Overview of Child Health Nursing Lecture Notes (pediatrics)

Overview of 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 o Number of deaths per 1000 live births during 1st year of life. o Infant mortality rate is an indicator of how healthy the nation is. o This rate is used to compare national health care to previous years and to other countries. o 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: o Motor vehicle accidents are the leading cause of death in children over 1 year of age. o Majority of deaths are due to no use of seatbelts.

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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 3rd in boys from 1-14 years old.

Childhood Morbidity (Illness) • •

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An illness or injury that limits activity, requires medical attention or hospitalization, or results in a chronic condition. Examples: o Congenital heart defects o Asthma o Cerebral Palsy o 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

Growth and Development • •

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Growth: used to show an increase in physical size or a significant change. Includes: o Height o Weight o Head circumference Development: Used to denote an increase in skill or ability to finction. Measured by: o Observing child do specific tasks o Parents description of child’s ability o Using standardized testing (Denver II screening test)

Principles of Growth and development

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G & D is a continuous process from birth to death o Rate of growth varies at different times G & D proceeds in an orderly manner o Growth from smaller to larger o 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 o Level of activity differs widely among children Rhythmicity o Have a regular rhythm is physiologic terms Approach

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o Child’s response to a new situation Adaptability o Is the child able to adapt to new situations? Intensity of reaction Distractibility Attention span and persistence Mood quality

Categories of temperament •

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The “Easy” Child o 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. o 40-50% of children fall into this category The “Difficult” child o Children are “difficult” if irregular in habits, have negative mood quality, and withdraw from new situations o 10% of children fit this image. “Slow to warm up” child o Describes children who are overall fairly inactive, respond only mildly, adapt slowly to new situations, and have a general negative mood. o 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 • •

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Know norms; child vs. adult A & P variations are farthest from adult norms at birth o Most of these variations mature quickly in the first year o 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 • • • •

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Always think of where the child is developmentally. Approach must be individualized 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. o Place left hand on shoulder while auscultating the heart. o Move unhesitatingly, firmly, and gracefully. o Talk pleasantly and reassuringly. Instructions to the child: o Use a directive voice o Have specific instructions o 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 o On parents lap o On the floor o Examiners lap

Conducting the exam •

Perform the least distressing procedures first and the most distressing last. o Heart and lungs; have the child lie down. o Abdomen, throat, and ears (throat and ears are the worst) o Genitalia and rectum

Physical exam technique • •

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Inspection o Observe before you touch Auscultation o Use diaphragm of stethoscope for high pitched sounds (bowel sounds) o Use bell of stethoscope to help localize sounds for infant Palpation o Use pads of fingers to determine tenderness and pulsations o Use palmar surface of fingers to determine masses and organ enlargement o Observe reaction to palpation rather than to ask if it hurts (don’t suggest that it does) Percussion o 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 •

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Temperature o Body temperature in infants is less constant than in adults o Use ax/tympanic for children less than 4 years of age o Values are the same as in adults Axillary: hold child’s arm firmly Tympanic: o Less than 3 years of age

Insert gently into ear Pull down on ear o Over 3 years of age  Pull up on ear Rectal temperature o Most hospitals are done only with doctors order, or there is a standing order. o Lubricate tip well o Insert 1 inch Pulse o Apical is best o May use femoral arteries, brachial arteries o Radials only in older children (at least 2 years old) Respirations o The younger the child, the more abdominal breathing o Observe the abdomen instead of the chest in infants and small children o May need to auscultate the chest or put the stethoscope in front of the mouth and nose. Oxygen saturations Blood pressure o Wide enough to over 75% of the upper arm o Narrow cuff elevates reading, wide cuff lowers reading o In infants less than 1 year:  Thigh BP = arm BP o Older than 1 year:  Systolic in thigh is 10-40 mm Hg higher than in arm  Diastolic is same in thigh and arm o 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. o Diastolic BP  Diastolic pressure reaches about 55 mm Hg at one year of age  Gradually increases to 70 throughout childhood o The most common cause of hypertension in children are:  Anxiety (increases BP in children)  Renal disease (78%)  Coarctation of the aorta (2%)  

