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  Life with HNPP

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The following comments have been made by people with HNPP, to describe what their lives are like. Some of these individuals work full time, some part time and some not at all. Most of these people have almost daily symptoms because of their HNPP.

 

The Diagnosis:

Before the Diagnosis

I have had symptoms for years, but did not know what was wrong.

I went through years of testing and guessing...you know, doctors and tests and still no answers to what was wrong. First they thought it would go away and then that maybe I had MS, then finally HNPP.

I underwent a lot of tests to find out what it was that caused the numbness and pain and progressive loss of dexterity, especially in my hands. I was rather scared at the time and unable to work.

I started complaining to my G.P. about general pain (like a deep bruise) in my right hand, which would get worse after a long day at my PC, mousing...immediate suspect was Carpal Tunnel. I was sent for an EMG and the neurologist and technician thought their equipment was faulty after the crappy results.

Getting tested - "Everybody come look"

They drag in all the students to look at me and watch my lack of reflexes. They nod and either smile or frown in concern. They took turns doing EMG's on me.

Over the past two years, I have had more tests where they yell "everybody come look"...all the med students are amazed...then you go to another doctor and he says he can not believe those results and wants to do his own...more needles and shocks...then he come to the same conclusion...and the cycle continues until you say enough.

 

Getting the Diagnosis

It was a relief to find out what was wrong.

I think when I found out, it was half a relief and half fear, sorrow and grief. I had been having problems for so long, it was a relief to find out that something was really wrong and I wasn't crazy. I believe there were people who thought I was faking it.

I guess I was somewhat relieved when I heard, but no one had explained it to me then.

 

Doctors

Some Success stories...

I finally went to our state University. It was a relief to finally have someone who actually knew about HNPP. It's funny, but I felt safe. Maybe because I didn't have to educate the doctor, the doctor educated me.

Last year I was referred to a great neurologist. ..He was genuinely interested and concerned. He felt that I might have CMT and encouraged me to do a bit of research (medical and family tree) and get back to him.

I go to my family doctor once a month and he is very supportive and willing to read anything I bring over. He even calls e if I miss an appointment just to make sure I'm ok.

...and some not so successful.

My local GP sent me to a specialist. Everybody thought that was the answer...they did the DNA test looking for CMT and found HNPP...but that's all I can say for that experience..we went 3 times...he didn't want to hear about how I felt, he just wanted to tell me what I had, then he was through with me.
 
He said I am the only one with HNPP that he has seen in his years as a doctor...
 
I have chronic pain in my right shoulder and arm pit...my back has started really hurting a lot lately. But the doctors act like this will not affect that...they said only the lower extremities...they act like I'm crazy when I tell the my shoulder and back hurts...I feel like I am isolated...doctors in my area have never heard of this before.
 
I think they have a lot to learn about us. They are just beginning and I think we need to tell them - sometimes over and over again - about our symptoms. It took 1 1/2 years for my doctor to figure out that HNPP hurts.
 
At my last visit to the neurologist, he told me that HNPP was painless, that it was just a numbing type thing. When my husband and I left, I just wanted to cry. I told my husband that I know I had HNPP from the DNA, but there must be something wrong with me elsewhere - cancer or something, - because of the pain. I knew I wasn't crazy, but I felt like the doctor thought I was...
 
I asked about the pain in my hands and legs and why it is getting worse, and he said "well, there is nothing I can do about it. Just live with it!"
 
My doctor told me that HNPP didn't hurt and that I had pain from a slightly bulged disk. Well I just don't think so. He also said that if my pain was from HNPP, they would have to change the text books.
 
The neurologist was so uninterested that all he wanted was for me to come back one year later. I did and my hands were weaker and the EMG was worse. all he could offer me was - "Well, some people just have undiagnosed neuropathy. Yours is a 'small fiber peripheral neuropathy." You're obviously a heavy drinker." ....I could have screamed at the man.
 
Well a few years ago, before I knew what was wrong with me, I went to a doctor because my left wrist and hand wouldn't work. It just hung there and I could not use it. My doctor asked me if I had been out drinking and leaned on the bar counter too long...I think about it now and it makes me mad that he didn't see it as a sign of something wrong and check further.
 
If they are not making you feel that it's all in your head, they make you feel stupid and like you are making up your symptoms....
 
They accused me of being an alcoholic too. My GP and the local neurologist.
 
It's kind of funny about the drinking thing because my neurologist asked me if I was an alcoholic after the first round on EMG's!
 
