Blood disorders summary

. , , , 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

nionoveno@yahoo.comblood disorders

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

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

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)

1. Increase dietary consumption of Green leafy vegetables Meat Fish Legumes Whole grains

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

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

2. Insidious onset: usually diagnosed because of an infection or chronic GI problems

1. Assessment a. Pallor, weakness, tachycardia, dizzinessb. Slow motor developmentc. Poor muscle toned. Hemoglobin level below normal for age

• general rule: below 11 dl

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

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

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

Sources of Fe: Fish Eggs Apricots Raisins Red meat Dried beans

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)

Viral infection Chemicals (benzene or airplane glue) Radiation

Pancytopenia Leukopenia, thrombocytopenia, and decreased

erythrocytes, agranulocytosis

Symptoms Fatigue Weakness Tachycardia DOB Susceptibilty to infection Petechiae Gingival bleeding Epistaxis

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

For <45 yo Bone marrow transplant

human leukocyte antigen- (HLA-) matched sibling donor Cyclosporine (Sandimmune) for graft rejection

For >45 yo ATG Cyclosporine

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

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

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

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

Sickle cell crisis

Vasoocclusive Splenic sequestration

Aplastic Hyperhemolytic

StrokeChest syndrome

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]

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]

Aplastic crisis: diminished red blood cell production

b. triggered by a viral or other infection

d. Profound anemia rapid destruction decreased production

Hyperhemolytic crisis: increased rate of red blood cell destruction

b. Anemia, jaundice, and reticulcytosis

d. Glucose-6-phosphate dehydrogenase deficiency

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

Chest syndrome: clinically similar to pneumonia

Overwhelming infection Streptococcus pneumoniae

Haemophilus influenzae type B

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

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

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

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

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

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

Classification1. Factor VIII deficiency (classic

hemophilia): hemophilia A

3. Factor IX deficiency (Christmas disease): hemophilia B

Clinical findings1. Prolonged bleeding from any wound

3. Bleeding into the joints (hemarthrosis), resulting in pain, deformity, and retarded growth

5. Intracranial hemorrhage

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

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)

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

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

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

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

A. Etiology and pathophysiology

2. Results in a decreased number of leukocytes

4. Thought to arise from physical agents heavy metals and cytotoxic drugs

A. Clinical findings

2. Subjective

a. Fatigue b. Malaise

b Objective a. High fever b. Necrotic ulcers of

mucosa c. Rapid weak pulse

A. Therapeutic interventions

2. Removal of causative agent

4. Transfusions

6. Antibiotics

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

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

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

A. Medical interventions

2. Corticosteroids

4. Immunosuppressive agents

6. Splenectomy

8. Platelet transfusions

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

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

Acute lymphocytic leukemia (ALL)

2) Primarily occurs in children

4) Most favorable prognosis with chemotherapy

6) Results from abnormal leukocytes in blood-forming tissue

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

Chronic myelogenous leukemia (CML)

2) Occurs after the second decade

4) Prognosis is poor

6) Results from abnormal production of granulocytic cells

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

A. Clinical findings

1. Subjectivea. Malaiseb. Bone pain

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

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

Th a n k y o u !Nio C. Noveno, RN, MAN, MSN

Blood disorders summary

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