Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease

Qureshi, Amrana; Kaya, Burak; Pancham, Shivan; Keenan, Russell; Anderson, Jeremy; Akanni, Magbor; Howard, Jo

doi:10.1111/bjh.15235

Cited by 64 publications

(67 citation statements)

References 88 publications

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“…Age restrictions were based upon concerns for adverse effects on growth and development, but subsequent clinical experience has determined these early concerns were unfounded . Based primarily on BABY HUG data, the NHLBI guidelines and newer British guidelines recommend offering treatment as early as age 9 months, but there has been no careful analysis of the effect of starting age on laboratory and clinical outcomes. Since many children have laboratory and clinical manifestations of SCA by 6 months of age, early initiation of therapy may be beneficial.…”

mentioning

confidence: 99%

Hydroxyurea for children with sickle cell anemia: Prescribe it early and often

Ware

McGann

Quinn

2019

Pediatric Blood & Cancer

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mentioning

confidence: 99%

Hydroxyurea for children with sickle cell anemia: Prescribe it early and often

Ware

McGann

Quinn

2019

Pediatric Blood & Cancer

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“…Hydroxyurea, also known as hydroxycarbamide, is a cytotoxic drug that has been successful in reducing the frequency of vaso-occlusive crises and the need for blood transfusions in some cases (NICE 2016). It is the only medicine licensed in the UK for the prevention of recurrent vaso-occlusive crisis in patients with sickle cell disease (Qureshi et al 2018). In sickle cell disease, hydroxyurea can increase haemoglobin levels and reduce intercellular adhesion, which results in improved blood flow and can lessen vaso-occlusions in some patients (Qureshi et al 2018).…”

Section: Hydroxyureamentioning

confidence: 99%

Enhancing the care of patients with sickle cell disease

Blackmore

Taylor

2019

Nursing Standard

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When typically round red blood cells intermittently elongate and become lodged in the body's microcirculation, this can result in painful vaso-occlusive crises, often referred to as 'sickle cell crises'. The sickling and unsickling process can result in acute pain, chronic anaemia, ischaemic injury and multiple organ damage. One of the main concerns raised by patients with sickle cell disease is the lack of knowledge and understanding of their condition among healthcare professionals in acute care settings. Therefore, this article aims to enhance nurses' understanding of sickle cell disease and the effective management of painful vaso-occlusive crises. While sickle cell disease was traditionally perceived to only occur in people of black African or African-Caribbean ethnic origin, this article seeks to challenge this belief and reconsider sickle cell as a public health concern for all.

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“…NIH guidelines in the US recommend that providers “offer hydroxyurea for children with SCA starting at 9 months of age” 31 . The British Society Of Haematology have recently published UK guidelines for the use of Hydroxycarbamide in adults and children, these have been summarised ( Supplementary File 1) 32 .…”

Section: Clinical Complications Of Sickle Cell Diseasementioning

confidence: 99%

Low-dose hydroxycarbamide therapy may offer similar benefit as maximum tolerated dose for children and young adults with sickle cell disease in low-middle-income settings

et al. 2018

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The multiple clinical benefits of hydroxycarbamide in sickle cell disease are supported by a large body of evidence. The maximum tolerated dose (MTD) is the regimen recommended by guidelines from a panel of National Heart, Lung, and Blood Institute (NHLBI) experts, but other dosage regimens have been used in babies (BABY-HUG) 9 to 18 months old (20 mg/kg per day) and developing countries such as India (10 mg/kg per day); however, there has been no direct comparison of the efficacy, effectiveness, or cost-effectiveness of these different regimens. The purpose of this review was to investigate the current situation with various hydroxycarbamide regimens with particular relevance to low-middle-income countries. In regard to methodology, a literature review was undertaken by using multiple databases in PubMed and Google and the search terms included sickle cell disease, hydroxyurea, hydroxycarbamide, sickle cell anaemia, low-middle-income countries, Sub-Saharan Africa, and India. Although MTD regimens have been widely used in research, especially within North America, clinical trials elsewhere tend to use fixed-dose regimens. In a survey of haematologists across Europe and Africa, 60% (75% response rate) did not use the MTD regimen for hydroxycarbamide treatment of sickle cell disease. The recommendations are (1) for practical purposes to commence using fixed-dose hydroxycarbamide in line with BABY-HUG recommendations and then (2) to consider or propose a trial comparing MTD escalation with various fixed doses and to include as end points health-related quality of life, haemoglobin F levels, adherence, and cost-effectiveness.

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Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease

Cited by 64 publications

References 88 publications

Hydroxyurea for children with sickle cell anemia: Prescribe it early and often

Hydroxyurea for children with sickle cell anemia: Prescribe it early and often

Enhancing the care of patients with sickle cell disease

Low-dose hydroxycarbamide therapy may offer similar benefit as maximum tolerated dose for children and young adults with sickle cell disease in low-middle-income settings

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