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Polycythemia rubra vera, pulmonary embolism, hypoxia, heart failure with severe pulmonary hypertension, one kidney. What can be done?

80 year old female, long term diagnosis of Polycythemia rubra Vera, recurrent pulmonary embolism and hypoxia, R heart failure with severe pulmonary hypertension.
Born with one kidney, now infected (treating with IV antibiotic)
Blood transfusion consisting of 2 units each time performed twice over last week - count from 1st transfusion went from 60-80 - 2nd result not in yet

Querying being told not candidate for anaesthetic for gastroscopy - also pt unable to travel for Haematologist review

Patient using Wheelchair for past 2 years - not walking more than 6-8 steps - from 1/52 ago has not left bed

What is best / worst outcome and considered ?

(daughter) Michelle
Asked On : Sat, 13 Apr 2013
Answers:  1 Views:  68
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Internal Medicine Specialist 's  Response
Hi Michelle,
Welcome to HCM,
After going through patient's history, she has Polycythemia vera with pulmonary embolism with pulmonary hypertension.
Infection and taking IV antibiotics she appears to be quite sick.
With available details why was transfusion given as polycythemia patients have high RBC count and hematocrit.
Her activity is very restricted has severe pulmonary Hypertension.
She should be seen by Hematologist to see if any treatment is possible .
Bloodletting (called venesection or phlebotomy) is one form of treatment, which often may be combined with other therapies including aspirin,and hydroxyurea.
There are some new drug for pulmonary hypertension like Bosentan is a dual endothelin receptor antagonist for treatment of pulmonary artery hypertension (PAH).
Hope this information helps.
Take very good care of her.
Good Luck.
Dr.Akhilesh Dubey M.D.

Answered: Mon, 27 May 2013
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