|"Hidradenitis suppurativa is a chronic, relapsing, suppurative cicatricial disease occurring in the apocrine follicles. The disease tends to become chronic and indolent because of subcutaneous extension leading to induration, sinus, and fistula formation."|
For laymen, and sadly even some medics too, hidradenitis suppurativa can simply be described as 'boils' or 'adult acne'. However, neither of those descriptions comes within a mile of the reality of what HS actually is for those that suffer from it.
Despite the fact the existence of HS has been known for over 160 years, little is still known about the disease. Sufferers are often left isolated and alone to battle with severe disease and the dramatic impact it often has on their lives. The rareness of the disease, combined with the lack of reliable information about its progression or even its precise cause, has compounded the problem - even when correctly diagnosed, the HS sufferer is still left with more questions than answers. The situation for those undiagnosed or misdiagnosed is, of course, even worse as they don't even have a name for their pain.
This has, quite understandably, led to sufferers feeling let down, neglected and most of all completely ignored by governments, the medical profession and pharmaceutical companies alike. Add to this the resulting lack of any reliable or effective treatments, as a result of the abysmal lack of detailed research into the true nature of the disease leading many doctors still wrongly believing HS is just a form of acne and treating it accordingly, despite the growing evidence this is clearly not the case. It is easy to understand the anger and frustration of those suffering from the disease.
Isolated and alone, most HS sufferers have not been able to get their voices heard. Just like a single ailing tree falling in the giant forest of the general population, nobody hears them fall or feels their pain, and often even those closest to them are sadly all too often unaware of their unsightly and painful plight.
The internet is helping as now, despite their isolation, individuals around the world can meet and discuss our common experiences and finally realise we are not alone. The various HS support sites worldwide have together accrued thousands of years of experience of living with this pernicious disease and, from that collective knowledge, sufferers are perhaps now in a better position than anyone else to truly appreciate and comprehend the problem. As a group they are no longer willing to accept being ignored, neglected and let down.
It is with the unique insight of a sufferer that an h2g2 Researcher has compiled a review of the sparse information currently available about the life-altering disease that is hidradenitis suppurativa. This entry does not claim to have all the answers, but should certainly highlight the questions that now urgently need to be answered.
Every aspect of the disease still has important unanswered questions falling into three distinct but interconnected areas.
1. The Nature of the Beast. (Clinical and sufferer's perspectives)
2. Quantifying the problem. (H.S. morbidity & prevalence statistics)
3. The Etiology, Diagnosis and Treatment of Hidradenitis Suppurativa.
This initial paper seeks to address the first of these key issues in a way that prompts those with the resources needed to ask the questions we pose to make those resources available and also provide the required motivation those who have the ability and expertise to seek the answers to these questions to undertake that work with our active participation and cooperation making use of our collective knowledge as sufferers to aid that process.
Even if successful, this process will clearly take some considerable time to achieve significant results. For those currently afflicted with the condition some action is urgently needed right now though, even if this is only a "band-aid" to ease the main symptoms we suffer even if not provide an effective cure for the condition itself.
Sadly, as will be seen from our collective observations as sufferers throughout this paper, the single most common symptom experienced by those with H.S. is the extreme difficulty we have had in finding those rare and special medical professionals fully aware even of the scant information currently available, let alone finding those who can translate that awareness into truly compassionate and effective case management and treatment commensurate with the full impact this dreadful disease so often has on our lives.
I am therefore also planning a postscript to this discussion paper in the form of a proposal for a new "contract" between ourselves and the medical professionals who treat us, based on our vast collective experience as sufferers, but also recognising that, despite the all too frequent shortfalls in appropriate diagnosis and care, there are still many shining examples of "best practice" which can be learnt from and replicated to the benefit of sufferers and physicians alike.
The Nature of the Beast (Defining Hidradenitis Suppurativa.)
Before looking in detail at what Hidradenitis Suppurativa means from a sufferer's perspective, it is perhaps useful to first refer to the accepted clinical descriptions of the condition in order to set the scene and provide suitable points of reference for that perspective.
There are many different "textbook" descriptions of Hidradenitis Suppurativa, many of which are woefully inadequate, which may explain some of the common misconceptions held not only by some sufferers but also lamentably by many members of the medical community.
Of the many available medical sources, I have decided to use extracts from the excellent eMedicine article about Hidradenitis Suppurativa written by Naveen Pokala, MBBS, MS, FRCS, Staff Physician, Department of Surgery, Bronx Lebanon Hospital, as it is clearly one of the most comprehensive, accurate, well researched and importantly up to date medical sources currently available.
For full article see: http://www.emedicine.com/med/topic2717.htm
If you want to know what they say go have a read then come back. LOL
Hidradenitis Suppurativa (The Sufferers perspective)
Text-book definitions are all very well, and even for lay-people can provide a valuable starting point in learning about the disease, but the reality of any medical condition is that only those that actually suffer from it fully appreciate the true nature of the beast.
Taking the first half of the "General" clinical description:
"Chronic, relapsing, suppurative cicatricial disease occurring in the apocrine follicles", or more often used, "Recurrent Cysts & Boils".