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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 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: o Anxious parents o Disengaged parents o Stressed families o 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

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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 o 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. o Hold pencil at right angle to the table and mark the head and toes (which are pointed toward the ceiling)

Height •

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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 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 o Around the occipital prominence at the back of the skull o Compare to 36 months

Denver Developmental

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The standard for measuring the attainment of developmental milestones throughout infancy and childhood. Designed for birth to 6 years Includes screening for: o Personal social skills o Fine motor adaptive o Language o Gross motor Denver screening for articulation and eyes

Example of DDST for One year of age: •

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Personal/Social o Drink from a cup, imitate activities, play ball with examiner, indicate wants, play pat-a-cake Fine motor/adaptive o Scribbles, puts block in cup Language o Dada/Mama specific, one word Gross motor o Stands alone

More on DDST: • • • • •

Only a measure of developmental attainment—not a measure of intelligence Not a highly specific test o Most normal children score as normal Not very sensitive o 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 • •

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: o 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 o 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 • •

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

Infant: communication • •

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Forms first social relationships Communicates needs and feelings through nonverbal behaviors and vocalizations o Smile and coo when content o Cry when distressed—hunger, pain, body restraint, loneliness Respond to adults’ nonverbal behaviors o Become quiet when cuddled, patted, or receive other forms of gentle, physical contact. o 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 • •

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Occurs as early as 4 months old; infants face shows disapproval as primary caregiver walks away. Three stages of separation anxiety: o Protest  Cries loudly; rejects attempt to be comforted by anyone but the caregiver o Despair  Crying stops and depression is evident  Much less active  Uninterested in food or play  Withdraws from others o 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: o Encourage parents to room-in with infant o Involve parents in child’s care whenever possible o Keep parent in infant’s line of vision o If parent is unable to be with infant, place familiar object with infant (stuffed toy, etc.) o Support sibling and grandparent visitation

Stranger anxiety • • •

Occurs as early as 5 months Usually peaks at 8 months Behaviors exhibited by infant: o Cries o Screams o Searches for parent with eyes o Clings to parent

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o Avoids and rejects contact with strangers Interventions for stranger anxiety: o 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. o Observe position in which parents hold infant and imitate this. o Hold infants where they can see their parents o 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 o Keep frightful objects out of view o Perform painful procedures in separate room, not in crib, o Use non-intrusive procedures whenever possible.

Immobilization •

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Infants explore life through activity and mobility. If taken away: o Feel helpless o May have difficulty with language skills o May have problems mastering developmental tasks o Problems with motor skills o Immobility impacts development. Immobilization interventions: o Play therapy o Transport infant outside of room by wagon of by carrying o Spend time interacting with infant o Encourage caregivers to do the same

Toddler • •

Ages one to three years old Erikson’s developmental task: Autonomy vs. shame and doubt. o Task: Gaining some basic control over self and environment o 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 o Toddler not interested in the experiences of others o

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Stressors of hospitalization • • • • •

Separation anxiety 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 o 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

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Loss of control occurs due to: o Restriction or limitation of movement o Altered routines and rituals  Eating  Toileting  Sleeping  Bathing  Play o When routines are disrupted, problems can occur in these areas Interventions o 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: o Tell child it’s ok to yell, cry, or do whatever is needed during procedure o Explain procedure in relation to child’s senses o Ignore temper tantrums o Use distraction techniques (Sing song with a child)

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 o Task: Becoming purposeful and directive o Resolution of Crisis: ability to initiate one’s own activities; sense of purpose. o Unsuccessful resolution of crisis: aggression-fear conflict; sense of inadequacy or guilt

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Developing a sense of initiative Preschooler is: o Egocentric o Has increased language skills o Concept of time and frustration tolerance is limited o 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 • •

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Tolerate separation better than toddlers; may develop substitute trust in other significant adults However, they may show other behaviors: o Refuse to eat o Have difficulty sleeping o Cry quietly for parents o Constantly ask when parent will be visiting o May express anger Interventions: o Have parents bring in familiar articles from home—pictures/radio o If child has attachment to special item, have it brought in o Same strategies for toddlers

Loss of control •

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Egocentric and magical thinking; perception of actual events are more frightening o 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

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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).

