Chapter 31- Care of Child with a Physical Disorder

1. Chapter 31- Care of Child with a Physical Disorder Jessica Gonzales RN, MSN

2. Cardiovascular assessment Assess height And weight, Growth failure Can occur with Sever cardiac disease Monitor apical and peripheral Pulses for rate, rhythm, and quality Auscultate for extra heart sounds Periorbital edema Engorged Neck veins Monitor respirations for rate and effort Ausculate for adventitious sounds Monitor BP for hypo or hypertension Abdominal distension Palpate for Hepatomegaly And splenogmegaly cyanosis clubbing • Peripheral edema • Palpate • inspect

3. Congenital Heart Disease Etiology and pathophysiology: • Family history of CHD • Mom comes in contact with certain substances during first few weeks of pregnancy • Mom with seizure disorder and on meds • Depression and lithium • Uncontrolled diabetes or lupus • Rubella • Chromosomal abnormalities (downs syndrome, turners) • infection

4. 1. Inferior and superior vena cavafrom • bodyintoright atrium 2. Right atriumtoright ventricle via • tricuspid valve 3. Throughpulmonary valveto • pulmonary artery 4. Pulmonary arteryto lungs 5. Topulmonary veinsfromlungs 6.Pulmonary veinsto left atrium 7.Throughmitral valveinto left ventricle 8.Through aortic valve to aorta 9. Tobody Left atrium Superior vena cava Right atrium mitral aortic pulmonic Left ventricle tricuspid Tissue Paper My Assests R u i o I l t r C m r t U o a i S n l c P i I c D Right ventricle Inferior vena cava

5. Types of defects: • Pulmonary Blood flow • Pulmonary Blood Flow • Obstruction to systemic blood flow • Mixed blood flow • Cyanotic • Acyonotic

6. Cyanotic Acyanotic

7. 4 T’s • Tetralogy of fallot • Truncus Ateriosus • Transportation of • the great vessels • Tricuspid Atresia Cyanotic R L • PDA • ASD • VSD R L Acyanotic

8. Clinical manifestations • Cyanosis • pallor • Cardiomegaly, • additional heart sounds (pericardial rubs, murmurs,) • Discrepancies between apical and radial pulses • Tachypnea • Dyspnea, grunting, crackles, and wheezes • Digital clubbing • Hepatomegaly, splenomegaly

9. Acyanotic Increased Pulmonary Blood flow Patent ductus arteriosus (PDA) is a condition in which a blood vessel called the ductus arteriosus fails to close normally in an infant soon after birth. (The word "patent" means open.) https://health.google.com/health/ref/Patent+ductus+arteriosus

10. Acyanotic Increased pulmonary blood flow Atrial septal defect (ASD) is a congenital heart defect in which the wall that separates the upper heart chambers (atria) does not close completely. Congenital means the defect is present at birth.

11. acyanotic Increased pulmonary blood flow Ventricular septal defect (VSD)describes one or more holes in the wall that separates the right and left ventricles of the heart. Ventricular septal defect is one of the most common congenital (present from birth) heart defects. It may occur by itself or with other congenital diseases.

12. A large ventricular septal defect(VSD): a hole in the part of the septum that separates the ventricles, the lower chambers of the heart. The hole allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood from the right ventricle. An overriding aorta: the aorta is between the left and right ventricles, directly over the VSD. As a result, oxygen-poor blood from the right ventricle flows directly cyanotic Decreased Pulmonary Blood flow Overriding aorta Pulmonary stenosis Opening between ventricles Right ventricular hypertrophy Right ventricular hypertrophy : This defect occurs if the right ventricle thickens because the heart has to pump harder than it should to move blood through the narrowed pulmonary valve. Pulmonary stenosis : This defect is a narrowing of the pulmonary valve and the passage through which blood flows from the right ventricle to the pulmonary artery. In pulmonary stenosis, the heart has to work harder than normal to pump blood, and not enough blood reaches the lungs.