My neuro gave me it's all in my head diagnosis once.
 
Once I brought her a news letter to show her that other people were experiencing pain also. She said she wasn't interested in something people just made up.

 

Lack of Information about HNPP

We got the computer to get on line and find out about HNPP

I had to do a lot of research on my own to get any information.

It took months for us to find out anything about HNPP...nobody knew and I went every where to public libraries, etc.

The whole thing is so frustrating now because we are finding so few answers.

 

Family Reactions

Some Success stories...

They are starting to show some interest in this.

...and some not so successful.

They want nothing to do with it (HNPP) or discussing it. You see, I think they look at me walking okay when they see me and think I am making the whole thing up. That is the trouble with HNPP. You don't always show visible signs of it..I only show when I walk a lot or do too much activity.

I sometimes think that my family thinks that it is all in my head. They cannot understand how one minute I am fine and the next I am walking strangely.

Symptoms

Balance

 I have no side to side balance and use a cane.

Cold Extremities

My feet stay cold most of the time.

My feet stay cold, even in the hot whirl pool. It is a weird feeling.

Right now the cold hands are driving me nuts!! It has been in the 70's and 80's and I still have to have gloves on in the house to try and keep warm.

My feet are always very cold and so are my hands.

Cramps

Do others experience the burning in the nerves and the cramping muscles after moderate exercise? Is exercise that causes pain and burning helpful or harmful? Just a few questions that seem to plague me a bit.

Has anyone gotten cramps in their neck? I have about 3 times in the past month and it makes any other foot or leg cramp feel like a baby.

I have cramps in my neck too.

I get cramps alot...my toes will start going every which a way and you have to jump around and put weight on them to make them stop...then, your fingers will start twisting and cramping!! What a weird thing!

I get them too, in the arches of my feet.They are painful enough that my whole body is incapacitated for 10-30 minutes.

Cramps I have had since I was an adolescent. Especially in my legs when I am tired. But now daily in my left hand, probably a mild form of atrophy/muscle degrading.

As far as muscle cramps...I had ultrasound done on my abdomen....it was cramping when I bent over to do something...I mean severe....I asked my mother what symptoms she was having and she said that every time she bent over to do something...she got severe charley horses in her abdomen...she said the doctors thought she was crazy.

I too have charley horses in my abdomen. Although I always thought it was scar tissue...I get them right in the incision mark where I had my gall bladder removed...some days they are so severe, that it hurts to do anything.

Falling

As I was going to work this morning my ankle gave way and I fell like an idiot. Luckily, I was not hurt, just embarrassed....

Fatigue

I have the fatigue thing bad...it has slowly gotten worse over the last two years and now I just have to rest.

Now I tire really fast. It is something we all have to learn to live with.

I have been sleeping a lot again lately...I slept 3 hours this afternoon..made me really mad, because the weather turned out pretty and I feel like it messes up my whole day when I do that.

Now I am lucky if I can make it to 9:00PM and I nap at lunch at work and on the weekends I can not seem to stay up all day. I always have to take a few hour nap (which I hate because I could be doing things) on the weekends.

On Sunday, I was so lethargic, I couldn't find the energy to get up off the couch. So I finally forced myself to get up and do some things that needed to be done, like cutting the grass. etc. I think I overdid it. Monday I could not pry myself out of bed. I did not make it to work. I ended up sleeping on and off the entire day. I was extremely fatigued. I woke up at 9:00 PM and went to bed. I did not experience any pain, only mild numbness and extremes fatigue. 36 hours of absolutely no energy.

After 3 hours of walking and bird watching, once I sat down I could hardly drive home, my wife did. Then I spent the next 2 days glued to my chair because every time I got up it hurt from my hips down. Also I was very tired all over.

I am not able to function much lately. I could stay up for an hour and then have to rest awhile. Just doing one load of laundry or cooking a meal has me out for at least 1-2 hours.

Feet

I remember the doctors saying I have high arches...which I never knew. I always thought my feet looked like everyone else's.

My feet are always tired and cramp a lot sorta where my toes all cross over and bend down.

I have crooked toes too. They cross. When I was a kid, I thought it was because my parents made me wear shoes that were too small....I too have high arches. When I was a kid, my parents would get mad at me for walking down the hall so hard that I disturbed them. My feet used to hurt real bad too. Lucky for me, they are so numb now, they don't hurt anymore.

I find that I have more of the CMT symptoms than HNPP but I guess the DNA and biopsy must be right. I have very high arches on both feet with severe foot drop.

