Hematologic Disorders

Question 1

Anemia

Answer 1

RBC count is low

Question 2

Polycythemia

Answer 2

RBC count is high

Question 3

Agranulocytes

Answer 3

Lymphocytes: increased with infections
Monocytes: in chronic conditions

Question 4

Granulocytes

Answer 4

Neutrophils: acute inflammation, bacterial infection
PMN: indicates infections
Bands: immature neutrophils
Eosinophil: allergic reactions
Basophils: healing phase of inflammation and hay fever

Question 5

Platelets

Answer 5

Used to stop bleeding
Made in bone marrow
Stored in spleen
Life span 8-10 days
150,000 - 300,000 is normal

Question 6

Thrombocytopenia

Answer 6

Decreased platelet count from a bleeding disorder

Question 7

Intrinsic pathway

Answer 7

Released by plasma

PTT measures abnormalities for pts on heparin

Question 8

Extrinsic Pathway

Answer 8

Released by the injured tissue

PT measures abnormalities for pts on warfarin

Question 9

Immunoglobulins

Answer 9

IgM - First responder, 2-3 days
IgG - Day 4-10, last for a new weeks
IgA - Located in GI tract, respiratory tract, and GU tract. Prevents adherence
IgE - Allergic response

Question 10

T-Helper (CD4)

Answer 10

Involved in immune surveillance, stimulate B- lymphocyte production
CD4 count - HIV

Question 11

T-Suppressor

Answer 11

Supresses production of immunoglobulins against specific antigens and prevents overproduction
Body fails to recognize self

Question 12

T-Cytoxic

Answer 12

Aid in destroying viral, fungal, protozoan and some bacteria
Goes out and eats the cells

Question 13

Natural Killer

Answer 13

Migrate to blood and spleen and aid the Tcells

Question 14

Risk for Infection

Answer 14

Good hand washing, monitor VS, antibiotics, G-CSF to stimulate granulocytes
Aseptic technique for all procedures, insure good nutrition, and monitor CBC and WBC
Teach prevention!

Question 15

Imbalanced Nutrition: Less Than

Answer 15

Promote nutrition with small high calorie feeds and maintain fluid balance
Dietary management, iron fortified formula for infants
Older child with PICA – eat ice

Question 16

Risk for Injury: Bleeding

Answer 16

SAFETY!
Assess CNS, joints, GI/GU bleeding. Recognize and control bleeding
NO ASA or NSAIDs, IM injections
Give factor replacement
Platelets <50-100,00 limit contact sports, no bike riding, skateboarding, or climbing
Encourage quiet activity

Question 17

Activity Intolerance

Answer 17

Assess color of skin and mucus membranes
Allow periods of rest
In bed activities
Good nutrition

Question 18

Risk for Disturbed Body Image

Answer 18

Provide emotional support and allow them to discuss feelings

Have teens talk to someone with their condition

Question 19

Impaired Physical Mobility

Answer 19

Promoting mobility is key
Physical therapy!!
Encourage ADL’s
Protect the joint

Question 20

Caregiver Role Strain

Answer 20

May need to evaluate family coping
Provide emotional support and allow families to verbalize feelings
Parents may have guilt for being a carrier

Question 21

Knowledge Deficit

Answer 21

Use a family approach
Teach disease process
Identify and avoid triggers
Treatment regimen

Question 22

3 Types of Anemia

Answer 22

Microcytic and Hypochromic
Normocytic and Normochromic
Macrocytic -- Rare
MCV- the size
MCH- the color, dependent on amount oh Hgb

Question 23

3 Causes of Anemia

Answer 23

Increased RBC destruction
Increased RBC loss
Decreased RBC production (aplastic anemia)

Question 24

Iron Deficiency Anemia

Answer 24

Low MCV, MCH
Serum Feritin is low
Fatigue, pale, irritable
Underweight or overweight from too much milk

