blood disorders summary
TRANSCRIPT
. , , , Nio C Noveno RN MAN MSN
An autoimmune disorder Parietal cells are destroyed Atrophy of gastric mucosa
Postgastrectomy
Onset: 50-60 years old Northern European & African American women
[email protected] disorders
2
Insidious onset Symptoms appear
several months after Extreme muscle
weakness Sore tongue Numbness &
tingling in the extremities
Edema of the legs Ataxia Dizziness
DOB Headache Fever Blurred vision Tinnitus Jaundice with pallor Poor memory Irritability Loss of bladder &
bowel control
[email protected] disorders
3
1. Lack of intrinsic factor • Prevents the absorption of vitamin B12 in the lower
portion of the ileum• Anemia
2. Assessment 2 Pallor2 Beefy red tongue2 Positive Romberg test (loss of balance when eyes
closed) Gastric analysis: no intrinsic factor: (+) Schilling’s test: urine test for B12 absorption
[email protected] disorders
4
È lifelong injections of cyanocobalamin (Rubramin PC) or hydroxocabalamin crystalline (Hydroxi Cysti12) 100 μg IM/SC qd x 5-10 days 100 μg IM 2x/week x 1 month 100 μg IM monthly x lifetime
2. Folic acid or folate (Folvite)
[email protected] disorders
5
1. Increase dietary consumption of Green leafy vegetables Meat Fish Legumes Whole grains
[email protected] disorders
6
Most common type of anemia Affecting premature infants or low birth weight patients Adolescent girls Alcoholics Elderly
Results from the decrease iron in the blood
Diagnostics test Decreased RBC Low Hgb & HCT Low serum iron High total iron binding capacity
[email protected] disorders
7
1. Lack of adequate sources of dietary irona. Infant usually has iron reserve for 6 monthsb. Premature infant lacks reservec. Children receiving only milk have no source of
iron
2. Insidious onset: usually diagnosed because of an infection or chronic GI problems
[email protected] disorders
8
1. Assessment a. Pallor, weakness, tachycardia, dizzinessb. Slow motor developmentc. Poor muscle toned. Hemoglobin level below normal for age
• general rule: below 11 dl
[email protected] disorders
9
Oral iron sources1) Drug: ferrous sulfate (Feosol) - most
absorbable form of iron Ferrous fumarate (Feostat)
3) Adverse effects: N/V; fatalities in children who ingest enteric-coated tablets
5) Drug interactions: • ferrous sulfate binds tetracycline and decreases
absorption• magnesium trisilicate decreases absorption of iron
[email protected] disorders
10
Parenteral iron sources Drug:
parenteral iron-dextran injection (Imferon, Infed)
Adverse effects: Tissue staining (use Z tract for
intramuscular injection) Fever Lymphadenopathy N/V Arthralgia Urticaria Severe peripheral vascular
failure Anaphylaxis Secondary
hematochromatosis
[email protected] disorders
11
Nursing Considerations: Vitamin C and hydrochloric acid aid absorption Folic acid acts as a coenzyme in the formation of heme
proteins are necessary for the synthesis of hemoglobin ascorbic acid promotes the conversion of folic acid to folinic acid
Oxalates, phosphate, and caffeine decrease absorption [MILK]
Use a straw because some liquid preparations stain teeth Discolors stools
May cause gastric irritation or constipation
[email protected] disorders
12
1. Unknown cause [congenital…]2. Secondary
Drug Chloramphenicol (Chloromycetin) Mephenytoin (Mesantoin) Trimethadione (Tridione) Mechlorethamine or nitrogen mustard (Mustargen) Methotrexate (Folex PFS) 6-mercaptopurine or 6-MP (Purinethol) Phenylbutazone (Butazolidin)
[email protected] disorders
14
Viral infection Chemicals (benzene or airplane glue) Radiation
Pancytopenia Leukopenia, thrombocytopenia, and decreased
erythrocytes, agranulocytosis
[email protected] disorders
15
Symptoms Fatigue Weakness Tachycardia DOB Susceptibilty to infection Petechiae Gingival bleeding Epistaxis
[email protected] disorders
16
Therapeutic interventions
3. Identify and eliminate causative agent Bone marrow aspiration
4. Blood transfusions (PRBC, platelets)5. Maintenance of fluid and electrolyte balance6. Corticosteroids and androgens 7. Splenectomy
[email protected] disorders
17
Therapeutic interventions
For <45 yo Bone marrow transplant
human leukocyte antigen- (HLA-) matched sibling donor Cyclosporine (Sandimmune) for graft rejection
For >45 yo ATG Cyclosporine
[email protected] disorders
18
Therapeutic interventions
Antithymocyte globulin or ATG (Atgam) Suppress the reaction Allow bone marrow recovery
Administration Given through CVP x 7-10 days
Improvement in 3-6 months
[email protected] disorders
19
1. Autosomal disorder affecting hemoglobin
3. Defective hemoglobin red blood cells sickle and clump together under
reduced oxygen tension initially fetal hemoglobin prevents sickling
5. Classification1. Sickle cell anemia: homozygous for sickle cell gene
3. Sickle cell trait: heterozygous for sickle cell gene
[email protected] disorders
