A journey to HSP/FSP: My On-line Research

I have undertaken ten on-line surveys (annually since 2013) for people with HSP. These were reported on Rare Disease Day the following year. The initial surveys each had around 100 respondents predominantly from the

USA

and the

, but also Europe,

Australia

and other places. Later surveys have had some 300-500 respondents, with more from Europe and Brazil.

Index for full blog pages for each survey:

2021: https://hspjourney.blogspot.com/2022/02/2021-survey-results.html

2022: https://hspjourney.blogspot.com/2023/02/2022-survey-results.html

The following table gives an overview of which topics have been covered in each year, you can click to get to the relevant post:

Year	13	14	15	16	17	18	19	20	21	22
Activities of Daily Living						ü
Bladder				ü					ü
Bowel				ü
Coronavirus								ü
Cure for HSP / Research								ü	ü	ü
Depression			ü		ü				ü	ü
Disclosure										ü
Disabled Y/N?					ü	ü	ü	ü	ü	ü
Employment					ü					ü
Exercise		ü					ü
Fatigue				ü
Frequency seeing professionals								ü
Funding										ü
Grief/Stigma/Acceptance									ü
Home modifications			ü
Life with HSP							ü	ü	ü	ü
Lifespace/ Distance of travel							ü
Medication		ü					ü
Menstrual health										ü
Misdiagnosis	ü					ü
Mobility and aid use	ü	ü	ü	ü	ü	ü	ü	ü	ü	ü
Multiple Long Term Conditions						ü
Needs								ü
OXPAQ								ü
Pain					ü					ü
Quality of life			ü
Relationships/ Friendships									ü	ü
Resources/ Information	ü			ü					ü	ü
Sleep						ü
SPRS questions			ü
Standing from chair			ü					ü		ü
Supplements		ü
Support groups						ü
Surgery								ü
Symptoms and tracking	ü					ü
Trips and falls									ü
Walking					ü
Walking distance			ü		ü	ü	ü	ü	ü	ü
Wellbeing					ü	ü	ü	ü	ü	ü

2022: Linking Wellbeing with Mobility, Pain, Menstrual Health and life with HSP.

513 respondents completed an on-line survey between September and December 2022, predominantly from the USA, the UK, Brasil, and France. 234 of these had also completed at least one other survey. 14% of respondents use no mobility aids, and 19% use them some of the time. Others use aids all or most of the time: 36% using sticks, 12% using frames and 19% using wheelchairs. Around 80% consider themselves disabled, with around 20% not considering themselves disabled. Mobility is often the main factor determining if someone feels disabled. Some people who have completed previous surveys have had a genetic test for HSP since their first survey, indicating that their diagnosis journey continues over time.

Respondents completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS) to assess wellbeing, with an average score of 45.6, in the ‘average wellbeing’ range. Respondents completed the PHQ2 depression screening tool. Around a quarter score 3 or more points, indicating they screen for depression.

Walking sticks/canes were identified most often as people’s favourite mobility aids amongst a wide range. Those who have negative feelings about using mobility aids tend to have a lower wellbeing. People appear to spend the longest time using either walking sticks or wheelchairs. Mobility aid use can vary significantly in the earlier stages of HSP as people learn about their altering gait. Benefits from aids include increasing independence, reducing symptoms and enjoying doing more. Many have uncertainty about the types of mobility aid they will need to use in the future. It is difficult to estimate how long people would spend with any particular mobility aid based on data from people answering mobility questions more than once.

Around three quarters of people indicate they get pain from their HSP. Respondents completed the Short- form McGill Pain Questionnaire 2 (SF-MPQ-2). The average score for people with pain from HSP is 3, higher for those using mobility aids. The most common description of pain from HSP is ‘tiring or exhausting’, followed by ‘cramping pain’ and ‘aching pain’. Most people describe their pain intensity from HSP as ‘discomforting’, and those that describe this as ‘distressing’, ‘horrible’ or ‘excruciating’ have a lower wellbeing. Overall, most people note their pain from HSP is in their legs, feet, back and hips. Looking at changes in pain since 2017, most people completing these questions in both years have no change or a small change in pain, and there may be evidence suggesting some get accustomed to their pain.

