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NUR 340 exam 1 > HEMATO > Flashcards

HEMATO Flashcards

1
Q

Pediatric Variations

A

Red Blood cell Production shifts from the liver to the bone marrow​

Hemoglobin F predominant for 1st ​
6-months of life​

Fetus receives iron through the placenta from the mother and “stores” iron for later use.

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2
Q

Assessment of Hematologic Function

A

Red Blood cell Production shifts from the liver to the bone marrow​

Hemoglobin F predominant for 1st ​
6-months of life​

Fetus receives iron through the placenta from the mother and “stores” iron for later use.

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3
Q

Complete Blood Count

A

RBC 4.5-4.8​

HGB 11-16​

HCT 35-41​

MCV 81-99​

MCH 27-31​

MCHC 32-36​

Retic 1-2%​

RDW 11-15%​

Platelets 2-4 X 100,000

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4
Q

RBC range

A

4.5 - 4.8

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5
Q

a

A

a

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6
Q

What to do with low platelets?

A

> 100,000-normal ​
- No contact sports​
- Protective equipment such as bike helmets when riding a bike​

50,000-100,000​
- Padding with activity​
- Protective equipment such as bike helmets when riding a bike​

<50,000​
- Extreme caution as kids can spontaneously bleed in their head​
- Quiet activities​

> 20,000​
- Kids can return to school

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7
Q

CBC with differential​

A

WBC (5-10) X 1000​

Lymphs (25-40%)​

Mono (2-8%)​

Neutrophils (54-75%)​

Eosino (1-4%)​

Baso (0-1%)​

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8
Q

Absolute Neutrophil Count (ANC)

A

% of Segs + % of Bands (total neutrophil %) X Total WBC= ANC

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9
Q

Nursing interventions for neutropenia

A

Monitor V/S especially temperature; Temperature > 100 is considered a medical emergency!!​

Good hand washing​

Inspect skin for breaks & redness​

Inspect mouth for ulcers​

No flowers & plants in room​

Low bacteria diet​

Change dressings & lines using sterile technique​

No live-virus vaccines​

Avoid contact with persons who carry viruses

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10
Q

Risk of injury to the ___ is high

A

mucus membranes

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11
Q

Erythrocytes (Red Blood Cells)

A

Regulation of Erythrocyte Production​
- Tissue oxygenation​
- Erythropoietin​

Lifespan; 120 days​

Abnormalities; Polycythemia or Anemia

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12
Q

Anemia

A

The most common hematologic disorder of childhood​

Decrease in number of RBCs and/or hemoglobin concentration below normal​

A state in which an individual can not conduct his/her activities because the oxygen carrying capacity of their blood is too low.

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13
Q

Etiology of Anemia

A

Hemorrhage​

Hemolysis​

Diminished Production​
- Bone Marrow Suppression​
- Absence of substances needed for production; iron, B complex vitamins, erythropoietin​

Classified by morphology or etiology.

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14
Q

Clinical Manifestations of Anemia​

A

Anorexia​

Pallor​

Skin break down​

Jaundice​

Tachycardia & Tachypnea​

Altered neurologic status/ behavior changes

Weakness or low exercise tolerance​

Gum hypertrophy​

Smooth tongue​

Blood in Urine or Stool​

Infections​

Cold intolerance

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15
Q

Effects of Anemia on Circulatory System​

A

Hemodilution​

Decreased peripheral resistance​

Increased cardiac circulation and turbulence​
- May have murmur​
- May lead to cardiac failure​

Cyanosis​

Growth retardation

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16
Q

Therapeutic Management​ of Anemia

A

Treat underlying cause​
- Transfusion after hemorrhage if needed​
- Nutritional intervention for deficiency anemias​

Supportive care​
- IV fluids to replace intravascular volume​
- Oxygen ​
- Bed rest

