History, Physical, and Initial Work-Up

History and Physical

Prior acute chest syndrome
Asthma
Fever
Respiratory symptoms (cough, shortness of breath, dyspnea)
Recent history of sedation without prior transfusion

Current chest pain
Restrictive lung disease
Nocturnal hypoxia

Initial Work-Up

CBC with differential, reticulocyte count, HbS level
BMP, liver function panel, LDH
Blood culture if febrile, hypotensive, or toxic-appearing
Type and Screen
Chest X-ray (2 views) if any respiratory symptoms, even in the absence of hypoxia or abnormal lung findings on exam

Consider blood gas
Consider respiratory panel PCR and COVID-19 testing

Typical Laboratory Findings in Sickle Cell Disease
Genotype	Hb (g/dL)*	HbS (%)	HbA (%)	HbA2 (%)	HbF (%)	HbC (%)
SS	6 - 9	> 90	0	< 3.5	< 10	0
Sβ⁰-thalassemia	7 - 9	> 80	0	> 3.5	< 20	0
Sβ⁺-thalassemia	9 - 12	> 60	10 - 30	> 3.5	< 20	0
SC	9 - 14	50	0	< 3.5	< 1.0	45
Note. Hb = hemoglobin; HbS = sickle hemoglobin; HbA = normal adult hemoglobin; HbA2 = minor variant of adult hemoglobin; HbF = fetal hemoglobin; HbC = hemoglobin variant that causes manifestations of sickle cell disease when paired with HbS *The hemoglobin values presented apply in the absence of a blood transfusion in the last four months, are not absolute, and are applicable only to adults and children, not neonates

Reference

National Heart, Lung, and Blood Institute. (2014). Evidence-based management of sickle cell disease. (NIH Publication). U.S. Department of Health and Human Services, National Institutes of Health. https://www. nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease

These pathways do not establish a standard of care to be followed in every case. It is recognized that each case is different, and those individuals involved in providing health care are expected to use their judgment in determining what is in the best interests of the patient based on the circumstances existing at the time. It is impossible to anticipate all possible situations that may exist and to prepare a pathway for each. Accordingly, these pathways should guide care with the understanding that departures from them may be required at times.

History, Physical, and Initial Work-Up