Tuesday, 28 February 2017

2016 Survey Results

Another year passes and we are round to Rare Disease Day again, this year with the theme of research which seems highly appropriate for me!

I am pleased to publish the results of my fourth HSP survey which I launched in September 2016. Many thanks are due to the 169 people who gave their time and completed the survey - this wouldn't have been possible without you. Most of the respondents were from the USA and the UK, and about a quarter had also completed at least one of my previous surveys.

The text below is a short version of the results. The full version goes into more detail on each of the different elements, with more graphs, tables and detailed breakdowns of different results and factors, and the questionnaire scoring systems. The full analysis can be found here: https://drive.google.com/open?id=0BzEoTkR5HCWhSEQ1UElwamVod3c

This analysis is by far my longest yet. Apologies for some readers, the lines on my tables are not behaving themselves this year!

Mobility Analysis
From the answers to the mobility questions it is possible to see which mobility aids are the most regularly used. Around half of respondents use walking sticks/poles/crutches/canes, and similarly, 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%. Broadly these results are similar to those from previous years.

I have devised an “HSP mobility score” which then 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. Walks without aids but has 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
The overall results are;

Mobility Aids Used - Overview:
Respondents
Percentage
Mobility Score
Those without aids
30
18%
0-1
Those who use mobility aids some of the time
42
25%
2-3
Those who use sticks most/all of the time
55
32%
4-5
Those who use frames most/all of the time
13
8%
6-7
Those who use chairs most/all of the time
29
17%
8-9

Fatigue
Fatigue is often noted as a symptom of HSP. My 2013 survey showed that 62% of people experienced fatigue as a significant symptom and 30% experienced fatigue as a minor symptom. I wanted to find out more about fatigue.

I could only find one paper on fatigue in HSP, a study from 2015-2016 which examined non-motor symptoms in people with SPG4 in Brazil – Non-motor symptoms in patients with hereditary spastic paraplegia caused by SPG4 mutations, by Katiane Servelhere and others. (https://www.ncbi.nlm.nih.gov/pubmed/26806216) This study used the Modified Fatigue Impact Scale for fatigue and reported that “patients with SPG4-HSP had more severe fatigue than controls (MFIS: 31.3±14.9 vs 14.9±15.4, p<0 .001="" o:p="">

There has also been research undertaken in assessing fatigue in Multiple Sclerosis (a common misdiagnosis for HSP) including development of the Neurological Fatigue Index. The Brief Fatigue Inventory also appears to be used in a number of different conditions, including cancer. Since all of these are available in a short-form version I chose to ask all three questionnaires, and would then be able to test if one is more suited to HSP. The questionnaire asked respondents to complete:
  • 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)
Neurological Fatigue Index - MS
The Neurological Fatigue Index (NFI-MS) asks people to select how much they agree with each of 9 questions, selecting from; Strongly Disagree, Disagree, Agree and Strongly Agree. The nine questions are:
·          Sometimes, I lose my body strength
·          Sometimes, I really have to concentrate on what are usually simple things
·          The thought of having to do something often puts me off doing it
·          By the end of the day I'm shattered
·          If I've overdone things, I know about it the next day
·          I soon become weak after physical effort
·          Resting allows me to carry on
·          I can become weak even if I've not been doing anything
·          When I awake in the morning, I feel unrefreshed

Details of the survey can be found here: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2834659/. Each question is scored depending on the answer selected, where Strongly Disagree =0, Disagree =1, Agree =2 and Strongly Agree =3. The overall score is the sum over the nine questions, and therefore can range between 0 (answering ‘strongly disagree’ to all questions) and 27 (answering ‘strongly agree’ to all questions).

The table below shows the results.

Mobility Score
Respondents
Minimum Score
Average Score
Maximum Score
0-1
30
3
16.6
26
2-3
41
7
15.4
24
4-5
52
9
17.5
27
6-7
13
9
17.0
22
8-9
27
10
19.5
25

These results show that the average and maximum scores are generally independent of mobility although 
the average score rises slightly with decreased mobility. Those who do not use mobility aids (mobility score 0 or 1) have lower minimum scores than those who use mobility aids (mobility score 2 or more).


