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This Could Well Be the Worst Inheritable Disease of All

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Surely hidradenitis suppurativa must be the worst genetic disease there is, and, ever since a pretty blonde lady came to see me and told me that she had been unable to find a doctor who knew anything about it (except the name), I have agonized about the situation to the extent that I have been able to think of little else. To add to her despair, none of those whom she has consulted even knew of a specialist to whom she might be referred! The more she described what she was going through the more agitated I became, and by the time she left I was so deeply concerned that I felt driven to find out as much as I possibly could about it.

What I have learned is that hidradenitis suppurativa is a chronic skin disease, one which does not become symptomatic until puberty. But this is no ordinary skin disease. The nodules, which it produces in many parts of the body, cause excruciating, chronic pain, severe infections, and unsightly scarring, and, when surgically removed, grow back, even reaching the size of baseballs. The afflicted victims will by then often have become hermits because of the agony exacerbated by the unsightly scar tissue, which may already have become skin cancer.

I Was Certainly Not Looking for Another Undertaking

Over the years, awareness of hemochromatosis (HH) has greatly increased. While, sadly, the gene for hidradenitis suppurativa has not been mapped, we now know which nationalities are most at risk of developing hemochromatosis (the disorder that caused the death of my beloved husband) and I never cease to give thanks for these developments. But having devoted the greater part of my adult life to creating awareness of the latter illness, I was certainly not looking for another undertaking that would inevitably involve a great deal of my time and perhaps become another obsession. However, I was now confronted with a very tragic situation—and now I am hooked!

After my visitor had poured out her heart, described some of the effects of her disease, and confided that her mother had not told her until the night before she died that she, her mother, was afflicted with it, I have not been able to sleep for agonizing about my desperate visitor for, by this time, she herself has a child with the prospect of the same suffering in store for her. Ever since our conversation I have been pondering what I can do, although that does not mean for one minute that hemochromatosis will be neglected. At least, as bad as it is, there is nothing revolting about HH, while that is certainly not the case with this other affliction—so much so that the mother never discussed it with her daughter, only admitting shortly before her death that she suffered from it.

Starting my research by referring to Wikipedia, I found out that this is usually the case with those afflicted with HS – they do not even tell the rest of their family, “because they are too ashamed!” The disease is now known to be an autosomal dominant disorder, in other words one which can be inherited from a parent who carries only a single copy of the gene. It is not infectious—a godsend because the suffering must be horrendous! Once thought to be a rare condition, probably because it was so rarely reported, it is now known that that is not the case and, for an unknown reason, women are several times more likely than men to develop it. The cause is unknown but, as far as I’m concerned, the fact that people can inherit it is bad enough.

Although the literature on the Internet seems to support the fact that the autosomal gene has not yet been mapped, I did find one reference in which the writer maintains that it has, but unfortunately provides no details. It would help if one could establish the gene pool that is at risk, but I’ve not been able to find such info for my distraught new acquaintance. Judging by her blonde colouring, I have even written to a good friend who is a doctor in Sweden, but he also could not find proof of any such details concerning this ghastly disease, which must be worse than leprosy.

Exploitation on YouTube

It was a huge mistake to consult YouTube at a friend’s suggestion—not because the articles and images posted there for the right reasons were so tragic that I wept, but mainly because, as is so often the case, it made me livid to see, among those postings, the usual exploitation of an illness for the purpose of marketing useless cures. Meanwhile there is little I can do but try to promote awareness!

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About Marie Warder

Born in South Africa, became a journalist and later trained as a teacher before establishing my own school - "Windsor House Academy, of which I remained the principal until I emigrated to Canada. Love to write, and have published 27 books. Played the piano in my husband's dance band for 33years. Founder and President Emerita of the the Canadian, South African and in International Association of Hemochromatosis Societies, http://en.wikipedia.org/wiki/Marie_Warder
  • http://tjkorri.blogspot.com/ Teri Korri

    Thank you for helping raise awareness of HS. It’s definitely painful both physically and mentally, a disease no one wants to speak about. My story is on my blog. There are many others who are gathering together on Facebook and Yahoo groups.