People were asked how the common HSP symptoms of spasticity, pain, fatigue and mental health varied during their menstrual cycle. Over half of people felt that their mental health was worse during their menstrual cycle, and around a third thought that their spasticity, pain and/or fatigue were worse during their menstrual cycle. Those with worse spasticity, fatigue and/or mental health tend to have a lower wellbeing than others. A few people also find that their bladder and/or bowel symptoms are more affected during their cycle. For a few with earlier onset HSP there are some reasons why people avoid using tampons.

People identified one piece of advice for others with HSP. The most common answers were around staying positive and keeping moving. Many people identified things now hard or impossible to do, including answers around movement and activities with other people. There are many with HSP who find making or keeping friends hard, which leads to a lower wellbeing. Similarly, people can have lower wellbeing when they find it hard to tell their friends or work colleagues about their HSP.

When thinking about research priorities for HSP many answers are around treatments/cures for aspects of HSP. It is often felt that healthcare professionals do not have enough HSP information to confidently deal with people seeking help on how their HSP can be treated or how their symptoms are likely to progress. When looking at funding, there are similar numbers using mobility aids and medication, and many use both. More than half have needed adaptions and improvements around their home, and around a quarter of people need adapted cars and/or carers at their homes. The four most common methods of funding for these things are Government benefits, Savings, Insurance and Health Services.

Action can be taken to improve wellbeing, including reviewing medications, mobility aids and exercise routines to manage HSP appropriately over time. Other areas associating strongly with wellbeing include:

Reducing depression,
Pain management,
Seeking adjustments at work or alternative jobs.
Friendships, conversations and views on the opinions of others

Blog post: https://hspjourney.blogspot.com/2023/02/2022-survey-results.html

Full analysis: https://drive.google.com/file/d/1eWWNwPDR6IcCHT6a6aXHRCoy3Trw1zZe/view

2021: Relationships, Acceptance, Life with HSP and Bladder issues.

565 respondents completed an on-line survey between October and December 2021, predominantly from the USA, Germany, Brasil, and the UK. 195 of these had also completed at least one other survey. 16% of respondents use no mobility aids, 19% use them some of the time. Others use aids all or most of the time: 29% using sticks, 15% using frames and 19% using wheelchairs. Up to 14% have experienced significant changes in mobility over a five year period. Around 80% consider themselves disabled, with around 20% not considering themselves disabled. Mobility is not the only factor determining if someone feels disabled.

Respondents completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS) to assess wellbeing, with an average score of 46.0, in the ‘average wellbeing’ range. Respondents completed the PHQ2 depression screening tool. Around a quarter score 3 or more points, indicating they screen for depression.

When looking at relationships, there is a larger proportion of people having positive experiences than having negative experiences. There are large differences in wellbeing for those having positive experiences compared with those having negative experiences. Key factors are the perceptions of family about the impacts of HSP. People (72%) feel stigma associated with their HSP, and this is most often perceived to be due to lack of understanding about HSP or the symptoms of HSP.

Respondents completed the Acceptance of Illness Scale (AIS). The average acceptance of people with HSP is 21.7, near the change-over between a low and moderate level of acceptance. There is a strong link between acceptance and wellbeing. Those able to accept their HSP have wellbeing significantly above average, and those having low acceptance of their HSP have wellbeing significantly below average. People may regard themselves as disabled before they accept that they have HSP.

Over 90% of people with HSP have problems with balance, tripping and falling, and 80% of people with HSP scuff their feet. Almost a third of people who lose their balance do so on a weekly basis, with around a quarter doing so more frequently. Around half of people tripping up do so on a weekly or monthly basis, with one in five doing so more frequently. Over half of people have fallen down a few times, with around one in five people falling on a monthly basis. A small proportion fall down on a daily basis.

Using the ICIQ overactive bladder questionnaire more than 80% of people with HSP have some degree of bladder issues. Bladder issues can affect people who do not use mobility aids, just as there are some who use mobility aids all or most of the time who do not have any bladder issues. Those with SPG7 tend to be more affected by bladder issues than others.

