Endometriosis is a chronic estrogen-dependent disease that can potentially cause incapacitating pain, organ failure, infertility, and other severe medical consequences. It was described in medical literature more than 300 years ago and was even alluded to in ancient medical texts from nearly 4,000 years ago. And, far from being a rare disease, it’s estimated that as many as 200 million women and pubescent girls from around the world have endometriosis.
For centuries the symptoms of endometriosis have been dismissed as a supposedly normal part of womanhood, nothing more than painful periods or cramps. However, endometriosis is a serious disease which can lead to serious medical consequences if inadequately treated. In fact, endometriosis actually shares some features with non-fatal cancers, such as the ability to spread throughout the entire body, potentially causing irreparable damage and disability (for an example of a non-fatal cancer, see http://www.ncbi.nlm.nih.gov/pubmed/11880756). Recently, one study even cited endometriosis as one of the top ten most painful medical conditions.
WHOLE-BODY SYSTEMIC DISEASE
While it’s true that extreme, incapacitating pain with menstruation is one of the most common symptoms of endometriosis, it can also cause chronic and disabling pain in essentially any region of the body and at any time during the month. Subfertility/infertility, excruciating pain during sexual intercourse, silent kidney loss, searing lower back, hip and leg pain, and severe bowel and bladder dysfunction are among other symptoms experienced by women with endometriosis. Endometriosis has been found in every organ and anatomical structure in the body except the spleen. However, the lower abdominal cavity (pelvic cavity) is the most common general area where endometriosis occurs, and the most common cites include the peritoneum (lining of the pelvic area), rectouterine pouch (also called the Pouch of Douglas or cul-de-sac), rectovaginal septum, uterosacral ligaments, ovaries, fallopian tubes, all over the outside of the uterus, including underneath it and behind it, the appendix, bowel, bladder, and rectum. Meanwhile, adenomyosis, thought to be at least a cousin of endometriosis, if not simply another form of the disorder, occurs when endometrial glands, putatively from the basal layer of the endometrium, invaginate into the muscular wall (myometrium) of the uterus itself.
In the broadest terms, many researchers hypothesize that the body’s immune system recognizes that these endometriotic growths are not growing where they should and therefore launches an immune response in order to destroy them. (Others believe that the immune system does not recognize them until it’s too late). As a result of this continued battle between endometriosis and your body’s immune system, the cytokine-prostaglandin response, inflammation, and other immune system factors appear to become chronically dysregulated.
CLASSIC VICIOUS CYCLE
It’s a classic vicious cycle. Partially as a result of this immune response and other factors, severe pain with menstruation, chronic pain independent of menstruation, inflammation, subfertility, infertility, tissue necrosis, and scar tissue (adhesions) can develop. In severe cases, endometriosis causes such extensive damage that serious complications ensue, like life-threatening bowel obstruction, bladder dysregulation, kidney dysfunction, silent loss of kidney, collapsed lungs, and destruction of the ovaries and fallopian tubes.
REPROGRAMMING GENETIC PATHWAYS
Meanwhile, scar tissue formation also damages organs by encasing them together in abnormal ways -in a sense strangling them – which, in severe cases, leads to the so-called frozen pelvis which can cause chronic pain and even loss of organ function. Like the aberrant agents of destruction that they are, it appears that these clever little endometriotic growths can also reprogram genetic pathways, allowing them to continue migrating throughout the body and even produce their own hormones, nerves, and blood supply. It’s as if endometriosis is determined to live and will do anything to ensure its own survival. In this sense, endometriosis behaves in similar ways as some cancers, even though it’s classified as a benign condition. (Endometriosis is, however, associated with an increased risk of certain cancers). A combination of genetic predisposition, epigenetic mutations, stem cell dysregulation, immune system dysfunction, and environmental triggers have all been proposed as potential factors in the pathogenesis of endometriosis.
PERNICIOUS, PROGRESSIVE DISEASE
Through all of these potential pathways, endometriosis can be a progressive disease; that is, it can continue to grow throughout the body and become worse, despite medical and/or surgical interventions and even if the uterus and ovaries have been surgically removed. (This is not to suggest that these growths move or travel; rather, it may be that these cells are in place from birth and are activated through certain environmental, epigenetic, immune, stem cell, or other unknown factors. But, there is no scientific consensus on the etiology, so these are still under the category of hypothesis).
