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Sickle Cell Anemia pp 231–267Cite as

Treatment of Childhood Sickle Cell Disease

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Abstract

With the advances in our understanding of the complex mechanisms that come into play in sickle cell disease (SCD), medical care improves and patients with SCD live longer. It is, thus, essential to have adequate knowledge of the available and potential treatment modalities for all SCD complications to reduce morbidity and mortality. As in all chronic illnesses, patient education is the most important aspect of treatment. Patients should be enrolled in a routine follow-up program with multidisciplinary care for better outcomes. Penicillin prophylaxis and adequate immunizations must be instated as soon as the diagnosis is made. Regular screening is warranted to predict the risk of central nervous system involvement, pulmonary hypertension, nephropathy and retinopathy. In this chapter, we also discuss the management of acute SCD complications including vaso-occlusive painful crises, fever, acute chest syndrome, acute splenic sequestration, cerebrovascular accidents, priapism, aplastic crisis, hepatobiliary complications and ophthalmologic complications. We also present approaches for chronic complications such as pulmonary hypertension, chronic kidney disease, chronic pain, sickle retinopathy, leg ulcers and avascular necrosis. The indications and risks of blood transfusions are discussed in addition to hematopoietic stem cell transplant, the only curative treatment for SCD.

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Abbreviations

AAP:

American Academy of Pediatrics

ACE:

Angiotensin-converting enzyme

ACIP:

Advisory Committee on Immunization Practices

ACS:

Acute chest syndrome

AHS:

Acute hepatic sequestration

AIC:

Acute intrahepatic cholestasis

ASS:

Acute splenic sequestration

ATS:

American Thoracic Society

AVN:

Avascular necrosis

CBC:

Complete blood count

CDC:

Centers for Disease Control and Prevention

CKD:

Chronic kidney disease

CRAO:

Central retinal artery occlusion

CT:

Computed tomography

ED:

Emergency department

ESRD:

End-stage renal disease

FDA:

U.S. Food and Drug Administration

G6PD:

Glucose-6-phosphate dehydrogenase

GvHD:

Graft versus host disease

Hb:

Hemoglobin

Hib:

Haemophilus influenzae type B

Hib-MenCY-TT:

Haemophilus b tetanus toxoid conjugate vaccine

HSCT:

Hematopoietic stem cell transplantation

HU:

Hydroxyurea

IOP:

Intraocular pressure

IV:

Intravenous

LIC:

Liver iron content

MenACWY:

Quadrivalent meningococcal conjugate vaccine

MRI:

Magnetic resonance imaging

MTD:

Maximum tolerated dose

NHLBI:

US National Heart Lung and Blood Institute

NHS:

UK National Health Service

NO:

Nitric oxide

NSAID:

Nonsteroidal anti-inflammatory drug

NT-Pro-BNP:

N-terminal pro-brain natriuretic peptide

PCA:

Patient-controlled analgesia

PCV13:

13-valent pneumococcal vaccine

PCV7:

7-valent pneumococcal vaccine

PH:

Pulmonary hypertension

PPSV23:

23-valent pneumococcal polysaccharide vaccine

PSR:

Progressive sickle retinopathy

PT:

Prothrombin time

PTT:

Partial thromboplastin time

RBC:

Red blood cell

SCD:

Sickle cell disease

SNRI:

Serotonin norepinephrine reuptake inhibitors

TCD:

Transcranial doppler

TIA:

Transient ischemic attack

TRV:

Tricuspid regurgitant velocity

VOC:

Vaso-occlusive crisis

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  1. Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon

    Rouba Abdennour M.D. & Miguel R. Abboud M.D.

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    Fernando Ferreira Costa

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    Nicola Conran

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Abdennour, R., Abboud, M.R. (2016). Treatment of Childhood Sickle Cell Disease. In: Costa, F., Conran, N. (eds) Sickle Cell Anemia. Springer, Cham. https://doi.org/10.1007/978-3-319-06713-1_10

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