Hematological Disorders Flashcards
(65 cards)
1
Q
What is epitaxis and what is its treatment?
A
- Nose bleed
- Lean forward
- Pinch nose for 10mins
- Petroleum jelly can be used to moisten nares and prevent recurrence
2
Q
What is the most common hematological disorder of childhood?
A
Anemia
3
Q
What is anemia?
A
- Decrease in number of RBCs
or
- Decrease in hemoglobin concentration
- Result in either case is poor tissue perfusion
4
Q
Relative hemoglobin levels for adult males and females?
A
- M = 14-18
- F = 12-15
- # s will vary by sources
- Children’s #s will be lower
5
Q
TRUE or FALSE
The body cannot adapt to anemia.
A
FALSE
- If anemia ocurs slowly, the body can adapt
- ex. Move to denver from a low elevation
6
Q
What are the basic s/s of anemia?
A
- Fatigue
- Pallor (pale)
7
Q
What are some risk factors for Iron-Deficency Anemia?
A
- Diet high in milk
- Poor dietary intake of iron
- Blood loss
8
Q
Why is too much milk a bad thing?
A
- It binds to iron and causes it to get excreted from the body reducing serum levels
9
Q
What is a negative effect of cow’s milk on the GI system?
A
- it can cause microbleeds
10
Q
Why should toddlers eat BEFORE they get their milk?
A
- So they get their nutrients from food before filling up on milk
11
Q
What is the max amount of daily milk for toddlers and teens?
A
- Toddlers = ≤ 32oz
- Teens = ≤ 3 cups (24oz)
12
Q
What is the treatment for iron-deficiency anemia?
A
- Treat the underlying cause
- Educate the family
13
Q
What is our supportive care for severe cases of iron deficiency anemia?
A
- Intravenous (IV) fluids to replace intravascular volume
- Transfusion may be needed
- Oxygen
- Bed rest
14
Q
What are the items to remember regarding iron supplements?
A
- Liquid admin for kids - tastes terrible
- stains teeth so give with straw and
- brush teeth immediately after
- Tablets for older kids
- Take supplements w/ vitamin C to help absorption!
15
Q
What is the milk rule regarding iron supplements?
A
None for an hour or two before and after meal
16
Q
What do we need to keep in mind for safety regarding iron supplements?
A
- IRON is extremely easy to overdose for kids
- Should be treated as a dangerous drug out of reach (even though its OTC)
- Hide grandmas iron pills!
17
Q
What is Aplastic Anemia?
A
- Bone marrow failure!
- Pancytopenia with triad of
- Anemia
- Thrombocytopenia
- Leukopenia
18
Q
Explain pancytopenia, each component and its s/s.
A
- Pancytopenia is the destruction of RBCs, WBCs and Thrombocytes (platelets)
- RBC destruction leads to poor oxygen perfusion
- s/s of hypoxia
- WBC destruction leads to susceptibility to infection
- s/s of poor immune response
- Platelet destruction leads to low clotting factor
- s/s unsual bleeding
19
Q
What is the cause of aplastic anemia?
A
We don’t know. It is idopathic.
However, some cases that have caused:
- Overwhelming infection
- Radiation
- Industrial/household cleaners
20
Q
What is the medical treatment for Aplastic anemia?
A
- Immunosuppression if autoimmune cause is suspected
- Bone marrow transplant
21
Q
What are the key points regarding bone marrow transplant?
A
- Transplant (better success rate the earlier its done)
- 85% survival rate
22
Q
What happens if you wait on a bone marrow transplant for aplastic anemia, what happens?
A
- reduces survival right to 70% as the child would to the human leucocyte antigen (HLA)
- body may now see it as “this isn’t me” and reject the new marrow
23
Q
If both parents have the sickle cell anemia trait, what are the chances each child will have of getting it?
A
25%
24
Q
What are the key points to keep in mind regarding sickle cell anemia?
A
- sickled cells can’t carry enough 02, leading to decreased perfusion
- sickled cells can stick together and cause blockages
25
When sickled cells stick together and create blockages, what happens?
* Tissue hypoxia
* Infarction (tissue death)
* Increased stress which causes even more sickling
26
SCA exacerbations can result in what 3 things?
* Vaso-occlusive crisis
* Sequestration crisis
* Infection
27
Describe the vaso-occlusive crisis of SVA.
* sickled cells cause blockages that result in a painful episode from hypoxia
* Leads to
* Acute Chest Syndrome (similar to pneumonia)
* crisis clogs chest
* leading cause for death in SCA
* CVA (Cerbrovascular Accident - *stroke*)
* Crisis clogs brain
* Infection
28
Describe the sequestration crisis of SVA.
* crisis will clog the spleen or liver causing blood to pool in the organ and can cause vascular collapse
29
What is sometimes done to prevent a sequestration crisis of the spleen from occuring.
When/why else would this be done?
* A splenectomy would be done to prevent the sequestration crisis
* This would also be done with a spleen that has been shredded by SCA
30
What happens as a result of a splenectomy?
It leaves the pt @risk for infections
31
What is the treatment for SCA?
* Treatment aimed at reduce sickling
* Teach family stressors to avoid
* Blood transfusions/ exchange transfusion in severe cases
32
In some blood transfusions for SCA, why would Desferal/deferoxamine be given?
