T3-Blueprint: Blood Disorders Flashcards Preview

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Flashcards in T3-Blueprint: Blood Disorders Deck (64):
1

What is key with sickle cell disease?

Hydration and oxygenation

2

Sickle cell disease is a genetic issue resulting ins ever reduction in ____

Circulating RBC

3

What is the average life span for sickle cell?

45

4

What is the hgb count in sickle cell? What is normal?

2-5 gm/dl

Norm is above 11

5

Sickle cell disease--RBC contain an abnormal form of HGB so what does that mean?

RBC are inflexible and rigid (sickle shape)

More fragile= hemolysis (death of cells)

6

Can sickle cell be cured?

No

7

What is treatment for sickle cell?

Controlling anemia and relieving pain

Blood transfusions

Ions and fluid replacement is needed

8

T/F: A small cold in a sickle cell patient will make them very ill

True

9

Nutrition: What do they need? What do they not need?

Need: folic acid, and can eat normal foods but don't need lots of sweets and fast foods

10

Sickle cell patients in crisis is a HIGH PRIORITY!--What are the normal complaints they are telling if they come to ED?

Severe pain and fever

11

Screening tests for sickle cell only detect the prescence of an abnormal hb. What are they NOT specific for?

Trait or anemia

12

What is the definitive test for sickle cell?

Hgb electrophoresis

13

Sickle cell: What is the Hgb electrophoresis test?

Definitive test for sickle cell that separates and measures the various HGB aka differentiates between trait and anemia

14

Sickle cell: Ophthalmic complications. What does this mean

-Vitreous hemorrhage
-Retinal detachment
-Blindness

15

Sickle cell: What is a vaso-occlusive crisis?

CVA
Chest syndrome
Dactylitis
Painful episodes

16

Sickle cell: Vaso-occlusive crisis

Chest syndrome: If they have had this in the past, will they have it again?

They probably will

It can cause life threatening issues

Chest syndrome= chest pain, fever, cough (can worsen with or result from pneumonia)

17

Sickle cell: Vaso-occlusive crisis. What may be the first symptom of vaso-occlusion?

Hand and foot syndrome (dactylitis)

18

Sickle cell: Vaso-occlusive crisis. What is the most frequent complication?

Painful episodes occuring in joints and limbs

*Pain is there bc there is obstruction of blood flow due to sickled RBC

19

What are some other complications of sickle cell disease?

-Cardiomegaly & systolic flow murmurs
-Abdominal pain, GU dysfunction
-Enlarged spleen
-Dilute urine

20

What is a main issue of sickle cell disease?

Enlarged spleen

21

SCD: Enlarged spleen is caused by picked blood. How is this treated? What can this cause?

Treat with blood transfusion

Can cause anemia, hypovolemic shock

22

SCD: What is the role of the spleen?

Filters out infection in the body

23

SCD: If the spleen is enlarged, there is risk for infection!!! Explain.

1. Normal cells last 3 months; sickled cells only last 1 month
2. Keep out of crowds
3. Penicillin is DOC for this issue

24

What is sickle cell crisis triggered by?

Viral infection or depletion of folic acid

Signs: profound anemia and pallor

25

What is aplastic crisis of SCD caused by?

Human parvo virus leading to lowered Hb, retake count

26

SCD: How is aplastic crisis treated?

Observation and transfusion

27

SCD: T/F: Once they've had one stroke, they will normally have another

TRUE

Stroke affects 10% of children with sickle cell

28

What do we do to treat the SCD complications?

-IV hydration
-Analgesia: morphine
-Transfusion/transplants

29

When is RBC transfusion used for sickle cell complications?

Aplastic, hyperhemolytic, and splenic sequestration crisis

30

SCD: Avoid heat or cold?

COLD!!!

31

What is treatment for FE overload?

Chelating agent that binds with iron in the bloodstream and enhances its elimination in the urine and feces

32

Who is more likely to get hemophilia: boys or girls?

BOYS

*this is an X linked recessive disorder--girls have 2x, boys only have 1

33

Hemophilia A is a deficiency in factor ?

Factor VIII defiency

"classic hemophilia"--80% of cases

34

Hemophilia B is a deficiency of what factor?

