Endometriosis—How Big is the Problem?Chapter 1

From a medical perspective endometriosis is defined as the presence of endometrial-like tissue outside the uterus, which induces a chronic, inflammatory reaction, predominantly in women of reproductive age, from all ethnic and social groups.³

From a woman's perspective, however, endometriosis is a disease of many illnesses which is surrounded by taboos, myths, delayed diagnosis, hit-and-miss treatments, and a lack of awareness, overlaid on a wide variety of symptoms that embody a stubborn, frustrating and, for some, chronic condition. These women are given a life sentence and are trapped by endometriosis (Figure 1-1).

“In its worst stages, this disease affects the well-being of the female patient totally and adversely, her whole spirit is broken, and yet she lives in fear of still more symptoms such as further pain …”. These are the words of Louis Brotherson MD, written in 1776,⁴ and these words so accurately describe what many women with endometriosis face today—233 years later.

Unfortunately little has changed in the last 200 years. This is the problem!

This problem of endometriosis is further intensified when we remind ourselves that this disease is not only a significant problem for the woman, and her family, who has to deal with its impacts on a day-to-day basis.

The problem extends to the physician, who may struggle to aid this woman in getting her symptoms under control and restore her quality of life and, for some, fertility due to a lack of efficacious treatments; many of which have significant side-effects, which further compromise these women's lives.

In addition, from a societal perspective, endometriosis compounds into a much wider issue, where the cost of illness is estimated at more than US $ 22 billion a year in the USA alone. These costs are considerably higher than those related to Crohn's disease or to migraine – illnesses much better understood and socially acknowledged.⁵

Thus, the problem that is endometriosis cannot continue to be ignored – from any perspective.

Consequently these factors contribute to a diagnostic delay ranging from 4-12 years in various health care settings between symptom onset, presenting with symptoms, and a definitive diagnosis, where endometriosis-related infertility is typically diagnosed sooner than endometriosis-related pain, and with a variance in whether the woman was seen within the context of a public or a private practice.^8–11

This “normalization” of symptoms, where pain is seen to be “part of being a woman, mean that women tended to consider their experiences as normal, even when periods were recognised as problematic and disruptive to their lives. They considered themselves “unlucky” rather than ill. And, if the mother had had similar symptoms this tended to further delay these women seeking medical help.

At a medical level there will by definition always be somewhat of a diagnostic delay for the pure reason that a definitive diagnosis requires a laparoscopy – an invasive procedure. Nonetheless, Ballard showed that the delay in referral from primary to secondary care varied from 1 month to 22 years, with most women reporting multiple visits to their primary physician before referral to a specialist. Women were often “dismissed” by their primary physician, who also “normalized” their painful periods.¹¹

It is clear, however, that the diagnostic delay goes both ways, and to a large extent is due to a breakdown in communication between the patient and physician. Without mutual physician-patient rapport and trust, and time to ask questions and “tease out” answers, establishing the diagnosis can be difficult because the symptoms of endometriosis may be one of those things we don't allow ourselves talk about.

Abdominal pain and/or bloating, irregular or excessive menstrual bleeding, pain during sexual intercourse, abdominal pain, nausea, diarrhea, back pain, and urinary retention are not topics that are addressed over Sunday lunch with friends and family, nor—often through embarrassment—with a physician, and thus remain unsaid and unacknowledged.

Furthermore, in terms of obtaining a diagnosis of endometriosis, these exact symptoms listed above taken together, or in combination with other physical symptoms, describe a mental disorder called “somatization disorder” according to the American Psychiatric Association.¹²

Whereas it is essential to assess the psychological status of any patient, it can be very detrimental to a woman, whose complaint of pain is valid, to be too easily dismissed as a “psychological case” because an organic cause of her symptoms have not been identified due to a persistent overall lack of awareness of the (combination of) major symptoms that indicate that she may suffer from endometriosis.

Two and three may be interchangeable depending on definitions. Definitions, however, mean less to women with endometriosis; they want number one: they want to be symptom free!

Cure is, of course, the ultimate goal, but symptom management is more often the reality in endometriosis. Since evidence supporting the use of most medical therapies for endometriosis may not be as robust as we might wish to believe, and since the success rate of surgery is surgeon dependent,¹³ treatment failures within this field are subsequently common.