13. The precise mechanism of these episodes is in doubt presumably results from a transient In resistance to blood flow to the lungs with flow of desaturated blood to the body characterized by a sudden, marked, increase in cyanosis followed by syncope, and may result in hypoxic brain injury and death, prolonged crying, irritability treatment: Calm infant- hold over shoulder or in knee chest position or have child squat (increases pressure on the left side of the heart, decreaseing the R to L shunt thus decreasing the amount of deoxygenated blood entering systemic circulation) Morphine (to decrease spasm and supress resp center) Oxygen (it is a potent pulmonary vasodilator and systemic vasoconstrictor. This allows more blood flow to the lungs) Consider sedation and parlaysis with intubation if these measures fail Children with TOF may develop "tet spells“ (acute hypoxia)

14. Increased Pulmonary blood flow cyanotic Transposition of the great vessels is a congenital heart defect in which the two major vessels that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched (transposed).

15. Normal pulmonary blood flow Acyanotic Aortic coarctation is a narrowing of part of the aorta (the major artery leading out of the heart). It is a type of birth defect. Coarctation means narrowing

16. Hematologicalassessment Jaundice, sclera, retinal hemorrhage Impaired thought Process or lethargy Gingival pallor or bleeding Tachycardia Auscultate for murmurs Lymphadenopathy or tenderness Tachypnea, orthopnea, dyspnea Abdominal tenderness, Hepatomegaly, splenomegaly Joint swelling, Bone and joint tenderness Blood in urine and abnormal Mentsraul bleeding Decreased muscle mass Pallor,flushing Jaundice, Purpura, Petichiae, Scratch marks cyanosis Palpate decreased cap fill time

17. Disorders of Hematological Function • Anemia: The condition of having less than the normal number of red blood cells or less than the normal quantity of hemoglobin in the blood. The oxygen-carrying capacity of the blood is, therefore, decreased

18. Iron containing O2 transport protein that carries O2 from the lungs to the body Failure to produce (hem)oglobin due to lack of iron In anemia selective vasoconstriction of blood vessels allows nonvital areas to be bypassed to allow more blood to flow into critical areas. The skin is one of the areas to be considered “nonvital” and the result is pallor. Iron needed to bind O2 Reduces O2 carrying capacity of the blood Tissue hypoxia= ↑cardiac input= ↓PVR & ↓blood viscosity (thinner blood) = tachycardia and heart murmur O2 state to the tissues: dyspnea on exertion, fatique, fainting, lightheadedness, tinnitus, headache

19. A genetic disorder characterized by an abnormal form of hemoglobin within the erythrocyte ↓ O2 = sickle shaped red blood cells break apart not acting effectively Ischemia in the small blood vessels and infarction in the small bones Damaged sickle RBC’s clump together and stick to the walls of blood vessels, blocking blood flow causing sever pain and permanent damage to brain, heart, lungs, kidneys, liver, bones, and spleen ↑Risk of infection due to damaged spleen from sickled cells getting trapped Ischemia in the small blood vessels and infarction in the small bones

20. Aplastic Anemia is a rare but potentially life threatening syndrome of bone marrow failure characterized by pancytopenia ↓RBC’s fatigue due to ↓O2 infections Bruising and bleeding

21. Iron replacement therapy • Nutritional or dietary counseling • Treatment of underlying cause Failure to produce hemoglobin due to lack of iron broad spectrum antibiotics • Infection • Pain • Fatigue • Shortness if breath Pain medications, local heat application ** hydration to prevent sickling • Pallor • Dyspnea • Petechiae • bleeding • Fever • Infection Administer O2, semi-fowlers postion Good oral hygiene, patient safety • Transfusions as needed • Isolation precautions per institute (reverse isolation) Prophylactic antibiotics

22. Idiopathic thrombocytopenic purpura (bleeding in the tissue) platelets bleeding A bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. Persons with the disease have too few platelets in the blood *antiplatelet antibody in the spleen • Prevent bruising • Control bleeding • Counsel family to not use salicylate drugs • Transfusion of RBC’s • IV gamma globulin and anti-D antibody therapy • splenectomy

23. Hemophillia • Hereditary (x-linked recessive transmitted by females found predominately in males) bleeding disorder characterized by deficincy in a blood clotting factor (*factor VIII{A} or IX {B}) Prednisone: decreases antiplatelet antibodies IVIG Bleeding into the tissue Minimize bleeding platelets Bruising and petichiae Anti D antibody

24. Disorders of Hematological Function Leukemia -ALL (acute lymphoblastic leukemia) uncontrolled proliferation of blast cells,which accumulate in the marrow causing crowding and depression of other cells • Hodgkins disease- This is a malignant lymphoma distinguished by painless, progressive enlargement of lymphoid tissue.