The answers to the questions allow the different fatigue factors to be examined. Overall, the three statements which the most people agreed with were “If I've overdone things, I know about it the next day” (81% agreeing), “Resting allows me to carry on” (79% agreeing) and “I soon become weak after physical effort” (78% agreeing). Overall, the three statements with he least people agreeing with were “sometimes, I really have to concentrate on what are usually simple things” (60% agreeing), “When I awake in the morning, I feel unrefreshed” (62% agreeing) and “I can become weak even if I've not been doing anything” (69% agreeing).

The factors do change with level of mobility. For those who do not need aids (mobility score 0-1). Most people agree with “I soon become weak after physical effort” (80% agree) and fewest agree with “The thought of having to do something often puts me off doing it” (53% agree). At the other end of the mobility score, those in wheelchairs all or most of the time (mobility score 8-9) most people agree with “By the end of the day I'm shattered” and “I can become weak even if I've not been doing anything” (93% agree with each) and fewest agree with “When I awake in the morning, I feel unrefreshed” (70% agree). 

Modified Fatigue Impact Scale 5
There are two versions of the Modified Fatigue Impact Scale (MFIS) scale, a full questionnaire with 21 questions and a shorter version with 5 questions. The five questions are those from the full survey whose answers correlated most strongly with the overall 21 question survey score. The full 21 questions on this survey are themselves a subset of the Fatigue Impact Scale. The Modified Fatigue Impact Scale asks people to select how often fatigue has affected them, selecting from; Never, Rarely, Sometimes, Often and Almost Always. The five questions are:
·          I have been less alert
·          I have been limited in my ability to do things away from home
·          I have had trouble maintaining physical effort for long periods
·          I have been less able to complete tasks that require physical effort
·          I have had trouble concentrating

Details of both versions of the MFIS survey can be found here (along with a number of other questionnaires associated with Multiple Sclerosis): http://www.nationalmssociety.org/NationalMSSociety/media/MSNationalFiles/Brochures/MSQLI_-A-User-s-Manual.pdf Each question is scored depending on the answer selected, where Never =0, Rarely =1, Sometimes =2, Often =3 and Almost Always =4. The overall score is the sum over the five questions, and therefore can range between 0 (answering ‘never’ to all questions) and 20 (answering ‘almost always’ to all questions).

The questions have been grouped to cover three key areas of fatigue; Physical (the two ‘maintaining physical effort’ questions), Cognitive (the ‘less alert’ and ‘trouble concentrating’ questions) and Psychosocial (the ‘limited in my ability’ question).

The table below shows the results.
Mobility Score
Respondents
Minimum Score
Average Score
Maximum Score
0-1
27
0
11.0
20
2-3
39
0
9.7
17
4-5
53
2
12.7
19
6-7
11
2
11.9
19
8-9
27
7
14.1
18

These results show that the average and maximum scores are generally independent of mobility although the average score rises slightly with decreased mobility. It is only those in wheelchairs all or most of the time (mobility score 8 or 9) who consistently have a higher minimum score. 

The MFIS scale allows us to look at the different areas of fatigue. The overall maximum score of 20 would comprise a score of 8 each from Physical and Cognitive sections and a score of 4 from the Psychosocial section, and the graph below splits the average score into the three areas:

This graph shows that the physical questions make the greatest contribution to the fatigue score across all levels of mobility, scoring slightly more than the cognitive questions. The psychosocial question contributes the least, and would still contribute the least if its score were doubled to give it equal weight to the physical and cognitive questions. From this, we can see that both physical and cognitive factors play a part in the fatigue of people with HSP.

The analysis of respondents with SPG4 shows results similar to the full dataset, whereas those with SPG7 tend to have a higher level of fatigue.

The MFIS-5 questionnaire shows similar results to the Brazil results, in that the physical score contributes the most to the overall fatigue level and the psychosocial score contributes the lowest level. In the full MFIS questionnaire there are 9 physical questions (with a maximum score of 36), there are 10 cognitive questions (with a maximum score of 40) and there are 2 psychosocial questions (with a maximum score of 8).


