Anemia part 2

Question 1

What is the main issue with Hemophilia?

Answer 1

It is a disease where you lack the ability to clot due to not enough coagulation proteins

Question 2

How is hemophilia passed down?

Answer 2

Familial in nature and genetically passed down in females

Question 3

What fraction of hemophilia is from gene mutation though?

Answer 3

1/3 from mutation

Question 4

Types of hemophilia?

Answer 4

Hemophilia A

Hemophilia B

Question 5

Explain Hemophilia A

Answer 5

Deficiency in factor 8

- the most common

Question 6

Explain Hemophilia B

Answer 6

Deficiency of factor 9

Question 7

Where are factors produced?

What is the patho of the factors?

Answer 7

In the liver. And those with hemophilia do have some factors just not enough.

Factors should release in order to get to fibrinogen. If there’s no factor = no fibrinogen or clotting

Question 8

Signs and symptoms of Hemophilia
Joints?

What will clotting factors test be like

Answer 8

Bruising easily
Bleeding too much
- can be internal or external

Also Hemarthorosis
- stiff, achy, red, edema, and loss of function of joints

Clotting factor test will be abnormal

Question 9

What replacement can help treat hemophilia?

Where can they get treatment?

Answer 9

Replacing the factor 8 or 9

• can either give the actual factor
• or give plasma w that factor capability

Can do it in home for convenience

Question 10

What is factor replacement treatment like with those who have mild hemophilia?

What if it is moderate to severe hemophilia?

Answer 10

Mild - receive infusion at time of bleeding incident

Moderate or Severe - prophylaxis infusions

Question 11

What hormone replacement can help treat hemophilia?

And which route?

Answer 11

Vasopressin (ADH) can increase the factors
- but this doesn’t work for everyone

Oral and nasal route of ADH can treat mild cases

Question 12

What medication can be given for hemophilia to make sure clots aren’t dissolving?

Answer 12

You would first give the factor dose but then give Amicar

- especially anytime before a surgery or procedure

Question 13

What can you substitute the factors with if someones body doesn’t recognize the proteins?

Answer 13

Can give Vlla or activated prothrombin complex concentrate

Question 14

What can help with joint inflammation or Hemarthorosis that comes with hemophilia?

Answer 14

Corticosteroids can be used for the inflammation

Question 15

In hemophilia, what do we need to educate parents about?

Answer 15

Educate parents about not letting their child do dangerous activities or ones that may make them bleed.
- instead of playing football, have them do cross country.

Question 16

What medications should those with hemophilia avoid? And why?

Answer 16

They need to avoid aspirin and nsaids.

They thin the blood and can increase the amount they bleed even more.

Question 17

Nursing care for Hemophilia acronym

Answer 17

Factor replacement

Rest

Immobilize so you don’t dislodge clots and ice

Compression

Elevation

Question 18

How expensive is factor replacement?

Is this an issue?

Answer 18

You pay per factor so very expensive.

Children get state health care but when they become an adult, this can be a problem.

Question 19

What is Idiopathic Thrombocytopenia Purpura or ITP

Answer 19

Platelets are being destroyed which causes red poetical dots on the skin
- platelets are usually under 100k but can go lower

Question 20

What is the trigger of ITP or idiopathic thrombocytopenia purpura?

Answer 20

The platelets being destroyed is usually due to an autoimmune response that was triggered by an illness of some sort

• may not even be an obvious correlation
• usually from upper respiratory, human parvo, or varicella

Question 21

What education should we give with ITP?

Answer 21

Try to avoid dangerous activities here too

Question 22

What will be done if platelets get too low with ITP?

Answer 22

A transfusion

Question 23

Are there different types of ITP?

Answer 23

Either acute or chronic

Question 24

What types of treatment are given for acute ITP?

Answer 24

Try to treat trigger if possible but next best thing is supportive tx

• restrict activity
• IVIG IG to lower immune response
• Anti-D antibody (but just know a hemodilution like anemia will occur but platelets will rise around 48 hours after
• Prednisone to see if reducing immune response stops the trigger
• Can do a transfusion of platelets if count gets too low

Question 25

What types of treatment will be used for chronic ITP?

