HNPP · Physical Health

How to travel with HNPP

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It’s summer in most of the northern hemisphere which means it’s time for vacation for many people. It can be challenging for many with HNPP who are planning to get away, either at home or abroad, therefore you may need to do some more research to make sure that your destination is going to meet your needs.

Although travelling abroad can be relaxing and rewarding, the physical demands of travel can be stressful, particularly for those with underlying chronic illnesses. With adequate preparation, however, chronic illness sufferers can have safe and enjoyable trips.

Preparing ahead of your holiday

First things first, before you make the journey there are few bits to consider including if you can manage your health while you’re abroad. It may seem like a formidable task, but it’s better to be prepared for any eventuality.

  • Ensure that any chronic illnesses are well controlled – it may be useful to see your health care provider to ensure that the management of your illness is optimised.
  • Vaccinations – if you are going abroad to tropical climes, make sure you get the correct vaccinations. As you may know, certain immunisations, could have adverse side effects with current medications that you may be taking so it’s essential to check and let your medical practitioner know that this could be a factor. Immunisations may also present symptoms at a later date, so you may have to consider the long term impact as well.
  • Think about where you are going – will you have access to your medical needs if required? Or medications if you run out?
  • Pack a travel health kit – travellers with preexisting medical conditions should carry enough medication for the duration of their trip and an extra supply, in case the trip is extended for any reason. If additional supplies or medications would be needed to manage aggravations of existing medical conditions, these should be carried as well. Your clinician managing your condition should be consulted for the best plan of action. Consider wearing an alert bracelet and making sure this information (in English and preferably translated into the local language of the destination) is on a card in your purse or wallet and with your other travel documents.
  • Medical supplies – make sure you carry on some of your medication if you are travelling by air, and with this a copy of your prescriptions. Take sufficient quantities of medications for the entire trip, plus extra in case of unexpected delays. Since medications should be taken based on elapsed time and not time of day, you may need guidance on scheduling when to take medications during and after crossing time zones.
  • Medication restrictions – check with your embassy or consulate to clarify medication restrictions in the destination country. Some countries do not allow visitors to bring certain medications into the country.
  • Drug interactions – certain medications used to treat chronic medical illnesses may interact with medications prescribed for self-treatment of travellers’ diarrhoea or malaria chemoprophylaxis. You may want to ask your GP about this ahead of time.
  • Provide a doctor’s letter – this can come in handy if heaven forbid you get ill during your travels. Handing this to a practitioner at your destination may avoid any mistakes from happening. The letter should be on office letterhead stationery and should outline existing medical conditions, medications prescribed (including generic names), and any equipment required to manage the condition.
  • Medical travel insurance – this is a given. It’s always better to be safe than sorry even if you’re going away for a few days. Three types of insurance policies can be considered: 1) trip cancellation in the event of illness; 2) supplemental insurance so that money paid for health care abroad may be reimbursed, since most medical insurance policies do not cover health care in other countries; and 3) medical evacuation insurance. Travellers may need extra help in finding supplemental insurance, as some plans will not cover costs for preexisting conditions. Make sure to also check the excess, as sometimes you end up paying more in excess than the policy actually pays you back. You must declare your condition. Many online companies now do offer “medical screening” as you go through the ordering process.
  • Medical assistance companies – this is less common everywhere and isn’t for everyone, however, there are organisations that can store your medical history so it can be accessed worldwide. Some companies are listed below:
  • Research your destination – check facilities that are available at your destination, such as ease of access and transport options. Consult widely including good guidebooks, disability organisations in the the country that you’re visiting, the embassy or high commission of the country you plan to visit, specialist tour operations and tourist boards.
  • Foreign office – check the travel advice by country before you travel and while you’re there which you can find on your government’s websites.
  • Contacting beforehand – when contacting holiday providers, airlines, hotels etc, clearly state your needs and what assistance you require – just telling people you have a particular illness doesn’t mean that they will understand your needs, so you may need to clearly explain them. Service providers are required by law in many cases to accommodate travellers with special needs. However, most need some time to make the necessary arrangements. Mention your needs at the time of reservation, and call the provider 24 to 48 hours before your arrival to confirm that proper accommodations have been made. This checklist from the ABTA travel board may help guide you.
  • Ensure everything is airtight – confirm enquiries, bookings and reservations in writing. Double check all arrangements before departure.

Resources: UK Foreign Office and Centers for Disease Control and Prevention

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Managing air travel

Flying abroad?  Airports are usually enormous, with vast distances to be traversed between check-in areas, and planes. Here are a few things to consider:

  • Avoid connecting flights – although wheelchairs are the last items to be checked into the luggage compartments, and thus first to be pulled off, flying direct can save you unnecessary time and hassle. One exception: If you have trouble manoeuvring into airplane lavatories, long flights may become uncomfortable — so a series of shorter flights might be a better option. If you do choose to connect, be sure to allow plenty of time between flights (at least 90 minutes, or two hours if you need to go through customs or security) to get from one gate to the next.
  • Allow plenty of time – you will need time before your flight to check in, get through security and transfer to your gate. Arrive at least two hours before a domestic flight and three hours before an international flight — more if you’re travelling at a peak time.
  • Mobility access – check in with your flight attendant before your plane lands to make a plan for exit.  All airports provide wheelchair assistance, which is usually excellent, to get from A to B – you can book this through your airline, either at the time of booking by ticking the relevant box, or phoning a dedicated disability booking line.  Make sure you do this well in advance, or at the very least, three days before travelling.
  • Transportation – don’t forget about transportation to and from the airport. If you have a wheelchair, make arrangements in advance to have an accessible vehicle pick you up in your destination city.
  • Wheelchairs – for those with wheelchairs, bring spare parts and tools. Assemble a small kit of spare parts and tools for emergency repairs. You may also be required to dismantle a wheelchair for certain flights or activities; make sure you and your travelling companions know how to do this.
  • Know your rights – before going through airport security, be aware of the rules for travellers with disabilities and medical conditions. I had a few unfortunate experiences with my walking stick.
Accommodation

Be specific about your requirements – ask for whatever you need to make your stay comfortable and ask for written confirmation that they are available. Your travel agent or tour operator should be able to advise you, but you may also decide to call the hotel, resort or cruise liner directly to speak to someone who is familiar with the rooms.

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You may want to think about the following:

Wheelchair access

  • Is there step free access to all the main areas of the hotel, resort or ship?
  • Are there charging facilities for electrical equipment such as a wheelchair?
  • If you have mobility needs or are visually impaired, you should check on the access to public rooms, restaurants, bars, toilets, swimming pool, beach etc
  • Can any equipment you need be hired locally, such as back rests, bathing equipment, hoists, ramps and special mattresses? Information may be available from local disability groups at your destination
  • Is a lift is available and if so, will your wheelchair or other equipment fit?

