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Sickle Cell Disease: Adult Care and Crisis Management

Pain crises, organ complications and modern disease-modifying therapy

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Sickle Cell Disease: Adult Care and Crisis Management?

Sickle cell disease is an autosomal recessive disorder with mutated beta-globin chains forming insoluble hemoglobin S polymers under deoxygenated conditions.

Sickled erythrocytes cause microvascular occlusion, hemolysis, endothelial dysfunction and chronic inflammation.

Genotypes include homozygous HbSS, compound heterozygous HbS-beta-thalassemia and HbSC, with variable severity.

Adults face cumulative organ damage to spleen, kidneys, lungs, brain, eyes and bones.

Diagnosis uses hemoglobin electrophoresis and high-performance liquid chromatography; newborn screening allows early intervention.

Symptoms

Acute vaso-occlusive pain crises affecting bones, chest, abdomen and joints often triggered by cold, dehydration, infection or hypoxia.
Acute chest syndrome presents with chest pain, fever, cough and hypoxia and is a leading cause of mortality.
Splenic sequestration with rapid spleen enlargement and circulatory collapse, more common in children but can recur.
Stroke from large vessel and small vessel disease, silent infarctions and progressive cognitive impairment.
Chronic complications include avascular necrosis, leg ulcers, retinopathy, priapism, pulmonary hypertension, kidney disease and gallstones.

Risk Factors

African, Mediterranean, Middle Eastern, Indian and Caribbean ancestry.
Genotype HbSS and HbS-beta-zero have the most severe phenotype.
Triggers for crises include cold exposure, dehydration, infection, stress, high altitude and pregnancy.
Coexisting conditions like alpha-thalassemia or hemoglobin F levels modify severity.
Limited access to comprehensive care, transcranial Doppler screening and disease-modifying therapy worsens outcomes.

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Severe pain unresponsive to home analgesia or persisting more than several hours requires emergency evaluation.
  • Fever above 38.5 degrees Celsius is a medical emergency due to risk of overwhelming infection from functional asplenia.
  • Chest pain, dyspnea, cough or low oxygen saturation may indicate acute chest syndrome.
  • Sudden weakness, slurred speech, vision changes or severe headache warrant urgent stroke workup.
  • Persistent priapism more than 4 hours, sudden vision loss, severe abdominal pain or rapid spleen enlargement need emergency care.

Treatment Methods

01
Acute pain crisis is managed with intravenous opioids, hydration, oxygen, incentive spirometry and treatment of triggers.
02
Acute chest syndrome requires antibiotics, oxygen, bronchodilators and exchange transfusion when severe.
03
Hydroxyurea increases fetal hemoglobin and reduces crises, acute chest syndrome and mortality.
04
Voxelotor improves anemia, crizanlizumab reduces crises through P-selectin inhibition and L-glutamine reduces oxidative stress.
05
Curative therapies include matched sibling allogeneic transplant, haploidentical and unrelated donor transplant and gene therapy (lovotibeglogene, exagamglogene); comprehensive care provides vaccinations, transcranial Doppler screening, retinal exams, kidney monitoring and reproductive counseling.

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.