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: https://www.tandfonline.com/doi/full/10.1080/09638288.2019.1610084

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.

DISCUSSION

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.

Conclusion 

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.