Friday, 31 May 2019

Research Paper: Stumbling, struggling, and shame due to spasticity

I found this paper really good. The text of this blog post heavily copies and pastes from the full paper. My key takeaway points are:
  • Applying strategies to manage spasticity was often perceived as a struggle. 
  • Giving up activities is common. Finding alternatives is a struggle.
  • Fear of functional deterioration and frustration from increasing social isolation are common.
  • Participants experience substantial day-to-day fluctuations in spasticity.
  • Limitations in gait and balance have an impact on almost all physical activities.
  • Fatigue is a major consequence of spasticity, impacting on both daily routines and night rest.
  • Participants often felt ashamed from negative judgements made by other people.
  • Frequently used tools for spasticity do not address relevant issues for patients (fatigue, pain, sleep, and general well-being.) 
  • Pain and fatigue have been scarcely addressed in the literature on HSP, even though they are regarded by the patients as some of their most disabling symptoms.
  • Outcomes that address fatigue, energy expenditure, and sleep appear to be lacking in the literature on chronic spasticity in persons with HSP.
The full details of the paper are:

Title: Stumbling, struggling, and shame due to spasticity: a qualitative study of adult persons with hereditary spastic paraplegia

Authors: Hans C. J. W. Kerstens , Ton Satink, Maarten J. Nijkrake, Bert J. M. De Swart, Bas J. H. Van Lith , Alexander C. H. Geurts  & Maria W. G. Nijhuis-van der Sanden

Link to abstract

The paper describes how 14 people with HSP from The Nederlands were interviewed about their HSP, their mobility and the consequences of spasticity, as well as the various treatments they receive. The 14 people all had one of the pure/uncomplicated forms of HSP. They were a mix of men and women over a wide age range.

The authors interviewed the people and looked for patterns in their results, and each of the patterns is introduced with a quote from one of the patients. Four different areas were identified, which are described below.

"I stumble"

All people identified stumbling as an issue. The stumbling was sub-divided into four categories:

  • Pain and stiffness - "Sometimes the pain feels like I’ve been run over by a train"
Almost all people consistently mention leg pain, and some also experience back pain. Stiffness and pain increased the time that people have to remain seated or laying down, and the stiffness makes movement inconvenient, 

  • Fatigue - "I am so tired that I could sleep standing up"
Many people identify that gait impairments require more effort and energy, leading to high levels of fatigue. Fatigue is exaggerated with poor sleep.

  • Impaired balance and gait - "I have difficulty with all the activities that require me to use my legs"
All people had problems with gait and balance control. Toe-walking and slower speed are common. Standing and walking are regarded as very energy demanding, and many experience limitations in a wide range of daily activities. (personal hygiene, housekeeping, employment, mobility and leisure activities - etc.). Getting in and out of cars is difficult for many. Some are accustomed to regular falls. Exercise and sport are viewed as important, but it is difficult to find a suitable one.
  • Day-to-day fluctuations - "Some days I feel like a wreck, but sometimes I don’t feel too bad at all"
Some people have stable conditions whereas others have symptoms with strong and unpredictable day-to-day variation. All participants notice a gradual long term increase in the progression of HSP.

"I struggle"

All people make adaptions to live with their HSP. There are two categories identified.
  • Continuous adaption - "I have to think before I do something: First I have to be standing steadily, then I can lift the groceries into the car"
A wide variety of aids and adaptions are used, and support is given by spouses or others. Walking aids are regularly used, routes are planned and obstacles/difficulties are remembered and avoided. The adaptions help people carry on performing activities, keeping independence. Finding the right balance between keeping active and taking rest is difficult - insufficient movement increases stiffness but too much activity reduces leg stability/balance.
  • Quitting - "Diving is now impossible because I can’t control my feet any more".
After a certain time applying adaption strategies is no longer possible. When an activity is stopped, some search for less demanding activities instead. Quitting activities also affects employment, sometimes the effects of HSP makes finding an alternative position more difficult.

