Re: Pachyonychia Congenita - any knowledge to share??

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Posted by Murray on February 20, 19100 at 16:19:54:

In Reply to: Re: Pachyonychia Congenita - any knowledge to share?? posted by Michelle Mays on February 16, 19100 at 18:24:09:

Michelle,

Here is a copy of a post (by me) from 12/99 which may give you more insight into P.C. One bit of advice... Instill into your child's mind that this is not a fungus and is not contagious and does not make him/her (? sorry I don't have your original posting in front of me) "inferior" to other kids... I got a lot of kidding by other kids as a child and the most important thing my Mom did was remind me I may be different, but that doesn't make me a bad person!!! As your child gets older, it may become worse (pain walking, nails regenerating, etc.) but I try not to let it get me down!!! Mental attitude is very important.
_______________________________________________________I am a 43 yr. old man and have had Pachyonychia Congenita since birth and my Dad had it and my younger brother (39) also has it, as well as his daughter. His son doesn't. We are the youngest of 4 brothers and 2 older ones didn't get it. Dr's think because my Dad's condition was more dominant in his genes since he was older when we were born. I have the "fungus appearing" condition on finger and toe nails, excessive sweating on feet and hands causing callous buildup on feet, harsh loud (annoying to others around me!!) chronic cough (though dr's don't connect this to the disease, but I read of hoarseness here SO who knows??), build up on my tonque so I have a tongue scraper I use daily. In younger years I used to experience episodes of dishydrotic eczema on my hands (itching, fluid filled blisters, weeping, and usually ran a cycle of 2-3 weeks and required soaking hands in BluBoro or Domeboro & water, ointment, wearing absorbent cosmetic gloves because of the fluids) & was told I got this "rash" because of sweat glands in my hands closing up. I also experience regeneration of finger nails (caused by
unconsciously sleeping on hands or by accidentally banging one on something, or cutting nails to close
to the nerves. This process is swelling, fluid (pus) forms in the nail and then it gets swollen to a point that it can be lanced to relieve the pressure and eventually the nail comes off to expose the nail bed and a new one grows out.

The foot pain is the worst because I cannot walk very long without painful throbbing, burning and sweating
of my feet (and hobbling like an 80 yr. old man). I have tried various "comfortable shoes" (Spenco sport
insoles help) to no avail, oiling my feet with Xylocaine Gel (that's useless), Triamcilone ointment (& wear socks to sleep in) before bed (helps some short term), but basically it is a debilitating disease that most dr's know nothing about so they refer you to a dermatologist (which is a useless waste of your time). My parents took me to dermatologists since I was 6 yr. old and nothing new in 37 years.

I sand my nails down periodically with a Dremel "Motor Sander" (look for it in hobby stores), go to a
podiatrist every 2-4 weeks to have my feet (callouses) scaled down & cut off, have looked into plastic surgery on my finger nails because psychologically it is hard for me because most people think of it as a contagious fungus (which I set them straight on when I get to know them).

I am on a regime of strong painkillers which does help, but am unable to do long walks, or swim or hot
tub too long because if my feet get too soft it's painful to walk even short distances... Either the skin is
too hard or it is too soft - Hard to find any in between. I am also told to be checked to have a biopsy
taken from tongue every 5 yr. or so to check for cancer. I am considered disabled and was able to get a
Handicapped Parking pass with my podiatrists help and urging.

I was called by a college in WA to see if I was eligble for an Ichthiosis study in England (MD/Professor
was traveling looking for eligible people) but I found as severe as my disease is, people have it on their
whole bodies so mine wasn't severe enough.

I've done a lot of research on it and still don't know that much about it. The main thing is it's hereditary
and we only traced it back to my Dad (no prior generations) and am told there is a 50/50 chance of
passing it on to your child... So I chose not to take the risk and won't have children. And as you get
older, it gets worse (more painful to walk) which I observed in my Dad until he passed away at age 62
(from heart disease unrelated to Pachyonychia Congenita).

My current doctor is very sympathetic and is trying to find out more about it and he has sent me to a Chronic Pain Management Clinic (which also included an oncologist) and at least I have a professional MD that doesn't shrug it off as something trivial and refer to it as a skin problem.

I hope this info helps others out there because I know there aren't that many of us... Very rare to have full
blown disease. Good luck and email me at MJE56@netscape.net or post here if you'd like more info or have some info to relate about "your situation."

