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.
Keywords
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
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
References
Abboud MR (2009) Hematopoietic stem-cell transplantation for adults with sickle cell disease. N Engl J Med 361(24):2380–2381
Adamkiewicz TV, Abboud MR, Paley C et al (2009) Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood 114(21):4632–4638
Adams R, McKie V, Nichols F et al (1992) The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 326(9):605–610
Adams RJ, McKie VC, Hsu L et al (1998) Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 339(1):5–11
Adams RJ, Brambilla D, Optimizing Primary Stroke Prevention in Sickle Cell Anemia Trial I (2005) Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med 353(26):2769–2778
Ahmed SG (2011) The role of infection in the pathogenesis of vaso-occlusive crisis in patients with sickle cell disease. Mediterr J Hematol Infect Dis 3(1):e2011028
Ahn H, Li CS, Wang W (2005) Sickle cell hepatopathy: clinical presentation, treatment, and outcome in pediatric and adult patients. Pediatr Blood Cancer 45(2):184–190
Al Hajeri A, Serjeant GR, Fedorowicz Z (2008) Inhaled nitric oxide for acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev 1:CD006957
Alvarez O, Miller ST, Wang WC et al (2012) Effect of hydroxyurea treatment on renal function parameters: results from the multi-center placebo-controlled BABY HUG clinical trial for infants with sickle cell anemia. Pediatr Blood Cancer 59(4):668–674
Alvarez O, Yovetich NA, Scott JP et al (2013) Pain and other non-neurological adverse events in children with sickle cell anemia and previous stroke who received hydroxyurea and phlebotomy or chronic transfusions and chelation: results from the SWiTCH clinical trial. Am J Hematol 88(11):932–938
American Academy of Pediatrics (AAP) (2009) Immunization in special clinical circumstances. In: Pickering LK, Baker CJ, Long SS (eds) Red book: Report of the Committee on Infectious Diseases, 28th edn. American Academy of Pediatrics, Elk Grove Village
Anim SO, Strunk RC, DeBaun MR (2011) Asthma morbidity and treatment in children with sickle cell disease. Expert Rev Respir Med 5(5):635–645
Aoki RY, Saad ST (1995) Enalapril reduces the albuminuria of patients with sickle cell disease. Am J Med 98(5):432–435
Ataga KI, Moore CG, Jones S et al (2006) Pulmonary hypertension in patients with sickle cell disease: a longitudinal study. Br J Haematol 134(1):109–115
Ataga KI, Derebail VK, Archer DR (2014) The glomerulopathy of sickle cell disease. Am J Hematol 89(9):907–914
Atz AM, Wessel DL (1997) Inhaled nitric oxide in sickle cell disease with acute chest syndrome. Anesthesiology 87(4):988–990
Ballas SK, Lieff S, Benjamin LJ et al (2010) Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol 85(1):6–13
Barst RJ, Mubarak KK, Machado RF et al (2010) Exercise capacity and haemodynamics in patients with sickle cell disease with pulmonary hypertension treated with bosentan: results of the ASSET studies. Br J Haematol 149(3):426–435
Baum KF, MacFarlane DE, Maude GH, Serjeant GR (1987) Topical antibiotics in chronic sickle cell leg ulcers. Trans R Soc Trop Med Hyg 81(5):847–849
Bellet PS, Kalinyak KA, Shukla R, Gelfand MJ, Rucknagel DL (1995) Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med 333(11):699–703
Benjamin LJ, Swinson GI, Nagel RL (2000) Sickle cell anemia day hospital: an approach for the management of uncomplicated painful crises. Blood 95(4):1130–1136
Benkerrou M, Alberti C, Couque N et al (2013) Impact of glucose-6-phosphate dehydrogenase deficiency on sickle cell anaemia expression in infancy and early childhood: a prospective study. Br J Haematol 163(5):646–654
