eMedicine Specialties > Emergency Medicine > Hematology & Oncology

Anemia, Sickle Cell: Follow-up

Author: Ali Taher, MD, Professor of Medicine, Division of Hematology and Oncology, Assistant to the Chair-Undergraduate Program, Department of Internal Medicine, American University of Beirut Medical Center
Coauthor(s): Adlette Inati Khoriaty, MD, Head, Division of Pediatric Hematology-Oncology, Medical Director, Children's Center for Cancer and Blood Diseases, Rafik Hariri University Hospital; Research Associate, Balamand University; Head of Post Bone Marrow Transplant Clinic and Consultant Hematologist, Chronic Care Center, Lebanon; Ziad N Kazzi, MD, Assistant Professor, Department of Emergency Medicine, Emory University; Medical Toxicologist, Georgia Poison Center
Contributor Information and Disclosures

Updated: Nov 16, 2009

Follow-up

Further Inpatient Care

  • Indications for hospital admission for patients with sickle cell disease are as follows:
    • Pulmonary, neurological, or infectious crisis
    • Vaso-occlusive pain that does not resolve after 4-6 hours and 2 doses of narcotics in the ED
    • Inability to maintain adequate hydration if discharged home
    • Uncertain diagnosis

Further Outpatient Care

  • If improvement is shown after 6 hours in the ED, patient may be discharged home with strict instructions to ingest large amounts of fluids and to return if pain recurs, temperature increases, or new symptoms develop.
  • Arrange follow-up in a hematology clinic so that appropriate counseling can be given and new drugs, such as hydroxyurea, can be tried. Such drugs are believed to decrease the frequency of sickling crisis by increasing the percentage of fetal hemoglobin (HbF) in blood.

Inpatient & Outpatient Medications

  • Folic acid should be prescribed to those who are not already taking it.
  • Discharge the patient on oral analgesics for a week. Up to 2 doses of narcotics can be administered in the ED over a period of 4-6 hours.
  • Oral drugs for mild pain include acetaminophen, ibuprofen, aspirin, and codeine. If pain is moderate, oxycodone or methadone can be administered.
  • Administer parenteral drugs for severe pain. Morphine is the drug of choice, but meperidine with promethazine can be used.
  • Antibiotics are indicated when an infection is suspected, when body temperature is higher than 38 degrees Celsius, or when a patient has localized bone tenderness. Fever in children is strongly suggestive of infection. It has been found that signs of infection are more accurate in children than in adults.
  • Common infections include pneumonia, bronchitis, cholecystitis, pyelonephritis, cystitis, osteomyelitis, meningitis, and sepsis. Recommended parenteral antibiotics include cephalosporins (eg, ceftriaxone, cefuroxime) and macrolides for acute chest syndrome.
  • If the patient is discharged home, oral antibiotics (eg, amoxicillin-clavulanic acid, clarithromycin, cefixime) are useful in selected cases. If the patient has localized bone tenderness, seek antibiotic coverage for S typhimurium and S aureus.

Transfer

  • Transfer is only applicable if exchange transfusion or ICU is not available.

Deterrence/Prevention

  • Measures to prevent sickle cell crisis include the following: 
    • Adherence to an immunization against pneumococcus, H influenzae, meningococcus, hepatitis B, influenza, and Salmonella typhi (in endemic areas) as well as routine vaccines.
    • Avoid temperature extremes, high altitudes, and cold weather
    • Enforce oral fluids
    • Foot care and protective shoes
    • Periodic health care visits and surveillance workups
    • Educate parents and elderly patients about symptoms of life-threatening complications.
    • Biannual medical visit for those older than 30 years

Complications

  • Pulmonary hypertension, attributed to hemolysis and hypoxia and seen in adult patients, heralds a poor prognosis and is refractory to hydroxyurea therapy. Cor pulmonale may ensue, and the management is that of patients with right-sided heart failure and chronic obstructive pulmonary disease (COPD).
  • Cholelithiasis may occur because gallstones form as a result of chronic hemolytic anemia. Ultrasonography is diagnostic. If symptomatic, cholecystectomy is indicated.
  • Ophthalmologic complications include retinopathy, which can be proliferative and nonproliferative, as well as retinal infarcts and retinal detachment. Findings on ophthalmoscopic examination include corkscrew vessels in the conjunctiva and salmon patches on the retina.
  • Transfusion-related complications include alloimmunization, exposure to pathogens, and iron overload. Therapy of iron overload is becoming easier with the new oral chelators.
  • Leg ulcers may result from venous stasis and chronic hypoxia and may become infected. Management is the same as with other stasis ulcers.
  • Avascular osteonecrosis may result from chronic hypoxia in weight-bearing joints, commonly the femoral head. Joint replacement is often necessary.
  • Psychological problems
    • Patients may experience depression, anxiety, and chronic pain behavior.
    • Counseling is crucial. Ensure an appropriate physician-patient relationship.
    • Anxiolytics and amitriptyline may be used.

