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64 Cards in this Set

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Diaper Dermatitis - Overview
Common in infants

One of several acute inflammatory skin disorders

Caused directly or indirectly by wearing diapers

Peak age of occurrence: 9 - 12 months

Greater in bottle-fed infants
Diaper Dermatitis - Pathophysiology
Caused by prolonged & repetitive contact with an irritant
- Urine, feces, soaps, detergents, ointments, friction

Combination of factors contribute to irritation
- Majority: urine and feces

Prolonged contact of skin with diaper wetness produces:
- higher friction
- greater abrasion damage
- inc. transepidermal permeability
- inc. microbial counts

* healthy skin: LESS resistant to potential irritants

*Ammonia: once thought to cause diaper rash
- associated with strong odor on diapers and dermatitis
- not sufficient alone

Irritant quality of urine r/t inc. pH from urea breakdown in the presence of fecal urease
- inc. pH promotes activity of fecal enzymes (principally proteases and lipases), which act as irrtants
-- fecal enzymes also inc. skin permeability to bile salts (another potential irritant)
Diaper Dermatitis - Clinical Manifestations
Eruption primarily manifested on convex surfaces or in folds

Lesions represent a variety of types/configurations

Eruptions involving skin in most intimate contact with the diaper (convex surface of butt, inner thighs, mons pubis, scrotum), but sparing the folds are likely to be caused by chemical irritants (especially from urine and feces)

Other causes: detergents, soaps from inadequately rinsed cloth diapers or disposable wipe chemicals

Perianal involvement: usually result of chemical irritation from feces (especially diarrheal stools)

*Candida albicans: perianal inflammation and maculopapular (beefy red central erythema) rash with satellite lesions that may cross the inguinal fold
- seen in up to 90% of infants with chronic diaper dermatits
- should be considered in diaper rashes that are recalcitrant to treatment
Diaper Dermatitis - Nursing Care
Interventions aimed at altering 3 factors that produce dermatitis
- wetness, pH, fecal irritants

Most significant factor amenable to intervention: moist environment created in diaper area

Change diaper as soon as it becomes wet
- Eliminates a large part of the problem
- Removing the diaper to expose healthy skin to air facilitates drying

* Hair dryer or heat lamp NOT recommended because they can cause burns

Diaper construction has significant impact on incidence and severity
- Superabsorbent disposable diapers reduce diaper dermatitits
-- contains absorbent gelling material that binds water tightly to decrease skin wetness
-- maintains pH control by providing a buffering capacity
-- decreases skin irritation by preventing urine and feces from mixing in the diaper

Another advancement: addition of inner layer or top sheet which is impregnated with petrolatum
- ex: Pampers Swaddler with Absorb Away

Common misconception: corn starch promotes growth of C. albicans.
- Neither cornstarch nor talc promotes fungi growth under normal conditions
- Cornstarch is more effective in reducing friction; tends to cake less than talc when skin is wet; also safer in terms of inhalation injury.
- Cornstarch is preferred than talc.
Family-Centered Care: Controlling Diaper Rash
Keep skin dry
- Use superabsorbent disposable diapers
- If using cloth diapers: use only overwraps that allow air to circulate
- AVOID RUBBER PANTS
- Change diaper as soon as it is soiled -- especially with stool -- whenever possible (preferably once during the night)
- Expose health or slightly irritated skin to air, NOT heat, to dry completely

Apply ointment, such as zinc oxide or petrolatum, to protect skin, especially if skin is very red or has moist, open areas
- Avoid removing skin barrier cream with each diaper change; remove waste material and reapply skin barrier cream.
-To remove ointment completely (especially zinc oxide), use mineral oil; do not wash vigorously

Avoid overwashing the skin, especially with perfumed soaps or commercial wipes (may be irritating)
- May use moisturizer or nonsoap cleanser (cold cream or Cetaphil), to wipe urine from skin
- Gently wipe stool from skin using water and mild soap (such as Dove)
- When traveling, fill an old baby wipe container with soft paper towels and warm water.
Atopic Dermatitis (Eczema) - Overview
AKA eczematous inflammation of the skin

Refers to a descriptive category of dermatologic diseases and not a specific etiology

A type of pruritic eczema that usually begins during infancy
- Associated with allergy with hereditary tendency (atopy)

3 forms based on age and lesion distribution
1) Infantile (infantile eczema)
- Usually begins @ 2 - 6 months of age; generally undergoes spontaneous remission by 3 years of age
2) Childhood
- May follow infantile form; occurs @ 2 - 3 years of age; 90% of children have manifestations by 5 years
3) Preadolescent & adolescent: Begins @ about 12 years of age; may continue into early adult years or indefinitely

Diagnosed based on history and morphologic findings

Children with AD have lower threshold for cutaneous itching than other children; many authorities believe dermatologic manifestations appear subsequent to scratching from the intense pruiritis
- Infants rub their faces against bed linen; crawling (a form of scratching) results in irritation of knees and elbows
- Lesions disappear if scratching is stopped

Majority of children with infantile AD have:
- Family hx of eczema
- Asthma
- Food Allergies
- Allergic rhinitis
* Strongly supports genetic predisposition

Cause: unknown; appears to be related to abnormal function of the skin
- including alterations in perspiration, peripheral vascular function, heat tolerance

Manifestations of chronic disease improve in humid climates; get worse in the fall & winter (homes are heated and environmental humidity is lower

Disorder can be controlled, not cured
Atopic Dermatitis (Eczema) - Clinical Manifestations
Infantile
- Distribution: Generalized, especially cheeks, scalp, trunk and extensor surfaces of extremities
- Appearance: Erythema, vesicles, papules, weeping, oozing, crusting, scaling, often symmetric

Childhood
- Distribution: Flexural areas (antecubital & popliteal fossae, neck), wrists, ankles, feet
- Appearance: Symmetric involvement, clusters of small eyrthematous or flesh-colored papules or minimally scaling patches, dry and possibly hyperpigmented, lichenification (thickened skin with crease accentuation), keratosis pilaris (follicular hyperkeratosis) common

Preadolescent & Adolescent
- Distribution: Face, sides of neck, hands, feet, face, antecubital & popliteal fossae (lesser extent)
- Appearance: Same as childhood manifestation; dry, thick lesions (lichenifed plaques) common, confluent papules

Other physical manifestations
- Intense itching
- Unaffected skin dry & rough
- African-American children likely to exhibit more papular or follicular lesions than Caucasion children
- May exhibit one or more of the following
-- Lymphadenopathy (especially near affected sites)
-- Increase palmar creases (many cases)
-- Atopc pleats (extra line or groove of lower eyelid)
-- Prone to cold hands
-- Pityriasis alba (small, poorly defined areas of hypopigmentation)
-- Facial pallor (especially around nose, mouth and ears
-- Bluish discoloration beneath eyes ("allergic shiners")
-- Increased susceptibility to unusual cutaneous infections (especially viral)
Atopic Dermatitis (Eczema) - Therapeutic Management
Major goals:
1) Hydrate the skin
2) Relieve pruritis
3) Reduce flare-ups or inflammation
4) Prevent & control secondary infection

General measures focused on reducing pruritis and other aspects of the disease
- Avoid exposure to skin irritants or allergens
- Avoiding overheating
- Administering medications (antihistamines, topical immunomodulators, topical steroids, mild sedatives [sometimes])

Enhance skin hydration and prevent dry, flaky skin: done in a number of ways (depends on skin characteristics and individual needs)
- Tepid bath with mild soap (Dove), no soap or emulsifying oil, followed immediately by applying an emollient (within 3 mins) assists in trapping moisture and preventing its loss
* Avoid bubble baths and harsh soaps
- Bath may need to be repeated 1x or 2x daily, depending on child's status; excessive bathing without emollient only dries out the skin
- Some lotions are not effective; emollients should be chosen carefully to prevent excessive skin drying.