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

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Erikson’s developmental task: Industry vs. Inferiority o Task: developing social, physical, and school skills o Resolution of Crisis: competence, ability to learn and work o Unsuccessful resolution of crisis: Sense of inferiority; difficulty learning and working Developing a sense of industry and concrete thought Has increased language skill o Interest in acquiring knowledge o Improved concept of time o Increased self-control o 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 o Concrete thinkers; no abstract thought

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Stressors of hospitalization • • • • •

Separation anxiety Loss of control Bodily injury Painful, invasive procedures Fear of death

Separation anxiety • •

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Younger school age children miss their parents more than older children Middle/late react more to separation from peers and usual activities o May feel lonely, bored, isolated, depressed due to separation, not the illness. o May try to be “brave and strong”. o May be irritable with parents Interventions o Make environment as home-like as possible  Continue school lessons  Have friends visit or call on phone  Decorate walls with cards

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

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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 o Need to express their feelings, for some this comes easily, for others it does not. o 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. o o o

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Stressors of hospitalization • • • •

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Loss of control Altered body image, disfigurement o Do not want to look different Separation from peer group Loss of control o Anything that interferes with sense of independence o Patient role fosters dependency o May withdraw, be uncooperative, angry, frustrated. Altered body image o Very relevant at this stage o Any change that makes them different from peers is seen as a major tragedy o Insecure with their bodies due to the many changes o May react with “know it all” attitude (but may be afraid, deep down) o 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

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More than 10 million Americans (1 in 31 or 3.3%) are symptom free carriers of the defective CF gene

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: o Lungs o Pancreas o Sweat glands o Salivary glands o Digestive glands

Pathophysiology •

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CF causes the body to produce an abnormally thick, sticky mucus which: o Clogs the lungs and leads to life-threatening lung infections; o Obstructs the pancreas, preventing enzymes from reaching the intestine to help break down and digest food Primary symptoms: o Thick, sticky mucus o Salty taste on skin Marked electrolyte changes in sweat glands o Chloride in sweat is 2-5X above normal

Lung involvement • •

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Thick tenacious mucus polls in the bronchial tree and obstructs bronchioles Results in: o Bronchiectasis: Chronic dilation of the bronchi. Involves a chronic cough that produces mucopurulent sputum. Over time results in destruction of the bronchial walls. o Pneumonia: Staph Aureus, pseudomona’s, and H. Influenzae Symptoms over time include: o 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 • •

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Thick secretions mix with pancreatic enzymes and block the pancreatic duct. Therefore: o Essential pancreatic enzymes cannot flow into the duodenum to aid with digestion (lipase, trypsin, and amylase) o Fats, proteins, and some sugars cannot be digested—are excreted in stools: o 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

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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 o 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) o Normal concentration of Chloride in sweat is 20 mEq/L o A level of 50-60 mEq/L suggests CF—test is repeated

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o 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 •

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Maintain respiratory function o Keep bronchial secretions as moist as possible to facilitate drainage:  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 o 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 goodtasting mouthwash. Need frequent check-ups and current immunizations/vaccines Adequate rest and comfort o Dyspnea can lead to exhaustion o 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 o 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. o High calorie, high protein, moderate fat diet o 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

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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 Pneumothorax related to rupture of pulmonary blebs

Parental involvement •

Parents assume a great deal of responsibility when taking care of a CF child. o Need to encourage a balance of work, the child, and the rest of the family o Encourage involvement of support group o 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.