The majority with bladder issues have mild effects, with less than one in ten having severe effects. Needing to rush to the toilet and having urine leak before getting to the toilet are common issues. Most feel their bladder does not empty fully at least some of the time. A range of techniques are used to manage bladder issues, with the most common ones being taking anti-spasticity medication and drinking less fluids.

There is a trend showing lower wellbeing for those with increased bladder effects. Within these results, those who always have to rush to the toilet have the lowest wellbeing, and those not having to rush have the highest. Those with urine leaking before they get to the toilet all or most of the time also have low wellbeing.

Action can be taken to improve wellbeing, including reviewing medications and exercise routines to manage HSP changes appropriately over time. Other areas associating strongly with wellbeing include:
·         Working towards acceptance of HSP,
·         Reducing depression,
·         Being more comfortable talking about HSP with important people,
·         Helping family, partners and friends understand the impacts and consequences of HSP.

Around a third of respondents have taken part in HSP research studies. Almost 90% of people would like to take part in future projects, but only 30% of people know how to find out about upcoming trials. People want research to improve learning, some for themselves, others for research teams. People want research to identify more/better treatments for HSP, and a cure.

HSP information from neurologists and support groups is trusted a great deal or quite a lot by over 80%. Similar information from doctors is trusted not very much or not at all by 48%.

Blog post: http://hspjourney.blogspot.com/2022/02/2021-survey-results.html

Full analysis: https://drive.google.com/file/d/1eB5CHux-V-S91vs3DRujuZP-znsHx0u9

2021: Wellbeing overview.

This post reports an analysis of wellbeing factors from surveys 2017-2020.

https://hspjourney.blogspot.com/2021/04/wellbeing-factor-overview.html

2020: Needs, Coronavirus, OxPAQ and Life with HSP.

317 respondents completed an on-line survey October to December 2020, predominantly from the UK, Brasil and the USA. 170 of these people had also completed at least one other survey. 17% of respondents use no mobility aids, 19% use them some of the time. Of those that use aids all or most of the time: 32% use sticks, 14% use frames and 18% use wheelchairs. Up to 12% have experienced significant changes in mobility over a five year period.

Needs were explored for people with HSP, grouped into five topics:

Treatment needs are important in all groups of people with HSP.
Meeting needs around ‘Living with HSP’ has the potential to benefit the greatest number of people, and is important for young people with HSP and for those providing support to those with HSP.
The types of needs change for different groups of people and change as people progress through different points on their HSP journeys.

The needs data can be used by HSP support groups, professionals, and supporters to identify where efforts should be focussed to ensure that the identified needs are being met. The top needs in each category are:

Topic	Top Needs
Diagnosis and Inheritance	Explanation of HSP on diagnosis
	Signposting about potential future needs
Living with HSP	Stretches and exercises that help
	Information on appropriate physical activities to take part in
Treatment needs	Treatment options for: spasticity, pain, bladder issues, fatigue
	Better information on existing treatments
Information about HSP	Predicting how HSP will progress in individuals
	Understanding the burden of HSP symptoms
Wellbeing	Information on improving mental health/wellbeing
	Information on finding/accessing relevant healthcare professionals

Coronavirus has increased access to digital healthcare. For those with HSP the key benefits for digital appointments are reduced effort and time travelling, while the key disadvantage is having less chance to show symptoms. Most consider face-to-face appointments to be better than digital appointments.

Four fifths of people consider themselves to be disabled and one fifth do not. Mostly, people think a cure for HSP would reverse their symptoms. The most common surgeries for HSP are tendon release and having a baclofen pump installed. Around one in five have had surgery for their HSP.

About a third of people consider medical professionals do not understand what it is like for them to live with HSP. 15% have avoided or delayed advice because of embarrassment about HSP. More than half feel they have not been believed about the severity of symptoms, or feel they have not been taken seriously.

