2014 Survey Results

So, it is rare disease day again. I am pleased to publish the results of my second HSP survey which I launched in September. Many thanks are due to the 97 people who gave their time and completed the survey - this wouldn't have been possible without you.

The full analysis can be found here: 

https://drive.google.com/file/d/0BzEoTkR5HCWhbEJMcVZncFNGV00/view?usp=sharing&resourcekey=0-5WakXsT4x1aiVBwu1sG2jw

Here is a short version of the 2014 suvey results....

This reports my analysis of the results of an on-line survey for people with HSP launched in September 2014. There were 97 respondents who completed the survey, predominantly from the USA and the UK.

Mobility Analysis

All 97 respondents gave answers to this question. Around half of respondents use walking sticks/poles/crutches/canes and around two fifths of respondents use a wheelchair or mobility scooter. FES is the mobility aid used by the least number of people, with a take-up of around 5%.

The results also allow the distribution of respondents within a scale of mobility which I devised last year. This is an “HSP mobility score” which allows me to cross-reference mobility against the other questions in the questionnaire. The definition of the HSP mobility score is;

0. No mobility effects
1. Can walk without aids but some effects
2. Orthotics/AFO/FES and/or Sticks/Poles/Crutches/Canes some of the time
3. Sticks/Poles/Crutches/Canes and Frame/Chair some of the time
4. Sticks/Poles/Crutches/Canes most of the time
5. Sticks/Poles/Crutches/Canes all of the time
6. Rollator/Walking frame most of the time
7. Rollator/Walking frame all of the time
8. Wheelchair/Mobility scooter most of the time
9. Wheelchair/Mobility scooter all of the time

Table 7 – Overview of mobility aids used

Mobility Aids Used - Overview:	Respondents	Percentage	Mobility Score
Those without aids	19	20%	0-1
Those who use mobility aids some of the time	24	25%	2-3
Those who use sticks most/all of the time	24	25%	4-5
Those who use frames most/all of the time	13	13%	6-7
Those who use chairs most/all of the time	17	18%	8-9

Medication

I wanted to explore which reasons people take which medicines for and how effective they perceive those to be. In total 91 respondents answered this question. There were 68 people who indicated that they took at least one medication and 23 respondents who indicated that they did not take any medication at all.

Around three quarters of people are prescribed at least one form of medication for their HSP. Of the 23 who responded that they did not take any medication 10 indicated that they have not ever been on medication for HSP. The other 13 had 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. Naturally, readers should consult with their doctor if they wish to change their medications.

The majority of respondents included which symptoms they took their medication for, as shown for symptom experienced by at least 10 respondents;

Table 10 – Symptoms that people take medication for

Symptom	Number of medications taken
Spasticity	49
Pain	29
Bladder	22
Spasm	19
Depression	12
Nerve Pain	10

Spasticity and Spasms

Almost half of the medication being taken is used to treat spasticity and spasms, the key features of HSP. The biggest proportion of this group of medications comprises people taking Baclofen. The list of medications where at least 5 respondents are taking is shown in the following table, with descriptions of the medications taken from the UK NHS and drugs.com websites.

Table 13 – Spasticity and Spasm Medication

Medication	Description	People taking medication
Baclofen	Baclofen reduces muscle tone and unwanted muscle contractions. This helps to reduce painful muscle spasm.	43
Botulinum toxin A / Botox / OnabotulinumtoxinA	Botulinum Toxin Type A is used to treat muscle spasms. It works by stopping muscle contraction in the muscle that it is injected into. This prevents muscle spasm. It also blocks pain signals, which indirectly block the development of a migraine.	7
Diazepam	Diazepam is a medicine which helps to control feelings of anxiety. It makes people feel less agitated and less tense. It also acts as a sedative and an anticonvulsant. It can help to relieve muscle spasm.	7
Tizanidine / Zanaflex	Tizanidine reduces muscle tone. This helps to reduce painful muscle spasm. The effect of Tizanidine usually lasts for a few hours.	6

Baclofen

43 Respondents indicate that they take Baclofen for HSP symptoms. The symptoms, as described, fall into two areas, spasticity, with 31 respondents and spasms, with 7 respondents. 6 of the respondents indicate that they take their Baclofen through an intrathecal pump whilst the other 37 take tablets.