    Again thank you

  • Michelle Veasey

    Thank you also for raising the awareness. We have all been treated in the same way by the doctors. There are quite a number of us who converse regularly sharing tips and methods for managing the disease or at least trying to lessen the symptoms and lead relatively normal lives. We all feel abandoned by the medical community, with the only solace being with other sufferers who know how we feel. If you wanted to speak to us to find out more about the disease I am more than happy to discuss my own experiences as I am sure many of us would. Thank God for social media is all I can say at least we don’t feel like freaks of nature on our own! Thanks again :-) x

  • Gandalf Parker

    I also thank you for posting this.
    In the HS support groups on FaceBook we comfort each other on bad days, we compare notes about how many wrong diagnosis we suffered thru, we complain of doctors who leave the room to google it then come back with horrible information that is 5-10 years out of date, we post pictures that we would never post publicly (and never should because the ones that are public are bad enough). We even make jokes. I HIGHLY recommend anyone diagnoses with HS to join one of the online support groups.

    By the way, the genetic thing is still in debate. It might not be the CAUSE of HS but the genetic link that some people seem to have might be due to a genetic predisposition to something else that makes HS more likely or more recognizable in those people.

    One of the surprisingly WORST sites for your doctor to find on HS.

    One of the best sites for your doctor to find Hidradenitis Suppurativa

    One of the most complete collections of the various theories of this no-known-cause no-known-cure disease
    SkinChannel.com the link for Hidradenitis Suppurativa

  • Marie Warder

    Thank you for your comments which are especially important to me if I am to learn more about HS. Once I get hooked on a worthwhile cause (which yours most certainly is)I put my heart,soul and prayers into it and I have already taken the plunge.

  • Mike W.

    Being someone who suffers with HS I appreciate this article and any other form of awareness. THANK YOU a million times over. It is so very frustrating having to go to doctors who know very little to nothing about it, you end up knowing more than they do. Tired of being told “there’s not much else we can do”. You get so tired of being treated like a drug addict for pain meds as well. Again, Thank you very much.

  • Libby Sandoz

    I will simply say THANK YOU. More awareness. Wish someone would force the Dr’s to read an article like this…I am SICK of living with this dreaded condition. Can only pray for awareness & treatment in the future.

  • Mechelle royce

    Thank you so much for this article…as I type this I am in a hospital bed and due for my 6th surgery in the last year because of HS.

  • Mandi Hanson

    Friend has this disorder and it makes me so said to hear what she and others are going through. The worst thing about it is the friend is unable to get benifits to help feed her family

  • http://hsf-uk.find-forum.net/ Bev Alderson

    Thank you so much for helping raise awareness of Hidradenitis Suppurativa you will never know how much your article is appreciated in the HS World.
    I suffer with HS and I’m co owner of a support group for people with HS once again many thanks for all your hard work!

  • Marie W.

    Thank you for the info. At last I have found a link to a support group. Not one of the organizations on the Net have bothered to respond.

  • Marie W

    I no longer have either the strength or the stamina that I devoted to Hemochromatosis to do anything significant to help fight this new battle, but—-using my voice-recognition device—-I intend to “write” about it, and plan to start by doing what I can to publicize Rare Disease Day this year. This is an annual, awareness-raising event co-ordinated by EURORDIS (Rare Disease Europe) at the international level and the National Alliances of Patient Organisations at the national level.
    February 29, 2012 marks the fifth international Rare Disease Day coordinated by EURORDIS and organized with rare disease national alliances in 25 European countries. On this day hundreds of patient organizations from more than 40 countries worldwide are organizing awareness-raising activities converging around the slogan “Rare but strong together”.
    Activities will take place across Europe, all the way to Russia, continuing to China and Japan, in the US and Canada, and as far as Australia and New Zealand!

  • Jo Redmond

    Thank you so much for taking time out to raise awareness for such a disabling soul destroying disease

  • Meg Stuart

    Responding to you request on Facebook, my family and I are praying.

  • Brett and Muriel

    You have our prayers and support, that’s for sure!

  • MW

    * When Velpeau identified and described hidradenitis suppurativa in 1839, it was primarily found among families of Sephardic Jewish, Italian, Greek, Middle Eastern and Northern African ancestry.

  • http://www.squidoo.com/lensmasters/IanMayfield Dr Dreadful

    Thanks for this article. My wife suffers from HS: in her case it seems to be abating somewhat as she gets older, but she still has regular outbreaks. Then of course there are the extensive scars that will never go away.

    One of our biggest frustrations is that the medical community knows so little about the condition. Though painful and unsightly, it’s not life-threatening, so it’s not one of the “glamour” diseases that gets research funding thrown at it.

    The baton of education and awareness therefore seems to be carried mostly by the community of sufferers, their friends and families, and the occasional interested physician.

