Exercise physiologist Lisa of Advance Rehab Centre in Sydney did a webinar on ‘Seated Exercise at Home’ for the HSP community in October 2022.
Building on that, here are two more exercises from her that can help increase step length, improve balance and counteract hamstring tightness.
Lisa writes:
General strengthening of hip extension helps with increasing step length (usually smaller step length in people with HSP if there’s glute weakness) and overall hip control to help with improved single leg balance or balance in reduced base of support. Quad strength – general for targeting weakness in quad muscles if they’re affected by HSP can have trouble straightening the leg, can also help offset any hamstring tightness.
Exercise 1: Kneeling squat to shoulder press
Start by kneeling on your feet with your head and chest tall, if you struggle with this end range, place a pillow/cushion on top of your lower legs to reduce the range of motion. When you’re ready, rise up into a high kneeling position. Extend your arms up from your chest to above your head, and then back down – you can add a weight as a progression. Lower your body back down, slowly by using the quads to control the kneeling back down.
Muscle groups targeted: Quads, glutes, core, shoulders
Benefit for HSP:
Provides a quad stretch at the beginning of movement
Works on core control
Targets hip extension (glutes)
Forces the quads to control eccentrically
Functional position.
Exercise 2: Banded glute bridges
This is a way to add load to a traditional glute bridge exercise. Lying on your back with your knees up and feet flat on the ground, hold a theraband (exercise band) across your hips (you can adjust the tension as needed). Lift your hips up by pushing off your heels, hold the top position for a second before lowering back down.
Muscle groups targeted: Glutes, hamstrings, core
Benefit for HSP:
Add load for hip extensors (glutes)
Core activation
Provides a stretch at end range for hip flexors and quads.
Chair of the UK HSP Support Group, Adam Lawrence, and Secretary of Euro HSP, Lori Renna Linton, both of whom have HSP, collaborated as members of the European Reference Network on Rare Neurological Diseases (ERN-RND) to chart the journey that people with HSP take.
The HSP Journey
A more detailed version of the chart here (need to Zoom to enlarge once open).
Patient Journeys are info-graphical overviews that visualize patients’ needs in the care of their rare disease. Because Patient Journeys are designed from the patient’s perspective, they allow clinicians to effectively address the needs of rare disease patients.
Patient Journeys consider that patients’ needs may differ at different stages of the disease – e.g., initial symptoms vs. treatment. They also reflect the patients’ personal experiences, which may vary depending on the person, clinic and country.
ERN-RND considers Patient Journeys working documents that patients and clinicians can use together to identify gaps in care and adapt care pathways to better meet the needs of patients living with these conditions. Patient Journeys can therefore be seen as a first step toward systematic patient engagement in the design of care pathways.
In addition, Patient Journeys are a useful resource for patients, families, non-specialist clinicians, and the general public to understand the care needs of patients living with a rare neurological disease.
The Patient Journey for Hereditary Spastic Paraplegias (HSPs) was developed together with Adam Lawrence, chair of the UK HSP Support Group and Lori Renna Linton, ERN-RND ePAG representative for: Ataxia/HSP.
If you are worried about someone’s mental well-being and unsure what to do about it, this guide to planning a conversation (from Mental Health First Aid Australia) can help.
Pick your moment
It’s important that you have adequate time to listen, focus and respond.
Right frame of mind
When preparing for a conversation about someone’s mental state, your principal focus should be on connecting and offering support. That includes being in the right frame of mind to discuss emotional or stressful things, and an understanding that the person may not be ready or willing to talk.
Choose the venue
Find somewhere that you won’t be interrupted or distracted, preferably somewhere calm, safe, neutral, conducive to conversation and sufficiently private.
Creating a safe and supportive space begins by showing a genuine, non-judgemental interest in what they are experiencing and their welfare. Use ‘I’ statements over ‘You’ statements e.g. “I’ve noticed a change in your behaviour lately” over “You have changed”
Ask open-ended questions to encourage the conversation e.g. “What’s that like for you?” over “So you feel sad?” Limit comparisons, especially if they have the potential to invite shame e.g. “How are you coping?” over “You’ve never acted like this before.”
