Download The Child With a Cardiovascular/Hematologic Disorder

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Describe the cardiovascular and hematologic systems and how they function.
Discuss ways the child’s cardiovascular and hematologic system differs from the adult’s
system.
Discuss congestive heart failure, including care of the child with CHF.
Name the bacterium usually responsible for the infection that leads to the development of
rheumatic fever.
List the major manifestations of rheumatic fever and describe the nursing care.
Explain Kawasaki disease and state the most serious concern for the child with Kawasaki
disease.
Discuss iron deficiency anemia and identify the common causes.
Explain how (a) sickle cell trait and (b) sickle cell anemia are inherited.
Describe the shape of the red blood cell and the effect it has on the circulation in sickle cell
anemia.
Discuss the nursing care for the child with sickle cell anemia.
Discuss the common complications and prognosis for the child with thalassemia.
Name the most common types of hemophilia and state how they are inherited.
Discuss the treatment and nursing care for the child with hemophilia.
Describe the symptoms noted in the child with idiopathic thrombocytopenic purpura.
Explain the diagnosis of leukemia including the symptoms, treatment, and nursing care
The Child With Cardiovascular/Hematologic
Disorders
 Cardiovascular & Hematologic Systems-work together
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to remove the waste products from the cells, so they can
be excreted from the body
Cardiovascular system: major organ is the heart;
carries needed chemicals to & from the cells so they can
function properly
Hematologic system: includes blood & blood forming
tissues
Usually serious
Many are hereditary and often present at birth
May be chronic or long-term conditions
The Child With Cardiovascular/Hematologic
Disorders
 Differences between child’s and adult’s systems
-at few months of age, the left ventricle is about
two times the size of the right (about the same
proportion as an adult)
-infant’s heart rate is higher than an older child’s
or adult’s
-by 5 years of age, the heart has matured, and
functions just as the adult’s
CONGESTIVE HEART FAILURE
QUESTION
When does Congestive Heart Failure
occur?
Why does this happen?
CONGESTIVE HEART FAILURE
 Clinical manifestations-differs by age
infants: subtle, fatigability, feeding problems,
tires easily, breathes hard, expiratory grunt
older child: failure to gain weight, weakness,
fatigue, restlessness, tachypnea, dyspnea
infants & older child-tachycardia
 Diagnosis-clinical symptoms are the primary basis for
diagnosis; chest xray-enlarge heart; EKG-ventricular
hypertrophy; ECHO-may be done to note cardiac
function
 Treatment-improving cardiac function using
glycosides (digoxin), removing excess fluid (diuretics),
decreasing workload on heart (limiting physical
activity), improving tissue oxygenation
CONGESTIVE HEART FAILURE (CONT.)
 Nursing process for the child with congestive heart
failure
 Assessment-interview the family (current illness, and
previous episodes); problems with feeding, rapid or
difficult respirations, episodes of turning blue; physical
exam of the child with complete vital signs: apical pulse,
respiratory status, skin and extremity color, signs of
fatigability or increase of symptoms with exertions
 Selected nursing diagnosis-page 806
 Outcome identification and planning-major goals
include improving cardiac output & oxygenation, relieving
inadequate respirations, adequate nutrition; family goals
include increasing understanding & prognosis
CONGESTIVE HEART FAILURE (CONT.)
 Nursing process for the child with congestive heart failure
(cont.)