Location of the bedrooms

  • Can you be on the ground floor if you wish, or near a suitable lift?
  • Do the bedroom facilities fit your needs, for example, is the door wide enough, does it open outwards or inwards?
  • Do the bathroom facilities fit your needs, for example, is the room large enough: is there a roll-in shower or grab-bars?
  • Can your dietary requirements be met?
  • Are there facilities for assistance dogs?

Rooms:

  • Wider entry and bathroom doorways. Easy to open?
  • Mid-height light switches and power outlets
  • Lever type door handles
  • Manoeuvring space on each side of the bed
  • Roll in shower
  • Wheeled shower chair and/or wall mounted shower seat
  • Grab bars in bathroom
  • Raised toilet
  • Lower hanging space in closet

Neighbourhood:

  • Proximity to markets, pubs, restaurants
  • Proximity to health services.

Be prepared, in the unlikely event that:

  • The hotel does not have the accessible room available for you when you arrive. The hotel will need to find you an accessible room, even in another hotel. You will need to ask “where will you put us up for the night?”
  • The complimentary hotel shuttle may not be accessible. The hotel will need to accommodate the service in some other way. “How will you provide alternate shuttle service for us?”

As an avid traveller, being diagnosed with HNPP felt like an end of my solo quests. But with the right information, being prepared, and calling ahead, being on holiday won’t seem like such an arduous task, rather it will be just what the doctor ordered.

Useful Websites and Resources

For more information on travelling with all types of disabilities, check out the websites and other resources below.

Read: Is walking good for those with HNPP?

HNPP · Physical Health

When HNPP ’causes breathing problems’

Lungs

If you didn’t think it was possible, but some individuals with HNPP claim they suffer from breathing problems due to anomalies with certain nerves. Breathing problems associated with hereditary neuropathy are often ignored because most doctors don’t know they can be part of the disorder.

While hyperventilation is less common, weakness of the thoracic diaphragm, which enables people to breathe, and the nerve linked to it is suggested to cause problems with breathing.

Disclaimer: Please ask your medical practitioner or doctor for more information. This article is based on various research, journals and testimonies.

Why does it happen?

HNPP makes the nerves very susceptible to trauma, which plays a pivotal role when it comes to the act of inhaling and exhaling. There are several major issues that create specific problems:

Hypoglossal Nerve

The hypoglossal nerve is the twelfth paired nerve in the brain. Its name is derived from ancient Greek, ‘hypo‘ meaning under, and ‘glossal’ meaning tongue. The nerve has a purely somatic motor function, innervating the majority of the muscles of the tongue.

According to the 2015 study Disorders of the Lower Cranial Nerves by authors Josef Finsterer and Wolfgang Grisold, the nerve directs not only voluntary activities of the tongue, but also involuntary functions, such as clearing the mouth of saliva by swallowing. They report that some people with HNPP have been reported to have issues with this specific nerve. The authors state: “Another [lower cranial nerve] involved in HNPP is the hypoglossal nerve. The affection of the hypoglossal nerve has been also reported in other types of hereditary neuropathy.”

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Researchers at the Department of Neurobiology, Tokyo Metropolitan Institute for Neuroscience, say that there is a distinct link between swallowing and breathing. Using six rats, they report: “The XII nerve activity preceding that of the phrenic nerve (pre-I XII nerve activity) was markedly exaggerated during periods when this relation was disrupted”.

The phrenic nerve plays an important role in breathing because the nerve originates in the neck passes down between the lung and heart to reach the diaphragm. It is responsible for controlling the contractions of the diaphragm, which allows the lungs to take in and release air and make us breathe properly. During the above experiment with the rats, the authors say that “nerve activity was also shown to couple with swallowing in the same manner as ‘real’ inspiratory activity.”

You may experience this when your breath arrests for a moment while attempting to swallow, which brings me neatly on to the significance of the phrenic nerve.

Phrenic Nerve

Nerve fibres of the phrenic nerve, spinal nerves, and laryngeus recurrens nerve project to the diaphragm, abdominal, intercostal, and laryngeal muscles. While it is considered “exceptional”, some HNPP sufferers do face these symptoms.

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In the 2016 report Laryngeal and Phrenic Nerve Involvement in a Patient with Hereditary Neuropathy with Liability to Pressure Palsies, the participant is said to have had vocal cord paralysis as well as “latency of the right phrenic nerve” and reduced right hemi-diaphragm – the muscle that separates the chest cavity from the abdomen and that serves as the main muscle of respiration.

The study suggests that a person with comorbidity, or someone who suffers from several separate illnesses which in this case was chronic obstructive pulmonary disease (COPD), can consequently end up with breathing issues. They also say that this was rectified through COPD treatment. Potential weight loss from HNPP could be a triggering factor.

This video by Professor Mary Reilly from the National Hospital for Neurology in London, UK, explains breathing issues for those suffering from the inherited condition Charcot Marie-Tooth syndrome:

Brachial plexopathy can be an issue withh HNPP sufferers. It is a form of peripheral neuropathy which occurs when there is damage to the brachial plexus, an area on each side of the neck where nerve roots from the spinal cord split into each arm’s nerves. Nerves outside the brachial plexus, extends from the spinal cord, through the cervicoaxillary canal in the neck, over the first rib, and into the armpit.

The phrenic nerve receives stimuli from parts of both the cervical plexus and the brachial plexus of nerves. As Satish Khadilkar and Snehaldatta Khade state in the Official Journal of Indian Academy of Neurology: “Hereditary neuropathy with pressure palsies can present phenotypically like acute brachial plexopathy. But, unlike the classical phenotype, recurrence is unusual.”

Hence damage to this area could potentially weaken signals and cause breathing difficulties.

How likely is it occur?

However, it’s important to note that respiratory issues are more common with the inherited condition Charcot Marie-Tooth syndrome. In a 2016 study including 49 patients with genetically confirmed CMT or HNPP, those with a duplication of the PMP22 gene as well as those with a mutation of the MPZ gene were more likely to face issues with breathing. The HNPP participants were recorded not to have faced respiratory problems.

The authors of Underestimated Associated Features in CMT Neuropathies say that those with the CMT2-I/J (Charcot Marie-Tooth syndrome Type 2) condition had certain mutations in the MPZ gene causing “respiratory insufficiency”, which obviously reiterates the rarity of this occurring when a person suffers from HNPP.

This video, made by CMTA, explains what’s going on in more detail:

The organisation CMT UK has recommended the following, which may be applicable to those with HNPP if you feel that you are suffering from breathing issues.

What to do if you think you’re having breathing difficulties

  • Have your doctor send you to a cardio-pulmonary specialist for a sleep study.
  • Suggest that your maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) be tested both sitting and lying down.
  • If you are having bad morning headaches you could be retaining CO2. The above tests will show that.
  • You can elevate the head (from the waist) of your bed two or three inches by putting bricks under the legs or a suitcase under the mattress.
  • You can keep your weight down.
  • Make sure you have had a pneumonia and flu vaccinations (ask your GP) and treat respiratory infections aggressively.

