Recumbent tricycle for HSPers

Modern ultra low ‘tadpole’ style

 

I use a recumbent ‘tadpole’ tricycle. I think three wheels are much better than two. I don’t know if you have ever seen one. Most bike shops do not even carry them, or know little about them. Many of us are familiar with upright bicycles and upright tricycles but how many of us are familiar with the modern ultra low recumbent tricycles with two wheels facing forward?

David on his tricycle
David on his tricycle

 

Recumbent tricycles have the pedals out the front, three wheels, a full comfortable supportive reclined seat, and are very low to the ground. ‘Tadpole’ configuration has two wheels at the front and one wheel at the back. They are much more comfortable than a regular upright bike or upright trike.

 

Recumbents of any form were banned from push bike racing in 1935 by the International Cycling Federation but are currently making a strong comeback. Many Australian schools now race 3 wheel recumbents under a new association.

 

There is a big difference between what we learned about riding a bike as a kid and learning to cycle efficiently as an adult. Many parents feel pride when their young child first learns to ride a bike. Learning to ride a bike has become a rite of passage for many children. It is often very difficult as adults living with HSP, especially when we can no longer ride a bike but our small children can.

 

HSPers can make good use of recumbent tricycles but we will never be as fast as many people who do not have HSP. I have found that I can travel faster than many youthful school leavers but not as fast as my regular cycling peers.

 

The key to good cycling technique on any cycle is to pedal your feet in ‘circles’, not in ‘squares’ and to have a proper ‘fit’ between your body and your cycle. Make sure it is properly set up for you. Much can be learned about cycling from the internet. Good cycling shoes can make a big difference.

 

Shoes that let you exercise your whole leg

Cycling shoes that clip onto special pedals let me exercise my whole leg not just part of it. I can both pull and push with my legs. Most bicycles are fitted with flat platform pedals that only allow you to push. People usually first learn to ride a bike with these flat pedals on it. The more serious rider clips their feet onto the pedals with cycling shoes and matching pedals. Proper cycling shoes are the key to exercising your whole leg.

 

There are two basic types of cycling shoes, ‘mountain bike’ and ‘road’ shoes. I find it very difficult to walk in road shoes due to the design of their pedal clips. I don’t need any more wobble in my walk than HSP gives me! I use mountain bike shoes and matching pedals. Mountain bike shoes can also be known as SPD shoes. These have the clip section indented in the outer sole of the shoe. I can walk in mountain bike shoes. They are ‘walk-able’ cycling shoes.

 

Mountain bike shoes clipped onto their pedals allow me to pull up with my feet as well as push down with my legs. Ordinary flat platform pedals do not allow me to pull up with my feet while cycling. I can use a far better cycling technique and spin the pedals at a much faster rate (higher cadence) using mountain bike shoes than flat platform pedals. Quickly spinning the pedals around also reduces the strain on the knees.

 

Another advantage of these shoes for HSPers is that my feet are kept in place while cycling by my shoes. Without them it is difficult for me to keep my feet in the right place due to my HSP.

 

Muscle exercise and development

I can exercise and develop all the different muscle groups in my legs, if I use mountain bike shoes with good cycling technique. Before I got my tricycle, my calf muscles were wasting away but now they get regular exercise and have developed some definition. My quadriceps at the front of my thighs are also ‘bulking up’. The hamstring muscles in the back of my thighs are stretching and developing. The muscles in my shin used to lift my feet are also being exercised and developed. My bottom muscles (glutes) are toning up. The small muscles in my hips used to lift my entire leg are also becoming stronger and being developed. My cycling will not cure HSP but I can help what muscles I have to become stronger.

 

Other health benefits

There are several other health benefits for HSPers, when they regularly ride three wheel recumbents.

 

I do not get swelling in my ankles any more. There is a basic cycling technique called ‘ankling’. ‘Ankling’ is where you bend your ankle to assist your pedalling. The regular bending of the ankle while pedalling assists pumping any fluids back up to my body. I can best use this technique while my feet are ‘clipped in’.

 

The comfortable recumbent seat also helps develop and tone core trunk muscles making it easier to keep my balance while walking or while using a wheelchair.

 

Riding a recumbent is a fully supported exercise. If it gets too much I just stop and have a rest. I also get a good general cardio and aerobic workout that strengthens the heart, fully uses the lungs and diaphragm, loses weight, improves circulation, improves cognitive skills and is not as boring as a static exercise bike in front of the TV.