Brief Fatigue Inventory
The Brief Fatigue Inventory (BFI) has 2 sections. The first section has three questions and asks people to rate their fatigue on a scale between 0 and 10 where 0 = ‘No fatigue’ and 10 = ‘As bad as you can imagine’. They are asked to rate their fatigue right now, their usual level of fatigue and their worst level of fatigue. They are then asked to rate how their fatigue has interfered with six items using a scale between 0 and 10 where 0 = ‘Does not interfere’ and 10 = ‘Completely interferes’. The six items are:
·          General activity
·          Mood
·          Walking ability
·          Normal work (both outside home and daily chores)
·          Relations with other people
·          Enjoyment of life

Details of the BFI are here:  http://onlinelibrary.wiley.com/doi/10.1002/(SICI)1097-0142(19990301)85:5%3C1186::AID-CNCR24%3E3.0.CO;2-N/full. The questionnaire is scored by taking the average of the scale score of the nine items, and therefore can range between 0 (score 0 to all questions) and 10 (score 10 to all questions).  The table below shows the results.
Mobility Score
Respondents
Minimum Score
Average Score
Maximum Score
0-1
26
0.7
5.5
9.0
2-3
40
0.3
4.8
8.2
4-5
54
2.0
6.0
9.3
6-7
12
0.6
4.8
7.3
8-9
27
1.7
6.5
9.4

These results show that the average and maximum scores are generally independent of mobility although t
here is a slight upward trend on the average score with decreased mobility. There is no clear pattern in the minimum score.

The scale has been interpreted within the previous Wiley link, and a number of different classifications are made for mild, moderate and severe fatigue in cancer patients, as shown in Table 4 in the link. Since the average score is unlikely to be a whole number, I am using the following similar definitions;
·          Score less than 3 – Mild fatigue
·          Score between 3 and 7 –  Moderate fatigue
·          Score greater than 7 – Severe fatigue.

The table below shows the number of respondents with mild, moderate and severe fatigue, along with the proportion of that mobility score group:
Mobility Score
Respondents
Mild Fatigue
Moderate Fatigue
Severe Fatigue
0-1
26
3 (12%)
15 (58%)
8 (31%)
2-3
40
6 (15%)
31 (78%)
3 (8%)
4-5
54
6 (11%)
28 (52%)
20 (37%)
6-7
12
2 (17%)
9 (75%)
1 (8%)
8-9
27
1 (4%)
15 (56%)
11 (41%)
Overall
159
18 (11%)
98 (62%)
43 (27%)

This table shows that between around 90% of people with HSP have moderate or severe fatigue.



Fatigue Summary
Of the people which answered the fatigue questions, 126 said that their fatigue at the time they answered the questions was similar to their normal level of fatigue. 7 people said their fatigue was better than normal and 36 said that their fatigue was worse than normal. This may suggest that the results of this survey present a slightly more adverse picture than ‘normal’ for the fatigue levels of people with HSP.

166 respondents answered the question about which fatigue survey they preferred. 132 said that they had no clear preference for one of the surveys. Of the 34 respondents who expressed a preference 19 preferred the NFI questionnaire, 9 preferred the MFIS-5 questionnaire and 6 preferred the BFI questionnaire. There were people from across the range of mobility scores who preferred the NFI and MFIS-5 questionnaires. Whereas people who preferred the BFI tended to have less reliance on mobility aids, with mobility scores between 1 and 5.

The average scores for each of the three fatigue 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 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. The BFI questionnaire shows that around 90% of people with HSP have moderate or severe fatigue.  


Bladder Issues
There have been a number of papers examining bladder issues in those with HSP. Bladder dysfunction in hereditary spastic paraplegia: a clinical and urodynamic evaluation, by Fourtassi et al in 2012 (Pubmed ID 22289900), reports urgency being the most common complaint affecting 72% of people, followed by increased frequency (affecting 66%), incontinence (affecting 55%) and hesitancy (affecting 52%). The study examined 29 patients and was carried out in France. These results are similar to Bladder dysfunction in hereditary spastic paraplegia: what to expect?, by Braschinsky et al in 2010 (Pubmed ID 197226407), which reports incontinence being the most common symptom, affecting 69%, followed by hesitancy (affecting 59%), increased frequency (affecting 55%) and urgency (affecting 51%). The study examined 38 patients with urinary symptoms out of 49 patients. The study was carried out in Estonia.

The various texts on HSP often describe having overactive bladders (OAB). I reviewed different short form questionnaires for overactive bladders, and found the International Consultation on Incontinence Modular Questionnaire (ICIQ). There are a number of different questionnaires for different aspects of different pelvic problems, and they have the objective of developing validated international standard questionnaires for wide use.

