T3 - Blueprint 2 (Josh)

Question 1

Hallmarks of DIC are – and –

Answer 1

bleeding

clotting

Question 2

What are the Vaso-occlusive complicatons w/ SCD?

Answer 2

Stroke

Pain assoc. w/ obstructed blood flow and ischemia

Question 3

Why is the Spleen effected by SCD?

Answer 3

Splenic sequestration caused by pooled blood, which enlarges the spleen significantly.

Treat with blood transfusion.

Question 4

Patho of DIC

Answer 4

When the clotting process gets started abnormally. Thrombin is made faster than body can neutralize it.

This is bad because the child is susceptible to uncontrollable hemorrhage into vital organs.

Question 5

Why is iron deficiency anemia common in infancy?

Answer 5

cow’s milk too soon

Question 6

Hemophilia A and B are X-linked recessive disorders which means that – are more likely to get it.

Answer 6

boys

• remember the Punnet Square

Question 7

Pathophysiology of Kawasaki Disease

Answer 7

inflammation in the walls of medium-sized arteries throughout the body (including the coronary arteries, which supply blood to heart muscle)

Question 8

What is the definitive test for SCD?

Answer 8

Hbg Electrophoresis

• mearsures the various HGBs
• differentiates b/t trait and anemia

Question 9

— is a secondary disorder of coagulation that occurs as a complication of a number of pathologic processes.

Answer 9

DIC (Disseminated Intravascular Coagulation)

Question 10

What is the analgesia of choice for SCD pain?

Answer 10

IV morphine

Question 11

Why is Kawasaki Disease serious?

Answer 11

can lead to MI

Question 12

S/S of RHD:

What is Erythema Marginatum

Answer 12

Classic eruption of RF

Flat, macular, circular, distinct wavy border (chicken wire–no itching) on trunk or arms

Question 13

Prevention of RHD?

Answer 13

uses ASPIRIN for polyarthitits (inflammation f joints)

Question 14

Hemophilia — is more common and is a deficiency in —

Answer 14

A (80% of cases)

Factor VIII

Question 15

Stopping a nose bleed

Answer 15

sit up and slightly lean forward

pinch bridge of nose–hold about 5 minutes;

*****longer if hemophilia

Question 16

What is the drug of choice to fight infection w/ SCD clients?

Answer 16

Penicillin

Question 17

What is one of the first S/S of Vaso-occlusive complications w/ SCD?

Answer 17

Hand and Foot Syndrome (dactylitis)

• sausage finger
• inflammation of an entire digit (finger/toe)

Question 18

SCD problems:

Aplastic Crisis is caused by — and leads to — and —-

Answer 18

human parvo virus

lowered Hgb

lethargy

Question 19

S/S of RHD:

What is SubQ Nodules?

Answer 19

Small, hard, nontender swellings

Contain Aschoff bodies

Question 20

What are the Ophthalmic complications with SCD?

Answer 20

hemmhorage and retinal detachment that can lead to blindness

Question 21

S/S of RHD:

What is Sydenhams Chorea?

Answer 21

sudden, aimless, purposeles movements

• relieved by rest and sleep

Question 22

RICE treatment for Hemophila

Answer 22

Rest

Ice

Compression

Elevation

Question 23

Prevention for Hemophilia clients:

Answer 23

padded crib

clutter-free (tripping)

soft bristle toothbrushes

immunizations

RICE (rest, ice, compression, elevation)

Question 24

Screening tests for Hgb are limited because…

Answer 24

they are not specific for trait or anemia

Question 25

--- is systematic vasculitis.

Answer 25

Kawasaki Disease

Question 26

SCD is an --- disease with an average lifespan of ---

Answer 26

inherited 45 years

Question 27

Hgb count for Iron Deficiency Anemia.

Answer 27

6-11 g/dL

Question 28

What can be given to increase clotting w/ Hemophilia clients?

