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<< Sickle Cell Disease And Other Hemoglobinopathies: Approaches To Emergency Diagnosis And Treatment

Risk Management

 1. "He just had a little cough, so I didn't think a chest film was warranted."

Acute chest syndrome is one of the most feared complications of SCD. Patients with pulmonary signs or
symptoms, and those with fever, deserve a chest film.

2.  "I know he had a fever of 103°F—but his mom told me that little Johnny was sneezing the day before, so I didn't think bloodwork or antibiotics were indicated."

Infection is one of the major causes of death in children with SCD. Always assume a bacterial infection (or ACS) and evaluate with chest film and bloodwork. Well-appearing children with reassuring diagnostic studies can be discharged after receiving IM or IV ceftriaxone if next-day follow-up is arranged.

3.  " I always use IM meperidine for people with SCD."

Hopefully not in those who are on dialysis! Meperidine has many shortcomings as an analgesic, and it may cause seizures in those with renal failure or insufficiency.

4.  "He wasn't using his arm normally, but I thought it might be a peripheral nerve palsy."

Neurologic deficits in a patient with SCD should be assumed to be secondary to stroke unless the neurologic exam proves otherwise. Have a low threshold for CT scanning of the head.

5. "He had a painful knee! Lots of people with painful crisis have knee pain."

True. But they don't have fever, excruciating pain with range of motion, and an effusion. Ask patients if the pain they have today is the same as their usual pain. Be suspicious of pain isolated to one joint, especially if it's accompanied by abnormal physical findings.

6.   "Who ever feels for a child's spleen?"

We all should. ASS, while rare, is a serious condition found in young children with SCD. The hallmarks
include splenomegaly, anemia, and, often, unstable vital signs.

7.  "I gave him antibiotics for his pneumonia and told him to come back if he had trouble."

Maybe that infiltrate was pneumonia, but maybe not. Patients who may have ACS should be admitted and given antibiotics, incentive spirometry, and perhaps steroids and nebulized beta-agonists.

8.  "But I gave the child Rocephin before I sent him home."

Not every child with fever and SCD can safely go home. There are high-risk features of both the clinical exam and diagnostic studies that mandate admission. (See "Clinical Pathway: Management Of The Febrile Child With Sickle Cell Disease")
9. "SCA patients usually exaggerate their pain in order to get large doses of narcotics."

Patients with SCA have real pain. Similar to childbirth and renal colic, pain from this condition cannot be adequately understood by someone who has not experienced it. Do not let personal suspicion of drugseeking behavior in a patient prevent giving pain relief to someone who is truly suffering.

10. "He just had some abdominal pain. Abdominal pain can be part of a painful crisis."

Yes, it can. But splenic abscess, pancreatitis, cholecystitis, appendicitis, and perhaps a hundred other diseases can cause abdominal pain in the patient with SCD. High-risk features of abdominal pain include localized tenderness, fever, peritoneal signs, and persistent pain despite hydration and opioids. Laboratory parameters, including a leukocyte count, may not distinguish a painful vasoocclusive crisis from a surgical condition. In these cases, consider surgical consult or diagnostic imaging such as CT scan or abdominal ultrasound, depending on the location of the pain.

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Last Modified: 07/23/2017
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