Bernini JC, Rogers ZR, Sandler ES et al (1998) Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood 92(9):3082–3089
Beverung LM, Strouse JJ, Hulbert ML et al (2015) Health-related quality of life in children with sickle cell anemia: impact of blood transfusion therapy. Am J Hematol 90(2):139–143
Bhatia M, Walters MC (2008) Hematopoietic cell transplantation for thalassemia and sickle cell disease: past, present, and future. Bone Marrow Transplant 41(2):109–117
Bolanos-Meade J, Fuchs EJ, Luznik L et al (2012) HLA-haploidentical bone marrow transplantation with posttransplant cyclophosphamide expands the donor pool for patients with sickle cell disease. Blood 120(22):4285–4291
Boyd JH, Moinuddin A, Strunk RC, DeBaun MR (2004) Asthma and acute chest in sickle-cell disease. Pediatr Pulmonol 38(3):229–232
Brousse V, Hertz-Pannier L, Consigny Y et al (2009) Does regular blood transfusion prevent progression of cerebrovascular lesions in children with sickle cell disease? Ann Hematol 88(8):785–788
Brousse V, Buffet P, Rees D (2014) The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol 166(2):165–176
Cacciola E, Giustolisi R, Musso R, Longo A, Cacciola E (1989) Antithrombin III concentrate for treatment of chronic leg ulcers in sickle cell-beta thalassemia: a pilot study. Ann Intern Med 111(6):534–536
Callahan ST, Winitzer RF, Keenan P (2001) Transition from pediatric to adult-oriented health care: a challenge for patients with chronic disease. Curr Opin Pediatr 13(4):310–316
Canan H, Ulas B, Altan-Yaycioglu R (2014) Hyperbaric oxygen therapy in combination with systemic treatment of sickle cell disease presenting as central retinal artery occlusion: a case report. J Med Case Rep 8:370
Centers for Disease Control and Prevention (CDC) (2012) Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine for adults with immunocompromising conditions: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rpt 61(40):816–819
Centers for Disease Control and Prevention (CDC) (2014a) Recommended adult immunization schedule, by vaccine and age group. http://www.cdc.gov/vaccines/schedules/hcp/imz/adult.html. Accessed 20 Nov 2014
Centers for Disease Control and Prevention (CDC) (2014b) Recommended immunization schedule for persons aged 0 through 18 years. http://www.cdc.gov/vaccines/schedules/hcp/imz/child-adolescent.html. Accessed 20 Nov 2014
Charache S, Terrin ML, Moore RD et al (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 332(20):1317–1322
Chinegwundoh F, Anie KA (2004) Treatments for priapism in boys and men with sickle cell disease. Cochrane Database Syst Rev 4:CD004198
Co JP, Johnson KB, Duggan AK, Casella JF, Wilson M (2003) Does a clinical pathway improve the quality of care for sickle cell anemia? Jt Comm J Qual Saf 29(4):181–190
Cohen RT, Madadi A, Blinder MA et al (2011) Recurrent, severe wheezing is associated with morbidity and mortality in adults with sickle cell disease. Am J Hematol 86(9):756–761
Crosby LE, Modi AC, Lemanek KL et al (2009) Perceived barriers to clinic appointments for adolescents with sickle cell disease. J Pediatr Hematol Oncol 31(8):571–576
Curran EL, Fleming JC, Rice K, Wang WC (1997) Orbital compression syndrome in sickle cell disease. Ophthalmology 104(10):1610–1615
Davies SC, Gilmore A (2003) The role of hydroxyurea in the management of sickle cell disease. Blood Rev 17(2):99–109
Davies EG, Riddington C, Lottenberg R, Dower N (2004) Pneumococcal vaccines for sickle cell disease. Cochrane Database Syst Rev 1:CD003885
Davies JM, Lewis PN, Wimperis J et al (2011) Review of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen: prepared on behalf of the British Committee for Standards in Haematology by a Working Party of the Haemato-Oncology Task Force. Br J Haematol 155:308–317