Imagine, if you can, how you would feel if out of nowhere strange and painful lumps began to appear, as if surgically implanted deep below the surface of your skin and without the benefit of an anaesthetic.
Sometimes they will be small but more often huge in size, ranging from pea sized ones that itch and burn, to golf ball size ones that stretch and tighten the surrounding skin with increasing discomfort and pain reaching levels no ordinary mortal should ever have to bear.
These are just the teenagers though and over time these "aliens", as we call them, often grow and grow and can eventually become the size of baseballs. The pain these ones cause is indescribable and radiates far wider than the lump itself as the unrelenting pressure forces the whole area surrounding the uninvited "alien" to also become inflamed.
Imagine how any one of these lumps or lesions would feel if you had to endure it for say an hour or two. Now stop and think how it would feel after a week and then a month and try to take in the reality for us is that these alien invasions or "flares" can, and often do, last for several months or even years at a time.
Remember too that we are not talking about individual one off cysts here, but all too often savage outbreaks with two, three or anywhere up to a dozen or more such horrors of all sizes invading our bodies at the same time. And then, as if this by itself were not enough, the putrid, suppurated (puss filled) content of the individual "aliens" will eventually and inevitably need to find a way to escape.
Picture if you will the famous scene from the movie "Alien", you know the one where John Hurt's character has been hosting an alien which suddenly decides it's time to break free while he is sitting enjoying a meal. The pain, the anguish and the sheer horror of that scene has entered movie folklore not least as part of the effect was created by deliberately not pre-warning the remaining members of the cast what was about to happen before they shot the scene.
OK at first sight this may appear to be a rather melodramatic way to describe the eventual bursting of our particular "aliens". But trust me, the eventual end result often looks and feels exactly the same to us, complete with the gaping hole, the blood and gore, and something that even the movie couldn't communicate, other than by the disgust shown by the rest of the crew, and that is the awful stench involved.
I hope from this you now have some small inkling of what the reality can be for us of a single episode or flair as Hidradenitis sufferers, but what the textbooks often describe as simply "recurrent cysts or boils" and even some physicians still call (and treat) as acne. I say small inkling because our story does not end there and there are many other important aspects to this disease which also come into play.
To borrow a slogan more often used in real-estate.
"Location, Location, Location."
In this case the specific locations our "alien" invaders choose to invade and infest us as Hidradenitis, being essentially a disease of the apocrine follicles, follows the distribution of the apocrine glands themselves which are found in the following key areas:
• Skin-bearing apocrine glands
• Perianal region
• Submammary region
This medically correct, inoffensive, polite but emotionally sterile list however belies the full impact on individual sufferers as these areas are also without doubt the most embarrassing and intimate parts of the human anatomy to have any problem with let alone suffering the invasion and infestation of the soul destroying "aliens" described above.
Remembering another well know slogan "Don't Die of Ignorance" used to great effect as part of the HIV/Aids awareness campaigns, I am tempted to suggest an appropriate slogan for Hidradenitis could be, "Don't die of embarrassment".
Many are so scared what others will think about them, should they admit to having such evil suppurating open sores in such intimate locations, they may often delay or even avoid seeking the appropriate medical advice and treatment.
Sadly that fear is often fully justified though as it is not uncommon for the initial reaction, even by many doctors, to be the suspicion that the lesions are connected to some sexually transmitted disease. When that initial wrong impression is then perpetuated to the point the first action by the doctor is a referral to an S.T.D. clinic, the emotional effect on the sufferer can be devastating.
You might think such errors are rare but research shows otherwise, as can be seen from Jemec's Danish studies which identified that over 4% of the patients being treated at an S.T.D. clinic showed clinical signs of Hidradenitis Suppurativa but had not been diagnosed as such.
I will return to this specific research in more detail in section two of this paper as the implications of that particular study are often misinterpreted in estimates of prevalence and lifetime risk. The key point is many sufferers are not just misdiagnosed but that the errors made often have dramatic emotional as well as clinical consequences.
Even where misdiagnosis is not a problem there is an important gender issue here in terms of the location of the lesions. As Hidradenitis is predominantly a condition suffered by women the corresponding under representation of women in the medical profession effectively provides yet another situation where women are often forced to suffer the indignity of exposing intimate areas of their bodies to men.
Whilst the intimate location of the majority of lesions allows both male and female sufferers some privacy from the world at large, this is not always the case and is also not always an advantage. Clearly the last six locations on the list, and to a lesser degree the second as well, are those normally hidden from view in everyday life but such a "hidden" condition often leads to a failure of appropriate allowances being made by friends, family and most of all employers.
Notwithstanding this, the locations do still present restrictions on everyday activities, like swimming and others where one usually wears far less clothing. The reason for this is lesions in both breast and genital areas usually spread out into the surrounding areas which are normally visible in a bathing suit and even after lesions eventually heal they tends to leave unsightly and clearly visible scars.
The locations also included some areas that one can never avoid being public other than by becoming a complete recluse as the first item on the list includes the scalp, face, ears and even eyelids and, although it is relatively less common to have large lesions in these areas, many of us, including myself, often do.