25. Assessment of the Immune System Conjunctival redness Temperature for hyperthermia Auscultate for tachycardia Palpate for adenopathy Palpate for spleneomegaly Auscultate for abnormal Breath sounds Assess the joints for Swelling, redness, Tenderness, decreased mobility Inspect skin for hives, edema, lesions

26. Disorders of the Immune System • Symptoms • may not • Appear for • 1 to 2 yrs • Nonspecific • clinical manifestations Infection with HIV produces Lymphopenia resulting in immunosupression and AIDS • Prevent opportunistic infections • Administer prophylactic therapy • for P. carnii (co-trimoxazole) beginning • at 6 mos of age • Immunizations • Pulmonary hygiene • Promote adequate nutrition • Foster healthy growth and development

27. Disorders of the Immune System • Clinical manifestations: • Daily afternoon temperature spikes • macular rash on trunk and extremities • joint involvement- swelling, pain, redness • Medical management • Nonsteroidal anti-inflammatory drugs • antirheumatic drugs • cytotoxic drugs • corticosteroids

28. Assessment of the Respiratory System Observe for Alertness, change In mental status Temperature for hyperthermia Auscultate for abnormal Breath sounds Monitor respirations for rate, depth, and quality, Note any dyspnea, use of accessory muscles Percuss for dullness which indicates fluid Intercostsal, suprasternal, Sternal and substernal retractions Chest diameter Inspect skin color changes, especially cyanosis

29. Disorders of the respiratory system Bronchopulmonary Dysplasia Premature lungs needing mechanical ventilation (high 02 and PIP) can injure the aveolar Saccules and lead to fibrosis of these structures • Long term O2 therapy • Cyanosis when breathing RA • Manifestations of right sided failure • Administer medications: bronchodilators, diuretic • Planned rest periods to decrease respiratory effort and • conserve energy • Small frequent meals to prevent over distension of stomach • Counsel parents in ways to prevent respiratory infection • Teach parents CPR

30. Disorders of the respiratory system • pneumonia Acute inflammation of the lung parenchyma (bronchioles, alveolar ducts, and sacs, and alveoli) • Respiratory • Distress • Wheezing, crackles • Use of accessory • muscles Impairs gas exchange • Antibiotics if bacterial • Assess for respiratory distress • Provide family teaching

31. Disorders of Respiratory Function-Bronchitis/Bronchiolitis • Assess for • respiratory • Distress • Contact isolation • Prescribed • Medications (RT) • O2 if needed • Fluids • Wheezing • Crackles • Tachypnea • Retractions Viral infection of the lower respiratory tract characterized by inflammation of the Bronchioles and production of mucous (usually caused by RSV)

32. Asthma is a chronic, reversible, obstructive airway disease, triggered by various stimuli Inflammation and edema of muscle (spasms) • Wheezing • Use of accessory • muscles Production of thick mucosa resulting in increased airway resistance, premature closure Of airways, hyperinflation, increased work of breathing, impaired gas exchange • Increased RR • Cough • Fatigue • Anxiety • dyspnea • Assess respiratory status • Administer prescribed meds • Promote adequate O2 • Fowler’s position

33. Disorders of the respiratory system • Respiratory distress syndrome- mainly caused by a lack of a slippery, protective substance called surfactant, which helps the lungs inflate with air and keeps the air sacs from collapsing. Common in premature babies whose lungs are not fully developed. • Sudden infant death syndrome • Acute pharyngitis (sore throat)-inflammation of the pharynx • Tonsillitis • Croup – inflamation of the larynx (voice box)** • Acute epiglotitis –bacterial infection of t he epiglottis • Pulmonary tuberculosis-chronicbacterial infection caused by bacillius mycobacterium tuberculosis • Cystic fibrosis- an inherited disorder of the exocrine glands characterized by excessive thick mucous that obstructs the lungs and GI tract

34. Assessment of the GI System Measure height And weight for growth failure Inspect mouth For caries, periodontal Disease, lesions, And clefts Palpate hard and soft palates for defects Temperature for hyperthermia Inspect abdomen for distention, depression, umbilical herniation Auscultate to assess bowel sounds (do first) Palpate for tenderness, rigidity, masses and organomegaly Inspect the anus for rectal bleeding and nonpatency Inspect skin for pallor, jaundice, carotenimia

