Sickle Cell Anemia Questions & Answers

Updated: Jan 29, 2019
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Questions & Answers

Overview

What is sickle cell disease (SCD)?

What is the role of imaging studies in the diagnosis of sickle cell disease (SCD)?

What are the signs and symptoms of sickle cell disease (SCD)?

What can trigger vaso-occlusive crisis in sickle cell disease (SCD)?

How is sickle cell disease (SCD) diagnosed?

Which lab tests are performed in with the workup of sickle cell disease (SCD)?

Which abnormal blood pressure (BP) patterns suggest sickle cell disease (SCD) in asymptomatic children?

What are the goals of treatment in sickle cell disease (SCD)?

Which medications are used to treat sickle cell disease (SCD)?

What are the nonpharmacological approaches in the treatment of sickle cell disease (SCD)?

What treatment approaches combine pharmacologic and nonpharmacological interventions for sickle cell disease (SCD)?

What is the benefit of the sickle cell trait (SCT) in malaria-endemic areas?

When is a less severe form of sickle cell disease (SCD) likely to occur?

What are the major sickle genotypes?

What is the sickle cell trait (SCT)?

What are the early manifestations of sickle cell disease (SCD)?

Is universal screening for sickle cell disease (SCD) mandated in the US?

What is the role of hemoglobin S (HbS) in the pathogenesis of sickle cell disease (SCD)?

What happens to hemoglobin S (HbS) under deoxy conditions?

What is the role of oxygen tension in the pathogenesis of sickle cell disease (SCD)?

What is the pathophysiology of the sickling process in sickle cell disease (SCD)?

What is the result of recurrent episodes of sickling in sickle cell disease (SCD)?

What happens when red blood cells (RBCs) sickle?

What is the role of very late antigen-4 (VLA-4) in the pathogenesis of sickle cell disease (SCD)?

What is the role of nitric oxide in the pathogenesis of sickle cell disease (SCD)?

What is the role of inflammatory activation of the endothelium in the pathogenesis of sickle cell disease (SCD)?

What is the role of adhesion molecules in the pathogenesis of sickle cell disease (SCD)?

What causes sickle red blood cells (RBCs) to adhere to endothelium?

What causes sickle red blood cells (RBCs) to adhere to macrophages?

What is hemolysis and what is its role in the pathophysiology of sickle cell disease (SCD)?

When do the symptoms of sickle cell disease (SCD) develop?

Which physiological changes in red blood cells (RBCs) suggest sickle cell disease (SCD)?

Which processes result in bone and joint destruction in sickle cell disease (SCD)?

What causes osteonecrosis in sickle cell disease (SCD)?

Where does bone infarction typically occur in sickle cell disease (SCD)?

What is the presentation of osteonecrosis in sickle cell disease (SCD)?

Which skeletal manifestations of sickle cell disease (SCD) are caused by infarction of bone and bone marrow?

What is the role of bone marrow hyperplasia in sickle cell disease (SCD)?

Which skeletal manifestations of sickle cell disease (SCD) are caused by bone marrow hyperplasia?

Which growth defects may occur in patients with sickle cell disease (SCD)?

What are the renal manifestations of sickle cell disease (SCD)?

What are the manifestations of sickle cell disease (SCD) in the spleen?

What is the pathogenesis of chronic hemolytic anemia in sickle cell disease (SCD)?

What is the pathogenesis of pulmonary hypertension in sickle cell disease (SCD)?

What is the major cause of morbidity and mortality in sickle cell disease (SCD)?

Why are patients with sickle cell disease (SCD) at higher risk of infection from common bacteria?

Where did sickle cell disease (SCD) originate?

How do variants of the hemoglobin S (HbS) gene affect the severity of malaria?

Which factors can precipitate the sickling process that prompts a crisis in sickle cell disease (SCD)?

Which factors increase the risk of vaso-occlusive crises in sickle cell disease (SCD)?

