Can some exercises ‘induce sporadic HNPP’?

Aug 22, 2017Aug 22, 2017 suswatibasuLeave a comment

It’s a fact that most people with HNPP know, but there are suggestions that certain exercises can actually do more damage than good. From walking to some types of yoga, while most forms of physical activity are encouraged by gym instructors, our bodies tend to rebel from the norm. But can exercise actually induce HNPP, even though it’s generally thought to be an hereditary condition?

As HNPP is an autosomal dominant disorder in which the condition is inherited and there is a deletion of one of the genes associated with PMP22, a child of an affected individual is at a 50 per cent risk of being affected by the condition. This suggests that the likelihood of a person developing HNPP out of nowhere is relatively low.

What are ‘sporadic’ cases of HNPP?

There have been recent reports that state that a few types of muscle training has actually brought about sporadic symptoms of HNPP without any previous familial history of the condition. Sporadic cases due to de novo deletion accounted for 21 per cent of the investigated HNPP families, as reported by some studies. De novo mutation is an alteration in a gene that is present for the first time in one family member as a result of a mutation in a germ cell of one of the parents or in the fertilised egg itself.

According to a 2017 study carried out by researchers at the Department of Neurology and Rheumatology, Shinshu University School of Medicine, Matsumoto, Japan, this apparently was the case in a 15-year-old boy. The authors suggest that this is the first instance of an adolescent “that developed neurological symptoms during muscle training in a school baseball club activity”, the first signs in a sporadic case of HNPP. The teenager developed bilateral painless brachial plexopathy through short-term barbell training and plank exercises.

“Patients sometimes show an atypical clinical phenotype, and a diagnosis of HNPP can therefore be challenging, especially in sporadic cases as in our patient.”

“Muscle Training-induced Bilateral Brachial Plexopathy in an Adolescent with Sporadic HNPP” – Kodaira, M., et al – July 2017

While brachial plexopathy can be a common feature of HNPP, bilateral involvement is thought to be unusual because HNPP is usually associated as a mononeuropathic condition, where there is a focus in only one area of nerves.

The authors in this case say: “As his clinical and NCS [nerve conduction study] findings indicated muscle training-induced bilateral brachial plexopathy in HNPP, genetic analysis for this disorder was performed, which revealed deletion of the PMP22 gene. Patients sometimes show an atypical clinical phenotype, and a diagnosis of HNPP can therefore be challenging, especially in sporadic cases as in our patient.”

The major difference in this occasion is that not only did he suffer from a bilateral injury, he also developed a sporadic case of HNPP triggered by relatively straightforward exercises. This is unlike some of the other examples where those in military training are far more likely to develop symptoms linked to HNPP, due to the strenuous physical activity undertaken by soldiers. However, this is not the first time ‘push ups’ are seen to be the main culprit as a trigger.

In another 2017 report A Case of HNPP due to Push-up Exercise, a 17-year-old man with no familial history of the condition developed “motor and sensory disturbance of the left upper limb a few days after starting push-up exercise”. Scientists from the Department of Neurology, Hirosaki University Graduate School of Medicine, say that the patient also developed brachial plexopathy, similar to the case above.

In the abstract, they conclude that: “Genetic tests revealed a diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP). HNPP should be included in the differential diagnosis for neuropathy due to slight exercise or nerve compression even when familial history is negative.”

The original text is in Japanese – read full document here.

Researchers from the Division of Neurology and the Department of Pathology, University of Missouri School of Medicine, Columbia, USA, found a case in 2004 akin to the above studies. A 21-year-old in good health began to develop symptoms of HNPP on her first day of military training. She began to show symptoms of severe pain, weakness, and atrophy in her right shoulder, foot and hands. Her mother and her family had no history of neuromuscular disease. She did not know her father or his family history.

jnnp-2004-November-75-11-1629-F1.large — (A) Teased fibre preparation of the right sural nerve showing focal sausage shaped enlargements of the myelin sheath (tomaculae) (indicated by solid arrows) and thinly remyelinated internodes (indicated by arrow head). (B) Epon section of sural nerve demonstrating marked variation in myelin thickness and several large hypermyelinated axons (indicated by solid arrows).

Researchers of the study Fulminant Development with Axonal Loss during Military Training say that the patient’s symptoms were “unique” and that she did not have the “cardinal features of HNPP” including inheritance and mononeuropathy.

They say: “While the presentation and severity of this patient’s condition may relate to a specific unknown genetic profile with very low PMP-22 mRNA levels, Schenone et al, in finding correlations between reduced PMP-22 mRNA levels and disease severity, also suggested that extrinsic factors—for example, level of physical activity, may be important in determining phenotypic features.

“This appears to be true of our patient—that she was neurologically normal, then developed symptoms on the first day of military physical training with progression as she continued the training over a three week period, suggests that disease severity and focal axonal damage were related to these intense activities.”

The authors add: “Additionally, as it has recently been recognised that sporadic cases of HNPP are common, either because of de novo mutations or asymptomatic carriers, her lack of family history did not preclude this diagnosis.”

How likely is it that a case of HNPP is sporadic?

It’s important to note that HNPP may not be easily traced in other family members, which means while it may appear sporadic, it could just have been overlooked. A 2013 case report reiterates this view, with authors saying that approximately one-third of deletion carriers are unambiguously detected on the basis of “electrophysiological criteria and confirmed by genetic analysis are asymptomatic and do not display significant signs at clinical examination.”

Researchers from Department of Paediatrics, Hospital de Guimarães, Portugal, say: “Thus, the family history is often uninformative, and a significant proportion of probands may be considered as apparently sporadic cases. However, a close questioning and examination of the relatives provided evidence for autosomal dominant inheritance in families that were originally stated by the probands to be normal. Therefore, HNPP can easily be overlooked in those cases in which familial involvement is not recognised unless intensive ascertainment techniques are used.”

Electrophysiologic studies are said to be “suggestive” and not “sufficient for diagnosis” which may be why it may be imperceptible to locate.

How exercise can trigger symptoms

The more frequent representations are in those who already have familial histories of HNPP, or in those who have already been diagnosed. In one such case in 2005, hiking and athletic training brought on the symptoms. A 10-year-old girl, who suffered from acute, recurrent monoplegic episodes affecting both the sciatic nerves and the left brachial
plexus since the age of 7, showed quite a lot extensive symptoms from relatively low impact exercise.

