T3-Blueprint: Blood Disorders

Question 1

What is key with sickle cell disease?

Answer 1

Hydration and oxygenation

Question 2

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

Answer 2

Circulating RBC

Question 3

What is the average life span for sickle cell?

Answer 3

45

Question 4

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

Answer 4

2-5 gm/dl

Norm is above 11

Question 5

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

Answer 5

RBC are inflexible and rigid (sickle shape)

More fragile= hemolysis (death of cells)

Question 6

Can sickle cell be cured?

Answer 6

No

Question 7

What is treatment for sickle cell?

Answer 7

Controlling anemia and relieving pain

Blood transfusions

Ions and fluid replacement is needed

Question 8

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

Answer 8

True

Question 9

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

Answer 9

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

Question 10

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

Answer 10

Severe pain and fever

Question 11

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

Answer 11

Trait or anemia

Question 12

What is the definitive test for sickle cell?

Answer 12

Hgb electrophoresis

Question 13

Sickle cell: What is the Hgb electrophoresis test?

Answer 13

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

Question 14

Sickle cell: Ophthalmic complications. What does this mean

Answer 14

• Vitreous hemorrhage
• Retinal detachment
• Blindness

Question 15

Sickle cell: What is a vaso-occlusive crisis?

Answer 15

CVA
Chest syndrome
Dactylitis
Painful episodes

Question 16

Sickle cell: Vaso-occlusive crisis

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

Answer 16

They probably will

It can cause life threatening issues

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

Question 17

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

Answer 17

Hand and foot syndrome (dactylitis)

Question 18

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

Answer 18

Painful episodes occuring in joints and limbs

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

Question 19

What are some other complications of sickle cell disease?

Answer 19

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

Question 20

What is a main issue of sickle cell disease?

Answer 20

Enlarged spleen

Question 21

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

Answer 21

Treat with blood transfusion

Can cause anemia, hypovolemic shock

Question 22

SCD: What is the role of the spleen?

Answer 22

Filters out infection in the body

Question 23

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

Answer 23

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

Question 24

What is sickle cell crisis triggered by?

Answer 24

Viral infection or depletion of folic acid

Signs: profound anemia and pallor

Question 25

What is aplastic crisis of SCD caused by?

Answer 25

Human parvo virus leading to lowered Hb, retake count

Question 26

SCD: How is aplastic crisis treated?

Answer 26

Observation and transfusion

Question 27

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

Answer 27

TRUE Stroke affects 10% of children with sickle cell

Question 28

What do we do to treat the SCD complications?

Answer 28

- IV hydration - Analgesia: morphine - Transfusion/transplants

Question 29

When is RBC transfusion used for sickle cell complications?

Answer 29

Aplastic, hyperhemolytic, and splenic sequestration crisis

Question 30

SCD: Avoid heat or cold?

Answer 30

COLD!!!

Question 31

What is treatment for FE overload?

Answer 31

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

Question 32

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

Answer 32

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

Question 33

Hemophilia A is a deficiency in factor ?

Answer 33

Factor VIII defiency "classic hemophilia"--80% of cases

Question 34

Hemophilia B is a deficiency of what factor?

Answer 34

Factor IX "Christmas disease"

Question 35

Hemophilia: What is there difficulty controlling bleeding?

Answer 35

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

Question 36

Hemophilia: When may difficulities become apparent?

Answer 36

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

Question 37

Hemophilia: What are s/s?

Answer 37

``` Active bleeding Hematomas/bruising Hemarthrosis Headache Slurred speech Decreased LOC ```

Question 38

Hemophilia: Avoid hot or cold?

Answer 38

AVOID HOT!

Question 39

What are some tests for hemophilia?

Answer 39

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

Question 40

Hemophilia: Ok to take rectal temps?

Answer 40

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

Question 41

What are Factor VIII and IX ?

Answer 41

Clotting factors necessary to begin clotting cascade

Question 42

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

Answer 42

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

Question 43

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

Answer 43

Hemoarthritis

Question 44

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

Answer 44

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

Question 45

Hemophilia: How do we treat prolonged bleeding to joints?

Answer 45

Must treat immediaely! RICE Active ROM after bleeding controlled Maintain ideal weight to avoid stress to joints Encourage regular exercise and PT

Question 46

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

Answer 46

Pretreat with their specific factor (VIII or IX)

Question 47

Hemophilia: How do we prevent bleeding at home?

Answer 47

Padded crib

Question 48

Hemophilia: How do we prevent bleeding for toddlers?

Answer 48

Environment free of clutter--padding corners of furniture Dress toddlers in extra layers of clothing to provide padding

Question 49

Hemophilia: How do we prevent bleeding for preschool?

Answer 49

Supervision/diversion

Question 50

Hemophilia: How do we prevent bleeding in school aged?

Answer 50

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

Question 51

Hemophilia basics--Whats important for client educatin?

Answer 51

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

Question 52

Why is iron deficiency anemia common in infancy?

Answer 52

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

Question 53

Why are adolescents at risk for iron deficiency anemia?

Answer 53

Poor diets Menses Obesity

Question 54

How should we administer iron for iron deficiency anemia?

Answer 54

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

Question 55

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

Answer 55

Through a straw *brush teeth to prevent staining

Question 56

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

Answer 56

Stools with be tarry green--this is normal

Question 57

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

Answer 57

z-track method--do not massage afterward!

Question 58

What is epistaxis?

Answer 58

Nose bleed

Question 59

How do you stop a nose bleed?

Answer 59

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

Question 60

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

Answer 60

Disseminated intravascular coagulation (DIC)

Question 61

What are the hallmarks of DIC?

Answer 61

Bleeding and clotting

Question 62

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

Answer 62

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

Question 63

S/S of DIC?

Answer 63

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

Question 64

Therapeutic management for DIC?

Answer 64

Control of underlying or imitating cause which usually stops problem spontaneously Platelets and fresh frozen plasma may be needed