Which factors often precede aplastic crises in sickle cell disease (SCD)?

What populations are most likely to develop sickle cell disease (SCD)?

What is the prevalence of the sickle gene in the US?

What is the prevalence of sickle cell disease (SCD) in the US?

What is the global prevalence of sickle cell disease (SCD)?

Is sickle cell disease (SCD) more common in males or females?

Does the clinical presentation of sickle cell disease (SCD) vary among age groups?

What is the prognosis of sickle cell disease (SCD)?

What is the socioeconomic impact of sickle cell disease (SCD)?

Which prognostic factors predict an adverse outcome in sickle cell disease (SCD)?

How does sickle cell disease (SCD) affect pregnancy?

What is the mortality rate of sickle cell disease (SCD)?

What is the survival rate for children with sickle cell disease (SCD)?

Has the mortality rate of sickle cell disease (SCD) improved over the past 30 years?

What is the life expectancy of patients with sickle cell disease (SCD) and end-stage renal disease (ESRD)?

What is the life expectancy of patients with homozygous HbS (HbSS) sickle cell disease (SCD)?

Which factors have contributed to an increase in life expectancy in sickle cell disease (SCD)?

What are the possible complications for aging patients with sickle cell disease (SCD)?

What education should patients with sickle cell disease (SCD) receive?

When should medical care be sought by patients with sickle cell disease (SCD)?

What should be avoided by patients with sickle cell disease (SCD)?

How should families be educated about sickle cell disease (SCD)?

What genetic information should be given to patients with sickle cell disease (SCD) or sickle cell trait (SCT)?

What is the likelihood that a child will have sickle cell disease (SCD) if both parents have the sickle cell trait (SCT)?

What encouragement can be given to families of patients with sickle cell disease (SCD)?

Presentation

What is the presentation of cholelithiasis (gallstones) in sickle cell disease (SCD)?

At what age do the symptoms of sickle cell disease (SCD) typically develop?

What is the most common clinical manifestation of sickle cell disease (SCD)?

How common is vaso-occlusive crisis in patients with homozygous HbS (HbSS) sickle cell disease (SCD)?

How do pain crises develop in sickle cell disease (SCD)?

Which patterns of bone pain suggest sickle cell disease (SCD)?

What triggers vaso-occlusive crises in sickle cell disease (SCD)?

Can dehydration precipitate pain in vaso-occlusive crises in sickle cell disease (SCD)?

Can changes in body temperature trigger vaso-occlusive crises in sickle cell disease (SCD)?

Do patients with sickle cell disease (SCD) experience chronic pain?

When is anemia present in sickle cell disease (SCD)?

What are the symptoms of anemia in children with sickle cell disease (SCD)?

What causes aplastic crisis in sickle cell disease (SCD)?

When is splenic sequestration most likely to occur in patients with sickle cell disease (SCD)?

How is splenic sequestration managed in sickle cell disease (SCD)?

What causes extreme susceptibility to infection in sickle cell disease (SCD)?

Which organisms pose the greatest risk for infection in sickle cell disease (SCD)?

What are the effects of sickle cell disease (SCD) on growth and maturation?

What is the hand-foot syndrome in sickle cell disease (SCD)?

What is the disease course of hand-foot syndrome in sickle cell disease (SCD)?

How does acute chest syndrome present in children and adults with sickle cell disease (SCD)?

Should acute chest syndrome in sickle cell disease (SCD) be treated as a medical emergency?

How does acute chest syndrome in sickle cell disease (SCD) begin?

What are the central nervous system (CNS) manifestations of sickle cell disease (SCD)?

When are patients with sickle cell disease (SCD) at risk for convulsions?

What are the potential central nervous system (CNS) manifestations of sickle cell disease (SCD) in children?

Are patients with sickle cell disease (SCD) at increased risk for hemorrhagic stroke?

Which cardiac complications may be present in sickle cell disease (SCD)?

What findings suggest cholecystitis in patients with sickle cell disease (SCD)?