As mentioned, breathing problems is rather a rare symptom to be associated with HNPP. That being said, there is still a lot of research to be undertaken to establish other symptoms associated with the condition. The main thing is not to hold back and visit your medical practitioner if you feel there may be a problem.

Read: Can HNPP lead to bone-related issues?

HNPP · Mental Health · Physical Health

HNPP and chronic pain flare-ups

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Like lightning, chronic pain can strike you down at any point during the day let alone the week. When you are so sick and unable to think clearly, it is easy to be ruminating in a downward spiral and become stuck in that hopeless state of mind, making your pain worse than it already is. Therefore it is essential to keep a strategy in place to manage the pain.

So what is a ‘flare-up’?

The London Pain Clinic describes chronic pain as a pain that persists for an unusually long period of time, which goes beyond the expected time of healing. It can lead to a series of long-term consequences such as loss of physical activity and sleep, a sense of uncertainty and insecurity about the future and feelings of despair and helplessness.

Pain flare-ups typically refer to those times when the chronic pain is more intense than usual. Flare-ups are often triggered by overdoing things, although the effects might not be felt until later, moving or sleeping awkwardly, or cold weather. From a statistical viewpoint, if pain is being measured on a 0 to 10 pain intensity scale, with the zero level implying no pain, a flare-up will mean episodes in which the pain is at level 7 or higher.

It can last from a few seconds to several hours, appearing in various guises such as muscle spasms, migraines, cramps, sharp and jabbing sensations and the like.

Brittani Daniels started up pamper company called Spoonie Essential Box after being hospitalised with the chronic illnesses, Crohn’s Diseases, Lupus, as well as colon cancer. More information about boxes below.

So what’s the plan?

Obviously, the old adage “prevention is better than cure” is an important notion when seeking to manage chronic pain flare-ups. First of all, it’s important to analyse the pattern in which the flare-up occurs and then develop a plan around it. Make a list of steps you will take the moment you recognise a flare-up. Keep the plan handy and resort to it immediately when you feel a flare-up rising.

  • Positive environment – keep affirming to yourself your will power to sail through the painful episode. Research shows that individuals who retain their self-confidence and positive attitude are able to handle the flare-up episodes much better. Think about what has made you feel better in the past and try utilise this during this time.
  • Alternative medicines – consult your healthcare provider beforehand and make note of any changes required to your medications during the flare-ups. Generally, short-term changes to the medicines are required in case of intense chronic pain flare-ups.
  • Monitor your breathing – tensed muscles and a quickened and shallow breathing immediately precede pain flare-ups. To help control the aggravation of a flare-up, it is important that you concentrate hard on the pace of your breathing and learn to take a deep breath.
  • Create a ‘spoonie box’ – consider keeping a stock of bottled water and nutrition bars near your bed. If you don’t eat anything, your blood sugar will drop and your muscles will become dehydrated, which causes the body to ache and will likely increase your pain. If your pain is already severe, by having easy access to food and water, you’re preventing it from getting worse. While there are many companies that provide pamper spoonie boxes, you can probably prepare one yourself at home. Items to consider include:
    • Snacks – from cereal bars and rice cakes to assorted nuts (obviously not if you’re allergic), having a stash of snacks can help get you through several hours without having to get up and raid the cupboard. This, however, isn’t a meal replacement. If push comes to shove, there are meal replacement drinks if it’s too difficult to heat up some soup. Some great spoonie boxes keep popcorn for those who just want to lie in bed and watch some Netflix. Chocolates are a significant part of my stash.
    • Bottle of water – essential while you’re in pain to keep drinking water and avoid getting dehydrated. If you have a thermos, then some chamomile or herbal tea maybe handy.
    • Additional medication / supplements – you may already have a pill box that helps divide up your medication for the week, but having an additional box for your recovery cache will save you from dealing with opening bottles and popping tablets.
    • Sleep masks and ear plugs – for those wanting a peaceful slumber without the blaring and glaring of the outside world.
    • Affirmations – positive messages during this time can help remind you that this is expected and not to give yourself a hard time for ‘failing to perform’. Even the Serenity Prayer is useful for this situation.
      • “I seek the serenity to accept what I cannot change; the courage to change what I can; and the wisdom to know the difference.”
      • “I know it hurts right now but I have been through this before. I know I can handle it because this will settle in time”.
      • “Stay calm, and relaxed. Tension isn’t going to help. Keep breathing slowly and deeply”.
      • “The pain is quite bad now, but I can get the better of this and stay positive. I must remember what I have done in the past which has helped to distract me for a while”.
    • Slipper socks – if they are loose and padded, these are literally the most comfortable pieces of footwear on earth.
    • Baths – sitting in a bubble bath is not everyone’s cup of tea, but for those who want to treat themselves to a luxury ‘spa’ type of day, then keeping bath bombs in your box can help distract you from what this day is all about.
    • Hot water bottles and cool packs – always useful for nerve or muscular related flare-ups.
    • Books, and DVDs – to pass the time, keep that film you want to watch or that book you want to read for during this time. If you’re in too much pain, revert back to sleep mode.
    • TENS machine – if you have one, these are known to provide quite a lot of help easing pain.
  • Alternative treatments – if it’s possible, getting a massage or some other type of holistic treatment may be beneficial.  You can hire professional services for the same, or request a friend or a family member. A gentle massage of the affected area could help reduce tension and pain – but do ask your medical practitioner in case this may aggravate your pain instead.
  • Stress management – prioritise what you will be able to do depending on the day. It’s a reminder that these things might be possible on a good day. Think about what has made you feel better in the past. As you learn to cope with the flare-ups, you should try and set only realistic pain goals or else you are likely to experience a sense of frustration and failure.. It is important to keep the stress levels under control, if you want to minimise the scope of flare-ups. Stress and anxiety aggravate chronic pain and might worsen the situation further.
  • Don’t ignore the pain – this is a given, and it’s unlikely most people can ignore a flare-up. Immediately begin the treatment of a flare-up as it happens, else it might worsen.
  • Make a diary – it helps to make a diary and make regular notes of your experience with the flare-up. It is worthwhile to note down the activities that preceded the flare-up, the mood-swings, medication taken and any other such information.
  • Don’t stop all activity – there is a high chance your pain will get a little better if you force yourself to get out of bed and shower. Getting out of bed with time, can increase your circulation, decrease headaches, and realign your body that was pushed into a misaligned position for many hours of sleeping in bed. Getting out of bed can distract the mind from focusing on the intensity of the pain. It is actually advisable to continue doing some gentle form of activity that does not cause too much of stress. Try to be purposeful with your activities. If getting out of bed and walking around the house for circulation is a goal to decrease pain, think about where to walk in the house. Even going to the garden, and walking around for several minutes can help lift your mood. If the pain gets worse, then do not forget to rest.
  • Relaxation techniques – if getting out of bed is not a possibility that day, consider downloading relaxation apps. These apps may help lower your stress and anxiety by guiding you through meditation and relaxation when sometimes it’s hard to do yourself.
  • Appointing friends and family to help – if you have the option, call a friend and let them help you in anyway suitable. This may include helping around with certain chores, taking a light walk, or just generally keeping you company. It is totally your prerogative to ask for support.

