Just because you’ve been diagnosed with Polycystic Ovarian Disease (PCO) doesn’t mean your doctor knows all the facts about your disease; in fact, you might not have PCO at all but rather it’s closely related cousin, Late Onset Congenital Hyperplasia (LO-CAH or non-class congenital hyperplasia). When you think of Polycystic Ovarian Disease (PCO) you think of insulin resistance, right? But did you know that insulin resistance is just one type of PCO? There are actually several types of polycystic ovarian disease and quite frankly, I’m a bit tired of the media and the public only knowing about one.
I was diagnosed with PCO way before it was even named Polycystic Ovarian Disease back in 1986, but back then it was called a ‘hormonal disorder’ and the proper name for it was Stein -Leventhal Syndrome – because gynecologists, Irving Stein and Michael Leventhal, first published their description of the disease back in 1935. For some reason this disease waited patiently in the shadows for its limelight and when it finally did gain recognition, a lot of misinformation came about with it.
The first infertility gynecologist I saw provided me with a pamphlet entitled Polycystic Ovarian Disease, A Guide for Patients (published in 1992 by the American Fertility Society, the Society for Reproductive Medicine and Biology; Birmingham, Alabama) that described three causes of Polycystic Ovarian Disease:
Diabetes: Some forms of diabetes with high insulin levels can alter the ovary’s normal cycle and create a situation that mimics polycystic ovarian disease.
Obesity Induced: The most common cause of polycystic ovarian disease is obesity. Fatty tissues produce estrogen, and this can deceive the pituitary gland into producing insufficient amounts of FSH (follicle stimulating hormone). This prevents ovulation and results in PCO.
Dysfunctional Glands: The adrenal glands, thyroid gland, or pituitary gland, can become overactive and produce PCO symptoms. This may cause excess androgen production and ovulation disruption. The excess androgen from the adrenal glands and the ovaries is converted into estrogen in fatty tissue.
For me it was a dysfunctional gland which spurred my symptoms, yet I never heard about it in the news and often wondered why it was so overlooked and cast aside like a step-sister scrubbing floors during the Ball. Women with congenital pituitary dysfunction don’t usually have insulin resistance and therefore, can’t control their symptoms through their diet – no, this is something that went wrong before they were even born.
Even on the PCO web sites they only talk about insulin resistance as a cause, and there’s never any discussion about my type – it’s bad enough being a pariah in society by having undesirable symptoms, but now the small percentage of women with the disease caused by a pituitary dysfunction have to be un-cool even within their own PCO community.
PCO has become so popular in recent years and for that I’m thankful, but I just wish people knew the facts instead of lumping all of us together and making gross generalizations. Not every woman with PCO gains weight, is infertile, has excess facial and body hair, and acne – and that is just one of the reasons why it is so difficult to diagnose and differentiate.
What you need to know is this: Polycystic Ovarian Disease is a misnomer – there are no multiple cysts – there are multiple follicles that never developed into eggs, which translates into lack of ovulation, and without ovulation the sperm has no egg to fertilize. But some women have polycystic ovaries without having the actual syndrome – no symptoms, no infertility – and these women might not even know they even have this condition and could be passing it onto their daughters. This disease isn’t cut and dry – its symptoms, severity, causes, and effects differ from woman to woman.
Some women develop their symptoms in their 20s or 30s while others develop them as a teenager – I was one of the unlucky few to have had my embarrassing symptoms make their appearance at the age of 13; imagine finding hair on your chin when you’re just learning how to shave your legs or wear your first bra. Then imagine going to the emergency room on a regular basis because you were having contractions as if you were labor and no one knew what was wrong or how to treat you; and that’s in addition to having heavy periods that lasted 10 full days – and of course there was no regular schedule so you couldn’t plan to stay in on certain days, no these lovely periods came when they wanted to with no warning.
Handling that when you first enter your teen years is a test of character and while it helped me develop a higher threshold for pain, it hasn’t made me stronger for having gone through it – it’s only made me resentful that I was born having to endure something most females don’t have to contend with. Fortunately for me, I wasn’t born with a strong desire to pro-create, otherwise that, too, would be another difficult hurdle to overcome. That’s my silver lining in this cloud but for many, it’s a thorn in their side (to say the least).
But then a new disease was added to the mix and now doctors are as confused as ever as to whether or not their PCO patients really have Polycystic Ovarian Disease. After many thorough tests on my ovary production, the last reproductive endocrinologist I saw diagnosed me with late onset congenital adrenal hyperplasia (also known as LO-CAH or non-classic congenital adrenal hyperplasia) – which apparently is the granddaddy of them all – and now doctors are debating if LO-CAH causes PCO, or if PCO is indeed a separate disease. The Office of Rare Diseases of the National Institutes of Health lists Late-onset congenital adrenal hyperplasia as a rare disease – this simply means it affects less than 200,000 people in the US population.
My reproductive endocrinologist then proceeded to tell me that I had to add a low dose steroid to my daily life regime but I declined – I didn’t want to add yet another pill to the mixture when steroids were known to cause further problems, or maybe I was just being ignorant about the role of steroids in the treatment of those with LO-CAH induced PCO. Part of the problem was that I didn’t fully understand LO-CAH so here’s the explanation of the disease according to the Mayo Clinic:
“Congenital adrenal hyperplasia is a family of genetic conditions affecting your adrenal glands. The adrenal glands, a pair of walnut-sized organs above your kidneys, typically don’t produce enough of the hormone cortisol. Congenital adrenal hyperplasia may also result in the adrenals manufacturing abnormal levels of two other classes of hormones – mineralocorticoids (for example, aldosterone) and androgens (for example, testosterone).”
“There are two major types of this disorder: classic congenital adrenal hyperplasia, the more severe form of the disease affecting very young children; and non-classic congenital adrenal hyperplasia, a milder form that usually develops in late childhood or early adulthood.”
“The cause of congenital adrenal hyperplasia is an inherited genetic defect that results in decreased formation of the enzyme 21-hydroxylase – resulting in reduced hormone production by the adrenal glands. Congenital adrenal hyperplasia may sometimes be called 21-hydroxylase deficiency.”
When signs and symptoms arise, they’re most often associated with declines in the manufacture of cortisol, which plays an important role in regulating your blood pressure, maintaining blood sugar and energy levels, and protecting your body against stress.
“If you and your spouse have congenital adrenal hyperplasia or if both of you are carriers of the genetic defect for the disease, your child is at increased risk of having the condition. People in some ethnic groups are more likely to develop this disorder, particularly Ashkenazi Jews. There’s also a higher prevalence among Hispanics, Italians and Eastern Europeans.”
I was happy with my testosterone free inhibitor and estrogen meds, but now my periods are heavier and heavier each month, as is the pain, so what happens in the future I don’t yet know. The ‘experts’ are waiting for my generation to go through menopause so they can finally figure out what happens to PCO patients when they stop menstruating.
I’m not sure if I should tell people I have late onset congenital adrenal hyperplasia or the differential diagnosis of congenital pituitary dysfunction form of Polycystic Ovarian Disease – both result in similar symptoms (irregular menstruation, facial and body hair, acne, infertility) and they both are hormonal disorders; therefore, the original diagnosis I received as a teenager wasn’t wrong.
I encourage all women who have been diagnosed with PCO to see a reproductive endocrinologist who can perform the necessary tests to determine if you have LO-CAH or a form of PCO; and until the doctors figure out which is which, find a treatment plan that works best for you as an individual.
