Use of Hydroxyurea with wheatgrass in Thalassaemia Major/Intermedia Dr Dr Vijay Ramanan ; MD, DM(Clinical Hematology) Introduction B-thalassaemia is caused by mutations in the β-globin locus resulting in loss of or reduced hemoglobin A ( HbA, α2β2) production. Hydroxyurea, an antimetabolite, is a potent inducer ofHbF production. Mechanisms: 1. A cytotoxic effect resulting in stress erythropoiesis, with increased HbF levels occurring as a result, is most commonly proposed. 2. More-complex effects involving the production of nitric oxide and the soluble guanylyl cyclase and cyclic guanosine monophosphate– dependent protein kinase pathway gene have been proposed as being responsible for this activity. Hydroxyurea therapy is not associated with considerable or steady effects on erythrocyte deformability in β- thalassemia, which may explain the reduced response to the drug in some patients. Wheatgrass contains Chlorophyll which makes up >70% of the solid content of wheat grass juice. Both chlorophyll and hemoglobin share a similar atom structure. Hemoglobin consists of iron, while in chlorophyll the metallic atom is magnesium. The believers of alternative system of medicine claim that as chlorophyll and hemoglobin are alike in atom structure, intake of wheat grass juice enhances hemoglobin production. The treatment of transfusion dependent b-thalassemia imposes a considerable burden on the family and institutional resources. In economically challenged nations, basic management(red cell transfusions, iron chelation) is a distant dream for the majority, who, consequently, endure a poor quality of life. Aims and Objectives:
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Use of Hydroxyurea with wheatgrass in Thalassaemia Major/Inter-media
Dr Dr Vijay Ramanan ; MD, DM(Clinical Hematology)
IntroductionB-thalassaemia is caused by mutations in the β-globin locus resulting in loss of or reduced hemoglobin A( HbA, α2β2) production.Hydroxyurea, an antimetabolite, is a potent inducer ofHbF production.Mechanisms:1. A cytotoxic effect resulting in stress erythropoiesis, with increased HbF levels occurring as a result, is most commonly proposed.2. More-complex effects involving the production of nitric oxide and the soluble guanylyl cyclase and cyclic guanosine monophosphate–dependent protein kinase pathway gene have been proposed as being
responsible for this activity.
Hydroxyurea therapy is not associated with considerable or steady effects on erythrocyte deformability in β- thalassemia, which may explain the reduced response to the drug in some patients.
Wheatgrass contains Chlorophyll which makes up >70% of the solid content of wheat grass juice. Both chlorophyll and hemoglobin share a similar atom structure. Hemoglobin consists of iron, while in chlorophyll the metallic atom is magnesium. The believers of alternative system of medicine claim that as chlorophyll and hemoglobin are alike in atom structure, intake of wheat grass juice enhanceshemoglobin production.
The treatment of transfusion dependent b-thalassemia imposes a considerable bur-den on the family andinstitutional resources. In economically challenged nations, basic management(red cell transfusions, iron chelation) is a distant dream for the majority, who, conse-quently, endure a poor quality of life.
Aims and Objectives:To study the effect of hydroxyurea and wheatgrass in reducing the frequency of blood transfusion.
Materials and Methods
The study was carried out between January 2008 and June 2013 on 74 patients diagnosed as Betathalas-saemia by HPLC.Randomly selected 74 patients with transfusion dependent B-thalassemia, were recruited for the study.Patients were enrolled irrespective of whether they were receiving chelation therapy with defiriperone /desferrioxamine or not.A medical doctor exclusively on the roll of thalassemia center maintained records of the study subjects.Exclusion criteria:1. Indiscipline in intake of wheat grass and hydrxyureatablets. This included interruption in intake exceeding3-days/week or more than 7-days month.2. Hydroxyurea was not administered below 2 years of
age.
Methodology• Our index case gave me insight into follow up in these case. He had been transfusedfor over 30 years but first transfusion was after 1 year of age.• I realised the real effect of Hydroxyurea +/- Wheatgrass can only be judged by allowingthe hemoglobin to fall upto 5 to 6 gm% with pulse rate never crossing 120/min.• This level allows the inherent hemoglobin synthesis to start i.e HbF synthesis. If apatient doesn’t receive transfusion for over 3 months then it is translates intoTRANSFUSION INDEPENDENCE.
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