practicality. Results Within
our CF center, 12 patients have an official diagnosis of ASD. Median age
of patients with CF-ASD was 8.5 years (range 3-20 years), 75% were
male, and 83% were on highly effective modulator therapy. Clinical
challenges included sensory processing issues, environmental
overstimulation, intolerance to procedures and disrupted routines.
Potentially impactful strategies include patient-specific coping plans,
guided behavioral interventions, parental advocacy, and improved
communication between the family and multidisciplinary team.
Conclusions Children with CF-ASD face extraordinary challenges
beyond the experience of neurotypical children with CF. Increased
awareness of this complex dual diagnosis will help providers be
sensitive to the unique needs of these patients, to help build
consistent and trustworthy relationships with families, and to provide
effective clinical care despite limitations.