CHILD HEALTH NURSING Child Health Nursing ● Focuses on protecting children from illness and injury ● Assists children

to obtain optimal levels of wellness Primary roles of the Pediatric Nurse ● Care provider ● Educator ● Advocate Pediatric Health Statistics ● Infant Mortality ○ Number of deaths per 1000 live births during 1st year of life. ○ Infant mortality rate is an indicator of how healthy the nation is. ○ This rate is used to compare national health care to previous years and to other countries. ○ There has been a great decrease in the number of deaths over the past century. ■ In 1997, 6.9 per 100 live births. Childhood Mortality ● For children older than one year, death rates have always been less than those for infants. ● In later adolescence, there is a sharp rise in deaths. Injuries; the leading killer in childhood ● Injuries cause more death and disabilities in children than any diseases. ● Some examples include: ○ Motor vehicle accidents are the leading cause of death in children over 1 year of age. ○ Majority of deaths are due to no use of seatbelts. ● Drowning is 2nd leading cause of death in boys 1-14; both sexes 15-24 years old. Drowning is the 3rd leading cause of death for girls 1-14. ● Burns are the 2nd leading cause of death from injury in girls and the 3 rd in boys from 1-14 years old. Childhood Morbidity (Illness) ● An illness or injury that limits activity, requires medical attention or hospitalization, or results in a chronic condition. ● Examples: ○ Congenital heart defects ○ Asthma ○ Cerebral Palsy ○ Cystic Fibrosis ● Concerned with helping to decrease these statistics as children miss school and other activities when ill. Advanced Practice roles for nurses in Child health nursing ● Family nurse practitioner ● Neonatal nurse practitioner ● Pediatric nurse practitioner ● Nurse midwife Compiled By: Bheru Lal Page 60

Growth and Development ● Growth: used to show an increase in physical size or a significant change. ● Includes: ○ Height ○ Weight ○ Head circumference ● Development: Used to denote an increase in skill or ability to finction. ● Measured by: ○ Observing child do specific tasks ○ Parents description of child’s ability ○ Using standardized testing (Denver II screening test) Principles of Growth and development ● G & D is a continuous process from birth to death ○ Rate of growth varies at different times ● G & D proceeds in an orderly manner ○ Growth from smaller to larger ○ Development: from sitting to crawling to walking ● Different children pass through the predictable stages at different rates ● All body systems do not develop at the same time. ● CNS, Cardiac develops quickly. ● Lungs are typically the last to develop. Development is Cephalicaudal, which is the 3rd principle of G & D. “Head to toe”. Development goes from proximal to distal, which is the 4th principle. ● Example: Development proceeds from gross to refined. ● A child cannot learn tasks until the nervous system is ready. ● Neonatal reflexes must be lost before development can proceed. Factors that influence growth and development ● Genetics ● Gender ● Health ● Intelligence Temperament ● Inborn in all of us ● A way of reacting to the world around us Reaction Patterns: ● Activity level ○ Level of activity differs widely among children ● Rhythmicity ○ Have a regular rhythm is physiologic terms ● Approach ○ Child’s response to a new situation ● Adaptability ○ Is the child able to adapt to new situations? ● Intensity of reaction ● Distractibility Compiled By: Bheru Lal Page 60

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Attention span and persistence Mood quality

Categories of temperament ● The “Easy” Child ○ Easy to care for if they have predictable rhythmicity, approach and easily adapt to new situations, have a mild to moderate intensity of reaction, and an overall positive mood quality. ○ 40-50% of children fall into this category ● The “Difficult” child ○ Children are “difficult” if irregular in habits, have negative mood quality, and withdraw from new situations ○ 10% of children fit this image. ● “Slow to warm up” child ○ Describes children who are overall fairly inactive, respond only mildly, adapt slowly to new situations, and have a general negative mood. ○ 15% of children display this pattern. Other factors that impact growth and development ● Environment ● Socioeconomic level ● Parent-child relationship ● Position of birth in the family ● Health ● Nutrition ● As a child normally develops somewhat predictably in growth and physical development, he also matures emotionally, intellectually, and spiritually along certain paths. Review: ● Erikson’s theory of Psychosocial development ● Piaget’s theory of cognitive development ● Kohlberg’s theory of Moral development ASSESSMENT OF THE CHILD Basic Principles ● Know norms; child vs. adult ● A & P variations are farthest from adult norms at birth ○ Most of these variations mature quickly in the first year ○ By 5-7 years of age, body is that of a “Small adult”; however, a child is not a miniature adult and should not be treated as such. ● As a child normally develops somewhat predictably in growth and physical development, he also matures emotionally, intellectually, and spiritually along certain paths. ● Try to see the child’s world and body through his/her mind. If you do this, you will connect with the child. Approach to exam ● Always think of where the child is developmentally. ● Approach must be individualized Compiled By: Bheru Lal Page 60

Usually the child will be frightened and anxious. May lack verbal skills to express fear or ask for information. ● Use both hands on child when possible – comforting touch. ○ Place left hand on shoulder while auscultating the heart. ○ Move unhesitatingly, firmly, and gracefully. ○ Talk pleasantly and reassuringly. ● Instructions to the child: ○ Use a directive voice ○ Have specific instructions ○ Do not ask, but instead tell a child ■ Example: Say “Roll over on your belly” rather than “Will you roll over on your belly?”

Physical Exam ● Can take place almost anywhere ○ On parents lap ○ On the floor ○ Examiners lap Conducting the exam ● Perform the least distressing procedures first and the most distressing last. ○ Heart and lungs; have the child lie down. ○ Abdomen, throat, and ears (throat and ears are the worst) ○ Genitalia and rectum Physical exam technique ● Inspection ○ Observe before you touch ● Auscultation ○ Use diaphragm of stethoscope for high pitched sounds (bowel sounds) ○ Use bell of stethoscope to help localize sounds for infant ● Palpation ○ Use pads of fingers to determine tenderness and pulsations ○ Use palmar surface of fingers to determine masses and organ enlargement ○ Observe reaction to palpation rather than to ask if it hurts (don’t suggest that it does) ● Percussion ○ A more advanced technique usually done by physicians and advanced practice nurses. The general survey ● Vital signs ● General appearance ● Mental status ● Body measurements Vital signs ● Temperature ○ Body temperature in infants is less constant than in adults ○ Use ax/tympanic for children less than 4 years of age Compiled By: Bheru Lal Page 60

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Values are the same as in adults Axillary: hold child’s arm firmly Tympanic: ○ Less than 3 years of age ■ Insert gently into ear ■ Pull down on ear ○ Over 3 years of age ■ Pull up on ear Rectal temperature ○ Most hospitals are done only with doctors order, or there is a standing order. ○ Lubricate tip well ○ Insert 1 inch Pulse ○ Apical is best ○ May use femoral arteries, brachial arteries ○ Radials only in older children (at least 2 years old) Respirations ○ The younger the child, the more abdominal breathing ○ Observe the abdomen instead of the chest in infants and small children ○ May need to auscultate the chest or put the stethoscope in front of the mouth and nose. Oxygen saturations Blood pressure ○ Wide enough to over 75% of the upper arm ○ Narrow cuff elevates reading, wide cuff lowers reading ○ In infants less than 1 year: ■ Thigh BP = arm BP ○ Older than 1 year: ■ Systolic in thigh is 10-40 mm Hg higher than in arm ■ Diastolic is same in thigh and arm ○ If BP in thigh is less than in arm: ■ Cardiac anomaly or decreased circulation to extremities ■ Be sure to use correct size cuff ■ Use the same extremities, the same size cuff, and same position whenever possible when trending values for an individual patient. ○ Diastolic BP ■ Diastolic pressure reaches about 55 mm Hg at one year of age ■ Gradually increases to 70 throughout childhood ○ The most common cause of hypertension in children are: ■ Anxiety (increases BP in children) ■ Renal disease (78%) ■ Coarctation of the aorta (2%)

General appearance ● To form a general impression of child’s health and well-being ● To pin-point specific areas that may require more detailed assessment Initial observations ● Degree of illness or wellness Compiled By: Bheru Lal

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Mood State of nutrition Speech, cry, facial expression, posture Apparent chronological and emotional age Respiratory pattern Parent and child interaction

Parent and child interaction ● Amount of separation tolerated ● Displays of affection ● Response to discipline ● Look for signs of: ○ Anxious parents ○ Disengaged parents ○ Stressed families ○ Possible abusive parents (no separation anxiety when removed from parent, or over-affectionate) Child cries or clings to parent ● Ignore the child temporarily ● Engage the parents in conversation, then place a small game, toy, or your stethoscope within reach of the child while continuing your discussion Mental status ● Is the child alert? ● Able to respond to questions easily? ● Assess appropriateness of behavior ● Assess memory Assessing Growth/Body measurements ● Height, weight, head circumference—important indicators of growth ● Measured and plotted on standard growth charts ● These charts are used to determine if the baby/child’s growth is falling within the accepted percentile for age Length ● Birth to 36 months ○ Fully extend the body by: ■ Holding the head midline ■ Grasping the knees together gently ■ Pushing down on the knees until the legs are fully extended and flat against the table. ○ Hold pencil at right angle to the table and mark the head and toes (which are pointed toward the ceiling) Height Child’s back is to the wall, with heels, buttocks, and back of the shoulders touching the wall and the medial melleoli touching if possible. ● Check for bending of the knees, slumping of the shoulders, or raising the heels of the feet

Weight ● Birth to 36 months, weigh nude Compiled By: Bheru Lal Page 60

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Older children with panties and light gown Balance (or zero) scale prior to weighing

Head circumference ● Measure at greatest circumference ● Slightly above the eyebrows and pinna of the ears ○ Around the occipital prominence at the back of the skull ○ Compare to 36 months Denver Developmental ● The standard for measuring the attainment of developmental milestones throughout infancy and childhood. ● Designed for birth to 6 years ● Includes screening for: ○ Personal social skills ○ Fine motor adaptive ○ Language ○ Gross motor ● Denver screening for articulation and eyes Example of DDST for One year of age: ● Personal/Social ○ Drink from a cup, imitate activities, play ball with examiner, indicate wants, play pat-a-cake ● Fine motor/adaptive ○ Scribbles, puts block in cup ● Language ○ Dada/Mama specific, one word ● Gross motor ○ Stands alone More on DDST: ● Only a measure of developmental attainment—not a measure of intelligence ● Not a highly specific test ○ Most normal children score as normal ● Not very sensitive ○ Many children with mild developmental delays also score normal ● Only a screening test ● Other more sophisticated tests are available if delay is suspected even when DDST is normal. Heart Murmurs ● 50% of all children develop an innocent heart murmur at some point during childhood. It is usually not something to be overly concerned about unless there are other symptoms. Must be determines if murmur is normal; therefore always report when one is heard. Abdomen ● Protuberant abdomen is typical in most children until adolescence. ● If child is ticklish on palpation, hold his/her hand over yours to reduce apprehension and increase relaxation of the abdominal musculature. Neuromuscular Compiled By: Bheru Lal Page 60

If possible, watch the child standing upright. Have them walk, stoop, and touch their toes ● Checking for scoliosis.

More tidbits: ● Always think of child’s development when assessing ● Know the BP and pulse variations ● When there is an abnormal finding – ALWAYS gather more data ● Weight is a huge concern for children. Many medications are weight dependent. ● The Denver Developmental is not very precise; it’s more of a screening tool ● As it says, the Denver Developmental is only developmental—not a cognitive or an IQ test. ● For breath sounds: ○ Encourage the child to “blow out” your light, in your pen light or flashlight. This will almost always produce full inspiration. CARE FOR THE HOSPITALIZED CHILD General communication guidelines ● Pay attention to infants and younger children through play or by occasionally directing questions or remarks to them. ● Include older children as active participants. ● With children of all ages, the nonverbal components of the message conveys the most. ● Communicate at the child’s level ○ Developmentally and physically Communication Guidelines ● Allow children time to feel comfortable ● Avoid sudden or rapid movements, extended eye contact, or other gestures that may be seen as threatening. ● Talk to the parent if the child is initially shy ● Communicate through puppets, dolls, or stuffed animals before questioning a young child directly. ● Give older children the opportunity to talk without the parents present ● Speak in a quiet, unhurried, and confident voice. ● Speak clearly, be specific, and use simple words and short sentences. ● Be honest with children ● Offer a choice only when one exists. ● Allow them to express their concerns and fears ● Use a variety of communication techniques; if one technique doesn’t work, try another. Infant
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One month to one year old Erikson’s developmental task: Trust vs. Mistrust ○ Task: Attachment to the mother ○ Resolution of crisis: Trust in persons; faith and hope about the environment and future. Page 60

Compiled By: Bheru Lal

Unsuccessful resolution of crisis: General difficulties relating to person’s effectively; suspicion; trust-fear conflict, fear of the future.

