eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Sickle Cell Anemia: Treatment & Medication

Author: Ariel Distenfeld, MD, Clinical Professor, Department of Medicine, New York University School of Medicine
Coauthor(s): Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Contributor Information and Disclosures

Updated: Aug 26, 2009

Treatment

Medical Care

Probably no other disease in existence has as much scientific information and knowledge available as SCD. The genetics, pathophysiology, and molecular biology of SCD are well established. Even so, no safe, effective, and curative therapy is available. The goals of treatment strategies are symptom control and management of disease complications. Treatment strategies include the following 7 goals: (1) management of vasoocclusive crisis, (2) management of chronic pain syndromes, (3) management of the chronic hemolytic anemia, (4) prevention and treatment of infections, (5) management of the complications and the various organ damage syndromes associated with the disease, (6) prevention of stroke, and (7) detection and treatment of pulmonary hypertension.

  • Vasoocclusive crisis is treated with vigorous hydration and analgesics.
    • Intravenous fluids should be of sufficient quantity to correct dehydration and to replace continuing loss, both insensible and due to fever.
    • Normal saline and 5% dextrose in saline may be used.
    • These fluids should be given intravenously, and treatment must be in an inpatient setting.
  • Pain control is best achieved by the administration of opioids.
    • Morphine is the drug of choice. Morphine dosing has to be individualized (vide infra).
      • It should be given intravenously, hourly at first. Once the effective dose is established, it should be administered every 3 hours via the intravenous regimen.
      • After 24-48 hours, as pain is controlled, equivalent doses of sustained-release oral morphine should be given.
      • When marked improvement occurs, the patient may be discharged home on sustained-release oral morphine, and the dose is reduced gradually over several days.
      • Morphine elixir can be used to control breakthrough pain.
    • Chronic pain is managed with long-acting oral morphine preparations and acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs).
      • NSAIDs are particularly effective in reducing deep bone pain. Many patients may require breakthrough oral opiates as well. The weak agents, codeine and hydrocodone, are used first. Sustained-release long-acting oral morphine is reserved for more severe cases.
      • The addition of tricyclic antidepressants may reduce the dose and need for opiates by interfering with pain perception. Many patients with chronic pain are depressed, and lifting the depression has a salutary effect on the pain.
    • Hydromorphone may be used but is considerably more expensive.
    • Meperidine should be avoided.
    • Nonpharmacological approaches to pain management are very important.
      • These include physical therapy, heat and cold application, acupuncture and acupressure, hypnosis, and transcutaneous electric nerve stimulation (TENS).
      • Support groups are also useful.
      • All of these modalities may have a substantial impact on pain reduction.
  • Prevention of infection improves chances of survival in SCD.
    • Penicillin prophylaxis, commencing in infancy and continued until age 5 years or early teens, and the use of a pneumococcal vaccine at age 2 years with a booster dose at age 5 years greatly reduces the frequency of infections with S pneumoniae.
    • In the adult patient, all infections must be treated promptly with broad-spectrum antibiotics until a causative organism is identified and therapy is tailored according to its antibiotic sensitivity.
  • Allogeneic bone marrow transplantation (BMT) can cure the disease, but it is difficult to decide which patients should be offered BMT.
    • Many risks are associated with BMT, and the risk-to-benefit ratio must be assessed carefully.
    • With the advent of cord blood stem cell transplantation and with the development of less immunoablative conditioning regimens, perhaps BMT will gain wider acceptance and use.
    • The lack of availability of a matched donor may limit the utility of BMT.
  • Although several attempts have been made to treat SCD with pharmacological agents over the years, the only drug currently approved by the US Food and Drug Administration (FDA) for the treatment of SCD is hydroxyurea.
    Effects of therapy with hydroxyurea.

    Effects of therapy with hydroxyurea.

    [ CLOSE WINDOW ]
    Effects of therapy with hydroxyurea.

    Effects of therapy with hydroxyurea.