Are you being the person they need?
Establishing the trust and connection required for a mental health conversation, requires more thoughtful consideration than a day-to-day conversational approach, and what might get someone through a tough day in the office or in their family life, may not be appropriate if they are experiencing a mental health concern.
Consider how you communicate your intentions through both your attitude and words:
Are you promoting disclosure through a genuinely supportive tone?
Are you reducing the chance of deflection by avoiding sarcasm and inappropriate humour?
Are your questions and statements judgement-free?
Are you able to speak about the problems at hand without dismissing or minimising their impact?
Are you able to listen without imposing your beliefs, experience and/or your own coping mechanisms?
Make it sustainable
When facilitating a mental health conversation, you may hear or feel things that impact you in ways you didn’t anticipate. Your safety and well-being is as important as the person you’re looking to support.
Consider the impact the length of the conversation may have on you and the person you are talking to. Overly long conversations about stress and/or mental health topics can emotionally fatigue both parties.
Normalise and encourage seeking additional. The conversation is the starting place, not the destination.
Having deep and difficult conversations may leave you feeling drained, both physically and emotionally, so it’s important to set aside time to reflect and practice self-care. Self-care techniques include:
Taking a walk
Engaging in exercise
Enjoying quiet time with cup of tea, puzzle, or a book
Reach out to someone you trust if you need support.
The Spastic Paraplegia Rating Scale (SPRS) is an HSP-specific clinician-reported outcome measure (CROM). It was the only measure that showed the clinical progression that had taken place over a year in this study of 55 people with HSP.
There are no specific patient-reported outcome measures (PROM) for HSP, and unsurprisingly, the PROMs used in the study failed to detect change in HSP status over the year.
The study authors propose creating a disease-specific PROM fully depicting the effect of HSP on the lives of people with HSP.
Background and objectives: Hereditary spastic paraplegias (HSPs) are a heterogeneous group of rare neurodegenerative diseases, characterized by a progressive spastic paraparesis. Currently, there is a HSP-specific clinician-reported outcome measure (CROM) called Spastic Paraplegia Rating Scale (SPRS). There are, however, no specific patient-reported outcome measures (PROMs) for HSP. In the present cohort study, we prospectively follow up a well-examined Austrian HSP cohort using validated rating scales and compared PROM with disease-specific and non-disease-specific CROM.
Methods: Patients were recruited and followed up at the Center for Rare Movement Disorders, Innsbruck, Austria. CROM included the SPRS, Scale for the Assessment and Rating of Ataxia (SARA), Barthel Index (BI), and Mini-Mental State Examination (MMSE). PROM included the EQ-5D questionnaire and the Patient Health Questionnaire 9 (PHQ-9). Standardized response means (SRMs) were calculated for all scales at follow-up (FU) after 1 year.
Results: A total of 55 patients (36 males) with HSP were included in the study. FU was performed for 30 patients (21 males). Apart from females reporting more problems in the EQ-5D domain of anxiety and depression (p = 0.008), other clinician-reported outcomes (CROs) or patient-reported outcomes (PROs) did not differ significantly across sex. SPRS showed significant correlations with SARA (p < 0.001), mainly driven by the gait item, as well as the BI. Although SPRS did not correlate with EQ-5D visual analogue scale and PHQ-9 scores, several EQ-5D domains correlated significantly with SPRS. At FU, SPRS showed the highest responsiveness (SRM 1.11), followed by SARA (SRM 0.47). Neither MMSE nor PRO significantly increased at FU.
Discussion: In this study, we present an Austrian cohort of patients with HSP and a prospective study evaluating correlations of CRO and PRO as well as their progression. Demographics from our cohort are comparable with several other European cohort studies. Our data highlight the capabilities of the SPRS to show clinical progression and warrant consideration of ataxia rating scales such as SARA in HSP cohorts. We also show that the generic PROMs are not suitable to detect change in HSP, and thus, we propose to create a disease-specific PROM fully depicting the effect of HSP on the patients’ lives.