Unfortunately, learning to manage pain isn’t always a prioritised discussion with healthcare providers. Doctors prescribe more pain medication, blame stress and diet, and your time is done. You are expected to just go home and figure out how to deal with these major life changes.

While medical professionals are only just beginning to understand the impact of chronic pain on daily lives, learning to master the pain yourself will give you a head start.

Resources:

Read: Relaxation Techniques to Help HNPP Sufferers

HNPP

Gadgets to help cope with HNPP

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With its muscle aches and chronic fatigue, HNPP can make the simplest activities painful and difficult. At its worst, it can make chores you once took for granted suddenly seem daunting. So what is available to help with these trying times?

“The number-one tool that one needs during a flare, by far, is this word called autonomy,” says Nortin Hadler, MD, a professor of medicine and microbiology/immunology at the University of North Carolina at Chapel Hill. “It means the ability to pace the day and choose activities.”

Fortunately, there are an array of tools to control pain and lessen the burden on sore muscles.

Seat lift

If you have problems with your knee, raising the seat by a few inches can make all the difference. A seat lift assistant is a mechanical lifting cushion that you can take with you wherever you go.

There are dynamic sitting pads which are air filled cushions thought to aid active sitting and outstanding sitting comfort, while encouraging an upright posture. Specially designed pads, like the Sissel Sitfit Plus, make it easy to retrofit chairs for more comfortable seating. So what’s the verdict?

It’s been mostly praised by those suffering from various neuromuscular injuries including an Amazon customer who had a herniated disc. However, others have echoed the above opinion that durability may be an issue, and with a one year warranty, you may need to think a little more about investing in this.

On the upper-end of the scale is the Upeasy’s Power Seat and Seat Assist (electric and nonelectric, respectively), which will give you a gentle push on your way up. They can be used with pretty much any chair or sofa, and they’re portable. The lifting action is activated automatically as the user stands, lifting up to 70 per cent of their weight. Make sure to buy this product from a reputable dealer, some sellers using Amazon’s marketplace sell knock-off versions instead which break, according to a few reviewers.

Ergonomic Jar Opener

These specialised jar openers provide a strong, safe grip for opening many items and feature a comfortable, ergonomic handle. There are several options if you’re looking for help with opening jars or cans. Most of these provide four to eight different sizes of circles in a bid to release a variety of condiments.

 

I have a standard one from Amazon – and it’s not perfect. The grip needed just on the handle puts pressure on the hand itself, and if the jar is shut tight, it may not budge at all. However, I can swear on a jar popper – the Jarkey opens jars in a jiffy by simply releasing the vacuum.

Portable Grips

Securely installed grip bars are a must for getting safely in and out of the bathtub.

If you don’t always need the help, there are portable versions that you can install as needed – these are also useful for travel. It’s important to get a good quality one, as a less than adequate grip can actually be dangerous if the suction isn’t strong enough while you’re trying access your bathtub or get up from anywhere.

Double grip

Bridge Medical has been recommended as the company makes a single-grip bar, as well as a telescoping pivot-grip grab bar that can be installed at a variety of angles. There are several reviewers on Amazon however, who have complained that although it is well-made, the bar would not attach properly on to ceramic tiles or tubs hence becoming a liability. I’d suggest shopping around and checking out reviews to see if it’s worth the purchase.

Gripped Cutlery

When you have arthritic fingers, everyday tasks, such as eating your dinner, can be painful and difficult. You may need knives, forks and spoons with handles that are easy to grip and won’t slip out of your hands.

These eating utensils from Good Grips fit the bill with large, cushioned handles made of a rubber-like material. Each utensil has a metal shaft that can twist in any direction, making it easy to hold in a position that’s comfortable to you.

Electric Can Opener

These little beauties are fully automated – you don’t even have to hold the can. You place the gadget on a can, press a button, and the can opens. The integrated magnet lifts the lid off the can for easy disposal. Cans are cut around the sides to minimise sharp edges. Ideal for those with limited manual mobility.

Some products can be rather noisy, and there seems to be a whole host of other problems encountered by users including breaking on the first try, lids getting stuck, cutting on the outside of the can instead even the fact that it refuses to switch off.

According to BestReviews, the West Bend 77202 Electric Can Opener comes out on top with a powerful 70-watt motor, a locking/cutting mechanism, and an automatic shut-off feature.

Ring Pullers

Made especially for ring pull cans this specific can opener has a J shape that opens them easily. No more struggling to get your fingers under the tab, simply slip the tip under the tab and rock your hand back.

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It has a comfort grip non-slip handle, perfect for those with arthritis, and is dishwasher safe. You may have to be slightly dexterous to try and insert this under the ring pull.

Slow Cooker

Slow cookers can make meals so much faster and easier. You have the ability to drop in all the ingredients in, turn it on and leave it… and if you buy one with the warming feature then your meal is still hot when everyone’s hungry.

It is perfect for making large batches of sauces, chillis, soups, etc. and freezing them for when you’re having a chronic pain flare up and all you can manage is too pull something out of the freezer.

Don’t assume more expensive options are better. Sometimes you’re paying for fancy controls, more timer options and even auto-stir functions. Be sure that you genuinely need these if you fork out for them.

Think about the size of the pot – it’s no good buying a cheap and cheerful slow cooker that’s on offer if it only feeds two people and you have a large family. Generally speaking, a 1.5-3L slow cooker will feed one or two people; a machine that’s 3-5L will serve three or four people; and anything between 5-6.5L will feed five or more people. You can get bigger ones too for six or more people.

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Lakeland Slow Cooker is recommended by the BBC Good Food site. It’s lightweight with a nice ceramic pot that you can take to the table, and it feeds three to four people easily. There’s an auto-warm function for keeping food at serving temperature, and Lakeland will give you your money back if you aren’t happy.

So as you can see, there is a whole host of gadgets at your disposal – whether it works or not differs from person-to-person. Having tried a few of these myself, it can be hit and miss, but mostly a success.

Please feel free to comment below about gadgets that you feel are essential for your daily living.

Read: When Small Tasks become Daunting with HNPP

HNPP · Mental Health

HNPP, belief and the impact of misdiagnosis

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The cause of HNPP remains unknown. Only the fact that it is an inherited condition is recognised. According to many websites, it’s considered “painless” or the symptoms are “mild”, which is obviously not the case for many living with the disorder. It definitely makes it difficult when it comes to being believed let alone getting a correct diagnosis.

“Pain is whatever the experiencing person says it is, existing whenever the experiencing person says it does.”

Margo McCaffery, 1968

Living with Hereditary Neuropathy with Liability to Pressure Palsies can be challenging. You may experience pain and fatigue that interferes with daily activity. But yet your family, friends, and even your doctor may not understand your concerns. Also, some people may not think HNPP is a “real” condition and might believe symptoms are imagined.