Acceptable lotions: Aquaphor, Cetaphil, Eucerin

Nighttime bath followed by emollient and dressing in soft, cotton pajamas may help alleviate most nighttime pruritis

Sometimes: colloid baths (adding 2 cups of cornstarch to warm water tub) can provide temporary relief of itching and may help the child sleep if given before bedtime

Cool wet compresses are soothing to the skin and provide antiseptic protection

Oral antihistamines (hydroxyzine or diphenhydramen) usually relieve moderate or severe pruritis
- causes sedation/drowsiness

Non-sedating antihistamines, like loratadine (Claritin) or fexofenadine (Allegra) may be preferred for daytime pruritis relief

Mildly sedating antihistamine at night (pruritis increases at night)

Topical steroids: used to diminish inflammation due to occasional flare-ups
- Low-, moderate-, or high-potency topical corticosteroids are prescribed based on degree of involvement, area of the body to be treated, child's age, potential for local side effects (striae, skin atrophy, pigment changes), and the type of vehicle to be used (cream, lotion, ointment)
- Patients receiving topical corticosteroid therapy for chronic conditions should be evaluated for risk factors (suboptimal linear growth and reduced bone density)

Topical immunomodulators, a new nonsteroidal treatment for AD
- best used at the beginning of a flare-up, just as skin becomes red and itches
- 2 new immunomodulator medications: tacrolimus and pimecrolimus

* Tacrolimus: 2 ointment strengths - 0.03% and 0.1%
-- 0.03%: approved for children 2 years +
-- Recommended for intermittent therapy in patients not adequately responsive to, or are intolerant of, conventional therapy

*Pimecrolimus: available in a 1% cream and has no systemic accumulation or effects
- approved for use in children with mild to moderate AD

** Both drugs can be used freely on the face without worry about steroid side effects

** If secondary skin infections occur in children with AD: use appropriate systemic antibiotics
Atopic Dermatitis (Eczema) - Nursing Care
Assessment:
- Family hx for evidence of atopy, hx of previous involvement, and any environmental or dietary factors associated with the present and previous exacerbations
- Skin lesions are examined for type, distribution, and evidence of secondary infection
- Parents interviewed re: child's behavior, especially scratching, irritability, and sleeping patterns
- Explore family's feelings and coping methods

Care is challenging

Controlling intense pruritis is imperative if the disorder is to be successfully managed
- Scratching leads to new lesions and may cause secondary infection.

In addition to medication regimen, other measures can be taken to prevent or minimize scratching
- Fingernails and toenails are cut short, kept clean, and filed frequently to prevent sharp edges
- Gloves or cotton stockings can be placed over the hands and pinned to sleeves
- One-piece outfits with long sleeves and long pants also decrease direct contact with the skin
- If gloves or socks are used, the child needs time to be free from such restrictions
-- Remove gloves, socks or other protective devices during bath or after receiving sedative or antipruritic meds

Eliminate conditions that increase itching when possible
- Woolen clothes or blankets, rough fabrics, and furry stuffed animals are removed from the child's environment
- Proper dress for climatic conditions is essential (Heat & humidity cause perspiration, which intensifies itching)

Pruritis is often precipitated by exposure to the irrtant efects of certain components of common products (soaps, detergents, fabric softeners, perfumes and powders)
- Children experience less itching when soft cotton fabrics are worn next to the skin
- During cold months, synthetic fabrics (NOT wool) should be used for overcoats, hats, gloves and snowsuits)

Avoid exposure to latex products (balloons, gloves)

Clothes & sheets are laundered in a mild detergent and rinsed thoroughly in clear water (w/o fabric softeners or antistatic chemicals)
- Put clothes through a 2nd complete wash cycle w/o detergent (reduces amount of remaining residue)

Preventing infection is accomplished by preventing scratching
- Baths as prescribed with tepid water
-- soaps (unless indicated) and buble baths are avoided, as well as oils or powders

- Skinfolds and diaper areas need frequent cleansing with plain water.

- Room humidifier or vaporizer may benefit children with extremely dry skin.

- Examine skin lesions for signs of infection; report any to HCP
-- honey-colored crusts or pustules with surrounding erythema

** If child is being tx'ed with baths for hydration, apply emollient immediately after bathing (while skin is slightly moist) to prevent drying

Wet soaks and compresses are applied and pruritis or infection meds are administered as directed.
- Give family explicit instructions on the preparation and use of soaks, special baths and topical medications, including the order of application if more than one is prescribed
* One thick application of topical medication is not equal to several thin applications; excessive use of an agent (particularly steroids) can be hazardous

If children have difficulty remaining still for a 10- or 15-minute soak, bath or dressing application, they can be carried out at naptime or when the child is engrossed in watching TV, listening to a story or playing with tub toys

Diet modification: another source of frustration
- Hypoallergenic diet: parents need help to understand the reason for the diet and guidelines for avoiding hyperallergenic foods
- Parents need reassurance that results may not be seen immediately (hypoallergenic diets take time before visible effects are apparent)

If airborne allergens make eczema worse: counsel family about allergy-proofing
Atopic Dermatitis (Eczema) - Nursing Care: Family Support
Assure parents that lesions will not produce scarring (unless secondarily infected from scratching) and that the disease is not contagious.

Child may have repeated exacerbations and remissions
- Spontaneous and permanent remission takes place at approx. 2 - 3 years of age in most children with infantile AD

During acute phases, emotional stress can become intense for the family.
- Needs time to discuss negative feelings and be reassured that these feelings are normal
- Efforts to relieve as much anxiety as possible in both the parents and the child have a beneficial emotional and physical effect
Atopic Dermatitis (Eczema) - Nursing Care Guidelines: Preventing Atopy in Children
Identify children at risk
- Family hx of allergy
- Inc. IgE in cord blood and post natal serum
- Dry, flaky skin

Prenatal Precautions (Last Trimester)
- Avoid any known food allergens
- Avoid milk and other dairy products, peanuts and eggs
- Minimize ingestion of other hyperallergenic foods

Postnatal Precautions
- Breast milk or casein-whey hydrolysate formula (Nutramigen, Pregestimil, Alimentum) exclusively for at least 6 months
- No solid food for first 6 months
- No cow's milk or soy formula for 12 months
- No eggs, fish, corn, citrus, peanuts, nuts or chocolate for 12 - 18 months
- One new food added at 5- to 7-day intervals to identify possible reaction

Environmental Control
- Limited exposure to dust, molds, furry animals, and cigarette smoke
Acne - Overview
Acne Vulgaris: most common skin problem treated by physicians during patients' adolescence

Not caused by dirt but by testosterone
- present in both males and females; increases during puberty
- stimulates sebaceous glands of the skin to enlarge, or produce oil, and plug the pores
- Whiteheads, blackheads and pimples are present in teenage acne

One half of the adolescent population experiences acne by the end of the teenage years

Peak incidence occurs in middle to late adolescence
- age 16-17 years in girls; 17-18 in boys
- disorder can appear before 10 years of age

More common in boys than girls

Degree to which an individual is affected may range
- from a few isolated comedones to a severe inflammatory reaction.

Disease is self-limiting and not life threatening; has great significance to adolescent
- HCP shouldn't underestimate the impact it has on teens

Numerous factors affect the development and course of acne.
- Distribution in families and high degree of concordance in identical twins suggest hereditary factors
- Premenstrual flares of acne occur in nearly 70% of adolescent girls, suggesting a hormonal cause

Studies do not indicate a clear association between stress and acne
- Adolescents commonly cite stress as a cause for acne outbreaks.

Cosmetics containing lanolin, petrolatum, vegetable oils, lauryl alcohol, butyl stearate, and oleic acid can increase comedone production
- Exposure to oils in cooking grease can be a precursor to adolescents who work over fast-food restaurant hot oils

No known link between dietary intake and development or worsening of acne
Acne - Pathophysiology
A disease that involves the pilosebaceous follicles (hair follicle and sebaceous gland complex) of the face, neck, chest and upper back

3 pathophysiologic factors involved
- Excessive sebum production
- Comedogenesis
- Overgrowth of Propionibacterium acnes

Comedogenesis: formation of comedones
- results in noninflammatory lesions that may be open (blackhead) or closed (whitehead)
- Inflammation occurs with the proliferation of P. acnes, which draws in neutrophils, causing inflammatory papules, pustules, nodules and cysts
Acne - Therapeutic Management: General
Depends on cooperative effort between HCP, adolescent and parents

Acne lesions resolve slowly, improvement may not be apparent for at least 6 weeks.
- Individual comedones can take several weeks to months to resolve, and papules and pustules usually resolve in about 1 week

Multifactorial causes of acne necessitate a combined approach for successful tx.