1. Centre for Rare Neurological Diseases (M.A., E.I., A.E., W.N., S.B.), Department of Neurology, Medical University of Innsbruck; and Department of Medical Statistics (J.F.), Informatics and Health Economics, Medical University of Innsbruck, Innsbruck, Austria.
Radboud University in the Netherlands has been highly regarded for a long time in its research of HSP gait.
This study of a 5 week gait adaptability training in 36 people with pure HSP employing a treadmill equipped with augmented reality (C-Mill) found no conclusive evidence of improvement across a range of measures related to obstacles, balance, falls and various gait parameters.
It would be premature to conclude that gait adaptability training has no value for people with HSP as there may be other machines and training regimens where improvement is possible.
C-Mill gait training
Background and objectives: In people with hereditary spastic paraplegia (HSP), reduced gait adaptability is common and disabling. Gait impairments result from lower extremity spasticity, muscle weakness, and impaired proprioception. The aim of this study was to assess the efficacy of a 5-week gait-adaptability training in people with pure HSP.
Method: We conducted a randomized clinical trial with a cross-over design for the control group, and a 15-week follow-up period after training. Thirty-six people with pure HSP were randomized to 5 weeks of (i) gait-adaptability training (10 hours of C-Mill training-a treadmill equipped with augmented reality) or (ii) a waiting-list control period followed by 5 weeks gait-adaptability training. Both groups continued to receive usual care. The primary outcome was the obstacle subtask of the Emory Functional Ambulation Profile. Secondary outcome measures consisted of clinical balance and gait assessments, fall rates, and spatiotemporal gait parameters assessed via 3D motion analysis.
C-Mill augmented reality
Results: The gait-adaptability training group (n = 18) did not significantly decrease the time required to perform the obstacle subtask compared to the waiting-list control group (n = 18) after adjusting for baseline differences (mean: -0.33 seconds, 95% CI: -1.3, 0.6). Similar, non-significant results were found for most secondary outcomes. After merging both groups (n = 36), the required time to perform the obstacle subtask significantly decreased by 1.3 seconds (95% CI: -2.1, -0.4) directly following 5 weeks of gait-adaptability training, and this effect was retained at the 15-week follow-up.
Conclusions: We found insufficient evidence to conclude that 5 weeks of gait-adaptability training leads to greater improvement of gait adaptability in people with pure HSP.
1. Department of Rehabilitation, Center of Expertise for Rare and Genetic Movement Disorders, Donders Institute for Brain, Cognition and Behavior, Radboud University Medical Center, Nijmegen, The Netherlands.
2. Department of Neurology, Center of Expertise for Rare and Genetic Movement Disorders, Donders Institute for Brain, Cognition and Behavior, Radboud University Medical Center, Nijmegen, The Netherlands.
3. Research, Sint Maartenskliniek, Nijmegen, The Netherlands.
4. Department of Rehabilitation, Sint Maartenskliniek, Nijmegen, The Netherlands.
Many people with HSP report that amongst all the natural stress, fear, anxiety and uncertainty that goes hand-in-hand with a diagnosis of HSP, having reliable and readily accessible information to answer the thousand questions you have would have been really helpful.
We plan to develop a section of the website to provide such information for people newly diagnosed with HSP and we need your help to do that, especially if you did not have such support when newly diagnosed yourself.
Please email us your thoughts or add them in the Comments box at the bottom of the page. Here are three questions as a prompt:
What questions did you have when you were newly diagnosed?
What information would you have liked to have access to?
What else would have been really helpful?
Thank you for your support in providing this information that will help others in the future.
The Foundation presented a webinar on ‘Seated Exercise at Home’ led by exercise physiologist Lisa Meng of Advance Rehab Centre in Sydney in late October.
The webinar topic was the top-rated choice in a survey of people at the previous AGM.
It was a highly informative and practical session with participants doing the exercises as they were presented.
