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Diagnosed with Sickle Cell anaemia. Had Splenectomy and Cholcystectomy. Taking Hydroxy urea, folic acid. Treatment?

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XXXXXX is 40 years old lady, works as a specialist in Ophthalmology in Germany. Diagnosed with Sickle Cell anemia since childhood. She is post Splenectomy and Cholcystectomy which were carried out in childhood. At age of 7 she had intracerebral Hemorrhage which was spontaneously resolved with no interventions after her admission due to major crisis and received blood transfusion
She is taking regularly Hydroxy urea 500 mg twice a day and folic acid. She was on Penicillin but which she stopped since last XXXXXXX 2012. Her last hospital admission was in 2008 with Major crisis.

Currently XXXXXX often complains of recurrent headaches (3-4 times in a week, mostly band like headache and sometimes in occipital region) and some mild to moderate sickle crisis which is controlled with NSAIDs , rehydration and rest. In September 2012 XXXXXX had an MRI head in London, there was an incidental finding of Aneuyrsms (left posterior cerebral artery aneurysm, which is less than 2 mm in diameter and 3 to 4 mm left XXXXXXX carotid paraophthalmic aneurysm). A repeated MRI scan in March 2013 showed no changes. The Neurosurgeon recommended to repeat the Scan in 6 month time.

In her recent blood tests, XXXXXX Platelets showed Thrombocytosis (ranges 700-900).

Our Query is: what is the best option available for XXXXXX recurrent Headaches and Thrombocytosis? and is there any other tests should be carried out in her case?
Posted Tue, 30 Apr 2013 in Brain and Spine
Answered by Dr. Gopal Krishna Dash 17 hours later


Thanks for the query,

I am really very sorry to note your problem

I understand your concern

Patients with sickle cell disease can have headache which can mimic like migraine headache. This could be multifactorial and could be related top altered cerebral hemodynamics due to repeated vaso- occlusive crisis. Further, this can compounded by increased risk of cerebral aneurysm in these patients without any other risk factors like hypertension and dyslipidemia.

Her main treatment will be to treat the sickle cell disease with medications, blood transfusion and prevent repeated crisis. Medications that may help relieve headache and altered red cell membrane are valproate, Azacytidine. Valproate will be helpful for headache. Aneurysms need careful follow up

Hope this clarifies
Please get back to me in case you have any other questions
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