Tx: general measures of care and specific txs (based on lesions involved)

General measures: adequate rest, moderate exercise, well-balanced diet, reduction of emotional stress, eliminating foci of infection

Cleansing: Dirt or oil on the surface of the skin DOES NOT cause acne
- Gentle cleansing with a mild cleanser 1x or 2x daily is usually sufficient
- Antibacterial soaps are ineffective; may be too drying when used in combination with topical acne meds
* For some adolescents: hair and scalp hygiene appears to be r/t to the clinical activity of the acne
-- Acne on the forehead may improve with brushing the hair away from the forehead and more frequent shampooing
Acne - Therapeutic Management: Medications
Success depends on commitment from the adolescent
- HCP should determine adolescent's level of comfort and readiness to begin tx

Tretinoin (Retin-A): the only drug that effectively interrupts the abnormal follicular keratinization that produces microcomedones, the invisible precursors of the visible comedones
- Alone is usually sufficient for management of comedonal acne
- Available as cream, gel or liquid
- Can be extremely irritating to the skin; requires careful pt. education for optimal usage
-- Patient should be instructed to begin with a pea-sized dot of medication, which is divided into the 3 main areas of the face, and then gently rubbed into each area
-- Should not be applied for at least 20-30 mins after washing to dec. burning sensation
-- Emphasize avoiding sun and daily use of sunscreen (sun exposure can result in severe sunburn)
--- Adolescents should be advised to apply the medication at night and use a sun protection factor SPF of at least 15 in the daytime

Benzoyl peroxide (topical): an antibacterial agent that inhibits growth of P.acnes
- Effective against both inflammatory and noninflammatory acne; an effective first-line agent
- Available in cream, lotion, gel or wash
- Teach patient that it may have a bleaching effect on sheets, bedclothes, towels, and eyebrows. Skin bleaching will NOT occur
- Accommodation can be gained with a gradual inc. in strength and frequency of application

Topical antibacterial agent: prescribed for inflammatory lesions that accompany comedones
- Used to prevent new lesions and treat preexisting acne
- Includes Clindamycin, erythromycin, metronidazole, azelaic acid, and the combination of either benzoyl peroxide and erythromycin (Benzamycin) or benzoyl peroxide and glycolic acid
* 5% benzoyl peroxide and 3% erythromycin is especially beneficial (exact mechanism unknown)
* Tretinoin improves the penetration of other topical agents
- combination therapy with Tretinoin and an antibacterial treatment is the only way to address 3 of the pathogenic causes of acne (keratinization, P. acnes, and inflammation)

Systemic antibiotic therapy: used when moderate to severe acne doesn't respond to topical treatments
- Tetracycline, erythromycin, minocycline and doxycycline are safe to use

* Females with mild to moderate acne may respond well to topic treatments and the addition of an oral contraceptive pill (OCP)
- Reduces the endogenous androgen production and decreases the bioavailability of the woman's circulating androgens, both resulting in dec. acne

Isotretinoin, 13-cis-retinoic acid (Accutane): a potent and effective oral agent that is reserved for severe cystic acne that hasn't responded to other treatments.
- Only agent available that affects factors involved in acne development
-- should only be managed by a dermatologist
- Adolescents with multiple, active, deep dermal or subcutaneous cystic & nodular acne lesions are tx'ed for 20 weeks
- Multiple side effects: dry skin and mucous membranes, nasal irritation, dry eyes, dec. night vision, photosensitivity, arthralgia, headaches, mood changes, aggressive or violent behaviors, depression, and suicidal ideation
* Adolescents on this drug should be monitored for depression, depressive symptoms, and suicidal ideation
- Drug should only be given at recommended doses for no longer than the recommended duration

** Most significant side effect: teratogenic effects
~ contraindicated in pregnant women
~ Sexually active young women must use an effective contraceptive method during tx and for 1 month after tx

* Patients receiving isotretinoin should also be monitored for elevated cholesterol and triglyceride levels
~ Significant elevation may require discontinuation
Acne - Nursing Care
Acne is so common and its appearance may seem mild
- Importance of the disease to the adolescent may be underestimated

Assess adolescent's individual level of distress, current management, and perceived success of any regimen before initiating a referral.
- If adolescents do not perceive the acne to be a problem, they may lack motivation to follow the tx. plan

Provide ongoing support for the adolescent when tx. plan is initiated
- Encourage family to support efforts of adolescent
- Discuss use of meds and basic skin care in detail
-- Accompany with written instruction
- Information to dispel myths re: use of abrasive cleansing products can prevent unnecessary costs and skin trauma

Educate teenagers about aggravating and damaging factors (vigorous scrubbing)
- Picking, squeezing, and manual expression with fingernails can break down lesion ductal walls and worsen acne
- Mechanical irritation (vinyl helmet straps that rub predisposed acne areas) can cause lesion development
Cerebral Palsy - Overview
2006: defined as, "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain"
- Involves motor disorders as well as disturbances of sensation, perception, communication, cognition and behavior; secondary musculoskeletal problems and epilepsy

Etiology, clinical features and course are variable; characterized by abnormal muscle tone and coordingation as primary disturbances

Most common permanent physical disability of childhood
- Incidence: 1.5 - 3 in every 1000 live births
- Prevalence has risen 20% since the 1960's, most likely reflecting improved survival of extremely LBW and very LBW infants

Though traditional hypothesis has been that CP results from perinatal problems (especially birth asphyxia), it is believed it results more often from existing prenatal brain abnormalities (exact cause elusive)
* Estimated 80% of CP cases are unknown prenatal factors
- Intrauterine exposure to maternal chorioamnionitis is associated with inc. risk of CP in infants with normal birth weight and preterm
-- Not all term infants develop CP

Prevalence of CP in infants born before 36 weeks of gestation & weighing less than 2000g (4.4 lbs): 12%

Strongest independent risk factor for CP development: periventricular leukomalacia

Survivors of shaken baby syndrome may also develop CP from the damage

Additional factors that may contribute to CP development postnatally include: bacterial meningitis, viral encephalitis, MVAs, child abuse

* Significant percentage (15 - 60%) of children with CP will also have epilepsy, compromising self-care issues and normalization
Cerebral Palsy - Pathophysiology
Difficult to establish a precise location of neurologic lesions based on etiology or clinical signs
- No characteristic pathologic pattern exists

Some patients have gross brain malformations; others may have evidence of vascular occlusion, atrophy, neuron loss and degeneration.
- Few exceptions occur and are related to anatomic areas such as spastic diplegia (associated with preterm births), caused by hypoxic infarction or hemorrhage in the area adjacent to the lateral ventricles
- Ataxic CP may occur in relation to cerebral hypoplasia and, in some cases, severe hypoglycemia

American College of Obstetricians & Gynecologists in conjunction with the American Academy of Pediatrics published report defining neonatal encephalopathy
- affirms that approx. 70% of cases of neonatal encephalopathy occur as a result of events before onset of labor
- establishes criteria to define events sufficiently capable of causing intrapartum asphyxia and CP
* Evidence indicates that events that cause the majority of CP occur not as a result of intrapartum asphxia, but for previously mentioned reasons
Cerebral Palsy - Diagnostic Evaluation
Infants at risk according to known etiologic factors associated with CP warrant careful assessment during early infancy to identify signs of muscular dysfunction as early as possible

Neurologic exam and hx are primary modalities for dx
- Neuroimaging of the child with suspected brain abnormality & CP are now recommended for dx assessment
* MRI preferred to CT

- Metabolic & genetic testing is recommended if no structural abnormality is identified by neuroimaging lab tests are no longer recommended in the dx process for CP

Early recognition is made more difficult by lack of reliable neonatal neurologic signs
- Infants with known etiologic risk factors should be monitored and evaluated closely in the first 2 years of life.
- Alert observer may be suspicious of early signs
- Because cortical control of movement doesn't occur until later in infancy, motor impairment associated with voluntary control is usually not apparent until after 2-4 months of age at the earliest
- More often the dx cannot be confirmed until age 2 years because motor tone abnormalities may be indicative of another illness

Persistence of primitive reflexes may be of value in establishing the dx.
- either the asymmetric tonic neck reflex or persistent Moro reflex (beyond 4 months) and the crossed extensor reflex
-- Tonic neck reflex normally disappears between 4-6 months of age; "obligatory" response is abnormal
-- Crossed extensor reflex, which normally disappears by 4 months, is elicited by applying a noxious stimulus to the sole of 1 foot with the knee extended; normally the contralateral foot responds with extensor, abduction, and then adduction movements
--- Possibility of CP is suggested if the reflexes are founda fter the age at which they should've disappeared
Cerebral Palsy - Early Signs
- Failure to meet any developmental milestones (rolling over, raising head, sitting up, crawling)

- Persistent primitive reflex (Moro, atonic neck)