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17
Q

Nursing considerations for anemia

A

Prepare child and family for laboratory tests​

Decrease oxygen demands​

Safety​

Good hand-washing and mouth care​

Maintain normal body temperature​

Prevent complications​

Support family

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18
Q

Major Anemia’s of Childhood​

A

Production​
- Iron Deficiency Anemia​
- Aplastic Anemia​

Hemolytic​
- Sickle Cell Anemia​
- Beta-Thalassemia

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19
Q

ANEMIA CAUSED BY DECREASED PRODUCTION: Etiology

A

Bone marrow fails to produce RBCs​
- Leukemia or other malignancy​
- Chronic renal disease​
- Collagen diseases​
- Hypothyroidism​
- Nutritional Deficiencies

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20
Q

Iron Deficiency Anemia

A

Most prevalent nutritional disorder in the US. ​

Happens when the body does not have enough iron to produce Hgb.​

Incidence has decreased with WIC

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21
Q

Clinical Manifestations Iron Deficiency Anemia​

A

Irritability, Anorexia​

Pallor of skin & mucous membranes​

Mild growth retardation​

Exercise intolerance​

Frequent Infections​

Cognitive delays & behavioral changes

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22
Q

Clinical Manifestations Iron Deficiency Anemia​

A

Irritability, Anorexia​

Pallor of skin & mucous membranes​

Mild growth retardation​

Exercise intolerance​

Frequent Infections​

Cognitive delays & behavioral changes

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23
Q

Etiology: Iron Deficiency Anemia (IDA)

A

Inadequate iron stores at birth ​

Deficient dietary intake​
- Rapid growth rates​
- Infancy, Toddler Period, Adolescence​
- Excessive milk intake​
- Poor general eating habits​
- Exclusive breast feeding after 6-months​

Impaired iron absorption ​
- Presence of iron inhibitors​
- Malabsorption disorders​
- Chronic diarrhea

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24
Q

Therapeutic Management IDA​

A

Prevention​
- Breast milk or commercial infant formula for first 12-months of life​
- Limit formula to < 1L/day (32 oz)​
- Limit Milk to < 24 ounces/day​

Nutritional supplements; by age 6-months​
- iron fortified formula​
- cereal​

Iron Supplements; ferrous sulfate

Blood transfusions for severe cases

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25
Q

​Nursing Implications IDA​

A

Assessment; pay particular attention to milk and iron intake.​

Determine and eliminate the cause​

General nursing implications for anemia​

Provide foods rich in iron​

Teach parents to administer supplements​

Administer parenteral iron safely​

Follow-up care

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26
Q

How To Administer Oral Iron​

A

Best absorbed in an acidic environment​ (oranges)

Give with straw or back in the mouth past the teeth​

Rinse mouth/ brush teeth after administration.​

Teach parents; measure accurately, increase fluids and fiber in diet.​

Avoid antacids, coffee, tea, dairy products, eggs, or whole grains one hour before or two hours after administration.

Between meals or with ascorbic acid to facilitate absorption

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27
Q

Adverse effects of iron supplements

A

Nausea​

Gastric Irritation​

Constipation​

Diarrhea​

Anorexia​

Staining of teeth​

Tarry stools​

Overdose is lethal!!!

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28
Q

Aplastic Anemia​ (AA)

A

Bone Marrow failure; All formed elements of the blood are simultaneously depressed—“pancytopenia”​

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29
Q

Pathophysiology: Aplastic anemia

A

red bone marrow converted to yellow, fatty marrow.​

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30
Q

Etiology: aplastic anemia

A

Primary (congenital)
- Fanconi’s anemia

Secondary (acquired)

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31
Q

Therapeutic management of AA

A

Bone marrow transplant ​

Stem cell transplant (HSCT)​

Immunosuppressive therapy

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32
Q

Nursing considerations for AA

A

same as Leukemia

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33
Q

ANEMIAS CAUSED BY INCREASED DESTRUCTION OF RED BLOOD CELLS

A

Hemolysis​

Decreased life span of RBC​

Hereditary spherocytosis (HS)​
- Splenectomy/partial splenectomy can correct hemolysis but does not correct underlying defect​
- Splenectomy rarely before age 5​

Aplastic crisis

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34
Q

Sickle cell disease

A

A group of inherited hemoglobinopathies in which the RBCs do not carry the normal adult hemoglobin, but rather a less effective type.