De Castro LM, Jonassaint JC, Graham FL, Ashley-Koch A, Telen MJ (2008) Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes. Am J Hematol 83(1):19–25
de Montalembert M, Guitton C, French Reference Centre for Sickle Cell D (2014) Transition from paediatric to adult care for patients with sickle cell disease. Br J Haematol 164(5):630–635
De Montalembert M, Abboud MR, Fiquet A et al (2015) 13-valent pneumococcal conjugate vaccine (PCV13) is immunogenic and safe in children 6-17 years of age with sickle cell disease previously vaccinated with 23-valent pneumococcal polysaccharide vaccine (PPSV23): results of a phase 3 study. Pediatr Blood Cancer 62:1427–1436
DeBaun MR, Telfair J (2012) Transition and sickle cell disease. Pediatrics 130(5):926–935
DeBaun MR, Gordon M, McKinstry RC et al (2014) Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med 371(8):699–710
Delicou S, Maragkos K (2013) Pain management in patients with sickle cell disease – a review. EMJ Hema 1:30–36
Downes SM, Hambleton IR, Chuang EL et al (2005) Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study. Ophthalmology 112(11):1869–1875
Ebong WW, Kolawole TM (1986) Aseptic necrosis of the femoral head in sickle-cell disease. Br J Rheumatol 25(1):34–39
El-Beshlawy A, Abd El Raouf E, Mostafa F et al (2006) Diastolic dysfunction and pulmonary hypertension in sickle cell anemia: is there a role for L-carnitine treatment? Acta Haematol 115(1-2):91–96
Emond AM, Collis R, Darvill D et al (1985) Acute splenic sequestration in homozygous sickle cell disease: natural history and management. J Pediatr 107(2):201–206
Emre U, Miller ST, Gutierez M et al (1995) Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr 127(6):901–904
Falk RJ, Scheinman J, Phillips G et al (1992) Prevalence and pathologic features of sickle cell nephropathy and response to inhibition of angiotensin-converting enzyme. N Engl J Med 326(14):910–915
Falletta JM, Woods GM, Verter JI et al (1995) Discontinuing penicillin prophylaxis in children with sickle cell anemia. Prophylactic Penicillin Study II. J Pediatr 127(5):685–690
Farber MD, Jampol LM, Fox P et al (1991) A randomized clinical trial of scatter photocoagulation of proliferative sickle cell retinopathy. Arch Ophthalmol 109(3):363–367
Files B, Brambilla D, Kutlar A et al (2002) Longitudinal changes in ferritin during chronic transfusion: a report from the stroke prevention trial in sickle cell anemia (STOP). J Pediatr Hematol Oncol 24(4):284–290
Foucan L, Bourhis V, Bangou J et al (1998) A randomized trial of captopril for microalbuminuria in normotensive adults with sickle cell anemia. Am J Med 104(4):339–342
Gaston MH, Verter JI, Woods G et al (1986) Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. N Engl J Med 314(25):1593–1599
Gbadoe AD, Atakouma Y, Kusiaku K, Assimadi JK (2001) Management of sickle cell priapism with etilefrine. Arch Dis Child 85(1):52–53
Gharaibeh A, Savage HI, Scherer RW, Goldberg MF, Lindsley K (2013) Medical interventions for traumatic hyphema. Cochrane Database Syst Rev 12:CD005431
Gibbs WJ, Hagemann TM (2004) Purified poloxamer 188 for sickle cell vaso-occlusive crisis. Ann Pharmacother 38(2):320–324
Gill FM, Brown A, Gallagher D et al (1989) Newborn experience in the cooperative study of sickle cell disease. Pediatrics 83(5 Pt 2):827–829
Gladwin MT, Sachdev V, Jison ML et al (2004) Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 350(9):886–895
Godfrey GJ, Lockwood W, Kong M, Bertolone S, Raj A (2010) Antibody development in pediatric sickle cell patients undergoing erythrocytapheresis. Pediatr Blood Cancer 55(6):1134–1137
Haberkern CM, Neumayr LD, Orringer EP et al (1997) Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Preoperative Transfusion in Sickle Cell Disease Study Group. Blood 89(5):1533–1542
