Care of the Child with a Physical Disorder

Chapter 31

Congenital Heart Disease

• 5-10% of newborns have congenital heart disease.

• Environmental factors include: intrauterine rubella exposure, maternal alcoholism, Diabetes, advanced maternal age, and maternal drug ingestion.

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• Genetic risk factors include: a sibling or parent with a congenital heart disease, chromosomal anomalies ( Down’s syndrome, Trisomy 13-15, and Turner’s syndrome ), and the presence of other non-cardiac congenital anomalies.

Types of Congenital Heart Defects

• Increased pulmonary blood flow• Decreased pulmonary blood flow• Obstruction to systemic blood flow• Mixed blood flow

Signs and symptoms

• Cyanosis, pallor• Cardiomegaly, murmurs• Tachypnea, dyspnea, grunting• Digital clubbing• Crackles, wheezes• Differences between upper and lower

extremity blood pressures

Diagnostic tests

• Urine culture, ABGs• Electrocardiogram, echocardiogram• Angiography, cardiac catheterization• Cardiac magnetic resonance imaging (MRI)

Increased pulmonary blood flow(1)

• Most common CHD• Communication exists between right and left

side of the heart.• The increased pressure on the left side of the

heart pushes blood BACK to the Right side of the heart.

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• Most common defects are: patent ductus arteriosis (PDA), atrial septal defect (ASD), and ventricular septal defect (VSD).

Patent Ductus Arteriosis

• Ductus arteriosis is a fetal artery that connects the pulmonary to the Aorta.

• Failure to close within the first few weeks allows blood to shunt from high-pressure areas to the low-pressure pulmonary artery.

Signs and symptoms

• Small PDAs may be asymptomatic• Large PDAs may exhibit signs of heart failure.• Other signs include a machine-like murmer,

widened pulse pressure, and bounding pulses.

Treatment

• Indomethacin – effective in closing PDAs • Surgical ligation (closure)• New surgical technique uses a thorascope to

place a clip on the PDA.

Atrial Septal defect

• An abnormal opening in the atrial septum, allowing blood to flow from the higher pressure LEFT atrium to the lower-pressure RIGHT atrium.

Signs and symptoms

• Some may be asymptomatic• Some have S/S of heart failure• Characteristic harsh systolic murmur

Treatment

• Open heart surgery• Small defects are repaired with sutures• Large defects may be repaired with a Dacron

patch.

Ventricular Septal Defect

• Abnormal opening in the intraventricular septum.

• Blood flows from the LEFT ventricle(oxygenated blood) to the Right ventricle(unoxygenated blood).

• Size can range from a pin-hole to the absence of the entire septum, leaving a single ventricle.

Signs and symptoms

• Initially may asymptomatic• Signs of heart failure eventually occur• Loud, harsh systolic murmur • Palpable thrill

Treatment

• 50% of VSDs close spontaneously close• 50% require open-heart surgery, usually

making repair with a Dacron patch.

Defects with Decreased Pulmonary blood Flow(2)

• Results when there is some type of obstruction to the lungs or if there is no connection between the right side of the heart and the lungs.

• Examples are pulmonary stenosis, pulmonary atresia, and tetralogy of Fallot.

• Most common CHD in this category is tetralogy of Fallot.

Tetralogy of Fallot

• A combination of four defects: (1)pulmonary stenosis, (2)VSD, (3)right ventricular hypertrophy, and (4)overriding aorta

Signs and symptoms

• Profound cyanosis at birth• Acute spells of severe cyanosis and

hypoxia(blue spells).• Systolic ejection murmur, clubbing of nailbeds,

dyspnea, squatting, poor growth, mental slowness, syncope(fainting) and stroke.

Treatment

• A pallative, temporary procedure done on infants: Blalock-Taussig shunt redirects blood back to the lungs for oxygenation.

• Complete surgical correction involves closure of the VSD, a pulmonary valvotomy and reapir of the overriding aorta.

Mixed Defects(3)

• Include the mixing of oxygenated and unoxygenated blood in the heart or great vessels.