At a psychological level though, it actually makes little difference whether the outward signs of Hidradenitis are detectable or not. We know the foul smelling, puss and blood leaking lesions are there, and inevitably for many sufferers this creates the perception that others are not just always aware of it but are disgusted by it.
This perception, however true or false, impacts greatly on the self image of sufferers and is often part of a vicious circle of depression and stress which, because the apocrine glands become more active at such times, leads to an exacerbation of the condition itself.
The problems with coping with the outside world are difficult enough but the impact on more intimate relationships can be devastating. Leaving aside the impact of depression, the mere physical location of the lesions frequently makes sex a practical impossibility, even if a sufferer and their partner are able to overcome such psychological barriers.
This also assumes the sufferer has a partner in the first place, of course, as forming such close relationships with new people obviously presents other problems over and above such physical considerations.
Before moving on to the second half of the "general" description used by the medical community to define Hidradenitis Suppurativa there is one last aspect to consider and that is the impact of the relapsing but recurrent and chronic nature of the disease.
At first sight one might well believe a condition which has periods of reduced intensity, and even full remission for brief periods of time, might bring some relief to sufferers. However, the reality of this pattern for us as sufferers is that it just creates a physical and emotional rollercoaster ride where we are constantly at the mercy of the disease which seems at times bent on our destruction by making each successive rise and fall far higher and faster than the last.
As with most if not all medical conditions, there is also a broad range of severity, and thus impact, on individual sufferers, both in terms of the individual episodes we experience and the overall course of the disease. This ranges from mild to moderate all the way up to extremely severe in some unfortunate cases. The general descriptions given so far are though appropriate, to some degree at least, for all Hidradenitis sufferers but we have not yet touched on the full impact of how the disease tends to progress over time for some.
As can be seen from the disease tends to become chronic and indolent over time and is recognised as having three distinct and identifiable stages which are important as they define not just the changing nature of the condition but will also have a major influence on the already rather limited treatment options which are available.
Though questions remain about the inevitability of such progression, sufferers understandably do have to come to terms with the fact it probably will be so for them and given the current lack of effective treatments the best we seem able to hope for currently is to try to delay the progression. From the sufferer's perspective though I would suggest it is far more important if one has mild, moderate or severe versions of Hidradenitis rather than which specific stage one is categorized in.
In fact for us there are actually three far more important "stages" to having the condition and these are as follows.
• Stage 1: Pre-Diagnosis phase
• Stage 2: Diagnostic phase
• Stage 3: Post-Diagnosis phase
To be fair to the medical profession, in the absence of a definitive diagnostic test, mild to moderate clinical stage 1 Hidradenitis will always be extremely difficult to confirm, not least as it is entirely possible that a patient is suffering from an ordinary individual cyst or boil which is not that uncommon in the general population.
Our main frustration is that, far too often, our medical history and current symptoms will show clear evidence of advanced stage 1, 2 or even in rare cases stage 3 of the disease but, despite this, we are still misdiagnosed. A further frustration is that in many cases even when the "professionals" are aware we have the condition, they do not deign to pass on that vital piece of information to us as patients.
Ultimately the problem for us, and equally for the professionals that treat us, is that even when we have finally been given a diagnosis there is little that can be done for us. We hope by providing this deeper insight into the nature of Hidradenitis for us as sufferers it will at least increase awareness of the true nature of the beast which originally inspired the following poem.
"The Need for Answers"
But then what's in a name?
Having you is worse by far
But the questions still remain.
What causes you to happen?
What makes you have to stay?
Why can't we find a cure for you?
Why won't you go away?
How many have you stricken?
How many feel the pain?
When Hidradenitis Suppurativa
It's you that is to blame.
The life we knew erodes away
never more to be the same.
You really are a shame.
You rob of us of our dignity.
You rob us of our lives.
Few doctors seem to know or care.
And most that do use knives.
Hidradenitis doesn't answer,
know its victims, much less care.
It just grabs hold and won't let go.
Hidradenitis Suppurative just simply isn't fair.
So let's all get together as family and friends.
Find answers to the questions on which we all depend.
And by our collective strength and will
Bring Hidradenitis Suppurativa to its end.
Original poem by Michelle Billingsly (2003/5)
Adapted By Peter J Farrington as an addition to the discussion paper: "The Need for Answers" with the full permission of the original author who is a long time friend and fellow sufferer of HIdradenitis Suppurativa.
Inevitably the content of parts of this paper has been shaped by my own personal experience suffering H.S. as a 50 year old, white, British male living in the United Kingdom and treated under the N.H.S.
I have, however, tried my best throughout to ensure that the "voice" I use to tell our story is the collective "voice" of as wide a range of individual sufferers as possible based on my discussions with many fellow sufferers and a review of the vast historical message database and survey data available from the growing number of Hidradenitis Suppurativa support groups that now exist worldwide.
Peter John Farrington (June 2009.)
NB: Further entries are planned for later covering H.S. morbidity & prevalence statistics and also exploring the Etiology, Diagnosis and Treatment of Hidradenitis Suppurativa.
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