If I ride a bike for any length of time my fingers will go numb from gripping the handle bars.

 

Legs/Walking/Sitting/Lying

I cannot stand for any length of time. Can't stand at the stove long enough to cook. When I try I have terrible pain in my back and my legs get real tired.

I have been out and about a lot. It's been making my legs achy and sore. You walk walk walk when you think you're ok, then after sitting for a while you go to get up and you get pains in your hips and knees and your legs feel like rubber as if they're going to give out on you.

I can still walk, but not for long distances. 15 minutes tops.

I wanted to mention I had a bad case of foot drop a while back, but it went away after about a month. But I don't really know what I did to get that, probably peddled my bike the wrong way :)

I wake up also because of back pain and it is frustrating when I want to sleep in.

Siting is definitely not a good thing for me, but too much standing is hard on calves and arches too - sometimes it's hard to know where to crawl.

 

Hand /Arms /Hand Function

My fingers don't work right when I do any kind of mechanical work - Using a screw driver, etc.

I have always worn a watch and that is beginning to bug me.

I can hardly tolerate things on my arms - watch, bracelet, tight sweater, etc.- So I always pull my sleeves up.

I have stiff, weak hands that have lost dexterity progressively. I'm blaming this on my computer mouse because that is when I can feel it the most.

I had drop shoulder long before anyone knew about HNPP. I could not pick up my left arm. My wife had to dress me. she had to literally put my clothes on me and then button all buttons. My shoulders are not wasted.

My right arm is losing allot of strength which is hard since I am right handed.

I have a lot of trouble typing these days.

My hands are waking me up again at night.

I have found that my left arm is starting again with me waking up with it asleep...so bad, that it usually lasts through part of the morning...my daughter handed me a pen to sign a paper for school and the the pen just plopped right out of my hand.

When I lift my arm up and lay it on my head for short while, I get an aching, heavy feeling in the arm and have to lower it down to my lap again.

 

Numbness

Even when I work the mouse on the computer, my hand will go numb after 3-4 minutes. I can shake it and that seems to help.

I wake up every morning with some numbness. I just got up and my left ear (of all things!) is really numb. It seems like how ever I sleep, it makes me numb - mostly on the left side of my body. Left arm, left calf, left side of face.

The numbness in both my arms from the elbow to my baby and ring fingers is always there.

I had a numb feeling in my back that went away. But what triggered my going to the neurologist was the numbness in the side of my hand and little finger. And up to now, this lack of feeling is still there.

I do get numbness in different parts of my body, but they never go away.

Pain

The pain is there just doing minor things around the house. I get cramps almost constantly in my feet, but they are so numb it seems to deaden the pain some.

Yes, There definitely is pain with HNPP. I have it in my lower back right now.

Today has been particularly bad - rainy with a chilly wind blowing. He has been in agony since about midnight last night.

Right now my nerve is so pinched in my lower bak that it is extremely difficult to sit for any amount of time at all.

Pressure Palsies

I have been painting a lot lately and have to take breaks for a day or so at a time to rest my hands and legs. They seem to want to go numb when used too much. The numbness seems to stick around longer and longer.

...you talk about an episode lasting 2 or 3 months. Well what about one lasting 2 1/2 years? I think I am over an episode. I still hurt and am not normal by any means, but...I am not as tired, have less pain and can do more than I have done since I quit working. (Note: this lasted 3-4 weeks)

Progressing Symptoms

I personally have been getting worse slowly from the start. I haven't had episodes...I used to recover after being off a few weeks, but now I either do nothing and live with minor pain and the odd sleepless night, or I do something and spend the next few days in alot worse shape.

 

Sensation

I can't really feel the difference between hot or cold and I got a severe burn on my back from an electric heating pad.

 

Weakness

I have noticed that if I am active one day, that the next day when I wake up I find it hard to walk the same in the morning upon first waking up and using my legs

 

Living with HNPP - the emotional aspects

Grieving - denial, anger, bargaining, depression, etc.

 I get mad alot of times because no one takes me serious on it and mostly cause no one wants to talk or hear about it. especially the doctors!!

When I got the news that I had HNPP, I was quite depressed. But I honestly feel better now knowing what I have.

Not a day goes by where I don't say "Why me?" and then say to myself "Thank God it's not something worse".

Acceptance

As far as my adjusting, I haven't. I'm glad I don't have to go to work because it is so hard on me to make a day. I get aggravated with my body. Hands and fingers don't work right. tripping. Falling down. Fatigue.