• Examples include transposition of the great vessels, truncus arteriosus, and hypoplastic left heart syndrome.

Transposition of the Great Vessels

• The pulmonary artery arises from the left ventricle and the aorta arises from the right ventricle.

• This causes venous blood returning to the right side of the heart to exit from the aorta without being oxygenated, and oxygenated blood returning from the pulmonary system is returned via the pulmonary artery to the lungs.

Signs and symptoms

• Profound cyanosis• Infants born with other defects such as PDA,

ASD, or VSD may have less cyanosis, but will have signs of heart failure.

• Cardiomegaly is usually present on x-ray.

Treatment

• Pallative treatments that encourage mixing of the blood properly.

• Complete correction involves open heart surgery and switching the great vessels back to their proper position.

Defects with Obstruction to Systemic Blood Flow(4)

• The blood is unable to reach the body from the heart.

• Show symptoms of lack of oxygenation, decreased peripheral blood flow, and symptoms of heart failure because the blood backs up into the lungs.

• Also includes aortic stenosis, pulmonary stenosis, and coarctation of the aorta.

Coarctation of the Aorta

• A narrowing of the lumen of the aorta(usually at the site of the ductus arteriosus.

• Results in increased pressure proximal to the defect(head and upper exremities) and decreased pressure distal to the defect(body and lower extremities)

Signs and symptoms

• Blood pressure in the arms will be 20 mm hg higher than in the legs(reverse of normal).

• Other signs include: bounding pulses in the lower extremities, signs of heart failure, leg cramping on exertion in older children, and epistaxis.

Treatment

• Surgical correction to remove the narrowed portion of the aorta with an end-to-end anastomosis or graft replacement if the narrowing is extensive.

Blood disorders

• Majority of blood disorders seen in children stem from chronic systemic illness, nutritional deficits, and inherited blood disorders.

Iron Deficiency Anemia

• Most common blood disorder in infancy and early childhood, peaks between 6-24 months.

• Prevalence has decreased due to participation in WIC.

• Preemies are especially at risk due to their reduced fetal iron supply.

• Adolescents are at risk due to rapid growth rates and poor eating habits.

Etiology

• Anemia is a decrease in red blood cell(RBC) volume, a decrease in hemoglobin or both.

• Classified as either hypo-proliferative(defective erythrocyte production)or hemolytic(premature destruction of erythrocytes)

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• Iron deficiency anemia becomes apparent at about 6 months of age in the term infant and about 3 months of age in the preemie, when their maternal stores of iron become depleted.

• Most infants with iron deficiency anemia are underweight, however babies fed exclusively on milk can become anemic( “milk babies”).

Signs and symptoms

• Mild to moderate anemia(Hg: 6-10g/dL)vague, non-specific symptoms including irritability, weakness, decreased play activity, and fatigue.

• When Hg falls below 5g/dL, will see anorexia, pallor, pale mucus membranes, inability to concentrate, tachycardia, and systolic murmurs. With chronic anemia will see growth retardation and developmental delays.

Diagnostic Tests

• Diagnostic evaluation including diet history, appetite, activity, weight and rate of growth, and any recent blood loss.

• Lab tests include a CBC and reticulocyte count, and a serum ferritin( a major iron storage protein).

Treatment

• Iron replacement therapy • Nutritional counseling: drinking too much

milk may cause iron deficiencies; iron-fortified food like whole grains, fortified cereals and bread products, leafy green vegetables, dried fruits. Meat, fish, poultry, eggs, and legumes.

• Treating underlying conditions(hemorrhage or mal-absorption conditions).

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• Iron replacement therapy: Fer-in sol(ferrous sulfate). Citrus helps aid iron absorption. Caution!!! As little as 6-12 iron tablets ingested at once by a child can be toxic.

• Breast-feeding, iron-fortified formulas, and iron-rich solid foods are recommended.

Sickle Cell Anemia

• A group of diseases called hemoglobinopathies, in which Hg(HgA) is partly or completely replaced by abnormal sickly hemoglobin(HbS).

• A genetic disorder.• Most common in afro-Americans• Occurs in 1:400-500 live births.

