1. The Child with a Cardiovascular/Hematologic Disorder
Chapter 37:
The Child with a Cardiovascular/Hematologic Disorder

2. The Child with Cardiovascular/Hematologic Disorders
The cardiovascular system carries needed chemicals to and from the cells in the body so they can function properly. The major organ is the heart.
The hematologic system includes the blood and blood- forming tissues.
The cardiovascular and hematologic systems work together to remove the waste products from the cells so that they can be excreted from the body.

3. The Child with ardiovascular/Hematologic Disorders (cont.)
At Birth both the right and left ventricles are about the same size.
By the time a child is a few months of age, the left ventricle is about two times the size of the right, about the same proportion as an adult.
The infant's heart rate is higher so that the cardiac output can provide adequate oxygen to the body;than the older child's or adult’s.
By the time the child is 5 years old, the heart has matured, developed, and functions just as the adult's.

5. Congestive Heart Failure
Congestive heart failure (CHF) occurs when blood and fluids accumulate in the organs and body tissues. This accumulation happens because the heart is not able to pump and circulate enough blood to supply the oxygen and nutrient needs of the body cells.
The signs and symptoms seen in the child with CHF often include fatigue; feeding problems; failure to gain weight; pale, mottled, or cyanotic color; tachycardia; rapid respiration; dyspnea; flaring of the nares; and use of accessory muscles with retractions.
Such children may also have edema, heart enlargement, and liver enlargement.

6. Congestive Heart Failure (cont.)
Treatment includes improving the cardiac function, removing excess fluids, decreasing the workload on the heart, and improving tissue oxygenation.
Cardiac Glycosides-Digoxin
Diuretics-Lasix
ACE inhibitors-captopril-vasodilator
Nursing care is focused on improving cardiac output and oxygenation, relieving inadequate respirations, maintaining adequate nutritional intake, and conserving energy.

7. Rheumatic Fever
Group A beta-hemolytic streptococcus is the bacterium usually responsible for rheumatic fever.
Major manifestations include carditis (inflammation of the heart), polyarthritis (migratory arthritis), and chorea (disorder characterized by emotional instability, purposeless movements, and muscular weakness).

8. Rheumatic Fever
The onset is often slow and subtle. The child may be listless, anorectic, and pale. He or she may lose weight and complain of vague muscle, joint, or abdominal pains. Often there is a low- grade late afternoon fever.
Nursing care for the child with rheumatic fever is focused on reducing pain, providing diversional activities and sensory stimulation, conserving energy, and preventing injury.
As long as the rheumatic process is active, progressive heart damage is possible, so bed rest is essential for the child with rheumatic fever to reduce the heart’s workload.
In addition, teach the caregiver to comply with drug therapy and the long-term care of the child.

9. Rheumatic Fever
Laboratory tests:Two commonly used indicators are the ESR and the presence of C-reactive protein.
The ESR is elevated in the presence of an inflammatory process and is nearly always increased in the polyarthritis or carditis manifestation of rheumatic fever.
ESR elevation in a choreic client may indicate cardiac involvement.
C-reactive protein in the body indicates an inflammatory process is occurring. It appears in the serum of acutely ill people, including people ill with rheumatic fever. As the client improves, C-reactive protein decreases or disappears.
Medications used in the treatment of rheumatic fever include penicillin, salicylates, and corticosteroids. Penicillin is administered to eliminate the hemolytic streptococci.

10. 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

11. 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.

12. Kawasaki Disease
Kawasaki disease is an acute, febrile disease seen in boys younger than 5 years old.
The disease appears to be caused by an infectious agent.
Intravenous immunoglobulin therapy is given to relieve the symptoms and prevent coronary artery abnormalities.
Aspirin is used to control inflammation and fever.
The most serious concern for a child with Kawasaki disease is development of cardiac involvement, which may not be seen for a period of time after the child's recovery.

13. Kawasaki Disease Clinical Manifestations
An elevated temperature 102°F to 104°is noted from the first day of the illness and may continue 1 to 3 weeks.
Irritability;
lethargy;
inflammation in the conjunctiva in both eyes;
strawberry-colored tongue;
dry, red, cracked lips; edema in the hands and feet;
and red, swollen joints are seen.
The skin on the fingers and toes peels in layers, and a rash covers the trunk and extremities.

14. Kawasaki Disease Cervical lymph nodes may be enlarged.
Inflammation of the arteries, veins, and capillaries occurs, and this inflammation can lead to serious cardiac concerns, including aneurysms and thrombus.
The child may report abdominal pain.
The disease occurs in three stages:
• Acute—high fever that does not respond to antibiotics or antipyretics; child is irritable.
• Subacute—fever resolves, irritability continues; greatest risk for aneurysms.
• Convalescent—symptoms are gone; phase continues until laboratory values are normal, and child’s energy, appetite, and temperament have returned.

