Ch. 21: Hematologic Disorders-Sickle Cell Anemia & Hemophilia

Question 1

Sickle cell disease (SCD) is a group of diseases in which abnormal sickle _____ replaces normal adult hemoglobin (Hgb A)

Answer 1

Hemoglobin S (HbS)

Question 2

What is the most common form of SCD?

Answer 2

Sickle cell anemia is the homozygous and most common form of SCD

Question 3

Manifestations and complications of SCA are the result of _____, which leads to increased ______, obstruction of ____, and tissue ____.

Answer 3

Result of RBC sickling which lead to

• increased blood viscosity
• obstruction of blood flow
• tissue hypoxia

Question 4

Why are manifestations of SCA not usually apparent until later in infnancy?

Answer 4

Due to the presence of fetal Hgb

Question 5

What causes the pain from SCA?

Answer 5

Tissue hypoxia, which causes ischemia, which results in pain

Question 6

What is sickle cell crisis?

Answer 6

The exacerbation of SCA

Question 7

SCD is what kind of disorder?

Answer 7

An autosomal recessive genetic disorder

Question 8

Who does SCA primarily affect? Can it affect others?

Answer 8

Primarily African American, other forms of SCD are individuals of Mediterranean, Indian, or Middle Eastern descent

Question 9

T/F: Children with sickle cell trait do not manifest the disease.

Answer 9

True…but they can pass the trait to their offspring

Question 10

What are S&S of SCA?

Answer 10

SOB
Fatigue
Pallor/pale mucous membranes
Jaundice
Hands and feet cool to touch
Dizziness
Headache

Question 11

SCA: What is a vaso-occulsive crisis and how long does it last?

Answer 11

A painful episode and it lasts 4-6 days

Question 12

SCA: What are the acute signs of vaso-occulsive crisis?

Answer 12

• Severe pain (bones, joints, abdomen)
• Swollen joints, hands, and feet
• Anorexia, Vomiting, fever
• Hematuria
• Obstructive jaundice
• Visual disturbances

Question 13

SCA: What are the chronic signs of vaso-occulsive crisis?

Answer 13

• Increased resp. infections and osteomyelitis
• Retinal detachment and blindness
• Systolic murmur
• Renal failure and enuresis
• Liver cirrhosis; hepatomegaly
• Seizures
• Skeleteal deformities; shoulder or hip avascular necrosis

Question 14

SCA: What is sequestration?

Answer 14

Excessive pooling of blood primarily in the spleen (splenomegaly) and sometimes in the liver (hepatomegaly)

Question 15

SCA: Sequestration–reducing circulating blood volume results in ____ and can progress to ___

Answer 15

Hypovolemia..progress to shock

Question 16

SCA: Sequestration–What are the signs of hypovolemic shock?

Answer 16

Irritability
Tachycardia
Pallor
Decreased urinary output
Tachypnea
Cool extremities
Thready pulse
Hypotension

Question 17

SCA: Aplastic crisis..What is it and what is it usually triggered by?

Answer 17

Extreme anemia as a result of decreased RBC production

Triggered by infection

Question 18

SCA: What is hyper hemolytic crisis?

Answer 18

Increased rate of RBC destruction leading to anemia, jaundice, and/or reticulocytosis

Question 19

Is screening for SCA a mandatory thing for newborns?

Answer 19

Yes, in most states

Question 20

What lab test detects anemia?

Answer 20

CBC

Question 21

What lab test is a screening tool that detects the presence of HbS. What does this test NOT do?

Answer 21

Detects the presence of HbS= Sickledex (sickle-turbidity)…This test DOES NOT differentiate the trait from the disease!

Question 22

What is the definitive diagnosis of sickle cell anemia? What does this test do?

Answer 22

Hemoglobin electrophoresis–separates the various forms of Hgb

Question 23

What happens in lab findings during a sickle cell crisis?

Answer 23

• Hgb= decreased
• WBC, biliruben, reticulocyte levels= elevated
• Peripheral blood smear reveals sickled cells

Question 24

What is the transcranial dropper (TCD) test and how often is this done?

Answer 24

Assesses intracranial vascular flow and detects the risk for CVA–done annually on children ages 2-16 who have SCD

Question 25

SCA: Should we promote rest or exercise?

Answer 25

Rest–to decrease oxygen consumption