Respondents completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS) to assess wellbeing, with an average score of 42.9. The average wellbeing is lower than shown in previous years. It is possible to take action to improve some factors associated with lower wellbeing which may result in an improvement in wellbeing. Factors with the strongest associations with wellbeing include:

·         Individuals understanding about HSP,
·         Embarrassment about HSP leading to delaying seeking advice,
·         Medical professionals understanding HSP and its effects on people,
·         Seeing relevant medical professionals at appropriate intervals.

There are strong overlaps between important wellbeing factors and the most important needs identified.

Respondents completed the Oxford Participation and Activities Questionnaire (Ox-PAQ) which measures participation and activity. Overall results for the HSP population are similar to those published for motor neuron disease, multiple sclerosis and Parkinson’s. Progression through mobility aids affects scores in the Routine Activities domain and Social Engagement domain, whereas the Emotional Wellbeing domain scores are independent of mobility aids.

Post: https://hspjourney.blogspot.com/2021/02/2020-survey-results.html

Country breakdown of needs: https://hspjourney.blogspot.com/2021/02/2020-survey-results-country-specific.html

Full analysis: https://drive.google.com/file/d/1Ect9mdluhSY3xGo8MZLezp22hk435l5r

2019: Medication, Activities, Travel Range and Life with HSP.

367 respondents completed the survey, predominantly from the USA, UK and Brazil. 150 of these had also completed at least one other survey, of which 22 had completed both the 2014 survey and this 2019 survey. Respondents completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS) to assess wellbeing, with an average score of 47.4. Although it is a small sample (7), those who do not know if they have HSP have a lower wellbeing score, suggesting diagnosis uncertainties have a negative impact on wellbeing. 20% of respondents use no mobility aids, 24% use them some of the time. Of those that use aids all or most of the time: 28% use sticks, 11% use frames and 19% use wheelchairs. Those with quicker the rate of change of mobility have a lower the wellbeing score. Less than 20% experience significant changes in mobility over a five year period.

Those who do are the most mobile take the least medication although there are several whose mobility is not affected who take several medications. For those whose mobility is affected by HSP there is a reasonable spread of numbers of medication taken across mobility bands. The most common number of medicines taken is 1 or 2. The most common medication taken is Baclofen (taken by about half of people). Other medicines taken regularly (each taken by less than 10% of people) include Botox, Gabapentin, Clonazepam, Oxybutynin and Tizanidine.

One fifth of medication is being taken for pain. One eight of medication is taken for bladder issues. Around 3% of medication is being taken for depression.

There is no consistent source of information for the treatment of HSP symptoms. Several of the more commonly used medications do not have HSP specific trial results published, including Clonazepam, Diazepam, Amitriptyline and others, which may prevent their consideration by medical practitioners who are not so familiar with HSP.

People identified the most important activities for exercise to be stretching, walking and aerobic activities. Music and stretches were identified as most important for relaxing. Stretches and physiotherapy were identified as most important for flexibility.

Almost all leave their bedroom every day and about three quarters go outside every day. Less than half go to their neighbourhood every day, and travel further is less frequent than this. The highest wellbeing is found in those people who travel the furthest every day. Patterns suggest that help sought to be mobile is more than just transferring and a broader level of assistance is given covering many aspects of movement.

Two thirds of people consider themselves to be disabled and one third do not. One third of people with HSP consider themselves to be lonely or isolated which tends to occur more often in those whose mobility is more restricted. Around half consider themselves to be vulnerable, and around one in seven people have suffered from abuse. Approximately three quarters of those who suffered from abuse indicated that this was as a result of their HSP.

Several factors are identified which often result in lower wellbeing. It is possible to take action to improve some of these factors which may result in an improvement in wellbeing.

Post http://hspjourney.blogspot.com/2020/02/2019-survey-results.html.

Full Analysis: https://drive.google.com/open?id=1Vrh5bd8ss0XDU_DR39kEpneo-3YZuaGm

2019:Poster for 2019 Spatax Meeting

In 2017 potentially significant wellbeing differentiators were identified:

• People without pain from HSP have a better wellbeing than those who get pain from HSP.

• People content with their employment/occupation have a better wellbeing than those not content.

• People whose employers are not supportive of HSP situations tend to have a lower wellbeing.

• Those who can walk as far as they want and those unable to walk have a better wellbeing.