Infant: communication ● Forms first social relationships ● Communicates needs and feelings through nonverbal behaviors and vocalizations ○ Smile and coo when content ○ Cry when distressed—hunger, pain, body restraint, loneliness ● Respond to adults’ nonverbal behaviors ○ Become quiet when cuddled, patted, or receive other forms of gentle, physical contact. ○ Get comfort from the sound of voice—usually respond to any gentle firm handling until they reach the age of stranger anxiety (5-8 months). Infant: Stressors of Hospitalization ● Separation anxiety ● Stranger anxiety ● Painful, invasive procedures ● Immobilization Separation Anxiety ● Occurs as early as 4 months old; infants face shows disapproval as primary caregiver walks away. ● Three stages of separation anxiety: ○ Protest ■ Cries loudly; rejects attempt to be comforted by anyone but the caregiver ○ Despair ■ Crying stops and depression is evident ■ Much less active ■ Uninterested in food or play ■ Withdraws from others ○ Detachment (denial) ■ Shows increased interests in surroundings ■ Interacts with strangers or familiar caregivers ■ Forms new but superficial relationships ■ Appears happy ■ Detachment usually occurs after prolonged separation from parents; rarely seem in hospitalized children. ■ Behaviors represent a superficial adjustment to loss. ● Interventions for separation anxiety: ○ Encourage parents to room-in with infant ○ Involve parents in child’s care whenever possible ○ Keep parent in infant’s line of vision ○ If parent is unable to be with infant, place familiar object with infant (stuffed toy, etc.) ○ Support sibling and grandparent visitation Stranger anxiety ● Occurs as early as 5 months ● Usually peaks at 8 months Compiled By: Bheru Lal Page 60

Behaviors exhibited by infant: ○ Cries ○ Screams ○ Searches for parent with eyes ○ Clings to parent ○ Avoids and rejects contact with strangers ● Interventions for stranger anxiety: ○ Holding out hands and asking the child to “come” will usually not work. If infant must be handled, better to pick up firmly without gestures. ○ Observe position in which parents hold infant and imitate this. ○ Hold infants where they can see their parents ○ Are usually more at ease upright than horizontal.

Fear of procedures—interventions ● During procedure, use sensory soothing measures (Stroking skin, talking softly, giving pacifier) ● Cuddle and hug after stressful procedure or encourage parent to do so if present. ● Older infants may associate objects, places, or persons with prior painful experiences and will cry and resist at the sight of them ○ Keep frightful objects out of view ○ Perform painful procedures in separate room, not in crib, ○ Use non-intrusive procedures whenever possible. Immobilization ● Infants explore life through activity and mobility. If taken away: ○ Feel helpless ○ May have difficulty with language skills ○ May have problems mastering developmental tasks ○ Problems with motor skills ○ Immobility impacts development. ● Immobilization interventions: ○ Play therapy ○ Transport infant outside of room by wagon of by carrying ○ Spend time interacting with infant ○ Encourage caregivers to do the same Toddler ● Ages one to three years old ● Erikson’s developmental task: Autonomy vs. shame and doubt. ○ Task: Gaining some basic control over self and environment ○ Resolution of Crisis: Sense of self-control and adequacy; will power ○ Unsuccessful resolution of crisis: Independence-fear conflict; severe feelings of self-doubt. ● Developing a sense of autonomy ● He wants to make choices and like the word “No!” ● Is egocentric ● Focus communication on toddler ○ Toddler not interested in the experiences of others Stressors of hospitalization ● Separation anxiety Compiled By: Bheru Lal Page 60

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Loss of control Painful, invasive procedures Bodily injury Fear of dark

Separation anxiety ● Verbally attack stranger (“Go away!”) ● May physically attack stranger (kicks, bites, hits) ● Tries to escape to find parent ● Tries to physically force parent to stay (clings) ● May have temper tantrums or refuse care ● Behaviors may last from hours to days Separation anxiety—interventions ● Child’s reaction to separation ○ Toddler might ignore parent. ● Other strategies are same as for infant. ● Feels more secure with familiar item ● Allow them to touch and examine articles that will come into contact with them ● Be direct and concrete ● They interpret words literally. Loss of control ● Very threatening to the toddler ● Many hospital situations decrease amount of control a child feels ● Loss of control occurs due to: ○ Restriction or limitation of movement ○ Altered routines and rituals ■ Eating ■ Toileting ■ Sleeping ■ Bathing ■ Play ○ When routines are disrupted, problems can occur in these areas ● Interventions ○ Promote freedom of movement ■ Encourage parent-child contact ■ Transport in carriage, wheelchair, carts, etc. ■ Maintain child’s routine ■ Encourage as much independence as possible (allow child to wear clothes from home, etc.) Bodily Injury/Invasive procedures ● Concept of body very poor ● Intrusive procedures (examining ears) create much anxiety ● React with intense emotional upset and physical resistance ● Interventions: ○ Tell child it’s ok to yell, cry, or do whatever is needed during procedure ○ Explain procedure in relation to child’s senses ○ Ignore temper tantrums ○ Use distraction techniques (Sing song with a child) Compiled By: Bheru Lal Page 60

Fear of the dark ● Keep night light on in room at all times ● Encourage parents to room-in with child Preschool ● 3-6 years of age ● Erikson’s developmental task: Initiative vs. Guilt ○ Task: Becoming purposeful and directive ○ Resolution of Crisis: ability to initiate one’s own activities; sense of purpose. ○ Unsuccessful resolution of crisis: aggression-fear conflict; sense of inadequacy or guilt ● Developing a sense of initiative ● Preschooler is: ○ Egocentric ○ Has increased language skills ○ Concept of time and frustration tolerance is limited ○ Illness and hospitalization may be viewed as punishment Fears of hospitalization ● Separation anxiety and fear of abandonment ● Loss of control ● Bodily injury ● Painful, invasive procedures ● Fears of the dark, ghosts, monsters Separation anxiety- preschooler ● Tolerate separation better than toddlers; may develop substitute trust in other significant adults ● However, they may show other behaviors: ○ Refuse to eat ○ Have difficulty sleeping ○ Cry quietly for parents ○ Constantly ask when parent will be visiting ○ May express anger ● Interventions: ○ Have parents bring in familiar articles from home—pictures/radio ○ If child has attachment to special item, have it brought in ○ Same strategies for toddlers Loss of control ● Egocentric and magical thinking; perception of actual events are more frightening ○ Typical fantasy: Illness is a punishment for their misdeeds ● Purely verbal instructions do not help them, have them practice on doll or toy. Bodily injury ● Differentiate poorly between themselves and outside world. ● Fear of mutilation ● Take things literally “Stick for blood” ● Fear if when given a shot, when needle is removed, their insides will leak everywhere (put a band aid in place). Compiled By: Bheru Lal Page 60

Interventions: ● Point out on drawing, doll, or child where procedure is performed ● Emphasize that no other body part will be involved ● Use non-intrusive procedures when possible ● Apply adhesive bandage over puncture site ● Encourage parental presence ● Allow child to wear underpants with gown ● Explain unfamiliar situations, especially noise or lights ● Involve child in care whenever possible (hold equipment, remove dressing) ● Praise child for helping and attempting to cooperate ● Never shame child for lack of cooperation School age child ● 6-12 years old ● Erikson’s developmental task: Industry vs. Inferiority ○ Task: developing social, physical, and school skills ○ Resolution of Crisis: competence, ability to learn and work ○ Unsuccessful resolution of crisis: Sense of inferiority; difficulty learning and working ● Developing a sense of industry and concrete thought ● Has increased language skill ○ Interest in acquiring knowledge ○ Improved concept of time ○ Increased self-control ○ Developing relationships with peers; peers are very important at this stage. ● Usually will want explanations and reasons for why things are being done ● Want to know more about procedures, activities, and objects ● Have a greater concern for privacy and body integrity ● Usually easier to communicate with than previous age groups ○ Concrete thinkers; no abstract thought Stressors of hospitalization ● Separation anxiety ● Loss of control ● Bodily injury ● Painful, invasive procedures ● Fear of death Separation anxiety ● Younger school age children miss their parents more than older children ● Middle/late react more to separation from peers and usual activities ○ May feel lonely, bored, isolated, depressed due to separation, not the illness. ○ May try to be “brave and strong”. ○ May be irritable with parents ● Interventions ○ Make environment as home-like as possible ■ Continue school lessons ■ Have friends visit or call on phone ■ Decorate walls with cards Compiled By: Bheru Lal Page 60

Bodily injury ● Less concerned with pain, more concerned about disability or death ● Take very active interest in their health ● Request facts Invasive procedures ● Want to know about a procedure (will it hurt? What’s it for?) ● Tolerate intrusive procedures well ● By age 9 or 10 should less fright to pain Adolescent ● Ages 12-20 years ● Erikson’s developmental task: Identity vs. Role confusion ○ Task: developing sense of identity ○ Resolution of Crisis: sense of personal identity ○ Unsuccessful resolution of crisis: confusion about who one is; identity submerged in relationships or group memberships ● Developing a sense of identity and abstract thought ● Fluctuate between child and adult thinking and behavior ○ Need to express their feelings, for some this comes easily, for others it does not. ○ Major sources of concern for this age group are attitudes and feelings toward sex, substance abuse, relationships with parents, peer-group acceptance, and developing a sense of identity. Stressors of hospitalization ● Loss of control ● Altered body image, disfigurement ○ Do not want to look different ● Separation from peer group ● Loss of control ○ Anything that interferes with sense of independence ○ Patient role fosters dependency ○ May withdraw, be uncooperative, angry, frustrated. ● Altered body image ○ Very relevant at this stage ○ Any change that makes them different from peers is seen as a major tragedy ○ Insecure with their bodies due to the many changes ○ May react with “know it all” attitude (but may be afraid, deep down) ○ Privacy is very important—may need to give them some time alone.

CYSTIC FIBROSIS Epidemiology ● Affects approximately 30,000 children and adults in the US today or 1 in 2500 live births ● Occurs most commonly in whites, rarely in blacks and Asians ● More than 10 million Americans (1 in 31 or 3.3%) are symptom free carriers of the defective CF gene Compiled By: Bheru Lal Page 60

Survival ● In 1955, few children with CF lived to attend elementary school. Today, the median age of survival is 32 years. ● In this decade, many CF survive into their 40’s. Etiology ● An inherited (genetic) disease ● CF is an autosomal-recessive disease (this means an individual must inherit 2 defective CF genes, one from each parent) ● The defective gene in CF occurs in Chromosome 7 ● The gene causes the dysfunction of the exocrine glands What are exocrine glands? ● Glands that secrete things such as: ○ Lungs ○ Pancreas ○ Sweat glands ○ Salivary glands ○ Digestive glands Pathophysiology ● CF causes the body to produce an abnormally thick, sticky mucus which: ○ Clogs the lungs and leads to life-threatening lung infections; ○ Obstructs the pancreas, preventing enzymes from reaching the intestine to help break down and digest food ● Primary symptoms: ○ Thick, sticky mucus ○ Salty taste on skin ● Marked electrolyte changes in sweat glands ○ Chloride in sweat is 2-5X above normal Lung involvement ● Thick tenacious mucus polls in the bronchial tree and obstructs bronchioles ● Results in: ○ Bronchiectasis: Chronic dilation of the bronchi. Involves a chronic cough that produces mucopurulent sputum. Over time results in destruction of the bronchial walls. ○ Pneumonia: Staph Aureus, pseudomona’s, and H. Influenzae ● Symptoms over time include: ○ Clubbing of fingers ■ Clubbing is related to lack of tissue perfusion Symptoms over time: ● Easily fatigued ● Physical growth stunted ● Chest may become enlarged from over inflation of alveoli because air cannot be pushed past the thick mucus on expiration (barrel chested) ● Increased carbon dioxide in blood (hypercapnia and/or chronic acidosis) Pancreas involvement ● Thick secretions mix with pancreatic enzymes and block the pancreatic duct. ● Therefore: Compiled By: Bheru Lal Page 60

