I was born with a rare form of Turner syndrome. Can I benefit from progesterone?

Hi Catherine,

This is rather complicated. I hope y’all don’t mind…

I was born with a rare form of Turner syndrome that used to be called Mixed Gonadal Dysgenesis. Essentially, it means that some of my cells have a Y chromosome and some don’t. So, I had both ovarian and testicular tissue and my genitals were hosed. Cancer rates are extremely high in ovatestes; they were removed when I was young. I’ve been on HRT for 30 years now. Currently I use an Estradiol patch.

Recently I was diagnosed with Meniere’s. The ENT doctor suggested that I take a diuretic. I used to take Prometrium so I suggested going back on it. But he’s unfamiliar with it so he wouldn’t prescribe it. Neither would my primary care physician nor my gynaecologist. My gynaecologist insists that progesterone isn’t a diuretic. And she wants me off of HRT now anyway. I’m 52. She says that there are drugs to prevent osteoporosis. And drugs for the depression I get when I’m not on HRT. And drugs for my Meniere’s. And she doesn’t want me to take progesterone because I don’t have a uterus. I have a small prostate (along with a clitoris and vagina). I know that progesterone is a precursor to testosterone. But, even if I were a man I could still take progesterone, couldn’t I? What does a prostate have to do with anything? In MGD it’s not even a guarantee that I don’t have uterine tissue somewhere.

Right now, however, I’m so angry with my doctors that I’m tempted to never go back. A friend of mine has been using a progesterone cream called “Happy PMS“. So, I’m trying it. For now I’ll keep using my Estradiol (.05mg/day) weekly patch.

Any advice you have would be appreciated,

Jen

Dear Jen,

This IS a curly one! But let’s not shirk from a unique opportunity to dig deep, do some research and learn more about this condition. Perhaps, while we’re at it, shed some light on where progesterone fits into the picture for you.

Humans are usually born with 46 chromosomes, which are arranged in 23 pairs. One of these pairs determines whether a baby is male or female, and these are known as the sex chromosomes. Boys are born with the sex chromosomes XY, and girls are born with two X chromosomes (XX).

Occasionally, however, a girl is born with only one X chromosome and this is known as Turner Syndrome. The second X chromosome is usually missing in Turner Syndrome. Occasionally it may be present, but abnormal in some way.

About 1 in 2,500 girls has Turner Syndrome. Even though these girls only have one normal X chromosome, they are 100 percent female.

Turner Syndrome is named after Dr. Henry Turner, who first described it in 1938.

Often a girl with Turner Syndrome is not diagnosed until early childhood, because she is not growing as well as expected. Some girls are diagnosed as teenagers when they are taken to the doctor because their periods or puberty seem to be late in starting.

Some of the most common features are listed below. Most girls with Turner Syndrome would have some, but not all, of these things.

• Short stature (not so tall as other girls).
• Widening of the neck (”webbed neck”).
• Ovaries that do not function and infertility.
• An underactive thyroid gland.
• A broad chest with widely spaced nipples.
• A heart murmur, sometimes associated with
• narrowing of the aorta (the main blood vessel that
• comes out of the heart).

Girls with Turner Syndrome are almost always infertile, because their ovaries are unable to produce eggs. A very small proportion of young women with Turner Syndrome may have a short time during their life when they are fertile.

Although girls with Turner Syndrome have nonfunctioning ovaries, they do have a normal womb and vagina, and will be able to have an entirely normal sex life.

Some women with Turner Syndrome have had successful pregnancies using donated eggs and in vitro fertilisation (IVF).

There is, at the moment, no way to prevent Turner Syndrome from happening or cure it once it occurs. Many of the features of Turner Syndrome can be medically monitored and treated.

According to research, girls with Turner Syndrome that are nearing the age of 13 may be given a low dose of estrogen for two years. During this time her growth rate may double. Since estrogen, when taken alone, can cause abnormalities of the uterus, progesterone is then included in the therapy. This estrogen and progesterone treatment will go on for the rest of her life from the age of about 15.