20
Union of person with sickle cell disease or trait (P1) and person with sickle cell trait (P2)
P1: A A
P2: A s
No sickle cell: AA As: Sickle cell traitNo sickle cell: AA As: Sickle cell trait
[email protected] disorders
21
Union of two persons with sickle cell trait
P1: A s
P2: A s
No sickle cell: AA As: Sickle cell traitSickle cell trait: sA ss: Sickle cell anemia
[email protected] disorders
22
Sickle cell crisis
Vasoocclusive Splenic sequestration
Aplastic Hyperhemolytic
StrokeChest syndrome
[email protected] disorders
23
A. Clinical findings Vasoocclusive crisis (pain episode): most
common and non–life threatening
b. sickled cells obstructing blood vessels, causing occlusion, ischemia, and potential necrosis
d. Symptomsa. Feverb. Acute abdominal pain (visceral hypoxia)c. Hand-foot syndromed. Priapisme. Arthralgia [without an exacerbation of anemia]
[email protected] disorders
24
1. Splenic sequestration crisis
b. pooling large quantities of blood in the spleen
c. precipitous drop in blood pressure [shock]d. Acute episode mostly commonly in children
8 months and 5 years of age result in death from anemia & CV collapse
e. Chronic manifestation [functional asplenia]
[email protected] disorders
25
Aplastic crisis: diminished red blood cell production
b. triggered by a viral or other infection
d. Profound anemia rapid destruction decreased production
[email protected] disorders
26
Hyperhemolytic crisis: increased rate of red blood cell destruction
b. Anemia, jaundice, and reticulcytosis
d. Glucose-6-phosphate dehydrogenase deficiency
[email protected] disorders
27
Stroke: sudden and severe complication with no related illnesses
b. Sickled cells block the major blood vessels in the brain
d. Repeat strokes in 60% of children who have experienced previous one
[email protected] disorders
28
Chest syndrome: clinically similar to pneumonia
Overwhelming infection Streptococcus pneumoniae
Haemophilus influenzae type B
[email protected] disorders
29
A. Therapeutic interventions1. Prevention of sickling phenomenon
a. Adequate oxygenationb. Adequate hydrationc. Administration of hydroxyurea to limit sickling
3. Treatment of crisisa. Restb. Hydration/electrolyte replacementc. Pain managementd. Antibiotic therapye. Blood products
[email protected] disorders
30
Hydroxyurea MOA: probable inhibitor of ribonucleotide reductase system
INDICATION: Sickle cell anemia, CML, head & neck, ovarian, colon CA, melanoma, acute leukemia, polycythemia vera, HIV
SE: leukopenia, N/V, rashes, facial erythema,
DOSE: 50-75 mg/kg for WBC counts >100,000 cells/ml 2-30 mg/kg for refractory CML; 1000-1500 mg/day in SD or divided doses
[email protected] disorders
31
During crisis provide for:a. Adequate hydration (may need IV therapy):
Avoid dehydration
2) May cause a rapid thrombus formation
4) Fluid intake per body weight 130 to 200 ml per kilogram [2 to 3 oz
per pound]
b. Proper positioning, careful handling
[email protected] disorders
32
During crisis cont…:
a. Exercise as tolerated – immobility
promotes thrombus formation respiratory problems
c. Adequate ventilation
e. Control of pain– use narcotics
f. Blood transfusions for severe anemia
[email protected] disorders
33
Provide for genetic counselinga. Disorder mostly of blacks / Mediterranean
people
c. If both parents are carriers– 25% chance of producing a child with the
disease
e. Screen young children for the disorder– clinical manifestations usually do not
appear before 6 months of age
[email protected] disorders
34
Defect in clotting mechanism of blood
Genetic disorder; X-linked recessive transmission
Usually occurs in males; females are carriers but do not have the disease
[email protected] disorders
35
[email protected] disorders
36
[email protected] disorders
37
[email protected] disorders
38
Classification1. Factor VIII deficiency (classic
hemophilia): hemophilia A
3. Factor IX deficiency (Christmas disease): hemophilia B
[email protected] disorders
39
[email protected] disorders
40
[email protected] disorders
41
Clinical findings1. Prolonged bleeding from any wound
3. Bleeding into the joints (hemarthrosis), resulting in pain, deformity, and retarded growth
5. Intracranial hemorrhage
[email protected] disorders
42
1. Severity of bleeding
b. Mild1) Factor VIII activity of 5% to 50%2) Bleeding with severe trauma or surgery
c. Moderate1) Factor VIII activity of 1% to 5%2) Bleeding with trauma
d. Severe1) Factor VIII activity of 1%2) Spontaneous bleeding without trauma
2. Anemia
[email protected] disorders
43
Therapeutic interventions
1. Control of bleeding2. Prevention of bleeding with use of factor
replacementa. Drugs that replace deficient coagulation factors
1) Factor VIII concentrate from recombinant DNA2) Factor IX complex contains factors II, VII, IX, X
(concentrated)
[email protected] disorders
44