  • MW

    Evidently Electron beam radiotherapy has been a successful treatment of hidradenitis, especially in Europe but it is not a common treatment option in most of the United States, as radiation oncologists generally refuse to treat patients with non-malignant diseases because of the potential for secondary radiation-induced tumors in the long term.

  • http://hsf-uk.find-forum.net/ Bev

    Please don’t do any more than you think you can Marie, we don’t want you to end up ill

    I’m sorry that none organizations on the Net have replied and I hope the link to the group helps you to understand HS a little more.

    I’m sure anything you do will help us and thank you again for trying.

  • Claire G

    Thank you so much. Just knowing someone cares enough to try raise awareness means a lot. You are a true angel, thank you again xx

  • Marie

    I have just been sent a link to an article by a physician in Denmark, Gregor B.E. Jemec, M.D., D.M.Sc., which appears in the New England Journal of Medicine J Med 2012; 366:158-164 and I count the fact that the article is evidence of renewed interest, as proof, also, that the prayers are working!

  • Tom Hennessy

    Are you VERY sure it isn’t connected to hemochromatosis ? Pretty much every disease so far has been linked to dietary iron overload , the same results as in hereditary hemochromatosis. Psoriasis is related and IT has high iron in the skin.
    “Increase in free reactive iron in psoriasis”
    The problem which has to be overcome in the treatment is noone wants to become vegetarian and NOT eat the high iron meat diet. If they don’t STOP their high iron diet , meat diet , they will never be able to totally eliminate the stored iron which accumulates.

    “Role of free reactive iron in psoriasis”

    “Our findings indicate the increase in level of free reactive iron and lower level of antioxidant status in psoriasis. This may contribute via free radical generation to the development of secondary cellular
    damage and pathological state in psoriasis vulgaris. Therapeutic use of iron chelator and antioxidant drugs may be investigated for beneficial role in psoriasis.”

    “Psoriatic patients exhibit high iron and low ascorbic acid concentrations in the dermis”

  • Tom Hennessy

    THIS shows a mouse doesn’t get hyperkeratosis when fed a low-iron diet.

    “Absence of hyperkeratosis in mice fed on low iron diet”
    “Low iron diet retards 12- O-tetradecanoyl phorbol-13-acetate-mediated tumorpromotion in murine skin”
    “Cutaneous iron levels were also lower in mice fed on low iron diet than in mice fed on normal diet. Histopathological sections of the skin portion adjoining tumors showed a lower degree of epidermal hyperplasia
    and lesser infiltration of inflammatory cells in the dermis, and absence of hyperkeratosis in mice fed on low iron diet. Thus, in this study we observe
    that the tumor promoting potential of TPA is reduced in mice fed on low iron diet, which is also accompanied by lesser inflammatory changes in the skin of
    tumor-bearing mice fed on low iron diet.”

  • Tom Hennessy

    “Iron chelators decrease Tumor necrosis factor alpha (TNF)”

    “Although deferoxamine’s protective actions can be attributed to inhibition of oxygen free radical generation, these experiments
    also document a decrease in TNF release.”

  • Marie Warder

    Thank you for all this info. Very useful!

  • Tom Hennessy

    Thank you for all this info. Very useful!

    I have much more information about iron because of my Herbivore Hypothesis.

    When I say it is related to ‘genetic’ hemochromatosis I mean the skin disease is due to iron so ‘could’ just as easily been diagnosed in your husband due to HIS increased iron.
    Increased iron is NOW being called ‘age-related iron accumulation’.
    They USED to say this GRADUAL iron increase didn’t exist and therefore my Herbviore Hypothesis couldn’t possibly be true.
    NOW that they HAVE recognised the FACT iron builds gradually in the body and so they have christened it , age-related iron accumulation.
    That is WHY there is disease in humans.
    Man is an obligate herbivore-frugivore eating meat which leads to disease.
    Herbivore Hypothesis.
    They are finding iron instead of being a boon , iron is instead , a bane.
    “The effectiveness of discontinuing iron-containing prenatal multivitamins on reducing the severity of nausea and vomiting of pregnancy.”
    “Avoiding iron-containing multivitamins is effective in majority”

  • Marie Warder

    I attempted to post a comment to your ‘network’ but it was rejected. Meanwhile I have ‘copied and pasted’ all of them and sent them by email to some of the Hemochromatosis experts with whom I work.

  • Iron Lady

    This assertion that a mainly vegetarian diet helps with keeping iron stores down relates to a very good documentary movie we recently saw called “FORKS OVER KNIVES”. While there is no mention of iron overload in the documentary, it does put forward the possibility of better health if all meat (amongst other things) were removed from the diet.
    Mr. Hennessy, you would probably find the film very interesting.

