HNPP · Physical Health

Do HNPP sufferers get brain fog?

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You walk into a room, knowing you’re there for a reason, but within moments you’ve forgotten why you’re there. Some sufferers of HNPP claim to suffer from forgetfulness, memory blanks, and being in a haze. I write this completely woozy, the computer screen and mobile phone a complete blur, so it will be less detailed than usual.

“Brain fog” isn’t a medical condition in itself, rather a symptom of an underlying health issue or even a side effect from a medication. It can affect your ability to think and you may feel confused or disorganised or find it hard to focus or put your thoughts into words. It can be fleeting, but for others dealing with multiple chronic conditions, it tends to last longer.

While some individuals with HNPP may face this, it’s still questionable whether it is a direct symptom of this inherited disorder.

Watch HNPP sufferer Jessica Kellgren-Fozard speak about her experiences of brain fog:

Asked if memory problems such as forgetting words or conversations, as well as losing your train of thought are common occurrences with HNPP, Gareth Parry, M.D, appears to disagree.

According to HNPP.info, the Professor and Head, Department of Neurology, University of Minnesota, says: “I am not aware of forgetfulness being a problem with HNPP patients other than related to drugs. One of the commonest causes of forgetfulness in young people is distractibility. For example, when one is anxious about an exam or some such life crisis, one tends to forget the less pressing things associated with every day life.”

“It is a possibility, but I suppose there may also be some direct effect on the brain although the protein for which the gene encodes is not a CNS protein.”

– Gareth J. Parry, M.D

While he doesn’t completely discount it, he adds: “I frequently see patients with serious illnesses have major problems with memory. If HNPP is creating significant problems perhaps it is distracting the victim from their everyday activities. It is a possibility, but I suppose there may also be some direct effect on the brain although the protein for which the gene encodes is not a CNS [central nervous system] protein.”

Cognitive fog may not be a direct symptom of HNPP, however, a 2013 study reports that some with either a duplication or deletion of the PMP22 gene, which is a fundamental tenet of the condition, had cognitive impairment.

Authors of the report Central Nervous System Abnormalities in Patients with PMP22 Gene Mutations said: “We found a decrease in the volume of WM [white matter] in 70% of patients, a reduced creatine level in WM in 28% and a cognitive impairment in 70%.”

White matter makes up half the human brain and has only recently been linked to cognition, the mental action or process of acquiring knowledge and understanding through thought, experience, and the senses. Research suggests that “white matter tracts mediate the essential connectivity by which human behaviour is organised, working in concert with grey matter to enable the extraordinary repertoire of human cognitive capacities.”

White matter HNPP hereditary neuropathy

Writing in the Journal of Neurology, Neurosurgery, and Psychiatry, researchers of the above 2013 study say that the results showed that 47 per cent of the patients with HNPP and those with CMT1A had “abnormal” levels of white matter volume, creatine level in white matter as well as cognitive testing.

They conclude: “The study demonstrates that altered PMP22 gene expression induces significant CNS alterations in patients with HNPP and CMT1A, including cerebral WM abnormalities and cognitive impairment.”

In the 2013 edition of Nervous System Diseases: New Insights for the Healthcare Professional, reporters from VerticalNews based in Strasbourg, France, reiterates the research, quoting the authors above: “Mutations of the peripheral myelin protein-22 (PMP22) gene are the most common cause of inherited diseases of the peripheral nervous system (PNS), with its deletion resulting in hereditary neuropathy with liability to pressure palsies (HNPP), and its duplication inducing Charcot Marie-Tooth 1A (CMT1A) diseases.

“Although mainly expressed in the PNS, PMP22 mRNA and protein are also present in the central nervous system (CNS).”

With only 30 participants tested in total, further research may be required for more definitive results.

What other causes could be a factor?

Medications

Some drugs – either prescribed or over the counter – can cause brain fog. If you take medicine and notice that your thinking isn’t as clear as it should be or you suddenly can’t remember things, it might be worth asking your medical practitioner if there is an alternative or if the side effects will pass.

Chronic Fatigue

An obvious part of feeling ‘foggy’ is dealing with chronic fatigue. With chronic fatigue, your body and mind are tired for a long time. You may feel confused, forgetful, and unable to focus.

Sleep

You need sleep to help your brain work the way it should, but too much can make you feel foggy, too. Aim for 7 to 9 hours.

Blogger Mark from Developing The Human Brain recommends allowing your body to wake up slowly and begin to move slowly. He adds: “Sit up in your bed for at least 12 sec after you wake up for stabilizing your blood flow.”

Dehydration

If you’re even the slightest bit dehydrated, it’s impossible to function at your full capacity. Every single cell in your body needs water, including those in your brain and your muscles. As the brain and heart is made up of 73 per cent water, it is the first organs to show signs of dehydration. Mild dehydration may affect your ability to take on mental tasks and cause you to feel foggy headed, according to a study from the British Journal of Nutrition.

