Ehlers Danlos and dysautonomic crisis

[daphne123]

Does anyone with Ehlers Danlos have severe dysautonomic crisis lasting up to a week with higth blood pressure,fever, nausea, wretching etc. These have been happening to my 38 yr old daughter with increasing frequency over the last few years.
With thanks,
Daphne

[daphne123]

She had a Nissan Fundoplication 10 yrs ago because of uncontollable acid reflux

[daphne123]

Thank you for your response. Over the nearly 20 years since she was first ill - she developed RSD following an arthroscopy - we have crossed paths with every sort of doctor. Her Ehlers Danlos was diagnosed in England by Prof Rodney Grahame. But lately the attacks have become more frequent and intense. After feeling better yesterday she is back in Crisis today.i.e this latest attack has lasted on and off for 2 weeks. On Sunday we are going to trhe Familal Dysautonomia unit though we know she doesn't have FD. Perhaps they can find a way to lesson the attacks. Her gastro/internist says they will have a case conference soon and any input would be useful
What is your field? In which country? You obviously know about Ehlers Danlos and dysautonomia.which it seems not many do.
I do appreciate your response. With thanks,
Daphne

[S. Kaul, MD]

Hi there!

Well, dysautonomia is appropriately managed by a multi-disciplinary approach (involving various specialists) based on the symptoms and may include a cardiologist, neurologist, endocrinologist or gastroenterologist. Mild to moderate symptoms may be managed by a primary care physician as well, while severe or frequent symptoms may require specialist care. If the symptoms develop acutely/ all of a sudden, it is best to rush to the ER; while severe symptoms may need referral of an intensivist. Without an evaluation/ knowing the details it would be difficult to suggest what would be an appropriate management approach in her case at this stage. You may discuss the situation in detail with her treating doctor.
Hope this is useful.

Take care!

[daphne123]

Dear Dr Kaul,
Thank you so much for your reply. She is under the care of a gastroenterologist for her stomach/bowel problems: in fact she was in his ward in hospital last week but the present attack with acid which tears her stomach apart, started 10 days ago. She came out of hospital on Thursday feeling somewhat better and even ate something yesterday. But today she is back in a crisis state. She is treated with Phenergan, morphine Zantac, Papaverine, Zofran  when in a crisis state but this time she seems to come out of it and then slip back. The nearest description of her state is that described in Familial Dysautonomia. She was tested for that here in Jerusalem where we live and they said she has aquired dysautonomia. This evening I just feel frightened and don't know what to do

[S. Kaul, MD]

Hey Daphne!

Patients with Ehler Danlos tend to have a chronic, progressive, generalized form of dysautonomia in the setting of central nervous system degeneration; the mechanism of which is not yet completely understood. Management is mainly supportive, though interventional measures may be tried in a few cases. I would suggest discussing the situation in detail with her treating doctor.
Hope this is helpful.

Take care!