Congenital Heart Disease and Cardiac Conditions

CARDIO CONGENITAL HEART DISEASE Acyanotic diseases Pulmonary circulation and systemic circulation are not connected If there is a connection, the pressure is higher in the left side than in the right side PATENT DUCTUS ARTERIOSUS Most common congenital heart defect Symptoms depend on size of the vessel and age of the child May have no symptoms; indication may be a murmur Increasing dyspnea, full bounding pulse, wide pulse pressure Spontaneous closure after infancy rarely occurs Without treatment – life expectancy short ATRIAL SEPTAL DEFECTS 10% of all forms of congenital heart disease Allows oxygenated blood returning from the lungs to pass into the right atrium VENTRICULAR SEPTAL DEFECT constitute 20% of all forms of CHD allows systemic venous and oxygenated arterial blood to mix may produce no symptoms at all require no specific treatment and often close spontaneously COARCTATION OF THE AORTA narrowing of the lumen of the aorta may be an isolated defect or associated with other cardiac malformation Assessment: measure BP in both arms and a leg and to assess the pulse in both upper and lower extremities surgical repair – only permanent treatment; usually deferred until 3 years of age PULMONIC VALVE STENOSIS usually do not produce symptoms; typical murmur AORTIC VALVE STENOSIS mild to moderate asymptomatic; typical murmur CYANOTIC DISEASES TETRALOGY OF FALLOT VSD Overriding of the aorta Pulmonary valve stenosis Enlarged right ventricular wall Assessment: Primary sign – cyanosis Hypoxic spells – usually initiated by crying Fainting – due to cerebral hypoxia Stunted growth, clubbed fingers and toes Squatting position – characteristic position to relieve dyspnea Implementation Decrease hypoxic spells – do not permit child to cry Place in knee chest position O2 as needed TRANSPOSITION OF THE GREAT VESSELS aorta arises from the right ventricle degree of cyanosis depends on the abnormal connections Assessment: cyanotic at birth develop polycythemia risk for emboli and thromboses may develop CHF or pulmonary vascular obstructive Implementation: require emergency medical treatment cardiac catheterization ballool atrial septostomy TRICUSPID ATRESIA condition in which tricuspid valve did not form no opening between the right atrium and right ventricle 2% of congenital heart defects profound cyanosis and dyspnea at birth emergency catheterization with balloon atrial septotomy TOTAL ANOMALOUS VENOUS RETURN condition in which all the pulmonary venous blood returns to the heart cyanosis and severe respiratory distress emergency surgical intervention TRUNCUS ARTERIOSUS single vessel arising from the ventricles just above a large VSD retarded growth; enlarged liver and heart usually infants die within the first year CARDIOVASCULAR DEFECTS Monitor vital signs closely Monitor respiratory status Auscultate breath sounds for crackles, ronchi or rales if respiratory effort is increased, place child in reverse Trendelenburg position administer humidified oxygen Monitor for hypercyanotic spells: Place infant in a knee chest position Administer 100% oxygen by mask Administer morphine as ordered IVF as prescribed Assess for signs of CHF Assess peripheral pulses Keep child stress free as possible; allow maximal rest CARDIAC SURGERY Postoperatively: monitor for signs of discomfort monitor for signs of sepsis (fever, lethargy, diaphoresis, altered LOC) Monitor lines, tubes or catheters ; remove promptly administer pain medications; note effectiveness encourage rest periods facilitate parent-child contact as soon as possible Home Care omit activities in which child could fall for 2-4 weeks avoid crowds for 2 weeks after discharge no added salt diet do not put creams, lotions or powders on the incision site child may return to school 3rd week after discharge no physical education for 2 months follow up after 2 weeks avoid immunizations, invasive procedure and dental visits for 2 months advise parents regarding importance of dental visit every 6 months inform dentist of cardiac problem instruct parents to call MD if with coughing, tachypnea, cyanosis, diarrhea CONGESTIVE HEART FAILURE inability of the heart to pump sufficiently to meet the metabolic demands of the body infants – most commonly caused by congenital heart defects combination of both left sided and right sided heart failure goal of treatment: to improve cardiac function, remove accumulated fluid and sodium, decrease cardiac demands, improve tissue oxygenation Assessment: tachycardia tachypnea profuse scalp sweating especially in infants fatigue and irritability sudden weight gain respiratory distress Implementation: monitor vital signs closely and for early signs of CHF monitor for respiratory distress monitor I and O; weigh diapers Monitor daily weight to assess for fluid retention; weight gain of 0.5 kg ( 1 lb /day) monitor for facial or peripheral edema , auscultate lung sounds elevate HOB maintain neutral thermal environment to prevent cold stress in infants administer cool, humidified oxygen organize nursing activities to allow uninterrupted sleep maintain adequate nutritional status provide rest; decrease environmental stimuli feed when hungry and soon after awakening infant should be well rested before feeding provide small, frequent feedings administer sedation administer digoxin check with physician parameters for witholding digoxin Note that infants rarely receive more than 1 mL (50 ug or 0.05 mg) of digoxin Administer diuretics; monitor for hypokalemia administer potassium supplements monitor serum electrolytes restrict fluid in acute stages check with physician regarding sodium restriction; infant formulas have slightly more sodium than does breast milk Home care Instructions for Administering Digoxin administer 1 hr before or 2 hrs after feeding do not mix medication with food or drink if the child vomits, do not administer a second dose if more than 2 consecutive doses have been missed, notify MD; do not increase or double the dose for missed doses keep the medication in a locked cabinet if the child becomes ill, notify MD RHEUMATIC FEVER an inflammatory autoimmune disease affects connective tissue of the heart, joints, subcutaneous tissues and blood vessels of the CNS most serious complication – RHD affecting the cardiac valves presents 2-6 weeks