Use this video to guide your seated exercise program at home. Just follow along with the exercises until you learn the routine. This could be a daily or every-other-day program.
Always consult your doctor before starting a new program.
HSP community member Michael talks about his challenges with mental health in a recent newspaper article in rural Victoria where he lives.
Michael
After hitting rock bottom, Michael took control of his mental health through his participation in the mental health support group GROW. Michael was at his lowest point when his physical health took a turn for the worst. After an accident that left him with a fractured spine, Michael was diagnosed with HSP along with spinal osteoporosis. Lying in a hospital bed in Tatura for 12 weeks coming to terms with the reality that he would slowly lose control of his legs, Michael felt despair. On the same day Michael’s sister was also diagnosed with HSP.
During his convalescence, Michael came across a pamphlet for the mental health support group GROW. Attending GROW has helped him to manage his mental health. He has been attending GROW for six years, and is now the organiser of his local group.
The GROW program is a 12-step process of personal growth, mutual help and support. “It’s a peer support group, so everybody is in a group of members, people with the lived experience of mental illness, which helps a lot,” Michael said.
Our meetings take place in a safe, caring and confidential way, whether the meetings are face-to-face, or online, known as eGrow groups. Our members meet weekly and follow the Group Method. Meetings run for about two hours. Groups vary in size from 3-10 members. Find a Group here.
eGrow groups run the same as the Grow Program. The only difference is it’s an online mental health support group! We use Zoom to connect with your eGrow group which is based on the traditional Grow Program.
You can join a group and participate in the Grow Program from the comfort of your own your house. If you’re unable to attend a physical Grow group you can access meetings online across Australia at a time that is suitable to you by using eGrow for your mental health support.
Over the course of each meeting, the group engages in a series of group discussions that follow the same structure and format as a face-to-face meeting; the only difference is it’s online!
Any member from any location can now receive mental health support and connect with others at a time most convenient to you.
If you’re interested in learning more about our Grow Groups, the Welcome to Grow mental health support group orientation takes place each Monday at 7.30pm (AEST) and on Thursday’s at 1pm (AEDT).
For more information or to see the times of our eGrow groups, please register your interest by using the sign up for eGrow button below.
I’m now 61 years old, with SPG4 HSP, use two trekking poles outside the house and a single cane (or furniture surfing) inside. Before the pandemic, for years I was often in the gym, doing weight work and stretching, as instructed by my physio. However, for the past two years with the pandemic, I stopped the gym, but I realized I needed to find some sort of cardio workout for good heart health. I found that in water exercise, I just wish someone had convinced me to do it 10 years ago! I think it’s a great workout.
I’d heard others talk about pool work and I thought they meant swimming. In googling around for HSP articles I learned that a group of German HSPers began doing pool work. I found this (translated), “Experience aqua fitness with lots of fun and music – gymnastics at a depth of 1.34 m and 28.5°C warm water of our indoor pool. Our aqua course is taught by qualified trainers”. They plan to create a video when they’ve learned more.
My local library had one book about water workouts (Water Exercise by Melissa Layne) and from it I learned this:
Hydrostatic pressure facilitates the efficiency of the cardiovascular system. With constriction of blood vessels, the heart is pumping through a smaller area so does not need to pump as often and the heart rate decreases.
Water provides resistance in every direction, so you work opposing muscle groups at the same time. This resistance is referred to as ‘drag’. Your body must exert muscular force that is 12-15 times greater than the force needed when moving through air, thus developing muscular fitness. Water also provides a stabilizing effect, meaning you don’t fall down.
Warmer water decreases muscles spasticity.
Ideal water depth is mid-chest deep. At this depth you can plant your feet flat on the pool bottom.
Water exercise improves all facets of heart health.