Question 25

Increased RBC Destruction S/Sx

Answer 25

``` Pallor Tachycardia, Heart Murmur Fatigue, HA Muscle Weakness Frontal Bossing ```

Question 26

Increased RBC Loss S/Sx

Answer 26

``` Jaundice Fatigue, HA Dark Urine Enlarged spleen and liver Low BP ```

Question 27

Decreased RBC Production S/Sx

Answer 27

``` Pallor Fatigue, HA Muscle Weakness Cool skin Tachycardia Decreased peripheral pulses ```

Question 28

Iron Deficient Anemia Causes

Answer 28

Cows milk is a poor source of iron Iron supplements can begin at 2 months for preterm, 4-6 months for term babies Appears around 9 to 12 months due to loss of iron stores from birth In adolescents from poor diet and menstruating girls

Question 29

Iron Deficient Anemia Nursing Care

Answer 29

Prevention and Education High risk- WIC children and low income families Administer medication via syringe at back of the mouth, or drink through a straw Take with something acidic, Orange juice Encourage a rich diet of leafy green veggies Medication causes constipation and tarry stool!

Question 30

Sickle Cell Anemia

Answer 30

A condition of normocytic, normochromis anemia with increased RBC destruction Normal Hgb A is replaced with abnormal Hgb S Cells are sickled Autosomal recessive! Girls > Boys African American > Newborn screening

Question 31

Sickle Cell Assessment Questions

Answer 31

Ask about past crisis Ask about triggers Past medical treatment and home management Assess psychosocial

Question 32

Sickle Cell Signs and Symptoms

Answer 32

Asymptomatic until 4-6 months Sickle cells cant flow easily Clogged up -- Trigger pain crisis Blindness, enlarged liver and spleen, chest crisis Joint swelling -- also in penis and testes Possible stroke, paralysis and death

Question 33

Sickle Cell Head to Toe Assessment

Answer 33

See Box 35-4 | Page 1437

Question 34

Sickle Cell Treatment

Answer 34

Limit activity to reduce energy expenditure Improve oxygenation HYDRATION! I/O's Reduce Metabolic Acidosis -- from hypoxia PAIN MANAGEMENT! Transfuse packed RBCs, Antibiotic -- If needed Home care: Penicillin K, multivitamin, folic acid

Question 35

B- Thalassemia Major

Answer 35

``` Cooley's Anemia Autosomal Recessive Defective in the synthesis of hgb A Microcytic and hypochromic Leads to increased RBC destruction Increased serum feratin EXTRA Iron- overload Mediterranean Middle Eastern Decent ```

Question 36

B- Thalassemia Major Treatment

Answer 36

PRBC transfusions -- prevent bone marrow expansion Keep Hgb >9.5 Take Desferal (Deferoxamine) Diet low in iron Prophylactic antibiotics Spleenectomy to decrease discomfort and rate of RBC destruction

Question 37

B- Thalassemia Major Nursing Care

Answer 37

Support family and child | Body Image disturbance -- bony changes, decreased growth and sexual immaturity

Question 38

Aplastic Anemia

Answer 38

Formed elements of the blood are low, RBC, WBC and Platelets Sudden onset Life threatening Autosomal recessive- primary or congenital

Question 39

Aplastic Anemia Acquired Causes

Answer 39

HPV, Hepatitis, an overwhelming infection Immune disorders Drugs, household chemicals

Question 40

Aplastic Anemia Congenital Cause

Answer 40

Fanconi's Anemia Patchy brown areas of skin discoloration Genetic counseling is needed! Autosomal Recessive Syndrome

Question 41

Aplastic Anemia Diagnosis

Answer 41

CBC is first step | Bone marrow aspirate-- appear yello and fatty instead od red

Question 42

Aplastic Anemia Treatment

Answer 42

Immunosuppressant to limit aplasia Stem Cell transplant Treatment is supportive

Question 43

Hemophillia A

Answer 43

X-linked recessive disorder of Factor 8 deficiency Boys > girls Mild, moderate and severe