Authors from the Department of Paediatrics, Yokohama City University Medical Centre, Japan, say: “This school child having HNPP is considered to be susceptible to the influence of abundant physical training, rather than minor trauma or compression at sites of entrapment of peripheral nerves.” However, this is hardly surprising given the nature of the condition. What’s more unusual are the situations where HNPP is not present beforehand or without familial background.

It goes without saying, exercise can cause many types of injuries even for those without HNPP depending on how extreme it may be. And as we have seen above, there have been at least several cases of ‘sporadic’ HNPP, which develops without any prior history of symptoms or familial connection.

However, it may be worth noting that without the correct tests to detect hereditary links, these may be incorrectly termed as ‘sporadic’. That being said, there have been established reports that suggest at least 21 per cent of HNPP cases are de novo mutations, so it may not be out of the realms of possibility that certain types of exercise can induce symptoms of HNPP.

Read: Is walking good for those with HNPP?

Is walking good for those with HNPP?

Jul 30, 2017Jul 30, 2017 suswatibasu4 Comments

From walking 15km a day to walking 15 minutes a day, my walking journey has been chaotic to say the least. But it was another essential question I asked my neurologist. Is it still possible to walk when you’re in pain?

It is shockingly difficult to do for most people living with HNPP, and yet it seems rather depressing to completely forgo what seems to be such a straightforward task. It’s a bit of a vicious cycle as it is difficult to exercise with painful neuropathy and yet it’s very important to stay active.

The major issues that seem to occur with HNPP include:

clumsiness
slight difficulty in walking because of trouble picking up the feet
weak leg muscles
fatigue
foot deformity (very high arched foot/feet)
difficulty lifting foot at the ankle (foot drop)
curled toes (known as hammer toes)
loss of lower leg muscle, which leads to skinny calves
numbness or burning sensation in the feet
“slapping” when walking (feet hit the floor hard when walking)
weakness of the hips, legs, or feet
leg cramps
loss of balance, tripping, and falling

These symptoms obviously cause problems while walking hence it seems next to impossible to even attempt it.

According to Patty Bonsignore, a Nurse Educator at Joslin Diabetes Center in Boston, U.S. dealing with neuropathies, it’s best to do exercises that are non-weight bearing, such as swimming, water aerobics, rowing and chair exercises. However, if walking is the exercise you prefer, and easiest for you, then it is important to make sure your shoes fit correctly, and that you have comfortable, well-padded socks that wick away moisture.

Disclaimer: Please ask your GP or medical practitioner before attempting any exercise included on this website.

Bonsigore suggests seeing a podiatrist or a pedorthist (a professional trained in creating customised footwear) who can help determine whether your shoes are fitting correctly, as correctly fitted shoes are an easy and important way to prevent foot issues.

She adds: “Neuropathy can also affect balance and shift the way you bear weight on your feet. Alterations in weight bearing can put you at greater risk for falls and foot ulcers. If you are having problems with balance, ask for a referral for physical therapy.”

Before you begin to attempt walking

According to CMT UK, a website dedicated to the inherited condition Charcot-Marie tooth disorder, stretching the calf daily is something that everybody with CMT or HNPP should get into the habit of doing to keep the calf muscles lengthened and slow down the development of the deformity.

These stretches are easy to perform and can be incorporated into your daily routine, for example during your morning shower when your muscles are warm and relaxed (just be careful not to slip) or standing up to a work surface while waiting for the kettle to boil.

Before exercising

Before you start to do any exercises, think about the following:

Have you talked to your physiotherapist, family doctor or gym instructor about the right exercises and level for you?

If needed, have you considered orthoses? The right one can make exercising more efficient and enjoyable.

Remember to pace yourself – don’t overdo it. And if you have worked hard one day, think about relaxing the next.

Put together an exercise plan – build up slowly so as not to injure yourself. (The 10% rule is a good one to stick to – aim to increase your exercise levels by no more than 10% to 15% each week.)

Why you wonder? In these types of conditions, the muscles on the shin tend to get weaker first which results in a ‘foot drop’. The stronger calf muscles overpower the weaker shin muscles setting up an imbalance between the two. Because of this the calf muscle will gradually get shorter and stiffer, as will the Achilles tendon, further increasing the foot drop.

Because the ankle needs to be at a right angle for the toes to clear the floor when walking, the result is an increased chance of tripping when walking, increased difficulty getting the heel to the floor and a greater chance of sprained ankles.

Orthoses can play an important role in helping to maintain flexibility, joint range and prevent the muscles tightening and shortening. This can be by means of insoles which may be thicker on the outside of the foot than the inside so the foot is stretched when weight is put on it. Orthoses used through the day can provide a very effective stretch as well as holding the foot in the best position for walking.

Stretching exercises

These exercises are only a general guide. It is strongly recommended that you
consult a physiotherapist to put together an exercise programme tailored to your individual needs.

Disclaimer: If you experience any pain or difficulty doing these exercises, stop immediately and seek advice from your family doctor or physiotherapist.

Calf stretch:
- With finger tips hold onto a wall or work surface.
- Keep your head up and back straight.
- Place one foot forward and one foot back with the back foot and heel fully on the floor. Make sure your toes are pointing forward.
- Let your front knee bend but keep your back knee straight.
- You should feel a stretch in the calf.
- Hold still for 20 to 30 seconds. Repeat three times and then swap legs.

Lower calf stretch:
- Get in the same position as above.
- Step your back leg forward so the toes are in line with the heel of the other foot.
- Letting both knees bend, sink down with your weight on your back leg.
- The stretch will not be as strong as with the previous exercise.
- Hold still for 20 to 30 seconds. Repeat three times and then swap legs.

Upper legs:
- Sit on the edge of your bed or a dining chair.
- Keep feet hip width apart. Keep your arms by your side.
- Stand up fully then slowly sit down.
- Repeat the movement 10 times.

Standing balance:
- Stand near a work surface or wall.
- Stand with your feet together keeping an upright posture.
- Hold for as long as possible using fingertip support on the work surface/wall as required.
- If you are able to, keep this position and turn your head right and left. Repeat the movement 10 times.
- If this is easy, extend your arms and rotate them around to the right and left. Repeat the movement 10 times.
- My physiotherapist recommended doing this while standing on a cushion or foam surface to test the balance a bit more.

This is for stroke recovery patients – however, these balance exercises were recommended to me by my neurophysiotherapist.