 Implementation
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Monitoring vital signs
Improving respiratory function
Maintaining adequate nutrition
Promoting energy conservation
Providing family teaching
 Evaluation: goals and expected outcomes
Rheumatic Fever
 Chronic disease of childhood, affects the connective
tissue of the heart, joints, lungs, & brain
 Autoimmune reaction to group A beta-hemolytic
strep infections: strep throat, tonsillitis, scarlet fever or
pharyngitis which may be undiagnosed or untreated
 Clinical manifestations-minor or major onset is
often slow and subtle; listless; anorectic; pale; vague
muscle, joint or abdominal pains; late afternoon fever
(none of these is diagnostic by itself)
 Major manifestations-(page 808)
 Carditis-inflammation of the heart, major cause of
permanent heart damage & disability among children
with rheumatic fever; signs may be vague enough to be
missed: poor appetite, low grade fever, listlessness, or
moderate anemia
RHEUMATIC FEVER cont’d
 Carditis cont’d-acute carditis-may be the presenting
symptom; abrupt onset of high fever; tachycardia; pallor; poor
pulse quality; rapid decrease in hemoglobin;
weakness; prostration; cyanosis, & intense precordial
pain
 Polyarthritis-AKA: migratory arthritis (moves from one
joint to another); painful to touch or movement; hot &
swollen; does not lead to disabling deformities
 Chorea-emotional instability; purposeless movements;
muscular weakness; onset is gradual with increasing
incoordination, facial grimaces, & repetitive involuntary
movements; movements may be mild or increasingly severe;
active arthritis is rarely present when chorea is the major
manifestation; carditis occurs (less commonly than when
polyarthritis is the major condition)
RHEUMATIC FEVER cont’d
 Diagnosis: difficult to diagnose & sometimes impossible
to differentiate from other diseases; Jones criteria (Fig. 372) divides criteria into major and minor
 Treatment: Chief concern is the prevention of residual
heart disease; bed rest-may be from 2 weeks to several
weeks, depends on degree of carditis present; digitalis,
restricted activities, diuretics, low sodium diet; lab tests:
ESR & C-reactive protein-used to help guide treatment;
medications: Penicillin, salicylates, corticosteroids (these
do not alter the course of the disease, but control of toxic
manifestations)
 Prevention-importance of teaching the public about the
need to have URI evaluation for group A strep and
treatment
Rheumatic Fever (cont.)
 Nursing process for the child with rheumatic fever
 Assessment-observe any minor & major manifestations;
elevated temp, pulse, erythema marginatum, subQ
nodules, swollen, or painful joints , or signs of chorea
 Selected nursing diagnoses-page 809
 Outcome identification and planning: reducing pain,
providing diversional activities ….
 Implementation
 Preventing infection
 Promoting skin integrity
Rheumatic Fever (cont.)
 Nursing process (cont.)
 Implementation
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Providing comfort measures and reducing pain
Providing diversional activities and sensory stimulation
Promoting energy conservation
Rheumatic Fever (cont.)
 Implementation (cont.)
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Preventing injury
Promoting compliance with drug therapy
Providing family teaching
 Evaluation: goals and expected outcomes
Question
In a child with a diagnosis of congestive heart failure,
what would chest radiographs show?
a. Pigeon chest
b. Enlarged heart
c. Lung infiltrates
d. Flail chest
Answer
b. Enlarged heart
Rationale: The clinical symptoms are the primary basis for
diagnosis of CHF. Chest radiographs reveal an enlarged
heart; electrocardiography may indicate ventricular
hypertrophy, and an echocardiogram may be done to
note cardiac function.
Kawasaki Disease
 Clinical manifestations-acute, febrile disease most
often seen in boys younger than 5 years old; etiology
unknown, but appears to be caused by an infectious
agent; late winter & early spring; major concern is
development of cardiac involvement; elevated temp (102104 F)from 1st day of illness-may continue 1-3 weeks;
irritability, lethargy, rash on trunk & extremities; skin
peels in layers; strawberry colored tongue, edema of
hands and feet; red, swollen joints; inflammation of the
arteries, veins, & capillaries, this leads to serious cardiac
concerns (aneurysms, thrombus); occurs in three stages:
acute, sub-acute, convalescent (page 811)
Kawasaki Disease
 Diagnosis-must have an elevated temp and four of the
following symptoms: cervical lymphadenopathy;
conjunctivitis; dry, swollen, cracked lips; strawberry
tongue; aneurysm; abdominal pain; peeling of hands
and feet; trunk rash; or red, swollen joints. The WBC
and ESR are elevated during the sub-acute stage, the
platelet count increases (this may lead to blood
clotting and cardiac problems); EKG shows cardiac
involvement
 Treatment and nursing care-IV immunoglobulin
therapy is given to relieve symptoms and prevent
coronary artery abnormalities, ASA to control
inflammation and fever and is continued for as long as
1 year in lower doses as an antiplatelet
Kawasaki Disease cont’d
 Nursing care-management of symptoms; monitor
temperature, cardiac status, I&O, daily weight; offer
extra fluids and soft foods; provide mouth and lip care;
encourage passive ROM exercises that increase joint
movement; promote rest…page 812
 Discuss follow up treatments, visits, medication
routines, and side effects.