following an untreated Group A beta hemolytic strep Jones criteria Asssessment: signs of carditis: SOB, edema of the face, abdomen or ankles, precordial pain signs of polyarthritis: edema, inflammation of the large joints, joint pain erythema marginatum: macular rash on trunk and extremities subcutaneous nodules fever elevated ASO elevated ESR elevated CRP Implementation: Assess vital signs Control joint pain and inflammation with massage Febrile phase – provide bed rest limit physical exercise in child with carditis Administer antibiotics (penicillin) as prescribed administer salicylates and anti-inflammatory agents Instruct parents about the importance of follow up and need for antibiotic prophylaxis for dental work, infection, invasive procedures advise child to inform the parents if anyone in school develops a strep throat infection KAWASAKI DISEASE known as mucocutaneous lymph node syndrome acute systemic inflammatory illness unknown cause cardiac involvement – most serious complication Assessment: fever conjunctival injection red throat Swollen hands, rash, enlargement of the cervical lymph nodes Implementation: monitor temperature frequently assess heart sounds and rhythm assess extremities for edema, redness, desquamation monitor mucus membrane for inflammation weigh daily administer IV immune globulin instruct parents in the administration of prescribed meds aspirin – need to monitor bleeding GASTROINTESTINAL SYSTEM Physiology of DIGESTIVE SYSTEM Mechanical functions of digestion immature at birth No voluntary control over swallowing until 6 weeks Stomach capacity decreased Relaxed sphincter contributes to tendency to regurgitate Liver functions immature throughout infancy Gastric acidity low in infants rises until age 10 Digestive processes are mature by toddlerhood Assessment: Presenting problem a. Vomiting b. Abnormal bowel habits: diarrhea, constipation, bleeding c. Weight loss, failure to thrive d. Pain Nutritional history Physical Examination: General appearance: a. Height and weight b. Measure mid arm circumference c. Observe color: jaundice Mouth: dentition Abdomen a. Skin integrity b. Abdominal distension; visible peristaltic waves c. Inspect for hernias d. Auscultate bowel sounds ( every 10-30 seconds) e. Palpate for tenderness f. Liver span (inferior edge palpated 1-2 cm below RCM) g. Spleen (felt on inspiration 1-2 cm below left costal margin) DISORDERS OF THE TEETH DENTAL CARIES erosion of the enamel and dentine of teeth results from combination of fermenting sugars and starched and acid forming organisms Prevention: dental hygiene regular dental hygiene good nutrition fluoridation CLEFT LIP AND CLEFT PALATE 1 in 1000 births multifactorial common among children with chromosomal abnormalities results from failure of the embryonic structures of the face to unite may occur separately or may combine to produce a single unilateral or bilateral cleft from the lip through the soft palate interfere with the child’s capacity to meet oxygenation and nutritional needs may seriously hamper normal bonding process of children with parents Assessment: facial abnormality visible at birth - cleft lip or palate or both, unilateral or bilateral difficulty sucking inability to form airtight seal around nipple formula/milk escapes through nose in infants with cleft palate predispose to infection – communication between mouth and nose difficulty swallowing abdominal distension – air swallowing Management: team approach otolaryngologist, audiologist, pediatrician, dentist, orthodontist, speech therapist Surgical correction: early correction – prevent speech defects Cheiloplasty correction of cleft lip unite edges to allow lips to be both functional; aesthetic reasons performed usually at age 2 months Cleft palate repair not done until age 18 months – anticipation for speech development child should be weaned and able to take liquids from a cup before palate repair NURSING INTERVENTION: Cleft Lip (PreOP) feed in upright position burp frequently press cleft lip together with fingers – to encourage sucking and to strengthen muscles if unable to suck, use a rubber tipped syringe; drip onto side of mouth finish feeding with water to wash away formula in palate area provide small, frequent feedings provide emotional support for parents Post OP: Cleft Lip repair maintain patent airway monitor amount of swallowing to detect hemorrhage do not place in prone position or with pressure on cheeks avoid any tension on suture line avoiding straining on suture line prevent crying keep child comfortable and contented use elbow restraint keep suture line clean – clean after each feeding with saline, peroxide or water to remove crusts pain relief Nursing Intervention: Preop cleft palate repair prepare parents to care fro child after surgery instruct concerning feeding methods and positioning Post Op cleft palate repair position on side for drainage of blood have suction available prevent injury or trauma on suture line Use cups only for liquids; no bottles avoid straws, utensils, popsicle sticks, chewing gum provide soft toys Use elbow and wrist restraints Provide liquid diet initially soft normal diet Give water after each feeding to clean suture line Hold and cuddle ESOPHAGEAL ATRESIA and TRAACHEOESOPHAGEAL FISTULA Esophageal atresia congenital defect; upper segment of the esophagus ends in a blind pouch TEF defect in which embryonic structures fail to divide into a separate esophagus and trachea opening between the two structures usually occur together Assessment: copious oral and nasal secretions –first sign of a defect choke or cough when suctioning or gavage is attempted – catheter cannot pass into stomach Medical and Surgical intervention prevention of aspiration drainage tube may be placed in the blind pouch – to suction secretions esophageal atresia – medical emergency - end to end anastomoses feeding tube maybe inserted into the stomach through gastrostomy until repair heals Nursing intervention: provide nutrition Provide gastrostomy tube feeding until anastomosis site has healed start oral feedings when infant can swallow well promote respiratory function 1. position properly PYLORIC STENOSIS narrowing of the outlet of the stomach caused by excessive growth of circular muscles that surrounds the pylorus hypertrophy develops over 4-6 weeks of life when