Most common type is Hgb SS or sickle cell anemia

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35
Q

Epidemiology of SCA

A

Autosomal recessive hemolytic anemia​

Most common in persons of African, Mediterranean, Middle Eastern, & Indian decent.​

1 in 12 African Americans have SC trait. People with SC trait have < 50% Hgb S​

1 in 500 African Americans have sickle cell anemia​

Usually Asymptomatic until age 6-months

In areas of the world where malaria is common, individuals with sickle cell trait tend to have a survival advantage over those without the trait​

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36
Q

Patho: SCD

A

inherited, autosomal recessive

several mutations in the B-globin gene that cause 6th amino acid to be changed from glutamic acid to valine.

resultant hemoglobin (called HbS) has abnormal physiochemical properties, and is prone to polymerization with other hemoglobin molecules under conditions of low oxygen tension.

number of adverse affects on erythrocytes.​

The normally freely flowing cytosol of red cells become viscous making the red cell much less deformable and impairing its ability to traverse tight capillary beds.

As HbS continues to polymerize the entire RBC is deformed giving the characteristic sickle shape.​

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37
Q

SCA complications

A

Acute Painful Episodes (Sickle Cell Crises)​

Stroke​

Sepsis​

Acute chest syndrome​

Reduced visual acuity​

Chronic leg ulcers​

Delayed growth & development​

Delayed puberty​

Priapism​

Enuresis

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38
Q

Vaso-occlusive crisis

A

Due to the deformed shape, HbS induces RBC membrane damage leading to calcium influx into the cell. Calcium influx leads to crosslinking of the membrane proteins and activating channels that allow for the efflux of potassium and water from the cell. This leads to RBC dehydration exacerbating the sickling.Vaso-occlusive crisis results from the sickle red cells obstructing and reducing blood flow to the vital organs leading to ischemia, necrosis and pain. Repeated episodes lead to bone infarction and necrosis; and bone marrow degenerationoccurs overtime. Long bones are affected most commonly, but pain episodes can affect any bone marrow-containing structure, including the ribs, sternum, vertebral bodies, and skull.Pulmonary fat embolism can be a life threatening complication of bone marrow infarction in patients with SCD and precipitate Acute Chest Syndrome (ACS).​

Furthermore, hemoglobin released from hemolyzed cells readily binds to and depletes NO, leading to vascular smooth muscle cell contraction and enhanced platelet aggregation. Occlusions lead to further hypoxia within the tissue, setting up conditions for a vicious cycle in which further sickling and hypoxia occur.

39
Q

Hemolysis

A

Sickle cells are mechanically weak and are prone to intravascular hemolysis. However, the more important mechanism leading to decreased red cell survival time is the extravascular hemolysis that occurs when inflexible cells are trapped in the spleen and phagocytosed by the reticuloendothelial systemBone marrow tries to compensate by increasing RBC production but it cannot match the rate of destruction.​

A normal RBC survives for an average of 90-120 days, whereas, a sickle cell survives 10-20 days.​

Complications of increased hemolysis include cholelithiasis; due to excessive bilirubin production.

40
Q

Hypo-splenism and Infection

A

Encapsulated bacterial infections: Splenic sequestration of sickle cells leads to splenic congestion, as manifested by splenomegaly, and reduced immune function. Since the spleen is important for macrophage phagocytosis of encapsulated bacteria, patients with SCD are prone to bacteremia with pathogens like Streptococcus pneumoniae (S. pneumoniae), Haemophilus Influenzae (H. Influenzae) and Neisseria meningitidis (N. Meningitidis). These pathogens, normally causing localized disease, may cause life-threatening sepsis in patients with sickle cell disease.Furthermore, patients with SCD develop pneumonias, predominantly from atypical organisms, such as, Mycoplasma pneumoniae, Chlamydia pneumoniae and Legionella. Respiratory viruses are also common causes of pulmonary infection, while S. pneumoniae and H. influenza type b are uncommon.Osteomyelitis and septic arthritis can affect minority of patients with SCD due to both, bone damage and poor splenic function. The leading pathogens include Salmonella, S. Aureus and other gram negative bacteria.​