"I feel ashamed"

Feeling judged and ashamed is a very significant category of emotions felt my many people, and fear and frustration is another category felt.

  • Judged/ashamed - "At parties I don’t drink much wine, otherwise they think I’m drunk."
People regularly encounter negative judgements from people or feel ashamed of how they walk because of looks/comments made. Several have to endure other peoples opinions particularly around the use of walking aids or mobility devices. People talk more freely amongst friends about spasticity and is consequences.
  • Fear/frustration - "I am so scared that I will deteriorate further"
People fear the slow but inevitable progression of HSP. People try to stay as active as possible to prevent functional decline. Frustration is felt about slowly reducing social roles and becoming more homebound leads to fewer topics of conversation. Some find it difficult to accept spasticity, some become angry when they cannot do things, some are depressed wondering why they are affected.

"I need support"

All people needed some support. Four categories were identified:

  • Adequacy of information - "I want more information than just the explanation about what the abbreviation HSP means"
There is a need from people with HSP and caregivers about spasticity and its consequences. Information should include how to deal with spasticity, and the advantages and shortcoming of different options.
  • Efficacy of interventions - "Thanks to the injections, the tips of my shoes don’t need to be repaired so often"
Different people have different approaches to medications, depending on their experiences and side effects. Many perform stretching exercises, either by themselves or with a physiotherapist. Many find a combination of home-based stretching with physiotherapy to be optimal as some need motivation to carry on. 
  • Quality of healthcare professionals - "My GP … does not know much about this"
People are usually satisfied with people working in centres of HSP expertise. Most other healthcare professionals are unaware of HSP. In these situations there is an absence of thorough assessment, personalised instruction, easy access, regular checks and shared decision making. 
  • Insight in HSP - "The symptoms creep up on me, but with a video I could see the difference"
Although people don't want to be confronted too much by their progression, they did want information about spasticity fluctuations and the influence of interventions on this. It would be easier to adhere to an exercise plan if the effects of efforts could be noticed. Objective feedback would identify if experienced changes were real or not. Monitoring movements is important but should not be time consuming.


Applying adequate strategies to manage spasticity and its consequences was often perceived as a struggle. Participants often felt ashamed as they experienced negative judgements by persons in their environment. People expressed the need for medical and practical support from professionals in both centres of expertise and the community. Key messages from the participants are:

  • Pain and stiffness are cardinal features of HSP. 
  • Fatigue is a major consequence of spasticity, impacting on both daily routines and night rest.
  • Limitations in gait and balance have an impact on almost all physical activities. 
  • Within the gradual progression of spasticity, participants experience substantial day-to-day fluctuations. 
  • Spasticity is energy demanding. 
  • Giving up activities is common in various domains of daily life. Finding alternatives is a struggle. 
  • Moving differently than others causes feelings of shame and being judged. 
  • Fear of functional deterioration and frustration about gradually increasing social isolation are common. 
  • There is a need for reliable medical information as well as practical information on how to deal with spasticity. 
  • Botulinum toxin injections induce fluctuations of spasticity, which are inconvenient.
  • Physiotherapy can provide coaching and motivation to continue home exercises over time.
  • Healthcare providers in expertise centres should support healthcare providers in the community with specific knowledge of how to treat and coach patients with HSP. 
  • Monitoring of physical impairments and activity limitations is important to get a better grip on the consequences of lower-limb spasticity on daily life
Spasticity is more than just stiffness 
Participants reported both well-known physical consequences of chronic lower-limb spasticity (stiffness, leg pain, stumbling gait pattern) and less obvious consequences (back pain, fatigue, and unpredictable day-to-day fluctuations). Nearly all participants complained about lower back pain, which they believed to be due to an altered posture and gait pattern. Both strenuous gait and disturbed sleeping pattern were responsible for high levels of fatigue. Together, these symptoms had a huge impact on their daily lives. 