Unlike normal endometrial cells found in the lining of the uterus, these errant endometriotic growths do not get expelled from your body each month as a period. Instead, many (but not all!) of these aberrant growths implant and begin reacting to the monthly hormones that trigger menstruation, causing them to bleed and shed and grow, month after month and year after year if left untreated. Although these endometriotic growths are benign (not cancer), it appears that the body still recognizes that they shouldn’t be growing outside of the uterus, and therefore usually launches an inflammatory response in order to try to destroy them. As a result, the affected areas become extremely inflamed and therefore potentially extremely painful. This chronic pro-inflammatory environment eventually leads to elevated cytokine-prostaglandin levels which contribute to the chronic pain. Blood and pus-filled endometriotic cysts may also form, which can become twisted (called torsion) and/or burst open and cause still more pain, bleeding, infection, and another cascade of acute inflammatory responses. Endometriosis can also grow directly onto nerves, which can cause excruciating pain, similar to the way that the nerve-invading disorder shingles does.
As mentioned, endometriotic growths can generate their own supply of blood vessels and nerves, which increases the number of pain receptors and therefore contributes to heightened pain responses. Eventually, scar tissue and other symptoms of endometriosis develop. In fact, scar tissue itself can cause severe pain. For example, in cases when endometriosis has invaded the ureters, scar tissue can cause these organs to close up (constrict), which can lead to severe kidney infections and an inability to completely void when urinating. Bowel endometriosis, on the other hand, can cause severe bowel obstructions and/or tiny perforations (holes), allowing the contents of the bowel to leak out into the pelvic cavity, which can cause an extremely painful, life-threatening medical emergency. In severe cases endometriosis can even completely destroy organs. For example, while most know that endometriosis can totally destroy the ovaries, in rare cases some women have even lost a kidney due to this confounding disorder. In rare cases endometriosis has been found in or on the heart, brain, skin, spine, eyes, liver, kidney, and lungs.
COMORBIDITIES AND/OR UNRECOGNIZED SURGICAL COMPLICATIONS
Other incredibly confounding aspects to treating those with endometriosis is that there can be a host of comorbidities or unrecognized surgical complications that the patient may have, which can also cause chronic pelvic pain. For example, ovarian remnant syndrome, non-endometriotic ovarian cysts,adenomyosis, ovarian torsion, ovarian hyperstimulation syndrome, adhesions, vulvadynia, chronic obstructive uropathy, fibroids,degenerating fibroids, fibroid torsion, pyelonephritis, fistulas, hernias, post-hysterectomy disorders, appendicitis, inflamed fallopian tubes (endosalpingiosis, salpingitis, etc.), ureterohydronephrosis, post-surgery pain from unrecognized complications, endometritis, adhesion-induced bowel obstructions, post-surgery bowel disorders, abdominal wall disorders, irritable bowel syndrome, severe proctitis, rectovaginal fistulas, rectus sheath hematoma, celiac disease, diverticulitis, colitis, Chrohn’s disease, gastroenteritis, ulcerative colitis, urinary tract obstruction, hydroureter, interstitial cystitis (also called painful bladder syndrome), bladder cystocele (prolapsed bladder),rectocele (prolapsed rectum), other prolapsed organs,neurogenic bladder, other neurogenic pain, other genitourinary disorders, pain from the muscles of the abdominal wall, bladder, or bowel, other pain from other muscles or joints (myofascial pain), pelvic floor disorders, pudendal neuralgia, , urethral disorders, ectopic pregnancy, post-childbirth complications, pelvic inflammatory disease (PID), other infections, chronic pelvic pain syndrome, endometrial hyperplasia, cervical stenosis, irradiation damage, infected or displaced intrauterine device (IUD), gynecologic malignancies, and many other conditions can be associated with chronic pelvic pain or co-exist with endometriosis.
Suffice it to say, this is certainly an unwieldy list, and, it isn’t even comprehensive! With so many potential disorders to sort through, this is why we encourage you to seek out a chronic pelvic pain specialist with expertise in recognizing these and dozens of other disorders that may be contributing to your pain.
HOW DOES ENDOMETRIOSIS SPREAD – OR DOES IT?
How endometriosis spreads – or whether it even does! – is a highly contested subject. Like many other diseases, its pathogenesis is likely multi-factorial. For example, many believe endometriosis can spread through the blood stream – hematogenously as the scientists like to say – or through the lymphatic pathways. Of note, these are the same pathways that most metastatic cancers take to spread – i.e. metastasize – to other parts of the body. And herein lies the controversy, because some scientists are hesitant to use the term ‘metastasize’ when referring to a benign condition like endometriosis. There is much more to be said on this subject, entangled as it is in a seething cauldron of academic debate. However, we’ll sign off for tonight to return to EndoMarch affairs, but we will revisit these and other popular hypotheses next time.
Thank you to a Facebook friend, who recently re-posted this long-forgotten white paper of sorts, written a few years ago by Barbara Page for Dr. Camran Nezhat’s website. It’s a compilation of a few different pages from http://www.nezhat.org, which can be found here and here and here.