35. Disorders of Gastrointestinal Function Cleft lip and cleft palate are birth defects that affect the upper lip and roof of the mouth. They happen when the tissue that forms the roof of the mouth and upper lip don't join before birth. The problem can range from a small notch in the lip to a groove that runs into the roof of the mouth and nose. This can affect the way the child's face looks. It can also lead to problems with eating, talking and ear infections. • Ensure adequate intake of food and fluids without aspiration. • Special feeding devices may be used. • Frequent burping • is necessary. • Assist parents in dealing with the diagnosis Treatment usually is surgery to close the lip and palate. Doctors often do this surgery in several stages. Usually the first surgery is during the baby's first year. With treatment, most children with cleft lip or palate do well.

36. Disorders of Gastrointestinal Function-constipation/dehydration The passage of hardened stools; may be associated with failure of complete evacuation of the colon with defecation • Add fluid or carbohydrate to the formula, add foods with bulk, and increase fluid intake. • Manually dilate the sphincter; administer mild laxatives/enemas. • Obtain history of bowel patterns • educate on dietary changes and normal stool patterns.

37. Disorders of Gastrointestinal Function- diarrhea/gastroenteritis • May be a result of a number of disease processes that cause abnormal losses through the skin, respiratory, renal, and GI systems –vomiting/diarrhea • Diarrhea- A disturbance in intestinal motility characterized by an increase in frequency, fluid content, and volume of stools • Assess for clinical manifestations of dehydration. • Observations should include I&O; vital signs; body weight; skin color, temperature, and turgor; capillary refill; presence or absence of the sensation of thirst; and in infants, assessment of the fontanels. • I&O, promotion of rehydration, correction of electrolyte imbalances, provision of age-appropriate nutrition, prevention of the spread of the diarrhea, prevention of complications, support of the child and family

38. Disorders of Gastrointestinal Function- • Gastroesophageal reflux • Hypertrophic pyloric stenosis • Intusseception • Hirschprungs disease The backflow of gastric contents into the esophagus resulting from relaxation or incompetence of the lower esophageal sphincter Pyloromytomy: Relieves obstruction Narrowing of pyloric sphincter at the outlet of the stomach Telescoping of one portion of the Intestine into an adjacent portion Causing an obstruction Congenital anomaly characterized by absence of nerves to a section of the intestine causing inadequate mobility Which leads to the absence of propulsive movements, causing accumulation of intestinal contents and distention of bowel

39. Umbilical Femoral Inguinal Hiatal Diaphragmatic Disorders of Gastrointestinal Function-hernias Usually closes by the time the child is 3 years old A protrusion of the bowel through an abnormal opening in the abdominal wall Most common in children Surgical repair

40. Assessment of the GU System Measure height And weight for growth failure Uremic encephalopathy- Lethargy, poor concentration, confusion Temperature for hyperthermia Ear abnormalities Monitor RR for abnormal rate and depth of respiration Monitor blood Pressure for hypo Or hypertension Abdominal distension Palpate kidneys for Tenderness, and enlargemnt Bladder For distension Hypospadias, epispadias Inspect the anus for rectal bleeding and nonpatency Inspect skin for peripheral cyanosis, slow cap refill time, pallor, peripheral edema

41. Disorders of Genitourinary Function- • UTI- characterized by inflammation, usually of bacterial origin, of the urethra, bladder, ureters, or kidneys • Nephrotic syndrome- characterized by proteinuria, hypoalbuminemia, hyperlipidemia, altered immunity and edema. Increased permeability to protein, protien leaks through the glomerular membrane resulting in albumin in the urine. Once albumin is lost colloidal osmatic pressure decreases permiting fluid to escape from the intravascular spaces to the intirstial spaces. The volume decrease stimulates antidiuertic hormone to reabsorb water = edema. • Acute glomerulonephritis- antibodies interact with antigens that remain in the glomeruli, leading to immune complex formation and tissue injury, filtration decreases and excretion of less Na and H2O. High Blood pressure, edema, and heart failure may result. • Wilm’s tumor- • Structural defects of gu tract