    • Hydroxyurea increases the production of Hb F, which retards gelation and sickling. The Hb level rises, and the frequency and severity of vasoocclusive crisis declines, sometimes being eliminated altogether. The reduction in the circulating leukocytes decreases the adherence of neutrophils to the vascular endothelium.
    • A decrease in chronic pain and the reticulocyte count occur.
    • Hydroxyurea is a potentially leukemogenic and carcinogenic agent.
      • Potential complications referable to long-term use are not yet known.
      • Its use also requires frequent blood testing and monitoring, with special attention to development of leukopenia and/or thrombocytopenia.
      • Children studied by a cooperative group remained on hydroxyurea for more than a year with only minor adverse effects.
    • A rigorous selection of patients for hydroxyurea treatment is required.
      • Candidates for this treatment have frequent painful crises (6 or more per year), severe unremitting chronic pain that cannot be controlled by conservative measures, acute chest syndrome, and a history of stroke or a high risk for stroke.
      • A good continuous doctor-patient relationship and rapport must exist to ensure that potential toxicity is identified at its onset.
  • Investigational treatments
    • Oral glutamine has been used to decrease resting energy expenditure in children.
    • Nitric oxide inhalation has been investigated in the treatment of pulmonary hypertension.
    • Topical GM-CSF has been reported to hasten the healing of leg ulcers.
    • Butyrate was studied to decrease vasoocclusive crisis.
    • Arginine has been proposed to use as a precursor of nitric oxide production.
  • Anemia is usually well tolerated.
    • Because of the high turnover, folate stores are often depleted. Folic acid supplementation may raise the Hb level and support a healthy reticulocyte response.
    • Women who are menstruating should be checked for coexisting iron deficiency and, if found, given iron supplements.
    • An adequate overall diet is essential.
    • Blood transfusion is indicated only in specific situations. These include acute chest syndrome, stroke, abnormal findings on transcranial Doppler in children (for stroke prevention), pregnancy, and general anesthesia.
      • The aim is to decrease the concentration of Hb S to 30% or less.
      • Transfusion may also be required during aplastic crisis.
      • Chronic and repeated transfusion leads to alloimmunization and iron overload and is associated with the risk of transfusion-transmitted infectious agents.

Surgical Care

  • Surgical care is limited to treating disease complications.
    • Skin grafts can help heal chronic leg ulcers.
    • Hip replacement or other orthopedic procedures can be used to treat avascular necrosis.
    • Resistant priapism may require surgical draining of the penile corpora. If impotence occurs, insertion of a penile prosthesis may be considered.
    • Cholecystectomy may be needed for gallstones, whether acute cholecystitis is present or not. Use either the classic abdominal incision or laparoscopy.

Consultations

  • Consultation with infectious disease specialists is recommended during febrile illness.
  • Consultations with pain management experts, social workers, psychiatrists and physical therapists, substance abuse counselors, vocational rehabilitation workers, and orthopedists may be required.
  • Each protean manifestation of SCD requires assistance from an expert in the involved area.

Diet

  • A general well-balanced diet is required. No restrictions are necessary.

Activity

  • Although activity is unrestricted, patients may not be able to tolerate vigorous exercise or exertion.
  • Patients with avascular necrosis of the femur may not be able to tolerate weightbearing and may be restricted to bed rest.
  • Patients with chronic leg ulcers may need to restrict activity that involves raising the legs.

Medication

The drugs used in treatment of SCD include antimetabolites, analgesics, antibiotics, and vaccines.

Antimetabolites

Hydroxyurea affects DNA. Administration results in increased production of Hb F, which inhibits sickling.


Hydroxyurea (Hydrea)

Inhibitor of deoxynucleotide synthesis. Myelosuppressive effects last a few days to a week and are easier to control than alkylating agents.

Adult

Initial: 10 mg/kg
After 6 weeks: 15 mg/kg
Goal: Maximally tolerated dose up to 35 mg/kg without signs of toxicity
Patient usually should start on 500 mg (1 tab/d PO) then increase to 1000 mg after 6-8 wk (2 tab/d PO); dose may be increased by 500 mg q6-8wk to a total dose of 35 mg/kg, ie, 4 tab/d (2000 mg)
Adjust dose and monitor q2wk in beginning, following blood counts; monitoring eventually is monthly; count pills at each visit to monitor compliance

Pediatric

Administer as in adults

Coadministration with fluorouracil can increase neurotoxicity

Documented hypersensitivity; severe anemia or bone marrow suppression

Pregnancy

D - Unsafe in pregnancy

Precautions

Careful frequent monitoring of blood counts is necessary, adjust dose accordingly; occasionally, patients may develop leg ulcers that improve only upon discontinuation of drug

Opioid analgesics

Used to control acute crisis and chronic pain.


Morphine sulfate (Duramorph, Astramorph, MS Contin)

An opioid analgesic and interacts with endorphin receptors in the CNS.

