Pediatric Pearls – Sickle Cell in Kids – An Update

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Nurses Edition Commentary

Lisa Chavez, RN and Kathy Garvin, RN

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Martin K., M.D. -

Can Dr. Sadowitz comment on the how the management of asthma in children with sickle cell disease differs from those asthmatic children who do not have sickle cell disease. Specifically:
1. There seems to be some concern about the use of systemic steroids triggering a vaso-occlusive crisis in a child with sickle cell anemia. Are there any differences in the way one should either initiate or discontinue a a short course of steroids in a sickle cell patient with an asthma exacerbation?
2. If a patient with sickle cell disease and asthma, presents with an asthma exacerbation with fever, wheezing, and a pulmonary infiltrate, and improves with ED treatment with nebs and steroids, would he have to be admitted because he meets criteria for an acute chest syndrome? If the same patient presents with the same symptoms but no fever, and he improves with nebs/steroids, would he need to be admitted?

Peter S., MD -

I would use steroids in the child with sickle cell disease and asthma in the same fashion as a child with asthma alone.

If the child with sickle cell has fever and infiltrates on chest x-Ray along with an asthma excerbatuon I would admit this child for IV antibiotics as fever and infiltrates are suggestive of an infectious etiology

If the child with sickle cell disease and asthma a has no fever , a normal chest x-Ray and normal
oxygenation and improves with bronchodilator therapy discharge home can be considered if there is close follow up and a reliable family. I would confer with hematology prior to discharge

Jay M, MD -

I'd be interested to hear your perspective on another aspect of sickle cell disease: opiate tolerance and oligoanalgesia due to provider bias. I found in my time in the States (left the US in 2016 for Australia) that the biggest barrier I had to treatment for sickle cell patients was overcoming the nurses reluctance to provide appropriate doses of IV analgesia for patients. This seemed to be less of a problem in younger paeds patients but as they approached the teen years the nursing staff seemed to drift toward lumping their medical need for analgesia in with the current opiate crisis...a very harmful cognitive error. Do you have any strategies or specific educational resources you've used to help educate staff of the need for appropriate analgesia in these patients?

Tracy G. -

From Ilene Claudius: We don't see alot of SCD patients at County, but we did at CHLA and they were put on PCA pumps fairly quickly- which helped take the onus off of nursing. The rub with these kids and adults is that a very small percent of SCD patients make up the vast majority of pain crises. I'm sure a few are opioid dependent, but the rest just get stuck with the stigma. I think protocols are very helpful- once stamped by the hospital, it is harder to disagree. Borrowing from the guidelines, an initial dose within 30 minutes (I love intranasal fent for this), reassess within 15-30 minutes then redose at same or 25% increase. Avoid merperidine and give only oral benedryl (not parenteral). NSAIDS if no contrindication. I feel like the IV benedryl thing ferrets out the addicts pretty quickly- if I get a big arguement on that one, my guard is up a bit. Usually 2 doses indicates need for admission, so I make the decision at that point. Would your hospital be amenable to a protocol that lays that plan out? Stupid question, but is there an Australian version of CURES? EMpowering a pharmacist or someone to check for multiple opioid prescriptions from different centers might help alter comfort levels as well, and assess this could be part of the protocol. Look up Evidence Based Management of Sickle Cell Disease, Expert Panel, 2014

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