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

ATI Pediatrics-MJ > Ch. 21: Hematologic Disorders-Sickle Cell Anemia & Hemophilia > Flashcards

Flashcards in Ch. 21: Hematologic Disorders-Sickle Cell Anemia & Hemophilia Deck (25):
1

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

Hemoglobin S (HbS)

2

What is the most common form of SCD?

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

3

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

Result of RBC sickling which lead to
-increased blood viscosity
-obstruction of blood flow
-tissue hypoxia

4

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

Due to the presence of fetal Hgb

5

What causes the pain from SCA?

Tissue hypoxia, which causes ischemia, which results in pain

6

What is sickle cell crisis?

The exacerbation of SCA

7

SCD is what kind of disorder?

An autosomal recessive genetic disorder

8

Who does SCA primarily affect? Can it affect others?

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

9

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

True...but they can pass the trait to their offspring

10

What are S&S of SCA?

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

11

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

A painful episode and it lasts 4-6 days

12

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

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

13

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

-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

14

SCA: What is sequestration?

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

15

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

Hypovolemia..progress to shock

16

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

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

17

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

Extreme anemia as a result of decreased RBC production

Triggered by infection

18

SCA: What is hyper hemolytic crisis?

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

19

Is screening for SCA a mandatory thing for newborns?

Yes, in most states

20

What lab test detects anemia?

CBC

21

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

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

22

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

Hemoglobin electrophoresis--separates the various forms of Hgb

23

What happens in lab findings during a sickle cell crisis?

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

24

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

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

25

SCA: Should we promote rest or exercise?

Rest--to decrease oxygen consumption