Essential pancreatic enzymes cannot flow into the duodenum to aid with digestion (lipase, trypsin, and amylase) ○ Fats, proteins, and some sugars cannot be digested—are excreted in stools: ○ Stools (The 4 F’s in CF) ■ Frothy (bulky/large) ■ Foul smelling (comparable to a cats stool) ■ Fat containing “greasy” Steatorrhea ■ Floaters ● Clogged ducts causes back pressure on the acinar cells (they make the enzymes) ● The cell lining of pancreas is damaged ● Over time the acinar cells atrophy and no longer produce the enzymes

Symptoms related to Pancreatic Involvement ● Protuberant abdomen because bulk of stool is setting in the intestines ● Signs of malnutrition—only benefit from 50% of food taken in ● Fat soluble vitamins are not absorbed A, D, E, K ● In infants, thick, sticky stools (meconium) which may cause intestinal obstruction ○ One of the first signs of CF in infants Sweat gland involvement ● The structure of the sweat glands is not changed, however: ● Level of chloride to sodium in the perspiration is increased 2 – 5 times above normal ● Some parents report they knew their newborn had the disease because when they kissed their child they could taste such strong salt in their perspiration How is CF diagnosed? ● By the history (cough, stool, abdomen, hx. of pneumonia) ● The abnormal concentration of chloride in sweat ● The absence of pancreatic enzymes in duodenum (can be tested through NG tube) ● Pulmonary involvement Common complaints that bring these patient to the doctor ● Newborn that loses 5-10% of weight after birth but does not gain it back ● Feeding problems—kids are always hungry because of their poor digestive function ● Frequent respiratory infections ● Cough Diagnostic tests ● A sweat test is a test for the chloride content of sweat ● Infants may not be tested until 6-8 weeks of age. (they don’t sweat a great deal) ○ Normal concentration of Chloride in sweat is 20 mEq/L ○ A level of 50-60 mEq/L suggests CF—test is repeated ○ A level >60 mEq/L = CF ● Duodenal analysis of secretions for detection of pancreatic enzymes ● Stool analysis: for fat content (although appearance may be enough) ● Pulmonary testing—chest x-rays or PFT’s Therapeutic management ● Maintain respiratory function ○ Keep bronchial secretions as moist as possible to facilitate drainage: Compiled By: Bheru Lal Page 60

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Moistened oxygen: Oxygen is supplied to children by mask, prongs, ventilators, or neubulizers, and rarely by tent ■ Aerosol therapy- 3-4 times/day via neubulizer to provide antibiotics and bronchodilators ○ Never give cough syrups or codeine Aggressive chest physiotherapy- usually needed 3-4 times a day Activity- need frequent position changes, especially when in bed. Helps facilitate drainage of various lobes, as well as prevent skin breakdown. Respiratory hygiene: frequent mouth care, toothbrushing and good-tasting mouthwash. Need frequent check-ups and current immunizations/vaccines Adequate rest and comfort ○ Dyspnea can lead to exhaustion ○ Need periods of rest during the day: ■ Rest period before meals so not too tired to eat ■ Rest periods before chest physiotherapy Promote optimal nutrition ○ Pancreatic enzyme supplements with meals and snacks ■ Pancreatic enzyme: Cotazym or pancrease ■ Comes in large capsule which can be opened and dissolved in a tsp. of food ■ Children usually begin to gain weight, and stools decrease in size and foul odor. ○ High calorie, high protein, moderate fat diet ○ Multivitamins and E, others when deficient. During hot months, extra salt may be added to food to replace that which is lost through perspiration Keep room temp at 72 degrees and have water available at all times. Parents need to supervise kids playing outdoors to prevent overheating. Keep well hydrated all of the time!

Complications ● Infertility in males related to blocking of vas deferens from tenacious seminal fluid ● Infertility in females related to tenacious cervical secretions that block sperm penetration ● Rectal prolapse in infants from straining to pass hard stool. Loss of blood supply to prolapsed rectal mucosa can occur if not replaced promptly and properly. ● Hypercapnia/respiratory acidosis from inability to adequately exhale carbon dioxide ● Exhaustion, slow growth patterns ● Skin irritation in diaper area from stool that is irritating due to acidic nature of stools ● Socialization and peer acceptance difficulties ● Cor Pulmonale (right sided heart failure) from increased respiratory resistance ● Anemia and bruising ● Frequent respiratory infections and compromised immunity ● Portal hypertension related to obstruction of bile ducts  area of biliary fibrosis  biliary cirrhosis Compiled By: Bheru Lal Page 60

Pneumothorax related to rupture of pulmonary blebs

Parental involvement ● Parents assume a great deal of responsibility when taking care of a CF child. ○ Need to encourage a balance of work, the child, and the rest of the family ○ Encourage involvement of support group ○ Requires extensive involvement of the discharge planner Nursing Diagnoses ● Ineffective airway clearance r/t thick mucus in the lungs ● Ineffective breathing pattern r/t thick tracheobronchial secretions and airway obstruction ● High risk for infection r/t presence of mucus secretions conductive to bacterial growth ● Altered nutrition: Less than body requirements r/t inability to digest nutrients ● Fear/Anxiety (parent or child) r/t prognosis and effect of illness on growth and development ● Knowledge Deficit (parent or child) Summary ● CF is an inherited genetic disorder ● Causes the exocrine glands to produce thick secretions ● Primary body organs involved are lungs and pancreas. ● Prone to respiratory infections r/t mucus. ● Digestion problems r/t pancreatic enzymes. ● Treatment centers around control and management. No cure for the disease.

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One month to one year old Erikson’s developmental task: Trust vs. Mistrust ○ Task: Attachment to the mother ○ Resolution of crisis: Trust in persons; faith and hope about the environment and future. ○ Unsuccessful resolution of crisis: General difficulties relating to person’s effectively; suspicion; trust-fear conflict, fear of the future. ○ Developmental tasks: Learning to eat solid foods

Toddler ● Ages one to three years old ● Erikson’s developmental task: Autonomy vs. shame and doubt. ○ Task: Gaining some basic control over self and environment ○ Resolution of Crisis: Sense of self-control and adequacy; will power ○ Unsuccessful resolution of crisis: Independence-fear conflict; severe feelings of self-doubt. ○ Developmental tasks: ■ Learning to walk ■ Learning to use fine muscles ■ Toilet training Compiled By: Bheru Lal Page 60

Learning to communicate

Preschool ● 3-6 years of age ● Erikson’s developmental task: Initiative vs. Guilt ○ Task: Becoming purposeful and directive ○ Resolution of Crisis: ability to initiate one’s own activities; sense of purpose. ○ Unsuccessful resolution of crisis: aggression-fear conflict; sense of inadequacy or guilt ○ Developmental tasks: ■ Independence of self-care ■ Learning sexual role identity ■ Forming reality concepts ■ Internalizing concepts of right and wrong ■ Learning to identify with family members and others. School age child ● 6-12 years old ● Erikson’s developmental task: Industry vs. Inferiority ○ Task: developing social, physical, and school skills ○ Resolution of Crisis: competence, ability to learn and work ○ Unsuccessful resolution of crisis: Sense of inferiority; difficulty learning and working ○ Developmental tasks: ■ Acquiring game skills ■ Learning to relate positively with peers ■ Building a wholesome self-concept ■ Refining communication skills Adolescent ● Ages 12-20 years ● Erikson’s developmental task: Identity vs. Role confusion ○ Task: developing sense of identity ○ Resolution of Crisis: sense of personal identity ○ Unsuccessful resolution of crisis: confusion about who one is; identity submerged in relationships or group memberships ○ Developmental tasks: ■ Forming peer relationships ■ Responding to an appropriate sexual role ■ Attaining emotional independence ■ Achieving a sense of economic independence

CARDIAC DEFECTS IN CHILDREN ALTERATION IN FLUID-GAS TRANSPORT Cardiac Defects in children: ● Divided into two major groups: ○ Congenital cardiac defects Compiled By: Bheru Lal Page 60

Acquired heart disease

Congenital Heart Disease ● Anatomic abnormality present at birth; the heart has not developed as it should in utero. ● Thus, the heart is unable to adjust to life outside of mom ● Results in abnormal cardiac function Acquired Cardiac disease ● Abnormalities that occur after birth ○ Can occur by self ○ Can occur with other congenital heart defects ○ Example; Rheumatic disease is the 2nd largest cause of cardiac problems in children over 5) Both congenital and acquired heart disorders can lead to heart failure Assessment of cardiac function ● History ○ History of heart disease in the family ○ Contact with known teratogens, such as rubella during pregnancy ○ Presence of chromosomal abnormalities (Down’s) ○ Poor weight gain and/or feeding behavior ○ Exercise intolerance and/or fatigue during feeds ○ Sweating during feeding ○ Frequent respiratory infections ○ Respiratory difficulties, such as tachypnea, dyspnea, and shortness of breath. ○ Recent streptococcal infection (may lead to valve damage) ● Physical exam ○ Begins with observation of general appearance, then the specifics ○ Use general assessment techniques but look specifically for the following: ○ Inspection: ■ Nutritional state: failure to thrive or poor weight gain ■ Skin color: cyanosis and pallor ■ Chest deformities- enlarged heart ■ Unusual pulsations of neck veins seen in some patients ■ Respiratory pattern- tachypnea, dyspnea, presense of expiratory grunt ■ Clubbing of fingers (now rarely seen in children d/t advances in surgical techniques) ○ Palpation ■ Quality and symmetry of pulses ○ Auscultation ■ Heart rate and rhythm ■ Presence of murmurs ○ Height and weight ○ Position of comfort ■ Remember, squatting/fetal positions are often comfortable for a child with a CHD. ● Tests of cardiac function Compiled By: Bheru Lal Page 60

Electrocardiography ■ Records electricity generated by the beating heart ■ Painless but scary, child must be still Exercise stress test ■ Monitoring of heart rate, BP, ECG, and oxygen consumption at rest and during exercise on a tread mill or bicycle Chest x-ray ■ Shows accurate picture of heart size and contour; size of the heart chambers ■ Used more as a screening tool Echocardiography ■ Ultrasound (high frequency sound waves produce an image of heart structures) ■ The primary diagnostic test for heart disease.

Cardiac catherization ● Radiopaque catheter is inserted through peripheral blood vessel into heart ○ Contrast material is injected and films taken (called angiography) ● Reasons for performing: ○ Diagnose specific heart disease ○ Measure pressures and O2 sats ○ Visualize heart structures ○ Determine blood flow patterns Preparation for Cardiac cath ● Although done frequently, there are some risks. Typical reactions include: ○ Acute hemorrhage from entry site (usually femoral artery) ○ Low grade fever (reaction to contrast media) ○ Nausea ○ Vomiting ○ Loss of pulse in the catheterized extremity ○ Transient dysrhythmia’s (d/t ventricular irritability) ● Done on an outpatient basis ● NPO for 2-4 hours before ● Older children should see the cath lab before procedure ● Accurate height and weight (for medications) ● Most children are sedated to decrease anxiety Post catheterization care ● Are usually on a cardiac monitor and pulse oximeter for the first few hours of recovery. ● Know the baseline pulse/BP before the procedure to compare ● Most important nursing responsibility is observation of the following for signs of complications: ○ Pulses, especially below the catheterization site, for equality and symmetry (pulse just distal to site may be weaker for the first few hours but gradually increase in strength. Observations of: ● Temperature and color of the affected extremity. Coolness or blanching may indicate arterial obstruction. Compiled By: Bheru Lal Page 60

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Vital signs are taken every 15 minutes. Special emphasis on heart rate. Must take for one full minute. Assess blood pressure, especially for hypotension. ○ Hypotension could indicate: ■ Hemorrhage ■ Too much medication ■ Dehydration Dressing, for evidence of bleeding. Fluid intake, both IV and oral, to ensure adequate hydration. ○ Remember sensible and insensible fluid loss (breathing too quick!). Hypoglycemia, especially in infants.