In 2018 three symptoms showed significant negative wellbeing effects; depression, stress, and HSP affecting learning/memory. Three further symptoms showed potentially significant negative effects; poor co-ordination, regular falls, and back/hip pain.

Several factors have large wellbeing effects, but many are not direct HSP symptoms. Help or treatment in these may result in improved wellbeing. Potential areas include;

• Decreasing: Depression, stress, anxiety, worries

• Lifestyle or other changes to improve sleep

• Reductions in pain

• Strength/balance training to reduce falls

• Activity/exercise to maintain mobility as long as possible

Post: https://hspjourney.blogspot.com/2019/11/spatax-meeting-poster-2019.html

2018:Symptoms, Sleep, Activities of Daily Living, Support and Diagnosis.

There were 304 respondents with Hereditary Spastic Paraplegia (HSP) predominantly from the USA, the UK and Brazil.

Around one third of respondents had been diagnosed with another condition before HSP. Six conditions were listed at least five times and are likely to be misdiagnoses; Cerebral Palsy, Multiple Sclerosis, Ataxia, Arthritis, Herniated disc and Neuropathy. Around one third of respondents are living with multiple long-term health conditions. There were five conditions affecting at least five people: High blood pressure, Depression, Arthritis, Asthma and Diabetes.

Using the Warwick-Edinburgh Mental Well-being scale the average wellbeing score is 45.9. This is lower than UK population norms however is still within the “average” definition and similar to those with a self-perceived health status of “poor”.

When recalling changes in mobility over five years, around 20% of patients may experience significant changes in mobility. The quicker a persons change in mobility the lower their wellbeing is likely to be. A longitudinal analysis of 38 respondents who completed surveys over four or five years shows most have a low change in mobility over a four or five year period, and less than 10% have a high change in mobility.

Respondents were asked to rate how much they are affected by each of a list of 36 HSP symptoms. There are five symptoms affecting almost everybody (loss of balance, muscles being stiff, difficulty running, difficulty walking, and difficulty using stairs). Respondents described which symptoms gave the greatest effect or had the biggest impact. Fatigue has the greatest effect for about a third of respondents.

Difficulty walking and loss of balance each affect about a fifth of respondents. Muscles feeling stiff, bladder problems and back/hip pain each affect approximately a sixth of respondents the most. Three symptoms (depression, stress and affecting learning or memory) show significant negative wellbeing effects, and three symptoms show potentially significant negative effects (poor co-ordination, regular falls and back/hip pain).

Of the 21 highest impact symptoms, all respondents had at least 5 and no respondents had more than 18. Across mobility bands the number of symptoms remains broadly the same but the effect of symptoms increases. A longitudinal examination of 13 respondents who answered symptom questions in 2013 and 2018 shows that not all symptoms persist from year to year and the number of symptoms tends to increase.

Respondents used a modified Global Sleep Assessment Questionnaire to describe their sleeping patterns, identifying if they are affected by 16 different sleep factors. The majority of respondents are affected by at least one sleep factor. Respondents affected by at least 6 factors (approximately one quarter) are shown to have a significantly lower wellbeing. There is no significant variation in sleep factors with mobility score, indicating that sleep factors are generally independent of mobility aids used.

Respondents answered the Katz Index of Independence in Activities of Daily Living (ADL). Over half of respondents are fully independent. The most common factor affecting people is their continence, their control of bladder or bowel. There are no clear patterns between ADL factors and the use of mobility aids.

Around 60% of respondents are members of a support group and 40% are not. The most common type of support groups identified were Facebook groups. Three key benefits people get from group membership are; getting to know other people with HSP, getting knowledge about HSP, and sharing stories. There is no difference in wellbeing between those that are members of support groups and those that are not.