In addition there are 26 other respondents who indicated that they have tried Baclofen in the past, but no longer take it, including 1 who used use an intrathecal pump.

The 38 respondents who currently take Baclofen for spasticity and spasms cover the full range of mobilities. Daily doses range between 10mg and 180mg for oral tablets. Generally, the dose of Baclofen is higher with increased spasticity/loss of mobility, but there are exceptions to this trend both ways. 15 Respondents indicate that their dose has increased over the years, with 5 respondents indicating no change.

31 of the 38 respondents who currently take Baclofen gave details of their side effects. 10 respondents had no side effects, 11 respondents found Baclofen made them sleepy or drowsy. The other 10 respondents indicated a range of side effects including bowel/bladder issues, dizziness, dry mouth, stomach ulcers, blood pressure issues and short term memory loss.

Of the 26 respondents who indicated that they no longer take Baclofen, 19 indicated why they had stopped taking it. 9 respondents indicated that they did not perceive any benefits from the medicine, 3 indicated it made them too drowsy/sleepy with the other 7 generally noting side effects outweighing benefits.

Most respondents indicated that the benefits of Baclofen have not changed over the years whereas other respondents were not sure if there had been a change. One respondent noted a “huge” difference moving from tablets to an intrathecal pump.

Respondents showed that, on the whole, people currently perceive benefits from the medicine, it is important to them and they follow the prescribed dose. There are some respondents who take the medicine but who do not perceive any benefits. The majority of respondents who currently take Baclofen would recommend it.

Baclofen is one of the recognised treatments for HSP. The first paper to mention the use of Baclofen to treat HSP seems to date from 1989, in the Journal of the Medical Association of Thailand (PubMed ID: 2738487). Papers continue to be published on this into 2015, with just over 20 papers altogether.

  

Botulinum Toxin Type A

7 respondents indicate that they take Botulinum Toxin Type A for treatment of HSP symptoms, predominantly spasticity but also spasms. This is commonly known as Botox, although there are several different types of treatment. For ease I will refer to this treatment as Botox in the remainder of this section. One respondent indicated that they have previously taken Botox but no longer take it because it ceased to be effective.

All respondents who take Botox use mobility aids to some degree with mobility scores ranging between 3 and 9. Typically Botox is injected every three months and the effects are noted as lasting for between a few weeks and a few months. Side effects are generally not experienced, although one respondent noted weakness for around a week after injection. Apart from one respondent, Botox is perceived as having benefits and would be recommended to others.

Botox is one of the recognised treatments for HSP. The first paper to mention the use of Botox to treat HSP seems to date from 2003, in the journal Developmental Medicine & Child Neurology (PubMed ID: 14667075). Papers continue to be published on this into 2015, with 4 papers altogether.

Diazepam

7 respondents indicate that they take Diazepam for treatment of HSP symptoms, predominantly spasticity but also spasms. No respondents indicated that they have previously taken Diazepam but no longer take it.

All respondents who take Diazepam use mobility aids to some degree with mobility scores ranging between 3 and 8. Typically one dose is taken daily, often at night, although some take more frequently and less frequently than this. The principal side effect noted is sleepiness, by 4 respondents. Diazepam is generally perceived as having benefits and would be recommended to others.

Diazepam is mentioned in one HSP paper from 1989, in the Japanese journal No to Shinkei - Brain and Nerve (PubMed ID: 2803825).

Tizanidine / Zanaflex

6 respondents indicate that they take Tizanidine for treatment of HSP symptoms, for both spasticity and spasms. 10 respondents indicated that they have previously taken Tizanidine but no longer take it, either due to side effects or to no perceived benefits.

Most of the respondents who take Tizanidine are able to walk, with 4 having a mobility score 3 or less, with one respondent scoring 5 and one scoring 8. The principal side effect noted is tiredness or fatigue, by 5 respondents.  Those who currently take Tizanidine generally perceive it to have benefits. There are some reservations recommending it to others.

Tizanidine is reported as having benefits for spastic paresis in 1982, in the Journal of the Neurological Sciences (PubMed ID: 7035623).

