sickle cell

Sickle Cell Disease (SCD)

An inherited, autosomal recessive disorder affecting red blood cells, including types such as sickle cell anemia, sickle cell-thalassemia, sickle cell Hgb C disease, and sickle cell trait.

Median survival rate for SCD

The median survival for individuals with SCD now exceeds 45 years.

Long-term effects of SCD

Can cause irreversible damage to the lungs, kidneys, brain, retina, and bones, impacting overall quality of life.

Triggers for sickling episodes

Common triggers include low oxygen levels, infections, dehydration, acidosis, low body temperature, high altitudes, and stress.

Reversibility of sickling

Sickling can initially be reversed with re-oxygenation, but eventually becomes irreversible.

Clinical hallmark signs of SCD

Vaso-occlusive phenomena and hemolysis.

Characteristics of sickle cell crises

Severe, painful acute exacerbations involving impaired blood flow, severe hypoxia, and tissue ischemia that can lead to necrosis and shock.

Onset and duration of crises

Sickle cell crises can have a sudden onset and may last for days, with severity depending on Hemoglobin S levels.

Common symptoms of SCD

Often asymptomatic; signs include pallor, jaundice, and pain, with possible fever, swelling, tachypnea, and hypertension.

Diagnostic tests for SCD

Tests include peripheral blood smear, hemoglobin electrophoresis, skeletal X-rays, MRI, Doppler, and chest X-ray.

Goals of nursing management for SCD

Prevent sequelae, alleviate manifestations, minimize damage, and treat serious complications like acute chest syndrome.

Organs involved in SCD complications

Spleen, lungs, kidneys, and brain.

Infection risks in SCD

Infections are a major concern due to splenic dysfunction, with pneumonia being the most common.

Acute chest syndrome

Involves pulmonary complications such as pneumonia and tissue infarction, characterized by fever, chest pain, and dyspnea.

Other complications of SCD

Include retinal vessel obstruction, kidney damage, pulmonary embolism/stroke, bone issues, and chronic leg ulcers.

Key nursing assessments for SCD

Auscultate lungs, noting SPO2 levels and adventitious lung sounds.

Main nursing interventions for SCD

Restore oxygen saturation above 90%, manage pain, and transfuse if necessary.

Medications for SCD

Hydroxyurea and oral glutamine are used to manage the disease.

Hematopoietic Stem Cell Transplant (HSCT)

A life-saving procedure that can cure some clients but may have delayed complications.

Patient and parent education for SCD

Educate on the disease, supportive care, avoidance of dehydration, and importance of immunizations.

Special pediatric considerations for SCD

Educate on fluid management, severity signs, and ensure awareness of the disease among health care teams.