They have an overactive bladder survey http://www.iciq.net/ICIQ.OABmodule.html which “provides a brief and robust measure to assess the impact of symptoms of overactive bladder on quality of life and outcome of treatment.” There are four questions in this questionnaire, and users select their answers from pre-determined answers.

The scale does not appear to show what a normal score would be. However, this report (http://bmcurol.biomedcentral.com/articles/10.1186/s12894-015-0009-6) uses the questionnaire to examine stress levels within a population with overactive bladders, carried out by Henri Lai (et al) in St Louis, USA. They had a range of age matched controls who had an average score of 2.0, plus or minus 1.5. This suggests that a score of 3-4 could be used to mark the start an overactive bladder (OAB).

Of note, that paper reports that “OAB patients reported psychological stress levels that were …. significantly higher than healthy controls. There was a positive correlation between perceived stress levels and urinary incontinence symptoms, and its impacts on quality of life among OAB patients.”

Overall score
These questions were answered by 165 respondents. The following table gives a summary of the results;
Mobility Score
Respondents
Minimum Score
Average Score
Maximum Score
0-1
30
0
5.4
10
2-3
41
0
4.4
14
4-5
53
0
5.6
12
6-7
13
0
7.5
14
8-9
28
1
7.1
16
Overall
165
0
4.6
16

This shows that there are people in each mobility band who do not have any bladder symptoms, and there are people within each mobility band who have bladder symptoms. Given that the score ranges between 0 and 16, for the purposes of this analysis I am grouping scores as follows;
·          3-6 = mild effects, things occur “occasionally”,
·          7-10 = moderate effects – things occur “some of the time”
·          At least 11 = severe effects – things occur “most” or “all” of the time.

Using this scoring system the results show:
Mobility Score
Respondents
No effects
Mild effects
Moderate effects
Severe effects
0-1
30
5 (17%)
14 (47%)
11 (37%)
0 (0%)
2-3
41
14 (34%)
17 (41%)
8 (20%)
2 (5%)
4-5
53
9 (17%)
29 (55%)
11 (21%)
4 (8%)
6-7
13
1 (8%)
4 (31%)
8 (38%)
3 (23%)
8-9
28
5 (18%)
9 (32%)
8 (29%)
6 (21%)
Overall
165
34 (21%)
73 (44%)
43 (26%)
15 (9%)

This shows that around 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 bladder effects tend to be more severe with those with higher mobility issues.

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.
·          The key bladder symptoms appear to be frequency and incontinence, affecting the greatest proportion of respondents.
·          Passing urine 4 to 6 times per day is normal. Just under half of respondents do this, just over half go more often.
·          Passing urine no more than once a night is normal, two thirds of respondents do this, one third go more often.
·          A third of respondents have to rush to the toilet most or all of the time, with a further quarter having to rush sometimes. Two fifths do not have to rush.
·          Urine leakage most or all of the time occurs in one fifth of respondents, a further third have this sometimes. Overall, half of respondents are affected by incontinence, one fifth of respondents are not affected and three tenths are affected occasionally.
·          The level of bother is generally above that expected by symptoms. Many respondents are bothered quite a lot by these symptoms.

Bowel Issues
There do not appear to be any papers examining bowel issues in those with HSP. Several reports describe bowel or sphincter issues, but from the abstracts available none appear to quantify this.

When reviewing the short form questionnaires for overactive bladders I noted the International Consultation on Incontinence Modular Questionnaire (ICIQ) included a questionnaire on the bowel. The website http://www.iciq.net/structure.html indicates that the bowel surveys are not finalised, however there are several other references to the ICIQ-B survey, e.g. https://www.kingstonmaternity.org.uk/media/187024/bowl-function-questionnaire-iciq-b-uk-english-.pdf.

There are twenty two questions in this questionnaire, with users selecting their answers from pre-determined lists. I decided that I would ask nine of these questions in order to get a picture of bowel function, following the general format of the overactive bladder questionnaire. The questions I selected were:
·          Q3A/3B: On average how many times do you open your bowels in 24 hours?
·          Q4: How often do you open your bowels during the night from going to bed to sleep until you get up in the morning?
·          Q5: Do you have to rush to the toilet when you need to open your bowels?
·          Q6: Do you use medications (tablets or liquids) to stop you opening your bowels?
·          Q7: Do you experience pain/soreness around your back passage?
·          Q8: Do you experience any staining of your underwear or need to wear pads because of your bowels?
·          Q13: Do you have bowel accidents when you have no need to open your bowels?
·          Q16: Do you need to strain to open your bowels?
·          Q23: Overall, how much do your bowels interfere with your everyday life?

