Saturday, 28 February 2015

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;
  1. No mobility effects
  2. Can walk without aids but some effects
  3. Orthotics/AFO/FES and/or Sticks/Poles/Crutches/Canes some of the time
  4. Sticks/Poles/Crutches/Canes and Frame/Chair some of the time
  5. Sticks/Poles/Crutches/Canes most of the time
  6. Sticks/Poles/Crutches/Canes all of the time
  7. Rollator/Walking frame most of the time
  8. Rollator/Walking frame all of the time
  9. Wheelchair/Mobility scooter most of the time
  10. 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


Wednesday, 11 February 2015

Bladder Medication Update

I have now been taking detrusitol for about 4 months now, and I thought time for a brief update.

I have found that my urgency has continued to be a much smaller issue than it was, and by and large I haven't had any accidents since then.

Also, I have spotted that my bowel urgency has also decreased similarly over the same time. A look at the detrusitol/tolterodine leaflet suggests that it is only for bladder issues. However, the common (less than 1 in 10) side effects include both diarrhoea and constipation so I may have found some unintended benefits here too.

In summary, I'm pleased that this issue is much more under control than it was before.


Saturday, 17 January 2015

Is HSP a disability?

In my work I belong to a professional association. They have started tracking disability information to try to demonstrate that they are not discriminating, as required by UK legislation.

The question begins with a statement which gives the legislative definition of disability and then gives a pick list of choices which you can select one.

Given my current minor symptoms it is clear that I choose "I do not have a disability".

But, I wanted to find out what they were using the data for and understand the process so asked a question. The interesting answer is that disability is a personal choice, and I hadn't really grasped that concept before.

It is entirely up to me to decide the point on the scale between now and when I end up (most likely) in a wheelchair when I would call myself "disabled".

It hadn't really occurred to me, assuming my symptoms progress a lot, that I could choose never to call myself disabled. In a lot of respects this is a very powerful and positive thought to carry forward.

Thursday, 15 January 2015

Life Expectancy

Last year I put up a post which compared HSP with other types of Motor Neuron Disease, and in which I summarised HSP as:

"The primary features of HSP are spasticity and weakness in the legs, varying between individuals. There is progressive difficulty walking and symptoms worsen over time. Initial symptoms are typically difficulty with balance, stubbing the toe or stumbling. Changes begin gradually. As the disease progresses, canes, walkers and eventually wheelchairs may become needed, although some people never require assistive devices. A wide variety of symptoms are observed across cases and over time, including balance, fatigue, bladder and back pain. The majority of individuals with HSP have a normal life expectancy."
http://hspjourney.blogspot.co.uk/2014/09/the-als-ice-bucket-challenge.html

As mentioned, I had borrowed the text from other HSP sites. I also used this post as the basis for one of my columns in the UK HSP Support Group Newsletter, and I was asked by one reader about life expectancy. This is an interesting question, and one I'm slightly nervous about writing about given that this is a sensitive issue. My advice for any readers who are concerned about this should contact their HSP specialist to get their own answer.

Some clarity is obtained from the NINDS website:
http://www.ninds.nih.gov/disorders/hereditary_spastic_paraplegia/hereditary_spastic_paraplegia.htm which indicates that "The majority of individuals with uncomplicated HSP have a normal life expectancy." I realise now that I should have included this important differentiation in my post (and I have now added an update accordingly). There is an increased risk with pure HSP due to falls and from infections, principally bladder.

Given that life expectancy for uncomplicated/pure HSP is usually normal this raises the question about what is the life expectancy for complex HSP. In summary, there is not much information which I can find, and this is an overview of what I have found, with links.

In my trawl of research papers there are only two papers which talk about life expectancy, both in relation to SPG11.
http://www.ncbi.nlm.nih.gov/pubmed/23443022 - A Dutch study covering 18 patients
http://www.ncbi.nlm.nih.gov/pubmed/210which - A study from France with 7 patients

The Dutch study suggests a life expectancy of 3-4 decades after the start of walking problems. The paper from France gives life expectancy of 30-40 years, but it is not clear from the abstract if this is from onset, the same as the Dutch study, or 30-40 years total. These two studies seem to have quite small sample sizes, and I dont have the benefit of the full papers to see what the uncertainty/confidence might be.

There is also a lot of information about HSP on this Washington University page http://neuromuscular.wustl.edu/spinal/fsp.html which indicates that SPG11 has an onset usually before 20 but sometimes up to 50, which perhaps ties in with the Dutch study in that some people don't get SPG11 until older than 30-40. The Washington page doesn't give life expectancy for SPG11.

The Washington page also describes all of the different forms of HSP and other related conditions, although it is not clear where all the data comes from. There are references in most areas of the page, but I don't have access to full papers to check. This page indicates that SPG13 has a life expectancy of less than 2 decades after onset.

Looking for further information starts to become more of a challenge needing to use other search terms like prognosis, life span, death, morbidity. This following paper presents all the HSP types and indicates which are complex/complicated and which are pure/uncomplicated, and could form a start.
http://download.springer.com/static/pdf/682/art%253A10.1007%252Fs00415-014-7598-y.pdf?auth66=1420585106_72fa6be6ff74353504b77ad14ce2220d&ext=.pdf

All of the other information/comments I find are not specific and I can find various answers to the question which contradict each other. Since I dont know which to believe, if any, I'll avoid any of those links here.

Originally I was quite surprised how little information there is out there on this topic. I couldn't decide if that's just because HSP is a rare condition and this aspect hasn't yet got the attention that it needs in the limited budget research world, or if it was the case that the other issues associated with complex HSP need more attention than looking at this issue. Some more thought, and discussion with others leads me to believe that life expectancy varies significantly from person to person, and perhaps that it can be varied with the level of care that is received. Which brings me back to my original observation - if this is important to you, talk to your specialist.