* Pt may have high iron serum levels due to xfusions.
* Desferal gets rid of excess.
* Must be infused over several hours
33
Blood xfusions can decrease sckling episodes. Which pts may need them every 3 wks for the rest of their lives?
* Those with a history of CVA or ACS
34
What is important to keep in mind regarding hydration and a pt in SVA crisis?
* Give them 1.5x's normal maintenance dose to help clear the blockages
35
Pain is a huge issue with SCA. What are the key points regarding medication?
* want to start meds as soon as possible,
* give PCA if they're old enough
* want them to feel in control
* nsaids, motrin, ibuprofen and opioid med is typical
36
What is our education to parents who are concerned about their child getting addicted to opioids?
* behavioral addiction is incredibly rare
37
How is Beta Thalassemia, "Cooley's Anemia" acquired?
inherited from parents
38
What is happening in Beta Thalassemia?
* Red blood cells hemolyze and become non-functional
* Body makes more RBCs to compensate
* Inside the bone marrow AND
* Outside the bone marrow (extramedullary hemotopoiesis)
39
How does Beta Thalassemia affect oxygenation?
Constant creation and destruction of RBCs and not enough oxygen-carrying hemoglobin to serve the body well
40
Simply put, what is Beta Thalassemia and what does the process lead to?
* Constant descruction and production of RBCs result in poor perfusion
* Process leads to
* Brittle bones, hepatosplenomegaly from the process
* Delayed growth
* Facial deformities
41
What are the key points of Hemophilia to keep in mind?
* X-linked recessive pattern
* Only males get it
* Males/Females can carry
* Factors VIII and IX help create clots
* Don't do things that make ya bleed
42
What are the key points to know for Von Willebrand Disease?
* Type of Hemophilia
* VW protein helps for clots
* VW Disease results in not enough or dysfunctional VW protein which leads to excessive bleeding
43
What is the treatment of Von Willebrand Disease?
* Goal: minimize episodes of bleeding
* Tx: DDAVP (desmopressin), which stimulates the release of VW factor
44
Symptoms of hemophilia may not occur until what age?
6mos
45
What are some s/s of hemophilia?
* Frequent and larger bruising and nosebleeds
* Hematuria
46
What is the challenge with pts with hemophilia?
* Internal, non-visible bleeding
* Need to know the s/s
47
What is most important regarding a head injury for a hemophilic pt?
Monitor for ICP
48
What is Hemarthrosis and what are the s/s?
* Bleeding into joint spaces
* S/s include warmth, pain, bruising, and decreased movement
49
What is a target joint?
* Hemarthrosis in the same joint on 4 different occasions w/in 6 months
50
What is the treatment for Hemarthrosis?
* RICE
* Analgesics
* Nsaids work really well, but need to be careful as nsaids can reduce production of platelets
* ROM exercises after bleeding stops to prevent contractures
* These are done by the pt, not us
* We encourage it when appropriate
* Physical therapy
51
What is the treatment for Hemophilia?
* Primary therapy is replacement of missing clotting factors
* Desmopressin (DDAVP) - for mild cases
* Increases Factor VIII activity
* Transfusions
* if loss of too much blood
* Prompt intervention to reduce complications
* Exercise and physical therapy
* No cure, just control of symptoms
52
What do we educate re: activity for our pts with hemophilia.
* physical activity is important, they just need to be safe
* No contact source
53
How does weight relate to hemarthrosis?
* Too much weight caues xtra stress on joints and increases incidence of hemarthrosis
54
What hygeine warnings do we give for pts with hemophilia?
* soft toothbrush
* No metal razors
55
TRUE or FALSE
Aspirin is ok for people with hemophilia.
FALSE
* Asprin increases risk for bleeding
* We also need to educate the pt that aspirin is also in things like alka-seltzer and pepto bysmal
56
What is Idiopathic Thrombocytopenic Purpura (ITP)?
* An aquired hemorrhagic disorder that is characterized by:
* Thrombocytopenia (destruction of of platelets - PLT\<20K
* Purpura (advanced peticea)
* NORMAL bone marrow
57
The cause of ITP is unknown, but it usually follows after a ....
viral infection
58
What is the treatment for ITP?
* IVIG
* Prednisone - to help body settle down and minimize platelet destruction
59
What is IVIG
* A super-concentrated and very diverse collection of antibodies against many possible infectious organisms your body might encounter.
* Administered via IV
60
Explain Acute vs Chronic ITP.
* Acute usually follows a viral infection and is self limiting
* Chronic will also resolve itself but will last 12 months or longer
61
What is a sign of DIC, why is it so important to recognize and how is it treated?
* Systemic bleeding all over
* Can be internal, external at puncture points, etc
* The next step is death
* Treated by tending to the underlying cause
62
Overstimulation of clotting has 4 major effects. What are they?
* Bleeding
* Organ damage
* Anemia
* Tissue hypoxia
63
All kids should be screened for lead poisoning by what age?
12mos
64
What are the CNS s/s of lead poisoning?
* Hyperactivity,
* impulsiveness,
* lethargy,
* irritability,
* loss of developmental progress,
* hearing impairment,
* learning difficulties
65
What is the treatment for lead poisoning and how does it work?
* Chelation therapy
* Uses special drugs that bind to metals in your blood and is then excreted through urine