Following a diagnosis, many women are therefore subjected to a roller-coaster of “hit and miss” treatments, with or without acceptable side effects, and/or repeat surgeries, with a subsequent impact on their quality of life.

Prevention and cure remains elusive. The problem of endometriosis has not yet been resolved.

Women with endometriosis may also need to learn coping mechanisms and consider adopting lifestyle changes in order to create a life with a disease, which for many is long term and chronic. This is not ideal—yet may be reality for the time being, if they do not have access to specialist care.

Reminding ourselves that the problem of how to deal with endometriosis does not stop at recognition and diagnosis alone: disbelief may be an early emotion that is felt by the woman, closely followed by a fear of the unknown, just as Brotherson described in 1776.⁴

She may fear further tests and treatments, and experience anxiety when having to face unfamiliar medical tests or invasive procedures. This woman is entering a whole new world where she has to navigate the patient-doctor relationship, the bureaucracies of hospitals and insurance companies – whilst still having to attempt to keep her day-to-day life together on a personal and professional level and deal with the disruption this chronic illness brings in its wake. Denial, anxiety, and/or helplessness are all emotions that now need to be tackled in parallel with coping with pain and/or infertility.

Gomel, in his 2007 opinion paper, summarized this perfectly: patients arrive frustrated with themselves, their partners, and the health care system, having lost trust in physicians as a result of having consulted many without any satisfactory outcome. The initial consultation with many such patients who have become difficult to communicate with may also be frustrating for the physician. It is important therefore to assign appropriate time for the initial consultation of patients with chronic pelvic pain and to adopt an attentive and interested approach.⁷

Early, specialist care, through careful attention and communication therefore, may take women with endometriosis off the roller coaster of taboos, hit and miss treatments, and mis-communication and ensure they get early, timely, specialized care.

Thus, any practitioner must never hesitate to seek advice, where appropriate, from more experienced colleagues or refer the woman to a center with the necessary expertise to offer all available treatments in a multi-disciplinary context, including advanced laparoscopic surgery.^3,7

This referral becomes crucial as soon as surgery is necessary since it is well accepted that the first surgery is the most important one. Thus, choosing a skilled endometriosis surgeon is extremely important. Inadequate 6and/or incomplete surgery, where for example the removal of endometriosis is not carried out during the same procedure as the diagnosis, will make subsequent surgery much more difficult and will ultimately impair outcome for the woman – and make the job for the next surgeon even more challenging!

All of these practitioners, with their different sets of skills, may play an important role in providing a holistic solution to an individual woman's needs. Assessment of therapeutic effectiveness may not exclusively rely on clinical data, but should incorporate patient based outcome measures and a willingness to explore combination therapies. If all these cogs in this treatment and management wheel are well-linked, the likelihood of positive long-term results become greater.¹⁴

Information is the cornerstone for any patient to understand what is happening in her body and to enable her to make informed decisions about her health care. Only the woman herself can decide how she values the benefits and risks associated with any given treatment, at any given time, and therefore it is crucial that she partakes in the decision making process not only in terms of investigations but also in the choice of the treatment of available options.^3,7 A well-informed patient is much more able to deal with any potential side-effects that may occur. As mentioned above, the resentment and frustration, which many with chronic diseases develop over time, more often than not, result in a disconnect between clinical management and the patient's expectations— expectations, which may have left the woman unprepared for the possibility of recurrence after treatment. It is important to not lose sight of this.

For example, if a patient has been promised a 100%“cure”, and yet she finds herself only 80% symptom free following treatment, the outcome in the patient's view was not successful. However, if it has been communicated to her, that any given treatment may only give her 75% or even 50% relief, and indeed it does, then expectations have been met, even if that treatment has not provided a long-term curable effect. Therefore, setting realistic expectations is vital, although this can be difficult when dealing with someone who just wants pain relief – or a child – and wants it now!

It is only fair to the woman to let her know what her chances are.

Nonetheless, several hundred years after endometriosis was first described in the literature¹⁶ we are no further in terms of satisfactory treatments. For women with endometriosis it thus becomes paramount to come to terms with living with a potentially chronic disease: to 7face the life sentence of pain and/or infertility they may have been given. But, this in itself brings limitations that are a challenge for anyone to cope with. As one woman with endometriosis expressed: “When you are healthy you have many dreams – when you are ill you have just one…”.

