Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Darshana, Thamal; Rees, David C.; Premawardhena, Anuja

doi:10.1186/s13023-021-01781-w

Cited by 8 publications

(4 citation statements)

References 73 publications

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“…Fixed-low dose hydroxyurea treatment (10 mg/kg/day) has shown encouraging outcomes in Indian SCD patients and may be appropriate for sickle patients from other South Asian nations who are of Indian descent. 9 Ataga et al conducted a randomized controlled trial of 198 sickle cell anemia patients and compared the efficacy of crizanlizumab with placebo and found that crizanlizumab therapy resulted in a significantly lower rate of sickle cell-related pain crises than placebo and was associated with a low incidence of adverse events. 10 It could be a potential treatment option for our patient for the prevention of further episodes of vaso-occlusive crisis.…”

Section: Discussionmentioning

confidence: 99%

Sickle cell anemia: a mimicker of rheumatoid arthritis

Selvaraju

Devipriya

et al. 2023

Int J Adv Med

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Sickle cell disease (SCD) is a genetic disorder characterized by presence of abnormal hemoglobin S, leading to sickling of RBCs. The prevalence of sickle cell carriers among different tribal groups varies from 1-40%. Rheumatoid arthritis closely mimics the bone crisis symptoms in sickle cell anemia hence prompt diagnosis should be made to commence correct choice of treatment. We reported an 18 year old female with sickle cell disease who presented multiple intermittent joint pain of both limbs for 6 years with acute worsening of pain for the past 7 days. Diagnosis of sickle cell anemia becomes important as musculoskeletal manifestations of the disease can mirror the symptoms of inflammatory arthritis and the treatment given for rheumatoid arthritis can potentially worsen the condition in patients with sickle cell anemia.

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Section: Discussionmentioning

confidence: 99%

Sickle cell anemia: a mimicker of rheumatoid arthritis

Selvaraju

Devipriya

et al. 2023

Int J Adv Med

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“…Malaria parasites, however, are now resistant to these drugs [ 9 ]. Hydroxyurea therapy for sickle cell disease has been utilized for many years and appears to be helpful for both the main and secondary prevention of stroke in sickle patients [ 12 ]. It does so by elevating the HbF levels that are present in the RBCs of patients.…”

Section: Reviewmentioning

confidence: 99%

Treatment Options That Reduce the Duration of Sickle Cell Vaso-Occlusive Crises: A Systematic Review

Akindele¹,

Jalkh²,

Eastmond³

et al. 2022

Cureus

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Most patients with sickle cell disease (SCD) seek hospital care because of pain symptoms. While some patients opt to treat themselves at home, some prefer to seek treatment in a hospital setting. There are, however, some patients with more complicated effects of the disease who seek treatment so often that they have been termed “super-users.” This paper seeks to determine, across the board, the treatments available for vaso-occlusive crisis (VOC), the most common complication of SCD. Due to the frequency and unpredictable nature of VOC, it is no surprise that the lives of so many patients dealing with SCD are constantly disrupted by this complication. Treatments that reduce the frequency of VOC and the need for hospital admissions will help these patients find some semblance of balance in their quality of life.

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“…Timely blood transfusion appears to prevent early signs and symptoms of the disease, however, transfusion complication such as excess iron is deposited in all body organs leading to heart failure (22,23), chronic liver diseases, endocrine problems, growth disorders, osteoporosis (24-26) …. etc.…”

Section: Introductionmentioning

confidence: 99%

Exploring the Economic Aspects of β-Thalassemia in Jordan in 2019

Alabbadi

Massad

Taani

et al. 2022

JJPS

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Thalassemia are inherited hematological disorders considered among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean Region. The WHO estimates that Beta-thalassemia affects 2.9% of the world’s population. In Jordan, the carrier prevalence rate of thalassemia is from 2-4%. Patients with thalassemia need a lifelong care, devastating their quality of life and imposing overwhelming psychological and financial burden on patients and their families. The Jordanian Ministry of Health (MOH) is the sole facility responsible for treating these patients from the pre-marital program until required medications regardless of their nationality. This study aimed to estimate the economic burden of thalassemia in Jordan in 2019. All 680 thalassemia patients admitted to thalassemia centers in Jordan and coming to out-patients’ clinics from July 1st to Aug 31st, 2019 are included. Data were collected using a pre-developed questionnaire from the electronic medical records. The economic burden was estimated from MOH perspective and societal perspective. The average annual cost was estimated to be 2,674 JOD for a single thalassemia Jordanian insured patient and 4,627 JOD for un-insured, while the non-Jordanian patient’ annual cost was estimated 4,751 JOD if insured and 6,651 JOD if un-insured. The total economic burden of thalassemia in Jordan in 2019 was estimated to be 2,148,741 JOD. Of this amount, 1,393,329 JOD was for Jordanians and 755,412 JOD for non-Jordanians. In conclusion, this high burden of thalassemia in Jordan requires adopting new controlling policies; pre-marriage counseling, education and raising awareness should be encouraged.

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Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Cited by 8 publications

References 73 publications

Sickle cell anemia: a mimicker of rheumatoid arthritis

Sickle cell anemia: a mimicker of rheumatoid arthritis

Treatment Options That Reduce the Duration of Sickle Cell Vaso-Occlusive Crises: A Systematic Review

Exploring the Economic Aspects of β-Thalassemia in Jordan in 2019

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