Several different factors have been shown to have large or significant effects on wellbeing, either positive or negative. Many of the factors identified are not direct HSP symptoms and it is possible to get help or treatment for some of these. Getting such help/treatment may result in improved wellbeing. Consultation with a doctor or other medical professional is needed for advice on the best course of action. Potential areas where changes could be made include:
• Decreasing: Depression, stress, anxiety, worries
• Lifestyle or other changes to improve sleep
• Reductions in pain
• Strength/balance training to reduce falls

• Activity/exercise programme to maintain mobility for as long as possible

Blog post: https://hspjourney.blogspot.com/2019/02/2018-survey-results.html

Full analysis: https://drive.google.com/file/d/1YaQsuXV7yhz1b5gVsUYLJT_rcAed-g0q

2017: Pain, Walking, Occupation and Disability, Wellbeing:

There were 222 respondents who completed the survey, predominantly from the USA and the UK, but also from Europe, South Africa and India. About a third of respondents had completed at least one of my previous surveys. Seven people have completed all five surveys. Of those who know their type of HSP, SPG4 and SPG 7 were most common.

There are a range of questions for each topic. I have designed my own questions for occupation and walking factors. Pain is assessed using the Short Form McGill Pain Questionnaire 2, with extra questions on where the pain is felt and how you treat it. Wellbeing is assessed using the Warwick-Edinburgh Mental Well-being Scale (WEMWBS) for assessing positive mental health and the Patient Health Questionnaire (PHQ2) used as a screening tool for depression.

211 respondents answered the pain questions. Of these 170 (81%) said that they get pain from HSP whereas 41 (19%) do not. The proportion of people who get pain from HSP is similar across the mobility bands. Those who use walking sticks all or most of the time get pain most often from HSP, and those who use frames all or most of the time get the pain least often from HSP.

Most people use medication, either prescription or over the counter, or physical therapy, either exercise, manipulation or massage to relieve pain. Heat and cold therapy, relaxation techniques and TENS machines are other methods used by at least 10% of respondents. The average number of methods used to treat pain is 3, and the most common number of methods used is 2. Three quarters (74%) of people use more than one method to control their pain. 13 respondents (8%) do not use any pain control.

Over 95% of people who have pain with HSP have some pain in their legs, and over 90% have pain in their feet. For legs and feet the common descriptors for pain intensity are 'discomforting' and 'distressing', accounting for more than half of respondents. Over 80% of people who have pain with HSP have some pain in their back and hips where the common description for pain intensity is 'discomforting'.

Taking a broad view of walking with HSP, the results shows that as a persons distance becomes limited the factors which affect walking each become more important. The factors which affect people walking the most seem to be:

Where stairs/steps are involved
Tiredness/fatigue
Going over uneven ground
Carrying something

Looking at combinations of factors, if people are affected by fatigue or tiredness, or they are in a rush, then walking is often more difficult. These factors are mentioned in combination with the widest number of factors indicating that they are key factors.

Looking at if people consider themselves disabled, 84% said they did consider themselves disabled, and 15% said they did not.

When people are in employment, results showed that if employers are positive to HSP then people are more likely to be content with their situation, and if employers are negative about HSP then people are less likely to be content with their situation. Respondents answered about changing jobs or stopping working as a result of HSP, and about two thirds of people have had to. The people who have had to change jobs the most are those who use walking frames all or most of the time, with more than 9 in 10 having to change jobs, followed by those who use wheelchairs all or most of the time at 7 in 10. Roughly two thirds of people think they will have to change jobs or stop working early in the future as a result of HSP.

There was a range of positive, negative and mixed consequences about changing jobs. Negative consequences account for about three quarters of responses, with the positive accounting for about one sixth.

By far the biggest negative consequence was financial, with about a third of respondents saying they had less money. Several (about 1 in 7) people said that they retired before they were ready to, and a similar number said they were more alone. Others commented that they were bored, frustrated or depressed and other that they were not making as much of a contribution or being a bigger burden. A few people said that they liked their current job less than their previous job. Positive consequences included several describing themselves in a “better situation”, with others specifying less pain, less fatigue, fewer falls, and others saying they felt less of a burden on others.

Respondents also answered questions on wellbeing. The average wellbeing score is lower than UK population norms, but still within the “average” definition and similar to those who perceive their health status as “poor”. The average score is more than 5 points below the UK population norms, suggesting a significant effect.