That being said, all of these exercises should be done with a touch of caution so please don’t overdo it.

Walking

If you really want to walk here are some tips worth noting:

Use walking aids – if necessary, take along a walking stick or your walker. Researchers in the U.S. showed that the use of a cane reduced the load on the knee by 10 per cent. By reducing knee joint stress, the pain, swelling, and stiffness is less likely to become debilitating. And remember, even expert hikers use trekking sticks.
Padding – make sure you wear all adequate padding such as knee pads, arm pads, splints and general orthotics if necessary.
Stretch before walking – See above for exercises.
Stop as soon as you feel pain – this also includes weakness in the foot.
Rest in between if necessary – it’s not a race, so don’t beat yourself up if you can’t manage.
Know your limits – if you know 10 minutes is enough to make you start feeling pain or fatigue then make sure you walk around the corner and make it back home in time.
Try focusing on your body mechanics – are your knees softly absorbing the impact of each step? Is your pelvis doing a waddling motion back and forth or is your pelvis staying level and steady? Are your shoulders back with your chest out? Each and every part of your body needs to be an active participant in your walk and doing their jobs correctly.
- Head and shoulders: Keep the head up and centered between the shoulders, with eyes focused straight ahead at the horizon. Keep the shoulders relaxed but straight – avoid slouching forward.
- Abdominal muscles: It is important to actively use the abdominal muscles to help support the trunk of the body and the spine. To do this, keep the stomach pulled in slightly and stand fully upright. Avoid leaning forward as you walk.
- Hips: The majority of the forward motion should start with the hips. Each stride should feel natural – not too long or too short. Most people make the mistake of trying to take too long of stride.
- Arms and hands: Arms should stay close to the body, with elbows bent at a 90 degree angle. While walking, the arms should keep in motion, swinging front to back in pace with the stride of the opposite leg. Remember to keep hands relaxed, lightly cupped with the palms inward and thumbs on top. Avoid clenching the hands or making tight fists.
- Feet: With each step, land gently on the heel and midfoot, rolling smoothly to push off with the toes. Be mindful about using the balls of the feet and toes to push forward with each step.
If trouble arises – shorten up your strides and slow down. Walking is a concerted effort that requires you to be mindful. Sometimes you need to relearn how to walk after experiencing a painful injury. That is when a physical therapy tune-up can be so very helpful.
Good walking shoes – your body will appreciate the added cushioning and support. According to peripheral neuropathy sufferer and author, Mims Cushing, Reebok lightweight trainers / gym shoes is a godsend to walkers. Find out more about the best shoes for fragile feet here.
Socks – in the same vein, get appropriate socks. People with neuropathy usually wear non-binding, with extra wide funnel tops. The seams are smooth and the soft, cushiony soles will not irritate your feet. If your feet sweat a lot, consider buying acrylic socks rather than cotton.
Inserts and insoles – inserts can often offload pressure points on your feet and will reduce the chance of ulcers
Avoid uneven terrain – this requires planning your route beforehand, and making sure you do a circular journey so it returns you home rather than walking further and further away. I walk near parks because if needs be I can take off my shoes and stand on the grass.
Plan your route beforehand – as noted above, circular journeys are important so that you make it home in one piece. Apps such as WalkIt and Map My Walk are fantastic for route mapping and journey suggestions.
Water – absolutely essential to keep hydrated throughout.
Snacks – I get exhausted quickly, but quick snacks such as rice cakes, cereal bars or even glucose tablets can keep the fatigue at bay.
Distractions – for those that need it, listening to music, books and podcasts sometimes helps ease the nerves of walking. Focusing on your breathing and posture may make the exercise less frightening.
If it’s all too much – then start on a treadmill, and build up slowly.

Walking is no walk in the park sadly, but it may get a little better each time if you make sure you don’t overdo it, listen to your body, and remember it’s a work in progress.

HNPP · Physical Health

When small tasks become daunting with HNPP

Jul 29, 2017 suswatibasu6 Comments

It’s taken me a week to prepare myself to wash my extremely long hair. The task seemed almost impossible after feeling fatigued the past several weeks. It got me thinking how do those with HNPP manage with menial chores?

People with chronic illness often have problems with housecleaning, chores and the control of clutter. Once a home reaches a certain stage of disarray, it can seem hopeless. Living this way contributes to emotional distress and social isolation, as people feel embarrassed about having guests or generally being near people.

“Have nothing in your house that you do not know to be useful, or believe to be beautiful.”

William Morris

It takes a combination of a lot of things to go from chaos to comfort. Identifying small practical steps you could take and then to do them one at a time. Putting your priorities in order however, is not an easy thing to do, especially when you are chronically sick. Even the simple act of making a small list gives can give you a headache.

After undertaking an interview to fill in a disability form for extra help, it became apparent how much help I needed. The interviewer said that if a task takes twice as long as previous while able-bodied – you do require some support.

So where to begin?

Now obviously, hiring a cleaning service or enlisting help from family members would save you a lot of heavy work. But that isn’t always an option. What’s realistically possible for you depends on your personal situation and health status, but here are general tips to keep a relatively clean and organised home if you’re chronically ill.

However, these are particularly important before commencing:

Rest – being tired doesn’t mean you’re being lazy. If you feel sleepy, then rest! Siestas will become your best friend. Schedule them in if needs be. And switch off your electronic devices.
Priority lists – take the things that you know cannot be avoided that day and put them at the top of your priority list. Things like feeding yourself / and your family, doing laundry. Then, take those same things and think about how you can simplify them. Slow cookers are great to just make a meal in one.
Delegating – for those with families who are more able, you have the gift of many hands. For others, it may be a good friend that you can call upon in a time of need. Learning to ask for help is a bit of skill in itself. The saying that many hands make light work is so true.
Do chores in an ‘ergonomically correct’ way – to overcome physical limitations or minimise pain, you might unknowingly adopt awkward postures that aggregate your symptoms. So pay attention to how you carry your body as you’re dusting high shelves or mopping up the floor. For example, try to keep your shoulders relaxed, use a step to reach spaces above your head or bend through your knees when lifting something off the ground. Stand firmly on both feet, hip-width apart, while you’re doing the dishes and rotate between different chores to avoid injury caused by repetitive motions. Think of housekeeping as a workout (which it is!) that needs to be performed with body awareness.
Substitution and flexibility – somethings unexpected always come up. You may need to practice how to be flexible. I used to be very OCD with cleaning my flat, now I just shrug my shoulders and wait for when the time is a little more suitable.
Congratulate yourself – come to see that, like other chronically ill people, you are always making a huge effort, so you always have something for which you can congratulate yourself.