It’s estimated that every 12.5 out of 100,000 people live with it but may not have been diagnosed. The condition can affect anyone at any age. But it is often misdiagnosed as either its sister disorder, Charcot Marie-tooth syndrome, Bell’s palsy among a huge list of other conditions including mental health issues.

But being wrongly diagnosed is not as uncommon as you think.

An estimated 12 million Americans a year are misdiagnosed with a condition they don’t have. In approximately half of those cases, the misdiagnosis has the potential to result in severe harm.

According to the Scientific American, writer Judy Stone says in cases where a condition is considered “rare”, in one survey, it took patients in the US an average of 7.6 years to be properly diagnosed, after visits to eight physicians. During that process, they received 2-3 misdiagnoses. In the UK, it was 5.6 years for a correct diagnosis.

So what impact does lack of belief have on sufferers?

In the 2008 study The Effects of Failing to Believe Patients’ Experience of Chronic Pain, the authors state: “Pain assessment depends on the patient’s self-report of pain and therefore accurate assessment cannot be achieved unless practitioners believe patients.” Taking a hermeneutic approach, where the methodology of the research is based on human experiences rather than quantitative analysis, patients were approached to record their daily life living with chronic pain.

The results showed that six out of the eight participants felt they were being stigmatised, while the other two, who did not have an issue with being believed both had a long-term physical disability.

“He (GP) was another one who blatantly said to me “I do not believe you are in pain” and he went to walk out the house – he wouldn’t give me pain relief”

“The Effects of Failing to Believe Patients’ Experience of Chronic Pain”, Clarke, K.A et al, 2008

As the authors also report that this could be due to the fact that they may have appeared more credible because they had a physical disability that made the pain appear more visible. The participants said that because they felt they weren’t being believed, it made them feel:

  • Like a burden
  • Alienated
  • Angry
  • Depressed
  • Suicidal
  • Not taken seriously

The report concluded that healthcare professionals “through attitude or actions such as withholding analgesia – demonstrate that they are not accepting patients’ reports of pain.”

It added that while each professional is entitled to their own opinion about the credibility of patients’ accounts but it is a professional responsibility to accept patients’ report and to help patients by adopting a positive and responsive manner.

What can happen with lack of belief?

The even bigger problem that this may pose is the fact that if a patient isn’t believed, it paves the way for doctors to incorrectly diagnosis or even completely ignore that there may be an underlying health concern.

This can be seen in the case of blogger, Nadia Tasher, where her doctors diagnosed her with anxiety, when in actual fact she had the rare chronic autoimmune condition Lupus. It took her 80 visits to a GP in the space of one year just to be diagnosed.

And there is a whole spectrum of conditions where sufferers appear to face issues with being believed and getting the right outcome. From Fibromyalgia, to chronic fatigue, unfortunately HNPP lists even further below this category of rare disorders. As seen in the report above, until medical practitioners get a little more clued about chronic pain, fatigue, illnesses as well as HNPP, the onus may have to fall upon the sufferer to push for answers.

What to ask a doctor if you feel they don’t believe you

Here are some tips to help you navigate doctors when they are sceptical of your pain.

  • Tell your entire relevant story – it’s important that you are able to tell the entire story about your pain. When did it start? What does it feel like? Do any activities make it worse? What makes it better?  If telling the story is difficult for you, write it down. If the doctor interrupts you as you are telling it, ask him or her nicely if you could continue before any questions, because it is important that you let them know everything that is going on with you. At the same time, keep the story succinct – the doctor doesn’t need to hear about unrelated events or conversations.
  • Medical records – if you have records from previous doctors, imaging (both the imaging itself and any reports from the radiologist), tests, or journals you have kept, definitely give them to the doctor to review. Some patients even send their records, journals, and questions ahead of time if the doctor is open to it.
  • Type of doctor – a general practitioner may not know very much about a specific type of pain. Always consider the source of any advice or opinion (medical professional or not).
  • Ask questions – if a doctor tells you he/she believes the pain is in your head, ask why they may think that. Sometimes at this point, the doctor will admit that they just aren’t sure how to help you. Ask for a referral. If they don’t know who to send you to, ask them if they have a colleague who might know. If he or she doesn’t have a colleague that knows, get on the internet and look for one right there with the doctor.
  • Searching for doctors – if you feel a doctor does not have your best interest at heart, then try and look for another one. It’s more than okay to look for a doctor who listens, helps you feel empowered, and treats your pain seriously. They are out there.
  • If all else fails – if you don’t have the option to change doctors, then agree to get a referral for a psychologist – they may actually be able to evaluate your pain correctly and then forward on the paperwork back to the doctor.
  • Believe in yourself – it’s very easy to become disheartened when you feel like no one is listening to you. Hence if needs be, practice what you are going to say, and take a list with you, so that you can effectively communicate with them. Another option is to take someone with you who sees the pain everyday and can explain it even better. Your doctor should be a partner in your health, not someone you fight with or against.

You might not be able to change some things that affect your doctor’s attention span, such as the shorter and shorter visit times that are common. But you can take steps to make sure your concerns are heard in the time you have.

Read: Patient-doctor relations and HNPP

HNPP · Physical Health

Can HNPP lead to bone-related issues?

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There appears to be a common trend of patients with HNPP having joint-related issues, which seems to go hand-in-hand with the fact that over-compensation on certain limbs, nerve damage, and issues with the feet eventually leads to problems with bones in general.

From osteoporosis to bone spur, entrapments of the nerves can cause a range of complications. Damage due to bone and joint problems at the elbow can be exacerbated by chronic pressure on the elbow and full elbow flexion and vice versa.

Disclaimer: Please ask your medical practitioner or occupational therapist for more information. This article is based on various research, journals and testimonies.

Why does it happen?

Compression of the nerves is an inability to transmit nerve impulses because compression has damaged nerve fibres either directly, or indirectly by restricting their supply of oxygen.

According to the 2000 study Is Bone a Target-Tissue for the Nervous System?, the authors state that there is strong evidence to suggest that bone can be a “target” of the nervous system.

The authors describe: “it seems reasonable that neural control could also apply to bone tissue, and several clinical and experimental observations support this concept, including Charcot’s neuropathy”.

“The distribution of different nerves during bone formation, combined with the observed effects of transmitters on bone metabolism in vitro, suggest that there is neuroendocrine regulation of bone physiology.”

“Is Bone a Target-Tissue for the Nervous System?” – García-Castellano, J. et al, Nov. 2014

Although there are few nerve fibres in bone, their presence may represent sophisticated and specialised regulatory elements able to deliver time- and site-specific stimuli according to demand.  This suggests that the peripheral nervous system is critically involved in bone metabolism, osteogenesis, and bone remodelling through nerve fibres. Various cells of the musculoskeletal system have receptors for sensory and sympathetic neurotransmitters.