Autosplenectomy: Continued splenic dysfunction eventually leads to infarction and loss of splenic function, which is referred to as autosplenectomy or functional asplenia.​

Dysfunctional complement system has been proposed to contribute to the infectious complications in SCD, however, this has yet to be proven.

41
Q

Pulmonary and Cardiovascular complications

A

Neurologic complications are related to vasoocculusive crisis and include strokes and silent strokes.The acute pulmonary complications of SCD, collectively referred to as the acute chest syndrome (ACS). It is defined as the appearance of new infiltrate with pulmonary symptoms, presence of fever, hypoxia and chest pain (minority of patients might not have an infiltrate initially). Precipitants of ACS include infections (see above for details), infarction and/or pulmonary fat embolism.No specific cardiomyopathies are present however, two cardiac complications occur; chamber enlargement related to significantly increased forward output due to chronic anemia and sleep/wake oxygen desaturations and secondly, myocardial infarction from vasoocclusion of the coronary arteries. Rarely, RV dysfunction (Cor pulmonale) may develop due to Pulmonary arterial hypertension.

42
Q

Acute Painful Episodes (Sickle Cell Crises)​

A

Acute exacerbations that vary in severity and frequency​

Precipitating factors​
- Anything that increases the body’s need for oxygen or alters transport of oxygen​
- Trauma​
- Infection, fever​
- Physical and emotional stress​
- Increased blood viscosity due to dehydration​
- Hypoxia ​
- From high altitude, poorly pressurized
airplanes, hypoventilation, vasoconstriction
due to hypothermia

43
Q

Types of Acute Painful Episodes (Sickle Cell Crises)

A

Vaso-occlusive (VOC) thrombotic​

Splenic sequestration

Aplastic crises

Acute Chest Syndrome

44
Q

Vaso-occlusive (VOC) thrombotic​

A

AKA painful event/ painful episode​

Most common type of crisis—very painful​

Stasis of blood with clumping of cells in microcirculation → ischemia → infarction​

Signs—fever, pain, tissue engorgement

Treatment: Hydration, Pain management, heat

45
Q

Splenic sequestration

A

Life-threatening—death can occur within hours​

Blood pools in the spleen​

Signs ​
- Profound anemia​
- Hypovolemia​
- Shock

Treatment: Splenectomy, Transfusions

46
Q

Aplastic crises

A

Diminished production and increased destruction of RBCs​

Triggered by viral infection or depletion of folic acid​

Signs include profound anemia, pallor

Treatment: Splenectomy, Transfusions

47
Q

Acute Chest Syndrome​

A

Similar to pneumonia​

VOC or infection results in sickling in the lungs​

Chest pain, fever, cough, tachypnea, wheezing, and hypoxia ​

Repeated episodes may lead to pulmonary hypertension​

Treatment: Adequate pain management, Antibiotics, with cover for atypical organisms, ICU, ventilator support, blood transfusion , exchange transfusion may be indicated if no improvement, bronchodilators if asthma like symptoms

48
Q

Prognosis Sickle Cell Anemia​

A

Prognosis varies​, highest risk are children under 5-years old from infection​

Life expectancy currently in the mid-forties ​

No cure (except possibly bone marrow transplants)​

Frequent bacterial infections may occur due to immunocompromise ​

Bacterial infection is leading cause of death in young children with sickle cell disease​

Strokes in 5% to 10% of children with disease​
- Result in neurodevelopmental delay, cognitive delays

49
Q

Medical Management SCA​

A

Diagnosed with Hgb Electrophoresis; part of the newborn screen, Cord blood in newborns​​
Newborn screening done in 43 states