Housekeeping

Create systems to deal with clutter – sort the mail as soon as it arrives, instead of letting it pile up. Do a little washing up daily to avoid pyramids of plates – even better if you have a dishwasher where you can wait several days to fill it up. Dust off crumbs when you finish a meal. If you can’t that day, try it on the next.
Change things gradually – if you have stacks of unused jars, bottles, newspapers, think about what you really need and if it is really necessary. Start throwing away bits and bobs, with your weekly recycling. This includes clothing, time yourself for 10-15 minutes, cleaning your closet a little every day. It may not clear it all – but it will start bringing the clutter down.
Create simple routines – this can include sorting laundry or laying out each day’s clothes in advance. If you have guests, make sure everyone takes their own plates, a serving dish and silverware to the sink.
Automate away – thanks to technology, many routine tasks can be set up on a schedule, from paying your monthly rent and utility bills to refilling your prescriptions. So find out if there’s a service or feature that will do recurring jobs automatically for you.
Simplify your meal planning – meal planning is an excellent way to limit tiring trips to the supermarket, but it can be daunting to figure out what’s for dinner. To make things easier, you could collect a month’s worth of healthy recipes for each season and use that to create a weekly menu. If that’s too much hassle for you, you can also build your own personalised template with seven days worth of delicious dinners.
List the grocery items you buy regularly – that way you’ll never forget to buy milk again.
Keep house supplies on each floor or generally accessible – having a bucket with spray bottles, microfiber cloths and sponges at hand wherever you need them saves you from needlessly carrying items up and down the stairs. Place baskets at the bottom of the stairs to collect things that need to be taken upstairs.
Create smart cleaning routines – clean rooms from top to bottom, so you won’t knock down dust from higher cupboards and shelves on recently vacuumed floors.
Experiment with other energy-saving tricks – this may be hanging your clothes instead of folding them, sorting dirty laundry in separate hampers and collecting all used home textiles (towels, rags, (table) cloths, bed sheets) in one go.
Consider a “no shoes indoors” policy – it drastically reduces the amount of dirt and germs tracked in.
Build in buffer time – don’t leave things like grocery shopping and laundry until the last minute, right when you run out of food and clean clothes, that just adds unnecessary stress. Also, don’t plan too many cleaning sessions in a row, but leave enough time for rest in between.
Find ways to conserve energy – sit down while you’re peeling potatoes or folding towels. Grab a stool during cooking if you have trouble standing. Consider home deliveries when going out to the shops is a tiring ordeal for you.
On low-energy days – it may feel like all you accomplish is keeping things in their proper places. But that is an important part of housekeeping.

Shopping

Check energy levels – if you’re extremely tired, divide the shopping list up into a couple little trips over several days.
Driving to stores – if possible park near a cart corral so it’s easy to drop it off when you’re done.
Pick a day that isn’t as crowded – if you’re not sure what days are less crowded you can always ask an employee. Or even check Google which shows when the quietest times are of most stores.
Grab a shopping trolley instead of those little baskets – even if you’re running in for three little items, the odds are you’ll pass the water or juice and remember you’re running low. Now you’re left trying to lug those heavy extras around with you. Also, the cart is great to help keep your balance and offer a bit more support while walking.
Bring a helper if you can – that way if you’re getting too tired to even let go of the cart you’ll have an extra set of arms to grab what you need. They also come in handy if you happen to remember you forgot an item, they can run back and get it.
Getting ill/tired – if you just can’t continue don’t beat yourself up. If you see an employee, let them know that you’re sick and have to leave. They’d rather be notified so they can put away the frozen food instead of happening upon a full trolley with thawed items that now need to be thrown away.
Arrange items on the conveyor belt according to where they go in the house – that way all the body wash and shampoo are in the same bags so you can just carry that bag to the bathroom. This also helps once you get home.
Don’t be afraid to ask – if you’ve used a trolley and the store doesn’t allow them to leave the building you can ask the bagger to grab the cart so you can load the groceries in there to take out to your car.
Online shopping – it’s an absolute godsend. You can sit in the comfort of your home, find what you need to buy online without even getting up. It does charge a delivery fee and most stores have minimum charge so wait for your big shop before ordering.

Assistive equipment

Having a helping hand when you suffer from pain, fatigue, weakened muscles or other symptoms can improve your independence and energy levels.

Put your appliances to work for you – the dish washer, dryer, a programmable slow cooker. You could even consider investing in smart helps around the house like a vacuuming robot.
Use supportive tools to make daily activities easier – stools, non-slip mats, long handled gardening tools or kitchen equipment specially designed for people with limited mobility and strength. Google which helpful aids are available for your specific health problems.

As anyone suffering with a chronic illness knows there are good days and bad days. And it’s important to remember you’re allowed to rest, ask for help and be as flexible as possible. Hopefully then, daily tasks will seem a little less daunting.

HNPP · Mental Health · Physical Health

Relaxation techniques to help HNPP sufferers

Jul 19, 2017Jul 20, 2017 suswatibasu3 Comments

After a night of tossing and turning, I found that my nerves tend to get worse. The problem is that when your nerves become frayed, lack of sleep can leave you in a vicious loop, so learning to relax is essential for daily life.

When looking to treat neuropathy considering treatments that can help a person learn how to relax so that their quality of life can improve not only physically but emotionally and mentally should be an option.

Mind-body approaches provide a variety of benefits, including a greater sense of control, improved coping skills, decreased pain intensity and distress, changes in the way pain is perceived and understood, and increased sense of well-being and relaxation.

This approach focuses on the interactions among the brain, the rest of the body, the mind, and behaviour. The ways in which emotional, mental, social, spiritual, experiential, and behavioural factors can directly affect health.

Can relaxation help?

According to a study in the International Journal of MS Care, 67 per cent of multiple sclerosis (MS) patients and 43 per cent of peripheral neuropathy (PN) patients reporting the use of at least one form of complementary and alternative medicine in 12 months.

The study itself used 40 sufferers of MS and PN, who took part in a meditation challenge for two months. While meditation does not change the underlying disease, “the effectiveness of mind-body therapies may lie in their ability to facilitate stress reduction, relaxation, and improvement of mood”.

