Sickle Flashcards
What is the leading cause of death in sickle cell disease?
Acute chest syndrome (ACS)
ACS is a major complication in patients with sickle cell disease, particularly affecting children.
What are recurrent ACS episodes a major risk factor for?
Sickle cell chronic lung disease (SCLD)
Recurrent ACS episodes significantly increase morbidity and can lead to chronic lung issues.
What type of lung function abnormalities are more frequently seen in children with sickle cell disease?
Obstructive abnormalities
Children more often exhibit obstructive lung issues, while older patients may show restrictive patterns.
What significantly correlates with increased airway obstruction in sickle cell disease?
Elevated pulmonary capillary blood volume
This condition results from chronic anemia associated with sickle cell disease.
At what age do symptoms of sickle cell disease typically begin to manifest?
10–12 weeks of age
Symptoms appear as levels of fetal hemoglobin (HbF) decline.
What is the most severe form of sickle cell disease?
Sickle cell anemia (HbSS)
This form results from homozygous inheritance of the gene for HbS.
What is the primary cause of sickling in red blood cells?
Deoxygenation leading to polymerization of HbS
This process causes the red blood cells to change shape and become less deformable.
What are the clinical features of acute chest syndrome?
- Chest pain
- Productive cough
- Dyspnea
- Fever
- Tachypnea
- Crackles and wheezes
Symptoms may vary with age; young children often present with fever and cough.
What factors increase the incidence and severity of acute chest syndrome?
- HbSS genotype
- Elevated white blood cell count
- Previous ACS episode
- Fever
The presence of these factors is crucial in assessing risk for ACS.
What is the most common pathogen implicated in infections leading to acute chest syndrome?
Chlamydia pneumoniae
Mycoplasma pneumoniae and RSV are also common causes of infections in these patients.
What is the expected prognosis for individuals with pulmonary hypertension in sickle cell disease?
Median survival reported to be 25.6 months
Sudden death is common due to pulmonary thromboembolism and cardiac arrhythmia.
What is a common complication of sickle cell disease that affects lung function over time?
Sickle chronic lung disease (SCLD)
This condition is characterized by hypoxemia and restrictive lung disease.
Fill in the blank: The HbS solubility test is ______ in the first 6 months of life.
unreliable
This is due to the predominance of fetal hemoglobin (HbF) in infants.
What treatment is recommended for patients with recurrent acute chest syndrome?
Hydroxyurea
Hydroxyurea increases the level of fetal hemoglobin and reduces sickle cell complications.
True or False: Exercise capacity in sickle cell disease is unaffected by the baseline degree of anemia.
False
Exercise capacity is significantly lower in subjects with a history of recurrent acute chest syndrome.
What monitoring is essential for children with sickle cell disease?
Annual respiratory monitoring
Early monitoring is vital as lung function deteriorates rapidly in young children.
What is the primary cause of death in adults with sickle cell disease?
Respiratory failure
This is often due to pulmonary emboli or bronchopneumonia.
What risk factors are associated with increased morbidity in children with sickle cell disease?
- Fever
- Acute neutrophil count > 9 × 10^9/L
- Hemoglobin < 8.6 g/dL
- Previous ACS episode
These factors heighten the risk for complications such as acute chest syndrome.
What is one method to avoid sickling during exercise in patients with sickle cell disease?
Hydration should be maintained
This helps prevent sickling and vaso-occlusive episodes during physical activity.
