Renee's Thoughts

I have a "rare" skin disease called Hidradenitis Suppurativa. This blog is about my struggle with this disease. Additionally, I am a Domestic Violence Victim & Survivor. I will be including entries regarding Abusive Relationships. Although this blog has a serious tone to it, I hope to be able to incorporate some fun stuff into the blog as well. Thanks!

Thursday, February 09, 2006

Hidradenitis Suppurativa - The Need for Answers

Hi everyone ... The entry contains a paper that has been written by a fellow HSer!  This paper has some very good information regarding this disease.  I am posting the paper in it's entirety (with Peter's permission, of course), so that you might be able to gain a better understanding of this disease, how the medical community views it, and what we (the sufferers) contend with on a daily basis.  I hope that you will gain some new information on this disease & perhaps it will help someone who has this, but doesn't know what it is ... ((((HUGS)))) Renee


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Hidradenitis Suppurativa?

"The Need for Answers"

Hidradenitis Suppurativa?
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.
Hidradenitis Suppurativa
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 as a preface to the discussion paper:

"The Need for Answers"

By Peter J Farrington (2005)
(Author & fellow H.S.Sufferer)



Hidradenitis Suppurativa

"The Need for Answers"


(A patient's perspective based on detailed analysis of the available information and research about Hidradenitis Suppurativa Worldwide)

Introduction

Despite the fact the existence of HS has been known for over 160 years, little is still known about the disease. Sufferers have often been left isolated and alone to battle with the extreme morbidity of the disease and the dramatic impact it often has on their lives.

The accepted comparative rareness of the disease, combined with the lack of reliable information about its progression or even precise cause, has compounded the problem as, even when correctly diagnosed, the H.S. 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.

Understandably this has led to sufferers feeling let down, neglected and most of all ignored by governments, the medical profession and the pharmaceutical companies. Add to this the 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 believing HS is just a form of acne and treating us accordingly despite the growing evidence this is clearly not the case, and you will understand our extreme anger and frustration as sufferers.

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 us fall or feels our pain and often even those closest to us are sadly all too often unaware of our unsightly and painful plight.

The internet is helping as now, despite our isolation, individuals as far apart as a small village in southern England or from Ohio in the heart of the USA or even all the way from the other side of the world in Australia and New Zealand can meet and discuss our common experiences and finally realise we are not alone.

Between the memberships of the various HS support sites worldwide we now have access to thousands of years of experience being victims of this pernicious disease and, from that collective knowledge, we are perhaps now in a better position than anyone else to truly appreciate and comprehend the problem in a way no others can. As a group we are therefore no longer willing to accept being ignored, neglected and let down. We have a voice now and we will be heard!

It is therefore as sufferers, with the added collective insight into the disease that brings, that we present this review of the sparse information currently available about the life altering disease which is Hidradenitis Suppurativa. Beyond this special insight we make no claim to be any more or less accurate in our conclusions about prevalence, cause or any other aspect of H.S. than those who have gone before us.

We make therefore make no claim to have any of the answers, but there can no longer be any doubt whatsoever that we most certainly know the questions which now urgently need to be answered.

Arrangement of Sections

The aim of this discussion paper is to provide a collective patient's perspective on the currently available information and research which will prompt governments, the medical community and the pharmaceutical industry to finally sit up and not just listen, but actually carry out the clearly urgently needed further research into this condition.

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 paper will therefore address each of these key issues in turn in what we hope is a logical way that enables those with the resources needed to ask the questions we pose to make those resources available and also provide those who have the ability and expertise to seek the answers to those questions the motivation 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.

We have therefore also included 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.

Author's Note

Inevitably the content of parts of this paper has been shaped by my own 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 many discussions with 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 (September 2005.)

Section 1: 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

Hidradenitis Suppurativa (The clinical perspective)

Historical Background:

Leper provided the first descriptions of Hidradenitis Suppurativa in 1839, when he noticed a peculiar localization of abscesses to the axillary and the perianal skin. Verneuil first described the apocrine involvement in 1854.