- Poor head control (head lag) and clenched fists after 3 months of age

- Stiff or rigid arms or legs; scissoring legs

- Pushing away or arching back; stiff posture

- Floppy or limp body posture, especially when asleep

- Inability to sit up without support by 8 months

- Using only 1 side of the body or only the arms to crawl

- Feeding difficulties

- Persistent gagging or choking when fed

- After 6 months of age, tongue pushing soft food out of the mouth

- Extreme irritability or crying

- Failure to smile by 3 months

- Lack of interest in surroundings
Cerebral Palsy - Clinical Classification
Spastic (Pyramidal)
- Characterized by persistent primitive reflexes, positive Babinski, ankle clonus, exaggerated stretch reflexes, eventual contracture development
~ 70-80% of all cerebral palsy cases
~ Diplegia: all extremities affected; lower more than upper (30-40% of spastic CP)
~ Quadriplegia: All 4 extremities involved: legs & trunk, mouth, pharynx and tongue (10-15% of spastic CP)
~ Triplegia: 3 limbs involved
~ Monoplegia: 1 limb only
~ Hemiplegia: motor dysfunction on 1 side of the body; upper extremit more than lower (20-30% of spastic CP)
- Other features:
~ Hypertonicity with poor control of posture, balance, and coordinated motion
~ Impairment of fine & gross motor skills

Dyskinetic (Nonspastic, Extrapyramidal)
- Athetoid: Chorea (involuntary, irregular, jerking movements); characterized by slow, wormlike, writhing movements that usually involve the extremities, trunk, neck, facial muscles, and tongue
- Dystonic: Slow, twisting movements of the trunk or extremities; abnormal posture
- Involvement of the pharyngeal, laryngeal, and oral muscles causing drooling and dysarthria (imperfect speech articulation)

Ataxic (Nonspastic, extrapyramidal)
- Wide-based gait
- Rapid, repetitive movements poorly performed
- Disintegration of movements of the upper extremities when the child reaches for objects

Mixed
- Combination of spastic & dyskinetic CP
- May be labled mixed when no specific motor pattern is dominant; however, this term is losing favor to more precise descriptions of motor function & affected area of brain involved
Cerebral Palsy - Clinical Signs & Symptoms
Spastic
- Inc. muscle tone (hypertonicity)
- Inc. deep tendon reflexes & clonus (sudden dorsiflexion of the ankle or rapid distal movement of the patella resulting in alternating spasm & relaxation of the muscles being stretched)
- Flexor, adductor, and internal rotator muscles more involved than extensor abductor & external rotator muscles
- Difficulty with fine and gross motor skills
- Most common contracture: heelcord
- Hip adductor contractures leading to progressive sublaxation & dislocation
- Knee contracures
- Scoliosis common
- Typical gait crouched, intoeing, scissoring
- Elbow, wrist, and fingers in flexed position with thumb adducted
- Motor weakness of antagonist muscle groups

Dyskinetic
- Purposeless, involuntary, uncontrollable movements of face & extremities
- Inc. movements with stress and voluntary movements, absent during sleep
- Contractures rare
- Normal deep tendon reflexes

Ataxic:
- Disturbed coordination
- Lack of equilibrium
- Unsteady gait
- Few orthopedic problems
- Hyporeflexia
- Loss of ability to gauge distance, speed, power of movement
- Muscles hypotonic
- Speech slurred, jerky, explosive
- Nystagmus common

Other
- Visual deficits (most common in spastic)
- Hearing impairment (most common in dyskinetic)
- Oral motor involvement resulting in drooling and feeding problems
- Developmental delay (40% to 60%; most common in spastic quadriplegia)
- Sensory impairment
- Seizures (approx. 40% of those with spastic hemiplegia)
Cerebral Palsy - Therapeutic Management
Goals: early recognition & promotion of optimal development to enable affected children to attain normalization & their potential within the limits of their existing health problems

* Disorder is permanent; therapy is primarily preventive and symptomatic

Therapy has 5 broad aims
1) Establish locomotion, communication & self-help skills
2) Gain optimal appearance & integration of motor functions
3) Correct associated defects as effectively as possible
4) Provide educational opportunities adapted to child's need & capabilities
5) Promote socialization experiences with other affected & unaffected children

Evaluated & managed on an individual basis
- Scope of child's needs requires multidisc. planning & care coordination among professionals & the family
- Outcome for child & family with CP is normalization & promotion of self-care activities that empower the child & family to achieve max. potential

Ankle-foot orthoses (AFOs, braces) are worn by many CP children
- used to help prevent or reduce deformity, inc. energy efficiency of gait and control alignment.

Other mobilization devices: wheeled scooter boards (allows children to propel themselves while on abdomen), wheeled go-carts (provides sitting balance and serves as an early "wheelchair"), bicycle walkers and special devices that leave the upper extremities free; strollers can be equipped with custom seats for dependent mobilization

Orthopedic surgery may be required to correct contractures or spastic deformities
- provide stability for an uncontrollable joint
- provide balanced muscle power
- Includes tendon-lengthening (especially heel-cord), release of spastic wrist flexor muscles, and correction of hip & adductor muscles to improve locomotion
- Selective dorsal rhizotomy has proveded marked improvement in some CP children.
-- Involves selectively cutting dorsal column sensory rootlets that have an abnormal response to electrical stimulants
-- Requires intensive PT and family commitment.
-- Results in flaccid muscles; child must be retaught to sit, stand & walk

Surgical intervention usually reserved for the child who doesn't respond to more conservative measures but is also indicated for children who's spasticity causes progressive deformities
- Primarily used to improve function than for cosmetic purposes
- Followed by PT

Intense pain may occur with muscle spasms in CP patients
- Oral pharmacologic agents have had little effect on improving muscle coordination, but are effective in dec. overall spasticity
-- Dantrolene sodium
-- Baclofen (Lioresal)
-- Diazepam (Valium)
* Common side effects: hepatotoxicity (dantrolene), drowsiness, fatigue and muscle weakness; less commonly, diaphoresis and constipation (baclofen)
* Diazepam (Valium) is frequently used but should be restricted to older children/adolescents

Botulinum toxin A (Botox) is also used to reduce spasticity in targeted muscles.
- Injected into a selected muscle (commonly quadriceps, gastrocnemius, or medial hamstrings) after applying a topical anesthetic.
- Acts to inhibit acetylcholine into a specific muscle group, thereby preventing muscle movement.
- When administered early in the course of the illness: contractures may be prevented; particularly in the lower extremities, thus avoiding surgical procedures with possible adverse effects
- Goal: allow stretching of the muscle as it relaxes and permit AFO assisted ambulation.
* Major adverse effect is pain @ the injection site.
- Prime candidates: children with spasticity confined to lowere extremities
-- drug weakens spasticity so the muscles can be stertched and the child may ambulate with or without orthoses
- Onset of action: within 24-72 hours; Peak observed @ 2 weeks; Duration: 3-6 months

Children with CP may also have pain as a result of surgical procedures intended to reduce contracture deformities, position & gastroesophogeal reflux, and PT.
- Pain management

Neurosurgical & pharmacologic approach to managing CP associated spasticity: Intrathecal baclofen pump, which infuses baclofen directly into the intrathecal space surrounding the spinal cord to provide relief of spasticity
- Best suited for children with severe spasticity that interferes with ADLs
- Screened before pump placement by a "test dose" of intrathecal baclofen via lumbar puncture
-- Observed for side effects (hypotonia, somnolence, seizures, n/v, headache, and catheter- or pump-related problems) and spasticity relief for several hours
-- If positive effect noted: considered a candidate for pump placement

- Performed by a neurosurgeon in the OR
-- Size of a hockey puck placed in the subcutaneous space of the midabdomen
-- Intrathecal catheter tunneled from lumbar area to the abdomen and connected to the pump
-- Pump filled with baclofen and programmed to provide a set dose using a telemetry wand and computer

- Benefits
-- Fewer systemic side effects than oral baclofen
-- Dosage titration for max. effect
-- Reversibility of therapy via removal if so desired

- Child may remain hospitalized for 3 - 7 days to adjust dosage and ensure proper healing
- Outpatient visits to refill pump and adjust dosages occur every 4-6 weeks, depending on the patient's response to tx
- Most suited for a multidisc. setting where rehab specialists are readily available & consistently involved in the patient's ongoing care
- Abrupt withdrawal (especially @ high doses) may result in rebound spasticity, pruritis, hyperthermia, rhabdomyolysis, disseminated intravascular coagulation, multiorgan failure, and death
-- Some cases: may mimic sepsis