symptoms begin to appear more common in Caucasian; first born, full term boys Assessment: olive size bulge under right rib cage vomiting – projectile; non bilous peristaltic waves during and after feeding failure to thrive dehydration diagnostic tests: a. UGIS – narrowing of diameter of pylorus b. Decreased serum Na, K, Cl c. Increased Hct d. Metabolic alkalosis Nursing intervention: Preop replace fluids and electrolytes prevent vomiting Give thickened feedings High Fowler’s Place on right side after feeding Minimize handling Strict I and O, daily weights, urine sp gravity Nursing Intervention: post Op Advance diet as tolerated Place on right side after feeding Observe incision for signs of infection Provide client teaching and discharge planning INTUSSUSCEPTION telescoping of bowel into itself ileocecal region edema, necrosis of bowel, obstruction most common at age 6 months more in boys than in girls associated with cystic fibrosis Assessment: piercing cry severe abdominal pain (pulls leg up) vomiting of bile stained fluid bloody mucus in stool “currant jelly” stool Management: 1. barium enema 2. surgery Nursing Intervention: 1. provide routine pre and post op care 2. monitor for peritonitis HIRSCHSPRUNG’S DISEASE absence of autonomic parasympathetic ganglion cells in large intestines results in decreased motility in that portion of the colon and signs of functional obstruction Assessment: failure or delay in passing meconium abdominal distension failure to pass stool temporary relief following rectal exam loose stools – only liquid can pass thru ribbon like stools nausea, anorexia, fecal vomiting weight loss, failure to grow volvulus – bowel twists upon itself diagnostic tests: rectal biopsy – confirms presence of aganglionic cells Nursing intervention: enema as ordered A. Mineral oil or isotonic saline Do not use water or soap suds – water intoxication Use volume appropriate to weight of child infants – 150-200ml children – 250-500 ml administer TPN as ordered Provide low residue diet provide client teaching and discharge teaching Colostomy care Low residue diet IMPERFORATE ANUS during embryonic life – membrane that separates the rectum and anus is absorbed absorption fails to occur -> imperforate anus anus appear as a dimple fistula may be present – rectovaginal, rectourethral Assessment: inspection of anus, check rectum for patency insert rectal thermometer absence of stool during 1st 24 hours development of a distended abdomen Intervention: surgery anoplasty – if possible temporary colostomy if with distension – nasogastric suction Nursing intervention: keep operative site clean observe infant for signs of hypovolemic shock and infection if anoplasty heals – regular dilatation of anus to prevent stricture colostomy care a. Kept clean b. zinc oxide after surgery – side lying or prone with hips elevated CELIAC DISEASE malabsorption syndrome characterized by intolerance of gluten, found in rye, oats, wheat and barley familial disease more common in Caucasians cause unknown flat mucosal surface and atrophy of villi of the intestine reduced absorptive surface -> marked malabsorption of fats Assessment: steatorrhea chronic diarrhea failure to thrive distended abdomen abdominal pain, irritability, listlessness, vomiting symptoms of ADEK deficiency diagnostic tests: 1. pancreatic enzymes 2. jejunal or duodenal biopsies Management: diet therapy gluten free diet TPN in children Nursing Intervention: gluten free diet (BROW – Barley, Rye, Oatmeal, Wheat) supplemental fat soluble vitamins client teaching 1. gluten free diet 2. importance of reading the label 3. avoidance of infection 4. adhering to diet even if symptoms are controlled 5. importance of long term follow up APPENDICITIS inflammation of the appendix ischemia, gangrene, rupture, peritonitis – if untreated school age children – most common due to mechanical obstruction or anatomic defects Assessment: diffuse pain RLQ nausea, vomiting guarding of abdomen rebound tenderness decreased bowel sounds fever diagnostic tests: 1. CBC 2. elevated acetone in urine Nursing Intervention: antibiotics/ antipyretics as ordered no enemas no heating pads routine preop care post op care: semi-Fowler’s position Monitor NGT Monitor penrose drains PARASITISM roundworms, pinworms Assessment: pinworms – anal itching, disturbed sleep roundworms – colic , abdominal pain, lack of appetite, weight loss Nursing intervention: obtain stool culture observe all excreta for worms Scoth tape swab instruct parents to change clothing, bed linens, towels and launder in hot water instruct all family members to scrub hands and fingernails prior to eating and after using toilet DIABETES MELLITUS most common endocrine disease of children onset maybe any age type 1 IDDM risk of complication is high – retinopathy, neuropathy, nephropathy, skin changes, predisposition to infection Assessment: rapid onset polyuria, polydipsia, polyphagia, fatigue weight loss ketoacidosis -Dry flushed skin Management: insulin diet therapy exercise prevention of complications Nursing intervention: administer insulin as ordered force fluids without sugar monitor blood glucose levels daily observe for hypoglycemia: behavior changes, sweating provide client teaching a. Daily regimen for home care b. Urine and blood glucose monitoring c. Nutrition management d. Effects of infection and exercise on CHO metabolism e. Prevention of acute and chronic complications RESPIRATORY SYSTEM EPIGLOTTITIS inflammation of the epiglottis H. influenzae Type b ; Strep pneumoniae age 2-5 abrupt onset; winter considered an emergency situation Assessment: fever sore, red and inflamed throat drooling, difficulty of swallowing inspiratory stridor muffled voice nasal flaring Implementation maintain patent airway assess respiratory status and breath sounds use of accessory muscles; presence of stridor assess temperature do not attempt to visualize the posterior pharynx or obtain throat culture lateral neck film do not force child to lie down do not restrain administer antibiotics; IV fluids cool mist oxygen high humidification have resuscitation equipment available immunization LARYNGOTRACHEOBRONCHITIS (LTB) inflammation of the larynx, trachea, bronchi most common type of croup; maybe viral or bacterial parainfluenza virus gradual onset ; preceded by URI Assessment: fever irritability