Lane and Brunstig described the clinical features of the disease. Experimental reproduction was achieved by Shelly and Cahn in 1955, which helped establish the pathogenesis of the disorder. Further work by Conway, Palette, Pollock, Letterman, and others in establishing the surgical management of the disease has been noteworthy.

General Description:

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.


Staging
: The disease can be divided into the following 3 clinical stages:

Stage 1: Single or multiple abscesses form, without sinus tracts and cicatrization.

Stage 2: Recurrent abscesses form, with tract formation and cicatrization. There may be single or multiple widely separated lesions.

Stage 3: Diffuse or near-diffuse involvement or multiple interconnected tracts and abscesses are observed across the entire area.

Age: Hidradenitis Suppurative usually begins in the post-pubertal age group, when the apocrine glands start developing. It is most common in the third decade of life, but the untreated disease may persist into the seventh decade.

Sex
: The condition is more common in women. Submammary, axillary, and inguinal involvement is more common in females, whereas the perineal form is more common in men.

Pathophysiology:

In the initial pre-destructive stages, keratin comedones are observed in the apocrine gland follicles, along with inflammatory cells around the apocrine glands and distended ducts filled with leucocytes and secretions. Later, groups of cocci may be observed within the gland and in the dermis, indicating infection and abscess formation. Some evidence suggests that the occlusion of abnormal hair follicles may lead to the initiation of these changes.

The suppuration later extends into the adjacent and subcutaneous tissue, where there may be chronic inflammatory cells involving histiocytes and giant cells around the apocrine gland remnants and the keratin plugs.

The chronic process leads to dense fibrosis and sinus and fistulous tracts lined partly by granulation tissue and partly by squamous epithelium.

Clinical Description:

The onset of disease is usually after puberty, in the second and third decades, when the apocrine sweat glands start developing. It begins in the epithelium containing apocrine and is more common in the neck, axilla, and the submammary region in females and in the perineal skin in males. The perineal disease tends to be more severe.

The early lesions are solitary, painful pruritic nodules that may persist for weeks or months without any change. If subcutaneous extension occurs, it may appear as indurated plaques, which in lax skin, such as the axilla and groin, manifest as linear bands. Multiple sites may be simultaneously affected.

The nodules develop into pustules and eventually rupture externally, draining purulent material. Healing occurs with dense fibrosis, and recurrences crop up in and around the original site. This leads to chronic sinus formation, with intermittent release of serous, purulent, or bloodstained discharge. Ulceration sometimes occurs, and the abscesses may burrow and rupture into the neighboring structures. Episodes of acute cellulitis are sometimes a feature and are accompanied by fever and toxicity.

Regional lymphadenopathy is characteristically absent. Chronic axillary hidradenitis suppurativa usually causes a reduction of the normal axillary odor.

Severity and course of the disease are variable, but untreated hidradenitis suppurativa is typically a relentless progressive disease with acute exacerbations and remissions that lead to sinus
tract formation and marked scarring.


Histologic Findings:

Early lesions show keratinous obstruction of the distal apocrine ducts, with dilatation and rupture of the adjacent tubules. Neutrophilic infiltrate is usually found within the tubule initially; after rupture, infiltrate is found in the surrounding tissue.

Chronic disease shows lymphocytic infiltration, granulation tissue, fibrous tissue, sinus tract formation, pseudoepitheliomatous hyperplasia, and obliteration of the glandular elements as the
disease progresses.

While the earlier lesions are diagnostic of Hidradenitis suppurativa, the later features can also be produced by severe cystic acne and by diseases that cause chronic sinus tract formation.