Eriksons Stages Infant • •

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

Toddler • •

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

Preschool

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3-6 years of age Erikson’s developmental task: Initiative vs. Guilt o Task: Becoming purposeful and directive o Resolution of Crisis: ability to initiate one’s own activities; sense of purpose. o Unsuccessful resolution of crisis: aggression-fear conflict; sense of inadequacy or guilt o 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

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Erikson’s developmental task: Industry vs. Inferiority o Task: developing social, physical, and school skills o Resolution of Crisis: competence, ability to learn and work o Unsuccessful resolution of crisis: Sense of inferiority; difficulty learning and working o Developmental tasks:  Acquiring game skills  Learning to relate positively with peers  Building a wholesome self-concept  Refining communication skills

Adolescent •

Ages 12-20 years

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Erikson’s developmental task: Identity vs. Role confusion o Task: developing sense of identity o Resolution of Crisis: sense of personal identity o Unsuccessful resolution of crisis: confusion about who one is; identity submerged in relationships or group memberships o 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: o Congenital cardiac defects o 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 o Can occur by self o Can occur with other congenital heart defects o 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 o History of heart disease in the family o Contact with known teratogens, such as rubella during pregnancy o 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. o Recent streptococcal infection (may lead to valve damage) Physical exam o Begins with observation of general appearance, then the specifics o Use general assessment techniques but look specifically for the following: o 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) o Palpation  Quality and symmetry of pulses o Auscultation  Heart rate and rhythm  Presence of murmurs o Height and weight o Position of comfort  Remember, squatting/fetal positions are often comfortable for a child with a CHD. Tests of cardiac function o Electrocardiography  Records electricity generated by the beating heart  Painless but scary, child must be still o Exercise stress test  Monitoring of heart rate, BP, ECG, and oxygen consumption at rest and during exercise on a tread mill or bicycle o Chest x-ray  Shows accurate picture of heart size and contour; size of the heart chambers  Used more as a screening tool o Echocardiography  Ultrasound (high frequency sound waves produce an image of heart structures)  The primary diagnostic test for heart disease. o o o o o

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Cardiac catherization •

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Radiopaque catheter is inserted through peripheral blood vessel into heart o Contrast material is injected and films taken (called angiography) Reasons for performing: o Diagnose specific heart disease o Measure pressures and O2 sats o Visualize heart structures o Determine blood flow patterns

Preparation for Cardiac cath •

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Although done frequently, there are some risks. Typical reactions include: o Acute hemorrhage from entry site (usually femoral artery) o Low grade fever (reaction to contrast media) o Nausea o Vomiting o Loss of pulse in the catheterized extremity o 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: o 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:

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Temperature and color of the affected extremity. Coolness or blanching may indicate arterial obstruction. Vital signs are taken every 15 minutes. Special emphasis on heart rate. Must take for one full minute. Assess blood pressure, especially for hypotension. o Hypotension could indicate:  Hemorrhage  Too much medication  Dehydration Dressing, for evidence of bleeding. Fluid intake, both IV and oral, to ensure adequate hydration. o 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 o Other than prematurity/low birth weight More than 35 well recognized defects Statistics improving due to more surgeries/treatments that help prevent death.

Etiology • •

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Not known in 90% of cases Factors associated: o Maternal rubella during pregnancy o Maternal alcoholism o Maternal age over 40 o 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 •

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Ductus venosus (DV): opening between umbilical vein and inferior vena cava. o 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. o 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 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. o 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.

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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 • • • • •

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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 o Clots to form ligamentus teres Foramen ovale o Closes to form interatrial septum Ductus arteriosis o 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

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

Defects with increased pulmonary blood flow • • •

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Ventricular septal defect o Hole between the ventricles Atrial septal defect o Hole between the atria’s Patent Ductus Arteriosis (PDA) o Allows blood to flow from higher pressure aorta to the lower pressure pulmonary artery, causing a left to right shunt. Atrialventricular septal defect o 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. o Pulmonary hypertension and cor pulmonae are frequent disorders associated with this.