Overall score
These questions were answered by 155 respondents. The following table gives a summary of the results;
Mobility Score
Respondents
Minimum Score
Average Score
Maximum Score
0-1
28
2
8.6
16
2-3
39
4
8.0
15
4-5
51
4
9.8
20
6-7
11
5
10.4
16
8-9
26
5
11.8
19
Overall
155
2
9.5
20

This shows that there are people in each mobility band who have no symptoms or minor symptoms, and there are people within each mobility band who have several symptoms. The minimum and average scores both generally increase as mobility issues increase. Given that the score ranges between 2 and 37, for the purposes of this analysis I am grouping bowel scores as follows;
·          5-11 = mild effects – things occur “rarely”
·          12-20 = moderate effects – things occur “some of the time”
·          At least 21 = severe effects – things occur “most” or “all” of the time

Using this scoring system the results show:
Mobility Score
Respondents
No effects
Mild effects
Moderate effects
Severe effects
0-1
28
5 (18%)
15 (54%)
8 (29%)
0 (0%)
2-3
39
3 (8%)
29 (74%)
7 (18%)
0 (0%)
4-5
51
2 (4%)
32 (63%)
17 (33%)
0 (0%)
6-7
11
0 (0%)
8 (73%)
3 (27%)
0 (0%)
8-9
26
0 (0%)
12 (46%)
14 (54%)
0 (0%)
Overall
155
10 (6%)
96 (62%)
49 (32%)
0 (0%)

This shows that around three fifths of people with HSP have mild bowel effects, and around one third have moderate effects. A few people have no effects at all, and no people showed severe effects. There is a general trend that the milder effects tend to be with those with lower mobility issues, and the bowel 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.
·          Opening your bowels between three times a week and three times a day is normal. 95% of respondents do this, 5% go more often.
·          It is not normal to open your bowels during the night. Nearly 90% of respondents do this, almost 10% need to go in the night.
·          A sixth of respondents have to rush to the toilet most or all of the time, with a further third having to rush sometimes. Half do not have to rush.
·          A third of people with HSP have to strain to open their bowels most or all of the time, with a further third having to strain sometimes. One third do not need to strain.
·          Some people have to both rush to the toilet and strain. 6 respondents have to rush and strain all or most of the time.
·          Wearing pads and/or staining underwear more than once a month occurs in about a quarter of respondents. Less than three fifths of respondents do not have this problem, and less than one fifth have this less than once a month.
·          One fifth of respondents have bowel accidents ‘sometimes’ of which only a small number have this ‘most of the time’ or ‘always’. More than half do not have this problem, and a quarter have it rarely.
·          The level of bother generally increases in line with symptoms. Those with more symptoms tend to have higher levels of bother.


HSP Information Sources
I also asked respondents where they got their information on HSP. 160 people gave answers to these questions, although some answers were partial. Most people get their information about HSP from social media, doctors and neurologists. Medical websites, support groups, physiotherapists and friends/family with HSP form another important group of information sources.

The sources which people regarded as most trustworthy were neurologists, support groups and physiotherapists. Social Media and friends/family with HSP form another important group. Of sources used by more than half of respondents, the ones with people being most unsure were doctors and other medical professionals.

Overall; people need to be selective in what they believe on social media – and check out the author. Support groups can be a good source of information. People may need to work on relationships with doctors so they understand people’s situations better. Talking about your situation with friends/family with HSP may be useful for you and them. 

Like this? in previous years:
Overview of all my surveys: http://hspjourney.blogspot.co.uk/p/my-on-line-resarch.html
2015: Modifications at home, depression, quality of life: http://hspjourney.blogspot.co.uk/2016/02/2015-survey-results.html
2014: Medication, exercise & relaxation: http://hspjourney.blogspot.co.uk/2015/02/2014-survey-results.html

2013: Symptoms and misdiagnosis: http://hspjourney.blogspot.co.uk/2014/02/hsp-survey-results.html

2 comments:

  1. This has been up for a little less than 24hours and has already had more than 600 views. Amazing! Thanks for reading everyone. (its nearly in my top-ten-all-time list already)

    ReplyDelete
  2. One week in - over 1000 views!

    ReplyDelete