This can be seen in the research Neurogenic Arthropathy and Recurring Fractures with Subclinical Inherited Neuropathy. Neurogenic Arthropathy, common in the hereditary condition Charcot Marie-tooth syndrome, is where due to damaged pain perception and position sense, the bones in the foot can rapidly degenerate. The authors say that patients with neuropathic arthropathy also suffer from recurrent long bone fractures owing to underlying sensory neuropathy.

With fractures, the researchers of the 2000 study for the Iowa Orthopaedic Journal explain: “This difference in healing may imply that in fractures with an abnormal nerve supply the sensory innervation does not recognize anomalous movement of the fracture and, with unstable fixation, nerves may mediate signals that lead to altered bone healing.”

They also suggest that with a neurectomy (surgical removal of all or some of the nerve), bones were less likely to grow back adequately, instead there is a decrease in bone mass.

How can nerves become compressed?

Compression can come from herniated discs in the spine, osteoarthritis can cause bone spurs that can compress a nerve, severe muscle injuries can compress nerves, and even prolonged use of tight clothing such as shoes or skinny jeans. It all depends on the nerve compressed.

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This type of pathology produces pain called radicular pain or nerve root pain leading to pain that may radiate to other parts of the body, such as from the low back down the leg or from the neck down the arm. Leg pain from a pinched nerve is usually described as sciatica.

With a herniated disc most compressed nerves will cause inflammation. This is likely to cause problems when the nerve is squashed between the disc and an adjacent bone.

What happens with injuries?

Traumatic injuries caused by accidents for example, can cause nerves to be partially or completely severed, crushed, compressed, or stretched, sometimes so forcefully that they are partially or completely detached from the spinal cord. Broken or dislocated bones can exert damaging pressure on neighbouring nerves, and slipped disks between vertebrae can compress nerve fibres where they emerge from the spinal cord.

What are the different types of musculoskeletal pain?

Musculoskeletal pain has varying symptoms and causes. Some of the more common types of pain include:

  • Bone pain: This is usually deep, penetrating, or dull. It most commonly results from injury. It is important to be sure that the pain is not related to a fracture or tumour.
  • Muscle pain: This is often less intense than bone pain, but it can still be debilitating. Muscle pain can be caused by an injury, an autoimmune reaction, loss of blood flow to the muscle, infection, or a tumour. The pain can also include muscle spasms and cramps.
  • Tendon and ligament pain: Pains in the tendons or ligaments are often caused by injuries, including sprains. This type of musculoskeletal pain often becomes worse when the affected area is stretched or moved.
  • Fibromyalgia: This is a condition that may cause pain in the muscles, tendons, or ligaments. The pain is usually in multiple locations and can be difficult to describe. Fibromyalgia is usually accompanied by other symptoms.
  • Joint pain: Joint injuries and diseases usually produce a stiff, aching, “arthritic” pain. The pain may range from mild to severe and worsens when moving the joint. The joints may also swell. Joint inflammation (arthritis) is a common cause of pain.
  • “Tunnel” syndromes: This refers to musculoskeletal disorders that cause pain due to nerve compression. The disorders include carpal tunnel syndrome, cubital tunnel syndrome, and tarsal tunnel syndrome. The pain tends to spread along the path supplied by the nerve and may feel like burning. These disorders are often caused by overuse.

How is musculoskeletal pain diagnosed?

Your doctor will begin by conducting a thorough medical history. They will then look for possible causes of your pain, and will also ask if the pain is ongoing or acute.

The doctor will then conduct a hands-on examination looking for the source of the pain. This may include palpating the affected area. This helps him or her locate the origin of the pain. However, to determine the underlying cause of the pain, the doctor will often follow the exam with laboratory tests and X-rays.

How is musculoskeletal pain treated?

Different types of physical therapy, or mobilisation, can be used to treat people with spinal alignment problems.

Medications such as nonsteroidal anti-inflammatories (NSAIDs) may be used to treat inflammation or pain.

In patients with musculoskeletal disorders such as fibromyalgia, medications to increase the body’s level of serotonin and norepinephrine (neurotransmitters that modulate sleep, pain, and immune system function) may be prescribed in low doses.

As if HNPP is not enough to deal with, it seems that there is a corresponding issue of joint problems. In this case, you may have to be extra careful with certain activities that may aggravate both issues, such as walking where it may not only cause numbness in the legs, but actual pain on the bone itself. Keeping vigilant is going to be absolutely vital as a result.

Read: Coping with multiple conditions including HNPP

HNPP · Medication · Physical Health

What to avoid with HNPP

Taxol
Rotation of the model of the Paclitaxel molecule – Andrew Ryzhkov

An interesting discussion emerged on one of the HNPP groups about how certain products can actually have a reverse effect on the nerves. While there are hundreds of results when it comes to what to eat or how to help neuropathic symptoms, it’s rather a different situation when you attempt to search foods, supplements or drugs to be cautious about. So what should we keep an eye on?

Disclaimer: Please ask your medical practitioner or occupational therapist for more information. This article is based on various research, journals and testimonies.

Vitamin C

This a hot topic of debate with some saying that high doses of vitamin C can actually reduce the amount of PMP22 produced, while others saying they have seen some benefits through increased energy. So what’s the deal?

While there is little research to show the effects of vitamin C on HNPP specifically, there has been studies revealing how it affects the inherited condition Charcot Marie-tooth syndrome.

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In the 2004 study Ascorbic Acid Treatment Corrects the Phenotype of a Mouse Model of Charcot-Marie-Tooth Disease, the authors claimed to see an improvement from ascorbic acid (vitamin C). They say: “Ascorbic acid treatment resulted in substantial amelioration of the CMT-1A phenotype, and reduced the expression of PMP22 to a level below what is necessary to induce the disease phenotype.

“As ascorbic acid has already been approved by the FDA [Federal Drug Administration] for other clinical indications, it offers an immediate therapeutic possibility for patients with the disease.”

While the authors saw benefits by reducing the expression of PMP22, it may be difficult to say whether this will have a reverse effect for those with HNPP, which consists of already having a deletion or defection of one of two of these genes.

On the other hand, a study of 277 persons with CMT1A found no significant effect of a daily 1.5-g dose of ascorbic acid after two years. The researchers of the 2011 study Ascorbic Acid in Charcot–Marie–Tooth disease type 1A states: “With respect to the size of the effect of ascorbic acid, if the effect is so small that only a biomarker can detect it, it would be unlikely to be clinically significant in a short-term study but might be important in the long term.

“Findings of this study suggest that ascorbic acid is not efficacious in adults with CMT1A.”

As with any supplements, it’s important not to take an excessive amount. As Thomas Bird, MD, writing for GeneReviews, a genetic resource page for clinicians, states: “No specific treatment for the underlying genetic or biochemical defect exists and no special diet or vitamin regimen is known to alter the natural course of HNPP.”

Always check with your medical practitioner about possible effects and let them know about your condition.

