A 25 year old female, a known case of sickle cell anemia is presented with bony pain, dyspnea, fever & cough. O/E: her HR is 110/min & BP is 140/88 mmHg, SaO2 is 85%. CXR shows bilateral diffuse alveolar infiltrates. Her mother gives history of four similar episodes in the last one year. All of the statements regarding her condition are true EXCEPT:
Given scenario suggests the diagnosis of acute chest syndrome- medical emergency that may require management in ICU. Rx: Hydration- monitored carefully to avoid the development of pulmonary edema Oxygen therapy- to avoid hypoxemia Transfusion to maintain a hematocrit >30% and emergency exchange transfusion if aerial saturation drops to <90%. Hydroxyurea- mainstay of therapy for patients with severe symptoms It increases fetal hemoglobin and exes beneficial effects on RBC hydration, vascular wall adherence, and suppression of the granulocyte and reticulocyte counts Trials of sildenafil to restore NO2 levels were terminated because of adverse effects.
Chronic therapy with oral hydroxyurea should be considered.
She is having a sickle cell acute chest syndrome.
She should receive daily sildenafil.
Hematocrit should be maintained at >30%.
Sickle Cell Disease, G6PD deficiency and other Hemolytic Anemia