Hirst C, Owusu-Ofori S (2012) Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev 9:CD003427
Howard J, Malfroy M, Llewelyn C et al (2013) The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet 381(9870):930–938
Hsieh MM, Fitzhugh CD, Weitzel RP et al (2014) Nonmyeloablative HLA-matched sibling allogeneic hematopoietic stem cell transplantation for severe sickle cell phenotype. JAMA 312(1):48–56
Hsu LL, Batts BK, Rau JL (2005) Positive expiratory pressure device acceptance by hospitalized children with sickle cell disease is comparable to incentive spirometry. Respir Care 50(5):624–627
Huang E, Parke C, Mehrnia A et al (2013) Improved survival among sickle cell kidney transplant recipients in the recent era. Nephrol Dial Transplant 28(4):1039–1046
Hulbert ML, Scothorn DJ, Panepinto JA et al (2006) Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia. J Pediatr 149(5):710–712
Irizarry K, Rossbach HC, Ignacio JR et al (2006) Sickle cell intrahepatic cholestasis with cholelithiasis. Pediatr Hematol Oncol 23(2):95–102
Issa H, Al-Salem AH (2010) Hepatobiliary manifestations of sickle cell anemia. Gastroenterol Res 3(1):1–8
Jacobson SJ, Kopecky EA, Joshi P, Babul N (1997) Randomised trial of oral morphine for painful episodes of sickle-cell disease in children. Lancet 350(9088):1358–1361
Jia L, Bonaventura C, Bonaventura J, Stamler JS (1996) S-nitrosohaemoglobin: a dynamic activity of blood involved in vascular control. Nature 380(6571):221–226
Johnson CS (2004) The liver in sickle cell disease. In: Okpala IE (ed) Practical management of haemoglobinopathies. Blackwell Publishing, Oxford, pp 120–129
Johnston RB Jr, Newman SL, Struth AG (1973) An abnormality of the alternate pathway of complement activation in sickle-cell disease. N Engl J Med 288(16):803–808
Kalpatthi R, Peters B, Kane I et al (2010) Safety and efficacy of high dose intravenous desferrioxamine for reduction of iron overload in sickle cell disease. Pediatr Blood Cancer 55(7): 1338–1342
Karam LB, Disco D, Jackson SM et al (2008) Liver biopsy results in patients with sickle cell disease on chronic transfusions: poor correlation with ferritin levels. Pediatr Blood Cancer 50(1):62–65
Khoury R, Abboud MR (2011) Stem-cell transplantation in children and adults with sickle cell disease: an update. Expert Rev Hematol 4(3):343–351
Kimmel AS, Magargal LE, Stephens RF, Cruess AF (1986) Peripheral circumferential retinal scatter photocoagulation for the treatment of proliferative sickle retinopathy. An update. Ophthalmology 93(11):1429–1434
Kinney TR, Ware RE, Schultz WH, Filston HC (1990) Long-term management of splenic sequestration in children with sickle cell disease. J Pediatr 117(2 Pt 1):194–199
Kinney TR, Helms RW, O'Branski EE et al (1999) Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group. Blood 94(5):1550–1554
Klings ES, Machado RF, Barst RJ et al (2014) An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med 189(6):727–740
Koshy M, Burd L, Wallace D, Moawad A, Baron J (1988) Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study. N Engl J Med 319(22):1447–1452
La Grenade L, Thomas PW, Serjeant GR (1993) A randomized controlled trial of solcoseryl and duoderm in chronic sickle-cell ulcers. West Indian Med J 42(3):121–123
Laurin LP, Nachman PH, Desai PC, Ataga KI, Derebail VK (2014) Hydroxyurea is associated with lower prevalence of albuminuria in adults with sickle cell disease. Nephrol Dial Transplant 29(6):1211–1218
Lebensburger JD, Miller ST, Howard TH et al (2012) Influence of severity of anemia on clinical findings in infants with sickle cell anemia: analyses from the BABY HUG study. Pediatr Blood Cancer 59(4):675–678