Question 44

Mild Hemophillia A

Answer 44

Bleeding with trauma or surgery

Question 45

Moderate Hemophillia A

Answer 45

Bleeding with trauma

Question 46

Severe Hemophilla A

Answer 46

Spontaneous bleeding without trauma | More common

Question 47

Hemophilla Concerns

Answer 47

Bleeding into the tissue -- Neck or thorax can impair breathing Intracranial can be fatal Retro peritoneal is an issue to due large space for blood accumulate Hematoma on spinal cord = paralysis Hemoarthosis in the joint-- lead to loss of function

Question 48

Hemophilla Treatment

Answer 48

``` Factor 8 Replacement as an infusion Goal is to stop[ bleeding Permanent IV access R.I.C.E. Parents can administer -- then child ```

Question 49

Hemophilla B

Answer 49

Christmas Disease Sex linked Recessive trait Factor IX deficiency Treatment with replacement therapy

Question 50

Idiopathic Thrombocytopenia Purpura (ITP)

Answer 50

Autoimmune disease Excessive destruction of platelets Usually resolves within 6 months

Question 51

ITP Symptoms

Answer 51

Bruising, purpura Bleeding that wont stop RISK FOR INJURY Wear helmets A LOT

Question 52

ITP Treatment

Answer 52

Supportive and observe if mild Symptomatic -- give IV IgG to raise platelet count Prednisone up to 3 weeks

Question 53

HIV Etiology

Answer 53

Vertical Transmission - perinatal from mother -- leading cause in children Horizontal from sex or parental contact with blood or body fluid Vertical # 1 way Look at CD4 count

Question 54

HIV Diagnosis

Answer 54

Done by ELISA and Western Blot Positive in infants with HIV+ mothers, maternal antibodies last 18 months PCR x2 for proviral DNA -- early as 1 to 3 months CDC surveillance for AIDS

Question 55

HIV Treatment Goals

Answer 55

Slow growth of HIV Virus Promote normal growth and development Decrease infections and cancers Improve quality of life and prolong survival

Question 56

HIV Treatment

Answer 56

``` Antiretroviral agents AZT first then HAART Bactrim starting at 4 - 6 weeks MMR and Varicella as scheduled Monitor viral load and CD4 Standard isolation techniques ```

Question 57

HIV Prognosis

Answer 57

Earlier presentation of AIDS = Poor, esp 100,000 and CD4 < 15%

Question 58

SCIDS

Answer 58

Congenital X-linked Autosomal Recessive Prents the formatio nof T-lymphocytes Affects maturation of B-lymphocytes

Question 59

SCIDS S/Sx

Answer 59

``` Early infection (oral candidiasis) Failure to thrive Chronic diarrhea Otitis media Pneumonia Failure to recover from an infection Low or absent B & T lymphocytes ```

Question 60

SCIDS Treatment

Answer 60

``` Goal: Restore immune function Give IV Ig Possible Stem cell transplant HIGH RISK FOR GRAFT vs HOST Prevent and promptly treat infections ```

Question 61

SLE

Answer 61

Girls > Boys Chronic inflammatory disease with an unknown origin Characterized by exacerbation and remission Involves more than one body system

Question 62

SLE S/Sx

Answer 62

Nephritis Arthritis or vasculitis Butterfly rash See pg 1683

Question 63

SLE Treatment

Answer 63

Goal is remission of symptoms and prevent complications May need steroids Anti-malarials help skin, renal and arthritis NSAIDs for pain

Question 64

SLE Nursing Care

Answer 64

``` Education and compliance with medication Understanding triggers and episodes Positive self- image r/t weight -- buffalo hump and moon shaped face Sunscreen and protective clothing Support groups ```

Question 65

Anaphylaxis

Answer 65

Clinical syndrome resulting from exposure to an allergen Result of IgE release -- histamine release -- vasodilaion -- multi-system involvement MEDICAL EMERGENCY EPIPEN!