Patient Presentation:
A 3-year old child, K.S. presented to the ER with acute onset chest pain, coughing, difficulty breathing, and fever. She also complains of severe generalized pain mostly in her chest and abdomen that is increasingly worsening as well as debilitating. The patient’s mother says she has had flu like symptoms for 2 days with a runny nose, congestion, fatigue, and decreased appetite. The mother states she has been pushing fluids but has not been too successful. Due to routine newborn screening tests, K.S. has had a diagnosis of sickle cell disease since birth and therefore is up to date with her current immunizations; however she has had multiple hospital admissions due to anemia in the past 3 years.
Her vital signs are RR 42, T 38.5 Celsius, BP 92/52, P 126, SaO2 90% RA.
She is immediately placed on 4 L O2 per NC and baseline Labs, Chest X-Ray, and EKG were completed. IV NS bolus of 500 ml was given along with maintenance fluid at 50ml/hr. IV morphine was given to control her pain.
EKG: Sinus Tachycardia
Chest X-Ray: Bilateral lower lobe infiltrate
Labs: WBC: 18,000, Hbg 7, Hct 22, Reticulocyte 10.2%, RBC 2.8
BMP: Na+ 136, K+4.0, Mg++ 1.5, Phos- 2.9, BUN 18, Cr 1.0
LFT: ALT 37, AST 46, Direct Bil 0.5, Total Bil 1.1
Diagnosis:
Sickle Cell Crisis with Acute Chest Syndrome
Sickle Cell disease is an autosomal recessive inherited disorder. Erythrocytes in sickle cell anemia contain abnormal hemoglobin that affects the beta-chain producing hemoglobin S or HbS. Valine (amino acid) takes the place of the normally appearing glutamic acid in beta-chains. Replacement of glutamic acid with valine causes the polymerization of HbS components to cohere forming long and insoluble particles. This distorts the RBC, which then assumes the inflexibility crescent or sickle shapes that become sharp and spiky when the RBC’s are deoxygenated. Sickle Cell Crisis refers to episodes of acute and severe sickling that blocks the circulation posing a threat of excessive organ damage and severe pain.
Sickle Cell Crisis may occur in any part of the body and may be brought on by cold or dehydration. Acute chest syndrome can be caused by a lung infection or by sickle cells blocking blood vessels in the lungs.
Treatment:
K.S. is admitted to the PICU where she will receive O2 to keep her SaO2 above 94%. She will receive 2 units PRBCs along with IV fluid to compensate for her dehydration. She is also given IV morphine to treat the acute pain. She is started on IV Penicillin G and given albuterol nebulizer treatments as a bronchodilator Q6 hours.
Outcome:
Due to quick action by her mother K.S. was stabilized in the PICU after the transfusions and hydration and will be discharged home. She will need close monitoring by a hematologist to prevent subsequent sickle cell events, as this is a life long disease. K.S. will be discharged on folic acid supplements to help continue to produce new RBC’s and PO penicillin b.i.d. until the age of 5 to prevent recurrent infections. Recommendations are now for infants from birth to age 5 to stay on prophylactic antibiotics. Children who develop serious complications (such as recurrent acute chest syndrome, severe anemia, or stroke) may receive regular blood transfusions to prevent or treat these complications.
Case created by Kimberly Schuelke, 2011.