 

Balance can be an issue for HSPers. I do not have good enough balance to walk or ride a bicycle. With three wheels the tricycle stays upright. Without the need to balance, I can put further effort into my cognitive abilities of pedalling, steering and braking.

 

There are improvements in my endurance and general fitness. I can ride a long way. I sit for most of my waking hours. It is easy for me to choose not to exercise. When I started to ride a recumbent tricycle, I could only travel 3.6km. Now that I am fitter, 50km is not difficult for me.

By riding my recumbent tricycle around, I’m improving my fitness and getting outside more often. I enjoy exercising this way.

 

My self esteem has also improved by riding a recumbent trike. I get to participate in large cycling activities with many able bodied cyclists. These events are not races. Some activities are fund raisers for people with disabilities similar to HSP. They are good fun to join in. The largest one I have been in had 10 000 cyclists. I get kudos from other cyclists for simply turning up with a recumbent tricycle. They notice the unusual mode of transport, not that the rider operating it is disabled. They do not need to know I cannot go as fast as many of them or that I have HSP. No one needs to know I’m disabled when I am riding one of these. Some people who previously know of my disability cannot believe I can participate or ride at all.

 

I also appreciate being able to ride with my wife and family and friends. It is good to be able to do something outdoors together. They are often surprised how well I can keep up with them.

 

Going over hills

At the start of the 'Ocean Ride for MS' in WA
At the start of the ‘Ocean Ride for MS’ in WA

Using all the different muscle groups in my legs adds about 10% more power to my cycling. This does not sound like much extra power but it can be the difference between comfortably getting over the hills I may encounter or not. Fortunately, most recumbent trikes have very low gears. If the hill looks too steep, just change to a lower gear and ride up the hill at a slower pace. If you find the hills are still too big you could add an electric motor to your recumbent tricycle.

 

Three wheel recumbents are also known as Human Powered Vehicles (HPV). Human Powered Vehicles are a viable alternative to using a car in your local area. They can carry small loads and can easily travel many kilometres from home.

 

If you have not done any exercise recently, I would recommend you first see a GP and tell them what exercise you are about to do. You may wish to consult your physiotherapist or occupational therapist or other health professional. They are not likely to know about recumbent tricycles and HSP but they do know what exercise is good for you.

 

Difficulties, what difficulties? There are no difficulties here!

The most difficult aspect of a recumbent tricycle is simply getting in and out of them. They are often very low to the ground. I use my arms a lot for this. A low wall or a shallow hole in the ground or a post to hang on to means I have no difficulty getting in or out of them, once I learned how. If there are none of these supports around, I lock the brakes and use the front wheels to push myself up or to sit back down again easily.

 

If I am unfortunate enough to get a flat tyre while riding, I simply get off and quickly fix it. I carry all the necessary equipment. The vast majority of times I never get a flat tyre. Some tyres are made so tough they almost never get a puncture. Recumbent trikes are less likely to get flats than bicycles.

 

If I have a crash, I’d much rather be on a recumbent than an upright bicycle or tricycle. At one time I crashed my recumbent trike at 51km/h. I was not injured and suffered only minor bruising. Being so low to the ground meant I did not have far to fall; also I crashed feet first. I’d rather crash feet first than head first. On a upright bike I would have gone over the handlebars head first. This crash did not hurt me much. Not all crashes are this good. Some crashes are quite injurious. I do not recommend crashing.

 

Many recumbents can quickly be folded in half and fit easily into a small car. It can be an advantage to get a recumbent tricycle that folds. I can easily get mine into a small car and drive to the start of a ride.

 

The two wheels in front ‘tadpole’ style means they can steer and brake very well; often better than a two wheel bike. They are so low to the ground that they are very stable going around corners. Nor do I need to ‘unclip’ my foot every time I stop at traffic lights. I simply watch my two wheeled peers try to unclip their feet in time before they fall over.

 

Medical equipment or an expensive indulgence?

Recumbent tricycles have many health benefits and can be seen as medical equipment for HSPers. They can also be very expensive. They are at least twice as expensive as the equivalent quality two wheel bike. Don’t let the expense put you off. Think of these tricycles as a piece of medical equipment for your HSP rather than an expensive indulgence. Learn to ride with good cycling technique, use mountain bike or ‘SPD’ cycling shoes, and most of all have fun.

