The burden of living with pure HSP

Posted - May 2020 in Living with HSP - Management & Treatment News

Large survey conducted in the Netherlands

 

The greatest burden identified by people with pure HSP is from muscle stiffness, standing and walking, and leg and/or back pain.

More than half reported falling at least twice a year and half reported being injured from falling. 75% reported fear of falling.

109 people with pure HSP in the Netherlands responded to an HSP-specific survey aimed at quantifying the burden of HSP to individuals and to better understand their needs.

 

Abstract

Background: Hereditary spastic paraplegia (HSP) is a group of inherited disorders characterized by progressive spastic paresis of the lower limbs. Treatment is often focused on reducing spasticity and its physical consequences.

To better address individual patients’ needs, we investigated a broad range of experienced complaints, activity limitations, and loss of motor capacities in pure HSP. In addition, we aimed to identify patient characteristics that are associated with increased fall risk and/or reduced walking capacity.

Methods: We developed and distributed an HSP-specific online questionnaire in the Netherlands. A total of 109 out of 166 questionnaires returned by participants with pure HSP were analyzed.

Results: Participants experienced the greatest burden from muscle stiffness and limited standing and walking activities, while 72% reported leg and/or back pain. Thirty-five and 46% reported to use walking aids (e.g. crutches) indoors and outdoors, respectively; 57% reported a fall incidence of at least twice a year (‘fallers’); in 51% a fall had led to an injury at least once; and 73% reported fear of falling. Duration of spasticity and incapacity to rise from the floor were positively associated with being a ‘faller’, whereas non-neurological comorbidity and wheelchair use were negatively associated. Higher age, experienced gait problems, not being able to stand for 10 min, and incapacity to open a heavy door showed a negative association with being a ‘walker without aids’ (> 500 m).

Conclusions: Our results emphasize the large impact of spastic paraparesis on the lives of people with pure HSP and contribute to a better understanding of possible targets for rehabilitation.

 

SOURCE: Orphanet J Rare Dis. 2020 Mar 4;15(1):64. doi: 10.1186/s13023-020-1338-4. PMID: 32131864

 

Experienced Complaints, Activity Limitations and Loss of Motor Capacities in Patients With Pure Hereditary Spastic Paraplegia: A Web-Based Survey in the Netherlands

 

Bas J H van Lith 1Hans C J W Kerstens 2 3Laura A C van den Bemd 4Maria W G Nijhuis-van der Sanden 3Vivian Weerdesteyn 5Rob J E M Smeets 6Klemens Fheodoroff 7Bart P C van de Warrenburg 8Alexander C H Geurts 5

1 Department of Rehabilitation, Donders Institute for Brain, Cognition and Behaviour, Radboud university medical center, PO Box 9101, 6500, HB, Nijmegen, The Netherlands. [email protected].

2 HAN University of Applied Sciences, PO Box 6960, 6503, GL, Nijmegen, The Netherlands.

3 Radboud Institute for Health Sciences, Scientific Institute for Quality of Healthcare, Radboud university medical center, PO Box 9101, 6500, HB, Nijmegen, The Netherlands.

4 Department of Rehabilitation, Sint Maartenskliniek, PO Box 9011, 6500, GM, Nijmegen, The Netherlands.

5 Department of Rehabilitation, Donders Institute for Brain, Cognition and Behaviour, Radboud university medical center, PO Box 9101, 6500, HB, Nijmegen, The Netherlands.

6 Department of Rehabilitation Medicine, Maastricht University, Research School CAPHRI, PO Box 616, 6200, MD, Maastricht, the Netherlands.

7 Gailtal-Klinik, Radnigerstrasse 12, 9620, Hermagor, Austria.

8 Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud university medical center, PO Box 9101, 6500, HB, Nijmegen, The Netherlands.

Comments on this story

  1. Robbie posted at 10:44 pm on 30 August 2020Reply

    I’m so ready to get back to healthy. 😥 😥

  2. Robbie posted at 10:56 pm on 30 August 2020Reply

    My HSP is getting worse off. I’m gonna to do my best to stay above water. I believe the drs I see 😐 😐

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