 Most children recover without long-term effects, but
the cardiac involvement may not be seen for a period
of time after recovery
Iron Deficiency Anemia
 Clinical manifestations-common nutritional
deficiency in children ages 9-24 months; hypochromic,
microcytic anemia (blood cells are deficient in
production of hemoglobin an are smaller than normal;
10% of dietary iron is absorbed (good diet needs 8-10 mg
of iron); “milk babies”; many children with iron
deficiency anemia are undernourished because of family
finances, knowledge deficit about nutrition
 Diagnosis-blood tests that measure hemoglobin (<11g/dl
or a hematocrit <33% g/dl); stool is tested for OB
 Treatment and nursing care-improved nutrition,
ferrous sulfate administered between meals with juice;
teaching caregivers; an iron-dextran mixture (Imferon)
Sickle Cell Disease
 Clinical manifestations-hereditary trait most commonly in
African Americans; production of abnormal hemoglobin that
causes the RBC’s to assume a sickle shape; asymptomatic trait
when inherited from 1 parent; if inherited from both parents
the child has sickle cell disease and anemia develops. Hbg (69 g/dl); tired, poor appetite, pale mucous membranes; sickle
cell crisis is the most severe manifestation-severe, acute
abdominal pain (enlargement of the spleen); muscle spasms;
fever; severe leg pain (muscular, osseous), or localized in the
joints (hot & swollen); several days after-become jaundiced;
could have a fatal outcome (cerebral, cardiac, or hemolytic)
 Diagnosis-Sickledex (page 822)
 Treatment-prevention of crises is the goal; hydration; rest;
oxygen; pain control
Sickle Cell Disease (cont.)
 Nursing process for the child with sickle cell disease
 Assessment-page 822
 Selected nursing diagnosis-page 822
 Outcome identification and planning-page 822
 Implementation
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Relieving pain
Maintaining fluid intake
Promoting energy conservation
Improving physical mobility
Promoting skin integrity
Promoting family cooping
Providing family teaching
Thalassemia
 Clinical manifestations-inherited mild to severe
anemias; major (Cooley’s anemia) presents in
childhood and is the most common (most often in
Mediterranean descent); anemia; fatigue; pallor;
irritability; and anorexia; bone pain and fractures;
 Treatment and nursing care-blood transfusions,
diet; medications to prevent heart failure; and
splenectomy or bone marrow transplants may be
necessary. Prognosis is poor even with treatment; the
child often dies of cardiac failure
Hemophilia
 Oldest know hereditary diseases
 Mechanics of clot formation-page 824, occurs in 3
stages
 Two Common types of hemophilia
 Factor VIII deficiency
 Factor 1x deficiency
Hemophilia (cont’d)
• Factor VIII Deficiency
 Includes: hemophilia A, antihemophilic globulin
deficiency and classic hemophilia
 Classic hemophilia is inherited as a sex-linked recessive
(transmission to affected males by carrier females)
 Hemophilia A (classic) 0ccurs in about 1 in 10,000
people
 The most severe
Hemophilia (cont’d)
 Factor IX Deficiency includes: Hemophilia B, plasma
thromboplastin component deficiency and Christmas
disease
 Accounts for about 15% of the hemophilias
 Is a sex-linked recessive trait appearing in male
offspring of carrier females
 Caused by a deficiency of one of the necessary
thromboplastin precursors
Hemophilia (cont’d)
 Clinical Manifestations:
 Prolonged bleeding, frequent hemorrhages externally
and into the skin, joint spaces, and intramuscular
 Bleeding from tooth extractions, brain hemorrhages,
and crippling disabilities are serious complication
 An infant/toddler bruises easily, serious hemorrhages
may result from minor lacerations
Hemophilia (cont’d)
 Diagnosis:
 Family history and type of bleeding present
 Abnormal bleeding beginning in infancy and a positive
family history suggests hemophilia
 Marked prolonged clotting time (severe Factor VIII or
IX deficiency)
 Slightly prolonged clotting time may be a mild
conditions
 Partial Prothrombin time demonstrates that Factor VIII
is low
Hemophilia (cont’d)
 Treatment:
 Only treatment in the past was fresh blood or plasma,
or fresh frozen plasma
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Helpful in emergencies, requires large volumes
FFP-must be administered within 30 minutes, Factor VIII
loses its potency at room temperature
 Commercial preparations
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Supply higher potency Factor VIII
Longer “shelf” life
Disadvantage-exposes the recipient to a large number of
donors (Hep B, HIV
Hemophilia (cont.)