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• Classified as either sickle cell trait or sickle cell disease.

• Sickle cell trait rarely causes disease symptoms

• Sickle cell disease is the most severe and potentially fatal form of the disorder.

Sickle Cell Etiology

• When oxygen is released into the tissues, the abnormal Hg becomes more viscous and crystalline which causes the erythrocyte to assume an elongated crescent or sickle shape. As the sickle cells clump together, this causes obstruction causing circulation to slow with sever tissue hypoxemia and necrosis. It is an intermittent condition.

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• Can be precipitated by infection, fever, hypoxemia, dehydration, high altitudes, cold or emotional stress.

• Greatest risk is in children under the age of 5 years.

• Most deaths are caused by overwhelming infection.

• Chronic illness with a potentially fatal outcome.

Signs and symptoms

• Starts with pallor, irritability, and jaundice.• May see growth below average levels.• Cardiomegaly and heart failure can devlop in

response to hypoxia.• Older children may have edematous joints.• Strokes are a risk.• Acute events are called sickle cell crises.

Diagnostic tests

• Newborn screening is mandatory in the U.S.• Sickledex is a sickle-turbidity test and gives

results in 3 minutes. If positive, a hemoglobin electroporesis is done to determine sickle cell trait or sickle cell disease.

Nursing Interventions

• Maintaining adequate hydration to prevent further sickling.

• Administering analgesics for pain as ordered.• Providing adequate oxygenation.• Promoting comfort measures.• Teach families to avoid infection, dehydration,

emotional stress, and strenuous physical activity. Medic-alert bracelets are recommended.

Aplastic Anemia

• Failure of the bone marrow to form blood components.

• Results in sever anemia, leukopenia, and thrombocytopenia.

• May be caused by exposure of the bone marrow to toxic chemicals, ionizing radiation, or antibiotics or other medications.

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• Diagnosed by bone marrow aspiration.• Treated by either immunosuppressive therapy

or bone marrow transplant(69% 15-year survival rate)

Coagulation Disorders

• Hemophilia: a serious bleeding disorder inherited as an X-linked recessive gene.

• Transmitted by female carriers and affects 1: 10,000 male births.

• Classic hemophilia is caused by a deficiency of Factor VIII( a necessary component of blood coagulation).

Signs and symptoms

• Diagnosis usually made after infancy.• Increasing episodes of oral bleeding and

bruising.• By age 4, 90% have demonstrated persistent

bleeding from minor lesions.• Most frequent type of internal bleeding is in

the joints(hemathrosis) and muscles. As bleeding persists pain grows.

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• Persistent hemarthrosis can lead to bone deformities with contractures.

• Intracranial hemorrhage can be life-threatening and cause more deaths than any other type of bleeding.

• Hematomas in the spinal cord can cause paralysis.

Diagnostic tests

• PT, INR, and PTT• Assay tests in specialized labs tests for Factors

VIII and IX.

Treatment

• For minor bleeding use pressure and cold packs.

• For more serious bleeding Factor VII may be given.

• Analgesics are prescribed for pain.• Blood products are often prescribed for

bleeding, and the child should be given the hepatitis B vaccine .

Nursing Interventions

• Teach family to avoid injury and to control bleeding. Use anticipatory guidance to avoid injuries.

• Early dental visits are recommended.• Always wear a medic-alert bracelet.• Inform the family about the National

Hemophilia foundation as a resource.• Teach RICE: rest, ice, compression,& elevation.

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• Encourage parents to be observant, yet not over-protective.

• Encourage parents to seek emergency care for any major or persistent bleeding, signs of intracranial bleeding such as: severe headache, slurred speech, vomiting, disorientation, and loss of consciousness.

Prognosis

• Progress over the last two decades has been tremendous.

• It has no cure, but it is controllable.• Unfortunately those hemophilia patients who

were treated prior to the purification techniques of Factor VII(1979-1985) were exposed to HIV. Estimated that 50% of these patients have sero-converted and have AIDS.

Idiopathic Thrombocytopenia Purpura