Antiepileptic drugs are routinely prescribed for children who have seizures
- Includes carbamazepine (Tegretol), divalproex (valproate sodium & valproic acid; Depakote)
- Gabapentin (Neurontin; used in adults with spinal cord injury): has decreased spasticity (no studies proving effectiveness on children)
- Clonidine (Catapres) and tizanidine (Zanaflex) used to dec. spasticity in adults with SCI & multiple sclerosis; use in children with CI yet to be reported
* Monitor these meds for maintenance of therapeutic levels & avoidance of subtherapeutic or toxic levels

Dental hygiene is especially important
- Regular visits to the dentist and prophylaxis (brushing, fluoride, flossing) should be instituted as soon as the teeth erupt
- Especially important for children receiving phenytoin (gum hyperplasia)

Additional problems: Constipation from neurologic deficits and lack of exercise; poor bladder control & urinary retention, chronic respiratory tract infections and aspiration pneumonia (occurs as a result of gastroesophageal reflux), abnormal muscle tone, immobility, and altered positioning (and skin problems as a result of altered positioning as well as poor nutrition and immobility)

Wide variety of technical aids to help improve functioning
- Electromechanical toys that employ biofeedback and operate from a head unit
-- Manipulated when head and trunk are in correct alignment
- Computerized toys and games enhance eye-hand coordination
- Microcomputers combined with voice synthesizers aid with speech difficulties by allowing them to "speak" and print messages on screen or paper

Other electronic devices allow independent functioning
- Sensors can be activated and deactivated with a headstick or tongue or other voluntary muscle movement over which the child has control
- Voice-activated computer technology may also allow for inc. mobility and ambulation with specially designed devices (wheelchairs)
-- Makes it possible for CP people to function eventually in their own residences and extended to the workplace

Physical therapy: one of the most frequently used conservative tx modalities
- Requires specialized skills of qualified therapist with extensive repertoire of exercise methods to make a program to stimulate each child to achieve their functional goals

Active therapy program involves family; PT; other members of the health team
- Most common approach: using traditional types of therapeutic exercises which consists of stretching, passive, active and resistive movements applied to specific muscle groups or joints to maintain or inc. ROM, strength or endurance
Cerebral Palsy - Prognosis
In general: more severe, the worse the prognosis
- Children with severe physical disability, mental retardation, tube feedings, and severe seizures are known to have a shortened life expectancy

- 30-50% of CP are mentally retarded; even a higher % have mild cognitive & learning deficits
-- Many children with severe spastic quadriplegic CP have normal intelligence.

Growth affected in children with spastic quadriplegia; many children remain below 5th percentile for age and gender

As CP children transition into adulthood, 30% remain in home and are cared for by parent or caregiver; 50% with spastic quadriplegia live in independent settings and function at appropriate social levels considering their disability

Vocational rehab and higher education are possible for adults with CP
- one study found 53% of all CP people were able to work outside of the home in regular jobs; 1/3 of the severely disabled CP adults worked outside of the home

** Discourage the use of mobile infant walkers with CP
- Possible injury risk for normal kids; especially hazardous for CP
** No jumping seats (that hang in doorways)
Cerebral Palsy - Nursing Care
Parents are participating earlier in tx programs for child (due to identification & tx at an earlier age)
- Taught proper handling and home care of young children with CP
- Requires a carefully planned program so that their role change from parent to caregiver can be melded into the already established relationship
- Close work with other multidisc. team members is essential
- Nurses reinforce the therapeutic plan and assist family in devising & modifying equipment and activities to continue at home

More frequent rest periods should be arranged to avoid fatigue in children with CP (expends a lot more energy)

Tailor diet to activity and metabolic needs
- Gastrostomy feedings may be necessary to supplement regular feedings & ensure adequate weight gain, particularly in the child who is at risk for growth failure and chronic malnutrition
- In severe CP and subsequent oral feeding difficulties: feeding gastrostomy should be considered
- Considered a supplement to oral feeding and often recommended when illness & dec. fluid or med intake affect well-being
- Oral feedings continued for oral motor skills
- Weight gain perceived important measure of efficiency of adequate oral feeding

Patients may need advice and assistance with med administration through a gastrostomy tube to prevent clots
- Skin-level gastrostomy is particularly suited for CP

Because jaw control is often compromised: normal control can be achieved if feeder provides stability of the oral mechanism from the side or front of the face
- Front: middle finger of the nonfeeding hand is placed posterior to the body portion of the chin, thumb placed below the bottom lip, and index finger placed parallel to mandible
- Manual jaw control from the side helps with head control, neck correction, trunk hyperextension, and jaw stabilization
-- Middle finger of the nonfeeding hand is placed posterior to the bony portion of the chin, index finger on chin below lower lip and thumb obliquely across the cheek for lateral stability

Safety precautions
- Protective helmet if subject to falls or capable of head injury on hard objects
- Adapt home & play environment to child's needs to prevent bodily harm (risk for altered proprioception and falls)
- Immunizations to prevent childhood illnesses and protect against resp. tract infections (influenza)
- Dental problems based on level of involvement

PT, Speech therapy, OT are particularly important in establishing and maintaining muscle function, adequate speech & phonation, and identification of modifciations for the child's environment and satisfactory ADL completion

Educational requirements determined by needs and potential
- Mild to moderate involvement are generally able to participate in regular classes (for varying amounts of time).
- Resource rooms available in most schools to provide more individualized attention
- Integrate CP children into regular classrooms: initial goal

- For those unable to benefit from formal education: vocational training program
- At adolescence: prevocational and vocational counseling/guidance are arranged
* At any phase or in any setting: education geared towards child's assets

Recreational outlets and after-school activities should be considered for the child unable to participate in the rgular athelteic programs and other peer activities
- Some children can compete in athletic & artistic endeavors; many games and pastimes suited to their capabilities
- Competitive sports becoming increasingly available to disabled children; offers an added dimension to physical activity
- Refer to National Disability Sports Alliance

Recreational activities stimulate children's interest & curiosity, helps them to adjust to their disability, improves functional abilities, and builds self-esteem
- Any accomplishment that helps approach a "normal" way of life enhances self-concept
Cerebral Palsy - Nursing Care: Family Support
Most valuable: support and help in coping with emotional aspects of disorder
- Supportive counseling directed toward understanding the implications of the dx and all the feelings it engenders
- Later clarification re: what they can expect from the child and HCP
- Educate in principles of family-centered care and parent-professional collaboration: essential
- May require assistance in modifying home environment for care of the child
- Special considerations for transportation to practitioner's office and other health care agencies

Care management for the child & family with CP is an important nursing role.
- Many cases: family assumes complete care and becomes adept in meeting the individual needs
- Home health nurse or care manage must support and encourage families who take primary role in care of CP
- Stress principles of normalization in times of managing daily tasks and family life changes

Support parents in frustration, problem solving, concerns, approaches to helping the child and lack of gratification as well as all the positives
- All aspects must be explored and discussed
- Family requires support and counseling
-- Siblings may respond to child's presence with overt or less evident behavioral problems
- Family needs nurses who can provided continued contact, support and encouragement through the long process of habilitization

Parents may find help & comfort from parent groups
- Can share problems and concerns
- Can derive comfort and practical information
- Groups most helpful through sharing experiences and accomplishments.
Cerebral Palsy - Nursing Care: Hospitalized Child Support
When CP children are hospitalized, it is for another reason or for corrective surgery
- Nursing care same as any other child with a disability
- Should be approached the same
- Speech impairment common
- Therapy program should be continued, insofar as the condition allows, during hospitalization
-- should be incorporated in the nursing care plan with every effort made to ensure ground gained hasn't been lost.
- Encourage parent to room-in and actively participate in care (facilitates home therapy program continuation; helps child adjust)
- Hospitalization may be the first time the parent can defer care
-- Respite may be crucial to parent's well-being
Spina Bifida (Myelomeningocele) - Overview
Abnormalities that derive from the embryonic neural tube (Neural Tube Defects)
- Constitutes largest group of congenital abnormalities consistent with multifactorial inheritance
- Normally spinal cord and cauda equina are encased in a protective sheath of bone and meninges
-- Failure of neural tube closure results in defects of various degrees (entire length of neural tube or a small area)

Rates of NTDs have declined from 1.3 per 1000 births to 0.3 with the introduction of folic acid
- Further data: 23% decline from 1996 to 2001
- Inc. use of prenatal dx techniques and pregnancy terminations have also affected overall incidence

Myelodysplasia: any malformation of the spinal canal and cord
- Midline defects involving failure of the osseous (bony) spine to close: Spina bifida, the most common defect of the CNS.