Answer 28

Factor VIII and Factor IX via port-a-cath at home

Question 29

S/S of Kawasaki Disease

Answer 29

High Fever Red Eyes Ring around Iris Strawberry Tongue Rash (skin peels off in flakes)

Question 30

S/S of Endocarditis

Answer 30

Low grade, intermittent fever Malaise Joint Pain (arthralgia) New Murmur

Question 31

In Endocarditis, what happens tot the heart valve?

Answer 31

infection/inflammation makes it stenotic - typically Mitral Valve

Question 32

SCD clients will likely need a --- due to severe pain.

Answer 32

PCA pump

Question 33

Jones Criteria (Major/Minor Manifestations of Rheumatic Heart Disease) ***memorize***

Answer 33

Major: - Carditis - Polyarthritis - Sydenham's Chorea - Erythema Marginatum - SubQ Nodules Minor: - Fever - Arthalgia - Elevated Acute Phase Reactants - Prolonged PR Interval

Question 34

Complications from SCD

Answer 34

Ophthalmic Complications Vaso-occlusive Crisis Cardiomegally Abd. Pain and GI dysfunction ENLARGED SPLEEN Stroke

Question 35

Enlarged Spleen (caused by SCD) can lead to...

Answer 35

Anemia Hypovolemic Shock INFECTION

Question 36

Normal RBCs live about -- Sickled RBCs live about --

Answer 36

3 mths 1 mth

Question 37

What is the cure for SCD?

Answer 37

nothing there is no cure, only treat symptoms - controlling anemia - relieving pain

Question 38

In treating Sickle Cell Disease (SCD), what is key?

Answer 38

Hydration and Oxygenation

Question 39

--- is a symptom of Rheumatic Fever.

Answer 39

Endocarditis - bacterial infection/inflammation of heart valves and inner lining

Question 40

What should we avoid w/ Hemophilia clients unless absolutely necessary?

Answer 40

Rectal temp Skin puncture

Question 41

Normal Hgb is ---. With SCD, Hbg is ---

Answer 41

Normal = 11 gm/dL SCD = 2-5 gm/dL

Question 42

S/S of Hemophilia

Answer 42

Active bleeding Hematomas Hemarthrosis Headache Slurred Speech Decreased LOC

Question 43

With Hemophilia, why avoid a Hot/Warm compress?

Answer 43

increases bleeding to the site!

Question 44

S/S of DIC

Answer 44

Petechiae Purpura Bleeding from openings in venipuncture site and surgical incision Bleeding from umbilicus or trachea (newborn) Bleeding evidence in GI Hypotension Organ dysfunction from infarction and ischemia

Question 45

Why is infection a problem with SCD?

Answer 45

Spleen filters out infection Spleen is one of the major organs affected by SCD due to blood sequestering in the organ

Question 46

What is the key sign of Hemophilia?

Answer 46

hemoarthritis - treat immediately - rest, immobilize, elevate, supply ice

Question 47

Treatment for Kawasaki Disease

Answer 47

High dose of IVIG and Salicylate therapy ASPIRIN

Question 48

How common is stroke w/ SCD children?

Answer 48

10%

Question 49

Nursing considerations when giving PO Iron.

Answer 49

If tolerated, give on empty stomach (with juice NOT MILK) Give with meals and start with reduced dose and gradually increase if GI disturbance occurs Give with Vitamin C to increase absorption Use straw to prevent staining of teeth Stools will be tarry green--this is normal Brush teeth after dose to minimize/prevent staining

Question 50

What helps keep SCD kids healthy?

Answer 50

``` good nutrition (folic acid) - (though they can eat normal food) ``` ****important b/c even a little cold can make them very ill

Question 51

Sickle Cell Crisis is triggered by --- or --- and signs include --- and ---

Answer 51

viral infection depletion of folic acid profound anemia pallor

Question 52

--- -- -- follows strep when it is not treated properly.

Answer 52

Rheumatic Heart Disease

Question 53

Treatment for Endocarditis

Answer 53

Prophylaxis Antibiotics (Penicillin best) - especially BEFORE dentistry

Question 54

In Kawasaki Disease, dilation of the Aorta from inflammation leads to ---

Answer 54

aneurysm