  • Marie Warder

    We seem to have “strayed” from the main subject to Hemochromatosis, but, regarding your remark that my husband’s illness could ‘easily have been diagnosed’
    I think you should read my book, The Bronze Killer to learn how little the medical profession knew about iron overload in those days.
    He was ill for 8 years before, by nothing short of a miracle, he was referred to a man who was a world authority on the subject.

  • Tom Hennessy

    Quote: I attempted to post a comment to your ‘network’ but it was rejected
    Answer: I will have to see what is going on , it’s been awhile since I updated The page.
    Quote: regarding your remark that my husband’s illness could ‘easily have been diagnosed’
    Answer: I didn’t mean he could easily have been diagnosed , I meant he could JUST have easily ‘caught’ hidradenitis suppurativa , just like he could have caught cystic fibrosis another disease linked to iron excess other than the arthritis. It just is what manifests in the particular person , just like iron is now being studied as THE cause of diabetes.

  • Marie Warder

    Sudden, rapid-onset diabetes is often a symptom, and until insulin was improved, it used to be a crippling factor without very severe blood-letting – in one case a gallon of blood a month to save a patient’s life…

  • Tom Hennessy

    That’s the first time I heard about the diabetes and bloodletting evidence ? I haven’t found a whole lot on the real early bloodletting and the known results thereof. I do know that it used to be the ONLY treatment available for hypogonadism which caused gynecomastia .
    The lastest in regards to ‘bloodletting’ would be the brain iron in women who have had hysterectomies. It JUST shows that women who lose the menses by hysterectomy begin to load iron and their brain iron begins to rise to those levels commonly found in men. Which sort of gives credence to the theory the menses are SPECIFICALLY FOR lowering the iron levels in the body for the safe gestation of an egg. They figure this brain iron buildup due to the lack of menses may lead to higher neurodegenerative disease in women too.
    “Premenopausal hysterectomy is associated with increased brain ferritin iron”
    Kind of makes one wonder now about the contraceptive pills which all but in not completely eliminate the menses ?

  • Hester McFarlane

    Sir,having come upon this word exchange while seeking info about hidradenitis suppurativa, I am now keen to find out how much you know about the lady with whom you are exchanging views; and I paste here, for your info, what I have copied from Wikipedia:
    Hemochromatosis activism
    Tom Warder, Marie’s husband, whose fight with hemochromatosis was the catalyst for her crusade against the disease.
    In 1975, Warder’s husband, Frederick Abinger (Tom) Warder, who had been seriously ill for eight years, was finally diagnosed with hemochromatosis at the age of 50, and died later in 1992.[13] In 1979 their daughter, then 32, was also diagnosed with hemochromatosis, Warder concluded that the disorder was hereditary and that much of what she had been told about it was incorrect: women could indeed develop hemochromatosis, and it was not only a disease of middle-age. Warder made it her mission to make the world aware of this disease, including an interview with Ida Clarkson on CHEK television.[14] For more than 28 years after that, except for a series of travel articles, Warder devoted her literary efforts to works about hemochromatosis. During this time she wrote The Bronze Killer, the first book devoted entirely to the subject of the genetic disorder, hemochromatosis. The term “Bronze Killer” has been used, among others, in the Toronto Star,[16] in British newspapers, in the magazine supplement of the Johannesburg Sunday Express[17] and in a Quebec French issue of the Reader’s Digest, where it is called “La tueuse au masque du bronze”. Having become acquainted with her, Hemochro-matosis was referred to as “the bronze killer” in an editorial by Clement Finch, Professor of Medicine Emeritus of the University of Washington, in the Western Journal of Medicine, September 1990.[19]
    In that same editorial, Finch says: “A strong case can be made for incorporating measurements of the plasma iron, iron-binding capacity and ferritin into the routine blood screen. Without such a survey, there is little hope of recognizing hemochromatosis at a time when treatment has the greatest promise.”[19] He also notes: “Lay Societies have been formed whose mission is to disseminate information about the “bronze killer”. . . Their information program is so effective that the people they reach are sometimes far better informed than their physicians.”[19] The Bronze Killer is recommended by professionals around the world.[20]

    Warder went on to found hemochromatosis societies in her native South Africa[1] and her adopted home of Canada.[2]
    Warder has also published more than 300 articles on the subject of hemochro-matosis, and as well as patient literature for individuals, hospitals and other medical facilities. Her newsletters and brochures have gone out to more than 16 countries.[2]

