Perceived fatigue in children and young adults with neurofibromatosis type 1

Abstract

Aim This study describes the prevalence and severity of perceived fatigue in a young neurofibromatosis type 1 (NF1) population. Methods Ethical approval was obtained and NF1 affected Individuals aged 2–18 years from the Manchester's NF1 clinic invited along with any unaffected siblings. The PedsQL Multidimensional Fatigue Scale Parental and child report was used. This validated measure explores cognitive, physical and sleep/rest domains on a 0–100 scale. Higher scores indicate less fatigue. Fatigue scores in affected children were compared to unaffected siblings after adjusting for age, sex and Index of Multiple Deprivation and with published population standards using z‐scores. Results A total of 286 families were invited and 75 affected and 16 siblings participated. There were significant differences between NF1 and controls in the aggregated fatigue core (child report 55 ± 19 vs. 75 (14), P < 0.001; parent 54 ± 20 vs. 73 ± 18, P = 0.001) and the three sub‐domains: cognitive (child 48 ± 27 vs. 75 ± 23, P < 0.001), physical (child 59 ± 19 vs. 82 ± 14, P < 0.001) and sleep/rest (child 59 ± 19 vs. 71 ± 15, P = 0.018). Similar differences were seen when compared with published controls (aggregated child z‐score −1.9 ± 1.4, P < 0.001; parent −3.2 ± 1.8, P < 0.001). Prevalence of severe fatigue indicated by scores <2 standard deviation below published means for healthy controls were also higher for children with NF on both parent and child reports. Agreement between child and parent reports were limited as is frequently seen in the literature. Conclusion This study suggests that children with NF1 are affected by perceived fatigue when compared with healthy children who do not have NF1.

Determinants of impairments in functioning, fatigue, and participation ability in pediatric brain tumor survivors

“…In our cohort PBTS with NF-1 reported increased general fatigue compared to those without NF-1, which was expected because children with NF-1 reported more perceived fatigue. 29 …”

Fatigue perception in a cohort of children with chronic immune thrombocytopenia and their caregivers using the PedsQL MFS: Real‐life multicenter experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP)

“…However, other studies have reported worse perception of HRQoL in females affected by chronic diseases 45,46 . The perception of fatigue in chronic conditions might be influenced by different gender‐related social behaviors, but further studies are required to clarify the association between fatigue and gender in chronic ITP 28,30,38 …”

“…The PedsQL Multidimensional Fatigue Scale (PedsQL MFS) is a generic symptom‐specific tool to measure fatigue in healthy children and adolescents as well as those with acute and chronic health conditions 26,27 . Importantly, it also measures cognitive fatigue, which focuses on memory and attention difficulties 28–30 . The use of these tools can be useful in clinical routine, because patients with ITP often report fatigue symptoms.…”

Development of tibia & fibula bone deficits in children with neurofibromatosis type I – A longitudinal case-control comparison

“…Children with NF1 display delayed motor development [17], which is associated with impaired bone loading and resultant deficits in skeletal development through childhood [18]. Previous studies have also identified muscle weakness in children with NF1 [19][20][21], although there is limited evidence of how this develops across childhood [20]. To date, we are only aware of one study which assessed physical activity in children with NF1, finding no difference from controls [19], but this was based on questionnaire assessments which may be less accurate and more prone to bias than objective accelerometer measures.…”