How is the renal system involved in sickle cell disease (SCD)?

What are the possible ocular (ophthalmic) complications of sickle cell disease (SCD)?

What causes leg ulcers in sickle cell disease (SCD)?

How common is priapism in sickle cell disease (SCD)?

Which patients with sickle cell disease (SCD) are at an increased risk for avascular necrosis (AVN)?

How is hip disease managed in patients with sickle cell disease (SCD)?

How does pulmonary hypertension develop in sickle cell disease (SCD)?

What is the incidence of pulmonary hypertension in patients with sickle cell disease (SCD)?

How is pulmonary hypertension characterized in sickle cell disease (SCD)?

What causes acute bone pain crisis in patients with sickle cell disease (SCD) and what are the symptoms?

What are the physical findings in sickle cell disease (SCD)?

What does hypotension and tachycardia indicate in sickle cell disease (SCD)?

Which symptoms suggest cardiovascular complications in sickle cell disease (SCD)?

What does a fever suggest in children with sickle cell disease (SCD)?

What is the role of auscultation in the physical exam in patients with sickle cell disease (SCD)?

How is spleen size measured in children with sickle cell disease (SCD)?

When do growth parameters typically fall in children with sickle cell disease (SCD)?

What physical exam should be performed in patients with sickle cell disease (SCD)?

What are ocular (ophthalmic) manifestations in sickle cell disease (SCD)?

When should meningitis be suspected in patients with sickle cell disease (SCD)?

Which skeletal findings are typical in children with sickle cell disease (SCD)?

Which physical findings suggest acute bone infarction in patients with sickle cell disease (SCD)?

What are the possible sequelae of chronic bone infarction in patients with sickle cell disease (SCD)?

What is hand-food syndrome in patients with sickle cell disease (SCD)?

What are the symptoms of osteonecrosis in patients with sickle cell disease (SCD)?

What causes osteomyelitis in patients with sickle cell disease (SCD)?

DDX

How is HbS-beta 0 thalassemia diagnosed?

How is the diagnosis of sickle cell disease (SCD) confirmed?

Which sickle cell variants must be distinguished from hemoglobin S (HbS) disease in sickle cell disease (SCD)?

What is the presentation of hemoglobin C (HbSC) disease?

How is hemoglobin C (HbSC) disease diagnosed?

What is the sickle cell trait (SCT)?

How do hemoglobin S (HbS) variants in sickle cell disease (SCD) occur?

How are Gaucher disease and sickle cell disease (SCD) differentiated?

Which conditions should be included in the differential diagnoses of sickle cell disease (SCD)?

What are the differential diagnoses for Sickle Cell Anemia?

Workup

When is screening for hemoglobin S (HbS) done in the US?

Which test is performed for the prenatal diagnosis of sickle cell disease (SCD)?

How often should pulmonary function tests (PFTs) be performed in children with sickle cell disease (SCD)?

What are the roles of transcranial near-infrared spectroscopy and cerebral oximetry in the workup of sickle cell disease (SCD) in children?

What is the role of transcranial Doppler (TCD) ultrasonography in the workup of sickle cell disease (SCD)?

What is the role of lumbar puncture in the management of children with sickle cell disease (SCD)?

Which high-risk features of sickle cell disease (SCD) require hospitalization?

What are the BCSH treatment guidelines for patients with sickle cell disease (SCD) admitted to the hospital?

What is the presentation of acute chest syndrome in patients with sickle cell disease (SCD)?

What are the newborn screening guidelines for hemoglobin disorders in the US?

Which hemoglobin screening results suggest sickle cell disease (SCD)?

What is the role of hemoglobin electrophoresis a in the diagnosis of sickle cell disease (SCD)?

Which hemoglobin electrophoresis findings suggest sickle cell disease (SCD)?

What are the typical baseline blood study abnormalities in patients with sickle cell disease (SCD)?