Lezcano NE, Odo N, Kutlar A, Brambilla D, Adams RJ (2006) Regular transfusion lowers plasma free hemoglobin in children with sickle-cell disease at risk for stroke. Stroke 37(6):1424–1426
Liem RI, Calamaras DM, Chhabra MS et al (2008) Sudden-onset blindness in sickle cell disease due to retinal artery occlusion. Pediatr Blood Cancer 50(3):624–627
Luffy R, Grove SK (2003) Examining the validity, reliability, and preference of three pediatric pain measurement tools in African-American children. Pediatr Nurs 29(1):54–59
Lusher JM, Haghighat H, Khalifa AS (1976) A prophylactic transfusion program for children with sickle cell anemia complicated by CNS infarction. Am J Hematol 1(2):265–273
Machado RF, Martyr S, Kato GJ et al (2005) Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension. Br J Haematol 130(3):445–453
Machado RF, Barst RJ, Yovetich NA et al (2011) Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity. Blood 118(4):855–864
Mahomed K (2000) Prophylactic versus selective blood transfusion for sickle cell anaemia during pregnancy. Cochrane Database Syst Rev 2:CD000040
Mantadakis E, Ewalt DH, Cavender JD, Rogers ZR, Buchanan GR (2000) Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood 95(1):78–82
Maples BL, Hagemann TM (2004) Treatment of priapism in pediatric patients with sickle cell disease. Am J Health Syst Pharm 61(4):355–363
Marti-Carvajal AJ, Knight-Madden JM, Martinez-Zapata MJ (2014a) Interventions for treating leg ulcers in people with sickle cell disease. Cochrane Database Syst Rev 12:CD008394
Marti-Carvajal AJ, Sola I, Agreda-Perez LH (2014b) Treatment for avascular necrosis of bone in people with sickle cell disease. Cochrane Database Syst Rev 7:CD004344
McCavit TL, Quinn CT, Techasaensiri C, Rogers ZR (2011) Increase in invasive Streptococcus pneumoniae infections in children with sickle cell disease since pneumococcal conjugate vaccine licensure. J Pediatr 158(3):505–507
McClellan AC, Luthi JC, Lynch JR et al (2012) High one year mortality in adults with sickle cell disease and end-stage renal disease. Br J Haematol 159(3):360–367
McGann PT, Ware RE (2011) Hydroxyurea for sickle cell anemia: what have we learned and what questions still remain? Curr Opin Hematol 18(3):158–165
McKie KT, Hanevold CD, Hernandez C et al (2007) Prevalence, prevention, and treatment of microalbuminuria and proteinuria in children with sickle cell disease. J Pediatr Hematol Oncol 29(3):140–144
McMahon L, Tamary H, Askin M et al (2010) A randomized phase II trial of Arginine Butyrate with standard local therapy in refractory sickle cell leg ulcers. Br J Haematol 151(5):516–524
McPherson ME, Anderson AR, Castillejo MI et al (2010) HLA alloimmunization is associated with RBC antibodies in multiply transfused patients with sickle cell disease. Pediatr Blood Cancer 54(4):552–558
McQuay H, Moore A (1998) An evidence-based resource for pain relief, 1st edn. Oxford University Press, Oxford
Mehari A, Alam S, Tian X et al (2013) Hemodynamic predictors of mortality in adults with sickle cell disease. Am J Respir Crit Care Med 187(8):840–847
Meloni A, Puliyel M, Pepe A et al (2014) Cardiac iron overload in sickle-cell disease. Am J Hematol 89(7):678–683
Miller ST (2011) How I treat acute chest syndrome in children with sickle cell disease. Blood 117(20):5297–5305
Miller ST, Wright E, Abboud M et al (2001) Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. J Pediatr 139(6):785–789
Minniti CP, Gorbach AM, Xu D et al (2014) Topical sodium nitrite for chronic leg ulcers in patients with sickle cell anaemia: a phase 1 dose-finding safety and tolerability trial. The Lancet Haematol 1(3):e95–e103
Moriarty BJ, Acheson RW, Condon PI, Serjeant GR (1988) Patterns of visual loss in untreated sickle cell retinopathy. Eye (Lond) 2(Pt 3):330–335