What meditation technique was used?

During each session, the patients practised three forms of meditation in a group setting that was divided into three parts, each lasting 30 minutes. The first part of the session consisted of walking meditation, the second part consisted of moving meditation, and the third part consisted of sitting meditation. Details regarding each technique are provided below.

Walking Meditation

With the six-part walking meditation technique, patients were instructed to focus their attention on each movement of the foot as they took a step forward. They were told to walk for 10 to 20 paces total, then turn around and walk back the same way. This was repeated for the 30-minute duration of the walking session. The six movements of the foot that they were instructed to concentrate on are as follows:
1. Lift heel
2. Lift toe
3. Move forward
4. Lower the foot
5. Heel down
6. Toe down
Participants were told to think of the movement first, and then concentrate as they physically took each step.
Moving Meditation

Qigong and tai chi are Chinese mind-body exercises that are considered moving meditation techniques in which awareness and concentration are placed on breathing and specific movements of the body. Study participants performed basic tai chi manoeuvres, including neck rolls in which the head was moved slowly from side to side, ankle rolls, shoulder rolls, hip rotations, knee bends, and alternating pulling and pushing movements with the arms. This was followed by more well-known forms such as “cloud hands,” in which they slowly rotated their body from left to right with sweeping motions of the arms in front of them.Qigong is a more physically rigorous form of moving meditation with shortened and very quick but much simpler movements coupled with deep inhalations and forced exhalations. Focus is placed internally with this type of moving meditation. In contrast, the forms in tai chi are more complex and require outward focus.

Participants unable to completely perform all movements while standing because of fatigue, instability, or weakness were allowed to sit in a chair and practice with their arms.
Sitting Meditation
Study participants performed samatha sitting meditation, a form of Buddhist concentration meditation in which the mind is focused on one point. Patients sat in a chair or on a cushion on the floor and were told to close their eyes and focus their attention solely on their breathing. They were instructed to breathe normally and observe the movements of the abdomen with each inhalation and exhalation.

Doing meditation at home

Meditation is one of the most widely used forms of complementary therapy, particularly as a palliative for chronic illness, but a lot of people are quite reluctant to practise it for either its possible religious and spiritual connotations. However, it is actually just a way of alleviating stress, hence it can be done by anyone.

Available meditation research is generally of low-to-modest quality, but tends to support this intervention for the reduction of stress and pain, and improving quality of life in a variety of medical conditions.

This meditation exercise is an excellent introduction to meditation techniques.

Sit or lie comfortably. You may even want to invest in a meditation chair – but really not necessary.
Close your eyes.
Make no effort to control the breath; simply breathe naturally.
Focus your attention on the breath and on how the body moves with each inhalation and exhalation. Notice the movement of your body as you breathe. Observe your chest, shoulders, rib cage, and belly. Simply focus your attention on your breath without controlling its pace or intensity. If your mind wanders, return your focus back to your breath.

Maintain this meditation practice for two to three minutes to start, and then try it for longer periods.

I found this guided meditation particularly therapeutic. Just make sure you don’t have too many distractions, and prepare your environment, such as switching off lights beforehand. It can be done both lying down or in a sitting position – whatever is more comfortable for you.

If you prefer to meditate by yourself with no distractions, there are entire channels on YouTube dedicated to supposed “nerve regeneration”.

Binaural Beats claims to show increased nerve regeneration in the brain. Using some frequency modulators they say they are able to recreate the frequencies that encourage the treatment by allowing the the nerves to begin to regenerate.

This hasn’t been proven but the music is wonderfully relaxing nonetheless. However, just to warn you, many of the videos are over an hour long and if you don’t have an ad blocker, it can be rather alarming when it gets cut off in between.

What other forms of relaxation are there?

Relaxation and biofeedback are directed toward helping persons with chronic pain become aware of their ability to exert some control over physiologic processes of which they are not normally aware.

Biofeedback – The stressors of nerve pain can be eased using biofeedback. Biofeedback is a mind and body relaxation technique that helps neuropathy sufferers learn about their body’s natural internal process to control relaxation. Patches, called electrodes, are placed on different parts of your body to measure your heart rate, blood pressure, or other function. A monitor is used to display the results. With help from a biofeedback therapist, they will describe a situation and guide you through relaxation techniques.
- EMG (Electromyograph) – The most common biofeedback therapy is the EMG. Because the EMG is used to help correct muscle pain and stiffness it can be the most useful for one suffering with stiff muscles as a result of nerve damage and lack of movement. The device that is used is called an electromyograph which is able to measure the electricity given off by the patient’s muscles.
- PST (Peripheral Skin Temperature) – A less common form of biofeedback is the PST. The PST is able to measure electrical impulses given off by the flow of a patient’s blood. By doing so, it is able to give information about skin temperature.
- EDR (Elecroderm Response) – An EDR is considered sweating biofeedback. It is able to monitor electricity produced by a patient’s sweating reflexes. This form is usually used to help with anxiety and depression.
- Electroencephalogram (EEG) – An EEG monitors the activity of brain waves linked to different mental states such as wakefulness, relaxation, calmness, light sleep and deep sleep. This process is also known as neurofeedback.
- Galvanic skin response training – Sensors measure the activity of a person’s sweat glands and the amount of perspiration on the skin, indicating the presence of anxiety. This information can be useful in treating emotional disorders such as phobias, anxiety and stuttering.
Hypnosis – a state of deep relaxation, which involves selective focusing, receptive concentration, and minimal motor functioning. A National Institutes of Health Technology Panel found strong support for the use of hypnosis for the reduction of pain. Individuals can be taught to use hypnosis themselves (self-hypnosis), and the use of self-hypnosis can provide pain relief for up to several hours at a time.
Massage Therapy – Massage therapy is looked at as a complementary therapy that when used in combination with other treatments can be beneficial in reducing nerve pain. One complication of neuropathy is the development of still muscles due to poor circulation or from lack of use. Adding weekly massage therapy sessions, one can improve blood circulation as well as help loosen stiffened muscles thus providing relief to the damaged area. It appears as though research has shown through several studies that massage therapy has a way of calming the stressed nerve endings and relinquishing the pent up stress found in the nerve endings.