Complications:

The complications of long-standing untreated disease include the following:

• Fistulae formation into the urethra, bladder, rectum, or peritoneum has been reported.
• Sequelae of chronic infection such as secondary anemia, hypoproteinemia, amyloidosis, and renal disease can occur in chronic disease.
• Peripheral and axial arthropathy are possible.
• Chronic malaise and depression are observed.
• Scarring of the tissue can lead to lymphatic obstruction and lymphoedema of the limbs and arthropathy.
• Marjolin ulcer (squamous cell carcinoma) has been reported in long-standing chronic disease.
• Scrotal elephantiasis has been reported.

Medical Therapy:

Treatment depends upon the stage of the disease. Early lesions are usually treated by medical therapy, whereas the patient with long-standing indolent disease requires surgical therapy.* Non-specific treatment measures include good hygiene, weight reduction, use of antiseptic detergents, and avoidance of tight-fitting clothes.

Outcome and Prognosis: (Post surgery)

Locoregional recurrence does arise, and reported rates vary by 30-50%. One series reports a 100% recurrence rate with incision and drainage alone, 42% recurrence with limited excision, and 27% recurrence after radical excision.

Reassessment for several months in the follow-up clinic is required before cure can be assumed with certainty.

* N.B. More detailed descriptions of the various acute and chronic relapsing stage treatment options from Naveen Pokala's full article at eMedicine are given in section 3 of this paper.

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.

For the first of these I need to borrow a slogan more often used in real-estate.

"Location, location, location."

I am talking here about the specific locations the "alien" invaders choose to infest as Hidradenitis, being a disease of the apocrine follicles, follows the distribution of apocrine glands themselves.

Hidradenitis suppurative is therefore found in the following areas:

• Skin-bearing apocrine glands
• Axilla
• Groin
• Perineum
• Perianal region
• Buttocks
• Scrotum
• Submammary region

This medically correct, inoffensive, polite, but, emotionally sterile list, belies the full impact on individual sufferers as these areas are, without doubt, the most embarrassing and intimate parts of the human anatomy to have any problem with, let alone infestation with the soul destroying "aliens" already described above.

Remembering another well know slogan "Don't Die of Ignorance" which was 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 still 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. Such a hidden condition often leads to a failure of appropriate allowances being made by friends, family and most of all employers.

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. Notwithstanding this, they 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 visible in a bathing suit. Even after an individual lesion eventually heals it tends to leave unsightly and clearly visible scars.

The list, however, also includes some areas that one can never avoid being public other than by becoming a recluse. The first item on the list includes the scalp, face, ears and even eyelids and, although it is relatively less common to have 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 also 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, 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 a broad range of severity, and thus impact, on individual sufferers, both in terms of the individual episodes 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 descriptions given so far are, however, appropriate, to some degree at least, for all Hidradenitis sufferers. I have also not yet touched on the full impact of how the disease tends to progress over time.

Returning now to the general description given earlier it continues:

"The disease tends to become chronic and indolent because of subcutaneous extension leading to induration, sinus, and fistula formation."

As can be seen from the full clinical definition, Hidradenitis is also recognised as having three distinct and identifiable stages linked to these factors.

Stage 1: Single or multiple abscesses form, without sinus tracts and cicatrization.

Stage 2: Recurrent abscesses form, with tract formation and cicatrization. There may be single or multiple widely separated lesions.

Stage 3: Diffuse or near-diffuse involvement or multiple interconnected tracts and abscesses are observed across the entire area.

Though questions still remain about the inevitability or not of such progression, sufferers understandably have to come to terms with the fact it probably will be in most if not all cases. Given the current state of knowledge and lack of fully effective treatments the best we seem to be able to hope for right now is to delay any such progress.

Clearly the stages themselves are also important as they define not just the changing nature of the condition but will also have a major influence on the treatment options which are available.

From the sufferer's perspective I would suggest it is more important if one has mild, moderate or severe versions of Hidradenitis rather than which specific stage one is categorized as. 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 insight into the nature of Hidradenitis for us as sufferers it will at least increase awareness of the true nature of the beast.

Friday September 23, 2005 - 02:36pm (EDT)

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