Which peripheral blood smear findings suggest sickle cell disease (SCD)?

What is the suggested schedule for routine lab testing for sickle cell disease (SCD)?

Can lab tests be used to distinguish pain crisis from the baseline condition of sickle cell disease (SCD)?

Are routine CBC counts and reticulocyte counts necessary in all patients with sickle cell disease (SCD) who have an acute illness?

Which tests should be performed in febrile children with sickle cell disease (SCD)?

Which CBC count findings suggest hematologic crisis in sickle cell disease (SCD)?

What is the role of reticulocyte percentage testing in sickle cell disease (SCD)?

What additional tests may be useful for a diagnosis of hemolysis in sickle cell disease (SCD)?

When are ABG measurements indicated in patients with sickle cell disease (SCD)?

When should a urinalysis be performed in patients with sickle cell disease (SCD)?

When is a sickling test indicated in patients with sickle cell disease (SCD)?

Is secretory phospholipase A2 a biomarker for acute chest syndrome in patients with sickle cell disease (SCD)?

When is chest radiography indicated in patients with sickle cell disease (SCD)?

What is the role of radiography of the extremities in patients with sickle cell disease (SCD)?

How sensitive is radiography compared to other studies for osteomyelitis in sickle cell disease (SCD)?

What is the role of MR) in the workup of sickle cell disease (SCD)?

What is the sensitivity and specificity of MRI for identifying bone marrow infarction in patients with sickle cell disease (SCD)?

What do abnormal MRI or neuropsychometric (NPM) findings in patients with sickle cell disease (SCD) indicate?

Are MRI and MRA important screening tools for sickle cell disease (SCD) in the prevention of primary stroke?

What is the role of CT scanning in the management of sickle cell disease (SCD)?

What is the role of nuclear medicine scanning in the management of sickle cell disease (SCD)?

What is the role of 99m Tc bone scanning in the management of sickle cell disease (SCD)?

What is the role of indium-111 (111In) WBC scanning in the management of sickle cell disease (SCD)?

How is acute osteomyelitis differentiated from bone infarction in patient with sickle cell disease (SCD)?

What is the role of transcranial Doppler (TCD) ultrasonography in the management of sickle cell disease (SCD)?

What are the indications for transcranial Doppler (TCD) screening in sickle cell disease (SCD) and how often should it be done?

Should transfusions be administered to patients with abnormal transcranial Doppler (TCD) results and sickle cell disease (SCD)?

What is the role of abdominal ultrasonography in the management of sickle cell disease (SCD)?

What is the role of ultrasonography of the kidneys in the management of sickle cell disease (SCD)?

What is the role of ECG in the management of sickle cell disease (SCD)?

Treatment

According to the NIH, how is optimal care for sickle cell disease (SCD) achieved?

What is self-treatment for sickle cell disease (SCD)?

How should patients with sickle cell disease (SCD) crisis be managed during transport by emergency medical services (EMS)?

What should be included in pain management for sickle cell disease (SCD)?

What are the goals of treatment for sickle cell disease (SCD)?

Is there a cure for sickle cell disease (SCD)?

What are the guidelines for the treatment of sickle cell disease (SCD)?

What is the role of L-glutamine oral powder (Endari) in the treatment of sickle cell disease (SCD)?

Which medications are approved by the FDA for the treatment of sickle cell disease (SCD)?

What are the effects of hydroxyurea in patients with sickle cell disease (SCD)?

Is hydroxyurea an effective treatment for adults with sickle cell disease (SCD)?

Is hydroxyurea an effective treatment for children with sickle cell disease (SCD)?

What are the indications for hydroxyurea in patients with sickle cell disease (SCD)?

What monitoring is required for patients with sickle cell disease (SCD) receiving hydroxyurea?

What are the possible adverse effects of hydroxyurea in patients with sickle cell disease (SCD)?

What are the treatment options after failed hydroxyurea therapy for sickle cell disease (SCD)?