Pain and fatigue have been scarcely addressed in the literature on HSP, even though they are regarded by the patients as some of their most disabling symptoms. 

The need to address outcomes that are meaningful to the patient 
The most frequently used measurement tools in the rehabilitation of chronic spasticity are focused on the level of physical impairments. These do not address the issues that are most relevant to the patients, such as fatigue, pain, sleep, and general well-being. Outcomes that address fatigue, energy expenditure, and sleep appear to be lacking in the literature on chronic spasticity in persons with HSP. 

Personal feedback enhances individual grip on spasticity 
Participants found it difficult to differentiate between disease progression and the wearing-off phenomenon following treatments. Participants also reported that both internal factors (stress, fatigue) and external factors (temperature) could influence spasticity. 

Strengths and limitations 
This study has strengths in the method of data collection and analysis. Limitations include: topics such as bladder issues or sexuality were not addressed, and results are limited to patients with a pure form of HSP.


The consequences of chronic lower-limb spasticity in patients with pure forms of HSP go beyond the well-known consequences such as muscle stiffness and impaired ambulation. HSP is associated with fatigue, pain, unpredictable day-to-day fluctuations, a wide range of physical activity limitations and social participation restrictions, and the continuous need to adjust compensation strategies to overcome these limitations. 

In addition, feelings of shame, fear, frustration, and depressed mood coincide with the disabilities experienced and the judgements made by persons in the social environment. The participants expressed a strong need for interventions not only to reduce the neuromuscular impairments, but also to alleviate fatigue and pain, to improve nighttime rest, and to restore the capacity to perform relevant physical activities and fulfil social roles. 

Spasticity rehabilitation regimens should therefore be tailored to the individual needs of the person with HSP, based on careful personalised monitoring that includes patient-reported outcomes. Enhancing self-management may empower patients to gain control of the consequences of chronic spasticity in their daily lives.

My Final observations

I'm very pleased that this paper has been published. It puts the frustrations that people often have with HSP into clear understandable language. A lot of the findings are similar to those reported in my survey results. 

Friday, 10 May 2019

Symptoms update: (all the) small things

No, its not the start of the song (Blink 182, 1999), but a collection of a few small differences that I've noticed in recent months. This is, therefore, a symptoms update post.

Bladder Medication

I had been finding that my bladder was beginning to annoy me a bit more, especially in the afternoons. I mentioned this when I went for my appointment in London at the National Hospital. It was suggested that we just increase my medication. When I went for my GP appointment afterwards we looked at the prescription I was on and the scope for increasing the dose.

It seems that I am already on the maximum dose for this medication, so we are trying out two different approaches. The first approach (I've not actually got round to trying yet) is that the medication is available in half doses, so the option is to take a half done in the morning and another in the evening.

The second approach (the one which I am trying first), which is simply to take the medication in the morning rather than in the evening before going to bed. I had read that one of the symptoms was that the medication might make you sleepy, so I had decided to take it at the end of the day. However, the thought is that the medication is wearing off before the 24 hours is up. By taking it in the morning I get the full day of benefit, and I'm asleep at night. This turns out to be effective. I'm not noticing the tiredness side effect, and the problem appears to have gone away.


I'm finding that it is slightly less comfortable to lay on my back with my legs straight than it is to lay on my side with my legs bent. I suspect that this is some residual spasticity in my legs. There's a part of me which wants to lay on my back and get a good long light stretch in my legs leaving them straight, whereas in practice I'm tending to roll onto my side and bend my legs to be more comfortable.


When we stayed in the Lake District at Easter we were in a small cottage. The stairs were very steep to get upstairs, and included a corner. I noticed that it was more difficult to get up and down those stairs than it is to get up and down the stairs I usually encounter at home and work. Progress on the steep stairs was manageable, but slow. I had to make much more use of handrails and other hold-ables than normal, and carrying a cup of coffee upstairs was somewhat a challenge! Perhaps this a preview of what is coming later down the line.