I found a heated indoor pool nearby with various classes, went to check it out, feel the water temp and caught a class in session. I decided to give it a try. It was hard for me! The instructor led the session from the pool’s edge. We did all this hard stuff and then the instructor said, “okay, that’s the 10 minute warm up, now we’ll begin”. I won’t tell you what I said to the woman next to me but she said, “it’s okay, we all just do what we can”. The instructor suggested I shift to a less challenging class and that was clearly a better option for me. That was in April this year and I’ve been doing aqua workouts ever since.
In the pool classes everyone wears water shoes (also called aqua shoes or reef walkers) as it’s important to have some grip on the pool bottom. I decided early on to also buy long water pants to keep the leg muscles warm and reduce spasticity. I also wear a long-sleeved pool top over my bathing suit, again to stay warmer. People in the classes who need them wear glasses or hearing aids. In typical hour-long aquatic classes, we walk, march, bicycle motion, walk backwards and do those movements with variations but we are all generally upright for the entire session. Some of these movements were very difficult with my lack of lower body strength and poor balance. When I was new to aqua workouts, I had a private session with the instructor. She clearly saw my poor balance and recommended I use the light weight pool barbells for balance. They are enormously helpful for maintaining stability.
I am now to almost 50 pool sessions and really enjoy them! I have built muscles and increased strength, including hip muscles. My legs are different – in a good way. I can tell by the way my shorts, underwear and pants fit. HSP causes weakness below the waist and poor balance for me, so building strength and improving balance is helping. I’m better going up stairs. The regular cardio workouts are good for my heart health and I’ve made some new friends in the pool.
I mentioned balance just above. I did a second private lesson with the instructor. She has a Master’s Degree in Recreational Therapy with a specialty in Aquatics and has been teaching aquatic workouts for over 20 years. I was asking her thoughts on building balance in the pool. She had me put down the pool barbells that I use so often and instead put on aquatic gloves. I can say more about that if anyone gets in the pool and is curious about balance work.
Now sometimes I do solo workouts. Whatever moving around in the water I do, I’m pushing against the water so it’s all good, but my time is valuable too, so I have worked out a routine that I like. I spend 15 minutes warming up by walking and marching forward, sideways and backwards. Then, standing chest deep next to the pool wall, I do some of the HSP exercises I found online here: https://treathsp.net/klinik/physiotherapie/uebungen. This is in German but the videos are self-explanatory. Although these are in a gym setting, in the pool I get the extra benefits of pushing against water and not falling down! I do about 15 minutes of exercises 2,3,9 and 10. Then 15 minutes doing exercises #1,5 and 6 on the pool steps and finish with about 15 minutes of balance work, walking in the pool wearing the aqua gloves.
I’m really happy to have discovered aquatic exercise.
I asked my instructor what leads to lifting the feet when walking, as I tend to drag my feet and not get enough ground clearance. She said it comes from two things, a backward kick followed by a forward kick. That’s TWO things I have trouble doing and I need to do them both in succession? But I’ve been working on this in the pool. When I’m warming up, I’ll walk this way, lifting my foot behind me (like I want to kick myself in the backside) and then swinging that leg forward… followed by the other leg. My hamstrings are weak but I’m gaining strength by practising walking this way in the pool.
The instructor also suggested spending some time facing a wall and lifting, lifting, lifting my toes while keeping weight on my heels. I find it easier to do this, not by working harder but by relaxing more.
SOURCES:
Human Kinetics Publishers
Water Exercise
by Melissa Layne
Gait Posture. 2014 Apr;39(4):1074-9. doi: 10.1016/j.gaitpost.2014.01.010. Epub 2014 Jan 29.
The effect of hydrotherapy treatment on gait characteristics of hereditary spastic paraparesis patients
1 Department of Sport and Exercise Science, University of Auckland, New Zealand.
2 Auckland City Hospital, Private Bag 92-024, Auckland, New Zealand; Centre for Brain Research, University of Auckland, Auckland, New Zealand.
3 School of Nursing, Faculty of Medical and Health Sciences, University of Auckland, New Zealand; Institute of Healthy Ageing, Waikato District Health Board, Hamilton, New Zealand.
4 Department of Mechanical Engineering, University of Auckland, New Zealand.
