Saturday, November 08, 2008

An Interesting Bone Case

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.
Do we:
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?


Anonymous Anonymous said...

How about IV bisphosphonates like Reclast or even SQ Forteo since she's already had fractures?

8:21 PM  
Blogger vagus said...

no reason to jump to Reclast without having even tried oral bisphosphonates.
and since we haven't given that a try, i didn't want to jump and put her on an expensive med like Forteo. moreover, one can argue that in an unusual case such as this, with her collagen disorder, if you put her on an anabolic agent will you only make more of the defective bone? will it work?
we'll see how she responds with time.

9:29 PM  
Blogger Nikki said...

Pardon my ignorance, but Ehler Danlos only affect the collagen, not bones, rite? Biphosphonate works to prevent osteoclast-mediated bone resorption. So, in terms of mode of action, not very relevant.

HRT? May I suggest raloxifene instead?

8:50 AM  
Blogger vagus said...

hi nikki. bone has collagen too. hence why patients with E-D can be prone to fractures.
between raloxifene and bisphosphonates, the latter has greater efficacy in fracture reduction, though raloxifene is a consideration too, I suppose.
thanks for the comments.

8:02 PM  
Blogger Mei said...

I don't see the harm of putting her on HRT. She has achieved surgical menopause after all... and she's a low risk patient as Vagus mentioned earlier.
It could treat her other menopausal symptoms too, not just in terms of bone health.

5:29 AM  

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