Readjusting

Isolation

For so long now, I have lived with this and not known one person who has it.

I feel alone with this. It is so different when you have something that others can't relate to.

 

Living with HNPP - the physical aspects

Symptom variability

This darn disease is the strangest thing I have ever seen. O.K. today, numb tomorrow, pain the next day, OK the next day, numb for two days, etc., etc., etc.

Can't figure out how to pace. What works today, doesn't work tomorrow.

Today I had much running around to do at the mall, but I could not hold up...my right arm gave out QUICK, went completely numb with sharp shooting pains up and down,,,then my legs wouldn't hold up with all the walking...isn't it weird how some days you can do anything and other days, just a simple shopping trip can tire you out!

Pacing: learning to walk the fine line between too much and too little activity

I find that what I can do one day, I may not be able to do the same amount on another day. It's very hard to pace myself.

I sure know this...when I push too hard, I pay dearly, usually the next day or several days.

I have to learns to take things one day at a time and maybe someday i will feel better. I find it really hard to know my limits and stop before overdoing it, but when I do feel better I always end up doing too much and paying for it for long periods of time.

Sometimes, even though you know you will pay for it, you want to do the things you used to to and enjoy. That is the way I feel about fishing. I always know I will pay. So what?

Slowing down...

Lately I have realized how much I can not do now that I used to do. More so now than ever! I am finding all the walking and running errands that I used to do tires me out faster and the feet and legs are not lasting as long as they use to. It's really starting to make me mad.

I can go shopping for about an hour and then I know its time to stop and get off my legs.

It seems too, that the less you do the harder it is to do things. I think being inactive is affecting me greatly.

Letting go...

The only sad thing is that I had to give up my houseboat which I rebuilt myself. I cannot do the maintenance like painting, derusting, repairing, etc.

I tried vacuuming last week and have paid for a week. I have to know when to stop but it is really hard to just watch others do your work.

But keeping active

 Your body will definitely tell you when you have done too much and you will not like the results.

 I work out and still try to. The doctor said don't do anything that will build muscle. If it hurts don't do it. Instead of using 40 lb. dumb bells, I use 15 lb.. I just do one set.

I do not think I have a very severe form of HNPP at the moment. My walking is a bit clumsy and I don't do sports, but I do not feel very restricted in my movements.

 Balancing acts

If I work all day, my family gets cheated when I get home. But if I rest some to feel good for my family, then my employer gets cheated some.

 Work

If he stayed off work for awhile, he would be near normal. But as soon as he went back, he was in trouble again. I wonder if he will ever get back to where he was...

I had to retire because I can no longer walk or really do much of anything for long periods of time. Guess I was lucky to hang on as long as I did.

Adaptive Devices

I want a scooter. I must have a mode of transportation. Not being able to go places is worse than people staring at me. I wish it were not this way. But it is. We have been to several places in the past two years when it really became apparent how badly I needed a scooter. I was miserable and in pain.

My doctor expressed concern about my becoming dependent on a scooter. I don't think they understand the frustration of trying to go someplace and having your legs give out on you. Or being in such pain, you don't think you can make it back to the car. You can't enjoy yourself.

I continue in my quest for decent working equipment at work. Today they brought me a large size track-ball. I knew within 15 minutes that it was worse than using my standard mouse.

Living with the unknown
As far as what the future holds...well, nobody can tell you. I think this is the hardest part for me to handle...isn't there a listing of percentages of people with certain disabilities (with this)? I am one of those people who can't deal with the unknown.

I think of each day as a surprise

 
It's also hard not knowing what to expect in our future, but listening to others with HNPP can help....things can happen daily where HNPP comes into play.

Looking forward

I really do believe though, that life can be full of happiness if we accept our fate and look at the bright things in life. There is always some even if they seem hidden.

I have always been strong (both physically and mentally) now I will have to focus all my energies on strengthening the mind. I would rather lose my physical strength than my mind though.

If I told you that I was not worried about my future, I would be lying. I also take it one day at a time.

I can just feel things getting worse and worse- it scares me so much. I had dreams of spending leisure filled days of retirement with quilt making. I hope I won't lose that dream.

I am concerned about the future and how bad this will get. and worried whether my two sons and their kids will get it.

 

Advice to others

I think the more you try to do the worse off you are. Take care of your body like you never have before. The damage builds.

 

Last updated: 1/00

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