There are a number of factors where there is more than a 5 point difference between the average of each answer, which may be am indicator of a significant differentiator in wellbeing. These are:

People who do not get pain from HSP have a better wellbeing than those who get pain from HSP.
People who are content with the employment/occupation situation tend to have a better wellbeing than those who are not.
People whose employers are not supportive of HSP situations tend to have a lower wellbeing.
Those who can walk as far as they want have a better wellbeing, and those who are unable to walk are likely to have a better wellbeing.

Blog post: http://hspjourney.blogspot.com/2018/02/2017-survey-results.html

Full analysis: https://drive.google.com/open?id=1d5H11BsMHSQplf0foiJrE6hQrXedUC6w

2016: Fatigue, Bladder and Bowel Issues, HSP information sources:

My fourth on-line survey took place between September 2016 and January 2017. 169 respondents with Hereditary Spastic Paraplegia (HSP) completed the survey, predominantly from the USA and the

. The survey covered fatigue, bladder and bowel effects, and sources of HSP information. Respondents also answered questions about their mobility allowing trends to be spotted with level of mobility.

Three short form fatigue questionnaires were selected as they have previously been used to assess fatigue in HSP or similar conditions:

Neurological Fatigue Index (NFI-MS, a 9 question survey)
Modified Fatigue Impact Scale (MFIS-5, a 5 question survey)
Brief Fatigue Inventory (BFI, a 9 question survey)

The MFIS-5 questionnaire shows that whilst the physical factors of HSP contribute the most to fatigue there is also an important cognitive aspect. Those with SPG7 tend to have a higher level of fatigue than those with SPG4. A 21 question version of the MFIS questionnaire was previously used to assess fatigue in people with SPG4 in

Brazil

, and these results are similar. The BFI questionnaire shows that around 90% of people with HSP have moderate or severe fatigue.

The average scores for all of the questionnaires show that fatigue is generally independent of mobility. Those respondents who do not use mobility aids tended to score slightly lower fatigue levels than those who use mobility aids all or most of the time, but there is a wide spread of data, and there are some who do not use mobility aids who have higher levels of fatigue than those who use mobility aids all the time.

The ICIQ-OAB and parts of the ICIQ-B questionnaires were used to assess bladder and bowel effects. The results of the bladder questions appear to be broadly in line with two previous studies examining bladder issues in people with HSP, from France and

Estonia

Two thirds of people with HSP have no or mild bladder effects, and around one third have moderate or severe effects. There is a general trend that the milder effects tend to be with those with lower mobility issues, and the effects tend to be more severe with those with higher mobility issues. The key bladder symptoms appear to be frequency and incontinence, affecting the greatest proportion of respondents. Many respondents are bothered quite a lot by these symptoms.

Around three fifths of people with HSP have mild bowel effects, and around one third have moderate effects. Severe effects are not reported. There is a general trend that the milder effects tend to be with those with lower mobility issues, and the effects tend to be more severe with those with higher mobility issues. The key bowel symptoms are urgency and hesitancy, affecting the greatest proportion of respondents. Those with more symptoms tend to have higher levels of bother about these symptoms.

Most people get their information about HSP from social media, doctors and neurologists. The sources which people regarded as most trustworthy were neurologists, support groups and physiotherapists.

Respondents identified factors of ‘accuracy’ and ‘being backed up by research’ as being most important when evaluating information sources. Neurologists, doctors and medical journals scored highest on these factors. Work on patient doctor relationships may be needed so that doctors understand patient situations better.

Blog post: http://hspjourney.blogspot.co.uk/2017/02/2016-survey-results.html

Full analysis: https://drive.google.com/open?id=0BzEoTkR5HCWhSEQ1UElwamVod3c

2016: Poster for International HSP meeting

https://drive.google.com/file/d/0BzEoTkR5HCWhNzllZWZWR05qWk0/view
Note, some browsers do not appear to like this file. You should be able to download the file and view in acrobat reader v8 or v9.
A more compatible, but bigger file size version is here: https://drive.google.com/file/d/0BzEoTkR5HCWhTklWZFNDWGlSYlU/view

2015: Modifications at Home, Depression and Quality of Life

Respondents indicated that there is a wide range of modifications that they have made around their properties. Modifications tend to be made after an accident or after noticing a change in mobility/symptoms, although some people are making modifications early and are planning for future changes. Frequently, the first modifications made are the installation of grab rails within the property, and these are often fitted in the bathroom first. Subsequent modifications are made depending on the rate of progression of HSP. The parts of properties which are modified the most after the inclusion of grab rails are the bathroom/toilet with a range of different modifications made. Adjustments to beds are also relatively common. Respondents indicated the benefits from the different modifications they had made.