Other Treatments (Fewer than 5 respondents)

Of the other treatments for spasms and spasticity taken by respondents, only Levodopa is described as a treatment for HSP in the Movement Disorders Journal in 2006 (PubMed ID: 16463348). It is interesting to note, however, that Nabiximols/Sativex is reported to also control pain and has benefits on bladder issues, that Dalfampridine/Ampyra is reported to reduce fatigue and that Levodopa is reported to improve the control of muscles. One respondent indicated that they had a spinal cord stimulator implanted which was the biggest benefit they had of all.

Pain

Many people are taking a range of painkillers, some are over-the-counter medicines like paracetamol right through to strong opioid medication like morphine.  At least 5 respondents are taking antidepressant or anticonvulsant medication as shown in the following table;

Table 16 – Pain Medication

Medication	Description	People taking medication
Amitriptyline / Elavil	Amitriptyline is a tricyclic antidepressant. It is also a medication that is used in management of long term (chronic) pain, especially nerve pain. It can also be used for improving sleep. It is prescribed in low doses for managing pain and is not addictive. Amitriptyline is also used for the treatment of bedwetting in children	6
Gabapentin / Neurontin	Gabapentin is an anti-epileptic medication, also called an anticonvulsant. It affects chemicals and nerves in the body that are involved in the cause of seizures and some types of pain. It is also used to treat restless legs syndrome (RLS).	6

Amitriptyline / Elavil

6 respondents indicate that they take Amitriptyline for treatment of HSP symptoms including nerve pain, pain and restless legs. 1 respondent indicated that they have previously taken Amitriptyline but no longer take it,  due to trying a different medication at the moment.

The respondents who take Amitriptyline include most of the full range of mobility, having a mobility score between 2 and 9. In terms of side effects, 4 respondents note no side effects with 2 noting tiredness. Based on the 6 respondents currently taking Amitriptyline, it is perceived as having benefits and would be recommended to others. None of the respondents indicated that they take Amitriptyline for bladder issues, but this medicine can be prescribed to treat bedwetting in children by helping the bladder wall to relax.

Amitriptyline is not reported in any HSP papers.

Gabapentin / Neurontin

6 respondents indicate that they take Gabapentin for treatment of HSP symptoms, predominantly nerve pain and also pain. 7 respondents indicated that they have previously taken Gabapentin but no longer take it, 2 indicating because of side effects and 2 because of lack of perceived benefit.

The respondents who take Gabapentin use mobility aids to some degree with mobility scores ranging between 3 and 8. In terms of side effects, all respondents note side effects including tiredness/drowsiness (5 respondents), dry mouth (1 respondent) and unpleasant dreams/thoughts (1 respondent). Based on the 6 respondents currently taking Gabapentin, it is generally perceived as having benefits and would generally be recommended to others.

Gabapentin is reported in one HSP paper from 2007, in the European Journal of Neurology (PubMed ID: 17539946) although this indicates no difference in tests between Gabapentin and a placebo.

Supplements and Diet

Respondents were asked to give details of supplements that they take. 58 respondents answered this question. 6 of these indicated that they did not take any supplements and 52 indicated that they took at least one. Generally people take up to 3 supplements with a small number taking more than this.

8 respondents are certain that they get benefits from taking magnesium to reduce spasms/cramps and to relax muscles, including bladder muscles. There is one paper which reports using magnesium glycerophosphate to reduce spasticity in paraplegia caused by MS (PubMed ID: 11136367). There were two respondents who were certain that taking cranberry tablets was beneficial in maintaining a healthy bladder.

There was also a general question about dietary changes. 39 respondents have not made dietary changes and 32 people had made changes. There were 7 respondents who made changes due to bowel issues, with some adding more or cutting down on fibre and/or fruit/vegetables. One respondent avoids caffeine and alcohol due to bladder issues. Low/No gluten and carbohydrate diets are mentioned as giving benefits to several respondents, with one noting improved mobility on a gluten free diet and another noting reduced inflammation with a low carb diet. One respondent limits alcohol to assist with balance, and another notes caffeine and sugar make their symptoms worse. 7 respondents have just made general health improvements in their diet, including two partly as a result of weight gain from reduced mobility.