In terms of coping a woman may be asked to indicate on a VAS scale of 1-10 how she feels (i.e. how severe is the pain?), but when it comes to coping, it may be more important to consider how she functions. Questions that may need to be asked include:

Her answers to these questions may assist her in “tailoring” her own response to her disease and to determine, which adjustments she needs to make in order to exist with endometriosis as part of her life. Because she is not given a choice as to whether she wants to have endometriosis or not, this is not necessarily an easy task to accept or to carry out. But for women with endometriosis it is necessary to accept this process and to ultimately find a solution, which enables her to accept that endometriosis may have limited some of her choices in life, and that her life has to be led with these limitations.

It does, however, bring us back to the topic of taboos. Because living a life of limitations exposes us to society and its perceptions of what is “normal” and what is not – and, what we choose/dare to talk about.

“Pain is a more terrible lord of mankind than even death itself” said Schweitzer.¹⁷ Many women with endometriosis will agree with this. Unfortunately pain is not well understood or accepted in society unless it is in connection with a physically visible injury: we feel sympathetic towards a person who has broken her arm; we will enquire about the healing process, when the cast comes off, and how the physiotherapy is progressing. After some six to eight weeks, the woman can report that her arm is back to normal, and everyone heaves a sigh of relief. A person gets sick, she gets treatment, and she gets better.

Except this is not always the case with endometriosis. Furthermore, because most people are not aware of chronic pain, persistent disease processes, or “invisible” illnesses, women with endometriosis will to a large extent have to “cope” with their pain alone.

With their pain alone— and deal with her challenges on her own because endometriosis is hard for others to understand.

Explaining the disease, with its difficult-to-pronounce name takes a degree of candor about her body, which not every woman possesses. It requires at least some discussion of female anatomy and menstrual cycles, which can be uncomfortable topics for both the ‘explainer’ and the ‘listener’.

Like pain, infertility is not a topic easily discussed at social gatherings, and thus can become a personal and private grief – a grief that is intangible because the couple's sense of loss is invisible. There is no funeral, no body, no flowers, and no messages of comfort; the pain and losses are invisible and subsequently difficult for others to understand. The couple may chose to not talk about their feelings to family and friends, and start avoiding situations that remind them of their infertility, including socializing with couples who have children.¹⁸

This combination of grief and the eternal hope of “maybe next month” should be borne in mind and acknowledged by those dealing with couples with endometriosis-related sub-fertility – even if such issues are not yet discussed socially and to a large extent remain a taboo in today's society.

When a disease affects an estimated 100 million women during the prime of their lives, the goal must be a future in which no woman's life is at risk of being compromised by endometriosis. Indeed a future in which these women's daughters should not fear what 10% of this generations' women have had to endure.

To achieve this we need to agree to a vision of a future in which millions of women are not prevented from fulfilling their dreams of completing their education, maintaining a career and having children because of a disease which could be prevented if only adequate funding for research was available.

This cannot happen without all stakeholders collaborating: i.e. women with endometriosis, clinicians, scientists/researchers, governments, and industry must come together.

The problem that needs solving is that collectively we need to find a way of taking care of women with endometriosis to safeguard our future. In other words: without significant investment into causal research it becomes impossible to safeguard that future. Developing treatments that actually work will for women with endometriosis mean preservation of their fertility, improved quality of life, and reduced socio-economic costs – and a life without pain.

Today, effective disease management requires a multidisciplinary treatment approach carried out within a specialized setting, where realistic expectations are well communicated. For now, women with endometriosis may also have to learn coping skills and consider lifestyle changes in order to create a life with a disease, which for many is long term and chronic.

However, it is said that to every problem there is a solution. For endometriosis this solution remains elusive; but, through global collaboration, prioritisation and coordination of research, and an open and inquisitive mind, we may just get there!

In conclusion: to solve the problem of endometriosis we need to apply and coordinate all of our faculties. The Danish philosopher, Piet Hein, summarized this perfectly:

With such commitment we can challenge the problem that is endometriosis and find a solution to improve the lives of those affected by the disease. With such commitment we can release these women – and future generations of women – from a life sentence of pain.

Until this happens, however, endometriosis will remain an unnecessary problem for too many millions of women worldwide.