Interventions ● Child must keep extremity straight 4-6 hours after venous catheterization and 6-8 hours for arterial cath. ● Child’s diet can be resumed as soon as tolerating sips of clear liquid ● Keep site clean and dry ● Encourage child to void. Congenital heart disease ● Incidence: 4-10 per 1000 live births ● The major cause of death in the first year of life ○ Other than prematurity/low birth weight ● More than 35 well recognized defects ● Statistics improving due to more surgeries/treatments that help prevent death. Etiology ● Not known in 90% of cases ● Factors associated: ○ Maternal rubella during pregnancy ○ Maternal alcoholism ○ Maternal age over 40 ○ Maternal insulin-dependent diabetes ● More likely to have other defects such as Down syndrome. Circulatory changes at birth ● In order to understand the pathophysiology of cardiac defects, it is important to understand fetal circulation and the changes that occur at birth. Review of prenatal circulation- 3 essential structures ● Ductus venosus (DV): opening between umbilical vein and inferior vena cava. ○ The ductus venosus is a vessel that allows blood to bypass the fetus's liver. It carries blood with oxygen and nutrients from the umbilical cord straight to the right side (right atrium) of the fetus's heart. The ductus venosus closes shortly after birth, when the umbilical cord is cut and blood flowing between the mother and fetus stops. ● Foramen ovale: opening between the right and left atrium—bypasses fetal lungs. ○ The foramen ovale is an opening in the wall that separates the upper right and left heart chambers (atria). This opening allows blood to flow to the left side of the heart without going to the lungs. Before birth, the foramen ovale is kept open by the pressure of blood that passes through it. When Compiled By: Bheru Lal Page 60

the baby takes the first breath, blood begins to flow through the lungs, and the foramen ovale closes ● Ductus arteriosis (DA): Opening between pulmonary artery and descending aorta; allows fetal blood to bypass the lungs. ○ There is still very little blood getting into the fetal lungs—just enough to help it grow in utero, even though they are collapsed until birth. Purpose of these structures ● Allow most of blood to bypass the liver and lungs. Fetal circulation ● Oxygenated blood from placenta to ductus venosus  inferior vena cava  right atrium. ● Blood then shunts over to the left atrium through the foramen ovale. ● Then over to the left ventricle  aorta  head/extremities. ● The unoxygenated blood returns to the right atrium via the superior vena cava  flows into the right ventricle  exits thought the pulmonary artery (which is connected to the aorta). ● Most of this blood shunts through the ductus arteriosis into descending aorta, and back into the placenta. Birth changes ● Infant cries, lungs expand ● Ductus arteriosis closes as resistance decreases though the pulmonary vasculature (infants now need to use their own lungs) ● Clamping cord causes ductus venosus to clot (infant needs to use own liver) ● Venous return from lungs causes increase in left atrium pressure. ● Increased left atrium pressure causes left to right blood flow through patent foramen ovale (which is a bad thing—we no longer want to bypass the lungs) ● The foramen ovale is a one way valve so it closes permanently. Birth changes, summary ● Ductus venosus ○ Clots to form ligamentus teres ● Foramen ovale ○ Closes to form interatrial septum ● Ductus arteriosis ○ Closes to form ligamentum arteriosus Altered hemodynamics ● Important to remember pressure gradients as blood will always flow (or shunt) from an area of higher to lower pressure. ● Heart defects cause a change in the direction of this normal flow of blood; create symptoms, especially those associated with congestive heart failure. Congenital heart defects ● Usual cause—heart structure fails to progress beyond earlier ● Was once classified as “Cyanotic” and “Acyanotic” ● New classifications below Classification of Congenital heart disease ● Increase in pulmonary blood flow Compiled By: Bheru Lal

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Decrease in pulmonary blood flow Obstruction to blood flow from ventricles Mixed blood flow

Defects with increased pulmonary blood flow ● Ventricular septal defect ○ Hole between the ventricles ● Atrial septal defect ○ Hole between the atria’s ● Patent Ductus Arteriosis (PDA) ○ Allows blood to flow from higher pressure aorta to the lower pressure pulmonary artery, causing a left to right shunt. ● Atrialventricular septal defect ○ Most likely to be a low artrial and a high ventricular defect. ● More blood to the lungs than needed. ● These defects allow blood to flow from area of higher pressure (left side of the heart), to area of lower pressure (right side of the heart). ● This creates increased blood volume on the right side of the heart which increases pulmonary blood flow. ● Usually results in CHF. ○ Pulmonary hypertension and cor pulmonae are frequent disorders associated with this. Defects causing Decreased pulmonary blood flow ● Tetralogy of the Fallot ○ Pulmonic Stenosis: Narrowing of the pulmonary artery or valve ○ Ventricular septal defect ○ Overriding of the aorta: position of the aorta is not correct. Blood may be shunted from both ventricles. ○ Hypertrophy of the right ventricle ■ Clinical symptoms: ■ O2 sats below 80% ■ Clubbing of fingers and toes ■ Polycythemia (increased hct) ■ Anoxia aeb: dizziness & convulsions ■ Squatting ■ Stunted growth ● Tricupsid Atresia ○ Extremely serious. ○ Tricupsid valve is completely closed. ○ No blood flow from the right atrium to the right ventricle ○ Blood passes through patent foramen ovale into the left atrium and through a ventricular septal defect to the right ventricle and out to the lungs. Defects causing obstruction to blood flow from ventricles ● Coarctation of the aorta ○ A segment of the aorta is too narrow, near the insertion of the ductus arterious. ○ High blood pressure develops Compiled By: Bheru Lal Page 60

Left ventricle is enlarged Oxygenated blood to the body is reduced. ● Pulmonary stenosis ○ Narrowing of the pulmonary artery or pulmonary valve just distal (under/below) to the valve. ○ Eventually causes right ventricular enlargement (hypertrophy) ● Aortic stenosis ○ Narrowing of the aortic valve ○ Prevents blood from passing freely from left ventricle unto aorta. ○ Causes left ventricular hypertrophy from increased pressure in the left ventricle.
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Mixed blood flow ● Cardiac anomalies that involve the mixing of blood from the pulmonary and systemic circulation in the heart chambers. ● Results in deoxygenation of systemic blood flow. ● Cyanosis is not always visible. Mixed blood flow involves: ● Transpositions of the Great Arteries ○ Aorta arises from the right ventricle instead of the left ○ Pulmonary artery arises from the left ventricle ○ Blood enters the heart from the vena cava. ○ Goes to the right atrium  to the right ventricle  then goes out the aorta to the body completely deoxygenated. ○ Very incompatible with life ○ Surgery indicated ● Total Anomalous Pulmonary Venous Return ○ Pulmonary veins return to the right atrium or the superior vena cava instead of to the left atrium as they normally would. (The oxygenated blood keeps going back into the lungs) ○ Blood must be shunted across a patent foramen ovale or ductus arteriosus in order to reach the systemic circulation. ● Truncus Arteriosus ○ One major artery or “trunk” arises from the left and right ventricles in place of a separate aorta and pulmonary artery. ○ Usually accompanied by a VSD. ○ Restructure common trunk to create two separate vessels (2 separate trunks) ● Hypoplastic left heart syndrome ○ Left ventricle of the heart is non-functional. ○ Unable to effectively pump blood into the systemic circulation. ○ Right ventricle enlarges as it tries to do all of the work. ○ Transplant or the 3 Staged Norwood procedures performed. Two principle clinical consequences of defects ● Heart failure ● Hypoxemia Heart Failure Compiled By: Bheru Lal Page 60

Results when myocardium of heart cannot circulate and pump enough blood to supply oxygen and nutrients to body cells ● Blood pools in the heart or in pulmonary or venous systems ● To increase cardiac output, the heart compensates in several ways: ○ Muscle fibers lengthen, causing ventricles to increase and handle more blood with each stroke (ventricular hypertrophy). ○ Heart rate can also increase ● Eventually the heart can no longer compensate—blood pools, unable to be pushed forward effectively.

First signs of CHF ● Tachycardia, at rest and on slight exertion ● Tachypnea ● Scalp sweating, especially in infants ● Fatigue and irritability ● Sudden weight gain ● Respiratory distress Implementation in CHF ● Reduce workload of the heart: ○ Decrease extra fluid (diuretics) ○ Strengthen cardiac function (digoxin) ○ Decrease afterload with vasodilators ■ Afterload is the amount of force needed for left ventricle to push blood through the body. ● Decrease cardiac demands ○ Allow for uninterrupted sleep periods ○ Small frequent feedings or gavage ■ The more food in the stomach, the more cardiac work it is to digest. ● Reduce respiratory distress ○ Count respirations carefully ○ Humidified O2 ○ Semi or high Fowlers Hypoxemia ● Color is not a great indicator ● SaO2 of 80-85%-- Saturation of Oxygen (arterial blood/Hemoglobin) ● Polycythemia (increase of the RBC’s d/t chronically low oxygen saturations) ● Clubbing ● Squatting to increase venous return ● Hypercyanotic spells Surgical intervention ● Early intervention prior to hypoxic episodes preferred ● Mortality rates vary from 2% to 25% ● Surgery should be done in major centers ALTERATION IN NUTRITION AND ELIMINATION Compiled By: Bheru Lal Page 60

Overview of function of GI system ● Responsible for taking in and processing nutrients for all parts of the body. ● Any problems can quickly affect other systems of the body ● In children, can affect overall health, growth, and development Overview of nutrition ● Infants ○ First 6 months can live off of breast milk or commercially prepared formula with iron added ○ May need to have Fluoride added if not already in the water. ● Cows milk is not recommended until 1 year of age d/t allergies ● First year is one of rapid growth ○ High protein ○ High calories ● A little about obesity ○ A baby who is overweight by the age of one will usually struggle with weight as an adult. Introducing solid foods ● 5-6 months: iron-fortified infant cereal mixed with breast milk, orange juice, or formula. ● 7 months: vegetables ● 8 months: fruit ● 9 months: meat ● 10 months: egg yolk Toddler Nutrition ● Appetite is usually smaller than infant because they are growing at a less rapid rate ● Tend to play with their food ● Want to feed themselves. Do not want to be fed. ● May also choose the same foods over and over. Preschooler and Nutrition ● Still not very big eaters at this age ● Parents should attempt to make meal times a pleasant experience for children. School age children and nutrition ● Good appetites ● Should begin the day with breakfast ● Usually hungry after school Adolescents and nutrition ● Growing so fast that they may always feel hungry ● May tend to eat faddish foods or those not very nutritious. ● May rebel against a parents wishes for them to eat good food ● This is a time when binging and unhealthy dieting may occur (even with athletes) ● Tend to not eat enough iron, calcium, and zinc Physiological differences: Adults vs. Children ● Internal distribution of water ○ Fluid is a greater fraction of their total body weight as compared to adults ■ Infants: 75-80% TBW Compiled By: Bheru Lal Page 60

2 years: 60% TBW Amounts stay approximately the same through later childhood and adult life ○ Body water is also distributed differently in infants than older children. ■ Infants have more interstitial fluids ■ Extracellular fluid compartment in infants includes 35-45% body water ● Insensible water loss ○ Loss of fluid through lungs and skin ○ Insensible water losses per unit of body weight are higher for an infant and younger child than an adult. ■ Total body surface is larger in infants/children ■ Body surface is the percentage of skin compared to total body weight. ■ Infants have more skin for their size. The more skin, the more fluid loss through skin ■ Infants and children have rapid respiratory rate and metabolic rate ○ All of these factors contribute to greater fluid loss through evaporation. ○ In addition, treatments or other conditions may increase fluid output ■ Activity, fever, diarrhea, vomiting ● Kidney function ○ During the first 2 years, kidneys are not mature ■ Do not excrete waste products efficiently ■ Difficulty concentrating or diluting urine ■ Sodium regulation mechanisms are not mature ○ Nurses want to make sure that kidneys are working before adding potassium to I.V. fluids. ● Other imbalances ○ Children are also more readily susceptible to imbalances in: ■ Serum glucose ■ Calcium ■ Potassium
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Glucose ● Infants and children have ○ Higher glucose needs due to high metabolic rate ○ Low glycogen stores ○ Hypoglycemia a threat under periods of stress Calcium ● Infants and children have: ○ Regulation of calcium less exact in infant than in older child or adult ○ When stressed, more growth hormone (GH) is secreted ■ GH increases result in increased calcium deposits in bone ■ Infant unable to keep up with these increased demands ■ Hypocalcemia results. Potassium ● Potassium concentration easily affected by decreased intake or intestinal illnesses (diarrhea) ● Decreased K can lead to cardiac arrhythmias Compiled By: Bheru Lal Page 60

