Beyond the Lungs—Emerging Challenges in Adult Cystic Fibrosis Care

Mulrennan, Siobhain; Sapru, Karuna; Tewkesbury, Daniel; Jones, Andrew M.

doi:10.1055/s-0042-1758734

Cited by 6 publications

(3 citation statements)

References 83 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In addition, a previous study on people with CF at the CF centre in Gothenburg showed no patient group-specific anatomical change or thoracic configuration with ageing [ 30 ]. Similar to the findings on thoracic mobility, these findings represent aspects of musculoskeletal health that need monitoring due to the demographic shift that is currently being seen in this patient group [ 11 , 31 ]. Regarding lung function, the results from this study were consistent with already well-established knowledge regarding the progressive nature of the disease on lung structure and function and will therefore not be discussed further at this time.…”

Section: Discussionsupporting

confidence: 60%

See 1 more Smart Citation

Musculoskeletal aspects of respiratory function in cystic fibrosis: a cross-sectional comparative study

Sinderholm Sposato,

Bjerså,

Gilljam

et al. 2024

European Clinical Respiratory Journal

View full text Add to dashboard Cite

Background Respiration is an intricate interaction between visceral and musculoskeletal structures. In cystic fibrosis (CF), the airways and lungs are subject to progressive obstruction and destruction. However, knowledge about the musculoskeletal aspects of respiratory function and symptoms is still limited in this patient group. Methods In a cross-sectional comparative study, 21 adults with CF enrolled at the Gothenburg CF Centre were matched with 42 healthy controls. The two groups were examined and compared in terms of thoracic mobility, respiratory muscle strength, lung function, and musculoskeletal pain in accordance with a predefined protocol. Results Significant differences were observed between the groups in the number of tender points, thoracic excursion, forced vital capacity (FVC), and forced expiratory volume (FEV). The CF group also demonstrated a tendency toward reduced function in other measurements, although these were not statistically significant. Conclusion This cross-sectional study revealed that people with CF have reduced thoracic mobility and an increased prevalence of muscular tender points, alongside decreased lung function, compared to healthy controls. These findings stress the need for greater emphasis on the often-overlooked musculoskeletal aspects of CF care, especially as people with CF are living longer and may require more musculoskeletal health support.

show abstract

Section: Discussionsupporting

confidence: 60%

“…As the average age of people with CF increases, so will the challenges of managing typical age-related musculoskeletal conditions, and it will be necessary to explore how these can best be managed. As also highlighted in previous research [ 12 , 31 , 32 ], this will probably require new and multidisciplinary approaches.…”

Section: Discussionmentioning

confidence: 85%

Musculoskeletal aspects of respiratory function in cystic fibrosis: a cross-sectional comparative study

Sinderholm Sposato,

Bjerså,

Gilljam

et al. 2024

European Clinical Respiratory Journal

View full text Add to dashboard Cite

show abstract

“…They are at risk for chronic kidney disease from microvascular complications and repeat exposure to nephrotoxic antibiotics. CFLD is the third largest cause of mortality in pwCF and studies show the incidence increase into adulthood [50 ▪▪ ]. There is also an increased risk for osteoporosis, hearing loss, and malignancy [51].…”

Section: Aging With Cystic Fibrosis – Uncharted Territorymentioning

confidence: 99%

Highly effective cystic fibrosis transmembrane conductance (regulator) modulator therapy: shifting the curve for most while leaving some further behind

Chun,

Somers,

Burgener

2024

Current Opinion in Pediatrics

View full text Add to dashboard Cite

Purpose of review Traditional cystic fibrosis (CF) care had been focused on early intervention and symptom mitigation. With the advent of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy (HEMT), in particular, the approval of elexacaftor/tezacaftor/ivacaftor in 2019, there has been a dramatic improvement in outcomes in CF. The purpose of this article is to review the benefits, limitations, and impact of HEMT as well as discuss the new implications, challenges, and hope that modulators bring to people with CF (pwCF). Recent findings HEMT has demonstrated sustained improvement in lung function, nutrition, quality of life, and survival for over 90% of pwCF. As HEMT has delivered such promise, there is a small but significant portion of pwCF who do not benefit from HEMT due to ineligible mutations, intolerance, or lack of accessibility to modulators. Summary HEMT has significantly improved outcomes, but continued research is needed to understand the new challenges and implications the era of HEMT will bring, as well as how to provide equitable care to those who are unable to benefit from HEMT.

show abstract