Hydroxyurea in Sickle Cell Disease: Our Experience in Western India

Deshpande, S. V.; Bhatwadekar, Seema S.; Desai, Parth; Bhavsar, Tarang; Patel, Ankit; Koranne, Amey; Mehta, Arpan R.; Khadse, Shweta Vikas

doi:10.1007/s12288-015-0542-1

Cited by 15 publications

(21 citation statements)

References 21 publications

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“…It is well known that HU increases HbF and total Hb level [1,10]. Similar to studies by Pondugala et al [13] and Deshpande et al [14], we observed a statistically significant increase in the total Hb level with HU therapy. Raised Hb level after starting HU therapy may be due to reduction in RBC destruction due to hemolysis.…”

Section: Discussionsupporting

confidence: 91%

Impact of hydroxyurea therapy on clinicohematological parameters in children with sickle cell anemia

Joshi¹,

Jain²,

Ramawat³

2021

Indian J Child Health

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ickle cell anemia (SCA) is an autosomal recessive hemolytic anemia caused due to qualitative defect in the hemoglobin (Hb) B chain synthesis. A single-gene mutation causes the base pair change in B chain of Hb. In the presence of hypoxic condition, these changes result in red blood cell (RBC) breakdown. These sickle RBCs not only decrease RBC life span but also are responsible for numerous dreaded complications of sickle cell crisis [1]. Till date, no effective cure is available for this condition which indirectly affects the person's quality of life and life expectancy. Hydroxyurea (HU) mainly used as a chemotherapeutic agent has shown promising results in the treatment of SCA patients due to its disease-modifying effect [2-5]. It is now being recommended as an effective supportive therapy in SCA patients to decrease risk of life-threatening complications such as stroke [6]. HU is believed to increase HB F concentration in RBCs which has good oxygen carrying capacity. Increase in Hb F concentration decreases the proportion of Hb in blood, thereby reducing the chances of sickling and vaso-occlusive crisis [1]. Since HU is recommended in all patients with SCA, many studies have tested the efficacy of HU in SCA patients. Results from developed and developing countries reported that HU is a safe and very effective treatment to prevent complications in SCA and it can be given at low doses for a longer duration [2,3]. It is a welltolerated medication with few side effects such as gastrointestinal discomfort, skin and nail hyperpigmentation, and transient and reversible myelosuppression. Limited studies are available in India evaluating the efficacy of HU in SCA [7,8]. The prevalence of SCA is significantly higher in Central India, with an increased incidence of hospitalizations secondary to various complications of SCA. Therefore, it is important to observe effect of HU therapy on person's well-being as well as the benefits and risks of prolong use. In view of this, the present study was conducted to evaluate the impact of HU therapy on clinical, hematological, and laboratory parameters in pediatric patients with SCA.

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Section: Discussionsupporting

confidence: 91%

Impact of hydroxyurea therapy on clinicohematological parameters in children with sickle cell anemia

Joshi¹,

Jain²,

Ramawat³

2021

Indian J Child Health

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“…There is now over 30 years of published evidence proving the efficacy of hydroxyurea in the treatment of both adults and children with SCD in the United States, Europe, Brazil, and India. [40][41][42][43][44][45] The results of the pivotal randomized trial of hydroxyurea in adults (MSH) showed benefits in the reduction of the number of vasoocclusive pain episodes, acute chest syndrome, requirement for blood transfusions, and hospitalizations. 41,42 Another placebocontrolled trial of hydroxyurea therapy in infants and toddlers with SCD (BABY-HUG) produced similar results.…”

Section: Hydroxyurea Therapymentioning

confidence: 99%

Sickle cell disease: Reducing the global disease burden

Mburu

Odame

2019

Int J Lab Hematology

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Sickle cell disease has been largely an invisible global health issue, especially in regions of high incidence mainly due to lack of awareness among both the local health policy makers and the public. Public health interventions, such as screening of newborns, provision of prophylaxis against bacterial infections, and immunizations against pneumococcal infections can have the greatest impact. Family education on assessment of spleen size and subsequent detection of splenic sequestration and promptness to seek medical attention for a febrile child is also important in the control of the morbidity and mortality of children with SCD living in resource‐poor countries. In addition to these affordable interventions, hydroxyurea therapy is necessary to decrease both the acute and chronic complications of sickle cell anemia. Sickle cell disease has been recognized to have global health significance by key institutions including the World Health Organization in 2006 and the United Nation is 2008. In 2010, the WHO released national health care management goals and set targets to be achieved by the countries in sub‐Saharan Africa for the control and management of SCD. These are yet to be translated into action. To do, this would require active and sustainable public‐private partnerships for sustainable program development in these regions. Effective interventions should be integrated into existing health systems, the best examples linking primary healthcare facilities to specialized sickle cell disease centers in regional and tertiary healthcare institutions.

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“…[27][28][29]45,[57][58][59][60][61][62] For example, hydroxyurea use has been shown to reduce the vasoocclusive crises, stroke, infections, malaria, transfusions, and death in children with SCD, warranting the need for wider access to this treatment. 29,[59][60][61][62] Hydroxyurea treatment has been shown to be feasible, safe, and effective in children with SCD living in India 63 and in sub-Saharan Africa. 29,59,64,65 In this manuscript, we describe the design, development, manufacturing, and clinical testing of a novel point-of-care hemoglobin test, HemeChip.…”

Section: Introductionmentioning

confidence: 99%