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Flashcards in HEME PART 2 Deck (66)
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1
Q

T or F. Iron deficiency anemia, sickle cell anemia, thalassemia, and hemophila are all childhood hematologic conditions that can either be fixed or treated?

A

True.

2
Q

Normal Hgb for newborns

A

14 to 24 g/dl

3
Q

Normal Hgb for infants

A

10 to 17 g/dl

4
Q

Normal Hgb for children

A

9.5 to 15.5 g/dl

5
Q

Anemia caused by an inadequate supply of dietary iron.

A

Iron deficiency anemia. It is also described as Hgb levels below normal range because of the body’s inadequate supply, intake, or absorption of iron.

6
Q

In regards to Iron, iron deficiency anemia can be caused by any number of factors that ________, ________, _________, or _______?

A

Decrease the supply of iron, impair its absorption, increase the body’s need for iron, or affect the synthesis of Hgb.

7
Q

Name 2 examples that would increase the demand of iron

A

Pregnancy and growth spurts increase volume

8
Q

What can cause a decreased supply of iron?

A

Surgery and poor diet

9
Q

T or F. For iron deficiency anemia, it is important to assess for s/s similar to dehydration?

A

True.

10
Q

Tachycardia and signs of poor oxygen delivery are common signs and symptoms of what condition?

A

Iron deficiency anemia

11
Q

What are common manifestations of poor oxygen delivery?

A

Fatigue, lethargy, SOB, chest pain, ice craving (picophagia), poor cardiac output, respiratory distress (tachypnea, apnea, low O2 sat, poor perfusion, and capillary refill greater than 4 sec

12
Q

What are 6 tips you can use when teaching families about the administration of oral iron supplements?

A

Give on an empty stomach for better absorption, give with citrus juices (vitamin C) to aid in absorption, use dropper or straw to avoid discoloring teeth, teach that stools will become tarry, teach that overdose can be fatal and the importance of keeping iron away from other children, and teach to not give with any dairy products.

13
Q

Why are preterm infants at increased risk for iron deficiency anemia?

A

Because of their reduced fetal iron supply.

14
Q

T or F. Many infants have iron deficiency anemia because of excessive milk ingestion (cow’s milk)?

A

True, because milk hinders the absorption of iron. Milk is a poor source of iron and contributes to a fecal loss of iron in the blood.

15
Q

Baby Christina has been Dx with Iron deficiency anemia. How should this condition be treated?

A

Treatment is mainly focused on increasing the amount of supplemental iron. This is usually done with diet modification (fortified formula or cereal) and oral supplementation (after 6 months and in exclusively breastfed infants).

16
Q

In regards to treatment, why is iron supplementation especially important for exclusively breastfed infants?

A

Because fetal Hgb has a short-life span.

17
Q

In peds patients, what Hgb levels indicate anemia?

A

Low level= 9.5- 13; Moderate= 8- 9.5; Severe-less than 8.

18
Q

T or F. To prevent nutritional anemia, a primary nursing objective is to prevention through family education, especially breastfeeding moms.

A

True. Because breast milk is a poor iron source after 5 months of lactation, the nurse must reinforce the importance of administering iron supplements to the exclusively breastfed infant by 6 months

19
Q

T or F. No evidence that screening in asymptomatic children results in improved health outcomes?

A

TRUE

20
Q

T or F. Evidence supports a substantial decrease in the incidence of anemia in healthy infants as a result of iron supplementation?

A

TRUE

21
Q

T or F. Fair evidence that iron supplementation may improve neurodevelopmental outcomes in children at risk for iron deficiency anemia?

A

TRUE

22
Q

T or F. AAP recommends screening for all infants between the ages of 9-12 months and then 6 months later; for children at high risk, screen once a year from ages 2 to 5 years.

A

TRUE

23
Q

Besides fortified formula and cereal, what are other dietary sources of iron?

A

Meat, green leafy veggies, fish, liver, whole grains, and legumes

24
Q

Baby Christina’s mother wants to help get her daughter better, but shares with you that she doesn?t think that she can afford to buy these iron-rich resources. What important resource should you refer her to?

A

WIC

25
Q

One of a group of diseases collectively termed hemoglobinopathies, in which normal adult Hgb (Hgb A) is partially or completely replaced by abnormal sickle Hgb S)?

A

Sickle Cell Anemia (SCA)

26
Q

At what age does SCA become evident? Why?

A

After 6 months because this is the time when FETAL Hgb starts to decrease and ADULT Hgb starts to take over.

27
Q

What 2 conditions are considered autosomal recessive inherited bleeding disorder?

A

Hemophilia and Sickle Cell Anemia

28
Q

T or F. SCA is an example of autosomal recessive inherited blood disease, meaning BOTH parents MUST be carriers of the recessive trait?

A

True. With each pregnancy, there is a 1 in 4 chance that these parents will have a child with the disease. However, ALL children of these parents can GET the disease, not just 25 percent of them.

29
Q

S/S of SCA are the primary result of 2 problems. What are they?

A

OBSTRUCTION caused by the sickled RBCs and increased RBC DESTRUCTION.

30
Q

What process contributes to the absence of blood flow to adjacent tissues, causing local hypoxia that eventually leads to cellular death?

A

The abnormal adhesion, entanglement, and enmeshing of rigid, sticky, and meshy sickled shaped RBCs with one another that intermittently block the microcirculation, causing vasooclusion.

31
Q

Why do patients with SCA tend to have gestational issues?

A

Because the destruction of RBCs and obstruction caused by the sickled shaped RBCs interfere with peristalsis and movement of the GI tract.

32
Q

When it comes to SCA management, _________ is the most important in regards to helping to identifying triggers to prevent crises.