 

David Moller

Western Australia

September 2013

 

Pain and spasm relief in the wardrobe

Elastic body suit technology developed

 

For those with neurological disorders — such as HSP, MS or Parkinson’s — treatment could be as close as the wardrobe.

 

Elastic Body Suit
Elastic Body Suit

Improved range of motion and reduced pain for people with brain injuries and neurological disorders may now be available with a specially-designed elastic body suit fitted with electrodes, which was designed at Stockholm’s KTH Royal Institute of Technology in collaboration with health care and business partners.

http://www.kth.se/en/aktuellt/nyheter/suited-for-treatment-of-brain-damage-1.421729

The Mollii garment provides an alternative to painful treatments and surgery, by treating the body with electrical stimulation to ease tension and spasms. The result is reduced pain perception and increased mobility.

 

The idea originated with a Swedish chiropractor, Fredrik Lundqvist, who worked with rehabilitation of brain-damaged patients. Lundqvist struck upon the idea of sewing electrical stimuli — similar to TENS (transcutaneous electrical nerve stimulation) electrodes — into garments that the patient can wear.

 

He turned to KTH researchers Johan Gawell and Jonas Wistrand at the Department of Machine Design at KTH. “They produced a prototype of the product, and today they are working full time on the development of Mollii,” Lundqvist says.

 

Designed with ordinary swimsuit material, the body suit has conductive elastic sewn into it, with electrodes located at the major muscles. Battery-powered light current is conducted via silver wires to 58 electrodes attached to the inside of the garment, which in turn stimulate as many as distinct 42 muscles, according to the patient’s needs.

Fitted with Electrodes
58 Battery-powered electrodes

 

Batteries are placed in a small control box fitted at the waistband. “The idea is that the clothes should be used for a few hours, three times a week, and the effect is expected to last for up to two days,” Lundqvist says. Users are advised engage in movement through training and stretching during the treatment. “To enhance the quality of life the patient may choose to use Mollii before it’s time to go to work, school or to a social event. That enables the body to function as well as possible when it is really needed,” he says.

 

The garment has been shown to be highly effective in patient examinations performed in collaboration with a PhD student Stockholm’s Karolinska Institute, Lundvist says. “One-hundred percent of the participants in the survey say they have experienced improvements in existing function or quality of life,” he says.

 

Stroke patients with paralysis on one side have been found to gain increased mobility in spastic limbs, in that they had improved gait and their arms and hands worked better after treatment. “As a bonus, the patients often sleep better, and their pharyngeal motor skills and speech improved after using Mollii,” Lundqvist says. “It can also help children with physical disabilities or motor difficulties in the feet, such as constantly walking on toes or with their feet at inward angles,” Lundqvist says.

 

Treating patients with movement difficulties and pain due to neurological damage can often require surgery, injections of botolinum toxin (neurotoxin) or strong medications. “These treatments mean high costs and side effects, while our clothes are simple and safe to use,” Lundqvist says. “You can reduce the number of hospital visits because the therapy can be performed at home. And when the mobility increases, there is less need for walkers or wheelchairs.”

 

Mollii is an approved CE marked medical device, but independent clinical tests haven’t yet been performed. But the company behind the treatment, Inerventions, has launched a scientific study of the clinical effectiveness of the garment, in partnership with Sweden’s Rehab Medical clinics. Lundqvist says the results should come next year.

 

Today, Mollii is available through the Swedish health care system as a personal tool prescribed by physical or occupational therapists. And the garment can also be purchased directly from Inerventions.

 

The price is about EUR 5,600 for two years guaranteed spasticity treatment. If the suit during that time becomes too small, the patient can switch to a new, tested garment at no additional cost. In Denmark, the garment is already subsidized with municipal funds for treatment of nerve damage, based on recommendations from a physiotherapist. Inerventions’ goal is to establish Mollii in Europe, the U.S. and Japan. The garment can in the future be used to help patients with chronic pain and people with Restless Legs Syndrome (RLS).