While it may not conclusively help to heal our battle scars, it is always wonderful to try and find new ways of relaxing, freeing our day-to-day constant worries whether for chronic pain and illness, or just life in general. Everyone has a different method of relaxing, it’s just important to acknowledge it as part of our daily routine.

HNPP · Mental Health

Things you shouldn’t say to those with HNPP

Jul 16, 2017 suswatibasu3 Comments

This was the subject of another article for those with multiple-sclerosis and it seems totally relevant for those with HNPP! It can be rather irritating for those with hereditary neuropathy, which can be an invisible illness for many, to hear people’s responses in a bid to stop the uncomfortable silences. But why do people feel edgy and how can we help stop the unwanted questions and replies.

To be fair, this seems to be the case across the spectrum for those with mental and physical health issues. Scope, Britain’s leading disability charity, conducted a survey into attitudes to disability, and apparently 67 per cent of British people feel so awkward around disabled people they either panic or avoid all contact. So what can dispel the awkwardness?

Disability blogger Goldfish explains that ‘awareness’ is not always something to rush towards. It not only creates the idea that a disabled person should be sharing their medical histories at will but creates a relationship between the disabled and the non-disabled that’s unequal, characterised by a damaging, charitable pity. Every ‘favour’ done for someone with HNPP or any kind of disability can be framed as care and take on a special charitable status instead of general eligibility. And that’s where the myth needs to be undone and it seems even more difficult when it’s not obvious.

Nothing less than a cultural and economic shift to full humanity for disabled people will improve attitudes to disability. But in the meantime here’s what not to say.

So what should people avoid saying?

You don’t look sick – This is rather an extreme thing to say obviously but with chronic conditions, those around us only see the incremental changes. As chronic illness patient and advocate at the Invisible Disabilities Association, Sherri Connell, says: “The biggest grievance those with chronic conditions have is that their loved ones often do not believe what they are going through is real, because to others they “look good.” Sadly, this makes the person feel as if they are being called a liar or a wimp.” This can actually set HNPP sufferers back, because it’s difficult enough to live with pain let alone trying to make sure you look a suitable way for the world to accept you.
You will get better – Not necessarily. Making the assumption that there will be improvements, which will only be short term with progressive conditions, only sets to ease the mind of those asking. And when we don’t improve and our symptoms even worsen, it is us who are frequently thought to be exaggerating. And as difficult as this is to admit, this maybe the new normal.
I have that too! – One of my favourites – unless you really do have the condition, it really isn’t the same. In an attempt to relate to you, people may compare themselves to you. It’s normal for people to think they can relate to something they don’t quite imagine how hard it actually is.
I have a friend/cousin/aunt who has the same and they’re doing great – ummm no. Everyone has an opinion and everyone knows someone who is friends with someone that has a certain disease–and that might be true. But not everyone is the same, and the exact same health condition can affect different people in very different ways.
It could be worse – Well of course it could be, but every health condition is a serious health condition. People tend to tell you that things could be worse, that you could have a more serious disease, but no one knows what you’re going through. Having a chronic illness means that you have to deal with it for the rest of your life. The only time comparisons might be okay is when we compare where we are now in this illness and our treatment to where we were when we first got sick.
You’re cancelling again – Believe me, this is only a last resort. Human beings are generally social creatures, hence cancelling is only so we don’t hurt ourselves further. And the issue with HNPP is that our symptoms can change hour-to-hour. The bottom line is – don’t stop making plans, it’s a postponement not a cancellation.
If you need anything… – this is said with great intentions. And it is a lovely gesture. The problem is that it is too broad-ranging. We mean it 100 per cent when we say it. So maybe be specific i.e. I’m going to (supermarket), do you need anything?
I wish I could stay home all the time – if only we were sitting at home running a Fortune 100 company from the comfort of our beds. But we’re not. Instead when we are home, we hate ourselves for our inability to do what once was the simplest most mundane tasks. We worry about being burdensome. We worry that we’re being lazy. And we compare ourselves to what we were – we don’t want to be home all day. We miss our active lifestyle.
God never gives us more than we can handle – Thankfully I live in the UK so I get this less. But it has happened. It is a bit of a ludicrous statement to make given that according to the National Center for Health Statistics there are 16,235 deaths from prescription opioids every year in the US. As patients feel alone and hopeless, suicidal thoughts often overwhelm them. They’d do anything to stop the pain. So please refrain from such statements, but instead think about communicating and reaching out to stop sufferers feeling lonely.
Everyone gets tired – That may be true. And most people are not getting enough sleep and rest. But the difference between someone with chronic illness associated fatigue and an otherwise healthy person is the level of fatigue. Unless you literally think to yourself “how much energy will that take?” for every single action you take during the day (including brushing teeth, combing hair, standing to do dishes, putting on makeup, cleaning, driving, etc.) then you experience a completely different kind of tired than people with certain chronic illnesses.
You’re just stressed – When we don’t understand something and don’t look physically sick we assume it is mental. It must be cultural or part of human nature based on how often this is said to people with chronic illnesses. Stress, depression, and anxiety can all make symptoms of chronic illnesses worse. But they do not usually cause them.
Be positive – It’s great to be positive but it isn’t a cure. Sometimes there’s also a level of denial when you overdo it. Positive thinking that is productive for chronic illness sufferers is not telling someone that thinking positively will help them with their symptoms. Instead, productive positive thinking is finding the positivity that comes with their illness.
Have you tried / you should stop – Unless you’re a medical professional or have some actual experience and knowledge with the condition, suggestions are unhelpful. Someone with a chronic illness doesn’t want to defend themselves to you on how they have already tried or don’t trust the efficacy of a certain treatment, especially if your evidence is only anecdotal.
All you need is exercise – Exercise is really important and no one is denying that. It helps pretty much any health condition. But it isn’t a cure-all. Those attempting to exercise usually do it as a form of physical therapy as there are so many limitations, especially when certain exercises can worsen the damage.
You need to get out more – While a change of scenery is wonderful, chronic fatigue and pain will stop you. That’s it. It also makes them feel guilty for not being able to do something they already want to and are probably trying to do.

The absolute best and most powerful thing you can ever say to someone with a chronic or invisible illness is “I believe you”.

I’m sure you’ve all heard a lot more. Feel free to add your comments!

Follow here if you want to find out more information about the End The Awkward campaign.

HNPP · Physical Health

How important is water to those with HNPP?