Genetic testing to identify carriers and children who have disease​

Sickle turbidity test
- Quick screening purposes in children older than 6 months​

Palliative Treatment-Erythropoietin or hydroxyuria ( increase Hgb F production), PCN prophylaxis​

Transcranial Doppler annually​

Preventing Acute Painful Episodes by Avoiding known triggers of the sickling phenomenon, infection, & other complications

Transcranial Doppler annually to assess intracranial blood flow and determine CVA risk

Crisis Management​

Hyper-transfusion; keep Hgb > 10Frequent transfusion leads to hemosiderosis (iron in tissues)​

Treat with iron chelation ​
- Parenterally—deferoxamine​
- Oral—deferasirox or deferiprone​
- Used alone or in combination​
+ Vitamin C to promote iron excretion

Bone Marrow Transplant​

50
Q

Crisis Management/Nursing Considerations SCA

A

Maintain Hydration​

Maintain Oxygenation​

Pain management​

Support for the Child & Family​

51
Q

Thalassemia

A

Inherited blood disorders of hemoglobin synthesis​

Classified by Hgb chain affected and by amount of effect​

Autosomal recessive with varying expressivity​
- Both parents must be carriers to have offspring with disease

52
Q

Pathophysiology​: Thalassemia

A

Alpha or beta-globulin chain in Hgb synthesis is reduced or entirely absent.​
- Large number of unstable globulin chains,​
- Causes RBCs to be rigid and easily hemolyzed​
- Severe hemolytic anemia leading to hypoxia​

Normal RBC size with decreased amounts of Hgb​

Chronic hypoxia​
- Headache, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxis​

Detected in infancy or toddlerhood​
- Pallor, FTT, hepatosplenomegaly, severe anemia (Hgb <6)

53
Q

Types of thalassemia

A

alpha

beta

54
Q

a - Thalassemia

A

α chains affected​

Occurs in Chinese, Thai, African, and Mediterranean people

55
Q

b -Thalassemia

A

Occurs in Greeks, Italians, and Syrians​

b is most common and has four forms​
- Thalassemia minor
- Thalassemia trait
- Thalassemia intermediate​
- Thalassemia major “Cooley anemia”

56
Q

Thalassemia minor

A

asymptomatic silent carrier​

57
Q

Thalassemia trait

A

mild microcytic anemia​

58
Q

Thalassemia intermediate

A

moderate to severe anemia + splenomegaly​

59
Q

Thalassemia major “Cooley anemia”

A

severe anemia requiring transfusions to survive

60
Q

Clinical Manifestations of​
b Thalassemia

A

Anemia; severe​

Bronze skin tone from hemosidorosis. (excessive iron storage in various tissues)

Growth retardation and delayed puberty​

Cardiac enlargement with flow murmur leading to heart failure​

Bone pain, skeletal deformities, fractures​

Death usually related to heart failure or iron overload

61
Q

Medical Management​ of b thalassemia

A

Diagnosed with Hgb Electrophoresis​

Blood transfusion to maintain normal Hgb levels​

Side effect—hemosiderosis​
- Treat with iron-chelating drugs such as deferoxamine​
- Binds excess iron for excretion by kidney​
- IV or SQ over 8-10 hours multiple times/wk​
- May be given at home with IV pump per parents​
- New oral chelation drugs—deferasirox ;Worldwide use ​

Also give oral vitamin C to facilitate binding of iron

62
Q

Nursing Interventions for ​
b Thalassemia

A

Administer packed Red Blood Cells safely. ​

General measures for anemia​

Monitor chelation Therapy​
- N/V/D​
- Decreased appetite​
- Rash​
- Increased liver enzymes​
- Neutropenia​
- *excreted via the kidneys so adequate hydration is vital​

Measures to enhance self-esteem & body image​

Measures to enhance home health maintenance

63
Q

how bleeding stops

A

1) blood vessels get smaller​

2) platelet plug is made:​
- Adhesion- platelets sticking to wall of torn blood vessel​
- Activation and secretion- platelets change shape to connect with other platelets​
- Aggregation- platelets stick to each other and to the blood vessel​
ALL OF THIS IS TEMPORARY​

3) clotting factors- A fibrin clot made from clotting factors is needed to make a fibrin weave to make strong clot or strong seal.