 

SOURCE:  KTH The Royal Institute of Technology (2013, October 14). Suited for treatment of brain damage. ScienceDaily. Retrieved November 20, 2013, from http://www.sciencedaily.com­ /releases/2013/10/131014093905.htm

Suited for Treatment of Brain Damage

 

Mobility Aids

Scooter, Cycle and in-home supports

 

Powered Scooter

Robyn posted the following about her scooter on the SPF’s chatline:

“I bought a scooter and I love it!!!  I was missing out on so much cause it was too hard to walk around with my cane.  I have 4 kids and a wonderful husband and I can’t miss out on stuff.  It has made my life awesome!!!  In fact my husband and I are in Vegas now and I am so thankful I have it.  I don’t have to miss out on anything!!!!!

Go Go Elite Scooter
Go Go Elite Scooter

 

I bought the Go Go Elite, I love it.  I can disassemble into 4 easy pieces and I can load and unload myself if I need to.  It has made life so much better.  I’m not afraid to go anywhere anymore, worrying about where to park, how far will I have to walk. The pressure is off!!  I don’t like that I have to use a scooter but that is reality and I don’t want to miss a thing!!  My kids are 21, 19,16, and 13 and they are thankful I have it and use it!!  We are on the go all the time with sports and life.  I now don’t worry when I travel away to baseball games because I have my scooter.

 

I know we need to walk and exercise which I do but life is so much better and easier with my scooter.  I’m over caring what people think, I need to do what is best for me and keeps me active and going with the rest of the world.  I rarely decline any opportunity now because I have no worries.  I hope this helps!”

 

Ed. Note: This model is available in Australia for $1750 + shipping.

 

GlideCycle

Glidecycle
Glidecycle

This cycle offers unweighted, low impact aerobic training. GlideCycle’s unweighting technology offers a new option in rehabilitation by allowing people to train without impact to sensitive bones and joints. Unweighted and low-impact training and rehabilitation are rapidly gaining momentum throughout the physical therapy, athletic rehabilitation, sports, baby boomer and general consumer segments. Available in Australia from this stockist.

 

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Lift System

Surehands is a lift system that allows even a quadraplegic to live in her own home. There is a line of motorized transfer devices to assist with lifting. Products include lifts appropriate for those with and without upper body strength. Here is a 10 minute video.

 

 

Superpole System

SuperPole
SuperPole

A very small and strong modular support system for people requiring a little assistance with standing, transferring or moving in bed. It consists of a vertical pole that fits by jackscrew expansion between a floor and ceiling.

 

Check it out on YouTube.

 

For more information go to these websites:

http://www.ilcnsw.asn.au/items/4867

http://www.acaciamedical.com.au/products/bedding/superpole-system.php

Our Unstable Genome

Everyone has genetic flaws

 

Most HSPers know that they have a genetic flaw but did you know that almost everyone has roughly 70 spontaneous, ‘single letter typos’ in their genetic coding?  Researchers are exploring genomic instability and with new technology seeing more than ever before.

 

How the quest to understand humanity’s shifting blueprint may better arm us to fight cancer and prevent birth defects

 

By Ian Demsky

 

A famous proverb holds that Persian carpets are perfectly imperfect, precisely imprecise. Intentional flaws in the weave symbolize a belief that true perfection is a quality reserved solely for the divine. Likewise, we humans are all imperfect copies of our ideal selves. For starters, within the billions of characters of DNA code we inherit from our parents, we each have roughly 70 spontaneous, single-letter typos.

 

“There are mistakes all over our DNA,” says Sally A. Camper, Ph.D., chair of the Department of Human Genetics at the University of Michigan Medical School. “The average person has dozens of tiny additions, deletions and changes in their genome ranging from those single base pairs up to several hundred kilobases — several hundred thousand base pairs.”

 

Read more …

Family planning and HSP

A second couple’s story

 

In the previous (Winter) edition of the website, we brought you the story of a couple in the early stages of undertaking an IVF program in the quest for a baby free of HSP.

 

Here, a 2nd couple, Sarah and John of New South Wales, tell the story of their IVF journey…

 

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When John and I decided it was time to start a family we were as excited as anyone would be. After a couple of months though, John and I admitted around the same time that he may be affected by whatever it was that affected his mum and brother – which was all a bit of a mystery to us at the time.  Trying to get pregnant naturally went on hold while we separately thought through possibilities and all the huge consequences both for John and for our future family.  We agreed we needed to make a deliberate decision about how to handle our family planning.