K must fall below 3 mEq/L before symptoms show

Critical! ● Any condition that interferes with normal water and electrolyte intake or causes excessive losses will produce a more rapid depletion of fluid and electrolyte stores in the infant and child than it will in the adult. Example: ● Adults, when they do not eat for a day due to GI upset, and whose kidneys are normal, will have 14% less body fluid by the end of the day ● Infants who do not eat for a day, and whose kidney function is normal, will be 40% short of fluid by the end of the day! Urine output norms ● Infants: 2-3ml/kg/hr ● Toddlers/preschoolers 2ml/kg/hr ● School age: 1-2ml/kg/hr ● Adolescents: 0.5-1ml/kg/hr Physical assessment ● Skin ○ Color indicates the state of perfusion ○ As extracellular fluid volume decreases (as with dehydration), peripheral circulation decreases. ○ Note the following: ■ Warmth ■ Skin color: from pink to pale as peripheral circulation decreases (cyanosis is a late sign) ■ Hands will get cold before core temperature ■ Cap refill increases <2 seconds ● Turgor: ○ In dehydrated states: ■ Skin is unusually dry ■ Tenting of skin (is a later sign) ■ The best place to check skin turgor is the abdomen and medial area of the thighs ● Skin temperature ○ Decreases in fluid status; results in vasoconstriction of small arterioles ○ Vasoconstriction causes a decrease in skin temperature ○ Extremities usually remain cold even if the child has a fever ● Mucous membrane ○ Mouth is the most mucous membrane to assess for fluid deficit. ○ Note: ■ Amount of saliva in mouth ■ Lips and nares may be dry ■ Mucous membranes may be cracked. ■ Wrinkled tongue can indicate dehydration ■ Be sure dryness is not due to excessive mouth breathing—look at other variables as well. ● Head Assessment Compiled By: Bheru Lal Page 60

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Usually lack luster May appear sunken and dark After 4 months—if the infant does not produce tears, could indicate dehydration ○ Fontanels ■ Should be soft and flat ■ Sunken can represent dehydration (especially anterior) ● Vital signs ○ Pulse: ■ Usually the most sensitive index of fluid status ■ Pulse over 160 for infants and 120 for children could indicate hypovolemia ■ Pulse will usually be weak and thready ○ Respirations ■ When fluid volume is decreased, children tend to compensate for changes in the blood by hyperventilating. This can lead to the development of cherry red lips ○ Blood pressure ■ Usually the last vital sign to show any changes. When it falls, it’s usually hours after changes in pulse and respiration ○ Weight ■ The single most important variable to follow in planning treatment of fluid imbalances ○ Intake and output ■ Record all intake ■ Output – usually a decrease in urine output and an increase in specific gravity (the higher the specific gravity, the more dehydrated the person is). ■ Every hour is documented ○ Output ■ Weigh diapers ■ 1 gm = 1 ml ■ document all stools, vomiting, wound drainage, perspiration ○ Behavioral assessment ■ Changes seen with fluid volume deficit usually include: ■ Anorexia ■ Drawn, flaccid expressions ■ Decreased levels of activity ■ Increased seeking of comfort and attention Diarrhea ● Increase in stool frequency and content of water ● Caused by abnormal intestinal water and electrolyte transport ● More water in the intestines Acute diarrhea ● Major cause of infant mortality in developing countries ● Most cases caused by infectious agents, viral or bacterial, and parasites ● Chronic diarrhea is more likely related to malabsorption or inflammatory cause Compiled By: Bheru Lal Page 60

Infectious causes of acute diarrhea ● Viruses ○ Rotavirus is responsible for 50% of hospital admissions for dehydration and diarrhea ■ Also a nosocomial infection ● Bacteria ○ Salmonella ○ Campylobacter (ingested by contaminated food) ○ Clostridium difficile (C-Diff) Food poisoning ● Staphylococcus ● Clostridium perfringens ● Clostridium botulinum Parasites ● Giardia lamblia ● Cryptosporidium Etiology ● Spread by contaminated food or water ● Also from person to person ● More common in crowded conditions, such as day care centers and schools ● Infants immune system plays a role (the younger the infant, the less developed the immune system). Assessment of mild diarrhea ● Fever of 101-102 degrees F may be present ● Anorexia ● Irritable ● Appear unwell ● Episodes of diarrhea consist of 2-10 stools per day ● Mucous membranes are dry ● Pulse rapid ● Skin feels warm ● Skin turgor is not yet decreased at this time ● Urine output usually normal ● Skin color is pale Therapeutic management of mild diarrhea ● Is not serious at this stage—children can be treated at home ● Rest the GI tract for at least one hour; then offer water or oral hydration solutions such as pedialye. ○ Approximately 1 tablespoon every 15 minutes x 4 ○ Then 2 tablespoons every ½ hour x 4 ○ If retained, give small sips of fluid; avoid giving large amounts of fluid. Give in small doses ○ Do not give OTC drugs such as Imodium or Kaopectate because they’re too strong for little tummies ○ Depended on developmental age of child; instruct on good handwashing technique. Compiled By: Bheru Lal Page 60

Severe diarrhea ● May result from mild or may appear on it’s own ● Temp: 103-104F ● Pulse and respirations are weak and rapid ● Skin is pale and cool ● Infants may appear apprehensive, listless, lethargic (appears as a hypovolemic state) ● Obvious signs of dehydration ○ Depressed fontanel ○ Poor skin turgor ○ Sunken eyes ● Bowel movement every few minutes ● Stool is liquid green, may be mixed with mucus and blood (because the intestines are inflamed and irritated) ● Stool may be passed with explosive force ● Urine output scanty and concentrated ● Lab findings: ○ Increased hematocrit, hemoglobin, serum protein levels ○ Falsely high because the percentage is low ● Loss of 2.5-5% of body weight- mild dehydration ● Severe diarrhea can cause of loss of 5-15% of body weight quickly ● Any infant who has lost more than 10% if body weight requires immediate treatment Therapeutic measures ● Assessment of the fluid and electrolyte imbalance ● Rehydration ● Maintenance of fluid therapy ● Reintroduction of adequate diet ● Antimicrobial agents if necessary Nursing considerations: assessment ● Observe general appearance and behavior ● Assess for dehydration ○ Decreased urine output ○ Decrease in weight ○ Dry mucous membranes ○ Poor skin turgor ○ Sunken fontanel in infant ● More severe dehydration ○ Increase in pulse ○ Increase in respirations ○ Decreased blood pressure ○ Prolonged cap refill time ○ All signs of impending hypovolemic shock ○ Also assess for septic shock Nursing goals ● Urine output is more than 1ml/kg/hr ● Bowel movements are formed and fewer than 4 per day Compiled By: Bheru Lal Page 60

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Stool tests negative Blood pH more than 7 (to prevent metabolic acidosis) Specific gravity < 1.030

Malabsorption syndromes ● Celiac Disease ● Short bowel syndrome Celiac Disease ● Sensitivity or immunological response to the gluten factor of protein ● When gluten is ingested, a autoimmune response destroys part of the small intestine mucosal ● Body is unable to properly digest food and absorb nutrients, especially fats ● As a result, these children develop: ○ Steatorrhea (bulky, foul-smelling, fatty stools) ○ Deficiency of fat-soluble vitamins (A, D, E, K) ○ Protuberant abdomen ○ Rickets (disease in bone formation) due to a loss of vitamin D ○ Hypoprothrombinemia may occur due to loss of vitamin K (need Vitamin K to make prothrombin in the liver) ○ May also have iron deficiency anemia and low albumin (blood carrier molecule that helps maintain blood volume and blood pressure) levels. Assessment of Celiac disease: ● These children may be anorexic and irritable ● A “typical” celiac baby: ○ Would be fit and well until after the ingestion of gluten-containing solids (usually between 6 and 18 months of age) ○ Then the baby would develop pale, bulky, offensive smelling stools (steatorrhea), become miserable and lethargic ● They gradually fall behind other children in their age in height and weight ● Appear skinny with spindly extremities and wasted buttocks ● Face, however, may be plump and well-appearing Diagnosis of Celiac disease: ● History ● Clinical symptoms ● Serum analysis of antibodies against gluten ● Biopsy of intestinal mucosa (too determine degree of inflammation) ● Stool may be collected for fat content analysis ● Children are also put on a gluten-free diet and observed—response to this diet is usually very dramatic ● Continue children on a gluten-free diet for life ● Need to take water-soluble forms of vitamins A and D ● May also need to take iron and folate to correct anemia Gluten is not found in rice or corn. Celiac crisis: ● Can occur when any child with celiac disease develops an infection ● Usually experiences acute vomiting and diarrhea ○ Cause electrolyte and fluid imbalances Compiled By: Bheru Lal Page 60

Short Bowel syndrome and its causes ● A decrease in the amount of absorptive surface ● Caused by congenital malformations such as gastroschisis (infant born with some or all of intestinal contents outside of body; loses absorptive surface), or intestinal atresia. ● May be seen in children with intestinal ischemia due to malroation (twisting of intestines), intussusception (the slipping of one part of an intestine into another part just below it; ensheathed), and volvulus (infection which makes bowel obsolete). ● Occasionally seen in patients with chronic irritable bowel disease (IBD). ● Necrotizing entercolitis in preemies (tissue death in bowels due to colonization/infection and/or inflammatory changes). Care of the infant/child with Short bowel syndrome: ● Parenteral nutrition ● Enteric feedings if tolerated ● Monitor for vitamin and mineral deficiencies Mortality-related complications of SBS ● Sepsis ● Liver and biliary tract infections associated with TPN-causes cholestasis ○ Seen in children under 4 years of age ○ Leads to liver failure Obstructive disorders ● Hypertrophic pyloric stenosis ● Intussusception Pyloric stenosis ● Pyloric sphincter is the opening between the lower portion of the stomach and the beginning portion of the intestine (duodenum). ● If there is hypertrophy (increase in size) or hyperplasia (excessive proliferation of normal cells in normal tissue) of the muscle surrounding the sphincter, there will be problems with the stomach emptying called pyloric stenosis. ● At 4-6 weeks of age, infants begin to vomit with feeds ● Vomiting grows increasingly forceful until it is projectile; can project up to 3-4 feet (this is the most common symptom) ● Occurs most frequently in first born white males ○ 1:150 males ○ 1:750 females ● Cause is unknown, but is probably inherited ● Vomitus usually smells sour (because of increased gastric acid) Diagnosis of pyloric stenosis ● Made primarily from history ● When the parent says their infant is vomiting, we need to find out: ○ What is the duration, intensity, frequency, description of vomitus ○ Is the infant ill in any other way? ○ Many infants show signs of dehydration at the time of diagnosis. ● A definite diagnosis is made by watching the infant drink; there is usually an olive-sized mass in the right upper quadrant which becomes more prominent with drinking the water (seen on the outside of the body). Compiled By: Bheru Lal Page 60

May also confirm with ultrasound

Therapeutic management of pyloric stenosis ● Surgical correction: pyloromyotomy ○ Muscle of pylorus is split allowing for a larger lumen ○ Is usually done laparoscophy ○ Prognosis is excellent Nursing care postoperatively for pyloric stenosis: ● Feedings usually begun 4-6 hours post-op with 1 tsp (5 ml) of 5% glucose in saline hourly by bottle for four feeds ● If no vomiting, 2 tablespoons given hourly for the next four feeds ● Next, half-strength formula is given every 4 hours ● By 24 to 48 hours, infants are taking their full formula diet or being breastfed. Usually discharged after 48 hours. ● Do not give more fluid than ordered; risks for breaking open the newly operated areas. ● IV fluids decreased as oral amount increases ● Infants need to be bubbled/burped well after each feeding to decrease swelling; we don’t want air or gas to be in tummies ● Lay them on their side, preferably right side, to aid the flow of fluid through the pylorus via gravity ● Monitor daily weights ● Usually no vomiting occurs after the surgery. If it does, report immediately! Feeding regimen may need to be adjusted. ● Some infants experience diarrhea due to the rapid functioning of pyloric sphincter ● Elevate the head of the bed ● Monitor intake and output carefully; weigh all diapers Nursing care for the pyloric sphincter incision ● Care of the operative site: ○ Observe for any drainage or signs of inflammation ○ Care of incision as dictated by hospital policy ○ Keep diaper folded low to prevent contamination of incision ○ Change diapers frequently Intussusception ● Invagination of one portion of the intestine to another (telescoping is a good synonym for it) ● Generally occurs at 6-12 months ● Typically idiopathic in patients under 12 months ● May be related to another disorder in patients over 12 months. Treatment of intussusception: ● Surgery ● Reduction by fluid/air/barium (done in radiology) Motility disorders ● Hirschsprung’s disease (once known as Congenital Aganglionic megacolon) ● GERD (low esophageal and gastric mobility) Hirschsprung’s Disease (Congenital Aganglionic megacolon) Compiled By: Bheru Lal Page 60

