scs4

Hydroxyurea Protocol

Common Treatment Methods

                  Transfusion
Patients with sickle cell disease may also benefit from a trial of regular transfusion for a period of 6 to 12 months to reduce the frequency of events. The goals of transfusion would be to keep the hemoglobin S percent at < 30%, the hemoglobin level at 10 to 11 gm/dl, and the reticulocyte at < 4%. Transfusion therapy must be discussed with the patient including risk of iron overload, alloimmunization (lack of an immune response), and blood-borne infections. Tranfusion for longer than a year must be based on clinical response and a discussion of the need to treat the patient for iron overload if transfusions are continued.
                    Hydroxyurea
Hydroxyurea is also very useful in reducing the frequency of pain episodes as was shown in a carefully controlled clinical trial and confirmed by extensive clinical experience. Patients with Hb SS and Hb SB thalassemia with more that three pain episodes a year should be offered hydroxyurea therapy. Informed consent must be obtained explaining the need for close monitoring of blood counts, the need to avoid pregnance, and all of the potential short and long-term side effects. Response is monitored by change in frequency of pain episodes or increase in total and fetal hemoglobin.
     Chronic pain in a patient with sickle cell disease is frequent and difficult to manage. The patient's past experience with treatment of acute pain episodes may set unobtainable expectations for management of chronic pain. The pain may not resolve or even be completely controlled without significant disruption of normal functioning. Complications like avascular necrosis may require changes in occupation and daily activities that have severe negative impact on self esteem, social status, and economis stability. Patients, significant others, and health care providers need to develop realistic plans for pain management that minimizes discomfort while maximizing physical, psychological, and social functioning.

Eligibility - Any patient who has:

1) more than 3 crisis in one year
2) chronic leg ulcers that fail conventional therapy
3) low creatinine (source of energy for muscle contraction)
4) symptomatic anemia with alloimmunization
5) persistent occurrences of priapism despite standard therapy
6) retic count of > 150,000 average value

Exclusion Criteria

1) failure to sign informed consent
2) active liver disease
3) patients (female) uwilling to use contraception
4) history of non-compliance

Patients should be warned of routine side effects of hydroxurea prior to starting the medication, those are:

1) weight gain
2) bone marrow suppression
3) hair loss, skin pigment changes
4) potential for birth derects
5) potential for leukemia or cancer is a concern

Pharmacologic Interventions

Pharmacologic pain control begins with non-narcotic analgesics. Non-steroidal anti-inflammatory drugs (NSAIDs) are very useful as initial therapy because of their effectiveness in bone pain and their antiinflammatory activities. Pain control with NSAIDs exhibits ceiling effects and higher doses are associated with toxicity without additional analgesia. Longer acting medications may be more helpful and antiiflammatory doses may be appropriate in patients with avascular necrosis. The patient must understand that these meds may not remove the pain as effectively as narcotics, but NSAIDs are still to be taken regularly because of their effects ands synergism with narcotics. Acetaminophen or aspirin should not be given in fixed dosage combinations with narcotics because of the tendencey of patients to take toxic levels to obtain adequate pain relief.

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