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After we both had time and space to think, we both knew that we didn’t want to bring a baby into the world to go through what John’s family had gone through, and what John may or may not go through in the future.  The uncertainty, the physical and emotional pain, and the difficulties with everyday life were all things we wouldn’t take the chance of passing on to our child knowingly. I knew that with every trip or stumble I would be wondering if it was the same condition. I would struggle to live with myself if it ended up being a severe case of HSP and I’d passed up the opportunity to remove that chance for my child.

I did some research and found our options to have our own child were prenatal testing or IVF (in vitro fertilisation). We were fortunate enough to have the means and the time to do IVF with PGD (pre-implantation genetic diagnosis). We chose to talk to Genea as there was some information on their website about testing for what looked like what we thought was the disorder. The really confusing part began here and this is why I wanted to share our story – if it makes this “adventure” easier for just one other couple, then it is worthwhile.  We thought we would be able to just rock up to the IVF clinic and be on our way to having a healthy baby.

Going to a GP to get a referral to a fertility specialist but trying to get them to understand that we didn’t think we had any fertility problems and wanted to find out about PGD was a nightmare. It seemed that the GP had never heard of PGD and didn’t understand what we wanted to do, and vaguely suggested it would be too hard/expensive/time consuming and why didn’t we just keep trying for a while – totally missing the point….  If we had understood more about HSP and knew what we needed to ask for I think it would have been easier, but it felt like it was the blind leading the blind.

Eventually we got the referral and then waited a couple of months to see the fertility specialist we had chosen, Dr Livingstone at Genea. On the same day we were to see a geneticist, Professor Graeme Morgan, who has previously contributed to the HSP website, who explained the inheritance pattern to us.  What we didn’t know before seeing Professor Morgan was that the disorder could be in a number of different genetic locations and that John would have to go through some testing which could take 6 months. We also didn’t know that we may not get a result, and then be faced with the decision about whether to go on and have children naturally, try testing overseas, or look at other options.

John’s family had previously been diagnosed based just on physical symptoms, so John had to go to a neurologist for a physical diagnosis and then be referred on to a clinical geneticist who would order the genetic testing.  John was diagnosed with HSP by a neurologist who didn’t offer much sympathy, compassion or any helpful advice on where to from there, but did write the referral for the genetic testing.

From the time we decided to try for a baby to the time John’s blood samples were taken, we were up to about 10 months and counting, and still not knowing if we would actually get a genetic result in the end.  7 months after that we finally got the phone call that the results were in and that there was a small never previously documented deletion found on the SPAST gene SPG4 – the most common location of HSP.  Though very sad that this meant a definite diagnosis for John, the relief was enormous, knowing we could move forward with the PGD.  I got back in touch with Dr Livingstone, and we were able to see him quite quickly and start the process for real this time….

We were warned there would be another period of waiting where Genea would create their own “work up” test where they took blood from John and me, combined it to create our joint DNA then tested it for HSP.  This took about another 12 weeks.  We were excited to start IVF finally, but very quickly it was absolutely overwhelming.  Lots of appointments leading up, waiting for my period to arrive, daily self administered injections, internal ultrasounds and blood tests every couple of days, the bloating & headaches, waiting for the phone calls with the results of how it was all going, the stress of whether it was going to work, both of us carrying on in our careers and John having to travel a lot for work in the middle of it all & leaving me home alone (though it was probably good for him with all the hormones flying around the house!).  It was absolutely exhausting both physically and emotionally and not as easy as we expected.

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The egg collection day finally arrived and we were so relieved to get heaps of eggs. It was not a fun procedure – I’ll leave it there! In the end we had 9 embryos develop far enough to be tested for HSP.  Waiting for the phone call to tell us how many were affected was probably the most nerve-racking part of the whole process because it could have meant we had done all of this for nothing – and it’s not cheap – though IVF is covered by Medicare, PGD is not at all (a whole other conversation that makes my blood boil). I think we were sitting at around $12,000 out of pocket, with all the other tests and expenses up to this point.  We were also thinking of our embryos as our future children while they were growing away in the lab, so knowing how they are progressing is totally surreal and emotional.

We had 4 HSP affected embryos and 5 unaffected embryos (little gold nuggets!) which was wonderful. We had a fresh transfer of one healthy embryo and I was getting positive home pregnancy tests, so we were excited & relaxed about the blood test. The news of my hCG hormone level wasn’t good though, and sadly that ended in an early miscarriage.

After the anticipation, it was a kind of devastation I won’t ever be able to describe in words.  No matter how early it is and what it is called clinically, a pregnancy loss is a pregnancy loss. We took a break for a couple of months & went on a couple of relaxing little holidays, which we both really needed. The medications affected me in ways I didn’t expect. I had a strange rash pop up all over my arms, legs, scalp and neck for a couple of months, my emotions were all over the place from the hormones & the loss, and my menstrual cycle really needed a couple of months to settle.  The subsequent frozen embryo transfer cycles was much easier to deal with physically as they worked in with my own natural cycle and even though the stress of waiting to find out if it had worked each time was still gruelling, there were no added hormones to make it worse!

It’s hard not to get obsessive about the process so it was really important to have support.  We kept the IVF to ourselves so we used a counsellor before & during the process, and it just happened that I ran into a good friend in the Genea waiting room one morning – it turned out she could use someone to talk to as well as they were also keeping their IVF to themselves.

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Finally, after the frozen embryo cycle, our precious baby arrived in May 2013. With 3 more embryos “in the freezer” in case we want to try for more children – though after all of this we agree we are happy just to start with one and see how we go!

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We appreciate and respect that PGD is not a process everyone would choose or agree with but we would be very happy to hear from anyone via the HSP Research Foundation who is considering PGD to share further.

We feel very humbled that we were able to donate our affected embryos for stem cell research and we truly hope this leads to better treatments and maybe even a cure for HSP.

We are deeply grateful to the compassionate, intelligent & committed people who have made it possible for us to have our own healthy baby – Dr Livingstone, Professor Morgan, the embryologists & nurses.  Sincere thanks also to the HSP Research Foundation and all who maintain & contribute to this website which helped us understand the basics of what we were dealing with.

Achieving and maintaining good mental health

Part 4 of a series – Taking in the Good

 

Neuroplasticity has been getting steadily increasing coverage in the media over the past couple of years related to a range of different aspects of the brain and its function.

 

Neuroplasticity refers to changes in the brain that are due to changes in behaviour, environment and neural processes, as well as changes resulting from bodily injury. Neuroplasticity has replaced the formerly-held position that the brain is a physiologically static organ, and explores how – and in which ways – the brain changes throughout life.

Neuroplasticity occurs on a variety of levels, ranging from cellular changes due to learning, to large-scale changes involved in cortical remapping in response to injury. The role of neuroplasticity is now widely recognized in healthy development, learning, memory, and recovery from brain damage.

Rick Hanson Ph.D.
Rick Hanson Ph.D.

Possibly due to the need for keen survival instincts in the earliest days of human beings, our brains are like Velcro for negative experiences and like Teflon for positive ones. Due to what is now known about Neuroplasticity, things don’t need to be like that.

 

Rick Hanson, a neuropsychologist and author, has been studying Neuroplasticity and has come up with a technique to train the brain to take in positive experiences with the potential for profound and welcome changes in our moods, outlook on life, happiness and overall satisfaction.

Here is his article – TakingintheGood – which takes you step-by-step through the technique that if practised and mastered until it becomes a habit, has the potential to be pleasantly life-changing.

 

Stretching for HSP

Exercises you can do at home

 

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This Foundation has been advocating the benefits of physiotherapy for HSP for some years now, specifically neurological physiotherapy where the physio has the specialist training in understanding and working with neurological conditions.

 

In this article, Sarah Daniel, a UK neurological physiotherapist, talks about the importance of stretches in managing the symptoms of HSP.

 

She details a program of 8 exercises in plain English with helpful pictures that many HSPers could benefit from.

 

She makes the point that not all of these exercises are right for everyone, so don’t do any that don’t feel right or that cause pain. Find a physiotherapist near you to learn more.

 

 

 

Effectiveness of Baclofen for HSP

Research shows mixed results

 

A review of recent research on the effectiveness of intrathecal baclofen for HSP shows mixed results. Some of the findings include:

 

intrathecal baclofen pump
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* some patients have good functional improvement.

 

  * satisfaction was high for patients who received implantation early instead of waiting for the natural course of the disease.