Jul 7, 2017Jul 7, 2017 suswatibasu4 Comments

Water is one of those debatable topics that seems to arise regularly. Can hot water help nerves? Or is it cold water that can help ease some of the pain? The jury is out there on this one.

HNPP and other hereditary neuropathic conditions can affect sensory and autonomic nerves (sensory neuropathies), or sensory and motor nerves (sensory and motor neuropathies). Sensory nerves carry sensory information – about such things as pain, temperature, and vibration – to the brain.

When the sensory and autonomic nerves are affected, the ability to feel pain and changes in temperature is impaired more than the ability to sense vibration and position (knowing where the arms and legs are). The hands and feet are affected most. Hence the temperature of water that you may need can depend on how the area affected is currently feeling.

According to LtCol Eugene B Richardson, who authors the Neuropathy Journal site, some patients have found that putting the affected area in cool tap water, not freezing, for 15 minutes before bed may calm the damaged nerves. Other patients have found that warm water, NOT hot, helps rather than the cold water. These suggestions featured in Peripheral Neuropathy in the American Academy of Neurology but Dr. Norman Latov and in You Can Cope With Neuropathy by Mims Cushing.

The bottomline is that it really depends on the individual. And the main thing is that it has to work for you. However, neurotherapists suggest you should always spend more time soaking in warm water than cool water.

But why is water important for HNPP’ers?

For people suffering from neuropathy, performing exercises is just not possible due to the severe nerve pain. Few low impact exercises can help control or reduce the symptoms of neuropathy. Though not all exercises will work for everyone, but there are some water exercises that can be of great benefit to people suffering from neuropathy.

Disclaimer: Please ask your GP or medical practitioner before attempting any exercise included on this website.

Julie O’Connor, Aquatic Specialist, who deals with neuropathic patients, told a 2016 conference for Neuropathy Alliance of Texas, that you get 17 times the resistance of being in the water than on land.

Why choose water exercise?

Improve muscle strength
Increased circulation and oxygen around the body through the blood – it is said that nerves regenerate better if there is more oxygen in the blood
Improve coordination
Improve range of motion
Decrease pain
Decrease weight bearing on joints
Improving balance prevents falls
Social interaction
Combat depression

Neuropathy and exercise

Safety first – safety on the deck of the pool is important, as tiles can be slippery, and you may have to consider how to get in and out of the pool. Aqua pool shoes can help for those with neuropathy in the feet. Keep a bottle of water, you’re still sweating!
Do what you enjoy – when you stop moving, your body starts rebelling. When you move, you bring oxygen to the tissue, staving off the initial neuropathy
Listen to your body – if it hurts, don’t do it, however, generally water doesn’t have a massive impact on your body.
Two hour rule – for any exercise that you’re doing, if you’re sore in your joints, or you’re feeling worsening fatigue in your nerves then you’ve done too much. Make sure you’re doing the exercises properly.
Move it or lose it – you’re likely to gain more issues if you don’t move at all.

Types of aquatic exercise

Shallow end – Using different equipment or even just doing poses with correct posture.
Deep (non-weight bearing)
Training for specific goals such as rehabilitation, weight loss, balance, cardiovascular and muscular endurance, pain management
Swimming – sometimes one-on-one can help at the beginning for those not ready for a group class. You may even not even need to swim but do easy positions instead.
Ai chi – Tai Chi in water
Water strolling – In water that is about abdomen high, stroll over the pool swinging your arms as you do when strolling ashore. Abstain from strolling on your tiptoes, and hold your back straight. Fix your abs to abstain from inclining too far forward or to the side.
Hydrotherapy – Alternating between hot and cold water helps expand and constrict the blood vessels – forcing the blood to move through the vessels to other areas in the body. In addition to improving circulation – the warm water releases pain-relieving endorphins that help block pain. The warm water also helps the body to relax, thereby reducing the stress and anxiety that can aggravate your symptoms.

It may or may not work for everyone, but personally, it’s great just being able to move without cramps for a change, and it’s important to be out and about to avoid isolation.

Will you take the plunge?

HNPP · Mental Health · Physical Health

HNPP and Tai Chi

Jul 3, 2017Jul 4, 2017 suswatibasu4 Comments

I’ve heard on the HNPP grapevine, Tai Chi (太极拳) is supposed to be incredibly beneficial for people with nerve disorders. You may think it’s only for a certain generation but believe me the science proves otherwise.

According to a 2010 study published in the American journal of Chinese Medicine from the Department of Kinesiology at the Louisiana State University, Tai Chi actually increases the nerves’ ability and speed of sending signals back to the brain and spinal cord. While that might be useful for the scientists and the academicians, is it useful for us?

Disclaimer: Please ask your GP or medical practitioner before attempting any exercise included on this website.

The best way to put it to the test is to try out some of the leading workouts. One of the moderators and organisers of the annual HNPP conference, which takes place in the US, recommended the video below.

This is perfect for beginners, zero strain on the limbs and it gives you a good idea about how to do / remember the positions and movements. It will then give you access to much more complex and challenging poses. The best thing is that no modifications are necessary! I’ve tried this personally. The first time I had to close the curtains to stop peering eyes out of sheer embarrassment. The second time, I did not give two hoots.

As you know, with our failing limbs, balance tends to go out the window. You can only be propped up by chair for so long, hence finding exercises that tackle that very problem are essential.

Something to keep an eye on

However, there are a few things to take into consideration even with the gentle pace of the movements.

Do not roll the knees, this could actually have an adverse effect. Make sure your hip is moving towards the different sides and plant your feet firmly on the ground.
Try and get some padding for your feet even if it’s something light as every time you press down it puts more strain on the calf.
Keeping a straight spinal posture is essential as you could end up hunching and hurting both the upper back and lower back.
A good tip that I’ve heard is to slightly lower the chin so you’re stretching the back of the top of your vertebra in the neck. This is mostly for people who can’t naturally keep their back straight.
Try really hard not to hunch or stretch the back too much backwards. The aim is to keep the axis of the spine in one place and completely straight.
Move the pressure downwards towards the legs in order to strengthen them without too much strain.
And finally do the movements slowly. Take your time, and enjoy the moment.

For those who want to try something a little less taxing, this video has been recommended by the Foundation for Peripheral Neuropathy, in which they say that the more someone with PN learns to use their whole body for both exercise and simple daily tasks, the less pain they experience.