and restlessness hoarse voice inspiratory stridor and suprasternal retractions crackles and wheezing cyanosis Implementation: patent airway vital signs elevate HOB humidified oxygen; IVF nebulized epinephrine resuscitation equipments available BRONCHITIS infection of the major bronchi assessment: cough, worse at night; becomes productive in 2-3 days Implementation: Monitor for respiratory distress Increased fluid intake Acetaminophen BRONCHIOLITIS/RESPIRATORY SYNCYTIAL VIRUS (RSV) Inflammation of the bronchioles; production of mucus that occludes bronchiole tubes and small bronchi RSV – highly communicable, usually transferred by hands Assessment: URI Lethargy, poor feeding, tachypnea Expiratory wheezes and grunt Diminished breath sounds The child with RSV: isolate in a single room or place with same patient good handwashing nurses caring for these children do not care for other high risk children wear gowns when soiling may occur ribavirin – may be aerosol nurses wearing contact lenses must wear goggles – ribavirin may dissolve contact lenses PNEUMONIA inflammation of the alveoli inhalation of causative agent bloodstream Primary atypical pneumonia – most common cause of pneumonia between 5-12 years Implementation: antimicrobial therapy oxygen cool humidification encourage child to lie on the affected side isolation procedures as needed antipyretics IVF; liberal fluid intake ASTHMA chronic inflammatory disease of airways commonly caused by physical and chemical irritants common symptoms – coughing in the absence of respiratory infection, especially at night Assessment: episodes of wheezing, breathlessness, dyspnea, chest tightness SOB, cough, wheezing child speaks in short, broken phrases retractions exercise induced bronchospasm severe spasm or obstruction Implementation continuously monitor respiratory status administer quick-relief (rescue) medication initiate an IV line; prepare to correct dehydration, acidosis or electrolyte imbalance Medications Quick relief To treat symptoms and exacerbations Short acting b2 agonists Anticholinergics – for relief of acute bronchospasm (ipratropium bromide) Long term control Corticosteroids Long acting b2 agonist Long acting bronchodilator Nebulizer, MDI Used to deliver many medications Non-CFC (chlorofluorocarbon) – albuterol Chest physiotherapy Includes breathing exercises Home care measures: instruct in measures to eliminate allergens avoid extremes of environmental temperature avoid exposure to individuals with a viral infection instruct the child in how to recognize early symptoms instruct the child in the cleaning of devices used for inhaled medications keep immunization up to date OTITIS MEDIA Infection of the middle ear occuring as a result of a blocked eustachian tube, preventing normal drainage common complication of ARI infants and children more prone – ET shorter, wider, straighter Assessment: fever irritability and restlessness rolling of head from side to side pulling or rubbing the ear earache; signs of hearing loss purulent ear discharge otoscopic exam Intervention Encourage fluids upright position when feeding avoid chewing – increases pain have the child lie with the affected ear down instruct on appropriate technique to clean drainage from the ear with sterile cotton swabs administer analgesics and antibiotics (10-14 days) screening for hearing loss otic medications If younger than age 3 – auditory canal is straightened by pulling the pinna down and back If older than 3 years – pull pinna up and back Myringotomy Insertion of tympanoplasty tubes into the middle ear to equalize pressure and keep ear aerated Keep ears dry Earplugs should be worn during bathing, shampooing, swimming TONSILLECTOMY AND ADENOIDECTOMY Assessment: persistent or recurrent sore throat enlarged bright red tonsils ; with exudates at times dysphagia mouth breathing; unpleasant mouth odor fever; cough Preop: assess for presence of infection assess bleeding and clotting studies assess for loose teeth prepare the child for a sore throat Postoperatively: Position – prone or side lying to facilitate drainage have suction equipment available monitor for signs of hemorrhage may give apply ice collar discourage coughing or clearing the throat provide clear, cool, noncitrus and non carbonated drinks avoid milk products intially – will coat the throat avoid red liquids do not give child any straw, forks, or sharp objects administer paracetamol as ordered instruct parents to notify MD if bleeding, persistent earache or fever occurs instruct parents to keep child away from crowds until healing occurred HEMATOLOGIC SYSTEM LEUKEMIA – the most frequent type of childhood cancer Brain tumors – 2nd Etiology: 1. Environmental Viruses Familial/genetic Host factors STAGES OF TREATMENT INDUCTION Goal: to remove bulk of tumor Methods: surgery, radiotherapy, chemotherapy, BM transplant Effects: often the most intensive phase Side effects are potentially life threatening CONSOLIDATION Goal: to eliminated any remaining malignant cells Methods: chemo/radio Side effects will still be evident MAINTENANCE Goal: to keep the child disease free Chemotherapy This phase may last several years OBSERVATION Goal: to monitor the child at intervals for evidence of recurrent disease and complications of treatment Method: treatment is complete Child may continue in this stage indefinitely LATE EFFECTS OF TREATMENT Impaired growth and development CNS damage Impaired pubertal development Development of secondary malignancy Psychologic problems related to living with a life threatening disease and complex treatment regimen NURSING INTERVENTION Help child cope with intrusive procedures Provide information geared to developmental level and emotional readiness Explain what is going to happen, why it is necessary, how it will feel Allow child to handle to handle and manipulate equipments Allow child some control in situations - positioning, selecting injection site Support child and parents Maintain frequent clinical conferences to keep all informed Always tell the truth Acknowledge feelings and encourage child/family to express them Provide contact with another parent or support group Minimize side effects of treatment Skin breakdown Keep clean and dry; wash with warm water; no soaps or creams Do not wash off radiation marks Avoid all topical agents with alcohol