Spina bifida occulta: defect that is not visible externally.
- Most frequently in the lumbosacral area (L5 & S1)
- May not be apparent unless there are associated cutaneous manifestations or neuromuscular disturbances

Spina bifida cystica: visible defect with an external saclike protrusion.
- 2 major forms of SB cystica
1) Meningocele: encases meninges and spinal fluid but no neural elements
-- not associated with neurologic defect
2) Myelomeningocele (meningomyelocele): contains meninges, spinal fluid, nerves
- Associated with neurologic defect in varying, often serious degrees

*Clinically: Spina bifida used to refer to myelomeningocele
Spina Bifida - Pathophysiology
Believed that primary defect is a failure of neural tube closure during embryo's early development (first 3-5 weeks)
- Evidence also implicates multifactorial etiology (drugs, radiation, maternal malnutrition, chemicals, possible genetic mutation in folate pathways)
- Evidence of genetic component in development of SB
-- Myelomeningocele may occur in association with syndromes like trisomy 18, PHAVER syndrome, Meckel-Gruber syndrome
- Additional factors predisposing children to inc. risk of NTDs: prepregnancy maternal obesity, maternal DM, previous NTD pregnancy, low maternal vitamin B12, maternal hyperthermia, AED use during pregnancy

Degree of neurologic dysfunction depends on where the sac protrudes through the vertebrae, the anatomic level of the defect, and the amount of nerve tissue involved
- Most myelomeningoceles involve the lumber or lumbosacral area

*Hydrocephalus is frequently associated in 80-90% of children
Spina Bifida - Diagnostic Evaluation
Made on the basis of clinical manifestations and examination of the meningeal sac.

Dx Measures used to evaluate the brain and spinal cord
- MRI
- Ultrasound
- CT
- Myelography

Lab exams are used primarily to determine causative organisms for common complications associated with myelomeningocele: meningitis and UTI

Prenatal Detection
- It is possible to determine the presence of some major open NTDs prenatally
- Ultrasonographic scanning of the uterus
- elevated maternal concentrations of alpha-fetoprotein (AFP or MS-AFP), a fetal-specific y1 globulin in amniotic fluid may indicate anencephaly or myelomeningocele

Optimum time for performing these dx tests is between 16-18 weeks of gestation, before AFP concentrations normally diminish and in sufficient time to permit a therapeutic abortion

It is recommended that such dx procedures & genetic counseling be considered for all mothers who have borne an affect child; testing is offered to all pregnant women

Elective prelabor cesarean birth may result in less motor dysfunction

Chorionic villus sampling is also a method for prenatal dx of NTDs; carries certain risks (skeletal limb depletion) and not recommended before 10 weeks gestation
Spina Bifida - Clinical Manifestations
Cystica
- Sensory diturbances usually parallel to motor dysfunction

* Below 2nd lumbar vertebra
~ Flaccid, partial paralysis of lower extremities
~ Varying degrees of sensory deficit
~ Overflow incontinence with constant urine dribbling
~ Lack of bowel control
~ Rectal prolapse (sometimes)

* Below 3rd sacral vertebra
~ No motor impairment
~ May have saddle anesthesia with bladder and anal sphincter paralysis

- Joint deformities (sometimes produced in utero)
~ Talipes valgus or varus contractures
~ Kyphosis
~ Lumbosacral scoliosis
~ Hip dislocation or sublaxation

Occulta
- Frequently no observable manifestations

- May be associated with 1 or more cutaneous manifestations
~ Skin depression or dimple
~ Port-wine angiomatous nevi
~ Dark tufts of hair
~ Soft, subcutaneous lipomas

- May have neuromuscular disturbances
~ Progressive disturbance of gait with foot weakness
~ Bowel & bladder sphincter disturbances
Spina Bifida - Therapeutic Management
Early surgical closure of the myelomeningocele through fetal surgery has been evaluated in relation to prevention of injury to the exposed spinal cord tissue and improvement of neurologic and urologic outcomes in the affected child.
- Initial success and survival rates seem positive
- Reports vary in relation to the success of fetal surgery in actual reduction of urologic problems, improvement of lower leg function, and prevention of hydrocephalus postanatal
- Overall mortality rate from fetal surgery: 4%
- Complications: oligohydramnios, preterm delivery, smaller birth weight

Management requires multidisciplinary approach involving the specialties of neurology, neurosurgery, peds, urology, orthopedics, rehab, PT, social services, along with intensive nursing care
- Collaborative efforts focused on
1) myelomeningocele and associated problems - hydrocephalus, paralysis, orthopedic deformities, genitourinary abnormalities
2) possible acquired problems that may or may not be associated (meningitis, hypoxia, hemorrhage)
3) other conditions such as cardiac or GI malformations
Spina Bifida - Therapeutic Management: Infancy
Preventing infection
Perform neurologic assessment
- observing for associated anomalies
Dealing with impact of anomaly with family

Although meningoceles are repaired early, the philosophy regarding skin closure of myelomeningocele varies
- Most authorities: early closure (within the first 24-72 hours)
- Early closure (withing 12-18 hours) prevents local infection and trauma to exposed tissues and avoids stretching of other nerve roots (which may occur when meningeal sac expands during first hours after birth) thus preventing further motor impairment
- Broad spectrum antibiotics are initiated and neurotoxic substances (povidone-iodine) are avoided at the malformation

Associated problems are assessed and managed by appropriate surgical & supportive measures
- Shunt procedures provide relief from imminent or progressive hydrocephalus
- Meningitis, UTI and ventriculitis are tx'ed with vigorous antibiotic therapy and supportive measures
- Surgical intervention for Chiari malformation (downward herniation of the brain into the brainstem) or tethered cord (scar tissue binding the spinal cord) indicated when child is symptomatic
- Improved surgical techniques do not alter the major physical disability, spinal defect, or chronic UTI that affect the quality of life for the children
- Superimposed on the physical problems are the effects that the disorder has on family life and finances, including the need for long-term specialized school and health care services
Spina Bifida - Therapeutic Management: Orthopedic Considerations
Evaluate musculoskeletal problems that will affect later locomotion and institute tx without delay (where indicated)

Neurologic assessment determines neurosegmental level of lesion and enable recognition of spasticity and progressive paralysis, deformity potential, and functional expectations

Orthopedic management
- Prevention of joint contractures
- Correction of existing deformity
- Prevention or minimization of effects of motor and sensory deficits
- Prevention of skin breakdown
- Acquisition of the best possible function of affected lower extremitites

Common orthopedic problems requiring attention in SB include:
- Knee, hip feet and spine deformities
- Fractures and insensate skin further complicate orthopedic care
- Later problems: kyphosis, scoliosis

Preventive skin care is important
- SB children have dec. sensitivity in lower extremities
- High % (60%) of children seen in wound clinic for skin breakdown had myelomeningocele

* Status of neurologic deficit remains the most important factor in determining child's ultimate functional ability

Lightweight orthoses available to provide mobility to children with SC lesions
- braces, "walking" devices, custom wheelchairs

Early infancy: intervention with passive ROM, positioning, and stretching may help dec. the incidence of contractures

Corrective surgical procedures, when indicated are best initiated at an early age so there will not be significant lag behind agemates in developmental progress

Surgery seldom recommended unless it will improve sitting position in a wheelchair and function for ADLS and mobility
Spina Bifida - Therapeutic Management: GU Function
Myelomeningocele is the most common cause of neuropathic (neurogenic) bladder dysfunction
- In infants: goal is to preserve renal function
- In older children: goal is to preserve renal function and achieve optimal urinary continence

Urinary incontinence is a chronic, often debilitating problem for the child.
- Neurogenic bladder can also produce urinary system distress
-- Symptomatic UTIs, uerterohydronephrosis, vesicouretal reflux or renal insufficiency

Bladder dysfunction in children vary according to the level of the neurologic lesion and influence of bony growth and development on the spine.
- Ongoing urologic monitoring is essential.