Once you’ve mastered these tips how about giving some of these videos a go? I particularly like this one as it is gentle and the instructor or Sifu explains every movement throughout. No Grand Canyon necessary.

This one is definitely only for slightly more advanced students and those who are having a good day. DO NOT ATTEMPT if you have any foot or wrist drop.

Disclaimer: Please ask your GP or medical practitioner before attempting any exercise included on this website.

With this, it’s important to keep an eye on your knees, especially as you can’t see the instructor’s. Hence I’m going to lay out the pose without the water (sorry folks!) The Sifu (master) also puts some good tips while the video continues to play, so watch the pointers. (Don’t worry, you won’t have to do the move above).

Make sure when you do the bend, your knees don’t track outwards. Keep them aligned going straight. Avoid doing a sumo squat.

Here is the posture, Rise and Sink, out of the water:

Similarly with the next move, make sure the knees don’t track outwards, and the bend is extremely slight. If you have hand splints and wrist drop, you don’t have to do the Anjali Mudra (hands in prayer) pose.

It starts getting a little complicated from here on out so look away if you want to stop.

Bend the knees in the same direction to avoid unnecessary strain. Otherwise you’ll end up with one facing forward and the other on the side.

Similarly, with the Withdraw and Push pose, keep the knees in the same direction and bend only slightly.

This goes without saying. If you have foot drop, you’d surely fall over doing this move so DON’T EVEN ATTEMPT THIS.

The next move is rather complicated and should be done on a good day in terms of your health. It literally consists of turning back and forth, first to your left and then when you reach backwards, turn back right to the front.

Put the leg back on the side you’re supposed to turn, so that if you are turning left, use the left leg to spin around.

Posture nine will require watching the description and movement carefully, described as carrying a ‘silk worm’, moving it side to side but the footwork is similar in terms of keeping your knees in the same direction.

The last pose is actually a bit of a workout on the arms! Just holding a ‘ball’ up for several minutes can make the lactic acid start building.

And I’m sure, after confusing your body into oblivion, it will be happy that it’s had a bit of a workout albeit a slow one.

HNPP · Mental Health · Physical Health

HNPP and Yoga

Jul 3, 2017Jul 5, 2017 suswatibasu3 Comments

Okay, I’m not going to lie, most of the positions (even the image above) are slightly off limits depending on how severe your HNPP is. That being said, it’s still doable, but it will take many modifications as necessary.

Why should you do yoga?

Despite the possible injuries it can cause, focusing on areas such as your back and shoulders will make sure you won’t become completely bedridden, and crippled with back pain (this happened during my early days). It was also recommended by my neuro physiotherapist so at least that’s good news!

Here, I will walk you through a YouTube video that I regularly use for CHAIR YOGA. Yes, you heard me correctly, it exists and it still works. The main focus of yoga is to allow yourself to take time out from the stresses of worrying, because it’s easy to disconnect from your body when you loathe it for failing you. But with some changes, yoga can keep many people from completely falling off the edge.

I’ve never been particularly good, but I’ve practiced Hatha, Sivananda and Ashtanga for most of my life. Believe me, if I can do it, I’m sure you’ll do an even better job! If not, no matter, your body needs to recover.

Remember, NEVER do any moves that can potentially injure your nerves. Symptoms include a tingling sensation while doing the move or numbness. Stop the pose immediately if that’s the case. You know your body better than anyone. AND KEEP THE CASTS / SPLINTS ON.

ADDITIONAL INFORMATION: Since writing this post, a very important issue has come to my attention by a fellow HNPP’er. Remember, breathing is the most significant part of yoga!!! Throughout the exercises, please breathe continuously in a natural flow in order to keep the muscles moving and stop lactic acid from building.

Disclaimer: Please ask your GP or medical practitioner before attempting any exercise included on this website.

So first things first – the chair has to be cushioned. Doing exercise on a metal chair could actually tweak the nerves, so either put a seat cushion on an ordinary chair or use an armchair that has the sides free! I actually use my sofa surrounded with cushions to grab at any point during the sequences.

Second of all, do not put your palms together into the Anjali Mudra position especially if you have wrist issues including wrist drop. In this case, you can just keep the arms up without contact.

I really don’t recommend the next move for people with hand issues. In this case, lightly intertwine the fingers together, keep head bowed and stretch forward. You can even hold one hand over the other instead and reach upwards into the next movement.

When holding wrists with the other hand, make sure you’re in a completely comfortable position to do so. Never do anything that makes the hands start to tingle or go numb.

In the next move, the hands can actually lightly touch the base of the skull on the back of the neck, looking upwards, without bending the wrists in this awkward position. The main stretch is the outward elbows and the neck stetch.

This move is particularly important for the back and shoulder blades. It was recommended by my neuro physiotherapist. It consists of bringing the shoulder blades back down together practicing with breaths in and out.

I wouldn’t suggest crawling on the floor for this move. The yoga instuctor also gives the suggestion to keep the arms on the thighs. Definitely stick to that.

Make sure your legs have padding underneath for this, and position the end of the chair right in the middle of your thigh avoiding the nerves near your hip or near the knee. If you have any kind of foot drop just sit in that direction. This sequence will be repeated on the both sides.

Eagle poses tend to tweak the ulnar nerves in the arms, so you can just lightly press the arms together instead. Do not lean down on to your knee, it will start blocking the nerve in your leg quite quickly.

At the beginning of the next sequence, the instructor pulls her arms backwards, intertwining the fingers. Feel free to just put one hand on top the other and reach backwards keeping the wrists straight.

In the twisting pose, do NOT lean on your leg. If there are no cushions available for your lower elbow to lean on and you’re feeling especially fragile, avoid this and just twist the body upright to that side.

As Laura (the instructor) also says, if the Warrior Two position (Virabhadrasana) tweaks your back leg, revert back to the original Goddess position.

Do not lean your arm on your leg during the side angle pose. In this case, just let your arm dangle towards the floor.

As mentioned before, eagle poses can tweak the arms so bringing the arms together lightly and stretch the neck, looking upwards. Similarly, I wouldn’t cross the legs.

In terms of holding the leg, if you can then do. If not, then try hold it underneath the thigh, and while twisting backwards, keep the leg floated and just lightly keep the hand against the knee, palm facing outwards.

And that’s the majority of modified moves! I hope it helps, it definitely gives your back a good stretch. Doing this daily keeps the strength around your spine.

Good luck yogis!