a. Adjunctive measuresr Aminocaproic acid (Amicar): inhibits the
enzyme that destroys formed fibrin and increases fibrinogen activity in clot formation
o Fibrinogen: maintains plasma fibrinogen levels required for clotting materials
t Thrombin: supplies physiologic levels of natural material at superficial bleeding sites to control bleeding
[email protected] disorders
45
Aminocaproic acid (Amicar)MOA: inhibits fibrinolysis via inhibition of TPA substances
INDICATION: excessive bleeding
SE: low BP & HR, dizziness, HA, fatigue, rash, GI disturbance, low platelet function
DOSE: 5 g IV or PO (1st h) followed by 1-1.25 g/h IV or PO
[email protected] disorders
46
Planning/Implementation1. Instruct the child and parents in the
treatment of bleedinga. Immobilization of the areab. Compression of the areac. Elevation of the body partd. Application of cool compresses
[email protected] disorders
47
1. Provide for appropriate activity2. Select safe toys and inform parents to safe-
proof house3. Avoid use of aspirin or ibuprofen4. Control joint pain5. Provide counseling6. Encourage parents to treat the child as
normally as possible
[email protected] disorders
48
A. Etiology and pathophysiology
2. Results in a decreased number of leukocytes
4. Thought to arise from physical agents heavy metals and cytotoxic drugs
[email protected] disorders
49
A. Clinical findings
2. Subjective
a. Fatigue b. Malaise
b Objective a. High fever b. Necrotic ulcers of
mucosa c. Rapid weak pulse
[email protected] disorders
50
A. Therapeutic interventions
2. Removal of causative agent
4. Transfusions
6. Antibiotics
[email protected] disorders
51
A. Planning/Implementation1. Prevent infection and observe for signs
of infection2. Provide careful oral hygiene3. Provide for bed rest4. Encourage diet consisting of high
nutrient density foods that are rich in vitamins and minerals
[email protected] disorders
52
A. Etiology and pathophysiology
2. Appears to result from the production of an antiplatelet antibody, which coats the surface of platelets
4. Coated platelets are then easily destroyed by phagocytic leukocytes
[email protected] disorders
53
A. Clinical findings 1. Subjective
b. History of epistaxis
d. History of gum bleeding
2. Objective
b. Low platelet count
d. Ecchymotis
f. Hemorrhagic petechiae
[email protected] disorders
54
A. Medical interventions
2. Corticosteroids
4. Immunosuppressive agents
6. Splenectomy
8. Platelet transfusions
[email protected] disorders
55
B Planning/Implementation 1. Prevent injury and bruises2. Encourage client to adhere to medical
regimen 3. Teach the side effects of medications,
particularly proneness to infection 4. Provide postoperative care related to
splenectomy
[email protected] disorders
56
A. Etiology and pathophysiology
2. Incidence highest in children ages 3 to 4• declines until age 35 [there is a steady
increase]3. Exposure to certain toxic substances
• radiation increases the incidence4. An uncontrolled proliferation of white blood
cells 5. Classified according to the type of white
blood cell affected
[email protected] disorders
57
Acute lymphocytic leukemia (ALL)
2) Primarily occurs in children
4) Most favorable prognosis with chemotherapy
6) Results from abnormal leukocytes in blood-forming tissue
[email protected] disorders
58
Acute myelogenous leukemia (AML)
2) Occurs throughout life cycle
4) Prognosis is poor with or without chemotherapy
6) Results from inability of leukocytes to mature
[email protected] disorders
59
Chronic myelogenous leukemia (CML)
2) Occurs after the second decade
4) Prognosis is poor
6) Results from abnormal production of granulocytic cells
[email protected] disorders
60
Chronic lymphocytic leukemia (CLL)
2) Occurs after age 35 years
4) Life expectancy: 4 to 5 years
6) Results from increased production of leukocytes and lymphocytes
• proliferation of cells within the bone marrow, spleen, and liver
[email protected] disorders
61
A. Clinical findings
1. Subjectivea. Malaiseb. Bone pain
[email protected] disorders
62
1. Objectivea. Anemiab. Thrombocytopeniac. Elevated leukocytesd. Decreased plateletse. Petechiaef. Gingival bleeding
A. Therapeutic interventions1. Chemotherapy2. Transfusions of whole blood or blood fractions3. Analgesics4. Bone marrow transplant5. Radiation to areas of lymphocytic infiltration
[email protected] disorders
63
A. Planning/Implementation1. Discuss the importance of follow-up care with the
client and family2. Provide emotional support for the client and
family3. Provide specific nursing care related to particular
chemotherapeutic therapy, transfusion, or diagnostic tests
4. Provide a safe injury-free environment5. Use appropriate infection control techniques6. Pace care to avoid fatigue and assist client as
necessary
[email protected] disorders
64
Th a n k y o u !Nio C. Noveno, RN, MAN, MSN