Do not use heating pads or hot water bottle Bone marrow suppression Provide frequent rest periods Avoid crowds Evaluate any potential site of infection Monitor temperature Avoid use of aspirin Select activities that are physically safe Nausea and vomiting Administer antiemetic at least half an hour before chemotherapy Eat light meal prior to administration of therapy Administer IVF if needed Alopecia Reduce trauma of hair loss Buy wig before hair falls out Discuss various head coverings Avoid exposing head to sunlight Nutrition deficits Establish baseline Provide high calorie, high protein 5. Developmental delay Facilitate return to school as soon as possible Discuss limit setting, discipline LEUKEMIA most common form of childhood cancer peak 3-5 years proliferation of abnormal wbc that do not mature beyond the blast phase blast cells – infiltrate other organs – liver, spleen, lymph tissue Types: Acute Lymphocytic leukemia (ALL) 80-85% Acute 75% chance of surviving Acute nonlymphocytic leukemia Includes granulocytic and monocytic types 60-80% will obtain remission 30-40% cure rate Assessment: anemia, weakness, pallor, dyspnea Bleeding, petecchiae, spontaneous bleeding, ecchymoses Infection, fever, malaise Enlarged lymph node Enlarged spleen and liver Bone pain Management: diagnosis: blood studies, BMA Treatment stages Induction CNS prophylaxis Maintenance Nursing Intervention: Provide care for the child receiving chemo and radiotherapy Provide support for the family/child Support child during painful procedures Use distraction, guided imagery Allow the child to retain as much control as possible Administer sedation prior to procedure as ordered Sickle Cell Disease hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin (HgbS) HgbS sensitive to changes in the oxygen content of the red blood cell Risk factors: African American Insufficient oxygen causes the cells to assume a sickle shape and the cells become rigid and clumped together, obstructing capillary blood flow The sickling response – reversible under adequate oxygenation Sickle cell crises – vaso-occlusive crisis, splenic sequestration, aplastic crisis Assessment: Vaso-occlusive crisis Most common type of crisis Caused by stasis of blood with clumping of the cells in microcirculation, ischemia and infraction Fever, pain and tissue engorgement Splenic sequestration Pooling of blood in the spleen Profound anemia, hypovolemia, shock Aplastic crisis Caused by the diminished production and increased destruction of RBCs, triggered by viral infection or the depletion of folic acid Profound anemia and pallor Implementation: Administer oxygen and blood transfusions administer analgesics maintain adequate hydration and blood flow with IV normal saline as prescribed and with oral fluids Assist the child to assume a comfortable position so that the child keeps the extremities extended to bed no more than 30 degrees avoid putting strain on painful joints encourage consumption of a high calorie, high protein diet with folic acid supplementation administer antibiotics as prescribed Monitor for signs of increasing anemia and shock (pallor, vital sign changes) IRON DEFICIENCY ANEMIA Iron stores are depleted, resulting in a decreased supply of iron for the manufacture for the manufacture of hemoglobin in RBCs Results from blood loss, increased metabolic demands, syndromes of GI malabsorption, dietary inadequacy Assessment: pallor weakness and fatigue irritability Implementation: Increase the oral intake of iron Instruct the child and parents in food choices that are high in iron Administer iron supplements as prescribed Liquid iron prep stains – stains teeth; with straw side effects of iron therapy APLASTIC ANEMIA a deficiency of circulating erythrocytes resulting from the arrested development of RBCs within the bone marrow causes: exposure to toxic agents, viruses, infection, autoimmune disorders, allergic states definitive diagnosis: BMA Therapeutic management: blood transfusions, splenectomy, corticosteroids, immunosuppressive therapy, bone marrow transplantation Assessment Pancytopenia Petecchiae, purpura, bleeding, pallor, weakness, tachycardia and fatigue Implementation: Blood transfusion corticosteroids and immunosuppresives splenectomy bone marrow transplant Medic Alert bracelet HEMOPHILIA x linked recessive trait Hemophilia A – deficiency of Factor VIII Hemophilia B – deficiency of factor IX Males inherit hemophilia from their mothers and females inherit the carrier status from their fathers Assessment: prolonged bleeding after minor injury At birth after cutting cord Following circumcision Following IM immunization Increase bruising as child learns to crawl and walk abnormal bleeding in response to trauma joint bleeding – pain, tenderness, swelling limited range of motion tendency to bruise easily prolonged PTT normal BT, PT, platelet count Implementation: prepare to administer Factor VIII concentrate /cryoprecipitate Thaw slowly Gently rotate bottle Infuse immediately; deteriorates at room temperature monitor for bleeding monitor for joint pain; immobilize the affected extremity if joint pain occurs assess neurological status ( child at risk for IC bleed) monitor urine for hematuria Control bleeding by immobilization, elevation, application of ice; apply pressure (15 mins) for superficial bleeding instruct parents regarding activities of the child avoidance of contact sports BETA THALASSEMIA MAJOR Cooley’s anemia autosomal recessive disorder characterized by reduced production of one of the globin chains in the synthesis of hemoglobin incidence highest in Mediterranean descent Assessment: severe anemia pallor failure to thrive hepatosplenomegaly microcytic, hypochromic RBCs Implementation Instruct the administration of folic acid (Vit B9) administer blood transfusion as prescribed Monitor for iron overload iron overload – chelation therapy with deferoxamine genetic counseling IDIOPATHIC THROMBOCYTOPENIC PURPURA increased destruction of platelets with resultant platelet count less than 100,000/mm3 characterized by petechiae and ecchymoses of skin exact mechanism unknown often preceded by a viral illness spleen not enlarge Assessment: petechiae: spider web appearance of bleeding under the skin due to small size of platelets ecchymosis blood in any body secretions, bleeding from mucus