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Absence of ganglionic innervation to a portion of the bowel Peristalsis does not occur in non-innervated bowel areas Patient’s have chronic constipation or ribbon-like stools

Treatment of Hirschsprung’s disease ● Surgery to remove the agangilionic colon segment ● May be done in 2 stages, with a temporary colostomy for 6-8 months to allow bowel to rest. Meckel’s Diverticulum ● In embryonic life, the intestine is attached for the umbilicus by a duct ● This duct becomes a ligament near term ● In 2-3% of infants, this duct remains open as a pouch off of the ileum Problems associated with Meckel’s diverticulum ● Some gastric mucosa (which secretes acidic fluids) may be displaced ● This may allow acidic, irritating secretions to flow into the intestine ● This irritates the bowel, leading to ulceration and bleeding ● Appendicitis symptoms ● Can serve as a lead point causing intussusception (from weakened area) ● A fibrous band extending from the diverticulum to the umbilicus can act as a constricting band around the bowel Treatment of Meckel’s diverticulum ● The diverticulum (pouch) is surgically resected. Structural defects ● Cleft lip and palate ● Esophageal atresia ● Tracheoesophageal fistula Cleft lip and palate ● Cleft lip occurs when there is a failure of the fusion of the maxillary and median nasal processes. ● Cleft palate occurs when there is a failure of the fusion of the palatal process (roof of the mouth) Associated Nursing Diagnoses for Cleft lip and palate ● Risk for fluid volume deficit ● Risk for altered nutrition; less than body requirements ● Risk for aspiration Treatment for cleft lip and palate ● Special nipples before surgery and while recovering ● Cleft lip surgery is usually done between birth and 10 months of age ● Cleft palate repairs are done at 18-24 months, so that anatomic changes in the palate contour are complete. ● Recovery is usually excellent ● Remember, these are typically stages surgeries Tracheoesophageal Fistula and Esophageal atresia ● Failure of the esophagus to develop normally, will end in a blind pouch or connect directly into the trachea instead of being a discrete passage. ● These defects may occur separately or in combination Compiled By: Bheru Lal Page 60

About half of the infants with TEF or EA also have associated anomalies, especially congenital heart disease and anorectal malformations.

Clinical manifestations of TEF and EA ● Excessive salivation (drooling) ● A newborn with drooling that is accompanied with choking, coughing, and sneezing, especially when fed. ● Fluid returns through the nose and mouth ● Infant becomes cyanotic and may stop breathing ● Most are corrected with one surgery or two or more staged surgeries. ALTERATION IN ACTIVITY AND MOBILITY Why study this topic? ● “Quest for mastery at every stage of development is related to mobility. Infant:

Speech, language, and overall development require sensorimotor activity.

Toddler ● Explore and imitate behaviors, a must for autonomy. Preschooler ● To express initiative, requires vigorous activity. School-age ● Strongly influenced by physical achievement and competition Adolescent ● Relies on mobility to achieve independence. Effects of Immobility: ● Affects all body systems in some way. ● Major physical consequences that impact children are: ○ Significant loss of muscle strength, endurance, and muscle mass (atrophy) ○ Bone demineralization leading to osteoporosis ○ Loss of joint mobility and contractures. ● Other effects: ○ Metabolism ■ Decreased metabolic rate ○ Cardiovascular system ■ Pooling of blood in extremities ■ Postural hypotension ■ Blood clots ■ Dependent edema ○ Respiratory system ■ Decreased chest expansion ■ Can lead to increased secretions, pneumonia ○ GI system ■ Constipation ○ Urinary system ■ Difficulty voiding ■ UTI’s (d/t retention and urinary stasis) ○ Integumentary Compiled By: Bheru Lal Page 60

Decreased circulation and increased pressure can lead to ulcers. Behavioral changes in Immobilized children: ■ Restlessness ■ Lack of concentration ■ Depression ■ Regression ■ Egocentrism ■ Decreased communication ■ Feel isolated ■ May become angry, aggressive or quiet and passive

Effects of Immobility and the family: ● Financial strain ● Siblings may feel ignored ● Guilt ● Caregiver becomes worn out Common Musculoskeletal problems ● Traumatic injury ○ Soft tissue injuries to the muscles, ligaments, and tendons are common in children. ○ In young children they occur from mishaps during play. ○ For older children and adolescents, they occur during sports. Soft Tissue Injuries ● Contusions (bruise) ○ Damage to soft tissue, subcutaneous structures, and muscles ○ Injured area is ecchymotic (black and blue discoloration) due to blood leaking into tissues; swollen and painful. ■ Cannot tell how long ago injury occurred by the bruise. ○ Immediate treatment is application of cold. ● Sprains ○ Occur when trauma to a joint is so severe that a ligament is partially or completely torn or stretched. Usually involves damage to blood vessels, muscles, tendons, and nerves. ○ Child may describe a “snap, pop, or tearing”. Therapeutic management of soft tissue injuries ● The first 6-12 hours is the most critical for almost all soft tissue injuries. ● Basic principles include RICE or ICES ○ R.I.C.E.: Rest, ice, compression, elevation ○ I.C.E.S.: Ice, compression, elevation, support ● Ice immediately; do not apply for more than 30 minutes ○ Reverse effects: vasodilation occurs ● Elevate the extremity above heart level. ● Torn ligaments, especially those in the knee, are usually made immobile with a cast or splint for 3-4 weeks. ● Passive leg exercises, gradually increased to active leg exercises, begun as soon as sufficient healing has occurred. Fractures Compiled By: Bheru Lal Page 60

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Common injury at any age but more likely to occur in children and older adults Usually due to traumatic incidents at home, school, in a motor vehicle, or associated with recreational activities Rarely occur in infants unless there is an underlying hematology disorder Toddlers- be suspicious of fractures at various stages of healing School-age children—usually due to bicycle-automobile or skateboard injuries Adolescents: bikes, motorcycles, sports

Common Fracture sites: ● Forearm: falling on the outstretched hand. ● Clavicle fractures are common ● Hip fractures are rare ● Auto/pedestrian—from 4 to 7 years of age ○ Triad of injury: ■ Level of the bumper, fractured femur ■ Hood of the automobile, injury to child’s truck ■ Contralateral (Originating in or affecting the opposite side of the body) head injury due to impact Types of fractures in children ● Bends: Deformity without breaking (think of a young, green stick that won’t break, but it bends) ● Buckle fractures: Compression of the porous bone, raised or buldging projection at site. (not broken all the way through) ● Greenstick fracture: incomplete fracture, bone is angulated beyond limits of bend. ● Complete fracture: Divides bone fragments Complete fractures ● Transverse: straight up and down ○ Crosswise, at right angles to the long axis of the bone ○ Oblique: Slanting but straight, between a horizontal and perpendicular direction (diagonal). ○ Spiral: slanting and circular, twisting along the bone shaft ○ Comminuted: broken into pieces (crushed). Fractures, continued ● Closed or simple ● Open or compound (protruding through the skin) Growth plate or Epiphseal injuries ● Weakest part of long bones ● Frequent site of damage ● Fracture may be through degenerating cartilage cells, without damage to growth ● Other fractures of the epiphysis may cause growth problems such as shortening of the limb Associated problems with fractures in general ● Muscles contract and spasm ● Bones are pulled out of alignment ● Severe hemorrhage in tissues, especially with femur fractures ● Vascular injuries ○ Femur fracture may cause injury to sciatic nerve ○ Bone marrow is high vascularized Compiled By: Bheru Lal

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5 P’s of Ischemia ● Pain ● Pallor ● Pulselessness ● Paresthesia ● Paralysis Nursing actions for fractures ● Support the injured limb; splint, DO NOT MOVE OR STRAIGHTEN OUT THE BONE. ● Ask the child to point to where it hurts ● Ask the child to wiggle fingers or toes ● Check distal pulses ● Get historical information from someone who witnessed the injury if possible. Diagnostic Evaluation ● History may be lacking! ○ Young children cannot tell ○ Older children may not tell the truth in fear of repercussions ● Radiography is the most useful tool Therapeutic management of fractures ● Reduction: regain alignment and length; “setting bone”. ● Immobilization: retains alignment and length ● Restore function ● Prevent further injury Bone healing and remodeling ● Rapid in children ○ Due to thickened periosteum (the fibrous membrane that forms the covering of bones). ○ Generous blood supply from bone marrow ● New bone cells are formed in immense numbers almost immediately after the injury—immediate treatment is detrimental ● Examples of healing times for femoral shaft in different age groups: ○ Neonatal period: 2-3 weeks ○ Early childhood: 4 weeks ○ Later childhood: 6-8 weeks ○ Adolescents: 8-12 weeks ○ The general rule of thumb is that the younger the child is; the quicker the bones heal. Delays in healing ● Gaps between fragments delays or prevent healing ● Prompt healing with end-to-end apposition (two normally contiguous parts) ○ Though, may cause bony overgrowth and greater length of extremity ● Angulated deformity may remodel in young children. Careful reduction and follow-up to prevent permanent disability. Fracture complications ● Circulatory impairment Compiled By: Bheru Lal Page 60

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Nerve compression syndromes Non-union Pulmonary emboli (fat, blood, and air)

Circulatory impairment—very important ● Absence of pulse, discoloration, swelling, pain ● Report immediately to practitioner ● Take steps to improve circulation (take action!) ○ Loosen ace wrap ● Most common lawsuits against nurses Compartment syndrome ● Typically caused by blunt trauma ● Pressure rises within this space with tight dressings or casts, hemorrhage, trauma, burns, and injury ● Most common symptoms ○ First sign: Pain out of proportion to injury ○ Second sign: Tenseness on palpation ○ Third sign: motor weakness Non-union fractures ● Failure of bone fragments to unite ● Failure to unite due to any of the following: ○ Separation of bone fragments at fracture site ○ Hematoma (swelling of comprising a mass of extravasated blood, usually clotted, caused by a break in a blood vessel) ○ Extra tissue between bone fragments ○ Necrosis of bone tissues or infection ○ Interruption of blood supply ● Bone grafting may be required Pulmonary Emboli ● Blood, air, or fat (fat tends to occur faster) ● May not occur for 6-8 weeks after injury ● Fat emboli, first 24 hours usually in adolescents ● Signs and symptoms: ○ Dyspnea ○ Chest pain ○ Onset of sudden pain ● Nursing interventions ○ First: Elevate the head ○ Second: Administer oxygen ○ Notify physician immediately Cast care ● Risk for altered peripheral tissue perfusion related to pressure from cast ● Expected outcomes: ○ No pain or numbness in extremity ○ Distal nail beds blanches and refills in less than 3 seconds ○ Pedal pulses palpable Compiled By: Bheru Lal Page 60

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Area surrounding case is warm and pink Interventions: ○ Keep extremity elevated ○ Check circulation every 15 minutes during the first hour, every hour for 24 hours, then every 4 hours thereafter ○ Assess for numbness or tingling (children under 6 or 7 may not be able to express this) ○ Edema that does not improve with elevation should be reported ○ Assess for the 5 P’s—if any of these symptoms are present, requires immediate attention! ■ Can lead to permanent paralysis of extremity. Risk for impaired tissue integrity related to pressure from cast ○ Edges of cast are usually covered by a fold of stockinet ○ If no stockinet, adhesive tape strips are applied to prevent skin irritation (petaling) ○ If the cast is surrounding the genital area, or there is any chance of cast coming into contact with stool or urine, need to cover with plastic. Prevention is very important as once urine has penetrated a cast, there is no way to remove it. Can clean cast with a damp cloth when soiled with food Knowledge deficit related to cast care ○ Instruct parents/child on how to care for cast ○ Instruct on 5 P’s

Casting complications ● Continued swelling could cause cast to become a tourniquet. ○ This is why we wait a day or two to cast ● Parents should receive verbal and written instructions ● Teach neuro/circulation checks to parent’s (5 P’s) ● Document parent’s response to teaching. Always have parents re-demonstrate Cast removal ● Most casts in place to 6-8 weeks ● Removed with electric cast cutter ● Skin usually looks macerated and dirty, a good bath will remove dirt. ○ Atrophy will resolve in its own ● Once healing takes place, the extremity is as strong as before—but children will usually favor the extremity. Allow them to regain full use on their own time schedule. Scoliosis ● Lateral (sideways) curvature of the spine ● May involve all or only a portion of the spinal column ● Functional scoliosis (in response to another condition) ○ Occurs as a compensatory mechanism ○ Usually due to unequal leg lengths ○ Created a pelvic tilt that is C-shaped ○ Must correct the initial problem ■ A lift placed in one shoe Compiled By: Bheru Lal Page 60

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Remind the child to maintain good posture (walking with book on head 3 x daily for 10 minutes) ■ Sit-ups and push-ups are good exercises Structural scoliosis Permanent curvature of the spine with damage to the vertebrae Spine has an S-shaped appearance Usually there is a family history 5x more common in girls than boys Usually peaks between 8-15 years (school age) Diagnosis is made on physical exam by having the child bend forward X-rays confirm diagnosis Therapeutic management: ○ If spinal curve is less than 20 degrees, no therapy except close observation until the child reaches 18 years of age) ○ If greater than 20 degrees, may use braces, traction, surgery, or combination.

