Monday, 29 February 2016

2015 Survey Results

It is rare disease day again, and this year on the leap day, 29th February.

I am pleased to publish the results of my third HSP survey which I launched in September 2015. Many thanks are due to the 109 people who gave their time and completed the survey - this wouldn't have been possible without you.

Here is a short version of the results. The full version goes into more detail on modifications made, and I hope that readers can use the information given to help themselves adapt to life with HSP. The full analysis can be found here: https://drive.google.com/file/d/0BzEoTkR5HCWhRjMtejZhSGpHSG8/view

This post reports a short version of the findings of an on-line survey undertaken between September 2015 and January 2016. 109 respondents with Hereditary Spastic Paraplegia (HSP) completed the survey, predominantly from the USA and the UK. The survey covered modifications at home, depression and quality of life. Respondents also answered questions about their mobility allowing trends to be spotted with level of mobility. Around a quarter of respondents had completed one or both of my previous surveys.


The full version includes further detail on the mobility and change in mobility of respondents, more observations on modifications made around the home, greater detail on depression and quality of life and a set of data looking at the spastic paraplegia rating scale.
  
Mobility Analysis

All 109 respondents gave answers to this question. From the results it is possible to see which mobility aids are the most regularly used. Around two fifths 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%.

In the remainder of this paper, whenever “sticks” are referred to as a mobility aid, this term includes poles, crutches and canes. Whenever “frames” are referred to this includes both walking frames and rollators. Whenever “chairs” are referred to this includes both wheelchairs and mobility scooters. Whenever AFO is mentioned it refers to Orthotics and/or AFO.


The results also allow the distribution of respondents within a scale of mobility to be understood. 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. 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
  Overview of mobility aids used
Mobility Aids Used - Overview:
Respondents
Percentage
Mobility Score
Those without aids
23
21%
0-1
Those who use mobility aids some of the time
20
18%
2-3
Those who use sticks most/all of the time
27
25%
4-5
Those who use frames most/all of the time
22
20%
6-7
Those who use chairs most/all of the time
17
16%
8-9


Modifications Around The Home
Overall, there were 99 respondents who answered these questions. An overview of the data is presented below, noting that respondents may appear in more than one of these categories:

Table 9 – Overview of Modification Data
Situation
Mobility 0-1
Mobility 2-3
Mobility 4-5
Mobility 6-7
Mobility 8-9
Total Answers
Total number of respondents
20
18
24
21
16
99
Zero modifications made
10
6
8
6
1
31
Furniture moved within the property
3
0
2
1
0
6
Have moved to a single storey property
5
2
5
5
7
24
Live on one floor within their property
1
2
0
1
1
5
Plan: Change property in the future
2
2
3
4
2
13
Plan: Stay at current property
3
9
7
6
6
31
Plan: Stay as long as possible
4
1
4
5
1
15


There were 31 respondents who indicated that they had made no modifications to their properties. These fell into two general groups;
  • those that had not made modifications yet, and
  • those who didn’t need to make modifications because they had moved into an accessible property which meets their needs.
Respondents across all mobility bands have moved into properties which are either single storey or they are able to live on a single storey within their existing property.

Plans to move properties reflect a range of attitudes of people, with some people preferring to stay in their current home and make whatever modifications they need to, with others planning to move properties as the effects of HSP on their lives change. Different respondents gave answers suggesting that moving property would be something which they would expect to do in the near future whilst others were planning to move in the longer term.

The overall conclusion of this appears to be that as HSP progresses modifications will need to be made to properties, and many respondents indicate that living in a single storey dwelling makes life much easier. The requirement to move to a single storey dwelling will depend ultimately on personal situations and preferences and the progression of HSP, and there will be plenty of other factors in any decision to stay or to move house.

There were 22 different types of modifications which were mentioned by more than one respondent. As these questions were free-form answers I have had to make a few assumptions on what respondents have meant in some cases, and therefore there may be a small amount of variance in the data in this table.

Modifications by more than 5 people
Modification
Mobility 0-1
Mobility 2-3
Mobility 4-5
Mobility 6-7
Mobility 8-9
Total Answers
Total number of respondents
20
18
24
21
16
99
Grab rails (all data, in any location)
3
7
8
13
8
39
Ramps (external or internal)
1
2
5
4
5
17
Grab rails (within the shower or bath)
2
5
3
3
1
14
Accessible/raised toilet
2
2
2
2
4
12
Stair lift
0
3
0
3
5
11
Bath seat/shower seat/bath board
2
1
2
2
4
11
Conversion of bathroom to wetroom
2
1
0
3
4
10
Hospital/power/electric/adjustable bed
0
0
1
2
4
7
Making the level of the bed lower
2
0
1
2
1
6
Modifications to the kitchen*
1
0
1
3
1
6









.

I have presented a commentary on each of the most common modifications made, i.e. those with 10 or more respondents, and there is more detail in the full version. The parts of properties that 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 relatively common. Modifications in other parts of properties are made less frequently. This would appear to reflect the importance of different activities – using the toilet and keeping clean are important as is getting sleep.

