COPY: Sickle Cell Disease and Thalassemia

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Questions and Answers

What is a recommended strategy to prevent illness in individuals with Sickle Cell Trait (SCT) during training or conditioning activities?

  • Pushing oneself to exhaustion to build endurance
  • Avoiding water intake during training to prevent dehydration
  • Setting your own pace and building your intensity slowly (correct)
  • Exercising in hot and humid temperatures without breaks

What is the primary goal of treatment in Sickle Cell Disease (SCD)?

  • Identifying the genotype of every child
  • Curing the disease
  • Reducing the financial burden of the disease
  • Symptom control and management of disease complications (correct)

At what stage should every child know their genotype, according to the primary prevention strategy?

  • Before entering secondary school
  • Before getting married
  • Before starting a job
  • Before entering primary school (correct)

What is a consequence of not managing Sickle Cell Disease (SCD) properly?

<p>Inability to achieve one's potential (D)</p> Signup and view all the answers

Why is it important to seek medical care immediately when feeling ill with Sickle Cell Disease (SCD)?

<p>To avoid crisis and manage disease complications (A)</p> Signup and view all the answers

How can individuals with Sickle Cell Trait (SCT) reduce their risk of illness during physical activities?

<p>By drinking plenty of water and resting frequently (A)</p> Signup and view all the answers

What is the primary reason for the significant declines in mortality rates in children with Sickle Cell Disease up to age 19?

<p>Prophylactic treatment with penicillin and vaccinations (C)</p> Signup and view all the answers

What is the purpose of transcranial doppler screening in children with Sickle Cell Disease?

<p>To identify children at risk of stroke (C)</p> Signup and view all the answers

Which age group has shown no significant decline in mortality rates for Sickle Cell Disease?

<p>20-24 (A)</p> Signup and view all the answers

What is a potential strategy to reduce the SCD burden in Africa?

<p>Establishing partnerships between high resource countries and countries in Africa (A)</p> Signup and view all the answers

What is a key factor contributing to the increase in death rates for Sickle Cell Disease in older adults?

<p>Increased risk of stroke (A)</p> Signup and view all the answers

What is a potential benefit of newborn screening for Sickle Cell Disease in Africa?

<p>Early diagnosis and treatment of SCD (B)</p> Signup and view all the answers

What is the primary function of hemoglobin in the human body?

<p>To allow red blood cells to carry oxygen to all parts of the body (B)</p> Signup and view all the answers

What is the genetic composition of people with HbS beta 0-thalassemia?

<p>They inherit one sickle cell gene and one beta thalassemia gene (C)</p> Signup and view all the answers

What is the probability that a child will have SCD if both parents have SCT?

<p>25% (or 1 in 4) (B)</p> Signup and view all the answers

What is the result of the pathophysiology of SCD?

<p>A rigid, sickle-like shape under certain circumstances (A)</p> Signup and view all the answers

What is the name of the abnormal oxygencarrying protein found in red blood cells in people with SCD?

<p>Haemoglobin S (C)</p> Signup and view all the answers

What is the effect of low oxygen levels on red blood cells in people with SCD?

<p>They become more rigid and take on a sickle-like shape (C)</p> Signup and view all the answers

What is the approximate average cost in hospitalizations due to SCD in the United States from 1989 through 1993?

<p>$475 million (A)</p> Signup and view all the answers

What is the estimated number of babies born with severe forms of hemoglobinopathies worldwide each year?

<p>300,000 (A)</p> Signup and view all the answers

What percentage of patients with SCD live in Sub-Saharan Africa?

<p>75% (D)</p> Signup and view all the answers

What is the average expenditure for children with SCD who had employer-sponsored insurance?

<p>$14,772 (C)</p> Signup and view all the answers

At what age do SCD symptoms typically start manifesting?

<p>6 months (D)</p> Signup and view all the answers

What is one of the risk factors for SCD crises?

<p>Increased pressure in the atmosphere (A)</p> Signup and view all the answers

What is the average lifespan of sickle red cells?

<p>20 days or less (C)</p> Signup and view all the answers

What is the main cause of anemia in SCD?

<p>Red cell destruction (C)</p> Signup and view all the answers

What is the term for the abnormal adhesion of sickled RBCs to endothelium and macrophages?

<p>Vaso-occlusion (A)</p> Signup and view all the answers

What is the approximate percentage of US patients with the Sβ° thalassemia genotype?

<p>2% (C)</p> Signup and view all the answers

At what age do the effects of sickling start manifesting?

<p>6 months (A)</p> Signup and view all the answers

What is the term for the recurrent episodes of pain in SCD?

<p>Recurrent vaso-occlusive pain (C)</p> Signup and view all the answers

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