When are transfusions indicated in the treatment of sickle cell disease (SCD)?

What are possible transfusion-related complications in sickle cell disease (SCD)?

Which intraoperative and postoperative complications are possible in patients with sickle cell disease (SCD)?

Does raising hemoglobin concentration prior to surgery protect patients with sickle cell disease (SCD) from complications?

Should hemoglobin concentrations be reduced in patients undergoing retinal surgery for sickle cell disease (SCD)?

Which test provides an accurate measure of tissue iron concentration in patients with sickle cell disease (SCD)?

Which agents are used for iron chelation in patients with sickle cell disease (SCD)?

What is the role of erythrocytapheresis in the treatment of sickle cell disease (SCD)?

How is an erythrocytapheresis performed in the treatment of sickle cell disease (SCD)?

What are the advantages of erythrocytapheresis over simple transfusion in the treatment of sickle cell disease (SCD)?

How are ophthalmic manifestations of sickle cell disease managed?

How are vaso-occlusive crises treated in patients with sickle cell disease (SCD)?

Which risk factors are associated with hospital admission for patients with sickle cell disease (SCD) in vaso-occlusive crisis?

Which risk factors are associated with longer length of hospital stay for vaso-occlusive crisis in patients with sickle cell disease (SCD)?

How can the incidence of vaso-occlusive crisis in patients with sickle cell disease (SCD) be reduced?

What are the NICE guidelines for pain control in sickle cell disease (SCD)?

What are the NHLB recommendations for pain management in the treatment of sickle cell disease (SCD)?

How should morphine be administered in patients with sickle cell disease (SCD)?

What are the BCSH guidelines for treatment of acute chest syndrome (ACS) in patients with sickle cell disease (SCD)?

How can acute chest syndrome (ACS) complications in patients with sickle cell disease (SCD) be prevented?

How is acute chest syndrome (ACS) treated in patients with sickle cell disease (SCD)?

Which medications can be administered to treat acute chest syndrome (ACS) in patients with sickle cell disease (SCD)?

Does an elevated level of serum phospholipase A2 predict acute chest syndrome (ACS) in patients with sickle cell disease (SCD)?

How is severe hypoxia treated in patients with acute chest syndrome (ACS) and sickle cell disease (SCD)?

What role do corticosteroids play in nonasthmatic patients with acute chest syndrome (ACS) and sickle cell disease (SCD)?

Which treatment may reduce the recurrence of acute chest syndrome (ACS) in patients with sickle cell disease (SCD)?

How is chronic pain treated in patients with sickle cell disease (SCD)?

What is the role of tricyclic antidepressants in the treatment of chronic pain in patients with sickle cell disease (SCD)?

Is hydroxyurea used to treat chronic pain in patients with sickle cell disease (SCD)?

What are the nonpharmacological approaches to pain management in sickle cell disease (SCD)?

What can family members and patients do to manage recurring pain of sickle cell disease (SCD)?

When is hospitalization indicated for the management of pain in sickle cell disease (SCD)?

Which dosage regimen is used to treat chronic pain in patients with sickle cell disease (SCD)?

Which opioids are used to treat chronic pain in sickle cell disease (SCD) and when is opioid dependence a concern?

How is megaloblastic anemia prevented in patients with sickle cell disease (SCD)?

What is the role of folic acid therapy in the treatment of chronic anemia in sickle cell disease (SCD)?

Does menstruation cause iron deficiency (chronic anemia) in women with sickle cell disease (SCD)?

When is blood transfusion indicated for treatment of chronic anemia in patients with sickle cell disease (SCD)?

How is anemic crisis with splenic sequestration treated in patients with sickle cell disease (SCD)?

When is a transfusion required for the treatment of anemia in patients with sickle cell disease (SCD)?

How is infection-related morbidity and mortality minimized and prevented in sickle cell disease (SCD)?

When is antibiotic therapy indicated in the treatment of sickle cell disease (SCD), and which antibiotics are used?