Answer 25

Above all try to treat the trigger if possible Can do a splenectomy if over 5 years old and not responding to other therapy since their spleen will be damaged already and that just makes immunity worse Earlier immunizations but NO live viruses whatsoever

Question 26

With ITP should you use Nsaids (ibuprofen)?

Answer 26

No - use Tylenol instead.

Question 27

What is Disseminated Intravascular Coagulation or DIC

Answer 27

When micro clots and excessive bleeding are occurring at the same time

Question 28

What triggers the abnormal clotting in DIC?

Answer 28

``` Hypoxia Endothelial injury like burns Acidosis Shock Severe systemic diseases ```

Question 29

What is the order of the clotting cascade with DIC?

Answer 29

Trigger > more thrombin > more fibrinogen > fibrin

Question 30

What is the patho of DIC?

Answer 30

Accelerated clotting > micro clots in vessels > tissue ischemia > loss of organ function and limbs > fibrinolytic mechanism activated - and then you have the break down of clots leading to bleeding)

Question 31

What risks can occur with DIC due to the clots in the vessels?

Answer 31

stroke or MI or anything that occurs when blood flow is occluded

Question 32

How to control bleeding in DIC?

Answer 32

Plasma factor- replacement is an option Cryoprecipitate - accumulated antibody given to stop autoimmune responses Platelet transfusion - done only if count is super low but do so cautiously Fibrinogen - can be given but just depends on situation

Question 33

In DIC , will they give heparin to prevent the clotting?

Answer 33

Controversial. If they prevent the clotting, that person can bleed.

Question 34

Things to monitor with people who have DIC?

Answer 34

Monitor vitals, O2, and fluids if in the hospital Illness should be monitored for so check temp. Check their limbs for signs of problems. Don't want any amputations

Question 35

Should we use nitroglycerin paste for those with DIC?

Answer 35

Well it dilates vessels to help with any obstruction from clots BUT it won't dissolve the clot. It'll just let the clot keep going. Wouldn't we rather let the clot form and know where it is in the body? Rather than have to guess where it is?

Question 36

Basic instructions for Pediatric CPR

Answer 36

Push hard and fast Minimize interruptions (10-15 sec max for pulse check) Full chest recoil Don't hyperventilate

Question 37

For CPR , there's one rescuer. What is the ratio How long should all respirations be?

Answer 37

30 compressions ; 2 breaths Respirations should be 1 sec - no matter how many rescuers. You only need 10 per minute

Question 38

We have two rescuers for CPR. What is the ratio How often should you rotate jobs

Answer 38

15 compressions ; 2 breaths Rotate every 2-3 minutes

Question 39

How many compressions should be given per minute

Answer 39

100 compressions/minute

Question 40

Depth of compression for older kids? Depth of compression for infants and young children?

Answer 40

1. 5 - 2 inches | 0. 3 - 0.5 of the AP diameter

Question 41

When a child goes into cardiac arrest - what is the cause

Answer 41

Respiratory issue & then they fail to compensate leading to the cardiac arrest

Question 42

How old does an infant have to be to use the AED? Will an aed shock someone back to life?

Answer 42

1 years old at least No. If a person is dead meaning no electrical activity going on in the heart, there is nothing you can do. - there must always be some sort of electrical activity left to work

Question 43

What part of your body do you use when doing compressions for infants? For older children?

Answer 43

Use your thumbs while supporting them with your hands (sort of hold them like your reading a book) Older children - use one hand

Question 44

If there is a code going on, what should someone try to establish? What if it is hard to establish?

Answer 44

IV access. Use intraosseous site if it is hard to establish Insertion device may be available - driven by facility policy

Question 45

If it comes down to using an endotracheal kit or bag and mask in CPR, what should you pick

Answer 45

Bag and mask