64
Q

Alterations in Clotting Function​

A

Idiopathic Thrombocytopenic Purpura​

Hemophilia

65
Q

Platelets

A

the cellular fragments needed to form a clot.

66
Q

Thrombocytopenia

A

low platelet count

67
Q

Decreased platelet production

A

leukemia, aplastic anemia

68
Q

Abnormal destruction of platelets

A

ITP

69
Q

Abnormal pooling of platelets

A

Sickle cell splenic sequestration crises

70
Q

Idiopathic Thrombocytopenic Purpura (ITP)

A

Most frequent occurring thrombocytopenia of childhood.​

Acquired Hemorrhagic disorder​

Autoimmune destruction of platelets in the spleen​

May be acute or chronic.​

Cause unknown​ - thought to be an immune response following a viral infection (measles, C-pox, or rubella) that produces antiplatelet antibodies. ​

Occurrence ; Caucasian children between 2 & 8 years old​

Most children recover spontaneously in 6-12 months

71
Q

Clinical Manifestations ITP​

A

Sudden onset of easy bruising, purpura, and petechiae​

Epistaxis​

Platelet count less than 20,000​

Prolonged bleeding time

72
Q

Medical Management ITP​

A

Primarily Supportive​; depends on platelet count

Anti D Antibody- Plasma derived immunoglobulin. One dose over 5-10 minutes IV. Causes transient anemia by clearing the antibody coated RBCs.​

Intravenous Immune Globulin (IVIG).​

Course of Steroids (1-2 weeks)​

Splenectomy and IV Gamma Globulin if chronic

73
Q

Nursing Interventions ITP​

A

Educate Parents​

Assess for bleeding​
- hematomas
- bruising
- bleeding gums
- tarry stools
- hematuria
- obvious bleeding.
Watch for intracranial bleed:
- irritability,
- change in level of consciousness,
- head ache,
- slurred speech.​

Safety measures to prevent trauma​
Soft toothbrush, restrict activity at the onset until platelet count is up,

Avoid unnecessary procedures, needle sticks, suppositories, rectal temps. etc.​

Apply pressure for 5 minutes after venipuncture.​

Follow-up care in home or clinic

74
Q

Nursing Care for Epistaxis

A

Seek medical attention for a nose bleed lasting longer than 30-minutes​

Sit up leaning forward​

Apply pressure for 10-minutes​

Apply ice bag to bridge of nose.

75
Q

Hemophilia

A

A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors

76
Q

Types of hemophilia

A

A and B

77
Q

Hemophilia A

A

Classic hemophilia​

Deficiency of factor VIII​

Accounts for 80% of cases of hemophilia​

Occurrence—1 in 5000 males

78
Q

Hemophilia B​

A

Also known as Christmas disease​

Caused by deficiency of factor IX​

Accounts for 15% of cases of hemophilia

79
Q

Etiology of Hemophilia A

A

X-linked recessive trait​

Males are affected​

Females may be carriers​

Degree of bleeding depends on amount of clotting factor and severity of a given injury​

Up to one third of cases have no known family history​
- In these cases disease is caused by a new mutation

80
Q

Clinical manifestations: Hemophilia A

A

Bleeding tendencies range from mild to severe​

Symptoms may not occur until 6 months of age​
- Mobility leads to injuries from falls and accidents​

Hemarthrosis​
- Bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobility​

Ecchymosis

Epistaxis

Bleeding after procedures​
- Minor trauma, tooth extraction, minor surgeries​
- Large subcutaneous and intramuscular hemorrhages may occur​
- Bleeding into neck, chest, mouth may compromise airway

81
Q

Early signs of hemarthrosis

A

stiffness, tingling, or ache followed by decreased mobility, warmth, redness, swelling, & severe pain.