Vincristine 

Vincristine is a known chemotherapy drug used to treat several types of cancer including acute leukaemia, malignant lymphomas and carcinomas. If you’re suffering from multiple illnesses, this is where it gets complicated.

Vincristine_3D
Vincristine 3D structure

The medication is said to be potentially “toxic” to those with peripheral neuropathy due to its side effects that resemble the same symptoms. These can include numbness, pin prick or a tingling sensation to full-blown motor difficulties and neuritic pain.

In one study, 61 per cent of the 23 patients treated for lymphoma developed neuropathy, while only 14 per cent of the 37 patients with other malignant diseases developed these symptoms.

There has also been a case report of a patient with a familial variant of Charcot-Marie-Tooth syndrome. After receiving two 2mg dosages, his weakness secondary to peripheral neuropathy rapidly progressed to complete paraplegia. Acute acoustic nerve palsy has also been reported.

Hence letting your medical team know about the condition that you have and how you are affected can help avert a crisis such as this.

Taxol

Paclitaxel, sold under the brand name Taxol among others, is a chemotherapy medication used to treat a number of types of cancer. This includes ovarian cancer, breast cancer, lung cancer, Kaposi sarcoma, cervical cancer, and pancreatic cancer.

toxic
“Toxic Neuropathies” – Kelley’s Essentials of Internal Medicine, H. David Humes, 2001

Drugs and medications such as taxol that are known to cause nerve damage should be avoided, says Vinay Chaudry, MD, in a 2003 study Toxic Neuropathy in Patients With Pre-Existing Neuropathy. Chaudry says that six patients with pre-existing neuropathy, who received “non-toxic” dosages of known neurotoxic agents including taxol, had significantly worsened. He concludes: “functionally disabling toxic neuropathy can occur in patients with pre-existing neuropathy at standard doses.”

The Charcot-Marie-Tooth Association has maintained a “Medical Alert” list of potentially neurotoxic medications. They define taxol as a “definite high risk” to those with the condition even if the individual may not present any symptoms.

Fluoroquinolones

The fluoroquinolones are a family of broad spectrum, systemic antibacterial agents that have been used widely as therapy of respiratory and urinary tract infections. It is an antibiotic used to treat some infectious or even common diseases.

However, in 2016, the U.S. Food and Drug Administration advised that the serious side effects associated with fluoroquinolone antibacterial drugs generally outweigh the benefits for patients with neuropathic issues.

“The peripheral neuropathy reported with fluoroquinolone administration can be severe, debilitating, and permanent.”

A Case Report on a Rare but Serious Debilitating Side-Effect of Fluoroquinolone Administration – Jacquelyn K. Francis and Elizabeth Higgins, MD, 2014.

In a statement, the FDA said: “An FDA safety review has shown that fluoroquinolones when used systemically (i.e. tablets, capsules, and injectable) are associated with disabling and potentially permanent serious side effects that can occur together. These side effects can involve the tendons, muscles, joints, nerves, and central nervous system.”

In one 2014 case report for the Journal of Investigative Medicine, a 57-year-old woman was treated for a urinary tract infection with a ciprofloxacin, an antibiotic under the fluoroquinolones umbrella. She had already been in remission for 12 years after suffering from trigeminal neuralgia. Two years after the initial onset of symptoms, she continued to suffer from polyneuropathies chronologically related to ciprofloxacin use.

As the report concludes: “the peripheral neuropathy reported with fluoroquinolone administration can be severe, debilitating, and permanent. It is for this reason that physicians need to practice due diligence when prescribing not only antibiotics, but any drug.”

While it’s important to understand how the wrong drugs can interact with hereditary neuropathy, it’s even more essential to talk about it with the right people. Letting health professionals know about your current situation will allow them to prescribe the right medication. As one HNPP’er says because there’s an assumption that something is safe, doesn’t mean it’s safe for us.

HNPP · Medication · Physical Health

Coping with multiple conditions including HNPP

multiple chronic multimorbidity hnpp hereditary neuropathy

Many people living with HNPP have the arduous task of coping with more than one condition on top of the neuropathic symptoms. From dystonia and asthma to peripheral oedema, or mental health conditions, those with HNPP battle a range of conditions. So how do you deal with multiple diagnoses?

Coping with multiple issues can be a bit overwhelming, and it adds a layer of challenge that might not be present for the friend of a friend who has one of your illnesses in common, but runs 5k races.

“In people with multiple chronic conditions, physical and emotional symptoms can compound and build off of each other, resulting in a larger negative effect on their daily lives.”

“Challenges of self-management when living with multiple chronic conditions”, Clare Liddy, 2014

More than one in four Americans have multiple (two or more) concurrent chronic conditions (MCC), according to the U.S. Department of Health & Human Services. As a result, people with several different illnesses tend to have poorer day-to-day functioning. In England, UK, the figure is said to be about 2.9 million people with multiple long-term conditions and the number is thought to be rising.

A 2012 study in the British Medical Journal says that doctors are still unprepared with dealing with ‘multimorbidity’ – that is, the coexistence of multiple chronic diseases and medical conditions in one individual. The authors say: “Despite the increasing numbers of patients with multimorbidity, evidence on the effectiveness of interventions to improve outcomes in such patients is limited.

“The clinical care of these patients is complex and the evidence base for managing chronic conditions is based largely on trials of interventions for single conditions, which too often exclude patients with multimorbidity.”

The impact of multimorbidity

And research shows that multimorbidity has an additional impact on those individuals including emotional challenges of dealing with a group of chronic conditions. Clare Liddy, MD, an Associate Professor in the Department of Family Medicine at the University of Ottawa in Ontario, says “In people with multiple chronic conditions, physical and emotional symptoms can compound and build off of each other, resulting in a larger negative effect on their daily lives.

“These symptoms are interdependent and symptoms of one condition can be aggravated by the symptoms, treatment, or medications of another condition. Some symptoms might overshadow others and reduce the patient’s ability to manage his or her care.”

Liddy suggests ‘re-prioritising’ to learn to cope with the negative effects of the various illnesses.

  • Changing cognitive approaches – patients with multiple conditions found that changing their thinking or conscious mental processes had a positive effect on them. Living with multiple chronic conditions became a way of life for some people, who reported fluctuating between “living a life and living an illness.” Liddy notes the current changes in those with multimorbidity:
    • reframing and regulating the amount of attention given to their situation
    • engaging in life and body listening
    • relinquishing control to another source – faith and doctors seem to be heavily relied upon
    • changing their beliefs (for example assigning new meanings to daily chores or activities)
    • self-monitoring – keeping an eye for any changes
    • self-advocacy – approaching and asking for help whenever necessary.