42. Palpate hair & nails Assessment of the EndocrineSystem Measure height And weight for growth Failure, plot size of head Lethargy, poor concentration, confusion, irritability Assess vision Monitor pulse Increase= hyperthyroid Decrease=hypothyroid Auscultate to note for murmurs Facial abnormalities, mouth for abnormal odors and Dental delay’s Monitor blood Pressure for hypo Or hypertension Assess for sexual development Assess muscle Strength and tone Inspect skin for color changes, hirsutism, easy bruising Palpate to note dryness, coldness, changes in texture

43. Disorders of Endocrine Function A chronic condition Characterized by inadequate amount of thyroid hormone to meet metabolic needs. Congenital- T4 is not produced which is essential for growth and development especially brian development, left untreated = MR. Acquired- inadequate amount of T4 • Hypothyroidism • Hyperthyroidism • Diabetes mellitus • 3 p’s • Polydipsia • Polyuria • Polyphagia A chronic metabolic disorder that results from either a partial or complete deficiency in insulin. Type 1- characterized by beta cell destruction, leading to absolute insulin deficiency. Type II- insulin resistance, progressive deterioration of Insulin secretion

44. Assessment of the Musculoskeletal System Measure height And weight for growth Inspect posture and gait Palpate spine to assess curvature Palpate boney Structures for tenderness, Masses, lesions Assess, muscle mass, tone Observe for structural abnormalities Asymmetrical limbs

45. Disorders of Musculoskeletal Function Scoliosis Legg-Calvé-Perthes Disease • Developmental Dysplasia of the Hip Surgery to correct A spinal deformity that usually Involves lateral curvature of the Spine, spinal rotation, and thoracic Kyphosis (hunch back) • A disorder caused by decreased blood supply to the femoral head; results in epiphyseal necrosis and degeneration • A developmental abnormality • of the femoral head, the acetabulum, • or both; subluxation of the hip

46. Disorders of Musculoskeletal Function Osteomyelitis Talipes (Clubfoot) • Congenital deformity of the foot and ankle • Varies in severity; may involve one foot or both feet • Manipulation and application of a series of short leg casts; changed weekly to allow for further manipulation • Infection within the bone • In children, the metaphysis of the femur, • the tibia, and the humerus are the areas • most affected. • It can occur at any age; the peak incidence • in children is between ages 3 and 15 years, • and boys are affected twice as often as girls.

47. Disorders of Musculoskeletal Function Septic Arthritis • A sex-linked inherited • disorder characterized by • gradually progressive • skeletal • muscle wasting and • weakness Joint aspiration and surgical irrigation Broad-spectrum IV antibiotics • Duchenne’s Muscular Dystrophy Fractures • An infection of a joint, • which can occur from • bacteria in the blood or • as a direct extension of • an existing infection Most common sites in children are long bones, clavicles, wrists, fingers, and skull. No effective treatment

48. Assessment of the Neurological System Assess LOC, Cerebullar status- gait Balance and coordination Measure head size, Palpate fontanels Cranial nerve function Esp pupillary response, Taste, olfaction, and tactile sense Tachycardia Increased ICP Hypertension Increased ICP Assess reflexes Assess muscle tone and strength

49. Disorders of Neurological Function Meningitis An infection of the meninges that is usually caused by bacterial invasion and less Common by viruses. The bacteria Enter the meniges through the blood stream and spread through the csf. Children under 2- poor feeding, irritability And lethargy, high pitched cry, bulging Fontanel, fever, resistance to being held, Opisthotonos (hyperextension of the Neck) • Check for neurolical signs • and monitor LOC • Administer prescribed meds • (antibiotic, steroid for cerebral • Edema, anticonvulsant) • Keep room quite and decrease • Environmental stimuli Older children- respiratory or GI problems, nuchal rigidity (stiff neck), HA, kernigs sign, bruzinski sign, petichial rash

50. Disorders of Neurological Function Occurs with a number of anomalies A condition caused by an imbalance in the production and absorption of CSF In the ventricular system. When production exceeds absorption, CSF Accumulates, usually under pressure and Produces a dilation of the ventricles. Communicating hydrocephalus- an impaired Absorption of CSF in the arachnoid space Noncommunicationg hydrocephalus- obstruction to the flow of CSF through the ventricular system Increased ICP- HA, emesis, irritability, Lethargy, apathy, and confusion