An 8-month-old boy with sickle cell disease is brought to an urgent care center by his parents following several hours of intermittent crying. Yesterday afternoon, the patient had been active and crawling around, but he seemed irritable in the evening and was awake most of the night. Today, his parents noticed swelling of his hands and feet, which appear painful to the touch. The pain seems to be only minimally relieved with over-the-counter acetaminophen and ibuprofen. The boy has been breastfeeding less than usual due to fussiness but has been voiding appropriately. The patient has never been hospitalized. He takes oral penicillin prophylaxis and a vitamin D supplement. Immunizations, including an annual influenza vaccination, are up to date. Temperature is 36.7 C (98 F), blood pressure is 90/60 mm Hg, pulse is 158/min, and respirations are 32/min. Pulse oximetry is 98% on room air. Physical examination shows a fussy infant who can be consoled for short periods of time but appears restless and grimaces occasionally during the examination. There is symmetric swelling of the hands and feet. There are no rashes or skin abrasions. Lungs are clear to auscultation, and there are no retractions or use of accessory muscles. The abdomen is soft, nontender, and nondistended; the spleen is not palpable. The remainder of the examination is normal. The last dose of ibuprofen was given 1 hour ago. Which of the following is the most appropriate next step in management of this patient?

Question

An 8-month-old boy with sickle cell disease is brought to an urgent care center by his parents following several hours of intermittent crying.  Yesterday afternoon, the patient had been active and crawling around, but he seemed irritable in the evening and was awake most of the night.  Today, his parents noticed swelling of his hands and feet, which appear painful to the touch.  The pain seems to be only minimally relieved with over-the-counter acetaminophen and ibuprofen.  The boy has been breastfeeding less than usual due to fussiness but has been voiding appropriately.  The patient has never been hospitalized.  He takes oral penicillin prophylaxis and a vitamin D supplement.  Immunizations, including an annual influenza vaccination, are up to date.  Temperature is 36.7 C (98 F), blood pressure is 90/60 mm Hg, pulse is 158/min, and respirations are 32/min.  Pulse oximetry is 98% on room air.  Physical examination shows a fussy infant who can be consoled for short periods of time but appears restless and grimaces occasionally during the examination.  There is symmetric swelling of the hands and feet.  There are no rashes or skin abrasions.  Lungs are clear to auscultation, and there are no retractions or use of accessory muscles.  The abdomen is soft, nontender, and nondistended; the spleen is not palpable.  The remainder of the examination is normal.  The last dose of ibuprofen was given 1 hour ago.  Which of the following is the most appropriate next step in management of this patient?

Quizplus · Accepted Answer

Explanation: 11ec2b69_c169_5a2d_8f63_97cf6764aade_MD0005_00 This patient with symmetric swelling and pain of the hands and feet in the setting of known sickle cell disease (SCD) has dactylitis (hand-foot syndrome). Dactylitis, resulting from vaso-occlusion of the blood vessels supplying the metacarpals and metatarsals, may be the earliest manifestation of SCD in young children (ages 6 months to 4 years). Similar to other vaso-occlusive episodes, triggers include stress, illness, temperature changes, and dehydration. Low-grade fever may also be present in addition to pain. Initial radiographs reveal only soft-tissue swelling; recurrent episodes can lead to a mottled appearance of the bones. Although most children with SCD are diagnosed by hemoglobin electrophoresis on newborn screening, the presence of dactylitis in a previously healthy child should prompt evaluation for SCD. Treatment of dactylitis involves pain control, hydration, and application of heat. Oral nonsteroidal anti-inflammatory drugs (NSAIDs) and oral opioids are typically initiated for mild to moderate pain. Because this patient still appears uncomfortable after treatment with NSAIDs, a short-acting oral opioid (eg, oxycodone) should be given. If the pain fails to improve, intravenous opioids are indicated. (Choice A) Cold compresses are not recommended as they can trigger local intravascular sickling and worsen vaso-occlusive pain episodes. In contrast, heat packs help by promoting vasodilation. (Choices B and E) Oral codeine and tramadol are contraindicated in children age <12 because ultra-rapid metabolism of codeine and tramadol causes an unpredictably high concentration of the active drug in the body, which can result in respiratory depression and death. (Choice C) Hydroxyurea reduces the frequency of vaso-occlusive episodes in patients with SCD but may take several months to take effect. It is not used in the treatment of an acute vaso-occlusive episode. Educational objective: Dactylitis presents in young children with sickle cell disease as symmetric swelling and pain of the hands and feet. Treatment includes pain control (eg, nonsteroidal anti-inflammatory drugs, oral opioids), hydration, and application of heat. References: Sickle cell disease. (http://www.ncbi.nlm.nih.gov/pubmed/20301551) Current management of sickle cell anemia. (http://www.ncbi.nlm.nih.gov/pubmed/23709685)

An 8-Month-Old Boy with Sickle Cell Disease Is Brought to an Urgent