: : : : : : I am a 42-year-old woman with MS and diffuse hypogammoglobulinemia.
: : : : : :
: : : : : : I've recently noticed that my skin is tasting salty, whether or not I am
: : : : : : sweating. I understand that this is in indicator for Cystic Fibrosis.
: : : : : : Are there any other diseases or conditions that cause salty skin? Should
: : : : : : I be tested for CF? I have chronic bowel problems and mild asthma. My
: : : : : : electrolytes are often out of balance. Am I worrying needlessly, or is
: : : : : : salty skin something to be concerned about? It's really salty!
: : : : : Dear Salty:
: : : : : Sorry to hear that you have MS. One of the diagnostic tests for CF is excess NaCl on the surface of the skin due to abnormalities in the chloride channel of the skin. Usually, it is only seen in people with both CF genes, making it a uncommon entity. Although it is not rare, especially in the caucasian population. Children with CF are now living into their late 20s and early 30s due to the advancement in medical treatment. However, their clinical course is very protracted with multiple lung infections throughout their lives. There are a few reported cases of people in their 40's having a very mild form of CF. Most of these patients have asthma as you have. In my honest opinion, if there is no family history of CF and your asthma is well controlled, then I would not pursue the diagnosis of CF. There is no treatment for CF, at present. However, if you are considering having (more) children then this might be an option for getting a diagnosis. I think the chances are remote. However, if you think
: : : : : that it might be important to you or your family, I would talk to your regular physician.
: : : : : Sincerely,
: : : : :
: : : : : CCF Neuro[P] MD, RPS
: : : : Dear CCF Neuro:
: : : : Thank you very much for the information. I know that there is no treatment for CF per se, but there are treatments for many of the symptoms and opportunistic infections that occur in CF. In my opinion, this is a valid reason to pursue a diagnosis. I do not have many asthma attacks, but my lungs are often wheezy. And I have the GI problems.
: : : : There is no CF in my family, but being that it is recessively inherited that would be the case in many (or most?) families were a member was found to have CF, wouldn't it?
: : : : Also, what is the significance of a Hoover sign in one lung and a reverse Hoover in the other?
: : : : Thanks,
: : : : Salty
: : : :
: : : Dear Salty:
: : : The characteristic lung infections of CF are pseudomonas. Have you had multiple infections with this organism? In fact, CF patients have so many infections with this organism, they develop resistence to the antibiotic. If you have had many pneuomonia infections with this organism then you might have CF. Although I wouldn't say for sure that even with multiple infections of your lungs without being pseudomonas, that you have CF. It is a simple test and if you think that it might be valuable then ask your primary physician. I have not heard of the Hoover sign for the lungs. In neurology, the hoover sign is when we ask a patient to move both heels downward. If the patient has a real hemiplegia or an organic problem, the one leg will not exert a forceful movement downward while the normal limb forcefully moves downward. It may be that one diaphram side is not moving as well as the other, but this is only a guess. As far as the recessive inheritance, yes since you would only have a 25% chance of having the disease if both your parents carried the gene, the disease might miss several generations. The GI problems result from the lack of pancreatic enzymes that break down food. Your stools would look very pale and have a foul smell. I hope that you find the results your after. Best of luck.
: : :
: : : Sincerely,
: : : CCF Neuro[P] MD, RPS
: : Dear CCF Neuro:
: : Thank you. Your replies have been quite helpful and illuminating.
: : Yours,
: : Salty
: Dear Salty:
Dear CCF Neuro:
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