Tips to reduce brain fog
  • Sleep – get enough sleep at night and stick to a reasonable bedtime. Also, find ways to manage mental and emotional stressors, such as conscious breathing or purposeful exercise.
  • Take note – either write down anything you need to do or create reminders and lists. Whether using post-it notes, a notebook, calendars or whatever works for you personally, all of these things help keep a record of what you need to remember.
  • Ask for help – if you have people around you, ask them to remind you of anything important.
  • Mobile apps – there are a whole host of mobile apps that can help you plan ahead including:
    • Cozi – marketed as a “family app”, you can keep everyone’s activities and appointments; as well as create and share shopping lists, chores.
    • MedCoach – medical app that helps you remember to take your medications and pills at the right time and day.
    • Evernote – you can capture, organise, and share notes from anywhere including taking pictures, websites, and making voice notes. It can be synced as well.
    •  Todoist – like Evernote – automatically syncs wherever you log in, which is useful for those moments of clarity when you need to write something down quickly.
  • Vitamin D – increased exposure to sunlight may help you to think more clearly. “The beauty of nature will give your prefrontal cortex – the area of your brain that helps you focus – an opportunity to recharge,” says neurologist Marie Palinski, at Massachusetts General Hospital.
  • Flare-up tool kit – just like with chronic pain, you may need to put everything you need to help you make it through your flare in the same place/in a flare-up tool-kit box.
  • Engage yourself – reading a book, seeing a play, or working a crossword puzzle or word game challenges your mind and stimulates your brain and your memory.  Dr Palinski, also a faculty member at Harvard Medical School and author of Beautiful Brain, Beautiful You says, “When stuck in a rut, we’re constantly treading the same brain pathways. Engaging in a new activity literally wakes up our brains.” That’s because the brain has to lay new neural pathways to process new information. At the same time too much stimulation can be overwhelming.
  • Postpone engagements – when you’re too tired and full of fog to think, put things off until the next day and get extra rest instead. Listen to the needs and signals your body gives. Use the presence of brain fog as a signal to slow down.
  • Manage your environment – move to a quiet place to minimise distractions when you are trying to concentrate. Reducing clutter in your living space helps you to be more organised, and remember where things are. Create a daily routine for yourself. Sticking to a routine may help you remember what tasks you have to do each day, and in what order to do them.
  • Relaxation techniques – relaxing activities such as yoga, tai chi and meditation can improve problems with sleep, fatigue, poor memory and anxiety – all of which are linked to brain fog, according to many studies.

While not everyone suffers from memory issues or general forgetfulness, it’s good to be prepared for any circumstance when you end up in a daze.

Read: When small tasks become daunting with HNPP

HNPP · Physical Health

When small tasks become daunting with HNPP

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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.

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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.

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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

Things you shouldn’t say to those with HNPP

HNPP hereditary neuropathy illness what not to say

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 to combat fatigue with HNPP

Between you and me I find fatigue to be one of the most common occurrences during daily activities. Even travelling to and from places can take its toll. So is there something that we can do to help control it?

Fatigue is unfortunately central to many neuropathies, but the causes can widely vary. It takes far more energy to walk, stand, balance and generally do normal, everyday things. Muscles have to constantly compensate for other areas having to do jobs they were not designed to do. 

According to Dr. Scott Berman, in his book Coping with Peripheral Neuropathy, a study showed fatigue in autoimmune neuropathy 80 per cent of 113 patients had severe fatigue. And that fatigue was independent of motor or sensory symptoms, being rated as one of the top three most disabling symptoms. Some medications also add to the endless tiring effect which can be unhelpful. 

DISCLAIMER: I am not an expert, everything written is based on personal and other’s testimonies, available journals and research.

So how do you manage it?

CMT UK, an organisation that deals with the sister condition of HNPP, Charcot Marie Tooth disorder, has made a few suggestions in order to counteract some of the lethargy.

  • Eat regular meals and healthy snacks – it is advised to eat every three to four hours instead of eating large meals with longer gaps in between.
  • Regular exercise – this might feel impossible when you’re feeling tired but it is supposed to help in the long run. A single 15-minute walk, (though many will find this difficult) or any other gentle exercise, can give you an energy boost, and the benefits increase with more frequent physical activity. Just start with a small amount and build up your physical activity incrementally over weeks and months until you feel comfortable and it doesn’t cause you any additional discomfort. 
  • Sleeping well – Two thirds of people are said to suffer from sleep issues when they have neuropathy problems. The Royal College of Psychiatrists suggests sticking to a routine of sleep every day, so getting up and going to sleep at same time daily. They also advise avoiding napping and if it helps, taking a hot bath before sleep.
  • Drinking lots of water – this is important for so many reasons. You may be dehydrated, or your medication requires a lot of H2O.
  • Weight management – excess weight can put a strain on your heart which can make you feel exhausted. Eating healthily and balanced meals as well as light exercise can help combat this.
  • Stress relief – relaxing activities can help relieve a bit of the stress which helps improve your energy levels. Even reading a book or listening to music can help take your mind off things.
  • Therapy – counselling or cognitive behavioural therapy (CBT) might help to fight fatigue. Talking about it may be a factor.
  • No caffeine – caffeinated drinks such as coffee and tea as well as fizzy drinks can potentially play havoc on your nerves. The RCP says caffeine hangs around in your body for hours afterwards. The guidance is to stop drinking tea or coffee by mid-afternoon. If you want a hot drink in the evening, try something milky or herbal. I find decaffeinated tea helps as a placebo!
  • Meals before bedtime – don’t eat or drink a lot late at night. Try to have your supper early in the evening rather than late.
  • Reduce alcohol intake – it may help you fall asleep at first but it’s likely to keep you up during the night and if you do sleep it won’t necessarily be deep. It can also make you feel tired in the morning.
  • The problems with sleep medication – there’s a chance it may interact with your current medication. It can also be addictive and eventually you may be required to take a higher dosage.
  • Muscle issues – speak to your doctor about any deficiencies that may make your muscles spasm more during the night.

    Sometimes fatigue can get the best of us. I say this as I head for a lie down.