Prognosis

  • Most patients with sickle cell anemia experience bacterial infections, painful crisis, and fatigue secondary to chronic anemia.
  • Half of patients with sickle cell anemia die when younger than 20 years. Most do not survive to the age of 50 years.

Patient Education

  • Teach patients to seek medical care in certain situations, including the following:
    • Persistent fever (>38.3°C)
    • Chest pain, shortness of breath, nausea, and vomiting
    • Abdominal pain with nausea and vomiting
    • Persistent headache not experienced previously
  • Patients should avoid the following:
    • Alcohol
    • Nonprescribed prescription drugs
    • Cigarettes, marijuana, and cocaine
    • Seeking help in multiple institutions
  • For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education articles Anemia and Sickle Cell Crisis.

Miscellaneous

Medicolegal Pitfalls

  • Carriers may show evidence of hemoglobinopathy under severe stress and hypoxic states.
  • Carriers may have leg ulcers, splenic infarcts, and hyphema.
  • Sickle beta-thalassemia and sickle cell disease   
    • Patients with either of these disorders can have splenomegaly and delayed autoinfarction of the spleen.
    • Patients with sickle beta-thalassemia tend to have more vaso-occlusive symptoms than do patients with sickle cell anemia. 
    • Patients with sickle beta-thalassemia tend to have more proliferative retinopathy, renal disease, acute chest syndrome, and avascular osteonecrosis than do patients with sickle cell disease.

Special Concerns

  • Sickle cell disease and pregnancy
    • Patients who are pregnant and have sickle cell disease are at increased risk for crisis, toxemia, pyelonephritis, thrombophlebitis, and spontaneous abortion compared to the general population.
    • Prophylactic transfusion with special concern for folic acid replacement has been shown to decrease the incidence of vaso-occlusive crisis during pregnancy.
    • In the past, pregnancy was strongly discouraged, and tubal ligation often was performed.
 


More on Anemia, Sickle Cell

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Follow-up: Anemia, Sickle Cell
Multimedia: Anemia, Sickle Cell
References
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Further Reading

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Keywords

sickle cell disease, sickle cell anemia, sickle cell treatment, sickle cell causes, sickle cell symptoms, blood disorder, crescent cell anemia, sickle cell, hemoglobin S, vasoocclusive crisis, avascular necrosis, isosthenuria, acute chest syndrome

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Contributor Information and Disclosures

Author

Ali Taher, MD, Professor of Medicine, Division of Hematology and Oncology, Assistant to the Chair-Undergraduate Program, Department of Internal Medicine, American University of Beirut Medical Center
Disclosure: Nothing to disclose.

Coauthor(s)

Adlette Inati Khoriaty, MD, Head, Division of Pediatric Hematology-Oncology, Medical Director, Children's Center for Cancer and Blood Diseases, Rafik Hariri University Hospital; Research Associate, Balamand University; Head of Post Bone Marrow Transplant Clinic and Consultant Hematologist, Chronic Care Center, Lebanon
Adlette Inati Khoriaty, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Hematology, European Hematology Association, and International Society of Hematology
Disclosure: Nothing to disclose.

Ziad N Kazzi, MD, Assistant Professor, Department of Emergency Medicine, Emory University; Medical Toxicologist, Georgia Poison Center
Ziad N Kazzi, MD is a member of the following medical societies: American Academy of Clinical Toxicology, American Academy of Emergency Medicine, American College of Emergency Physicians, and American College of Medical Toxicology
Disclosure: Nothing to disclose.

Medical Editor

Roy Alson, MD, PhD, FACEP, FAAEM, Associate Professor, Department of Emergency Medicine, Wake Forest University School of Medicine; Medical Director, Forsyth County EMS; Deputy Medical Advisor, North Carolina Office of EMS; Associate Medical Director, North Carolina Baptist AirCare
Roy Alson, MD, PhD, FACEP, FAAEM is a member of the following medical societies: American College of Emergency Physicians, American Medical Association, National Association of EMS Physicians, North Carolina Medical Society, Society for Academic Emergency Medicine, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Jeffrey L Arnold, MD, FACEP, Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center
Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Physicians
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Jonathan Adler, MD, Attending Physician, Department of Emergency Medicine, Massachusetts General Hospital; Division of Emergency Medicine, Harvard Medical School
Jonathan Adler, MD is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

 
 
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