82
Q

Medical management of hemophilia

A

Prevent Bleeding​
- infant-padded crib.
- Home; no sharp corners, clutter free, soft carpeted floors, use protective equipment,
- avoid contact sports and risky activities​

Replacement of deficient factor​
- Factor VIII concentrate​
- purified from large plasma pools or synthetic, long shelf life. Powder that can be mixed just prior to IV administration. Blocks aldosterone so potassium supplements not needed.​
or
- DDAVP (desmopressin)​
- Synthetic form of vasopressin, increases factor VIII production. Intranasal.​

8-10 yo can self-administer

Inhibitors- FEIBA (Anti Inhibitor Coagulant Complex) can cause blood clots​

Regular program of exercise & PT
- helps to keep muscles & joints stronger so they have fewer bleeds.​

83
Q

Nursing Interventions Hemophilia : Measures to prevent bleeding

A

safe environment​

teach the use of protective equipment​

avoid aspirin​

SQ rather than IM, venipuncture rather than finger stick, no suppositories, adolescents need electric razors, avoid contact sports.​

Soft toothbrush

84
Q

Nursing Interventions Hemophilia : Recognize and Control Bleeding

A

Pressure X 15 minutes for frank bleeding​

RICE ; Rest (Immobilize), Ice, Compression, elevation​

Children are aware of internal bleeding and can tell you. ​

Be suspicious of head ache, slurred speech, altered LOC, black tarry stools.

85
Q

Nursing Interventions Hemophilia: Prevent side effects of bleeding;

A

long term effects of bleeding into joints & muscles = incomplete re-absorption of blood & limited ROM results in bone & muscle changes, flexion contractures, & joint fixation.

active ROM after the acute phase​

physical therapy​

nutritional counseling

86
Q

Nursing Interventions Hemophilia: Family Support and Home Care

A

Med Alert bracelet​

Notify school nurse and teacher.

87
Q

von Willebrand Disease (vWD)​

A

A hereditary bleeding disorder involving deficiency of von Willebrand factor (a plasma protein and the carrier for factor VIII)​

von Willebrand factor needed for platelet adhesion​

Transmitted as autosomal dominant trait​

Occurs in both males and females​

The gene for the disease is located on chromosome 12

88
Q

von Willebrand Disease (vWD)​ clinical manifestations

A

Easy bruising​

Epistaxis​

Gingival bleeding​

Excessive bleeding with lacerations or surgeries​

Menorrhagia

89
Q

Diagnosis—Laboratory Findings (vWD)

A

Decreased von Willebrand factor levels​

von Willebrand antigen levels ​

Decreased platelet agglutination​

Prolonged bleeding time ​

PTT may be normal or prolonged​

90
Q

von Willebrand Disease (vWD)​ treatment

A

Infusion of von Willebrand protein concentrate​

DDAVP infusion before surgery, during menses, or to treat bleeding episode

91
Q

von Willebrand Disease (vWD)​ nursing inverventions

A

Avoid aspirin or NSAIDs (increase bleeding time and inhibit platelet function)​

Manage bleeding episodes with prompt infusion therapy ​

Children with vWD have normal life expectancy if well managed

92
Q

Henoch-Schönlein Purpura (HS Purpura)​

A

Occurs in ages 6 months to 16 years ​

Generalized vasculitis of dermal capillaries ​

Extravasation of RBCs, produces petechial skin lesions ​

Inflammation and hemorrhage in GI tract​

May lead to ongoing nephrotic syndrome​

93
Q

Henoch-Schönlein Purpura (HS Purpura)​ etiology

A

unknown; often follows URI

94
Q

Therapeutic management/Nursing of HS Purpura​

A

Most cases resolve without treatment​

Corticosteroids and anticoagulants for severe or persistent cases​

Symptomatic treatment​

Maintain hydration​

Monitor renal function

NUR 340 exam 1 (1 decks)

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