It is worth noting that the Spastic Paraplegia Foundation website indicates that “There are no known studies indicating that vitamins or food supplements are an effective treatment for HSP.” (https://sp-foundation.org/understanding-hsp-pls/treatments-and-therapies/). Any readers wishing to make changes to their diet or to change any supplements being taken may wish to consult their doctor.

Exercise and Relaxation

The final section of the questionnaire looked at respondents exercise and relaxation routines. Overall 90 respondents answered this question. The following table shows the distribution of answers given.

Table 17 – Exercise and Relaxation

	Stretches	Yoga etc.	Weights	Power plate etc.	Cardiovascular	Walk/Run/Cycle	Swimming	Massage	Aromatherapy	Acupuncture	Physiotherapy	Chiropractic
I do this several times a day	8	0	0	1	1	1	0	0	0	0	1	0
I do this daily	38	8	8	2	1	15	2	1	0	0	5	0
I do this weekly	9	5	13	4	13	17	14	9	3	2	13	1
I do this monthly	4	2	0	1	0	1	0	10	1	2	10	1
I do this occasionally	17	3	13	2	8	20	23	13	3	4	10	5
I dont do this	11	55	42	61	50	24	35	42	59	63	34	62
Total Answers	79	73	76	70	72	77	74	75	66	71	72	69

This shows that stretches are the most common form of exercise being undertaken with over 85% of respondents doing this. Other activities which are commonly undertaken include weights, cardiovascular machines, walking/running/cycling, swimming and physiotherapy.

Of the 90 respondents only 1 did not undertake any of the activities or indicate other activities. Respondents who undertake activities weekly or more frequently are grouped together as undertaking that activity “regularly”. Overall 71 respondents undertake at least 1 activity regularly.

To look at this in more detail Yoga/Tai-Chi/Pilates, Weights, Power plate/circulation booster, walking/running/cycling, swimming and physiotherapy are grouped together as “exercise”. Overall 81 respondents undertake at least one exercise activity, and 66 of these undertake at least one of those activities regularly (weekly or more often). Grouping the other activities together shows that these activities are undertaken regularly, but the general take-up of the other activities is lower than the exercise activities.

This information shows that for people who do not use mobility aids at all (mobility score 0 or 1) over 80% undertake at least one activity regularly. Activities are likely to include walking/running/cycling. Many respondents also do stretches and take part in swimming.  Other activities undertaken include physiotherapy, massage, yoga/tai-chi/pilates, weights and cardiovascular.

Almost 80% of people who use mobility aids some of the time (mobility score 2 or 3) take part in at least one activity regularly. Activities are likely to include stretches and walking/running/cycling. Many respondents also take part in weights, swimming and physiotherapy.  Other activities undertaken include cardiovascular, massage and yoga/tai-chi/pilates.

           

Three quarters of people who use sticks most or all of the time (mobility score 4 or 5) take part in at least one activity regularly. Activities are likely to include stretches. Many respondents also take part in physiotherapy and walking/running/cycling. Other activities undertaken include massage, swimming and weights.

Two thirds of people who use walking frames most or all of the time (mobility score 6 or 7) take part in at least one activity regularly. Activities are likely to include stretches. Many respondents also take part in walking/running/cycling. Other activities undertaken include physiotherapy and weights.

Almost 90% of people who use wheelchairs most or all of the time (mobility score 8 or 9) take part in at least one activity regularly. Many respondents take part in stretches. Other activities undertaken include swimming, physiotherapy, massage, weights, power plate/circulation booster and aromatherapy.

Like this? in other years:
Overview of all my surveys: http://hspjourney.blogspot.co.uk/p/my-on-line-resarch.html
2016: Fatigue, bladder, bowel & information: http://hspjourney.blogspot.co.uk/2017/02/2016-survey-results.html
2015: Modifications at home, depression, quality of life: http://hspjourney.blogspot.co.uk/2016/02/2015-survey-results.html
2013: Symptoms and misdiagnosis: http://hspjourney.blogspot.co.uk/2014/02/hsp-survey-results.html