 Nursing process for the child with hemophilia
 Assessment
 Selected nursing diagnosis (page 826)
 Outcome identification and planning
Hemophilia (cont.)
 Implementation
 Relieving pain
 Preventing joint contractures
 Preventing injury
 Providing family teaching
 Promoting family coping
 Evaluation: goals and expected outcome
Idiopathic Thrombocytopenic
Purpura (ITP)
 Clinical manifestations:
 Onset is often acute
 Bruising and a generalized rash
 Hemorrhage may occur into mucous membranes,
hematuria, or epistaxis (in severe cases)
 Diagnosis:
 Platelet count may be 20,000/mm3 or lower
 Bleeding time is prolonged
 Clot retraction time is abnormal
ITP (cont’d)
 Treatment:
 Corticosteroids to reduce the severity and shorten the
duration in some cases
 Intravenous immunoglobulin to increase the number of
platelets
 Nursing Care:
 Protect the child from falls and trauma
 Observe for signs of external or internal bleeding
 Provide regular diet and general supportive care
Acute Leukemia
 Most common type of cancer in children-accounts for
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about 30% all childhood cancers
Acute lymphoblastic leukemia (ALL) accounts for about
70-75%; Acute myelogenous leukemia (AML) accounts for
almost all the rest
ALL is the most curable of all major forms of leukemia,
AML cure rate is about 40%
Incidence of ALL is greatest between 2 and 6 years, higher
in boys
ALL more common in white children
Acute Leukemia
 Pathophysiology (rest of discussion focuses on ALL):
 Leukemia is the uncontrolled reproduction of deformed
WBCs (monocytes, granulocytes, lymphocytes)
 Cause is unknown
 Lymphocytes reproduce so quickly (mostly in the blast
or immature stage)
 This rapid increase causes crowding, which decreases
the production of RBCs and platelets
 This decrease in RBCs, platelets, and normal WBCs
causes: extreme/easily fatigued and susceptible to
infection and increased bleeding
Acute Leukemia
 Clinical Manifestations:
 Symptoms appear with abruptness, often without
warning signs
 Presenting signs and symptoms caused by anemia
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Fatigue
Pallor
Low-grade fever
Bone and joint pain-caused by invasion of periosteum
Wide spread petechiae & purpura-results from low
thrombocyte count
 Other signs: anorexia, nausea & vomiting, headache,
diarrhea, abdominal pain (during the course of the
disease)-as a result of enlargement of liver & spleen
Acute Leukemia
 Diagnosis:
 History, symptoms and labs
 Bone marrow aspiration MUST be done to confirm the
diagnosis
 Radiographs of the long bones demonstrate changes
caused by the invasion of the lymphoblasts
Acute Leukemia
 Treatment:
 Advances in the treatment of ALL have dramatically
improved long-term survival
 If the initial prognosis is good, about 90% have longterm survival
 Relapses reduce survival rates greatly
 Initial treatment
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3 phases of intensive chemotherapy: induction, sanctuary,
maintenance (page 829)
Combination of drugs
 Relapses
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2 additional phases: reinduction, bone marrow transplant
Acute Leukemia (cont.)
 Nursing process for the child with leukemia
 Assessment-page 829
 Selected nursing diagnosis
 Outcome identification and planning
Acute Leukemia (cont.)
 Nursing process for the child with leukemia (cont.)
 Implementation
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Preventing infection
Preventing bleeding and injury
Reducing pain
Promoting energy conservation and relieving anxiety
Promoting a positive body image
Promoting family coping
 Evaluation: goals and expected outcomes
Question
What disease is a sex-linked recessive trait caused by a
deficiency of factor IX?
a. Hemophilia B
b. Hemophilia A
c. Factor VIII disease
d. Christmas disease
Answer
d. Christmas disease
Rationale: Christmas disease is a sex-linked recessive trait
appearing in male offspring of carrier females, and it is
caused by a deficiency of one of the necessary
thromboplastin precursors: factor IX, the plasma
thromboplastin component.