* Evidence showing that early intervention serves to improve bladder function, reduces risk of subsequent urinary system distress and dec. need for reconstructive surgery of the lower UT
- based on evaluation during neonatal period and before complications

Tx of renal problems
1) Regular urologic care with prompt and vigorous tx of infections
2) Regular emptying of the bladder, such as clean intermittent catheterization
- Taught to and performed by parents and self-cath taught to children
3) Medications to improve bladder storage and continence
- Oxybutynin chloride (Ditropan) and tolderodine (Detrol)
4) Surgical procedures
- Vesicostomy (bladdger surgically brought out to the abdominal wall, allowing continuous urinary drainage)
- Augmentation enterocystoplasty (using a segment of bowel or stomach to inc. bladder capacity, reducing high bladder pressure)

Despite combined efforts, some children with myelodysplasia may continue to experience debilitating urinary incontinence
- Urinary diversion (Mitrofanoff procedure) to gain social continence
-- Cath channel surgically created from appendix, ureter, or tapered bowel; proximal end of channel connected to the bladder with the distal end brought out as a small stoma on the abdominal wall (usually near umbilicus); bladder neck may be sutured to prevent urinary leakage from the urethra
-- CIC through the easily accessible route fosters greater independence in children, especially those unable to transfer from wheelchair to toilet for CIC
Spina Bifida - Therapeutic Management: Bowel Control
Some degree of fecal continence can be achieved with diet modification, regular toilet habits, prevention of constipation and impaction
- Frequently lengthy

Dietary fiber (recommended 10g/day), laxatives, suppositories or enemas, aid in producing regular evacuation

Older children and adolescents seeking more independence may attain bowel incontinence and higher quality of life after undergoing antegrade continence enema
- Similar to Mitrofanoff; appendix or ileum is used for a cath. channel with attachment of the proximal end to the colon.
-- Distal end of channel exits through a small abd. stoma; every 1-2 days, a cath is passed through the stoma, allowing enema solution to be instilled directly to the colon
-- After admin., child sits on toilet for 30-60 mins as stool is flushed out through rectum

Frequency of enemas and volume of solution used to completely evacuate the bowel varies among individual
Spina Bifida - Therapeutic Management: Prognosis
Early prognosis depends on neurologic deficit present @ birth, including motor ability, bladder innervation and associated neurologic anomalies

Early surgical repair of the spinal defect, abx therapy to reduce the incidence of meningitis and ventriculitis, prevention of urinary system dysfunction and early detection & correction of hydrocephalus have significantly inc. survival rate and quality of life

Many children with SB achieve partial independent living and gainful employment
- Report vary, and many include adults who were born before medical advances and surgical techniques in the past 25 years

Research has noted that as adolescents with SB transition to young adulthood, they have inc. difficulty obtaining centralized health care for the different health problems associated with SB
- Chronic condition has an array of associated complications (hydrocephalus, shunt malfunctions, scoliosis, bowel & bladder management issues, latex allergy, epilepsy

Based on current medical knowledge and ethical consideration: early aggressive management is favored for child with myelomeningocele
Spina Bifida - Therapeutic Management: Prevention
Widespread use of folic acid among women of childbearing age is expected to significantly dec. SB incidence
- Daily intake of 0.4 mg (400 mcg) of folic acid in women of childbearing age will prevent 50-70% of all NTD cases

For women with previous NTD pregnancy: inc. folic acid intake to 4 mg/day
- supervised by practitioner beginning 1 month before a planned pregnancy and continuing during the first trimester
- Supplementation of 4 mg of folate should not be given in multivitamins because of overdose risk of other vitamins

To ensure adequate daily intake of recommended folic acid amount
- Folic acid supplement
- Fortified breakfast cereal containing 100% of the RDA of folic acid (Kellogg's Product 19, General Mills Total, Multigrain Cheerios Plus)
- Inc. consumption of other fortified foods (cereal, bread, rice, grits, pasta) and naturally rich foods (green leafy vegetables, citrus fruits)

*Only population where folic acid hasn't proved effective is epileptic women taking AED during pregnancy

Adolescent girls and women of childbearing age need to be educated about folic acid to prevent NTDs (half of all pregnancies are unplanned)
- 0.4 mg (400mcg) is most easily obtained from multivitamin supplement
Spina Bifida - Nursing Care
At birth: examination performed to assess the intactness of the membranous cyst

During transport: every effort made to prevent trauma to protective covering

In addition to routine assessment of newborn, assess infant for level of neurologic involvement
- Movement of extremities or skin response (especially anal reflex) may provide clues to degree of motor or sensory impairment is noted
- Important to observe the infant's behavior in conjunction with the stimulus (limb movements can be induced in response to spinal cord reflex activity that has no connection with the higher centers)
- Observe urinary output, especially if diaper remains dry (may indicate urinary retention)
- Abd. assessment revealing bladder distension, even with a wet diaper, may indicate urinary overflow in a retentive bladder
- Head circumference measured daily and fontanels are examined for signs of tension or bulging
Spina Bifida - Nursing Care: Care of Myelomeningocele Sac
Place infant in an incubator or warmer
- Temperature is maintained without clothing or covers that may irritate the spinal lesion
- If overhead warmer used: dressings over defect require more frequent moistening because of the dehydrating effect of the radiant heat

Before surgical closure: myelomeningocele is prevented from drying by the application of a sterile, moist, nonadherent dressing over defect
- Moistening solution: sterile normal saline
- Dressing changed frequently (every 2-4 hours) and sac is closely inspected for leaks, abrasions, irritation or signs of infection
- Sac must be carefully cleansed if it becomes soiled or contaminated
- Sac sometimes ruptures during delivery or transport; any opening in the sac greatly inc. the risk of infection to the CNS

To prevent stool contamination of the SB defect preop: obtain a surgical drape, cut a portion to fit the sacrum and secure drape using nonlatex tape.
- Place rest of drape loosely over dressing, covering stool and preventing stool exposure

* Observe for early signs of infection
- Temp. instability (axillary), irritability, lethargy and signs of ICP (indicative of developing hydrocephalus)

* Avoid rectal temperatures (can cause irritation and rectal prolapse)

Most important and challenging aspects in early care of myelomeningocele is positioning
- Before surgery: prone to minimize tension on the sac and risk of trauma
- Allows for optimal positioning of the legs (especially in cases of associated hip dysplasia
- Prone with hips slightly flexed and supported to reduce tension on the defect
-- Legs maintained in abduction with a pad between the knees to counteract hip subluxation and a small roll is placed under the ankles to maintain neutral foot position
- Aids include: diaper rolls, pads, small foam pads or specially designed frames and appliances
Spina Bifida - Nursing Care: Prevent Complications
Prone position affects other aspects of care
- Infant is more difficult to keep clean
- Pressure areas a constant threat
- Feeding is a problem
* Turn head to one side for feeding while still in prone position
- Fortunately most defects repaired early; infant can be held for feeding soon after surgery
* Special care to avoid pressure on the operative side

Diapering may be contraindicated until defect has been repaired and healing is well advanced or epithelialization has taken place
- Padding beneath diaper area is changed as needed to keep skin dry and free of irritation
- When urinary retention is detected, employ CIC
- Continuous passage of stool, often misinterpreted as diarrhea, a constant irritant to the skin and source of infection to the spinal lesion

Areas of sensory and motor impairment are subject to skin breakdown, requiring meticulous care
- Place infant on special mattress or mattress overlay to reduce pressure on knees and ankles
- Periodic cleansing, lotion application, gentle massage to aid circulation

Gentle ROM exercises to prevent contractures; contracture stretching when indicated
- May be restricted to knee, ankle and foot joint
- When hip joints are unstable, stretching against tight hip flexors or adductor muscles (which act as bowstrings) may aggravate a tendency toward sublaxation
- Consult PT for short- and long-term management

Infants with unrepaired myelomeningocele may be held in the arms and cuddled as unaffected infants are to meet need for tactile stimulation
- Caressing, stroking, other comfort measures
Spina Bifida - Nursing Care: Postoperative Care
Same care as any postsurgical infant
- Monitor vitals
- Monitor I&O
- Nourishment
- Observe for s/s of infection
- Pain management as needed

Care of operative site carried out under direction of the surgeon
- Includes close observation for signs of CSF leakage

General care continued postoperatively

Prone position maintained after surgical closure
- Many neurosurgeons allow side-lying or partial-lying position unless it aggravates a coexisting hip dysplasia or permits undesirable hip flexion
-- allows opportunity for position changes, reducing the risk of pressure sores and facilitates feeding
- If permitted: infant can be held upright against body, with care taken to avoid pressure on operative site

After effects of anesthesia subsides and infant is alert, feedings may be resumed unless there are other anomalies or associated complications
Spina Bifida - Nursing Care: Support & Education
Parents encouraged to become involved in care as soon as they can cope with the condition
- Learn to continue hospital care @ home (positioning, feeding, skin care, ROM when appropriate)
- Teach CIC when prescribed
- Teach s/s of complications (urinary, neurologic, orthopedic) and how to obtain help when needed

Mothers who wish to breastfeed is encouraged to do so
- Shortly after delivery: start a program to pump to initiate and maintain milk supply until child can breastfeed
- May require support from nurses, physicians, family members because of separation from infant for surgery and recovery

Long-range planning with & support of parents and newborn begin in the hospital; continues throughout childhood, even to young adulthood
- Life expectancy: well into adulthood
-- Planning should involve long-term goals and plans for optimum function as an adult
--- Aspects of adulthood include receiving educational or vocational training and education, living independently, having a mate, having sexual relationships, and bearing & rearing children; all are important and shouldn't be overlooked.