Nursing care of the immobilized child: Goals and interventions ● Goal One: Increase physical mobility related to mechanical restrictions, physical disability ○ Transport child by gurney, stroller, wagon, bed, WC from room to play room, lobby, or other area as allowed ○ Change position of bed in room ○ Out of bed in chair, wagon, etc. as allowed ● Goal Two: Assist child to maintain optimal autonomy ○ Provide mobilizing devices (crutches, WC) ○ Assist with acquiring specialized equipment ○ Instruct in use of equipment ● Encourage activities that require mobilization ● Allow as much freedom as possible and encourage normal activities ● Encourage child to participate in own care ● Allow child to make choices High Risk for Impaired Skin integrity ● Goal: Maintain skin integrity ○ Place child on pressure-reducing mattress ○ Change position frequently, unless contraindicated ○ Protect pressure points with proper positioning and cushioning ○ Inspect skin surfaces for signs of breakdown ○ Eliminate mechanical factures that cause friction ○ Good skin care ○ Gently massage skin area until contraindicated High risk for injury ● Child will experience no physical injury ○ Teach correct use of devices (child and parent) ○ Assist with moving and/or ambulating ○ Remove hazards from environment ○ Modify environment as needed ○ Keep call button within reach Compiled By: Bheru Lal Page 60

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Keep siderails up at all times Help child use bathroom or commode if possible Implement safety measures appropriate to child’s developmental stage Diversional activities MUSCULAR DYSTROPHIES

The largest group of muscle disorders of childhood Three types: ● Congenital myotonic dystrophy ○ Begins in utero ○ Newborn may already have significant myotonia (muscle weakness) ○ Usually die before one year of age due to respiratory failure ■ Assessory muscles for respiration fail Facioscapulohumeral Muscular dystrophy ● Begins after 10 years of age ● Facial weakness is the predominant symptom ● Very slow progression of symptoms ● Less disability than the other types ● Normal lifespan is possible. Pseudohypertrophic Muscular Dystrophy/ Duchenne’s Disease ● Most common form of MD ● Symptoms usually appear by age 3 years ● Occurs only in boys ● Late in meeting motor milestones ● Muscle weakness gradually becomes more pronounced ● Usually wheelchair-dependent by adolescence ● Heart and respiratory failure may occur by early adulthood Cerebral Palsy Cerebral Palsy ● A group of non-progressive disorders of upper motor neuron impairment that result in motor dysfunction. ● Can happen before, during, or after birth ● Occurs 2:1000 births ● Most common permanent disability of childhood Incidence and Causes of CP ● Most frequently associated with brain anoxia that leads to cell destruction ○ Symptoms can range from very mild to quite severe, depending on the extent of brain damage ● Also can be caused by: ○ Kernicterus (a form of jaundice from hyerbilirubinemia; staining of the brain with bilirubin) ○ Meningitis (viral is the most common) ● Occurs most frequently in very low weight infants (born prematurely), or those small for their age. ○ Their lungs haven’t been fully developed Compiled By: Bheru Lal Page 60

CP has increased over the past decade due to: ○ Preemies are living longer ○ Multiple births from artificial reproductive technologies ○ Prenatal technology

Types of CP ● Two main categories based in type of neuromuscular involvement ○ Pyramidal or spastic (50-70% of children with CP) ○ Extrapyramidal (outside of the pyramidal tracts of the CNS) ■ Ataxic (awkward, unsteady gait) ■ Dyskinetic (defect in ability to perform voluntary movement) ■ Athetoid (slow, irregular, twisting, snakelike movements occur in the upper extremities, esp. in the hands and fingers) ■ Mixed Spastic or pyramidal CP ● Pyramidal system: conveys nerve impulses that create voluntary movements ● Problems in this area result in: ○ Hypertonicity: excessive tone in the voluntary muscles ○ Abnormal clonus: rapidly alternating involuntary contraction of skeletal muscle ○ Exaggeration of deep tendon reflexes ■ Abnormal reflexes such as a positive Babinski reflex ● Continue to have neonatal reflexes past usual age (tonic neck reflex) ● Arch their back and extend arms/legs abnormally when held in a ventral suspension position ● Fail to do a “parachute” reflex if lowered suddenly (do not extend arms/hands in front of self) ● Assume a “scissors gait” due to tight adductor thigh muscles which cause their legs to cross when held upright. ● May have tightening of heel cord which causes the child to walk on toes; unable to stretch heel to touch the ground. ● Spastic involvement may affect: ○ Both extremities on one side (hemiplegia) ○ All four extremities (Quadriplegia) ○ Primarily lower extremities (paraplegia or diplegia) ● Children with quadriplegia: ○ Usually have impaired speech ○ Swallowing is difficult—drool, problems eating ○ May have cognitive impairment Extrapyramidal CP ● Extrapyramidal nerve tract conveys nerve impulses that effect autonomic movements: ○ Help coordinate body movements ○ Maintain skeletal muscle tone ○ Play major role in equilibrium ● Ataxia (defective muscular coordination) ● Dyskinetic (a defect in the ability to perform voluntary movements) ● Athetoid—“wormlike” Compiled By: Bheru Lal Page 60

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Limp and flaccid muscles as an infant Later, child makes slow, writhing motions (in place of voluntary muscles) May involve all four extremities, face, neck, tongue Due to poor tongue and swallowing movements, child may have poor speech and problems with drooling ■ Concerned about aspiration With emotional stress, involuntary movements may become irregular and jerky Children have an awkward, wide-based gait On neurologic exam, unable to touch finger-to-nose or due rapid, repetitive movements Combination of more than one condition listed above

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Diagnostic evaluation for diagnosis of CP ● Neurological exam ● History—especially born prematurely ● Ultrasound of brain ● CT scan ● MRI Physical findings that may suggest CP ● Delayed motor development ● Abnormal head circumference (head is typically larger) ● Abnormal postures ● Abnormal reflexes ● Abnormal muscle performance and tone ● 25-75% of children have cognitive defects ● may have visual problems Medical management of CP ● Overall goal—develop a rehabilitation plan to promote optimum function ● Multidisciplinary teams ○ OT, PT, Speech ● As child grows, would include therapeutic exercises, splints, braces ● Antispasmodic drugs may also be used (Baclofen), but may have little effect ● Surgery to lengthen heel tendons may be done ● Wheeled walkers or scooter boards ● Cerebellar pacemakers may decrease spasticity in some children ○ Also called Baclofen pumps Nursing Diagnoses ● Altered growth and development ● Impaired physical mobility ● Self-care deficits (bathing/toileting/dressing) ● Self-esteem disturbance ● Impaired social interactions General interventions ● Promote maximal functioning of joints ● Provide adaptive equipment for activities of daily living Compiled By: Bheru Lal Page 60

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Modified utensils for eating Push panels for computer Electric switches for battery operated toys Position to prevent contractures ○ Perform active and passive ROM exercises, must be done daily Provide adequate nutrition—often have difficulty swallowing Encourage verbalization of feelings about altered body image Encourage social interaction with peers Teach patient and family how to maintain independence Identify support groups
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Long term care for CP ● Sometimes children are not diagnosed with CO until 2-4 years later. This can be upsetting to parents. Will need much support and education. Febrile convulsions Febrile convulsions ● Seizures associated with high fever (102-104 degrees F) ● Most common in preschool children or between 5 months and 5 years of age ● Usually no more than 5-7 of these episodes occur in a child’s life Seizure activity ● Seizure usually lasts 15-20 seconds ● Shows an active tonic-clonic pattern (alternately contracting and relaxing the muscles) ● EEG tracing usually normal ● Usually a family history ● Seizures subside once the fever is gone Prevention of Febrile convulsions ● Give Tylenol to keep fever below 101 ● Often fever develops during the night when parent/caregiver is not with child ● If child has one febrile seizure, no further treatment given other than to advise parents to administer Tylenol to keep fever below 101 ● If more than one seizure, child may be put on Phenobarbital (controversial) Therapeutic management of seizures ● Teach parents that after the seizure subsides, they should: ○ Sponge the child with tepid water ■ Do not put child in bathtub ■ Do not use rubbing alcohol or cold water ■ Do not give Tylenol right after the seizure (not awake enough to swallow) ● If unable to decrease temperature by sponging, advise parents to: ○ Put a cool washcloth on child’s forehead, axillary, and groin area’s (which are temperature receptors) Healthcare facility will: ● Determine underlying cause ● Lumbar puncture to rule out meningitis ● Antipyretic drugs Compiled By: Bheru Lal

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Antibiotic therapy if needed Assure parents that febrile convulsions do not lead to brain damage and child will be well. BACTERIAL MENINGITIS

Infections or inflammation of the cerebral meninges (the membranes covering the brain and spinal cord) ● 90% of cases are between 1 month and 5 years ● Peak incidence is in the winter. ● Causative organisms (95% of cases) ○ H. Influenzae (type B) ○ Streptococcus pneumoniae ○ Neisseria meningitis- epidemic form; droplet from nasophargeal secretions ■ MUST be put on droplet isolation Pathophysiology of Bacterial meningitis ● Pathologic organism spreads to the meninges from upper respiratory tract or by lymphatic drainage from the sinuses. ● Once pathogen enter the meningeal space, they spread rapidly ● Produces an inflammatory effect that leads to thick exudates that blocks CSF flow. ● Brain becomes edematous, covered with purulent exudate. ● Spreads VERY quickly through CNS Clinical signs in Children and Adolescents ● Usually have 2-3 days of a cold, upper respiratory infection and occasionally and ear infection. ● Become VERY irritable due to headache ● May have convulsions ● photophobia ● As the disease progresses, more signs of meningeal irritability occurs: ○ Positive Brudzinski’s (image on page 674) ■ When child’s head is flexed forward (while laying on back), both hips, knees and ankles flex. This shows meningeal irritation ○ Positive Kernig’s (image on page 674) ■ Flex child’s hips and knee (while laying on back) ■ Then extend leg—this will cause pain, resistance and spasm which indicate irritation. ○ Nuchal rigidity occurs (neck stiffness) ○ In the newborn—poor sucking, weak cry, lethargy Diagnostic Evaluation—Lumbar Puncture ● Obtained by history and analysis and CSF via Lumbar puncture ○ Culture and gram stain identify causative organism ● Blood cell count—WBC elevated ● Lowered glucose ○ Increased metabolic rate due to the body and brain trying to fight off infection; draws glucose out of blood for energy. ● Protein content increased ○ Due to extra cells and metabolism occurring in the CNS Compiled By: Bheru Lal Page 60

Therapeutic Management ● Medical emergency! ○ Directly put on droplet isolation precautions ○ IMMEDIATE antimicrobial therapy ○ Hydration ○ Ventilation (not in all cases) ○ Reduction of increased ICP ○ Management of shock and Disseminated intravascular coagulation (DIC) ■ Tidbits on DIC: Normally, when you are injured, certain proteins are turned on and travel to the injury site to help stop bleeding. However, in persons with DIC, these proteins are abnormally active. Small blood clots form throughout the body. Overtime, the clotting proteins become "used up" and are unavailable during times of real injury ■ This disorder can result in clots or, more often, bleeding. Bleeding can be severe. ○ Control of seizures, temperature

