Addition of Multimodal Therapy to Standard Management of Steady State Sickle Cell Disease

Iheanyi Okpala,Osita Ezenwosu,Kingsley Agu,Uche Anike,Sunday Ocheni,Charles Nonyelu,Anazoeze Madu,Ifeoma Ajuba,Barth Chukwu,Augustine Duru,Obike Ibegbulam,Ogechukwu Ugwu,Uche Ololo,Anthony Ikefuna,Theresa Nwagha,Awele Chukwura,Chukwudi Anigbo,Ifeoma Emodi

doi:10.1155/2013/236374

Iheanyi Okpala, Osita Ezenwosu + Show 16 more

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https://doi.org/10.1155/2013/236374

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Abstract

Most people on folic acid to boost erythropoiesis and prophylactic antimicrobials, the standard management of steady state sickle cell disease (SCD), have unacceptable numbers of crises. The objective of this study was to evaluate the effects of adding multimodal therapy with potassium thiocyanate and omega-3 fatty acids to the standard management of steady state SCD. Pre- and post-treatment numbers of crises and other disease indices were compared in 16 HbSS individuals on folic acid and paludrine after 12 months of adding eicosapentaenoic acid 15 mg/kg/day, docosahexaenoic acid 10 mg/kg/day, and potassium thiocyanate 1-2 mL/day, each milliliter of which contained 250 mg of thiocyanate and 100 micrograms of iodine to prevent hypothyroidism: a possible side-effect due to competitive inhibition of the transport of iodide into the thyroid gland by thiocyanate. Median number of crises reduced from 3/yr to 1/yr (P < 0.0001). There was no evidence of impaired thyroid function. Plasma level of tri-iodothyronine improved (P < 0.0001). Steady state full blood count and bilirubin level did not change significantly. The findings suggest that addition of potassium thiocyanate and eicosapentaenoic and docosahexaenoic acids to standard management of steady state SCD reduces the number of crises. This observation needs to be evaluated in larger studies.

Highlights

The inherited blood condition sickle cell disease (SCD) affects 20–25 million people worldwide and constitutes a major health problem on a global scale
Sickle cell disease in steady state is currently managed with folic acid to boost production of red blood cells, antimicrobial drugs to prevent infections, and, in severely affected individuals who constitute less than 5% of all patients, either haemopoietic stem cell transplantation or the cytotoxic drug hydroxycarbamide which increases the proportion of foetal haemoglobin inside erythrocytes and so inhibits sickling
There is need to explore new approaches to specific treatment of steady state SCD, in order to advance beyond the level of efficacy attainable by the current standard management using folic acid and prophylactic antimicrobials

Summary

Introduction

The inherited blood condition sickle cell disease (SCD) affects 20–25 million people worldwide and constitutes a major health problem on a global scale. This multiorgan disease is characterised by crescent-shaped (sickle) red blood cells, premature destruction of erythrocytes (haemolysis) resulting in anaemia, susceptibility to infections, and recurrent obstruction of blood vessels which causes tissue ischaemia or infarction—the pathological process underlying the episodes of generalised (ischaemic) pain called vasoocclusive crisis. The multimodal approach to the specific treatment of steady state SCD is analogous to combination chemotherapy of cancers with cytotoxic drugs each of which inhibits cell proliferation by a different mechanism

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