Other important tasks to note include:

  • Social support – an important part of dealing with comorbidity is to have a support network of some sort. However, if you have the incorrect kind of support, friends and family may become a barrier to self-management, and they may end up interfering where unnecessary. The key is to create clear-cut boundaries and let them know how and when they can help you. Group activities such as walking have been shown to help with psychological issues, such as loneliness and depression. Joining a support group can also help.
  • Read about your condition extensively – the better equipped you will be when approaching your healthcare providers if you know what to expect. At times, you may find practitioners giving you contradictory information. Note it down, and then approach them carefully with what you have observed.
  • Multiple care approach – you may detect that some medical practitioners are still not completely skilled at dealing with multiple conditions, they may prioritise one condition over the other, so it’s important to keep that in mind and see that they can also deal with the other issue(s) in a similar manner. The best chance of this is to have a multidisciplinary team working with your needs.
  • Organise your medication – if you have lots of medicines to take, it can be hard to keep track. Some people find a dosette box or pill organiser (a plastic box which is separated into different compartments for each day and each time of day) helpful. You can get these from pharmacies or buy them online. Usually you would fill these once a week – ask someone to help you if necessary. Or you could try making a daily chart to show when you should take each medication. Or you could label your medication containers with the time you should take them, or keep medication where you are likely to take it at the time – for example, put breakfast tablets in the kitchen, and bed time pills on your bedside table. If you can’t manage with taking 18 tablets a day, it may be worth revisiting your doctor and being honest about it.
  • Take it day-to-day – this means prioritising your needs on any given day, for example, if you feel more tired due to one of your illnesses, then rest, or if you’re feeling depressed more so than usual, then address those needs first.

It’s unbelievably difficult to cope with one let alone several chronic illnesses. And it’s exacerbated by the fact that doctors are only just coming to see how big an impact this is having on society as a whole. However, thinking of it as a list of things to keep crossing off, over time with good care, more coping skills, better management of medication changes, surgeries and therapies, it will seem that tiny bit easier.

HNPP · Profiles

Profile: Jessica Kellgren-Fozard on living with an ‘invisible disability’

“I received a lot of online hate based on how I am not visibly disabled,” says TV presenter Jessica Kellgren-Fozard. Jessica, 28, from Brighton, UK, is one of the only known people with Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) to be in the public eye.

As the presenter of Latest TV, a Brighton-based TV station and a former contestant on BBC’s Britain’s Top Missing Model, keeping up appearances can be tough, especially with a hidden illness.

“When I was working for a TV station in Brighton I received messages on how I wasn’t disabled enough. And that was it, I decided to put myself out more and talk more about life with invisible disabilities in order to help people like me,” says the model and writer.

“I received a lot of online hate based on how I am not visibly disabled, and supposedly not disabled enough.”

At a young age, Jessica began showing symptoms of HNPP, including extreme palsy in her arms as well as patches of skin being numb, difficulty gripping things, weakness in limbs, as well as fatigue – common symptoms of this inherited condition. “As a child I thought it was normal to not feel the chins in my knees,” she says, after being eventually diagnosed at the age of 17 with the disorder.

She adds: “My reaction was one of relief because I finally had a name for what had been happening to me, and also for why it was happening though my childhood.”

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This is me eight years ago, this is me this year and here are some things you might not know about me… I missed most of my teenage milestones because I was either in hospital or lying in the dark. I was too ill to open my eyes, to sit up or to eat. I missed exams, parties and the friendships I lost. I was made to feel like a burden, like a problem, as if my presence was a hindrance to other people's lives: "how good you are to look after her"… I'm still in the room. But there were some amazing people who stepped in to my lonely space, took my hand and helped me through those rough years. They didn't mind helping out physically or spending time making me laugh and always let me know it was no big deal- I was never a burden to them. That was the best gift they could have given me. That was love. All of this is why I'm so angry when media like #MeBeforeYou comes along! It's a dangerous thing to tell people that the only way you can thank those who have helped you is by removing yourself from their lives! I'm so much better than I was but still need help and I know the best way to repay that help is to live the best life I can; to be brave, to be bright, to be happy! I'm proud of everything I have achieved and where I am now. You can watch my latest vlog for more: https://youtu.be/nE8CESB0NeI Love to everyone still in that place ❤️ #MeBeforeAbleism #LiveBoldly #DisabledandProud #disability #ableism #chronicallyill #beforeandafter #mylifeisworthy #spoonie #disabilityawareness #wheelchair #bestfriend #nowandthen #instagood #instadaily #bichon #deaf #ootdsocialclub #happiness #love #vintage #retro #pinupgirl #glamour #iger #youtuber #celebratedontdiscriminate #chronicallyfabulous

A post shared by Jessica Kellgren-Fozard (@jessicaoutofthecloset) on

The condition affects one or more of the peripheral nerves, which connect the brain and spinal cord to the muscles and to the sensory cells that detect touch, pain and temperature. One copy of the gene PMP22 is lost, causing the protective outer layer of each nerve (myelin) to become fragile and more susceptible to injury.

On top of having HNPP, Jessica battles with Mixed Connective Tissue Disorder (MCTD), an autoimmune disease in which the body’s defence system attacks itself. MCTD causes chronic joint pain, muscle inflammation, hypermobility, pulmonary hypertension alongside a huge list of other symptoms.

And owing to weak connective tissue and damage to the nerves, Jessica is also deaf. Hence, fighting these conditions while attempting to live a relatively steady life poses its own issues.

“I avoid sitting, standing or laying on the same position for too long,” she says.

After recovering from a long bout of illness, the then 19-year-old Jessica applied to an advert looking for people to take part in a BBC TV show about people with disabilities, specifically focusing on young women who were interested in fashion. “It ended up being a six part series about disabled girls becoming models.”

The show, Britain’s Top Missing Model, featured an array of young hopefuls with various disabilities from across the globe. Sadly, Jessica received a torrent of online abuse for her invisible illness. She says: “Afterwards I received a lot of online hate based on how I am not visibly disabled, and supposedly not disabled enough.”

Watch more here.

The stigma surrounding hidden illnesses spurred Jessica on to continue keeping up a public profile so that others can speak out about their own experiences about invisible disabilities.

“I did struggle in school and university, because I felt they weren’t prepared even if they were willing to help. There was just not enough funding or education on how to properly help students with a disability.”

Thankfully, Jessica is backed by her loving wife, Claudia, her two adorable dogs, as well as her parents who have been an important part of her support network. “My wife lovely rolls me through the night,” says Jessica, who married her partner last year.

Managing the condition is critical to how Jessica functions, as she recommends listening to your body for the correct answers. She asserts: “[Your body will] tell you when you rest and when to go for it and do all those things you want to do. Don’t overwork yourself, that’s very important. I also take a lot of prescribed medication, so talk to your doctor if you are doing to pain daily.”

So what next for the feisty fashionista? Jessica says she plans to continue spreading the word: “I am going to carry on with my YouTube channel and my writing. Just generally be there and support and inspiration for people going through something and need someone to look up to.”

Find out more about Jessica’s journey at Jessica Out Of The Closet.

Follow Jessica’s YouTube channel here: Jessica Kellgren-Fozard.

HNPP · Physical Health

Is walking good for those with HNPP?

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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.

calf stretch

  • 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.

lower calf

  • 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.