15. Iron-Deficiency Anemia
Iron-deficiency anemia is a common nutritional deficiency in children.
is common between the ages of 9 and 24 months.
A child needs to absorb 0.7 to 1.5 mg of iron per day.
It is difficult to get enough iron from food the child eats, and if the iron intake is inadequate, anemia quickly results.
Clinical Manifestations
The signs of iron-deficiency anemia include below- average body weight, pale mucous membranes, anorexia, growth retardation, fatigue, lethargy and listlessness, in addition to the characteristics of milk babies described earlier. Concave or “spooning” fingernails may be seen

16. Iron-Deficiency Anemia
Foods High in Iron
• Baby cereals fortified with iron.
• For older children, fortified instant oatmeal and cream of wheat are good sources of iron.
• Some infant formulas are iron fortified.
• Egg yolks are rich in iron. Avoid egg whites for young children because of allergies.
• Green, leafy vegetables are good sources of iron.
• Dried beans, dried peas, canned refried beans, and peanut butter provide good iron sources for toddlers and older children.
• Fruits that are iron rich include peaches, prune juice, and raisins (do not give to a child younger than 3 years of age because of danger of choking).

17. Sickle Cell Disease
In sickle cell anemia, the abnormal hemoglobin causes the red blood cells to assume a sickle shape.
When sickling occurs, the affected red blood cells become crescent-shaped and the blood viscosity increases (blood becomes thicker), causing slowdown and sludging of the red blood cells.
The impaired circulation results in tissue damage and infarction.

18. Inheritance patterns. A. Heterozygous type
Inheritance patterns. A.Heterozygous type. One parent carries a hemoglobin S gene, and one does not. Two children will be free of the gene (AA), and two will be carriers (AS). B. Homozygous type. Each parent is carrying one hemoglobin A gene and one hemoglobin S gene. One child is free of the gene (AA), two are carriers (AS), and one has sickle cell disease (SS).

19. Sickle Cell Disease (cont.)
Nursing care for the child with sickle cell anemia is focused on maintaining comfort and relieving pain, increasing fluid intake, conserving energy, improving physical mobility, and maintaining skin integrity.
Remind the family caregivers that fluid intake is important, and intake should be maintained at 1,500 to 2,000 mL when the child is not in crisis.
Work closely with the caregivers to decrease anxiety and increase their knowledge about the causes of crisis episodes.

20. Thalassemia
blood disorders are inherited mild-to-severe anemias in which the hemoglobin production is abnormal.
Clinical Manifestations: Anemia, fatigue, pallor, irritability, and anorexia are noted in children with thalassemia. Bone pain and fractures are seen.
The skin may appear bronze colored or jaundiced. Skeletal changes occur, including deformities of the face and the skull. The upper teeth protrude, the nose is broad and flat, and the eyes are slanted.
Common complications of thalassemia include enlargement of the spleen, overstimulation of bone marrow, and heart failure.
Prognosis remains poor, and the child often dies of cardiac failure despite treatment with blood transfusions (to maintain the hemoglobin levels), diet and medications (to prevent heart failure), and splenectomy or bone marrow transplants.

21. Hemophilia
Hemophilia is a syndrome of several distinct inborn errors of metabolism; all result in the delayed coagulation of blood.
Defects in protein synthesis lead to deficiencies in any of the factors in the blood plasma needed for thromboplastic activity.
The most common types of hemophilia are factor VIII deficiency and factor IX deficiency, which are inherited as a sex-linked recessive trait with transmission to male offspring by carrier females.

22. Hemophilia (cont.)
Hemophilia used to be treated by the use of fresh blood or plasma, but newer commercial preparations are now available that supply higher-potency factor VIII, including a synthetic preparation, DDAVP (1-deamino-8-D-arginine vasopressin), which is used in mild factor VIII deficiencies.
Nursing care is focused on stopping the bleeding, decreasing pain, increasing mobility, and preventing injury.
Work with the caregiver to increase knowledge about the child's condition and care and to help the family learn to cope.

23. Idiopathic Thrombocytopenic Purpura
Purpura is a blood disorder associated with a deficit of platelets in the circulatory system causing hemorrhages into the skin or mucous membranes. The most common type of purpura is idiopathic thrombocytopenic purpura (ITP). Purpura is preceded by a viral infection in about half of the diagnosed cases.
Symptoms of idiopathic thrombocytopenic purpura include bruising, a generalized rash, and in severe cases, hemorrhage in the mucous membranes, hematuria, or difficult-to-control epistaxis.
Corticosteroids are useful in reducing the severity and shortening the duration of the disease in some but not all cases of ITP. Intravenous immunoglobulin has been used to increase the production of platelets until recovery occurs spontaneously.
Nursing care consists of protecting the child from falls and trauma, observing for signs of external or internal bleeding, and providing a regular diet and general supportive care.

24. Acute Leukemia Leukemia, the most common type of cancer in children
Acute lymphoblastic leukemia (ALL) is responsible for about 70% to 75% of the childhood leukemias and acute myelogenous leukemia (AML) is responsible for almost all the rest.
ALL is also the most curable of all major forms of leukemia. The incidence of ALL is greatest between the ages of 2 and 5 years and is higher in boys
The child with leukemia often has fatigue, pallor, low- grade fever, bone and joint pain, petechiae (pinpoint hemorrhages beneath the skin), and purpura (hemorrhages into the skin or mucous membranes).
The lymph nodes may be enlarged and bruising is a constant problem.

25. Acute Leukemia (cont.)
Four drugs commonly used in the treatment of acute lymphatic leukemia are methotrexate, vincristine, prednisone, and 6-mercaptopurine.
Nursing care is focused on preventing infection, preventing injury, relieving pain, and reducing fatigue.
Work with the child to help promote normal growth and development and improve body image.
Encourage caregivers to verbalize feelings and help them to increase their coping abilities.