Defects causing Decreased pulmonary blood flow •

Tetralogy of the Fallot o Pulmonic Stenosis: Narrowing of the pulmonary artery or valve o Ventricular septal defect

Overriding of the aorta: position of the aorta is not correct. Blood may be shunted from both ventricles. o 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 o Extremely serious. o Tricupsid valve is completely closed. o No blood flow from the right atrium to the right ventricle o 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. o

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Defects causing obstruction to blood flow from ventricles •

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Coarctation of the aorta o A segment of the aorta is too narrow, near the insertion of the ductus arterious. o High blood pressure develops o Left ventricle is enlarged o Oxygenated blood to the body is reduced. Pulmonary stenosis o Narrowing of the pulmonary artery or pulmonary valve just distal (under/below) to the valve. o Eventually causes right ventricular enlargement (hypertrophy) Aortic stenosis o Narrowing of the aortic valve o Prevents blood from passing freely from left ventricle unto aorta. o Causes left ventricular hypertrophy from increased pressure in the left ventricle.

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: •

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Transpositions of the Great Arteries o Aorta arises from the right ventricle instead of the left o Pulmonary artery arises from the left ventricle o Blood enters the heart from the vena cava. o Goes to the right atrium  to the right ventricle  then goes out the aorta to the body completely deoxygenated. o Very incompatible with life o Surgery indicated Total Anomalous Pulmonary Venous Return o 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) o Blood must be shunted across a patent foramen ovale or ductus arteriosus in order to reach the systemic circulation. Truncus Arteriosus o One major artery or “trunk” arises from the left and right ventricles in place of a separate aorta and pulmonary artery. o Usually accompanied by a VSD. o Restructure common trunk to create two separate vessels (2 separate trunks) Hypoplastic left heart syndrome o Left ventricle of the heart is non-functional. o Unable to effectively pump blood into the systemic circulation. o Right ventricle enlarges as it tries to do all of the work. o Transplant or the 3 Staged Norwood procedures performed.

Two principle clinical consequences of defects • •

Heart failure Hypoxemia

Heart Failure • • •

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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: o Muscle fibers lengthen, causing ventricles to increase and handle more blood with each stroke (ventricular hypertrophy). o 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 •

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Reduce workload of the heart: o Decrease extra fluid (diuretics) o Strengthen cardiac function (digoxin) o Decrease afterload with vasodilators  Afterload is the amount of force needed for left ventricle to push blood through the body. Decrease cardiac demands o Allow for uninterrupted sleep periods o Small frequent feedings or gavage  The more food in the stomach, the more cardiac work it is to digest. Reduce respiratory distress o Count respirations carefully o Humidified O2 o 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

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Mortality rates vary from 2% to 25% Surgery should be done in major centers

Alteration in Nutrition and Elimination

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 •

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Infants o First 6 months can live off of breast milk or commercially prepared formula with iron added o 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 o High protein o High calories A little about obesity o 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

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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 o Fluid is a greater fraction of their total body weight as compared to adults  Infants: 75-80% TBW  2 years: 60% TBW  Amounts stay approximately the same through later childhood and adult life o Body water is also distributed differently in infants than older children.  Infants have more interstitial fluids

Extracellular fluid compartment in infants includes 3545% body water Insensible water loss o Loss of fluid through lungs and skin o 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 o All of these factors contribute to greater fluid loss through evaporation. o In addition, treatments or other conditions may increase fluid output  Activity, fever, diarrhea, vomiting Kidney function o 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 o Nurses want to make sure that kidneys are working before adding potassium to I.V. fluids. Other imbalances o Children are also more readily susceptible to imbalances in:  Serum glucose  Calcium  Potassium 