Grab Rails
Grab rails are by far the most common modifications that are made around homes, and are present in some homes at all levels of mobility. The majority of grab rails are installed in bathrooms/toilets although respondents also included them by doors, in bedrooms, kitchens, hallways, garages and other rooms.

The reasons for installing grab rails fall generally into two groups, one group includes reasons around helping to keep balance and move around, and the other group includes reasons around helping to get up/down in/out off/on from things like showers/baths/chairs/beds and getting up/down stairs. Reasons for installing are often following similar incidents or being increasingly unable to do something.

Advice for others includes “Definitely help to keep you on your feet and preventing falls”, “it is a small step to take but it makes life so much easier”, “Safety is more important [than] decor or vanity”, “more confidence while showering”. Several respondents mention talking to occupational therapists about this.

Ramps
Ramps are also a common modification, again made by people at all levels of mobility. There are two general types of ramps mentioned, the larger scale purpose built external ramp used for access to the property, and smaller portable ramps which may be for use either outside or inside the property.

Whilst many of the respondents include in their reasons for installing ramps that it gives them wheelchair access to parts of their property, other respondents indicated that they have ramps because of their issues getting over/up/down steps when walking. Most of the ramps are used by respondents who rely on mobility aids of one kind or another all or most of the time.

Advice for others includes looking on Amazon to purchase directly and purchasing second hand ramps. “Worth doing provided you can” and “Very good, not too expensive.”

Raised/Accessible Toilets
This modification includes toilets that were described either as raised or accessible and has been made across the full range of mobility. Generally this was described as making it easier to stand up/sit down from the toilet and was installed because people were finding it difficult to do so. Advice for others includes “Really makes a big difference” and “Make sure that the height of the [seat] suits you”.

A couple of respondents who had been having work done on their bathrooms had elected to install a taller toilet in preparation for expected future changes to their mobility.

Stair lift
The stair lift tends to have been installed by respondents who rely more frequently on mobility aids, although a few respondents have had one installed earlier.

Stair lifts are reported as giving access to otherwise inaccessible parts of the property or, installed because it makes access easier to parts of the property by people who have difficulties getting up or down stairs.

Advice for others includes “Best thing I did! I'm not the only one who uses it!”, “Do it- though ugly and expensive my back is better for it” and “It is beneficial if you struggle to get upstairs”. Of those with lower mobility scores, the reasons for installing are “Used a lot of energy and time”, “Assessment by Occupational Therapist” and “to make life easier and safer”.

Bath seat/shower seat/bath board
This modification covers several things. Some respondents describe having a seat, chair or stool in their bath or shower and others describe having a bath board – i.e. a board which spans the bath which you can sit on. This modification has been made by people across the range of mobility.

What is not clear from all of the descriptions is if these seats are fixed to wall/bath or if free standing seats have been added. From the descriptions some clearly are permanent. These are described as helping people keep from falling, prevention of dizziness, helping get in/out of the bath/shower, relieving fatigue. These are installed in showers generally when people are no longer able to stand, or after a fall. Advice for others includes “makes showering much more enjoyable” and “Just do it. It helps so much”

Conversion of bathroom to wetroom, or conversion of bath to shower.
The wetroom modification has been made by a number of respondents across the range of mobility. Some have specifically referred to this modification as a wetroom whereas others have described it as having a shower level with the floor. Where it is not clear if the shower is level with the floor or not I have grouped as “conversion of bath to shower”, and there is some uncertainty here.

The main reason for making this modification is enabling the respondent to shower because getting in/out of the bath has become difficult or impossible. Advice for others includes “It has made bathing so much easier.” “it helps so much”, “Strongly consider keeping a bath as laying in the bath reduces stiffness.” “Bathroom mods are expensive. Get professional advice and plan carefully if you need to modify an existing bathroom.” These comments show that the decision to make this modification may difficult for some.

Modifications Conclusions
There is a wide range of modifications that people have made around their properties and the approach depends heavily on personal preferences. Modifications tend to be made after a change in mobility/symptoms has been noticed, particularly after an incident/accident. Although, some people are planning for future changes in mobility. I asked respondents for the length of time that they have had these modifications, but there is sufficient information from the mobility scores to establish the general pattern.

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 relatively common. Modifications in other parts of properties are made less frequently.

Some people prefer to make modifications within their existing property whilst others prefer or have to move into accommodation which has been or can be set up to meet their needs. Some people are designing and building their own property to their own specification. Other key factors in modifications and moving home are practicality and affordability.