Adult

Initial: 0.05-0.08 mg/kg IV q15min until pain is controlled or oversedation occurs
Pain-controlling dose: Converted to IV q3h, adjust to pain level and sedation; ketorolac may be added (vide infra)
After 24 h: Add 30 mg PO SR q12h; reduce IV dose by 2 mg q3h
After 48 h: Change IV dose to prn for breakthrough pain, adjust dose to achieve pain control for 3h; if IV prn doses are required, increase PO SR to 30 mg q24h
For resolving pain: Reduce prn dose by 2-3 mg/dose q24h; discharge on PO SR at dose that kept patient pain-free without prn IV doses; reduce oral morphine by 30 mg q2-3d until no longer needed; morphine elixir can be used for breakthrough pain

Pediatric

0.05-0.08 IV mg/kg

Phenothiazines may antagonize analgesic effects of opiate agonists; tricyclic antidepressants, MAOIs, and other CNS depressants may potentiate adverse effects of morphine with coadministration; CNS depressants (eg, phenothiazines, benzodiazepines, barbiturates, alcohol) may increase effects

Documented hypersensitivity, respiratory depression, and intestinal ileus

Pregnancy

B - Usually safe but benefits must outweigh the risks.

Precautions

Hypersedation, respiratory depression, and constipation should be monitored and corrected; special care must be exercised in treating tolerant or addicted patients

Nonsteroidal analgesics

Add to effects of opioids during painful crisis and allow use of lower doses of narcotics.


Ketorolac (Toradol)

IV NSAID and very powerful analgesic. Inhibits prostaglandin synthesis by decreasing activity of the enzyme, cyclooxygenase, which results in decreased formation of prostaglandin precursors, which, in turn, results in reduced inflammation.

Adult

30 mg IV q6h for a maximum of 5 d

Pediatric

Not established
<50 kg: Reduce dose
Maximum: 30 mg/dose

Coadministration with aspirin increases risk of inducing serious adverse NSAID-related effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of beta-blockers, hydralazine, and captopril; may decrease diuretic effects of furosemide and thiazides; may prolong PT when administered concurrently with anticoagulants; monitor PT closely and instruct patients to watch for signs and symptoms of bleeding; may increase risk of methotrexate toxicity such as stomatitis, bone marrow suppression, and nephrotoxicity; phenytoin levels may be increased when administered concurrently

Documented hypersensitivity, active peptic acid ulcer disease, renal impairment, and suspected cerebral hemorrhage

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; low WBC counts occur rarely and usually return to normal in ongoing therapy; discontinuation of therapy may be necessary if persistent leukopenia, granulocytopenia, or thrombocytopenia occur

Vitamins

Folic acid replenishes depleted folate stores secondary to hemolysis and is necessary for erythropoiesis.


Folic acid

Necessary for proper nucleotide metabolism. Important cofactor for enzymes used in production of RBCs.

Adult

1 mg/d PO

Pediatric

Administer as in adults

Increase in seizure frequency and a decrease in subtherapeutic levels of phenytoin reported when used concurrently

Documented hypersensitivity; undiagnosed pernicious anemia

Pregnancy

A - Safe in pregnancy

Precautions

Benzyl alcohol present as a preservative in some products and has been associated with fatal gasping syndrome in premature infants; resistance to treatment may occur in patients with alcoholism and deficiencies of other vitamins

More on Sickle Cell Anemia

Overview: Sickle Cell Anemia
Differential Diagnoses & Workup: Sickle Cell Anemia
Treatment & Medication: Sickle Cell Anemia
Follow-up: Sickle Cell Anemia
Multimedia: Sickle Cell Anemia
References
Further Reading
[ CLOSE WINDOW ]

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  85. Al-Subaie AM, Mahdi N, Al-Absi IK, et al. Human platelet alloantigens (HPA) 1, HPA2, HPA3, HPA4, and HPA5 polymorphisms in sickle cell anemia patients with vaso-occlusive crisis. Eur J Haematol. Aug 21 2009;[Medline].

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Further Reading

Related eMedicine Topics

Clinical Trials
National Guideline Clearinghouse

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Keywords

sickle cell disease, hemoglobin SS disease, sickle cell trait, homozygous hemoglobin S disease, SCD, mutant hemoglobins, Hb S, Hb SS, Hb A, Hb SA, anemia, red blood cells, RBC, sickle shaped, vasoocclusive crisis, sickle cell crisis, hemoglobin sc disease, hemoglobin c disease, thalassemia, congenital hemolytic anemia, hemoglobinopathies

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Contributor Information and Disclosures

Author

Ariel Distenfeld, MD, Clinical Professor, Department of Medicine, New York University School of Medicine
Ariel Distenfeld, MD is a member of the following medical societies: American Academy of Hospice and Palliative Medicine, American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society of Clinical Oncology, American Society of Hematology, International Society of Blood Transfusion, International Society of Hematology, Medical Society of the State of New York, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Coauthor(s)

Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Disclosure: Nothing to disclose.

Medical Editor

Wadie F Bahou, MD, Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook
Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Nothing to disclose.

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

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
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