A

Education

33
Q

T or F. Besides teaching patients with SCA to minimize or avoid triggers, education related to preventing volume depletion (dehydration) is also important?

A

TRUE

34
Q

What are 6 examples of TRIGGERS that can lead to a crisis?

A

Fever, Infection, Emotional stress, Physical stress, Dehydration, Increased oxygen needs

35
Q

What is the number reason for ED visits in patients with SCA?

A

Sickle- cell crisis, also known as Vasooculsiveness or a PAIN episode. Therefore major focus of home management for people with sickle cell disease

36
Q

What serious complications can occur as a result of sickle-cell crisis?

A

Sequestration, Aplastic crisis, Hyperhemolytic crisis, Acute chest syndrome (occlusion in heart or lungs), Stroke

37
Q

To help manage SCA, the majority of patients have a splenectomy. Why?

A

Because the spleen is the main source of sickled cell production. This organ produces RBCs

38
Q

T or F. Patient who undergo a splenectomy are at high risk for infection?

A

True. The risk of bacterial infection is 400x higher in patients with sickle cell anemia and are asplenic.

39
Q

In asplenic patients, what interventions are most important?

A

Start prophylactic penicillin by age 2-3 months, promote vaccines, and administer IV antibiotics for temp over 101 (spikes in temp should NEVER be taken lightly, fever is an EMERGENCY).

40
Q

T or F. 11 percent of patients with SCA anemia will have a stroke by age 20 years, with silent infarction in another 22 percent?

A

TRUE

41
Q

In silent or micro-strokes that occur in patients with SCA, how would you determine if in fact a stroke occurred?

A

Assess for cognitive changes or challenges

42
Q

T or F. Chronic transfusion therapy is required to prevent a second stroke?

A

TRUE

43
Q

As a nurse caring for a patient with SCA, what are things to keep in mind?

A

Provide education to patient and family to help identify triggers, fear of addiction to pain meds, that iron supplement is not recommended, and that folic acid should be given to stimulate RBC synthesis.

44
Q

Why is iron supplementation NOT recommended?

A

Because it is contraindicated in RBC transfusions and the anemia is not caused by iron deficiency. Administration can lead to too much iron, which can be toxic.

45
Q

As a nurse, what is your main role is helping SCA patients manage this disease?

A

Education in regards to triggers (how to identify and avoid them), hydration, and narcotic use.

46
Q

In a sickle cell crisis, _______ is the first intervention to perform, then ________.

A

Fluids; pain management (either by pharmacological or non pharmacologic methods. Other interventions include monitoring I and O, Administering blood products and antibiotics as prescribed, and using a warm compress.

47
Q

T or F. The median life expectancy is 35 years for patients with SCA?

A

False. 45 years

48
Q

Examples of treatment for SCA include?

A

Replacement of normal hemoglobin with HgbF (controversial), STEM CELLS or Bone Marrow transplant (comes with RISK and finding a DONOR).

49
Q

To better prevent SCA, newborn screening is available in 44 states.

A

True.

50
Q

In regards to PREVENTIVE care for SCA, important interventions include?

A

Penicillin started by 2 months of age, Getting all immunizations including influenza and meningococcal, Monthly transfusions for severe pts , Iron-chelation for hemochromatosis, Hydroxyurea (PO) to increase production of HgbF, and Genetic counseling.

51
Q

HgbA

A

Sickle cell TRAIT

52
Q

HbS

A

Sickle cell DISEASE

53
Q

HgbS

A

Sickle cell DISEASE and TRAIT.

54
Q

Why is HYDRATION so important in the treatment of SCA?

A

Because it promotes hemodilution and circulation of RBC through the blood vessels.

55
Q

What are common S/S of a sickle-cell crisis?

A

Fever, severe abdominal pain, hand-foot syndrome (edematous in hands and feet), arthralgia.

56
Q

To prevent crisis (hypoxia), it is important to teach the family of a child with SCA to what?

A

Keep child from exercising strenuously, keep child away from HIGH altitudes, seek care at first sign of infection, keep child well hydrated, do NOT withhold fluids at night because ENURESIS is a complication of both the disease and the tx.

57
Q

What vaccines are important for SCA patients?

A

Pneumococcal, meningococcal, and Hib

58
Q

An inherited autosomal recessive blood disease that is characterized by deficiencies in the rate of production of specific globin chains in Hgb.

A

Thalassemia, a RARE but SEVERE abnormal production of Hgb. The Hem component is affected, not the RBC

59
Q

There are 4 different TYPES of Thalassemia. What are they?

A

Alpha Thalassemia, Beta Thalassemia, Cooley?s Anemia, and Mediterranean Anemia

60
Q

What is the most SEVERE type of Thalassemia?

A

Alpha Thalassemia. 4 chains are affected.

61
Q

How many chains are affected in Beta Thalassemia?

A

2 chains

62
Q

S/S of the most severe form of Thalassemia include?

A

Pallor, unexplained fever, FTT, severe anemia, hepatomegaly (spleen starts to mass produce, which can affect the liver as well)

63
Q

T or F. Bone marrow compensates with increased production of erythrocytes (bone changes)?

A

TRUE

64
Q

The objective of supportive therapy for Thalassemia is to maintain sufficient Hgb levels to prevent bone marrow expansion and the resulting bony deformities, and to provide sufficient RBCs to support normal growth and normal physical activity. How is this established?

A

Blood transfusions, chelation (deferoxamine or deferasirox or desferal)

65
Q

Besides transfusions, what are other ways in which to treat Thalassemia?

A

Splenectomy and antibiotics.

66
Q

In regards to treating Thalassemia, what are important nursing interventions to consider?

A

Monitoring for excess iron (hemosiderosis), Changes in VS, and S/S of infiltration from transfusions.