Which prophylaxis antibiotic is effective in reducing the incidence of infection in patients with sickle cell disease (SCD)?

Are pneumococcal vaccines (PCVs) effective in preventing invasive infection in children with sickle cell disease (SCD)?

What is the role of immunization in preventing infection in children with sickle cell disease (SCD)?

How is acute cholecystitis treated in patients with sickle cell disease (SCD)?

What is the role of cholecystectomy in asymptomatic patients with cholelithiasis (gallstones) and sickle cell disease (SCD)?

What is the initial treatment for priapism in patients with sickle cell disease (SCD)?

When is emergency department (ED) treatment indicated for priapism in patients with sickle cell disease (SCD)?

What are the complications of priapism in patients with sickle cell disease (SCD)?

How is recurrent priapism prevented in patients with sickle cell disease (SCD)?

How are leg ulcers treated in patients with sickle cell disease (SCD)?

What are the AHA/ASA guidelines for prevention of primary stroke in patients with sickle cell disease (SCD)?

What is the standard care for stroke prevention in children with sickle cell disease (SCD)?

How many children with sickle cell disease (SCD) and stroke experience subsequent events if long-term transfusion therapy is not provided?

What is an alternative to simple transfusion in the treatment of sickle cell disease (SCD)?

What are the AHA/ASA treatment guidelines for children with sickle cell disease at high risk for stroke if RBC transfusion is contraindicated?

What is the efficacy of transfusions or hydroxycarbamide for the prevention of stroke in patients with sickle cell disease (SCD)?

What is the significance of pulmonary hypertension in patients with sickle cell disease (SCD), and what factors contribute to pulmonary hypertension?

What is associated with severe reduction in exercise capacity, in addition to pulmonary hypertension, and what is associated with accelerated mortality in sickle cell disease?

How is symptomatic pulmonary hypertension managed in patients with sickle cell disease (SCD)?

How are hip complications of sickle cell disease (SCD) (avascular necrosis) treated?

Which psychiatric disorders can be caused by sickle cell disease (SCD) and how can they be treated?

What is the efficacy of stem cell transplantation (SCT) for the treatment of sickle cell disease (SCD)?

What are the restrictions to stem cell transplantation (SCT) for sickle cell disease (SCD)?

Are there dietary restrictions for patients with sickle cell disease (SCD)?

Are there activity restrictions for patients with sickle cell disease (SCD)?

Which activities should be encouraged in children with sickle cell disease (SCD)?

Are there investigational treatments for sickle cell disease (SCD)?

Which specialist consultations may be required in the treatment of sickle cell disease (SCD)?

Which specialist consultations may be required for complications of sickle cell disease (SCD)?

When is consultation with an ophthalmologist or urologist indicated in the treatment of sickle cell disease (SCD)?

When is consultation with a retina or glaucoma specialist indicated in the treatment of sickle cell disease (SCD)?

How often is follow-up required in the treatment of sickle cell disease (SCD)?

What education should patients receive about long-term management of sickle cell disease (SCD)?

What follow-up care is required for patients with proliferative sickle retinopathy (PSR)?

Should fluid intake and output be monitored in the treatment of sickle cell disease (SCD)?

What are the increased risks for patients with sickle cell disease (SCD) who have undergone a splenectomy?

What is recommended long-term monitoring for infants and children with sickle cell disease (SCD)?

Medications

What drugs are used in treatment of sickle cell disease (SCD)?

Which medications in the drug class Antimetabolites are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Opioid Analgesics are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Nonsteroidal Analgesics are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Tricyclic Antidepressants are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Vitamins are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Nutritionals are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Antibiotics are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Phosphodiesterase Type 5 Inhibitors are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Endothelin Receptor Antagonists are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Iron Chelators are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Antiemetics are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Adrenergic Agonists are used in the treatment of Sickle Cell Anemia?

Which medications in the drug class Vaccines are used in the treatment of Sickle Cell Anemia?