Anemia part 2 Flashcards
What is the main issue with Hemophilia?
It is a disease where you lack the ability to clot due to not enough coagulation proteins
How is hemophilia passed down?
Familial in nature and genetically passed down in females
What fraction of hemophilia is from gene mutation though?
1/3 from mutation
Types of hemophilia?
Hemophilia A
Hemophilia B
Explain Hemophilia A
Deficiency in factor 8
- the most common
Explain Hemophilia B
Deficiency of factor 9
Where are factors produced?
What is the patho of the factors?
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
Signs and symptoms of Hemophilia
Joints?
What will clotting factors test be like
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
What replacement can help treat hemophilia?
Where can they get treatment?
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
What is factor replacement treatment like with those who have mild hemophilia?
What if it is moderate to severe hemophilia?
Mild - receive infusion at time of bleeding incident
Moderate or Severe - prophylaxis infusions
What hormone replacement can help treat hemophilia?
And which route?
Vasopressin (ADH) can increase the factors
- but this doesn’t work for everyone
Oral and nasal route of ADH can treat mild cases
What medication can be given for hemophilia to make sure clots aren’t dissolving?
You would first give the factor dose but then give Amicar
- especially anytime before a surgery or procedure
What can you substitute the factors with if someones body doesn’t recognize the proteins?
Can give Vlla or activated prothrombin complex concentrate
What can help with joint inflammation or Hemarthorosis that comes with hemophilia?
Corticosteroids can be used for the inflammation
In hemophilia, what do we need to educate parents about?
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.
What medications should those with hemophilia avoid? And why?
They need to avoid aspirin and nsaids.
They thin the blood and can increase the amount they bleed even more.
Nursing care for Hemophilia acronym
Factor replacement
Rest
Immobilize so you don’t dislodge clots and ice
Compression
Elevation
How expensive is factor replacement?
Is this an issue?
You pay per factor so very expensive.
Children get state health care but when they become an adult, this can be a problem.
What is Idiopathic Thrombocytopenia Purpura or ITP
Platelets are being destroyed which causes red poetical dots on the skin
- platelets are usually under 100k but can go lower
What is the trigger of ITP or idiopathic thrombocytopenia purpura?
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
What education should we give with ITP?
Try to avoid dangerous activities here too
What will be done if platelets get too low with ITP?
A transfusion
Are there different types of ITP?
Either acute or chronic
What types of treatment are given for acute ITP?
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
What types of treatment will be used for chronic ITP?
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
With ITP should you use Nsaids (ibuprofen)?
No - use Tylenol instead.
What is Disseminated Intravascular Coagulation or DIC
When micro clots and excessive bleeding are occurring at the same time
What triggers the abnormal clotting in DIC?
Hypoxia Endothelial injury like burns Acidosis Shock Severe systemic diseases
What is the order of the clotting cascade with DIC?
Trigger > more thrombin > more fibrinogen > fibrin
What is the patho of DIC?
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)
What risks can occur with DIC due to the clots in the vessels?
stroke or MI or anything that occurs when blood flow is occluded
How to control bleeding in DIC?
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
In DIC , will they give heparin to prevent the clotting?
Controversial. If they prevent the clotting, that person can bleed.
Things to monitor with people who have DIC?
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
Should we use nitroglycerin paste for those with DIC?
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?
Basic instructions for Pediatric CPR
Push hard and fast
Minimize interruptions (10-15 sec max for pulse check)
Full chest recoil
Don’t hyperventilate
For CPR , there’s one rescuer. What is the ratio
How long should all respirations be?
30 compressions ; 2 breaths
Respirations should be 1 sec - no matter how many rescuers. You only need 10 per minute
We have two rescuers for CPR. What is the ratio
How often should you rotate jobs
15 compressions ; 2 breaths
Rotate every 2-3 minutes
How many compressions should be given per minute
100 compressions/minute
Depth of compression for older kids?
Depth of compression for infants and young children?
- 5 - 2 inches
0. 3 - 0.5 of the AP diameter
When a child goes into cardiac arrest - what is the cause
Respiratory issue & then they fail to compensate leading to the cardiac arrest
How old does an infant have to be to use the AED?
Will an aed shock someone back to life?
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
What part of your body do you use when doing compressions for infants?
For older children?
Use your thumbs while supporting them with your hands
(sort of hold them like your reading a book)
Older children - use one hand
If there is a code going on, what should someone try to establish?
What if it is hard to establish?
IV access.
Use intraosseous site if it is hard to establish
Insertion device may be available
- driven by facility policy
If it comes down to using an endotracheal kit or bag and mask in CPR, what should you pick
Bag and mask