Photo of Disseminated intravascular coagulation

INCREASED INTRACRANIAL PRESSURE Not a single disorder, but a sign that may occur with many neurological disorders One such cause is a brain tumor The rate at which symptoms develop is: ○ Cause ○ Ability of child’s skull to expand to accommodate the increased pressure ■ The younger the child, the more flexible the skull ○ Location ○ Size and growth rate of tumor ● Children with open fontanels withstand more pressure than older children
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Signs of ICP: Compiled By: Bheru Lal Page 60

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Increased head circumference ○ >2cm/month in the first 3 months ○ >1cm/month in the 2nd three months ○ >0.5cm/month for the ext six months Fontanel changes ○ Anterior fontanel tense and bulging; closes late Vomiting ○ Occurs without nausea (rarely with nausea) ○ Occurs upon awakening in the morning or after a nap ■ The fluid is not moving when sleeping ○ May be projectile Eye changes ○ Diploplia (double vision) from pressure on abducens nerves ○ White of sclera evident over top of cornea (“sunset eyes”) ○ Limited visual fields ○ Papilledema (retinal edema—cannot see on physical assessment; need special equipment) Visual sign changes ○ Elevated temperature and blood pressure (body regulators are compressed) ○ Increase in pulse pressure (the gap between the systolic and diastolic) ○ Decreased pulse and respirations due to the growing pressure on brain stem which controls: ■ Cardiac rate and respirations Pain ○ Headache on awakening and standing. Increases with valsalva maneuver) or holding breath ■ Each increased intrathoracic pressure Mentation ○ Irritability, decreased level of consciousness, altered LOC Later signs: ○ Personality changes ○ Seizures ○ Head tilt (because of heavy tumor) ○ Decreased LOC ○ Decreased motor response to stimuli ○ Decreased sensory response to painful stimuli ○ Alterations in pupil size and reactivity ○ Cheyne-stokes respirations ○ Decerebrate or decorticate posturing (pg. 1674) ■ This is very bad 

Glasgow coma scale ● Most widely used of the pediatric coma scales ● Standardized scale to describe and interpret the degree of LOC in persons with brain injury ● The lower the score, the deeper the coma ● 3 parts: ○ eye opening ○ verbal response Compiled By: Bheru Lal Page 60

motor response ● highest score is 15 ● lowest score is 3 ● Medical management is based on these scores

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BRAIN TUMORS The most common solid tumor that occurs in children, secondary to leukemia Tends to occur between 1 and 10 years of age (5-years is the peak) In children, tends to occur in areas of the brain where they are difficult to remove. Signs and symptoms due to increased intracranial pressure (ICP)

Treatment of Brain tumors ● 4-6 months may pass from first symptom before diagnosed ● diagnostic tests: ○ skull films ○ bone scan ○ MRI ○ Cerebral angiography (difficulty getting dye through due to abnormal vascularization) ○ CT scan Therapeutic management of brain tumors ● Includes a combination of surgery, radiotherapy, and chemotherapy ● Preoperative care ○ Will usually receive a stool softener—do not want child to strain after their brain is operated on ○ A portion of the head is shaved; very traumatic for adolescent ○ If going to the ICU after surgery, have child meet the ICU staff Postoperative care ● Positioning ○ Depends on location of tumor; usually on the opposite side of incision ○ Bed is flat or slightly elevated ○ DO NOT lower the head of the bed ○ Child will be very lethargic due to brain swelling ● Assess VS every 15 minutes initially until stable. Eventually will decrease to every 4 hours ● Monitor IV fluids very carefully. Too much fluid can cause edema in the brain ○ We don’t want the child to be dehydrated, but we do want them on the “drier” side ● Prevent nausea and vomiting Therapeutic management of ICP ● Identify source and remove ASAP ● Keep coughing, vomiting, and sneezing to a minimum ● Place child in a semi-fowler’s position (infant seat for infants) ● Monitor IV fluids very carefully ● Meds: ○ Corticosteriods (Decadron)—reduces inflammation ○ Osmotic diuretic (mannitol)—pulls fluid out of tissues Compiled By: Bheru Lal Page 60

Will usually insert foley catheter prior to administration for accurate I & O’s

DOWNS SYNDROME A generalized syndrome—1:800 to 1:1000 live births Etiology unclear 90% + cases attributable to an extra chromosome 21 (trisomy 21) Statistically greater risk if mother is over 35, but 80% born to women under the age of 35. ● Paternal age may also be a factor ● Degree of physical and cognitive development impairment related to the percentage of cells with abnormal chromosomal makeup
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Clinical manifestations of Down syndrome ● Intelligence varies from severely affected to near-normal intelligence ● Social: 2-3 years behind mental age, especially in childhood ● Congenital anomalies: 30-40% has a congenital heart disease, especially septal defects. May also have GI and ortho alterations. ● Respiratory—infections very prevalent ○ Due to hypotonia; swallowing muscles are weak—prone to aspiration ● Growth—rate reduced in height and weight as children; but often overweight as teens/adults ● Sexual development—may be delayed, incomplete, or both Physical manifestations ● Head—separated sagital suture ● Face: flat profile ● Eyes: upward, outward slant ● Nose: small and depressed ● Ears: small, sometimes low set ● Mouth: high-arched palate, downward curve, especially when crying ● Hands: broad, short, transverse palmar crease (simian line) ● Feet: wide space between great and first toes, plantar crease between great and second toes. ● Hypotonia Prognosis with Down syndrome ● Improved in recent years ● Significantly lower than for the general population ● Survival at one year with CHD: 76%; at 20 years of age: 53% ● Survival at one year without CHD: 91%; at 20 years of age: 82% ● Dramatic increase in mortality after the age of 44, virtually all have neuro changes similar to Alzheimer’s disease Possible nursing diagnoses for DS ● Potential for infection related to hypotonia, increased susceptibility to respiratory infection ● Impaired swallowing related to hypotonia, large tongue, cognitive impairment ● Altered family processes related to having a child with Down syndrome ● Altered growth and development related to impaired cognitive functioning ● Potential for injury due to hypotonia/cognitive impairments high risk for falls Compiled By: Bheru Lal Page 60

FETAL ALCOHOLIC SYNDROME Characteristic facial and associated features due to excessive ingestion of alcohol by mother during pregnancy ● Degree of alcoholism not related to defects of FAS ● Is related to liver’s ability to detoxify ● Circulating (unmetabolized) alcohol has an affinity for brain tissue

Newborns with FAS ● Measures to prevent seizures ● Avoid overstimulation (can bring on seizure) ● Provide sedation ● Anticonvulsants as ordered and indicated Major features of FAS ● Facial: thinned upper lip with vertical ridge, short upturned nose ● Neuro: mental retardation, motor retardation, microcephaly, poor coordination, hypotonia, hearing disorders ● Behavior: irritable, hyperactive ● Growth: Prenatal growth retardation (IUGR), persistent lag after birth, very small and thin HYDROCEPHALUS ● Caused by imbalance in production and absorption of CSF ● CFS accumulates within ventricular system of brain, producing dilation of ventricles. Mechanisms of Fluid imbalance in Hydrocephalus ● Tumor of choroid plexus (the area that produces CSF in brain) may cause increased secretion of CFS. ● Choroid tumors are rare, but structural malformations may cause impaired absorption or obstruction to outflow of CSF. ● Imbalance of secretion and absorption of CFS causes CFS to accumulate in the ventricles, which dilate and compress against cranium ● Skull also enlarged ● Most are a result of developmental malformations (in ventricular system) ● Usually presents in infancy, but can also be up to early childhood ● Other causes: infections, neoplasms, trauma, brain damage Clinical manifestations of hydrocephalus ● Influenced by acuity of onset and presence of pre-existing structural lesions ● In infancy, head grows at an abnormal rate ● Anterior fontanel tense, bulging, dilated scalp veins (due to the skin stretching) Manifestations of Hydrocephalus in childhood ● Caused by increased ICP ● Headache upon awakening with improvement following emesis or upright posture ● Papilledema (edema and inflammation of optic nerve) ● Strabismus ● Ataxia ● Irritability/lethargy ● Confusion Compiled By: Bheru Lal Page 60


Diagnosis ● Head circumferences ● Associated Neuro signs ● CT, MRI, skull x-ray ● Dye inserted into ventricle through anterior fontanel—will not appear in CSF from lumbar puncture if non-communicating Therapeutic management ● Relief of hydrocephalus ● Treatment of complications ● Management of issues related to psychomotor alterations Surgical treatment ● Direct removal of obstruction if present (cyst, neoplasm, Hematoma) ● A shunt is inserted under the skin the drain ventricles, may include a reservoir to add medications and remove fluid. More on Shunts… ● Valves open at a predetermined intraventricular pressure and close when the pressure falls below that level (prevents backflow) ● Ventriculo-Peritoneal (VP) shunt is preferred for infants and young children ● Ventriculo-Atrial (VA) shunt (ventricle to right atrium) reserved for older children who have attained most of their growth and for children with abdominal pathology (perforation of bowel, etc.) Complications of shunts ● Mechanical obstruction within ventricles from tissue or exudates, displacement related to growth, thrombus (clot) ● Often presents as an emergency; increased ICP and worsening of neuro status ● Infection—the most serious complication. May occur at any time but the greatest risk is 1-2 months after placement. (tubing colonized with bacteria) Postoperative care after shunt placement ● Position on un-operated side ● May need to keep flat to avoid too rapid reduction of intracranial fluid ● Observe for signs of increased ICP ○ If increased ICP occurs, elevated the HOB to 15-30 degrees to enhance gravity flow through the shunt ● Monitor I & O’s carefully, may be on a fluid restriction ● Presence of bowel sounds determined before feeding infant with VP shunt (in case the bowel was perforated at the time of placement—do not want shit leaking into the tube) Signs of CSF infection ● Elevated vital signs ● Poor feeding ● Decreased LOC ● Seizures Associated nursing diagnoses ● Potential for injury related to increased ICP ● Potential for infection related to presence of mechanical drainage system Compiled By: Bheru Lal Page 60

Altered family processes related to having a child with a chronic illness NEURAL TUBE DEFECTS Spina Bifida: defect in closure of the vertebral column with varying degrees of tissue protusion Spina Bifida Occulta: ○ Posterior vertebral arches fail to close in the lumbrosacral area ○ Spinal cord remains intact and usually is not visible. ○ Meninges are not exposed on the skin surface ○ Neurological deficits are not usually present Spina Bifida cystica: ○ Protrusion of the spinal cord and/or it’s meninges occurs ○ Defect results in incomplete closure of the vertebral and neural tubes, resulting in a sac-like protrusion in the lumbar or sacral area, with varying degrees of nervous tissue involvement Meningocele: ○ Protrusion involves meninges and a sac-like cyst that contains CSF in the midline of the back, usually the lumbosacral area ○ Spinal cord is not involved ○ Neurological deficits are usually not present. Myelomeningocele: ○ Protrusion of meninges, CSF, nerve roots, and a portion of the spinal cord occurs. ○ The sac (defect) is covered by a thin membrane that is prone to leakage and rupture ○ Neurological deficits are present.

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Clinical manifestations ● Vary according to degree of spinal defect ● Neuro dysfunction related to anatomic level of defect and nerves involved ● Defective nerve supply to bladder often causes urine dribbling or overflow incontinence ● Poor anal-sphincter tone; lack of bowel control and rectal prolapse ● There may be saddle anesthesia with bladder and anal sphincter paralysis ● May also have ortho involvement; joints, kyphosis, scoliosis, hip dislocations Diagnostic evaluation ● Clinical manifestations ● Meningeal sac (can be transilluminated) ● Ultrasound prenatally Care of the myelomeningocele sac ● Evaluate the sac and measure the lesion ● Protect the sac; cover with a sterile, moist (normal saline) dressing. May include an antibiotic in the solution. Change every 2-4 hours. ● Device to maintain body temperature without clothing or covers that irritate the sac. ● Place in prone position to minimize tension on the sac and the risk of trauma; the head is turned to one side for feeding. Compiled By: Bheru Lal Page 60

Assess for early signs of infection; elevated temperature, irritability, lethargy, nuchal rigidity.

Associated Nursing Diagnoses ● Potential for infection related to presence of infective organisms, nonepithelialized meningeal sac ● Potential for trauma r/t delicate spinal lesion ● Potential for impaired skin integrity r/t paralysis, continual dribbling of urine or feces ● Potential for trauma r/t impaired cerebrospinal circulation ● Potential for injury r/t neuromuscular impairment

Compiled By: Bheru Lal

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