It's a common scenario: A young woman goes to the Emergency Room with pelvic pain. She's told that it must be an ovarian cyst or Pelvic Inflammatory Disease (a condition often caused by an untreated STI), but she's monogamous and has no risk factors or fever. She's treated with antibiotics but all of her cultures come back negative and her pain persists. Or maybe she has a tiny ovarian cyst and her primary care doctor scratches her head. That grape-sized cyst can't be the cause of her excruciating pain... can it? No, it cannot.
She comes to see the supremely intelligent gynecologist, who takes a thorough history. It is revealed that the patient has had this before, just not as bad. The gynecologists asks, "Any women in your family have endometriosis?"
What is endometriosis?
Endometriosis is a condition where the glands that line the inside of the uterus, called the endometrium, attach outside of the uterus. It is not cancer or life-threatening, so the chief concern is symptoms. You may have endometriosis and not know it, and that is okay if you are not having pain or infertility.
What are the symptoms?
As mentioned above, pain and infertility are the primary symptoms. The pain often starts as just painful periods (dysmenorrhea) that gradually becomes more persistent throughout a woman's cycle. It also can present as painful sex (dyspareunia), painful urination (dysuria) or painful bowel movements (dyschezia). The average age of diagnosis is 28, but symptoms are often present for 7-8 years before it is diagnosed. The youngest patient that I have had with documented endometriosis was 11 years old.
Why is endometriosis painful?
There is definitely an inflammatory reaction and even an increase in the nerve cells that perceive pain. This is a phenomenon called neuroangiogenesis. Women with endometriosis often develop other pain syndromes such as interstitial cystitis and irritable bowel. Pain specialists call this central sensitization — unfortunately, our pain signals learn to travel more efficiently.
What causes endometriosis?
Theories abound. A common theory is retrograde menstruation: having blood flow backwards into your pelvis during your period. (No, standing on your head will not cause endometriosis.)
There are a few things wrong with this theory, or at least that make it incomplete. For one thing, endometriosis has been found very distant from the uterus. Many years ago, I had a healthy patient in her late 30s who had a spontaneous pneumothorax. Her lung collapsed while flying cross-country and when the surgeons were correcting the collapse, they determined the cause to be endometriosis. This woman had two kids, no problems with fertility, and her periods were light and just slightly uncomfortable. You read correctly: there was endometrial tissue around her lungs.
Another fact: Lots of women have evidence of retrograde menstruation but do not have endometriosis. It is perhaps an immunity issue in which these wayward endometrial cells are able to implant where they shouldn't. It's complicated.
How is endometriosis diagnosed?
The only reliable diagnosis is with surgery, specifically laparoscopy. CT scans and ultrasounds detect masses but endometriosis is usually a thin layer of cells on the abdominal lining. Endometriosis ususally does not form a mass unless it forms a cyst on the ovary, called an "endometrioma." This lining is called the peritoneum and endometriosis like to live there, especially behind the uterus. Sometimes the endometriosis can be deeply infiltrating, almost like a cancer. However, it is not deadly, just painful.
If I am diagnosed with endometriosis, does that mean I am infertile?
No, you may be totally fertile. I have had many patients with endometriosis that have had no problems conceiving. In fact, one of the worst cases I ever saw was a woman with three kids who developed pelvic pain years after her youngest was born. She had a big complex ovarian cyst. We were worried she had cancer. Instead, it was an endometrioma with extensive endometriosis everywhere. Stage 5 on a scale of 1 to 4.
However, there is an increased risk of infertility. There are lots of mechanisms: scarring of the fallopian tubes, inflammation making sperm unwelcome, and even poor implantation of an embryo.
What are the best treatments for endometriosis?
Let's talk about gynecologic surgery in general. Women are often surprised to hear that all board-certified gynecologists are trained surgeons. Trust me, I do a much better hysterectomy than the general surgeon who took out your appendix. However, I do not think all gynecologic surgeons are created equally, and the level of experience with endometriosis surgery varies greatly.
If your OB-Gyn spends more than half their time delivering babies, they probably do not have the same skill set as a surgeon who has given up obstetrics and just focuses on gynecology. If I had endometriosis, I would seek out a surgeon who removes the endometriosis and not just burns it, and who can confirm that they have done many such procedures. In other words, a "high volume" surgeon. I am not one of these surgeons, but I make a point of working with those who have extra training is Minimally Invasive Surgery (MIS). A good resource to find a qualified surgeon is at www.AAGL.org. (This stands for American Association of Gynecologic Laparoscopists, although it is international.)
Pelvic physical therapy (PT) can also aid in pain management. I use PT for a variety of pelvic conditions. It is well-known to improve bladder function, but much of pelvic pain is musculoskeletal. Example: tensing pelvic muscles in anticipation of painful sex. A good pelvic PT can help you retrain those muscles.
What about medication?
If you are diagnosed with endometriosis, your gynecologist is going to recommend hormones, specifically progesterone. Women hate to hear this. Many women erroneously believe that all hormones will turn them into crazy bitches. This is an urban myth, perpetuated by men (and women) who assume any mood swing is due to hormones. Some women will get moody on hormones, but different hormones affect different women . . . differently. If one method causes side effects, try another method.
Estrogen and progesterone are the yin and yang of female hormones. Estrogen builds up the lining of the uterus and progesterone thins it. Endometriosis tends to calm down with progesterones, and this can be in the form of birth control pills, progesterone IUDs, Nexplanon or Depo Provera.
But don't birth control pills have estrogen?
Yes, but they have more progesterone than estrogen, and thus the progesterone has the dominant effect. Estrogenis still helpful in preventing irregular bleeding.
There are other medications that work well in women with endometriosis:
--Lupron is an injection given every 1-3 months, and this was one of the first medications that specifically targeted endometriosis. It shuts down estrogen production and starves the endometriosis by temporarily mimicking menopause.
--Elagolix is an oral medication that has largely replaced Lupron. It can be expensive and can cause similar side effects to menopause: hot flashes, night sweats, vaginal dryness and osteoporosis. For many women in pain, this is a worthwhile trade-off.
--Gabapentin and similar nerve modulators block the pain signals. They are used off-label for endometriosis pain, and can cause side effects such as dizziness and blurred vision. I have prescribed this for women who did not tolerate hormonal suppression, and I have had some good results.
--Danazol is an androgen — it acts like testosterone and can cause hirsutism (hair where you don't want it), weight gain and irritability, as well as elevated cholesterol. It's an old medication and I have never had a patient use this. Usually, most women will learn about the side effects and say "no thanks, I'll take the pain."
--Letrazole is related to Tamoxifen and blocks synthesis of estrogen in some tissues. It too can cause menopausal symptoms. It needs to be taken with progesterone in order to avoid ovarian cyst formation. It is still considered experimental but I have used it on a few patients.
Is there anything I can do to prevent endometriosis?
Women who drink alcohol are at higher risk, and then there are other, more random associations: low body weight and freckles and moles (nevi). We do not know why some women have endometriosis, but there is a definite genetic component: If you have a sister or mother with endometriosis, you are up to 10 times as likely to have it yourself.
This is far from a comprehensive discussion about endometriosis. The take home point: If you are having chronic pelvic pain, very painful periods, pain with sex or unexplained infertility, see a gynecologist. It might be endometriosis and we may be able to help you, a lot.