membranes, nosebleeds diagnostic test: platelet count decreases, anemia Management: steroids and immunosuppressives platelet transfusion surgery; splenectomy Nursing Intervention control bleeding Administer platelet transfusion as ordered Apply pressure to bleeding sites as needed Position bleeding part above heart level if possible Prevent bruising Protect from infection Measure normal circumference of extremities for baseline Administer meds orally, rectally or IV rather than IM; hold pressure on site for 5 mins Avoid aspirin Provide client teaching and discharge planning Pad crib and playpen Provide soft toys Provide protective headgear during toddlerhood Use soft toothbrush Avoid contact sports INTEGUMENTARY SYSTEM ECZEMA atopic dermatitis - often the first sign of an allergic predisposition in a child usually manifests during infancy Asssessment: erythema, weeping vesicles that rupture and crusts severe pruritus; scratching causes thickening and darkening dry skin, sometimes urticaria Intervention: topical steroids antihistamines coal tar preparation colloid baths diet therapy: elimination of offending food Nursing Intervention: avoid heat and prevent sweating check materials in contact with child’s skin (sheets, lotions, soap) avoid frequent baths avoid use of soap provide lubricant immediately after bath administer topical steroids as ordered use cotton instead of wool keep child’s nails short; use elbow or glove restraints if needed apply wet saline or Burrow’s solution compresses DIAPER RASH contact dermatitis plastic/rubber pants and linings of disposable diapers exacerbate the condition by prolonging contact with moist, warm environment skin further irritated by acidic urine Assessment: erythema/excoriation in the perineal area irritability Nursing intervention: keep area clean and dry; clean with mild soap and water after each stool and soon as child urinates take off diaper and expose area to air during the day client teaching: Proper hygiene Avoid use of plastic pants or disposable diapers with a plastic lining Avoid commercially prepared diaper wipes containing alcohols Avoid cornstarch; good medium for bacteria MUSCULOSKELETAL SYSTEM Care of the child with a Cast if cast is of plaster – will remain wet for at least 24 hrs use only the flats of their hands to move children casts must remain open to the air until dry casted extremities are elevated to help blood return and reduce swelling Initial chemical hardening reaction may cause a change in an infant’s body Choose toys too big to fit down cast do not use baby powder near cast – medium for bacteria prepare for anticipated casting by having child help apply cast in a doll Diagnoses and interventions: Potential for alteration in tissue perfusion related to constriction of cast Check color, sensation and motion distal to the cast every half hour Check pedal or radial pulse Check for tightness by slipping finger under edge; if impossible – cast is too tight Ask child to move toes or fingers Elevate casted extremity Potential for alteration in skin integrity Remove plaster flakes from skin Handle wet cast carefully so as not to cause indentations Expose wet cast to air to hasten drying Support heavy cast with sling or pillow to decrease pressure of cast edges Check cast for foul or musty odors Potential for fear and loneliness Encourage expression of feelings Provide diversional play Encourage friends and family to visit children as often Provide educational opportunity for children confined for long periods Potential for knowledge deficit of family Encourage discussion of feelings and fears Provide information and reassurance as appropriate Involve family in child’s care in hospital Prepare family for some emotional regression CONGENITAL HIP DISLOCATION displacement of the head of the femur from the acetabulum present at birth although not always diagnosed familial disorder unknown cause; may be fetal position in utero acetabulum is shallow and the head of femur is cartilaginous at birth Assessment: maybe unilateral or bilateral limitation of abduction (cannot spread legs to change diaper) Ortolani’s click With an infant supine, bend knees and place thumb on bent knees, fingers at hip joint Bring femur 90degrees to hip, then abduct Palpable click – dislocation Barlow’s test With infant on back, bend knees Affected knee will be lower because the head of the femur dislocates towards the bed of gravity additional skin folds with knees bent when lying on abdomen, buttocks of affected side will be flatter Trendelenburg test – if child can walk Have child stand on affected leg only Pelvis will dip on normal side as child attempts to stay erect Management: Goal : to enlarge and deepen the socket Early treatment: positioning the hip in abduction with the head of the femur in the acetabulum and maintaining it in position for several months Traction and casting (hip spica) Surgery Nursing intervention: Maintain proper positioning: keep legs abducted Use triple diapering Use Frejka pillow splint (jumperlike suit to keep legs abducted) Place infant on abdomen with legs in “frog” position Use immobilization devices Provide adequate nutrition Provide sensory stimulation Client teaching and discharge planning: CLUBFOOT (Talipes) abnormal rotation of foot at ankle Varus – inward rotation; bottom of feet face each other Valgus – outward rotation Calcaneous – upward rotation; would walk on heels Equinas – downward rotation; would walk on toes Most common – talipes equinovarus Assessment: foot cannot be manipulated by passive exercises into correct position Management: exercises casting Denis Browne splint (bar shoe) surgery and casting Nursing Intervention: perform exercises as ordered provide cast care child who is learning to walk must be prevented from trying to stand; apply restraints if necessary provide diversional activities provide skin care client teaching SCOLIOSIS lateral curvature of the spine most commonly in adolescent girls familial pattern; associated with other nueromuscular condition idiopathic majority Assessment: failure of curve to straighten when child bends forward with knees straight and arms hanging down feet uneven bra strap marks uneven hips uneven shoulders asymmetry of rib cage xray: reveals curvature Management: stretching exercises Milwaukee brace – worn 23 hours/day for 3 