For those interested in learning more, I did manage to locate a paper on sex determination and differentiation that examines how mutations in genes cause important clinical syndromes such as Mixed Gonadal Dysgenesis.

Jen, all things considered, despite the fact you no longer have an intact uterus, it could be argued your body still needs progesterone at age 52. One of progesterone’s key functions is its broad range of core biologic effects on metabolic health. Furthermore, progesterone is a precursor (or building block) of other hormones that include estrone, estriol, estradiol, testosterone, and all the important adrenal cortical hormones (see image below).

Progesterone benefits include:

• Maintains the secretory endometrium
• Protects against fibrocystic breasts
• Helps use fat for energy
• It is a natural diuretic
• Natural anti-depressant / mood enhancer
• Facilitates thyroid hormone action
• Normalises blood clotting
• Restores sex drive
• Normalises blood sugar levels
• Normalises zinc and copper levels
• Restores proper cell oxygen levels
• Prevents endometrial (uterine) cancer
• Helps prevent breast cancer
• Simulates osteoblast for bone building
• Restores normal vascular tone
• Necessary for the survival of the embryo
• Precursor of corticosteroids and other hormones
• Modulates other hormones helping to restore balance
• Promotes sleep
• May help improve libido
• Contributes to reducing anxiety and panic attacks
• Reduces estrogen dominance symptoms

Medical conditions associated with Turner Syndrome that include osteoporosis, high blood pressure, hypothyroidism (Hashimoto’s), fluid retention, and depression have all been known to respond favorably to progesterone supplementation.

Medical research implicates estrogens (including the natural hormones estradiol and estrone) as a major cause of breast cancer. If you need to supplement estrogen (determined by a salivary hormone profile), ask your doctor to prescribe estriol instead of estradiol. Estriol transdermal cream is a safer Estrogen Replacement Therapy (ERT) alternative that protects women against breast cancer, vaginal atrophy, urinary tract infections, helps protect our bones, relieves hot flashes and night sweats, improves blood lipids, and protects the skin from aging.

Bioidientical progesterone replacement therapy should not be discounted by your GP just because you’ve had your uterus and ovaries surgically removed. As demonstrated above, progesterone DOES play a signicant role in our overall health, and can actually help protect us against some forms of cancer, including breast cancer.

Prometrium is a form of oral micronized progesterone. Whilst both Prometrium capsules (that contains peanut oil, so anyone allergic to peanuts should not use this!) and transdermal cream contain bioidentical progesterone, progesterone cream that is rubbed directly onto the skin is up to 80% more bioavailable than equivalent doses administered orally. In fact, in practical terms, an oral dose of 100 mg progesterone might be equivalent to 20 mg progesterone cream delivered transdermally. Prometrium can also be used as a vaginal suppository which would deliver a much higher dose. We know that high oral doses can cause drowsiness and sleepiness. Therefore, low dose transdermal delivery systems like progesterone cream tend to be safer and more natural, and kinder on our liver and gall bladder.

You posed the question if you were classified a man could you likewise benefit from progesterone supplementation? The answer is “yes”. Progesterone in men is vital to good health. Both the prostate gland (in men) and the uterus (in women) develop from the same embryonic cells, and both respond to the same hormones - estrogen, progesterone, and testosterone. Men synthesise progesterone in smaller amounts than women do but it is still important.

A little info for our readers about M?ni?re?s disease. It is a chronic, incurable vestibular (inner ear) disorder where a recurring set of symptoms such as attacks of vertigo, ringing in the ear (tinnitus) and hearing loss result from abnormally large amounts of a fluid called endolymph collecting in the inner ear.

In a pilot study, researchers found that older women receiving synthetic HRT did 10 percent to 30 percent worse on hearing tests than women not receiving HRT. Please note, all references to ‘progesterone’ in this report relate to synthetic progestin, not bioidentical progesterone.

Jen, please stay in touch, let us know how your health progresses on progesterone cream. You really shouldn’t be forced to go it alone (self-medicate) but that’s the reality for some of us, I’m afraid. The medical community will not be swayed in favour of bioidentical progesterone until much needed funds are injected into progesterone research to convince them otherwise.