Morris CR, Morris SM Jr, Hagar W et al (2003) Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med 168(1):63–69
Mouttalib S, Rice HE, Snyder D et al (2012) Evaluation of partial and total splenectomy in children with sickle cell disease using an Internet-based registry. Pediatr Blood Cancer 59(1):100–104
Mukisi-Mukaza M, Manicom O, Alexis C et al (2009) Treatment of sickle cell disease's hip necrosis by core decompression: a prospective case-control study. Orthop Traumatol Surg Res 95(7):498–504
NIH National Heart, Lung and Blood Institute (NHLBI) (2014). Evidence-based management of sickle cell disease. Expert panel report
Neumayr LD, Aguilar C, Earles AN et al (2006) Physical therapy alone compared with core decompression and physical therapy for femoral head osteonecrosis in sickle cell disease. Results of a multicenter study at a mean of three years after treatment. J Bone Joint Surg Am 88(12):2573–2582
Noe HN, Wilimas J, Jerkins GR (1981) Surgical management of priapism in children with sickle cell anemia. J Urol 126(6):770–771
Okomo U, Meremikwu MM (2012) Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database Syst Rev 6:CD005406
Okpala IE (2004a) Treatment modalities for pain in sickle cell disease. In: Okpala IE (ed) Practical management of haemoglobinopathies. Blackwell Publishing, Oxford, pp 72–75
Okpala IE (2004b) Sickle cell crisis. In: Okpala IE (ed) Practical management of haemoglobinopathies. Blackwell Publishing, Oxford, pp 63–71
Okpala IE (2004c) Pulmonary hypertension: a complication of haemolytic states. In: Okpala IE (ed) Practical management of haemoglobinopathies. Blackwell Publishing, Oxford, pp 130–133
Okpala I, Westerdale N, Jegede T, Cheung B (2002) Etilefrine for the prevention of priapism in adult sickle cell disease. Br J Haematol 118(3):918–921
Oniyangi O, Cohall DH (2013) Phytomedicines (medicines derived from plants) for sickle cell disease. Cochrane Database Syst Rev 1:CD004448
Oppert M, Jorres A, Barckow D et al (2004) Inhaled nitric oxide for ARDS due to sickle cell disease. Swiss Med Wkly 134(11-12):165–167
Oringanje C, Nemecek E, Oniyangi O (2013) Hematopoietic stem cell transplantation for people with sickle cell disease. Cochrane Database Syst Rev 5:CD007001
Owusu-Ofori S, Hirst C (2013) Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database Syst Rev 5:CD003425
Parent F, Bachir D, Inamo J et al (2011) A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 365(1):44–53
Pashankar FD, Carbonella J, Bazzy-Asaad A, Friedman A (2008) Prevalence and risk factors of elevated pulmonary artery pressures in children with sickle cell disease. Pediatrics 121(4):777–782
Pegelow CH, Adams RJ, McKie V et al (1995) Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr 126(6):896–899
Platt OS (2005) Preventing stroke in sickle cell anemia. N Engl J Med 353(26):2743–2745
Platt OS, Orkin SH, Dover G et al (1984) Hydroxyurea enhances fetal hemoglobin production in sickle cell anemia. J Clin Invest 74(2):652–656
Platt OS, Brambilla DJ, Rosse WF et al (1994) Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 330(23):1639–1644
Porter J, Garbowski M (2013) Consequences and management of iron overload in sickle cell disease. Hematology Am Soc Hematol Educ Program 2013:447–456
Powars DR, Johnson CS (1996) Priapism. Hematol Oncol Clin North Am 10(6):1363–1372
Powars D, Wilson B, Imbus C, Pegelow C, Allen J (1978) The natural history of stroke in sickle cell disease. Am J Med 65(3):461–471
Quinn CT, Rogers ZR, McCavit TL, Buchanan GR (2010) Improved survival of children and adolescents with sickle cell disease. Blood 115(17):3447–3452
Rachid-Filho D, Cavalcanti AG, Favorito LA, Costa WS, Sampaio FJ (2009) Treatment of recurrent priapism in sickle cell anemia with finasteride: a new approach. Urology 74(5):1054–1057
