manifestations-of-sickle-cell-disease?search=sickle cell
anemia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank
=1.
Painful vaso-occlusive crisis (VOC) is the most frequent complication of Sickle Cell
Disease. Common sites of pain include bone pain which can be in the extremities causing
dactylitis or hand/foot syndrome and abdominal pain. Bone pain is the most common type of
VOC and may or may not be accompanied by swelling, low-grade fever, redness, and warmth, as
presented with Miah in the case study this week.
Dactylitis is a common presentation in infants

and toddlers; where back and abdominal pain are more common in older children. Abdominal
pain in children with sickle cell disease is usually a simple VOC, but other diagnoses may
present which include splenic sequestration, liver sequestration, appendicitis, pancreatitis, biliary
colic and cholecystitis, urinary tract infection, and pelvic inflammatory disease and should be
ruled out. In addition, pneumonia and chest crisis may present as, or accompany abdominal pain.
During a severe pain crisis, a patient may also develop an acute chest syndrome, or a central
nervous system event. Pain should be treated early and as aggressive as possible (“Acute Painful
Episodes Vaso-occlusive Crisis,” 2019).
In 2017,
the Food and Drug Administration (FDA) granted regular approval to hydroxyurea
to reduce the frequency of painful crises and the need for blood transfusions in pediatric patients
from 2 years of age and older with sickle cell anemia with recurrent moderate to severe painful
crises(Center for Drug Evaluation and Research, 2017
).
Benefits associated with the use of
hydroxyurea in sickle cell patients include (Agrawal, Patel, Shah, Nainiwal, & Trivedi, 2014
):
Single-agent, inexpensive, orally administered, once-daily dosing
Increases HbF and total Hb, reduces WBC and reticulocytes, and lowers LDH
Leads to fewer vaso-occlusive events and hospitalizations
Works in all age groups
Prevents acute events and chronic organ dysfunction
Benefits continue over time without medication resistance or tolerance
Reference
Acute Painful Episodes Vaso-occlusive Crisis: Guidelines for Management in Children with
Sickle Cell Disease. (2019, April 30). Retrieved from -
practice-guidelines/clinical-practice-guidelines/Export/CLINS214/Main Document.pdf.
Agrawal, R. K., Patel, R. K., Shah, V., Nainiwal, • L., & Trivedi, B. (2014). Hydroxyurea in
Sickle Cell Disease: Drug Review.
Indian Journal of Hematology and Blood
Transfusion
,
30
(2), 91–96. doi: 10.1007/s12288-013-0261-4
Center for Drug Evaluation and Research. (2017, December 21). FDA approves hydroxyurea for
treatment of pediatric patients with sick. Retrieved from
-
treatment-pediatric-patients-sickle-cell-anemia.

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- Summer '17
- Sickle cell anemia, Sickle-cell disease