I saw an interesting case the other day. One that's left me thinking still about the possibilities.
This was a pleasant lady in her 30's with a history of recurrent fractures. After one too many, her doctors decided to send her to a specialist some years ago who did some skin fibroblast studies and diagnosed her with Ehlers-Danlos.
In addition she has a history of chronic colitis that is managed by her gastroenterologist; this apparently has been refractory to everything but Prednisone 7.5 mg daily.
To top it up, she had total abdominal hysterectomy with bilateral salpingo-oopherectomy.
Yup. Wham, wham and wham. Three whacks to the skeletal system. Premature osteoporosis, a collagen disorder, and supraphysiologic steroid therapy.
It's no wonder she is deathly afraid of breaking more bones.
We talked about some options aside from the calcium and Vitamin D shpiel. But really, I told her we don't know what works with the Ehlers Danlos.
1) Put her on estrogen therapy, since she is young, and is surgically post-menopause? She is a nonsmoker, has no risk factors for HRT.
2) Put her on a bisphosphonate because of her steroid therapy? However, the efficacy data is from physiologically post-menopausal women, and no study has been done on Ehlers-Danlos patients. Basically we don't know if it works.
3) Do both?
4) Do nothing?
In the end, for the lack of anything else, we both agreed on starting Alendronate. I'll probably start her on estrogen too, the next time I see her, to optimize her bone health. Despite her bad luck, she remains amazingly resolute and active, wanting to live life to the fullest.
What would you do?