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Glucose •

Infants and children have o Higher glucose needs due to high metabolic rate o Low glycogen stores o Hypoglycemia a threat under periods of stress

Calcium •

Infants and children have: o Regulation of calcium less exact in infant than in older child or adult o When stressed, more growth hormone (GH) is secreted  GH increases result in increased calcium deposits in bone

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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 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 o o o

Color indicates the state of perfusion As extracellular fluid volume decreases (as with dehydration), peripheral circulation decreases. Note the following:

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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 2cm/month in the first 3 months o >1cm/month in the 2nd three months o >0.5cm/month for the ext six months Fontanel changes o 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 o May be projectile Eye changes o Diploplia (double vision) from pressure on abducens nerves o White of sclera evident over top of cornea (“sunset eyes”) o Limited visual fields o Papilledema (retinal edema—cannot see on physical assessment; need special equipment) Visual sign changes o Elevated temperature and blood pressure (body regulators are compressed) o Increase in pulse pressure (the gap between the systolic and diastolic) o Decreased pulse and respirations due to the growing pressure on brain stem which controls:  Cardiac rate and respirations Pain o Headache on awakening and standing. Increases with valsalva maneuver) or holding breath  Each increased intrathoracic pressure Mentation o Irritability, decreased level of consciousness, altered LOC Later signs: o Personality changes o Seizures o Head tilt (because of heavy tumor) o Decreased LOC o Decreased motor response to stimuli o Decreased sensory response to painful stimuli o Alterations in pupil size and reactivity o Cheyne-stokes respirations o Decerebrate or decorticate posturing (pg. 1674)  This is very bad  o o

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Glasgow coma scale • • • •

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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: o eye opening o verbal response o motor response highest score is 15

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lowest score is 3 Medical management is based on these scores

Brain Tumors

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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: o skull films o bone scan o MRI o Cerebral angiography (difficulty getting dye through due to abnormal vascularization) o CT scan

Therapeutic management of brain tumors • •

Includes a combination of surgery, radiotherapy, and chemotherapy Preoperative care o Will usually receive a stool softener—do not want child to strain after their brain is operated on o A portion of the head is shaved; very traumatic for adolescent o If going to the ICU after surgery, have child meet the ICU staff

Postoperative care •

Positioning o Depends on location of tumor; usually on the opposite side of incision o 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 o We don’t want the child to be dehydrated, but we do want them on the “drier” side Prevent nausea and vomiting o o

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Therapeutic management of ICP • • • • •

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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: o Corticosteriods (Decadron)—reduces inflammation o Osmotic diuretic (mannitol)—pulls fluid out of tissues Will usually insert foley catheter prior to administration for accurate I & O’s

Down syndrome

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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

Clinical manifestations of Down syndrome

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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 o 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

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Potential for injury due to hypotonia/cognitive impairments high risk for falls

Fetal Alcohol syndrome

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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

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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 • • •

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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 Incoherence

Diagnosis • • •

Head circumferences Associated Neuro signs CT, MRI, skull x-ray

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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

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May need to keep flat to avoid too rapid reduction of intracranial fluid Observe for signs of increased ICP o 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 Altered family processes related to having a child with a chronic illness

Neural Tube Defects

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Spina Bifida: defect in closure of the vertebral column with varying degrees of tissue protusion Spina Bifida Occulta: o Posterior vertebral arches fail to close in the lumbrosacral area o Spinal cord remains intact and usually is not visible. o Meninges are not exposed on the skin surface o Neurological deficits are not usually present Spina Bifida cystica: o Protrusion of the spinal cord and/or it’s meninges occurs o 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 o Spinal cord is not involved o Neurological deficits are usually not present. Myelomeningocele: o Protrusion of meninges, CSF, nerve roots, and a portion of the spinal cord occurs. o The sac (defect) is covered by a thin membrane that is prone to leakage and rupture o Neurological deficits are present. o

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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. 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