Hyperhemolytic crisis accelerated rate of rbc

Hyperhemolytic Crisis : Accelerated rate of RBC destruction characterized by anemia, jaundice
and reticulocytosis.
Vaso-Occlusive Crisis
Results from sickled cells obstructing blood vessels = PAIN in area of involvement
Signs & Symptoms
 Distal ischemia and pain
 Hand foot syndrome = occlusion in the small distal bones of the hands and feet = pain
and swelling
*Immediately report symmetric swelling of the hands and feet in the infant or toddler. Termed
dactylitis, aseptic infarction occurs in the metacarpals and the metatarsals
 Abdomen = severe pain resembling acute surgical condition
 Cerebrum = Stroke, visual disturbances
 Chest = Resembles pneumonia, chest pain, SOB, cough
 Arthralgia = pain in the joint
 Priapism
 Kidney = hematuria
HHOP TO IT!!! (Heat, Hydration, Oxygenation & Pain Management)
 Prevent Sickle Cell Disease
 Prevent Sickle Crisis
 Maintain Adequate Hydration / Avoid dehydration
 Maintain Oxygenation
 Prophylactic antibiotics
 Treat medical emergencies
 Vaccines
 Control Pain
*Do not use meperidine for pain management during sickle cell crisis because multiple dosing
has been associated with an increased risk of seizures
HYDRATION FORMULA
Pediatric Hematologic & Neoplastic Alterations - 10 of 17
1
st
10 Kg Kg x 100 mL (Max 1000 mL)
Next 10 – up to 20 Kg 1000 + Kg x 50 mL (max 1500 mL)
Over 20 Kg 1500 + Kg x 20 mL

Acute Chest Syndrome -SCA complication
 Presence of pulmonary infiltrate
 Acute Chest Syndrome is a medical emergency! One of the most common reasons for
hospitalization, and a common cause of death for adolescent patients with sickle cell
disease.
 Increased risk for pneumonia due to decreased oxygen to lung tissue
 Clinical Manifestations:
o Chest pain, back pain, abdominal pain, fever 101.3 or higher, congested cough,
dyspnea/wheezing, tachypnea, retractions and declining O2 sats.
 TX: deep breathing, antibiotics, O2, pain meds, blood transfusion, chest tube
CVA (Stroke)-SCA complication
 Sudden and severe complication, may occur in the absence of related illness
 Major blood vessels of the brain are blocked leading to cerebral infarction, may cause
variable degrees of neurologic impairment.
 Current treatment involves chronic transfusion therapy, performed monthly to prevent a
repeat stroke
 Clinical Manifestations:
o Severe headache, vomiting, jerking of the face, legs, arms, seizures, strange
behavior, inability to move arm or leg, unsteady gait, slurred speech, weakness in
hands and feet and vision changes.
Therapeutic Management
Aims of therapy include prevention of sickling and treatment of medical emergencies due to
crises.
 Crisis management is generally supportive and symptomatic
 MINIMIZE TRIGGERS! Fever, hypoxia, emotional or physical stress.
 Rest to minimize oxygen use - Short term O2 therapy
 Oral and IV hydration
 Electrolyte replacement to combat MA that results from hypoxia
 Pain management
o Treat mild moderate pain with acetaminophen or ibuprofen.