  • Tom Hennessy

    Quote: I am now keen to find out how much you know about the lady
    Answer: For someone ‘just’ happening across the blog “while seeking info about hidradenitis suppurativa” you seem to presume I am unable to figure out whom to I am speaking ? I actually spoke to Marie long ago about the proper English for the phrase , Man Is A Herbivore ,as opposed to Man Is An Herbivore like everyone seems to think is right. I started my ‘quest’ in ’92 because of something which I ran into during my life and so I have heard of the ‘Bronze Killer’ book but have never read it. I read medical studies and articles , the last book I read was Dr. Lauffers’ book , Iron And Your Heart , and that being twenty years ago. Has it been that long ? I probably would have read some of Maries’ work over the years due to the link of iron and disease as opposed ‘generally’ diet induced iron excess due to humans , as herbivores , eating meat and building iron and causing disease. If I seem to , take liberties , as in speaking to medical studies , which one might think Marie is well aware of , quite the contrary , most of what I speak to is quite novel to most , JUST like it was to Marie and HER ‘audience’ over the years. It was almost twenty years ago they made the link to malaria and only this year have they made the link , officially , of diet to the induction of sickle cell disease.
    MY ‘book’ was rejected for “too much non-original material” which was in regards to the references I had included in the book and so since I had more ‘references’ , medical studies , in the book it turned out to be MORE medical studies as opposed to actually ME ‘writing’. If you see what I mean. It destroyed me as a writer. Just kidding.

  • Marie Warder

    I discussed our exchanges of info with Professor Patrick Macphail – an acclaimed expert on the subject of HH – and this is what he had to say:
    “There is some truth in most of what is said but maybe some things have been taken to extremes – to make the point perhaps. There is nothing new in the age related accumulation of iron. Tom Bothwell’s group showed this years ago but whether it has clinical significance remains an open question. There is a tendency to extrapolate the undoubted dangers of too much iron in iron overload states to the much more modest accumulation seen in other conditions – including age. All this does underline the basic fact that free iron is toxic – why else would so many protective mechanisms have evolved to protect organisms against iron when stored or moving about?”

  • Marie Warder

    I don’t know where those odd little quotation marks came from? They were certainly not in the preview of the material I posted.

  • Marie Warder

    By the way to which which group were you referring when you wrote that you were rejected?

  • Tom Hennessy

    When I say my book was rejected , I meant it was rejected by the publisher. They wanted me to ‘pad’ the book with more original material and THAT wasn’t something I wanted to do. I realized at that point the only reason I wrote the book was to ‘get the message out’ and so I decided it would work just as well to put the book directly on the web , which I did. Akin to an ebook before ebooks became commonplace.
    I have become known as a ‘net kook’ and when THAT happens people seem to feel they can say and do whatever they ‘feel’ like to you and that disrespect led to many
    ‘heated’ exchanges.

  • Leslie Morantine

    Interesting reading here. Now you all have me wondering about build up of iron being the culprit of my HS. I had a hysterectomy 17 years ago but didn’t start with HS until 2006. I do eat red meat, chicken and fish. I think I will do some experimentation with diet as I’m at my wits end with this horrible condition. I will also look for someone I know in the health profession to get a copy of New England Journal of Medicine to review article referred to prior.
    Thanks,
    Leslie Morantine

  • Marie Warder

    Herewith an excellent link: Too Much Iron newsletter (PDF)
    Included is a link to the CHS and if you look at the various links on the front page of that you may find much that will interest you.

  • Marie Warder
  • Shari Lubbe

    I appreciate your article on this awful disease…HS. Both my brother and I inherited it from my mother so we believe it can be passed down.

    Since 2010 I have had 10 surgeries to try and remove some of my HS (groin, breasts, underarm and under my chin) and it’s sinus tracts. I am now on SSDI because of it.

    The more we can raise awareness the better chance others may have of it not getting so bad. I fear for my daughter but we know what to watch for after so many years of my family having HS.

  • Lisa

    This horrific condition can be controlled with diet. Gluten, grain and dairy free, along with eliminating nightshades (tobacco is a nightshade). I hope this helps fellow sufferers.

  • Marie Warder

    I am so grateful for all the comments I receive because I learn more every time any of you post one.
    I’ll be “off the air” for a few weeks as my computer is badly in need of help — and so am I.More bones crumbling by the day and the pain is excruciating so it’s agony to write.
    (Check out Chondrocalcinosis on Wikipedia.)