years plaster jacket vest spinal fusion Nursing Intervention: teach/encourage exercise provide care for the child with Milwaukee brace Child wears brace 23 hours/day Monitor pressure points Promote positive body image with brace Provide cast care Assist with modifying clothing for immobilization devices Adjust diet with decreased activity Provide client teaching and discharge instructions Exercise Cast care Correct body mechanics Alternative education for long term hospitalization Availability of community agencies JUVENILE RHEUMATOID ARTHRITIS systemic, chronic disorder of connective tissue autoimmune reaction results from eventual joint destruction affected by stress, climate and genetics Types: Monoarticular JRA Fewer than 4 joints involved (usually legs) Asymmetric Good prognosis Mild signs of arthritis Polyarticular JRA Multiple joints affected Symmetrical Involvement of TMJ Remissions and exacerbations poor prognosis Systemic disease with polyarthritis (Still’s disease) Fever, rash, LADP, anorexia, weight loss Exacerbations and remissions Assessment: No specific diagnostic tests ESR, ASO, RF- not specific Intervention Drugs: ASA, corticosteroids NSAIDS Physical therapy – strengthening muscles, preventing deformities Splints – used for knees, wrists and hands – to reduce pain and prevent or reduce flexion deformities Nursing intervention: Assess joints for pain, swelling, tenderness promote maintenance of joint mobility a. ROM exercises b. Isometric exercises change position frequently; alternate sitting, standing, lying promote comfort and relief provide firm mattress maintain proper body alignment keep joints mainly in extension, not flexion cold treatments: in acute episodes focus on child’s strength GENITOURINARY SYSTEM Nephrons continue to develop after birth GFR is 30% below adult level at birth reaches normal level by age 2 years tubular function immature at birth; reach adult level by age 2 years urethra shorter in children and more prone to ascending infection UNDESCENDED TESTES (Cryptorchidism) unilateral or bilateral absence of testes in scrotal sac testes normally descend at 8 months of gestation increased incidence in those with genetic disorder 75% will descend spontaneously by age 1 year Assessment: unable to palpate testes in scrotal sac Management: if testes remains in abdomen, damage to testes is possible because of increased chorionic gonadotropin orchipexy: surgical procedure to retrieve and secure testes placement; performed between ages 1-3 yrs Nursing Management: provide treatment options support parents if surgery will be done post op: avoid disturbing the tension mechanism avoid contamination of incision HYPOSPADIAS urethral opening located anywhere along the ventral surface of penis Assessment: urinary meatus misplaced inability to make straight stream of urine Management: minimal defects – no intervention neonatal circumcision delayed, tissue maybe needed for corrective repair surgery at age 3-9 months Nursing Intervention: diaper normally provide support for parents post op: check pressure pressure dressing monitor catheter drainage PHIMOSIS an abnormal narrowing of the foreskin so that it cannot be retracted over the glans penis may be present at birth or may develop as a result of poor hygiene with accumulation of smegma Management: Prevention – regular pulling the foreskin back and cleaning circumcision Nursing intervention: circumcision care: Close observation for bleeding Ice application Administration of analgesics ENURESIS involuntary passage of urine after the age of control is expected (4 years) types: Primary – in children who have never achieved control Secondary: in children who have developed complete control May occur anytime but most frequent at night More common in boys No organic cause Etiologic possibilities: Sleep disturbances Delayed neurologic development Immature development of bladder Psychologic problems Assessment: PE normal History of repeated voluntary urination management: bladder retention exercises drug therapy TCA – imipramine Anticholinergics Nursing Intervention: Provide information/counseling to family: Confirm that this not conscious behavior and that the child is not purposely misbehaving Assure parents that they are not responsible and that this is a relatively common problem Involve child in care; give praises and support with small accomplishments Age 5-6 years – can strip wet beddings Age 10-12 years – can do laundry and change bed Avoid scolding and belittling the child EXSTROPHY OF THE BLADDER congenital malformation ; nonfusion of abdominal and anterior walls of the bladder during embryologic development anterior surface of bladder lie open on abdominal wall Assessment: Associated structural changes Prolapsed rectum Inguinal hernia Widely split symphysis Rotated hips Associated anomalies Epispadias Cleft scrotum or clitoris Undescended testes Chordee (downward deflection of the penis) Management: reconstructive surgery urinary diversion delayed until 3-6 months Nursing intervention: Preop: Provide bladder care; prevent infection Keep area as clean as possible Change diaper frequently; keep loose fitting Wash with mild soap and water Cover exposed bladder with vaseline gauze Postop: Design play activities to foster toddler’s need for autonomy - child will be immobilized for extended period of time Prevent trauma; as child gets older and more mobile, trauma is more likely NEPHROTIC SYNDROME autoimmune process structural alteration of glomerular membrane results in increased permeabilityb to plasma proteins course: exacerbations and remissions over a period of months to years commonly affects preschoolers; boys>girls Assessment: Proteinuria Hypoproteinemia Hyperlipidemia Dependent edema Puffiness around the eyes in morning Ascites Scrotal edema Ankle edema anorexia, vomiting, diarrhea Pallor, lethargy Hepatomegaly Management: drug therapy Corticosteroids Antibiotics Thiazide diuretics Nursing Intervention: Provide bed rest Conserve energy Find activities for quiet play Provide high protein, low sodium diet – during edema phase Maintain skin integrity Avoid IM injections – meds not absorbed in edematous tissues Obtain morning urine for protein studies Provide scrotal support Monitor I and O, vital signs Daily weight Administer as ordered Protect from sources of infection