HSP affecting Quality of Life

Study of HSP

I found a research paper from 2016 which looks at the differences between people with HSP and a control population to evaluate the burden of HSP. The study was undertaken in Norway, comparing 108 people over 30 yrs old with HSP against an age and gender matched sample from a study of 46 thousand people. 

The paper is called “Health survey of adults with hereditary spastic paraparesis compared to population study controls”, by Krister W. Fjermestad, Øivind J. Kanavin, Eva E. Næss, Lise B. Hoxmark and Grete Hummelvoll. You can read the full paper here: https://ojrd.biomedcentral.com/articles/10.1186/s13023-016-0469-0. I've taken my highlights from the paper and repeated below.

The study is a broad survey of health and everyday life domains among persons with HSP, including life satisfaction, mental wellbeing, social support, problems with sleep, memory, pain, gastrointestinal/urinary functioning, and ability to perform activities of daily living (ADL). 

The HSP sample more frequently lived alone. Overall, the HSP sample reported lower life satisfaction, lower mental wellbeing and lower social support, as well as poorer memory and sleep, compared to controls. Furthermore, the HSP sample more frequently reported musculoskeletal pain, constipation, and urinary incontinence compared to controls. There was no difference between samples in frequency of physical activity and alcohol and tobacco use. Men with HSP reported higher impact of HSP, lower life satisfaction, and less ability to perform activities of daily living compared to women with HSP.

Adults with HSP experience disease burden on a larger number of areas than previously documented, and men with HSP may represent a particularly vulnerable group.

Results

All of the comparisons in this section are with the control sample.

Participants in the HSP sample less frequently lived with a partner/spouse and more frequently lived alone. There was no difference between the samples in frequency of living with parents or children.

Sleep

Study considers daytime drowsiness, frequent night awakenings, trouble falling asleep and waking up early. People with HSP reported more sleep problems on all items

Pain

Study considers chronic pain of >3 months duration in the past year. The most frequent pain sites are: feet, knees, lower back, and hips. People with HSP confirmed more frequent musculoskeletal pain, more frequent pain in the lower body pain sites and less frequent pain in the upper body pain sites.

Comorbid disease prevalence

The most frequently reported diseases were mental health problems, osteoarthritis, hand eczema, psoriasis, asthma and brain hemorrhage. People with HSP more frequently reported brain hemorrhage and psoriasis.

Gastrointestinal problems

The study considered constipation, alternating constipation and diarrhea, bloating,  heartburn, diarrhea, nausea and fecal incontinence. People with HSP more frequently reported much problems on alternating constipation and diarrhea, constipation and fecal incontinence.

Urinary problems

The study considered urinary incontinence. People with HSP more frequently reported urinary incontinence 

Oral health

There was no difference in oral health but people with HSP reported more frequent dental visits during the last year compared to controls.

Physical activity

Study considered the frequency of physical activity (daily, 2–3 times pr. week, once a week, less than once a week and never). There is no difference in frequency. Those with HSP spent more hours sitting daily compared to controls.

Medication use

The study considers the percentage of participants taking nonprescription medicines 1–3 times weekly: for general pain, constipation, headache and heartburn. People with HSP more frequently reported taking medication for constipation and general pain. Otherwise no difference.

Alcohol and tobacco use

There was no difference for participants drinking alcohol at least 2–3 times pr. week, never drinking alcohol and smoking daily.

Social support

The study looked at the percentage of participants who confirmed practical support and emotional support. those with HSP reported lower practical support and emotional support.

Mobility and activities of daily living

In terms of mobility, 35 % reported to walk without aids outdoors, while 56 % reported to walk without aids indoors. Around a third (31 %) reported to use a wheelchair indoors, while 45 % reported to use a wheelchair outdoors. The majority (80 %) confirmed having a driver’s license. 

Men reported more activities they could not perform without assistance compared to women. Fisher’s exact tests showed that the two activities men reported to be able to perform less frequently compared to women were simple household chores and laundry.

Frequency of falling

In the HSP sample, 47 % reported to have fallen in the last 3 months. There was no gender difference in frequency of falling and no significant age difference between those who confirmed having fallen and those who did not.

HSP sample medication use

In the HSP sample, 15 % reported using Botox injections, 10 % reported using a baclofen pump, and 33 % reported taking oral spasmolytics. Of these, the percentages of participants reporting having some or large effect of the medication were 83 % for Botox, 86 % for baclofen pump, and 82 % for oral spasmolytics.

Gender differences within the HSP sample

There were no significant gender differences on any of the variables shown in Table 2, except overall life satisfaction. Males rated significantly lower life satisfaction compared to females

Pure versus complex HSP

There was no difference between the proxy pure/complex types in terms of age, total body impact, pain, mental well-being, memory, gastrointestinal/urinary problems, number of additional diseases, BMI, or physical activity.

Discussion

Compared to controls, persons with HSP reported lower scores on life satisfaction, mental wellbeing, as well as perceived practical and emotional support. Furthermore, compared to controls, persons with HSP reported more problems with memory, sleep, gastrointestinal and urinary function, and pain in the lower body. The results showed persons with HSP experience large total physical impact of their disorder. This total impact was significantly correlated with age, mental wellbeing, memory problems, gastrointestinal problems, extent of pain, number of co-morbid diseases, and life satisfaction. Thus, the disease burden for adults with HSP is multifaceted, and involves problem areas not previously documented.

Our results also showed considerable impact on activities of daily living for persons with HSP. Over half the sample reported not being able to take the bus, and nearly half the sample reported not being able to do more than basic house chores. They were surprised to find that age was only correlated with total physical impact and ability to perform ADL, and not with any other health-related variable. This implies that the burden of disease experienced by adults with HSP is considerable across the lifespan. Importantly, older participants reported less practical support compared to younger participants, possibly indicating a particular need for the older HSP group.

There are some important gender differences within the HSP sample. Specifically, men reported significantly higher overall impact of HSP, higher impact on sexual function, more ADL they could not perform without assistance, and lower overall life satisfaction compared to women with HSP. In summary, the results indicate that men with HSP represent a particularly vulnerable group in terms of overall HSP impact and quality of life.