Clinical Presentation and Diagnosis of Sequestration Crisis in SCD General

• Acute exacerbation of anemia due to sequestration of large blood volume by the spleen

• More common in patients with functioning spleens

• Onset often associated with viral or bacterial infections

• Recurrence is common and can be fatal Symptoms

• Sudden onset of fatigue, dyspnea, and distended abdomen

• Patients may present with vomiting and abdominal pain Signs

• Rapid decrease in Hgb and Hct with elevated reticulocyte count

• Splenomegaly

• May exhibit hypotension and shock Evaluation

• Spleen size changes

• Oxygen saturations

• CBC with reticulocyte count

Clinical Presentation and Diagnosis of Vasoocclusive Crisis in SCD General

• Most often involves the bones, liver, spleen, brain, lungs, and penis

• Precipitating factors include: infection, extreme weather conditions, dehydration, and stresses

• Recurrent acute crises result in bone, joint, and organ damage and chronic pain Symptoms

• Patients may complain of deep throbbing pain, local tenderness Signs

• Erythema and swelling of painful area

• Dactylitis in young infants

• Leukocytosis Laboratory tests

• CBC with reticulocyte

• Urinalysis

• Abdominal studies (if symptoms exist)

• Cultures (blood and urine)

• Liver function tests and bilirubin

Severe pain should be treated with an opioid such as morphine, hydromorphone, methadone or fentanyl. Moderate pain can be effectively treated in most cases with a weak opioid such as codeine or hydrocodone, usually in combination with acetaminophen. Meperidine should be avoided because of its relatively short analgesic effect and its toxic metabolite, normeperidine. Normeperidine may accumulate with repeated dosing and can lead to CNS side effects including seizures.

IV opioids are recommended for use in treatment of severe pain because of their rapid onset of action and ease in titration. Intramuscular injection should be avoided. Analgesia should be individualized and titrated to effect, either by scheduled doses or continuous infusion. The use of continuous infusion will avoid the fluctuations in blood levels between doses that is seen with bolus dosing. As needed dosing of analgesia is only appropriate for breakthrough pain or uncontrolled pain. Patient-controlled analgesia (PCA) is commonly used and allows the patient to have control over his or her analgesic breakthrough dosing. As the pain crisis resolves, the pain medications can be tape8ed. Physical therapy and relaxation therapy can be helpful adjuvants to analgesia.4 -48

Tolerance to opioids is seen when patients have had continuous long-term use of the medications and can be managed during acute crises by using a different potent opioid or using a larger dose of the same medication. Adverse effects associated with the use of opioids include respiratory depression, itching, nausea and vomiting, constipation, and drowsiness. Patients on continuous infusions of opioids should be on continuous pulse oximeter to assess oxygen saturations. Monitor the patient for oxygen saturations less than 92%. Oxygen should be administered as needed to keep the saturations above 92%. Itching can be managed with an antihistamine such as diphen-hydramine. Nausea and vomiting can be treated and managed with the administration of antiemetics such as promethazine or the 5HT3 antagonists, but the use of promethazine is contraindicated in children younger than 2 years of age. Assess stool frequency in all patients on a continuous opioid, and start stool softeners or laxatives as needed. Excessive sedation is difficult to control and the concurrent use of an opioid with diphenhydramine or other sedative medications can exacerbate the drowsiness, leading to hypoxemia. A continuous very low dose of naloxone, an opioid antagonist, has been used in some cases where the adverse effects such as itching are unbear-able.49

Table 68-4 Management of Acute Pain of SCD

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