ACUTE GLOMERULONEPHRITIS immune complex disease beta hemolytic strep More frequently in boys; 6-7 years resolves in 14 days self limiting Assessment: History of strep infection (URTI or impetigo) Edema, anorexia, lethargy Hematuria or dark colored urine Fever Hypertension Diagnostic tests: Urinalysis – rbc,wbc,protein, cellular casts Urine specific gravity increased BUN, crea increased ESR elevated Hgb, Hct decreased Management: antibiotics Antihypertensives digitalis – if with CHF fluid restriction peritoneal dialysis – if severe renal complication occurs Nursing Management: 1. Monitor I and O, BP Weigh daily Provide diversional activity Provide client teaching and discharge planning Medication administration Prevention of infection Signs of renal complications Importance of long term follow up WILM’S TUMOR (NEPHROBLASTOMA) large, encapsulated tumor that develops in the renal parenchyma, more frequently in left kidney originates during fetal life peak age : 1-3 years Assessment: non tender mass, usually midline near liver hypertension hematuria test: IVP Nursing intervention: Do not palpate abdomen – to avoid dissemination of CA cells Handle child carefully when bathing and giving care Provide care for the client with a nephrectomy Provide care for the child receiving chemotherapy MALFORMATIONS OF THE NERVOUS SYSTEM Cranial Defects: Craniosynostosis one or more of the sutures close too soon ICP increases; interfeeres with normal brain growth MR Assessment: suture lines of the skull manually palpated Radiographs are made to confirm Microcephaly The brain fails to grow May be due to a chromosomal defect or from drugs, toxins or radiation MR Hydrocephalus Imbalance of CSF absorption or production caused by malformations, tumors, hemorrhage, infection, trauma Types: Communicating – impaired absorption within arachnoid space Non-communicating – obstruction of CSF flow within the ventricular system Assessment: Infant – increased HC Macewen’s sign – cracked-pot sound on percussion of bones of head Anterior fontanel tense, bulging Scalp veins dilated Frontal bossing, sunsetting eyes Child – behavior changes Headache, nausea and vomiting Ataxia, nystagmus Surgical Implementation: Hydrocephalus Goal: to prevent further CSF accumulation by bypassing the blockage and draining the fluid from the ventricles to a location where it may be reabsorbed VP Shunt – CSF drains into the peritoneal cavity from the lateral ventricle AV shunt – CSF drains into the right atrium PostOP Care: Keep child flat as prescribed – to avoid rapid reduction of intracranial fluid Observe increase ICP – if present, elevate HOB 15-30 deg Monitor for infection Measure HC Monitor I and O Provide comfort measures; administer medications (diuretics, antibiotics, or anticonvulsants) Toddler – headache and anorexia earliest common signs of shunt malfunction SPINA BIFIDA CNS defect that occurs as a result of neural tube failure to close during embryonic development defect closure usually done during infancy Types: Spina bifida occulta Posterior vertebral arches fail to close in the lumbosacral area Spinal cord intact; not visible Meninges not exposed on the skin surfaces Meningocoele Protrusion involves meninges and a sac-like cyst Lumbosacral area Myelomeningocoele Protrusion of meninges, CSF, nerve roots, portion of spinal cord Sac covered by a thin membrane may rupture or leak Neuro deficit evidence Assessment: Depends on spinal cord involvement Visible spinal defect Flaccid paralysis of legs Altered bladder and bowel function Implementation: Evaluate sac; measure lesion neuro check monitor for increase ICP measure HC; assess fontanelles Protect the sac 1. Cover with sterile, moist (normal saline) non-adherent dressing 2. Change dressing every 2-4 hours Place prone position head is turned to one side for feeding diapering may be C/I until defect repaired Aseptic technique Watch for early signs of infection Administer antibiotics Administer anticholinergics – improve urinary continence Administer laxatives , antispasmodics MENINGITIS infectious process of the CNS caused by bacteria and viruses acquired as a primary or as a result of complications diagnosis – CSF analysis (increase pressure, cloudy CSF, high protein, low glucose bacterial or viral Assessment: signs and symptoms vary depending of age group fever, chills vomiting, diarrhea poor feeding or anorexia altered LOC bulging anterior fontanel nuchal rigidity Implementation: isolation; maintain for at least 24 hours after antibiotics are initiated administer antibiotics as prescribed monitor VS and neuro status Monitor I and O assess nutritional status determine close contacts of the child with meningitis SEIZURE DISORDERS Sudden transient alterations in brain function resulting from excessive levels of electrical activity in the brain Assessment: obtain information from parents about the time of onset, precipitating events and behavior before and after the seizure seizure precautions: Raise side rails Pad side rails Place waterproof mattress on bed Instruct child to swim with companion Alert caregivers to the need for special precautions Emergency Treatment for Seizures: Ensure patency of airways If the child is standing or sitting, ease the child down to the floor place pillow or folded blanket under the child’s head loosen restrictive clothing clear area of any hazards if vomiting occurs, turn child to one side do not restrain child; do not place anything in the child’smouth Remain with the child until fully recovers Prepare to administer medications CEREBRAL PALSY disorder characterized by impaired movement and posture resulting from an abnormality in the extrapyramidal motor system spastic type- most common Assessment: extreme irritability and crying feeding difficulties stiff and rigid arms and legs delayed gross development abnormal motor performance alterations of muscle tone abnormal posturing persistence of primitive reflexes Implementattion: early recognition PT, OT, speech therapy, eduaction and recreation assess the child’s developmental level and intelligence early intervention encourage communication and interaction with the child on a functional level provide safe environment position upright after meals provide safe, appropriate toys for age and developmental level

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