Rao S, Gooden S (1985) Splenic sequestration in sickle cell disease: role of transfusion therapy. Am J Pediatr Hematol Oncol 7(3):298–301
Reagan MM, DeBaun MR, Frei-Jones MJ (2011) Multi-modal intervention for the inpatient management of sickle cell pain significantly decreases the rate of acute chest syndrome. Pediatr Blood Cancer 56(2):262–266
Rees DC, Olujohungbe AD, Parker NE et al (2003) Guidelines for the management of the acute painful crisis in sickle cell disease. Br J Haematol 120(5):744–752
Rees DC, Williams TN, Gladwin MT (2010) Sickle-cell disease. Lancet 376(9757):2018–2031
Savlov D, Beck CE, DeGroot J, Odame I, Friedman JN (2014) Predictors of bacteremia among children with sickle cell disease presenting with fever. J Pediatr Hematol Oncol 36(5): 384–388
Scothorn DJ, Price C, Schwartz D et al (2002) Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke. J Pediatr 140(3):348–354
Segal JB, Strouse JJ, Beach MC et al (2008) Hydroxyurea for the treatment of sickle cell disease. Evid Rep Technol Assess (Full Rep) 165:1–95
Serjeant GR, Galloway RE, Gueri MC (1970) Oral zinc sulphate in sickle-cell ulcers. Lancet 2(7679):891–892
Serjeant GR, de Ceulaer K, Maude GH (1985) Stilboestrol and stuttering priapism in homozygous sickle-cell disease. Lancet 2(8467):1274–1276
Serjeant GR, Ceulaer CD, Lethbridge R et al (1994) The painful crisis of homozygous sickle cell disease: clinical features. Br J Haematol 87(3):586–591
Shaiova L, Wallenstein D (2004) Outpatient management of sickle cell pain with chronic opioid pharmacotherapy. J Natl Med Assoc 96(7):984–986
Shao SH, Orringer EP (1995) Sickle cell intrahepatic cholestasis: approach to a difficult problem. Am J Gastroenterol 90(11):2048–2050
Sharpe CC, Thein SL (2011) Sickle cell nephropathy - a practical approach. Br J Haematol 155(3):287–297
Sharpe CC, Thein SL (2014) How I treat renal complications in sickle cell disease. Blood 123(24):3720–3726
Shenoy S (2013) Hematopoietic stem-cell transplantation for sickle cell disease: current evidence and opinions. Ther Adv Hematol 4(5):335–344
Shihabuddin BS, Scarfi CA (2014) Fever in children with sickle cell disease: are all fevers equal? J Emerg Med 47(4):395–400
Singh PC, Ballas SK (2015) Emerging drugs for sickle cell anemia. Expert Opin Emerg Drugs 20(1):47–61
Smith WR, Penberthy LT, Bovbjerg VE et al (2008) Daily assessment of pain in adults with sickle cell disease. Ann Intern Med 148(2):94–101
Sobota A, Graham DA, Heeney MM, Neufeld EJ (2010) Corticosteroids for acute chest syndrome in children with sickle cell disease: variation in use and association with length of stay and readmission. Am J Hematol 85(1):24–28
Sokol JA, Baron E, Lantos G, Kazim M (2008) Orbital compression syndrome in sickle cell disease. Ophthal Plast Reconstr Surg 24(3):181–184
Steinberg MH, Barton F, Castro O et al (2003) Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA 289(13):1645–1651
Steinberg MH, McCarthy WF, Castro O et al (2010) The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up. Am J Hematol 85(6):403–408
Strouse JJ, Hulbert ML, DeBaun MR, Jordan LC, Casella JF (2006) Primary hemorrhagic stroke in children with sickle cell disease is associated with recent transfusion and use of corticosteroids. Pediatrics 118(5):1916–1924
Sullivan KJ, Goodwin SR, Evangelist J, Moore RD, Mehta P (1999) Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent. Crit Care Med 27(11):2563–2568
Sundaram N, Bennett M, Wilhelm J et al (2011) Biomarkers for early detection of sickle nephropathy. Am J Hematol 86(7):559–566
Thomas V (2000) Cognitive behavioural therapy in pain management for sickle cell disease. Int J Palliat Nurs 6(9):434–442