8) Depression

I included the two question Patient Health Questionnaire-2 (PHQ2 http://www.cqaimh.org/pdf/tool_phq2.pdf and http://www.apa.org/pi/about/publications/caregivers/practice-settings/assessment/tools/patient-health.aspx) in my survey, and followed the scoring given for these questions. 104 respondents completed this part of the survey, and the following table shows the results, by mobility score and by total score:

PHQ-2 Scores
Mobility score
Respondents
Score 0
Score 1 or 2
Score 3 or 4
Score 5 or 6
Percent 1 to 5
Percent 3 to 6
Percent 5 or 6
0 or 1
21
9
7
3
2
57%
24%
10%
2 or 3
19
9
8
0
2
53%
11%
11%
4 or 5
26
7
12
7
0
73%
27%
0%
6 or 7
21
8
4
6
3
62%
43%
14%
8 or 9
17
6
7
3
1
65%
24%
6%
Overall
104
39
38
19
8
63%
26%
8%

Overall this study shows 63% of respondents having some symptoms of depression and 37% without those symptoms. Additionally, it suggests that around one quarter of people with HSP may require further assessment for depression, particularly for those who are using walking frames all or most of the time to get around. Figure 1 shows the results in more detail, giving the split of assessment scores in each mobility band.

Looking at the highest scores, where people have “Little interest or pleasure in doing things” and/or “Feeling down, depressed or hopeless” nearly every day, it is my hypothesis that this seems to occur at the beginning of peoples’ journeys with HSP and at the point where people are beginning to lose the ability to walk. These highest scores are not seen in whose who have accepted the use of walking sticks and are not often in those who have accepted the use of a wheelchair, and perhaps the acceptance of these mobility aids relieves the depression. I repeat, this is just my hypothesis and I accept there is not much to back this up. Interestingly, the three respondents with the most rapid change in mobility in five years score 2, 2 and 3.

As a comparator, According to the World Health Organisation (http://www.who.int/mental_health/management/depression/who_paper_depression_wfmh_2012.pdf) 350million people in the world were affected by depression. The population in 2012, when that was published, was around 7 billion, giving a prevalence of around 5%. It is not clear what those people affected by depression would score using PHQ2.

There is also one paper which estimates the prevalence of depression in HSP in Estonia. The paper is: The prevalence of depression in hereditary spastic paraplegia, by L Vahter, M Braschinsky, S Haldre, K Gross-Paju, published in Clinical Rehabilitation in 2009 (PubMed ID 19561033, DOI 10.1177/0269215509337186). The abstract indicates that the Beck Depression Inventory was used and that, 44% (21/48) had mild, 13% (6/48) moderate and one person revealed severe depression. My interpretation of this study is that around 60% of people with HSP have some form of depression, which I estimate by summing the different percentages together: 44% (mild) + 13% (moderate) + 2% (severe – 1 out of 48) = 59%.

My conclusion is that the responses to this survey show that people with HSP appear to suffer from depression more than the general population. It is not possible to correlate scores between the PHQ2 used here and the Beck Depression Inventory, however, the Estonian study shows around 60% of people with HSP having some form of depression and my results shows that 63% having a score above zero. This may indicate a similar result.

Quality of Life

The next section of the questionnaire looked at respondents’ quality of life. Respondents were asked 3 questions about physical functioning and 2 questions about social functioning from the Patients Like Me Quality of Life survey (https://www.patientslikeme.com/ and  https://www.openresearchexchange.com/public/library/instruments/16/instructions ). In the full survey there are 11 physical functioning questions, 8 mental functioning questions and 5 social functioning questions. The questionnaire is used across many conditions and I selected a few general questions as a sample.

The questions on physical functioning were:
·         How much has your health limited you in accomplishing as much as you would like to?
·         How much has your health limited you in the type of work or other activities you can do?
·         How much has your health limited you in doing your work or other activities?

The questions on social functioning were:
·         Did your physical health interfere with your social activities with family, friends, neighbours or social groups?
·         Did your emotional problems interfere with your social activities with family, friends, neighbours or social groups?

Respondents selected from options which each have a score out of 4, which is multiplied by 25 to covert it to a percentage, and an average is taken. The average score is then ranked as follows:
·         85-100% - Best
·         50-85% - Good
·         15-50% - Bad
·         0-15% - Worst

In total 102 respondents answered this question, and the following results are shown:



The physical functioning results show that over 60% of respondents score good or best when using no mobility aids or when they are used some of the time (mobility score 0 to 3). There are no clear differences between these two mobility bands. Once mobility aids are used most or all of the time (mobility score 4 to 9) the physical functioning score lowers, with around 30% of respondents scoring good or best. Again, there are no clear differences between these three mobility bands. There may be an upturn in physical function for those most affected by HSP, perhaps as they have optimised their lives to their mobilties.

This sample analysis appears to show that a step change in quality of life occurs at the point when mobility aids are needed to be relied on more often.



The social functioning results show that before mobility aids are needed (mobility score 0 or 1) around 90% of respondents score good or best for social functioning. Once mobility aids need to be used (mobility score 2 to 9), the percentage of respondents scoring good or best drops to around 75%.

Within this, the proportion of respondents scoring best drops from 50% with no mobility aids to around 20% when some are needed. Once mobility aids are used the social functioning score does not change significantly, and this sample analysis appears to show that a step change in quality of life occurs at the point when mobility aids are needed.

The conclusion I draw from this is that HSP does affect quality of life and there appear to be two step changes, the first step change is a reduction in social functioning at the point when mobility aids are needed and a step change in physical functioning when mobility aids need to be relied on most or all of the time.

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
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