Hamostaseologie 2020; 40(S 01): S33-S52
DOI: 10.1055/s-0040-1721584
VII. Pädiatrie

Evaluation of Individualized Physiotherapy in Pediatric Patients with Severe Bleeding Disorders

Elisabeth Kahre
1   Department of Pediatric Haemostaseology, University Hospital Carl Gustav Carus Dresden, Dresden, Germany
,
Tina Adelhardt
2   Division of Pediatric Physiotherapy, University Hospital Carl Gustav Carus Dresden, Dresden, Germany
,
Claudia Nitze
1   Department of Pediatric Haemostaseology, University Hospital Carl Gustav Carus Dresden, Dresden, Germany
,
Ursula Range
3   Institute for Medical Informatics and Biometry, Medical Faculty Carl Gustav Carus Dresden, Dresden, Germany
,
Sylvia von Mackensen
4   Department of Medical Psychology, University Medical Centre Hamburg-Eppendorf, Eppendorf, Germany
,
Ralf Knöfler
1   Department of Pediatric Haemostaseology, University Hospital Carl Gustav Carus Dresden, Dresden, Germany
› Institutsangaben
› Weitere Informationen
Auch verfügbar auf
 

Background Physiotherapy (PT) is important for the prevention, treatment, and rehabilitation of joint bleeds in severe hemophilia and von Willebrand’s disease type 3 (VWD). However, only few studies regarding the effect of PT in bleeding disorders in the pediatric age are available.

Aims To examine the effect of a regular and individualized PT in children with severe hemophilia and VWD.

Methods Patients were examined by the same physiotherapist prior to regular PT (for 1 year every second week for 30 minutes), after 6 and 12 months. Outcome measures were the evaluation of the Haemophilia Joint Health Score (HJHS), circumferences of the extremities (CE), muscle strength (MS) by myometry, range of motion (ROM) by goniometry, posture by postural analysis (PA), balance by one-leg-stand, coordination, endurance, velocity, and skills by the self-developed Soft-Barrier-test (SOBA). Furthermore, self-reported physical functioning and health-related quality of life (HRQoL) were assessed using the HEP-Test-Q and the Haemo-QoL/Haem-A-QoL questionnaires. In addition, annual bleeding rate (ABR) and the annual joint bleeding rate (AJBR) were registered.

Results Eleven patients (8 with severe hemophilia A, 2 with severe hemophilia B, and 1 with VWD) with a mean age of 9.6 ± 4.4 years were enrolled. HJHS improved significantly in all enrolled patients. A significant increase in CE in some locations of the upper and lower extremities were observed. MS remained stable. No significant changes in ROM, PA, and SOBA test with tendencies to improvement were found. Results of the HEP-Test-Q also showed tendencies to an improvement but without statistical significant changes. The HRQoL improved significantly. With a median of 0 the ABR and AJBR remained unchanged. The total consumption of factor concentrates did not elevate during the study compared to the year before.

Conclusion The approach of this pilot project in children with severe bleeding disorders is promising because the results show an improvement of joint function and HRQoL accompanied by an unchanged ABR and the preservation of physical fitness.



Publikationsverlauf

Artikel online veröffentlicht:
13. November 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany