FDA approves drug for MS

Helps with walking

By Georgiann Caruso, CNN Medical News

January 22, 2010 8:03 p.m. EST

http://www.cnn.com/2010/HEALTH/01/22/fda.approval.ms.drug/index.html

The second-to-last time EJ Levy was at Disney World, she used a scooter to navigate the enormous park. Her legs were weak and she suffered from foot drop caused by multiple sclerosis. That was 4½ years ago. On her most recent trip, a few months ago, Levy walked the entire time, thanks in part to a drug approved by the FDA on Friday.

The FDA says the drug, Ampyra (generic name dalfampridine, formerly known as fampridine), is the first MS therapy that is taken orally and the first of its kind to receive FDA approval. It is designed help people with any type of MS improve their walking speed.

The prime of her life

In 2002, Levy was in her 30s and an active hiker and skier with a job on Wall Street and later in San Francisco, California. But her life took a turn when she started stumbling, falling down and dragging her right leg. Her doctor’s diagnosis? Secondary Progressive MS, a less common form of MS and, as the name implies, one that usually plagues people with the disease after its initial course.

The National Multiple Sclerosis Society defines MS as a “chronic, often disabling disease that attacks the central nervous system, which is made up of the brain, spinal cord and optic nerves.” Symptoms of MS include extreme fatigue, difficulty walking, problems with memory and heat sensitivity.

The most common form of MS is relapsing-remitting, in which people have acute attacks followed by periods of remission. In secondary progressive, however, the disease worsens steadily and there are no acute flare-ups. People with relapsing-remitting may later develop secondary progressive MS.

Levy says she lived in the same building as her parents at one point because she needed them to help take care of her. She says she mostly stayed at home, and when she did venture out she usually walked only a block or two with a cane. She relied on a wheelchair for longer distances.

She says she realized she would eventually be unable to walk. “I was scared of being in a wheelchair. I was scared of getting worse and worse. I had traveled around the world before I got sick, I was super independent,” Levy says.

Trying things out

After exhausting the usual MS treatments — and developing intolerable side effects — Levy also became frustrated that the treatments were primarily designed for people with the more common course of the disease, and not the secondary progressive course.

Levy’s neurologist then discussed the possibility of trying 4-aminopyradine — a version of the same drug the FDA approved Friday that was available only through compounding pharmacies. Just three days after taking the drug, Levy was able to walk unassisted.

“I never thought I could put my cane away for good,” she said.

Now she hopes the same drug that helped her will be able to help other people. She addressed an FDA advisory panel late last year and recommended approval of it.

“It’s about quality of life,” Levy says.

How it works

Two phase III clinical trials of Ampyra showed 35 and 43 percent of patients experienced, on average, a consistent improvement in their walking speed, increasing it by about 25 percent.

According to the National Multiple Sclerosis Society, even a modest improvement in walking ability could mean that thousands of people could benefit from the drug.

Dr. John Richert, executive vice president of Research & Clinical Programs at the National Multiple Sclerosis Society, says the drug can be used by most people with MS. However, the drug is not for people with a history of seizures or people who have moderate to severe kidney disease.

Richert says approval means patients can now skip the compounding pharmacy and get a consistent, exact dosage in a guaranteed time-released formula, which would lower the risk of getting a toxic dose instead of a therapeutic dose.

“It’s likely that further study and clinical practice may help to determine the extent to which the drug may impact other functions, and may also provide hints as to which patients are most likely to respond positively to the therapy,” Richert said.

He added that the drug would not help nerves that have been destroyed and emphasized that people should try the drug to see if it works for them.

Ampyra may not work for everyone. “Like any medication, people will have different responses, so they should talk to their doctors about whether it’s appropriate to start therapy on Ampyra,” according to a spokesperson from Acorda, Ampyra’s manufacturer.

Moving with MS

Today, Levy is doing well. She established the not-for-profit organization MS Hope for a Cure, and in three years the organization has raised $1.8 million for research and programs to help people living with MS.

She has also completed several five-mile hikes. But she says she is always conscious of where she steps. She says she occasionally has some weakness in her leg, but that she is thankful for every day: “I don’t ever take jumping out of bed for granted.”

Beating the Blues

Services

Support is available for anyone who may be depressed or for a family member or carer who may be concerned about someone by calling:

beyondblue 1300 22 46 36 – information & referral service; not counselling; information on depression in languages other than English can be obtained from the website. http://www.beyondblue.org.au/index.aspx?

SANE Helpline 1800 18 7263 – information, referral and advice; not counselling.

Lifeline 131 114 – 24hr phone counselling service.

Salvo Care Line 1 300 363 622 – 24hr phone counselling service and referral.

All these services are completely confidential.

.

Websites

http://www.blackdoginstitute.org.au/

http://www.beyondblue.org.au/index.aspx?

http://moodgym.anu.edu.au a free, interactive, online self-help guide

 

Reading

Beating the Blues: A Self-Help Approach to Overcoming Depression by Susan Tanner and Jillian Ball

Publisher: Doubleday
ISBN-13: 9780646366227
available at the Australian Online Bookshop

 

Exercise Program

Jill from Melbourne shares her Exercise Program …

This is the exercise regimen that works for me. I’ve included a few tips, some I have picked up from experts and some are my own ideas. I have no professional health expertise, so check any changes you may want to make to your program with your health professional.

One of the hardest things about the illness is understanding it and learning to manage it. It seems that only parts of the information you need about HSP can be provided by doctors and therapists, the rest you must work out yourself.

I am new to ‘serious’ exercise. It has been interesting to learn as I go, while also trying to understand what HSP means. In my case there was no clear onset but diagnosis a couple of years ago helped explain a lifetime of leg and hip pains. It seems that I have always had HSP and it has been slowly creeping up until it was clearly identifiable. A slight limp became a hobble, falling over was common, spasticity was increasing and fatigue was persistent.

Neurologists and therapists have helped me enormously in understanding HSP, but mostly living with it has been how ‘I’ve got my head around it’.

I’m stronger now than I’ve been for many years and it’s been quite a while since I last fell over as I am better able to ‘save’ myself when I do trip. My stamina is also greater and now I can enjoy doing more each day.

Hope there is something useful here for my fellow HSPers.

Jill


A few tips:

If you haven’t already done so link in with a good physiotherapy rehabilitation unit (usually in public hospitals).

Ask the physios lots of questions, such as, why this exercise, which muscles is it working, how can I tell which muscles, etc.

Form is all-important in all of your movements. Try to understand this information and so be able to recognize when you are doing each exercise correctly.

Do your exercises every day, they’re not a drag, they are fantastic and will help you with your pain, and may also significantly increase your mobility.

Play some music that you particularly enjoy while you do your ‘routine’.

Avoid ‘parasitic’ movements like grimacing and jaw clenching, they don’t help at all. But do occasionally try smiling, and think about how doing what you are doing is helping you to feel good. (If not immediately then in the longer run.)

Weights: do roughly half of what you think you can do. If you go to a gym (more about that later) and are new to gyms, then the advice given to me by a power-lifting champion (my brother) is: do roughly half of what you think you can do, e.g. if a 2kg weight feels fine to lift, put it back and choose 1kg instead. Do 5kgs instead of 10. This applies to the machines with fixed weights and resistance as well as dumb bells. Always do less than you think you can do, and always do less than you think you should do, and especially do less than others tell you to do.

It is the movement which is important and that you do it regularly. If you don’t enjoy it and if you feel too tired afterward then you won’t do it regularly and in the right frame of mind. You can increase the weights and the repetitions as you go, give yourself challenges and so on, but not too soon. Most gyms have well-trained staff who will help you to design a program to suit and to show you how the various machines work – this is excellent but again follow the advice of an expert and do much less than they recommend at least to begin with. It will help to see how you feel after each gym visit and over a course of visits. If you feel more tired than usual then wind it back (weights, repetitions, speed, etc) a bit for a while. You can increase it later. I never go to the gym two days in a row.

Rest between each set – as long as you feel like, more than a minute, maybe three or more. Learn to listen to your body, after all this is about helping your body and not someone else’s rules, whoever they are.

At first don’t worry about reaching heart rates, etc. Too much detail can make it seem all too hard. Accomplish one thing at a time, and let it be a very small thing. You are only doing this for yourself, there is no exam afterwards and nothing to prove to anyone. Check with your doctor before you start any new exercise program.

The exercise that you get from doing the things you enjoy and have to do (sometimes this is called ‘incidental exercise’), whatever you are able to do, is valuable but do add the purposeful, therapeutic exercises that your physios have given you.

Exercise in water was lovely to do and extremely helpful for my legs but I had to abandon it because the pool chemicals were making me sick – perhaps you can enjoy the benefits without this problem.

Doing some exercise every day seems to be the key. It seems to be about warming, stretching and using your muscles – and the mind likes it too. It’s a great feeling to be actively engaged in improving and helping your own well-being, and exercise itself makes you feel happy.

Have a look into Feldenkrais. There are practitioners listed in phone and web directories. This can be expensive but many of the community exercise groups include Feldenkrais at a low cost. For more information try www.feldenkrais.org.au and www.feldenkrais.com.au

…And if you feel like a day off from all this, to do something else, or to have a complete rest, then do it. Sometimes the body just wants to rest. Get up the next day though and start again, as long as you are otherwise well it is always worth starting again.


My exercise plan:

· Home exercises as prescribed by physios. I do these everyday and they include exercises to strengthen abductor and gluteous muscles (outside of hip and bottom) and knee, with stretches for a variety of muscle groups. It takes up to an hour to do them all.

· Gym – three times a week. Cross-trainer to warm up followed by fixed weight, dumb-bell, body-weight, balance exercises, upper body strengthening exercises, with cool down on the rowing machine. The physio exercises are included in the work-out and I finish off with stretches. Go home have something to eat and rest, even a snooze.

· Horse riding once a week. This is not for everyone but if you like horses then give Riding for the Disabled a call – they have branches everywhere. The benefits of sitting astride a horse for strength, stretch, balance, even spasticity, and fun, have proved to be immeasurable. Grooming, being with the horses and other riders, and volunteering to help in other ways makes for a great day of exercise and enjoyment.

· Feldenkrais. One and often two classes each week. This is not exercise as such but a learning experience for you and your body. What I learn from these classes is helping me to improve the way I move. The benefits so far have been profound with more to come …

· Pilates for core strength occasionally.

Function Study on HSPers

Walking speed for HSPers declines with disease duration, age, level of spasticity (higher) and Range of Motion (lower).

OBJECTIVES: To characterize the spasticity and range of motion (ROM) in patients with hereditary spastic paraplegia (HSP) and to correlate these parameters with walking speed.

DESIGN: An observational population-based cohort study. SETTING: Patient data were acquired from a population-based epidemiologic study performed earlier in Estonia.

PARTICIPANTS: Persons (N=46) (mean age, 50.1y) with clinically confirmed HSP diagnosis (mean duration, 20.9y) participated in the study.

INTERVENTIONS: Active and passive ROMs were measured with a plastic 360 degrees goniometer. Spasticity was evaluated by using the modified Ashworth scale (MAS). The time it took a patient to walk 10m was recorded.

MAIN OUTCOME MEASURES: Measurements included testing of active and passive ROM as a marker for mobility, the MAS for spasticity, and time to complete a 10-m walk.

RESULTS: A higher degree of spasticity in hip muscles was associated with lower values of active ROM and slower walking. Walking speed was negatively correlated to disease duration and participant age.

CONCLUSIONS: The present study provides analysis of the contributions of spasticity and ROM to walking speed in HSP, both factors negatively influence gait in persons with HSP.

SOURCE: Arch Phys Med Rehabil. 2009 Nov;90(11):1887-90.

Functional assessment of lower extremities in hereditary spastic paraplegia.

Braschinsky M, Parts K, Maamägi H, Gross-Paju K, Haldre S.

Tartu University, Department of Neurology, 8 L. Puusepp St, Tartu, 51014, Estonia. mark.braschinsky@kliinikum.ee

Jason Jones – HSP sportsman

HSP community member and active sports enthusiast Jason Jones of NSW wants to encourage other community members to think about giving competitive sports a go.

Jason tried out for soccer for the Paralympics and currently has his focus firmly on road cycling. He sends along the following details for anyone interested to check it out.

The Australian Paralympic Committee is conducting a talent search. Read about it on their website www.paralympic.org.au or contact them by phone on (02) 9704 0500 or e-mail: auspara@paralympic.org.au

Others may want to check out Wheelchair Sports, which are State-based organisations. Just Google ‘Wheelchair Sports + your State’ to find information relevant to you.

Congratulations and well done Jason! We all wish you every success.


Dancer with spasticity

Learning His Body, Learning to Dance

http://www.nytimes.com/2009/11/25/arts/dance/25palsy.html?_r=1&scp=1&sq=Learning%20His%20Body&st=cse

By NEIL GENZLINGER

Published: November 24, 2009

The New York Times

Gregg Mozgala, a 31-year-old actor with cerebral palsy, had 12 years of physical therapy while he was growing up. But in the last eight months, a determined choreographer with an unconventional résumé has done what all those therapists could not: She has dramatically changed the way Mr. Mozgala walks.

Photo: Gregg Mozgala rehearsing with Emily Pope-Blackman.

She said it had been a slow process to find out how much force they could place on his body. In the process, she has changed his view of himself and of his possibilities.

Mr. Mozgala and the choreographer, Tamar Rogoff, have been working since last winter on a dance piece called “Diagnosis of a Faun.” It is to have its premiere on Dec. 3 at La MaMa Annex in the East Village, but the more important work of art may be what Ms. Rogoff has done to transform Mr. Mozgala’s body.

“I have felt things that I felt were completely closed off to me for the last 30 years,” he said. “The amount of sensation that comes through the work has been totally unexpected and is really quite wonderful.”

Cerebral palsy, a neurological disorder in which the brain does not send the proper signals to the muscles, affects gait and other movements. Those with severe cases use wheelchairs.

Mr. Mozgala’s condition is less severe but disruptive enough to have caused him to walk for most of his life like “a human velociraptor,” as he put it: up on his toes, lower extremities turned in, seesawing from side to side to maintain balance. “My knees were going in, my hips were totally rotated inward,” Mr. Mozgala said. “Gravity was just taking me down. So my upper body — arms and chest — overcompensated, curling back and up.”

That is how he looked when Ms. Rogoff saw him in March 2008 playing the male lead in a production of “Romeo and Juliet” by Theater Breaking Through Barriers, a group whose shows mix actors with disabilities and those without. Ms. Rogoff has often worked outside normal dance parameters — with prison inmates, for instance — and knew immediately that she wanted to try to create a piece for Mr. Mozgala. “I didn’t know what I was going to do for him,” she said, “but I just knew he was inspiring to me.”

Originally, she envisioned a simple study, maybe 10 minutes long. Mr. Mozgala’s expectations when he agreed to the project were equally narrow: he said that he thought that she would either merely create a dance that made use of the physical abilities he already had or, after seeing his limitations, tell him, “Thanks but no thanks.”

Once they began working together, though, Ms. Rogoff realized that a broader approach was needed. “Every time he tried to move in a way that wasn’t specific to his habitual pattern, he would fall down or just not know how to address it,” she said, “because he had a certain amount of patterning linked to his C.P., and I was asking him to step out of these patterns. I realized I couldn’t ask him to do that unless I supported it with a lot of body knowledge.”

She introduced Mr. Mozgala to a tension-releasing shaking technique, and it was immediately revelatory. “My body just really took to it,” Mr. Mozgala said. “I did that for about 20 or 30 minutes, and when I stood up, I was walking completely differently. My feet were flat on the ground.”

They knew they were onto something. They began doing intensive one-on-one sessions they call body work, Ms. Rogoff using her knowledge of the body and dance-training techniques to help Mr. Mozgala “find” individual bones, muscles and tendons that he had had no command of before.

They started at the top and worked down — sternum, sacrum, knees — with Mr. Mozgala’s body and brain opening paths of communication that had not existed. “There’s a lot of howling, screaming, crying, sweating,” Ms. Rogoff said. But “we often have these huge eureka moments.”

The other day, for instance, it was brain, meet lower-leg tendon.

“I said today, ‘I can feel my Achilles,’ ” Mr. Mozgala said. “You have to realize, I have never felt my Achilles before.”

Dr. Stephen A. Paget, chief of rheumatology at the Hospital for Special Surgery in Manhattan, who has been acting as a sort of sounding board for Ms. Rogoff during the project, said the changes being wrought in Mr. Mozgala support a relatively new way of thinking about neurological impairments. “In the past, people thought that a neurological deficit was fixed and immutable,” Dr. Paget said. “Now there’s this whole concept of neuroplasticity: the neurological system has this ability to change itself and constantly grow.”

Apparently Mr. Mozgala’s has done just that. Before, his gait was extreme enough that it would draw stares on the street. (“The lurch,” he and Ms. Rogoff have come to call it.) Now, when he is fully concentrating, a passer-by might have to look twice to realize he has a disability at all.

Unlike his earlier physical therapists, he said, Ms. Rogoff has given him knowledge of his body and specific instructions that he can employ while going about his everyday life: “Sternum down, tailbone up,” and so on.

“I have the key now,” he said. “Before, I was always being manipulated by someone else.”

Ms. Rogoff, whose father was a doctor, said that she knew little about cerebral palsy when she and Mr. Mozgala began, and that she had made a point of not learning too much. “That way I didn’t have any ideas about what he could and couldn’t do,” she said.

The physical changes Mr. Mozgala has experienced have had ramifications beyond the gym and the studio. Since high school, he has had a comical routine he would employ when he fell (“falling with style,” he called it) as a defense mechanism, to get people on his side; that is now gone.

So are the swings of self-image in which he would go from not caring if people were staring at him to feeling like “John Merrick on Fleet Street,” as he put it.

As Mr. Mozgala changed, so did Ms. Rogoff’s concept for the dance. The 10-minute study she had envisioned is now a work of more than an hour, with a cast of four. The piece has antecedents in “Afternoon of a Faun,” the Nijinsky ballet. Mr. Mozgala plays a 5,000-year-old Faun who turns up in a modern-day hospital as the work explores the intersection of science and art.

Emily Pope-Blackman, an experienced dancer who has a very physical, sensuous duet with Mr. Mozgala in the piece, had the task of helping him translate the progress he was making in the body-work sessions onto the dance floor. It was, she said, a slow process of “finding out between the two of us how much force, as he got stronger, we could risk: how hard could I pull him toward me; how much could he push me over without falling over himself.”

The piece, which also features Lucie Baker and Dr. Don Kollisch (a real-life family physician), is financed with a grant from VSA Arts, a nonprofit group that supports arts by people with disabilities. After its New York run, it will be seen at the VSA International Arts Festival in June at the Kennedy Center in Washington.

Ms. Rogoff calls Mr. Mozgala “the best student I’ve ever had.” Yet both of them are emphatic about what they have not achieved. “This isn’t a cure,” Mr. Mozgala said. “I’m always going to have cerebral palsy.”

But now he doesn’t feel so enslaved by it. “Everybody told me there was nothing I could do,” he said. “That’s just what you hear, from the time you’re 5 to adulthood. Tamar gave me an option.”

Whether the methods they have used can translate to others remains to be seen. But Dr. Paget said their progress held a message for anyone with a neurological impairment. “It’s not over,” he said. “There’s always a chance to change. You should not — you dare not — give up.”

Bungee jumping

Check out this video from You Tube. You won’t believe your eyes.

If you think you can … !

http://www.youtube.com/watch?v=OtAJFIOz8xs

Fitness – a key for HSPers (part 2)

Fitness – a key to maintaining mobility for HSPers

by Lea McQuade, Physiotherapist*

Part 2 of a 2 part series(read Part 1)


CONTENTS

Walking

Running

Treadmill walking

Cycling

Static/exercise cycling

Dancing

Swimming

Aquatic exercise

Pilates & Yoga

Boxing style fitness sessions

Aerobic Classes / Circuit training / Boot camps

Gym and weights based resistance programs

Rowing machines

Kayaking

Other Forms of Exercise

Stretching

3 Final Tips


Let’s talk about different forms of exercise.While variety in exercise is good, every form of exercise does not suit everyone equally. HSPers vary in age and level of symptoms, and what suits one person will not be practical for another. Always seek individual advice from a qualified professional such as a physiotherapist.

Walking

The most common form of regular exercise, requiring no special equipment is adaptable to almost any situation. Many HSPers describe walking as something they have to pay attention to, step by step, to avoid tripping. For those HSPers who can manage walking, it is a good mainstay of fitness.

Walking Technique

Here are 3 things to help make it easier for you to transfer the weight from one leg to the other with more control.

1. “Walk Tall’ as the old song says, and find your natural rhythm. Think of lifting your body weight up off the legs, to make it easier for them to move under you. Get more ‘up and over’ your feet with your body weight.

2. Keep your stance narrower, feet closer together – less shifting side-to-side as you walk.

3. Keep step length fairly small, rather than long striding.

The stronger the large muscles are in the centre of the body (core stability) the more ‘held together’ the trunk is, and the more easily the legs can be used to propel efficiently. This is the focus of much Physiotherapy research in recent years.The popular Pilates approach focuses on core stability and is discussed in a section later on in this article.

Many HSPers lock their knees during the ‘Stance’ phase of walking. To counter-act this, concentrate on getting the front of the hip/groin area forwards (bottom under) as your body advances over your planted foot, rather than let the hip slump and bend which pushes your bottom behind you and makes your lower back over-arch inwards.

Visualise an elastic strap making a connection between the backs of both legs at the very top, and between the tops of the legs and the underside of your buttocks. Try not to completely release that connection from the moment you prepare to stand up and whilst walking, etc. When walking, consider a leg moving forward as a preparation stage, stretching the elastic bands so that the leg recoils back underneath you with some momentum, pulling the ground under your foot with it – so driving your body upwards and forwards over that ground.

When going up slopes or steps – tuck your bottom under you, point your chest and groin up to the top & consider your legs making a cycling motion underneath to drive you forwards and up the whole rise. As you raise each leg, consider how you are stretching the elastic band that will drive the leg back down again. It is normal and efficient to use rising up and down on the toes for stairs.

Coming down, again tuck your bottom under and consider your trunk and pelvis moving upwards, whilst your legs control the bending for the descent. This keeps your weight back over the heel of the rear foot until the toes of the leg moving forward find the lower ground without you leaning back. Again use whatever your ankles can do to assist you.

Shoes & Aids

Well fitting shoes will help the efficiency of rolling from heel to toe along the length of the foot and increase confidence on more uneven ground. There are some shoes on the market specifically designed to assist with the front to back roll of the foot, which may assist with propulsion. You could seek advice from a podiatrist if these are suitable for you.

The greatest overall health benefits are gained by moving at a moderate to faster tempo. However, for many HSPers, walking at any pace can be quite a workout. Strive to push yourself along within your comfortable tolerance, and as spasticity allows. Walking at a slower speed generally requires much more control and effort.

If you feel an aid is sensible for balance or just to assist with the ‘push along’ technique then try ‘walking poles’ (also called Nordic Poles) such as a ‘Happy Hiker’. Walking sticks on the other hand encourage leaning down on them, whereas the longer poles are gripped vertically. Try one or two walking poles used like ski poles – helping to drive you forward over the terrain. The correct length (they are telescopic) is with the forearm at 90° when your arms are by your sides.

To use them effectively – keep your shoulders wide, your lower back tucked in, your elbows fairly close into your sides and the poles at an angle slightly backwards from the vertical. It’s a short ‘push-back, press down’ action as part of normal arm swing. They are available from $50 each (& upwards) at outdoor retailers, and adjust in length to suit each person. Several colleagues and I have observed that when someone has already developed an altered walking pattern, focusing on how they use their arms as described above, may allow the body to find a different and improved pattern for the affected legs.

Running

HSPers who are experienced runners can have a highly automated pattern of activity that can often be maintained well despite greater difficulties with walking. Fast manoeuvring can often present the greatest problem for game playing. However, if you are a ‘jogger’ – beware! because at slower speeds patterns of muscle activity vary greatly, postural stability is readily lost and the additional pounding on hips and knees can quickly result in joint problems and worsen spasm. Running training for people with Neurological conditions is a new area, with few experienced professionals to guide you, as yet.

Treadmill walking

A substitute for normal walking especially in inclement weather – HOWEVER – the rolling belt action detracts from the normal drive back of the legs under the body which we use to propel us forward, meaning there is less use of some of the most important muscles of walking. Also because of the ‘ground’ movement, many people feel that they need to hold on to the machine rail, and often lean back to counterbalance, and so hang on by pulling themselves forward with their arms rather than propelling with their legs. This alteration in alignment often results in more ‘pick up’ leg action, especially if there is concern of catching the toes on the belt. This is likely to exaggerate abnormal muscle activity and is to be avoided.

From my observations I would suggest that using a treadmill is NOT a recommended option for HSPers, UNLESS they are familiar and comfortable walking without holding on and can push back with the belt at a reasonable pace. If one side of the body is considerably less affected than the other, then the treadmill could be a reasonable option, with the above considerations.

Cycling

Like many of the other activities, this can be difficult if there is already established toes-down spasticity. The focus should be on keeping the heel down, driving down underneath you from the top of the stroke, so lengthening the back of the leg.

In cycling one uses the inner thigh muscles (hip adductors) often, especially when pushing up hills or rising out of the saddle – in both cases the focus should be on the rear adductors underneath, squeezing you up off the saddle or shifting weight from pedal to pedal. Even without toe clips or shoe cleats there can be a tendency to ‘pull up’ especially at the top of the pedal rise – this is best avoided as it can affect the hip muscles needed for walking and climbing stairs.

If balance, or getting on and off become an issue for a seasoned cyclist, attaching ‘stabilising wheels’ or converting to a tricycle could be a good option to continue a favourite activity. If terrain is a problem, the recent advent of powered bicycles, where assistance from a small motor can be added when needed to normal leg power, may make cycling more feasible for an HSPer.

Static/exercise cycling

This can be an excellent form of exercise for HSPers. Whether on the road or in the gym, Bike Seats are frequently too wide and too far back from the pedals for efficient ‘press back and down’, especially for taller people, so experiment to find your best position. In all cases, advice on best seat height and position is paramount. The pedal crank should be close to vertically underneath the point of the hip and the knee still slightly bent when the heel is pushed well down at the bottom of the pedal stroke.

Aim to keep the pelvis level and stationary on the saddle, separating the leg action from the body. If you prefer to bend forward and rest on the handlebars, try to keep the chest pushed out forwards and the shoulder blades stabilised down your back, with elbows close together to support the neck.

I would advise against the recumbent versions of exercise bikes, as it appears much harder for many to use the rear push ‘down & back’ muscles in this position.

Dancing

Ballet, classical, salsa, line dancing – all dance is excellent for balance, body alignment, lightness on feet, fluidity and co-ordination with superb CV benefits.

And FUN!

Start early. Close partnered dancing has a significant effect on enhancing excellent posture. Dance around the house.

(For every up on the toes, ensure smooth lengthening of the calves to lower heels again, not fall backwards down onto heels or stick bottom out)

Not so sure about ‘break dancing’!!!

Swimming

Swimming is often advocated for those with ‘joint wear’ issues especially osteoarthritis of knees, feet or hips, because there is minimal joint loading with movement in swimming. It also has the benefit of whole body use, involving many muscles at once, which is why it places a workload on the heart and breathing. You need to be fairly efficient to get reasonable cardiovascular benefits from swimming. Many people fatigue and stop before that point, mostly because of breathing inefficiency. A snorkel could help that initially. I recommend inexperienced swimmers get good coaching for efficient technique.

Many HSPers report swimming or other aquatic activity as their exercise of choice and with which they have the least problems. I would imagine that crawl/freestyle’s controlled rotatory action of the body around the spine would limit spastic effects whereas breaststroke kick may be more difficult in someone with established spasm. Core stability of the trunk is essential to allow the limbs freedom to work, so work on that ‘saggy’ belly if you currently have one!

Aquatic Exercise

Many forms of aquatic exercise are potentially excellent as a long-term, enjoyable and central part of an HSPer’s physical activity and fitness regime. However there are pros and cons of different forms of aquatic exercise for someone with HSP. Buoyancy makes some exercises easier; the resistance of the water makes others harder.

Walking faster in waist depth water requires push back against the ‘drag’ resistance and could be a beneficial activity; however, walking slowly in waist depth water has little benefit and could worsen spasm.

Balancing sitting on a ‘pool noodle’ (a flexible, cylindrical polythene float device) under the buttocks or between the legs can be an effective core stability exercise. Similarly ‘water running’ in deeper water with a float waist belt could also have value, while working on standing in deeper water tends to lift people up onto their toes and makes lengthening the calves even harder than normal. A lot of aqua-aerobic classes encourage people to ‘pick the knees up’ or do half-squats to involve the arms under the water a lot of the time – I would not recommend this for HSPers as again, it can affect the hip muscles needed for walking and climbing stairs.

For HSPers, or anyone with a neurological condition, I suggest personal, professional advice by a Physiotherapist experienced in Aquatic Therapy about how to do your preferred water exercise safely and effectively.

Pilates & Yoga

Both of these activities focus on core body stability, control, strength and balance, through smooth and specific exercises designed to keep muscles long and lean. They focus on achieving stability of the pelvis, trunk and shoulder girdles in preparation for separate movements of the limbs. This is very similar in approach to the current thinking of Physiotherapists.

Both are excellent activities for HSPers provided they are done well. HSPers would be advised to pick small group sessions where the instructor can analyse your abilities and difficulties throughout the class to ensure you are achieving the desired techniques and getting results. Some people are put off because many exercises occur on a mat on the floor, which is difficult for them, but this problem can generally be overcome in a small group through discussions with the instructor. Pilates can also be performed on specific machines, but this usually requires one-on-one or one-on-two instruction.

There are many types of Yoga approaches, and anyone interested should discuss the appropriateness or goals with the tutor. A class where the flow of exercises is maintained can also have some cardiovascular benefits.

Boxing style fitness sessions

Increasingly popular with many age groups, and works on balance, reflexes, coordination, strength and stamina. Good coaching is essential for HSPers!

Aerobic Classes / Circuit training / Boot camps

Many have a fast-paced focus for the cardiovascular benefits.This can be difficult for someone with deteriorating coordination and increasing spasm. They often have large participant numbers and are not as focused on the individual. Rarely is there a second instructor who gives ‘on the floor’ guidance. The frequent change of movement also requires fast responses and challenges the very systems most damaged in HSP. For those HSPers inspired by this type of activity – class choice is paramount!

Similarly, home exercise videos do not allow interaction and feedback on your technique, but they do often frequently repeat the key coaching points of each exercise and you can take it at a more personal pace and repeat sections as you wish.

Gym and weights based resistance programs

Consider that if you are taking ‘Baclofen’ or other anti-spasmodic drugs, they work by inhibiting the conduction of some nerve impulses, therefore many people with neurological conditions taking these medications report feeling generally weaker. They report that these drugs can seem to exaggerate the difference between the strength of affected muscles and unaffected muscles.

Therefore, regular consultation with an experienced trainer should be a must and probably in consultation with an experienced Physiotherapist to ensure that the balance of muscles trained is appropriate in someone affected by HSP.

A mix of both strength and endurance is ideal and will be different for each individual.

Progressive resistance exercises generally involve ‘free weights’ e.g. dumbbells, or machines, and often both. Free weights and pulleys have some advantages – they require greater postural stabilising; also greater concentration on, and consideration of, technique for both effectiveness and safety compared to resistance machines. The movements on most resistance machines are isolated and somewhat divorced from the normal functional use of the strength, but they can have their place if targeting of specific muscles is desired.

Developing specific strength in individual muscles can be beneficial providing it is then trained into functional movements. Recent studies with the Stroke population have demonstrated that progressive resistance training does not increase spasticity, however this may not be the case for everyone.

Poor and possibly harmful technique is evident in many people who attend gyms and do resistance training.Get professional advice and guidance, at least until you really know what you are doing.

Rowing machines

The strong ‘leg press’ action and need for considerable flexibility required in the legs and low back for sitting with legs outstretched can be a problem with spasticity. As can the timely co-ordination of upper body and legs as power is required which needs faster movements. This is a demanding exercise both in terms of co-ordination for desired technique as well as the physical stress. However balance requirements are minimal, but getting up from the low machine (similar to out of the boat) can be difficult when fatigued. Good initial technique coaching is essential.

Kayaking

There are machines around in fitness centres that simulate the action well, with the ability to vary the resistance. Good paddling technique relies on developing good body control around the pelvis and lower trunk to allow the smooth spinal rotation required for the arm action, otherwise there can be considerable strain at shoulders and forearms. This is likely to be a more suitable activity for HSPers but is much less available than rowing machines. Good initial technique coaching is essential.

The same considerations apply for rowing or kayaking, plus additional difficulties, when taking these activities onto the water.

Other Forms of Exercise

Horse riding – many similarities to cycling with less effort.

Surfing / body surfing – just being challenged in the waves can be excellent strengthening, CV and balance training. Beware of too much sand walking as there can be a tendency to overuse less desirable muscles and a sideways slope can be difficult.

Sailing – (being in the crew that is, not just lazing back under the canvas).

Tennis – if tennis is beyond you then try ‘Swingball’ or ‘totem tennis’ in the backyard or park. Great for CV, coordination, balance and some strengthening.

Golf – often too much standing around, and slower speed walking – using a ‘driving range’ and ‘putting green’ is probably a better option as you dictate the pace. You need good core stability and efficient muscle use to be effective.

Bowls/Bowling – specifically good for balance and weight shifting – very difficult to do well unless most leg muscles are working as a team. No CV benefit.

Basketball – shoot basketball hoops with the kids. You might want to stay off their scooters and skateboards though!!!

Whole body vibration (WBV) – 2 types. This is a new concept beginning to appear in Fitness and medical specialist centres. Purports to give overall workout just through standing on a vibrating platform for a few minutes. Claims to improve strength and coordination and bone strength. Many of these claims have some evidence behind them, but use with people with neurological conditions requires more consideration of the individual than will generally be found in a fitness centre. Such units are now turning up in Physio practices, but experience with this treatment is still in it’s infancy.

Stretching

This is a difficult topic because there is little scientific evidence regarding the efficacy of stretching regimes in those with neurological conditions. There is also little guidance on the most effective way to stretch, e.g. prolonged or short duration. Muscles generally become shortened or tight if overactive especially if other muscles around the same joint or nearby joints are weak or inhibited for some reason. This could just be the habit of the individual to ‘favour’ some muscles over others because they have ‘learned‘ to move that way or because of neurological changes or injury. Other muscles become short or tight because the joint over which they act is never taken to it’s full range to lengthen them out, often for the same reason as above.

Aggressive stretching of muscles that have not been warmed up by gentle activity pumping blood through them can easily cause damage within the muscle tissue. This can then lead to further restriction of range of movement and ease of lengthening.

Aggressive stretching of a muscle that is short for a reason, as above, can also cause problems with joint control and stability and lead to injury if the muscle relaxes or is stretched so that it no longer contributes to control of the joint motion.

As stated before, incorporating a mixture of activities using different body parts in different activities that include opposite movements, e.g. pushing and pulling, taking them to as full a range of movement as feels safe and controlled is probably the best management strategy. Considering what postures and positions the body is experiencing throughout the day or week, especially those that are prolonged can give clues as to why some muscles seem tight or movement restricted.

Where possible, stretching should be through active use of opposite muscles, e.g. stretch the upper front area of the shoulder by contracting the rear lower shoulder blade muscles, and vice versa.


3 Final Tips

 

1. ‘No Pain – No Gain’ does not apply to any of the above! Exercise within your limits and back off if experiencing pain.

 

2. The greatest benefit of these and all activities is when the principles and the gains are carried over into all daily movements!

 

3. Frequent use maintains the skills. “Use it or lose it” or should that be ‘Use it well or lose it’.

 

Lea has many years experience as a practising physiotherapist.  She has patients with HSP and works to manage their symptoms, maintain their mobility and keep them fit.

Depression study on HSPers

Condition found to be prevalent

Over half the population in a depression study of HSPers were diagnosed as depressed.

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Three-quarters of those with depression had mild depression, with most of the rest having moderate depression.

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A link was established between depression and mobility, with those least mobile experiencing worse depression.

 

OBJECTIVE: To evaluate the prevalence of depression and sensitivity and specificity of the single-item interview ‘Are you depressed?’ for people with hereditary spastic paraplegia in Estonia.

DESIGN: Single-item interview ‘Are you depressed?’ was used as a screening question for depression; all participants then completed the Beck Depression Inventory.

SETTING: People with hereditary spastic paraplegia identified from the epidemiological database who agreed to participate in the study.

MAIN MEASURES: Beck Depression Inventory, clinical interview.

RESULTS: The epidemiological database consisted of 59 patients with clinically confirmed diagnosis of hereditary spastic paraplegia. Forty-eight of these consented to participate in the study. The Beck Depression Inventory score was higher than cut-off point in 58% (28/48) and lower in 42% (20/48). Of the study group, 44% (21/48) had mild, 13% (6/48) moderate and one person revealed severe depression. There was a statistically significant correlation between Beck Depression Inventory score and level of mobility; no other significant correlations with other measures were detected. Of the participants, 54% (26/48) had subjective complaints about depression and answered ‘Yes’ to the single-item interview ‘Are you depressed?’. The sensitivity of the one-item interview in the hereditary spastic paraplegia group was 75% and specificity 75%.

CONCLUSIONS: Our results show that mild depression is prevalent among people with hereditary spastic paraplegia. Although the single question may be helpful, it cannot be relied upon entirely when assessing a person for depression.

SOURCE: Clin Rehabil. 2009 Sep;23(9):857-61. Epub 2009 Jun 26

The prevalence of depression in hereditary spastic paraplegia.

Vahter L, Braschinsky M, Haldre S, Gross-Paju K.

Neurology Department, West-Tallinn Central Hospital, Tallinn and Institute of Psychology, University of Tallinn, Tallinn, Estonia. liina.vahter@ltkh.ee

New HSP detection technology for embryos

Embryo test could help end HSP

Finding the faulty gene: Embryology test that could kill off inherited illnesses


http://www.timesonline.co.uk/tol/life_and_style/health/article5004111.ece

The development of a universal embryo test that can detect almost any genetic disease is a landmark in medical science’s ability to help couples touched by inherited illness to have healthy children.

While it has been possible since 1989 to screen embryos for certain hereditary conditions, using a technique called preimplantation genetic diagnosis (PGD), its scope has always been limited by technological constraints.

With the fresh approach, known as karyomapping, doctors will be able to offer this service to couples affected even by very rare conditions, on a much faster timescale than is possible at present.

Karyomapping builds on the standard PGD procedure, which was developed by the same doctor, Alan Handyside, in the late 1980s. In PGD, embryos are created by IVF, and when they have grown to eight cells a single cell is removed from each embryo for genetic analysis. Only unaffected embryos are then implanted into the womb.

This can already be done for about 350 genetic conditions, such as cystic fibrosis and Huntington’s disease. Its use is limited, however, because scientists must first identify the precise DNA mutation that affects a family and then develop a test for it. This can take more than a year, and costs several thousand pounds.

A newer version of PGD developed two years ago at Guy’s Hospital in London can screen embryos when precise mutations are unknown, but it still requires lengthy test development.

Karyomapping, by contrast, can be performed within a few weeks, for any inherited condition. At present, costs are comparable to the £1,500(UK) price tag of standard PGD, but these are expected to fall significantly as the technology is developed.

DNA samples are first obtained from the parents and a close relative, usually a child who has the disease in question. This can be done using a noninvasive cheek swab.

This information is then compared with DNA from an embryo, obtained by a biopsy in similar fashion to PGD, to map how its chromosomes are built from the genetic material of its four grandparents. The map can determine whether the embryo has inherited chunks of chromosome that contain any faulty gene.

“What we’re basically doing is mapping family trees, so you can work out which parts of your chromosomes came from which grandparents,” Professor Handyside said. “This turns out to provide a truly universal method for PGD – that’s why we’re excited about it.

“At the moment, there are preimplantation tests for only a small fraction of the 15,000 genetic conditions that are known. This test is capable of detecting any of them. There is no need to find the mutation that is affecting a family, and work up a test. You do the analysis, and just read off the results.”

As the technique maps all the embryo’s chromosomes, it can check any gene, allowing several to be screened at once. It could also be used retrospectively, once an embryo has become a child, to provide wider information about its genetic inheritance.

“At the end of all this, potentially you could give advice to parents about genes linked to things like heart disease in the embryos you’re transferring,” Professor Handyside said. He acknowledged that this raised genetic privacy issues that need to be resolved.

The only kind of genetic conditions that karyomapping cannot detect are those that arise spontaneously, through a random mutation. Even in these cases, it can streamline traditional PGD tests.

Karyomapping can also detect chromosomal abnormalities that often cause embryos to die. It could thus also be used to select embryos with the best chance of developing, enhancing IVF success rates.

The technique could not be used for sex selection for social reasons, which is banned in Britain. Embryos can be screened for sex to prevent diseases that are inherited only by boys.

Professor Handyside will present details of the test next week at a conference in Hinxton, near Cambridge, and at the American Society for Reproductive Medicine’s annual meeting in San Francisco next month. The technique was developed in collaboration with Gary Harton, of the Genetics and IVF Institute in Fairfax, Virginia.

Independent scientists welcomed the development. Dagan Wells, a reproductive geneticist at Oxford University, said: “This is potentially very valuable because the real limitation of PGD is it is time-consuming and expensive. Virtually every patient who comes through the door ends up with a unique test being designed for them. Karyomapping means we can use one platform for almost every single patient and every single disease.”

Anne Child, reader in cardiovascular genetics at St George’s, University of London, said it would help many of her patients with Marfan syndrome, a rare condition that affects the heart. She said: “In the 75 per cent of patients who have other affected family members able to contribute blood samples for study, Professor Handyside’s new approach will be a very welcome addition, permitting the couple to know from the very beginning of the pregnancy that the gene will not be passed on to their children and grandchildren.”

Karyomapping works by analysing chromosomes – the packets that hold genes. Humans have 46 chromosomes arranged into two sets of 23, one provided by the mother and one by the father, through eggs and sperm.

When these are made, they each receive just one set of 23 chromosomes, so that when they fuse they create an embryo with the normal complement of 46.

During this process, a man’s sperm acquires some blocks of DNA that originally came from his mother (the embryo’s grandfather), and some that he inherited from his father (the embryo’s grandfather). The same applies to a woman’s eggs.

Karyomapping involves drawing up a chart of where these grandparental chunks lie on an embryo’s chromosomes. This can be done by comparing DNA from the parents, at least one close relative, and the embryo itself.

Scientists examine more than 300,000 DNA markers throughout the genetic code, which together can show which grandparent provided a particular block of DNA. Once the map is complete, it can be examined for faulty genes.

It is not necessary to know the DNA code of a particular mutation, only its position on a chromosome and the grandparent who passed it on. The gene that causes cystic fibrosis, for example, lies on chromosome 7. If the embryo’s paternal grandfather was a carrier, and the embryo has inherited a chunk of his DNA at the critical position, it will have the faulty gene.

The same thing can be done again and again across all the chromosomes, to allow screening for multiple genes. In practice, this is difficult for more than two or three traits, because few embryos will have the desired DNA at every point. A karyomap could also be reused after a screened embryo has developed into a child. This could reveal genes that give a raised risk of Alzheimer’s or heart disease.