People with HSP appear to suffer from depression more than the general population. Respondents completed the PHQ-2 depression screening questionnaire, which showed that around a quarter should seek further assessment. Results have been compared with the 2009 Estonian study into depression with HSP, and a similar proportion of people with scores of zero, indicating no depression, is shown.

Respondents also completed a sample of questions from the Patients Like Me Quality of Life survey and it is concluded that HSP appears to affect quality of life. From the data there appears to be two step changes in quality of life. The first step change is in social functioning at the point when mobility aids are needed and the second step change is in physical functioning when mobility aids need to be relied on most or all of the time.

Blog post: http://hspjourney.blogspot.co.uk/2016/02/2015-survey-results.html

Full analysis: https://drive.google.com/file/d/0BzEoTkR5HCWhRjMtejZhSGpHSG8/view

2014: Medication, Diet, Exercise and Relaxation

The results showed that around three quarters of people are prescribed at least one form of medication for their HSP. Of those who do not take medication around half indicated that they have never been on medication for HSP with the others having previously been prescribed at least one medication, but no longer take any either because of side effects, because the medication was not effective or a combination of both. Medication is most commonly taken for Spasticity, Pain, Bladder, Spasms, Depression and Nerve Pain. Respondents indicated their medications, their perceptions of the benefits and any side effects.

Almost half of the medication being taken is used to treat spasticity and spasms. The biggest proportion of this group of medications comprises people taking Baclofen (half of people). Other spasticity/spasm medications, with at least 5 respondents taking are: Botulinum toxin A / Botox / OnabotulinumtoxinA, Diazepam and Tizanidine / Zanaflex. One third of all medication being taken is for pain, ranging from over-the-counter medicines like paracetamol through to strong opioid medication like morphine.

There is little published research to support the use of many of the medications used for treatment of HSP.

Blog post: http://hspjourney.blogspot.co.uk/2015/02/2014-survey-results.html

Full analysis: https://drive.google.com/file/d/0BzEoTkR5HCWhbEJMcVZncFNGV00/view

2013: Mobility, Symptoms, Resources and Mis-diagnoses

An analysis was undertaken between respondents’ mobility levels and the number of other symptoms they have from the list: bladder problems, bowel problems, back pain, fatigue, stress, depression, clonus, pes cavus, numbness, stiffness when it is cold, loss of vibration sensitivity in legs, hammer toes, loss of balance.

Those who can walk unaided tend to have 4-5 minor symptoms, up to three moderate symptoms and no major symptoms. All respondents in this group had at least three symptoms, at least two of which were minor.
Those who use mobility aids some of the time tend to have 4-5 minor symptoms, up to three moderate symptoms and up to one major symptom. All of the respondents in this group had at least five symptoms, at least one of which was minor.
Those who use mobility aids all or most of the time tend to have 2-5 minor symptoms, up to 5 moderate symptoms and up to 5 major symptoms. All of the respondents in this group had at least 7 symptoms.

One fifth of respondents indicated that they had been correctly diagnosed with HSP the first time. The most frequent misdiagnoses were; Multiple Sclerosis, Cerebral Palsy, Arthritis and Charcot-Marie-Tooth disease.

Blog post: http://hspjourney.blogspot.co.uk/2014/02/hsp-survey-results.html

Full analysis: https://drive.google.com/file/d/0BzEoTkR5HCWhTlFsY3k0Y1IzWlE/view

Note from June 2016 - when preparing the poster I had a look at the data for the number of symptoms for the different levels of mobility, and there are slightly different numbers shown on the poster.

A journey to HSP/FSP

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