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PEDS Unit 3 > Hematological Disorders > Flashcards

Hematological Disorders Flashcards

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
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2
Q

What is the most common hematological disorder of childhood?

A

Anemia

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3
Q

What is anemia?

A
  • Decrease in number of RBCs

or

  • Decrease in hemoglobin concentration
  • Result in either case is poor tissue perfusion
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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
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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
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6
Q

What are the basic s/s of anemia?

A
  • Fatigue
  • Pallor (pale)
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7
Q

What are some risk factors for Iron-Deficency Anemia?

A
  • Diet high in milk
  • Poor dietary intake of iron
  • Blood loss
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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
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9
Q

What is a negative effect of cow’s milk on the GI system?

A
  • it can cause microbleeds
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10
Q

Why should toddlers eat BEFORE they get their milk?

A
  • So they get their nutrients from food before filling up on milk
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11
Q

What is the max amount of daily milk for toddlers and teens?

A
  • Toddlers = ≤ 32oz
  • Teens = ≤ 3 cups (24oz)
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12
Q

What is the treatment for iron-deficiency anemia?

A
  • Treat the underlying cause
  • Educate the family
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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
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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!
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15
Q

What is the milk rule regarding iron supplements?

A

None for an hour or two before and after meal

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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!
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17
Q

What is Aplastic Anemia?

A
  • Bone marrow failure!
  • Pancytopenia with triad of
    • Anemia
    • Thrombocytopenia
    • Leukopenia
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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
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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
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20
Q

What is the medical treatment for Aplastic anemia?

A
  • Immunosuppression if autoimmune cause is suspected
  • Bone marrow transplant
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21
Q

What are the key points regarding bone marrow transplant?

A
  • Transplant (better success rate the earlier its done)
  • 85% survival rate
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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
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23
Q

If both parents have the sickle cell anemia trait, what are the chances each child will have of getting it?

A

25%

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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
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25
Q

When sickled cells stick together and create blockages, what happens?

A
  • Tissue hypoxia
  • Infarction (tissue death)
  • Increased stress which causes even more sickling
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26
Q

SCA exacerbations can result in what 3 things?

A
  • Vaso-occlusive crisis
  • Sequestration crisis
  • Infection
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27
Q

Describe the vaso-occlusive crisis of SVA.

A
  • 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
Q

Describe the sequestration crisis of SVA.

A
  • crisis will clog the spleen or liver causing blood to pool in the organ and can cause vascular collapse
29
Q

What is sometimes done to prevent a sequestration crisis of the spleen from occuring.

When/why else would this be done?

A
  • 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
Q

What happens as a result of a splenectomy?

A

It leaves the pt @risk for infections

31
Q

What is the treatment for SCA?

A
  • Treatment aimed at reduce sickling
  • Teach family stressors to avoid
  • Blood transfusions/ exchange transfusion in severe cases
32
Q

In some blood transfusions for SCA, why would Desferal/deferoxamine be given?

A
  • Pt may have high iron serum levels due to xfusions.
  • Desferal gets rid of excess.
  • Must be infused over several hours
33
Q

Blood xfusions can decrease sckling episodes. Which pts may need them every 3 wks for the rest of their lives?

A
  • Those with a history of CVA or ACS
34
Q

What is important to keep in mind regarding hydration and a pt in SVA crisis?

A
  • Give them 1.5x’s normal maintenance dose to help clear the blockages
35
Q

Pain is a huge issue with SCA. What are the key points regarding medication?

A
  • 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
Q

What is our education to parents who are concerned about their child getting addicted to opioids?

A
  • behavioral addiction is incredibly rare
37
Q

How is Beta Thalassemia, “Cooley’s Anemia” acquired?

A

inherited from parents

38
Q

What is happening in Beta Thalassemia?

A
  • 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
Q

How does Beta Thalassemia affect oxygenation?

A

Constant creation and destruction of RBCs and not enough oxygen-carrying hemoglobin to serve the body well

40
Q

Simply put, what is Beta Thalassemia and what does the process lead to?

A
  • Constant descruction and production of RBCs result in poor perfusion
  • Process leads to
    • Brittle bones, hepatosplenomegaly from the process
    • Delayed growth
    • Facial deformities
41
Q

What are the key points of Hemophilia to keep in mind?

A
  • 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
Q

What are the key points to know for Von Willebrand Disease?

A
  • Type of Hemophilia
  • VW protein helps for clots
  • VW Disease results in not enough or dysfunctional VW protein which leads to excessive bleeding
43
Q

What is the treatment of Von Willebrand Disease?

A
  • Goal: minimize episodes of bleeding
  • Tx: DDAVP (desmopressin), which stimulates the release of VW factor
44
Q

Symptoms of hemophilia may not occur until what age?

A

6mos

45
Q

What are some s/s of hemophilia?

A
  • Frequent and larger bruising and nosebleeds
  • Hematuria
46
Q

What is the challenge with pts with hemophilia?

A
  • Internal, non-visible bleeding
  • Need to know the s/s
47
Q

What is most important regarding a head injury for a hemophilic pt?

A

Monitor for ICP

48
Q

What is Hemarthrosis and what are the s/s?

A
  • Bleeding into joint spaces
  • S/s include warmth, pain, bruising, and decreased movement
49
Q

What is a target joint?

A
  • Hemarthrosis in the same joint on 4 different occasions w/in 6 months
50
Q

What is the treatment for Hemarthrosis?

A
  • 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
Q

What is the treatment for Hemophilia?

A
  • 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
Q

What do we educate re: activity for our pts with hemophilia.

A
  • physical activity is important, they just need to be safe
  • No contact source
53
Q

How does weight relate to hemarthrosis?

A
  • Too much weight caues xtra stress on joints and increases incidence of hemarthrosis
54
Q

What hygeine warnings do we give for pts with hemophilia?

A
  • soft toothbrush
  • No metal razors
55
Q

TRUE or FALSE

Aspirin is ok for people with hemophilia.

A

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
Q

What is Idiopathic Thrombocytopenic Purpura (ITP)?

A
  • An aquired hemorrhagic disorder that is characterized by:
    • Thrombocytopenia (destruction of of platelets - PLT<20K
    • Purpura (advanced peticea)
    • NORMAL bone marrow
57
Q

The cause of ITP is unknown, but it usually follows after a ….

A

viral infection

58
Q

What is the treatment for ITP?

A
  • IVIG
  • Prednisone - to help body settle down and minimize platelet destruction
59
Q

What is IVIG

A
  • A super-concentrated and very diverse collection of antibodies against many possible infectious organisms your body might encounter.
  • Administered via IV
60
Q

Explain Acute vs Chronic ITP.

A
  • Acute usually follows a viral infection and is self limiting
  • Chronic will also resolve itself but will last 12 months or longer
61
Q

What is a sign of DIC, why is it so important to recognize and how is it treated?

A
  • 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
Q

Overstimulation of clotting has 4 major effects. What are they?

A
  • Bleeding
  • Organ damage
  • Anemia
  • Tissue hypoxia
63
Q

All kids should be screened for lead poisoning by what age?

A

12mos

64
Q

What are the CNS s/s of lead poisoning?

A
  • Hyperactivity,
  • impulsiveness,
  • lethargy,
  • irritability,
  • loss of developmental progress,
  • hearing impairment,
  • learning difficulties
65
Q

What is the treatment for lead poisoning and how does it work?

A
  • Chelation therapy
  • Uses special drugs that bind to metals in your blood and is then excreted through urine

PEDS Unit 3 (9 decks)

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