Peds Test # 3 Blood Dyscrasias

Question 1

What are the 4 methods used to diagnose Sickle Cell Anemia (SCA)?

Answer 1

1) Prenatal amniocentesis
2) Newborn screening: Sickledex
3) Hemoglobin electrophoresis - Differentiates trait from the disease.
4) Transcranial Doppler (TCD) - Assesses intracranial vascular flow (ages 2-16).

Question 2

What is the result of RBC sickling?

Answer 2

RBC sickling > blood flow obstruction > tissue hypoxia > tissue ischemia > pain

Question 3

What are the 6 SxS of Sickle Cell Anemia which usually starts around 6 months of age?

Answer 3

1) Shortness of breath > fatigue
2) Tachycardia
3) Pallor
4) Impaired healing and frequent infections
5) Delayed growth
6) Systolic heart murmur and heart failure

Question 4

What are the 3 conditions that cause RBCs to sickle?

Answer 4

1) ⬇ O2 concentrations
2) Acidosis
3) Dehydration

Question 5

What are the 7 SxS of an Acute Vaso-Occlusive Crisis resulting from SCA?

Answer 5

1) Sever pain in bones, joints and abdomen
2) Swollen joints, hands and feet
3) Anorexia, vomiting and fever
4) Hematuria
5) Jaundice
6) Visual disturbances
7) CVA

Question 6

What are the 8 SxS of an Chronic Vaso-Occlusive Crisis resulting from SCA?

Answer 6

1) Respiratory infections
2) Blindness
3) Systolic murmurs
4) Renal failure and enuresis
5) Liver enlargement/failure
6) Spleen enlargement/infections
7) Seizures
8) Skeletal deformities

Question 7

List the 6 nursing diagnoses discussed in lecture that are appropriate for a patient with SCA.

Answer 7

1) Acute pain
2) Altered tissue perfusion
3) Fluid volume deficit
4) Risk for injury
5) Risk for infection
6) Delayed growth and development

Question 8

What are the 8 methods of treatment used to manage SCA?

Answer 8

1) Bedrest
2) Hydration
3) Pain management
4) Electrolyte replacement
5) Give blood products
6) Antibiotics
7) Heat and passive ROM
8) Give O2

Question 9

What are the 3 methods used to prevent SCA complications?

Answer 9

1) Transfusion Program - Every 4-5 wks to maintain Hgb
2) Hydroxyurea - Oral med used to ⬆ fetal hemoglobin a that don’t sickle.
3) Bone Marrow Transplant - Only cure

Question 10

What are the 7 appropriate patient teachings for a patient with SCA?

Answer 10

1) Take frequent rest periods
2) No contact sports if spleen is enlarged
3) Avoid infections
4) Avoid low oxygen environments
5) Ensure adequate hydration (even though it may cause enuresis)
6) Apply heat to painful area
7) Maintain vaccination schedules

Question 11

Describe the disorder of Hemophilia.

Answer 11

It is a group of bleeding disorders from a congenital deficiency of specific coagulation proteins. It is an X-linked recessive disease carried by females (mothers) and transmitted to sons.

Question 12

How is Hemophilia diagnosed in males? How is the trait identified in females?

Answer 12

1) Males - Ptt

2) Females - DNA testing for trait

Question 13

What are the 6 SxS of Hemophilia?

Answer 13

1) Prolonged bleeding
2) Hemorrhage
3) Excessive bruising
4) Spontaneous Hematuria
5) Hemarthrosis (bleeding into joint cavity)
6) Hematomas (swelling, pain, limited ROM)

Question 14

What 4 methods are used to prevent complications from hemophilia?

Answer 14

1) Primary - Regular schedule of factor VIII
2) Secondary - Factor infusion after joint bleed
3) Mild Cases - DDAVP: A des oppression nasal spray which ⬆ factor VIII.
4) Antifibrinolytic Agents (Amicar) - Inhibits breakdown of fibrin

Question 15

What 6 methods are used to manage Hemophilia?

Answer 15

1) Blood transfusions - Includes FFP or PRBCs
2) RICE - Rest, Ice, Compression, Elevation
3) Aspiration - For severe bleeding
4) Pain Management - No NSAIDs
5) Use bleeding precautions
6) Active ROM (not passive)

Question 16

Describe the disorder of Immune Thrombocytopenic Purpura (ITP)?

Answer 16

ITP is an autoimmune disorder that destroys platelets via platelet antibodies. The antibodies bind to the plasma membrane of platelets causing them to be removed from circulation in the spleen and liver.

Question 17

What are 3 common etiologies/causes of ITP?

Answer 17

1) Viral infections
2) Drugs
3) Collagen vascular diseases (lupus)

Question 18

What are the two types of ITP?

Answer 18

1) Acute - Majority of cases, self limiting, resolves in days to weeks.
2) Chronic - Lasts > 6 months

Question 19

How is ITP diagnosed?

Answer 19

There are no definitive tests to diagnose ITP but the patient usually has platelet count < 20,000.

Question 20

What are the 6 different methods used to manage ITP?

Answer 20

1) Restrict activity
2) Anti-D antibody
3) Platelet placement
4) IVIG (an immunoglobulin)
5) Prednisone
6) Splenectomy for chronic severe form

Question 21

Describe the disorder of Aplastic Anemia.

Answer 21

When all formed elements of the blood are depressed.
⬇ RBCs
⬇ WBCs
⬇ Platelets

Question 22

What two methods are used to diagnose Aplastic Anemia?

Answer 22

1) Bone marrow aspiration

2) Labs - ⬇ RBCs, ⬇ WBCs, and ⬇ platelets

Question 23

What 3 methods are used to manage Aplastic Anemia?

Answer 23

1) Immunosuppressive therapy
2) Colony stimulating factors
3) Bone marrow transplant

Question 24

What are the 7 SxS of Anemia?

Answer 24

1) Petechiae and Ecchymosis
2) Pallor
3) Epistaxis
4) Fatigue & Weakness
5) Tachycardia
6) Anorexia
7) Infection

Question 25

What are the 4 factors that contribute to Iron Deficiency Anemia (IDA)?

Answer 25

1) ⬇ Iron intake
2) ⬇ Iron stores
3) ⬆ Iron loss
4) Poor utilization of iron by body

Question 26

What are 5 the SxS of IDA?

Answer 26

1) Overweight milk baby
2) Pallor
3) Pale mucous membranes
4) Content
5) Tired/fatigued

Question 27

What are the 4 methods used to diagnose IDA?

Answer 27

1) ⬇ Hbg
2) ⬇ Hct
3) ⬇ Serum iron level
4) ⬆ TIBC (Total Iron Binding Capacity)

Question 28

What are the 3 methods of treatment used for infants and children with IDA?

Answer 28

1) Give Iron formula, supplements and foods
2) Limit milk intake to < 32 oz/day for infants and 24 oz/day max for children older than 12 months.
3) Limit activities (need rest)

Question 29

What 7 patient teachings should be taught to your patient who is taking Iron Supplements?

Answer 29

1) Take on an empty stomach
2) Use dropper to avoid teeth stains
3) Stools may be tarry and constipation may ⬆
4) Do not take with dairy products
5) For max effect, give in 3 divided doses/day
6) OD can be fatal - child proof supplements
7) Over 6 months of age - give with citrus to ⬆ absorption