Estrogen Replacement Therapy is a common treatment for patients with Turner Syndrome. In this post, find the answers to some frequently asked questions about ERT. Thank you to Lournaris Torres-Santiago, MD and Nelly Mauras, MD of Nemours Children’s Health System, Jacksonville, FL, for contributing your expertise to answer these questions!
Note: The information in this post is educational and is not meant to replace medical advice from your doctor(s). Always consult with a medical professional regarding any specific health concerns.
The transdermal patch was considered a good option for girls to feminize. Is this still the recommendation? Do adults switch to ’the pill’. If so, when?
Transdermal estradiol (patch) is the recommended and most physiologic option for estrogen replacement. Ideally it should continue to be the estrogen of choice for ERT throughout the duration of treatment, cycled with progesterone. The use of oral contraceptive pills – not for birth control in this case – can be considered on an individual basis once puberty induction and linear growth are complete.
Some people claim emotional changes when initiating estrogen therapy treatment. What are these possible symptoms and what is recommended? Stop, change, push through symptoms?
Symptoms produced by hormone replacement therapy vary per individual. Feminization using ERT, similar to spontaneous occurring puberty, can be associated with emotional changes or mood swings. Therapy is started at low doses and slowly increased to full doses over approximately 2 years in attempt to mimic natural puberty progression. Over time, any symptoms related to the changing hormonal milieu should improve, but if not, it is important to address any emotional distress taking into account the whole patient as mood and behavior are more complex than our hormones. Alternative forms of estrogen can be considered, but stopping treatment altogether is not recommended given the importance adequate ERT has on promoting bone and cardiovascular health, amongst other body functions.
Can growth hormone and estrogen therapy be used together?
Growth hormone can be used in combination with ERT. Your doctor will be monitoring the bone growth and puberty development trying to keep a fine balance between the two in attempt to maximize both height and overall health.
If estrogen therapy is not maintained, what are the consequences?
Estrogen has important roles in the body supporting bone health, cardiovascular protection, breast and uterus growth and development, influence on mood, fat distribution amongst others. If ERT is not taken during the appropriate reproductive years it can have negative impact in these areas.
In the rare case a girl has feminized on her own, does she need to take estrogen?
If a girl feminizes spontaneously she may not need ERT unless pubertal development stops or if there is a significant evidence of ovarian dysfunction. This can be detected through regular blood work and physical examination as well as a careful menstrual history.
Low estrogen is known to cause osteoporosis. What should a woman do to maintain bone health throughout her lifetime?
There are several important components to maintaining good bone health including adequate estrogen replacement, weight bearing exercises, adequate consumption of calcium and Vitamin D in the diet. Smoking and excessive alcohol consumption should be avoided.
More FAQ’s about Estrogen Replacement Therapy and Growth Hormone Therapy can be found on our website!
Torres‐Santiago L, Mericq V, Taboada M, Unanue N, Kelin KO, singh R, Hossain J, Santen R, Ross JL, Mauras N. Metabolic effects of oral versus transdermal 17beta‐estradiol (E(2)): a randomized clinical trial in girls with Turner syndrome. J Clin Endocrinol Metab. 2013;98(7):2716‐2724.
Gravholt CH, Andersen NH, Conway GS, Dekkers OM , Geffner ME , Klein KO, Lin AE, Mauras N , Quigley CA , Rubin K , Sandberg DE , Sas TCJ , Silberbach M, Söderström-Anttila V , Stochholm K, Alfen-van derVeldenJA, Woelfle J , Backeljauw PF for International Turner Syndrome Consensus Group. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol. 2017;177(3):G1‐G70.
Sweetland S, Beral V, Balkwill A, Liu B, Benson VS, Canonico M, Green J, Reeves GK, The Million Women Study C.Venous thromboembolism risk in relation to use of different types of postmenopausal hormone therapy in a large prospective study. Journal of thrombosis and haemostasis: JTH. 2012;10:2277–2286.
Mauras N, Torres-Santiago L, Santen R, Mericq V, Ross JL, Colon-Otero, G, et al. Impact of route of administration on genotoxic oestrogens concentrations using oral vs transdermal oestradiol in girls with Turner syndrome
Klein KO, Rosenfield R, Santen RJ, Gawlik AM, Backeljauw PF, Gravholt CH, Sas TCJ, Mauras N. Estrogen replacement in Turner syndrome: literature review and practical considerations. J Clin Endocrinol Metab. 2018;103(5):1790‐1803.