Thompson WE, Eriator I (2014) Pain control in sickle cell disease patients: use of complementary and alternative medicine. Pain Med 15(2):241–246
Udezue E, Herrera E (2007) Pain management in adult acute sickle cell pain crisis: a viewpoint. West Afr J Med 26(3):179–182
UK National Health Service (NHS) (2010) Sickle cell disease in childhood. Standards and guidelines for clinical care, 2nd edn. London. ISBN:0-9554319-7-2
van Beers EJ, van Tuijn CF, Nieuwkerk PT et al (2007) Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. Am J Hematol 82(11):955–960
Vichinsky E, Hurst D, Earles A, Kleman K, Lubin B (1988) Newborn screening for sickle cell disease: effect on mortality. Pediatrics 81(6):749–755
Vichinsky EP, Haberkern CM, Neumayr L et al (1995) A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med 333(4):206–213
Vichinsky EP, Neumayr LD, Earles AN et al (2000) Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 342(25):1855–1865
Vichinsky E, Onyekwere O, Porter J et al (2007) A randomised comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. Br J Haematol 136(3):501–508
Voskaridou E, Christoulas D, Bilalis A et al (2010) The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood 115(12):2354–2363
Walters MC (2005) Stem cell therapy for sickle cell disease: transplantation and gene therapy. Hematology Am Soc Hematol Educ Program 66–73
Wang WC, Dwan K (2013) Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease. Cochrane Database Syst Rev 11:CD003146
Wang WC, Ware RE, Miller ST et al (2011) Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet 377(9778):1663–1672
Ware RE (2010) How I use hydroxyurea to treat young patients with sickle cell anemia. Blood 115(26):5300–5311
Ware RE, Rees RC, Sarnaik SA et al (2010) Renal function in infants with sickle cell anemia: baseline data from the BABY HUG trial. J Pediatr 156(1):66–70 e61
Ware RE, Helms RW, Investigators SW (2012) Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood 119(17):3925–3932
Wethers DL, Ramirez GM, Koshy M et al (1994) Accelerated healing of chronic sickle-cell leg ulcers treated with RGD peptide matrix. RGD Study Group. Blood 84(6):1775–1779
Wilimas JA, Flynn PM, Harris S et al (1993) A randomized study of outpatient treatment with ceftriaxone for selected febrile children with sickle cell disease. N Engl J Med 329(7):472–476
Williamson TH, Rajput R, Laidlaw DA, Mokete B (2009) Vitreoretinal management of the complications of sickle cell retinopathy by observation or pars plana vitrectomy. Eye (Lond) 23(6):1314–1320
Working Party of the British Committee for Standards in Haematology Clinical Haematology Task F (1996) Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. BMJ 312(7028):430–434
Wright J (2004) Acute chest syndrome in sickle cell disease. In: Okpala IE (ed) Practical management of haemoglobinopathies. Blackwell Publishing, Oxford, pp 88–98
Wright J, Ahmedzai SH (2010) The management of painful crisis in sickle cell disease. Curr Opin Support Palliat Care 4(2):97–106
Yang YM, Shah AK, Watson M, Mankad VN (1995) Comparison of costs to the health sector of comprehensive and episodic health care for sickle cell disease patients. Public Health Rep 110(1):80–86
Yusuf HR, Atrash HK, Grosse SD, Parker CS, Grant AM (2010) Emergency department visits made by patients with sickle cell disease: a descriptive study, 1999-2007. Am J Prev Med 38(4 Suppl):S536–S541
Zimmerman SA, Schultz WH, Davis JS et al (2004) Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood 103(6): 2039–2045
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-319-06713-1_10
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-06712-4
Online ISBN: 978-3-319-06713-1
eBook Packages: MedicineMedicine (R0)