 

  * a possible compromise between decreased spasticity and muscular weakness.

 

  * the baclofen dose needed for hereditary spastic paraplegia (HSP) is significantly smaller than that in other diseases.

 

  * baclofen causes some clinically significant adverse reactions.

 

 

Research Abstracts

http://www.ncbi.nlm.nih.gov/pubmed/17368612

Satisfaction was high for patients who received implantation early instead of waiting for the natural course of the disease. Some patients with hereditary spastic paraparesis have good functional improvement with chronic intrathecal delivery of baclofen if walking is still possible. Despite the natural history of the disease, functional results are stable during the first 5 years of treatment. The data indicate a possible compromise between decreased spasticity and muscular strengthening with the treatment.

 

http://www.ncbi.nlm.nih.gov/pubmed/15785266

Intrathecal baclofen therapy has been used for several years, despite the fact that long-term gait modifications in ambulatory patients have not been thoroughly investigated. A 31-yr-old male patient affected by hereditary spastic paraparesis was evaluated clinically and by gait analysis. Evaluations were made before and at 6, 12, 16, and 24 mos after implantation. The patient showed a clear improvement in self-selected speed, step and stride length, knee and ankle kinematics, and ankle kinetics. Moreover, the response observed on self-selected speed is consistent with the intrathecal baclofen dose administered. To our knowledge, this is the first report of a long-term instrumental gait analysis assessment of a patient receiving intrathecal baclofen. Gait analysis could be a reliable and feasible assessment tool to evaluate ambulatory patients receiving intrathecal baclofen therapy over time and to help clinicians in determining exact dose requirements.

 

http://www.ncbi.nlm.nih.gov/pubmed/21447848

Compared with pretreatment values, there was an improvement in clinical efficacy, but the baclofen dose needed for hereditary spastic paraplegia (HSP) was significantly smaller than that in other diseases. The result shows the importance of knowledge of the adequate baclofen dose in each disease in that baclofen causes some clinically significant adverse reactions.

 

Botox fails to make a difference

Treating spasticity in HSP

 

Botox injection
Botox injection

A new study from Brazil that has yet to be published shows that Botox failed to make much of a difference to outcomes when used to treat spasticity in adults with HSP.

 

This is in contrast to Israeli research from 2010 were Botox was used to treat spasticity in children with HSP and found to have beneficial effects for a period of up to 6 months after injection.

The Brazilian study was presented at a recent conference in Sydney with the results of treatment for 11 patients with pure hereditary spastic paraplegia. The average age of the patients was 41.5 and they had been diagnosed with the disorder for 23.5 years on average. All were without significant lower limb weakness but all had significant spasticity.

One of the reviewers of the conference presentation said:”The problem with this disease is that there are two major aspects, spasticity which can be improved with botulinum toxin and generalized lower body weakness, which is not helped by the toxin injections. Hence the overall improvement is minimal.”

Read the full report.

 

New walking aid device

Trials underway in the US

 

Kickstart is a new device designed with a pulley system to use and support the wearer’s own energy. It works without expensive electronics.

 

Kickstart Walking System

 

The Kickstart Walking System is an orthosis intended for individuals with lower extremity weakness – particularly in the hip flexors and ankle plantarflexors – and poor endurance, secondary to neurological injury or disease. This device is new in the US with trials being offered in various cities in the US and Canada, but is not yet available in Australia.

Who can benefit from the Kickstart Walking System?

It’s designed for those who have difficulty walking independently, especially if you experience any of the following:

  • Difficulty lifting your knee
  • Catching your toes often when taking a step, even while wearing a brace for foot drop
  • Lack of endurance – fatigue after walking short distances
  • Difficulty with coordination, balance or stability
  • Circumduction – tendency to swing your leg out to your side (instead of straight forward) in order to take a step
  • Step-to Gait – stepping forward with one foot and bringing the other to meet it

Ideal candidates for Kickstart are those who have:

  • The ability to initiate at least one step (with use of walker or crutches if needed)
  • Good core strength (back/abdominals)
  • The ability to stand up straight for two minutes (with only slight assistance from a walker or crutches for balance)

Other requirements include:

  • A strong motivation to walk
  • A strong, motivated support structure from family and caregivers

Contraindications include:

  • Severe knee flexion contractures
  • Severe quadriceps weakness
  • Cognitive impairments.