Factor IX

"Christmas disease"

35

Hemophilia: What is there difficulty controlling bleeding?

Bleeding time is exteneded due to lack of factor required for blood to clot

36

Hemophilia: When may difficulities become apparent?

Male circumcision...but may not become apparent until infant becomes more active and prone to injuries during toddler years

37

Hemophilia: What are s/s?

Active bleeding
Hematomas/bruising
Hemarthrosis
Headache
Slurred speech
Decreased LOC

38

Hemophilia: Avoid hot or cold?

AVOID HOT!

39

What are some tests for hemophilia?

aPTT
Factor specific assays
Platelet and prothrombin time
Whole blood clotting time

40

Hemophilia: Ok to take rectal temps?

Avoid this, as well as skin punctures and use surgical aseptic technique

41

What are Factor VIII and IX ?

Clotting factors necessary to begin clotting cascade

42

Why do we give factor VIII and IX to hemophilia patients?

This is given via port-a-acth bc this is what hemophilia pts ar deficient in

43

What is the key sign to prolonged bleeding to the joints in hemophilia pts?

Hemoarthritis

44

What are s/s of prolonged bleeding to the joints in hemophilia pts.?

-Joint pain stiffness
-Warmth
-Swelling
-Redness
-Loss of ROM
-Deformitis

45

Hemophilia: How do we treat prolonged bleeding to joints?

Must treat immediaely!

RICE

Active ROM after bleeding controlled

Maintain ideal weight to avoid stress to joints

Encourage regular exercise and PT

46

Hemophilia: If we need to give injections or perform an invasive procedures, how should we pretreat?

Pretreat with their specific factor (VIII or IX)

47

Hemophilia: How do we prevent bleeding at home?

Padded crib

48

Hemophilia: How do we prevent bleeding for toddlers?

Environment free of clutter--padding corners of furniture

Dress toddlers in extra layers of clothing to provide padding

49

Hemophilia: How do we prevent bleeding for preschool?

Supervision/diversion

50

Hemophilia: How do we prevent bleeding in school aged?

Set activity restrictions

Low contact sports!!! (tennis, swimming)--use protective equipment

51

Hemophilia basics--Whats important for client educatin?

-Use soft bristle toothbrush
-Up-to-date on immunizations
-Med ID wristband or tags need to be worn
-RICE

52

Why is iron deficiency anemia common in infancy?

Ages 12-36 months are most at risk due to consuming a diet high in cow's milk without adequate intake of foods high in iron

*Hgb requires iron...Low iron=low hgb

53

Why are adolescents at risk for iron deficiency anemia?

Poor diets
Menses
Obesity

54

How should we administer iron for iron deficiency anemia?

Give on empty stomach (with juice, NOT MILK)

Give with meals and start with reduced dose and gradually increase if GI distrubce occurs

Give with Vit. C to help increase absorption

55

If we are given liquid iron, how should child drink it?

Through a straw

*brush teeth to prevent staining

56

What should we tell parents about stools of children who are taking iron?

Stools with be tarry green--this is normal

57

If we give iron IM, how should it be given?

z-track method--do not massage afterward!

58

What is epistaxis?

Nose bleed

59

How do you stop a nose bleed?

Sit up and slightly lean forward and pinch bridge of nose for 10 min

60

A secondary disorder of coagulation that occurs as a complication of a number of pathologic processes (hoopla, acidosis, shock, burns)

Disseminated intravascular coagulation (DIC)

61

What are the hallmarks of DIC?

Bleeding and clotting

62

DIC: What is the patho and why is this bad?

Starts when the clotting process gets started abnormally--thrombin is made faster than body can neutralize it...bad bc child is susceptible to uncontrollable hemorrhage into vital organs

63

S/S of DIC?

-Petechia
-Purpura
-Bleeding form openings
-Bleeding from umbilicus or trachea (newborn)
-Bleeding evidence in GI
-Hypotension
-Organ dysfunction from infarction and ischemia

64

Therapeutic management for DIC?

Control of underlying or imitating cause which usually stops problem spontaneously

Platelets and fresh frozen plasma may be needed

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