----ME

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: Michelle,

: Here is a copy of a post (by me) from 12/99 which may give you more insight into P.C. One bit of advice... Instill into your child's mind that this is not a fungus and is not contagious and does not make him/her (? sorry I don't have your original posting in front of me) "inferior" to other kids... I got a lot of kidding by other kids as a child and the most important thing my Mom did was remind me I may be different, but that doesn't make me a bad person!!! As your child gets older, it may become worse (pain walking, nails regenerating, etc.) but I try not to let it get me down!!! Mental attitude is very important.
: _______________________________________________________I am a 43 yr. old man and have had Pachyonychia Congenita since birth and my Dad had it and my younger brother (39) also has it, as well as his daughter. His son doesn't. We are the youngest of 4 brothers and 2 older ones didn't get it. Dr's think because my Dad's condition was more dominant in his genes since he was older when we were born. I have the "fungus appearing" condition on finger and toe nails, excessive sweating on feet and hands causing callous buildup on feet, harsh loud (annoying to others around me!!) chronic cough (though dr's don't connect this to the disease, but I read of hoarseness here SO who knows??), build up on my tonque so I have a tongue scraper I use daily. In younger years I used to experience episodes of dishydrotic eczema on my hands (itching, fluid filled blisters, weeping, and usually ran a cycle of 2-3 weeks and required soaking hands in BluBoro or Domeboro & water, ointment, wearing absorbent cosmetic gloves because of the fluids) & was told I got this "rash" because of sweat glands in my hands closing up. I also experience regeneration of finger nails (caused by
: unconsciously sleeping on hands or by accidentally banging one on something, or cutting nails to close
: to the nerves. This process is swelling, fluid (pus) forms in the nail and then it gets swollen to a point that it can be lanced to relieve the pressure and eventually the nail comes off to expose the nail bed and a new one grows out.

: The foot pain is the worst because I cannot walk very long without painful throbbing, burning and sweating
: of my feet (and hobbling like an 80 yr. old man). I have tried various "comfortable shoes" (Spenco sport
: insoles help) to no avail, oiling my feet with Xylocaine Gel (that's useless), Triamcilone ointment (& wear socks to sleep in) before bed (helps some short term), but basically it is a debilitating disease that most dr's know nothing about so they refer you to a dermatologist (which is a useless waste of your time). My parents took me to dermatologists since I was 6 yr. old and nothing new in 37 years.

: I sand my nails down periodically with a Dremel "Motor Sander" (look for it in hobby stores), go to a
: podiatrist every 2-4 weeks to have my feet (callouses) scaled down & cut off, have looked into plastic surgery on my finger nails because psychologically it is hard for me because most people think of it as a contagious fungus (which I set them straight on when I get to know them).

: I am on a regime of strong painkillers which does help, but am unable to do long walks, or swim or hot
: tub too long because if my feet get too soft it's painful to walk even short distances... Either the skin is
: too hard or it is too soft - Hard to find any in between. I am also told to be checked to have a biopsy
: taken from tongue every 5 yr. or so to check for cancer. I am considered disabled and was able to get a
: Handicapped Parking pass with my podiatrists help and urging.

: I was called by a college in WA to see if I was eligble for an Ichthiosis study in England (MD/Professor
: was traveling looking for eligible people) but I found as severe as my disease is, people have it on their
: whole bodies so mine wasn't severe enough.

: I've done a lot of research on it and still don't know that much about it. The main thing is it's hereditary
: and we only traced it back to my Dad (no prior generations) and am told there is a 50/50 chance of
: passing it on to your child... So I chose not to take the risk and won't have children. And as you get
: older, it gets worse (more painful to walk) which I observed in my Dad until he passed away at age 62
: (from heart disease unrelated to Pachyonychia Congenita).

: My current doctor is very sympathetic and is trying to find out more about it and he has sent me to a Chronic Pain Management Clinic (which also included an oncologist) and at least I have a professional MD that doesn't shrug it off as something trivial and refer to it as a skin problem.

: I hope this info helps others out there because I know there aren't that many of us... Very rare to have full
: blown disease. Good luck and email me at MJE56@netscape.net or post here if you'd like more info or have some info to relate about "your situation."

: ----ME

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