Nurses assume an important role as the central member of the health team
- Reviews information with the family, takes responsibility for teaching, acts a liaison between inpatient and outpatient services

The child will need numerous hospitalizations over the years, each being a source of stress to where they can be vulnerable

Habilitation involves not only solving problems of self-help and locomotion, but also solving the most distressing problem of urinary or bowel incontinence, which threatens social acceptability
- Assistance in preparing the child and school regarding the special needs helps provide better initial adjustment to broader social experience

*Spina Bifida Association of America
Latex Allergy - Overview
Identified as a serious health hazard when a report linked intraoperative anaphylaxis with latex in children with SB.

High prevalence (80%) attributed to repeated exposure to latex during surgery & numerous bladder caths and possible disease-associated factors
- Evidence suggests children with SB are at risk for latex allergy as a result of the disease itself

Allergic reactions range from urticaria, wheezing, watery eyes, & rashes to anaphylactic shock.
- More adverse reactions tend to occur when latex comes in contact with mucous membranes, wet skin, bloodstream, or an airway

Cross reactions to a number of foods: banana, avocado, kiwi, chestnuts

Diagnosed in infants: manifests as wheezing, facial swelling, facial rash, anaphylaxis

In addition to SB, high-risk populations include urogenital anomalies, multiple surgeries and health care workers

Most important goals are prevention of latex allergy and identification of a child with a known hypersensitivity
- High-risk and latex-allergic individuals must be managed in a latex-free environment
-- Care must be taken so they do not come into contact with products or equipment containing latex at any time during medical treatment (direct or secondary)
- Allergy testing has been used to identify latex allergy with varying success
* Skin prick and provocation carry the risk of allergic reaction or anaphylaxis.
* Radioallergosorbent test (RAST) used to measure serum level of latex-specific immunoglobin E
- 90-95% sensitive
- Pretreatment with antihistamine and steroids (dexamethasone) before and after surgery to reduce possibility of serious reaction is controversial (interferes with healing)

Reduce exposure from birth on may decrease allergy development in children with SB
- Many health care facilities are establishing latex-safe environments when patients and health care workers are at risk
- Important to use products with lowest potential risk of sensitizing patients and staff members

US FDA proposed user labeling for latex-containing devices that come into contact (direct or indirect) with live human tissue

Identification of latex sensitive is best accomplished through careful screening of ALL patients
- Children with latex allergy should carry or wear some medical ID; should carry injectable epinephrine pen and a pair of latex-free gloves for emergencies
- Education programs re: latex hypersensitivity aimed at those who care for high-risk groups (children with SB; includes relatives, school nurses, teachers, childcare workes, babysitters)

Ask all patients, not only those at risk, about allergic reactions to latex during health interview with parent or child
- Make it routine of all preop and preprocedure hx.
- Stress importance of allergy hx to all personnel
Latex Allergy - Selected Medical Items Possibly Containing Latex
Adhesive bandage strips

Airways, masks (O2)

Anesthesia vent circuits, bags

BP cuffs and tubing

Bulb syringe

Catheters (indwelling, condom)

Chux (washable rubber pads)

Crutches (axillary, hand pads)

Dressings and wraps (various)

Elastic bandages

Electrode pads, bulbs

Endotracheal tubes

Finger cots

Gloves (sterile and examining, surgical and medical)

Hep-lock adapter

IV tubing, injection ports, bags, burets

Medication vials

NG tubes

Ostomy supplies

Penrose drains

Pulse oximeters

Spacer (metered dose inhaler)

Stethoscope tubing

Suction tubing

Syringes

Tape (cloth adhesive, paper)

Tourniquet

Urodynamics rectal pressure catheters

Vascular or compression stockings

Wheelchair cushions, tires
Latex Allergy - Selected Home/Community Items Possibly Containing Latex
Art supplies (paint, markers, glue)

Balloons (not Mylar)

Balls (Koosh, tennis, bowling)

Cardiopulmonary resuscitation manikins

Carpet backing, gym floor, gym mats (Broadloom carpets contain no natural rubber latex. For other products, provide barrier cloth or mat)

Chewing gum

Cleaning or kitchen gloves

Condoms, contraceptive sponges, diaphragms

Dental dams and equipment

Disposable diapers

Elastic exercisers

Elastic on legs, waist of clothing, some disposable diapers

Feeding nipples

Foam rubber lining on splints, bracers

Electric toothbrush or massager (for CPT)

Latex paints, sealants, stains (No natural rubber in latex paint; although it may be present in some waterproof paints and sealants)

Pacifier

Playpits, playground surfaces with
natural rubber latex

Racquetball handles

Rubber bands

Water toys, swim, scuba equipment

Wheelchair cushions, tires

Zippered plastic storage bags
Latex Allergy - Associated Medical Conditions
Spina bifida

Urogenital anomalies

Imperforate anus

Tracheoesophageal fistula

VATER association (Vertebral defects, imperforate Anus, TracheoEsophageal fistula, Radial and renal dysplasia)

Preterm infants

Ventriculoperitoneal shunt

Mental retardation

Cerebral palsy

Spinal cord injuries

Multiple surgeries

Atopy
Latex Allergy - Identifying Latex Allergy
Does your child have any symptoms (coughing, sneezing, rashes, wheezing) when handling rubber products (balloons, tennis or Koosh balls, adhesive bandage strips) or when in contact with rubber hospital products (gloves, catheters)?

Has your child ever had an allergic reaction during surgery

Does your child have a hx of rashes, asthma, or allergic reactions to medication or foods, especially milk, kiwi, bananas or chestnuts?

How would you identify or recognize an allergic reaction in your child?

What would you do if an allergic reaction occurred?

Has anyone ever discussed latex or rubber allergy or sensitivity with you?

Has your child had any allergy testing?

When did your child last come in contact with any type of rubber product? Were you present?
Duchenne (Pseudohypertrophic) Muscular Dystrophy - Overview
Most severe and most common MD of childhood

Inherited as X-linked recessive trait
- single-gene deficit located on short arm of X chromosome

Reportedly high mutation rate
- Positive family hx in 65% of all cases
* Genetic counseling important aspect of care

Males affected almost exclusively
- Results from mutations of the gene that encodes dystrophin (skeletal muscle protein product)
- Absent in DMD, reduced or abnormal in Becker MD
-- Becker MD have later onset, not as severe

Incidence: 1 in 3600 male births
Becker: 1 in 30,000

Most children with DMD reach appropriate developmental milestones early in life, with mild, subtle delays
- Evidence of muscle weakness usually appears during the 3rd to 7th year
* History of delay in motor development, particularly walking
- Difficulties in running, riding a bike, and climbing stairs are usually the first symptoms noted
- Later: abnormal gait on a level surface is apparent
-- In early years, rapid developmental gains may mask progression of the disease

Question parents to reveal child having difficulty rising from sitting or supine

Affected boys will have waddling gait and lordosis, fall frequently and develop a characteristic manner of rising from squatting or sitting position on the floor (Gower's sign)
* kneeling, pushing torso upright with knees straight; "walking" his hands up his legs

Enlarged calves

Pseudohypertrophy: applied to muscular enlargement by fatty infiltration
- Profound muscular dystrophy occurs in later stages
- As disease progresses, contractures and deformities involving large and small joints are common complications
- Ambulation impossible by 12 years
- Facial, orophayngeal and resp. muscles are often spared until terminal stages
- Ultimately: disease process involves the diaphragm and auxiliary muscles of respiration, and cardiovascular involvement (cardiomyopathy, dysrhythmias, heart failure) is common
- Mild mental delay is common in roughly 30% of all people with MD; many will have permanent learning disabilities.
-- Children with DMD should be transferred to early learning programs and eventually into regular classrooms as much as possible

Eventual cause of death: respiratory tract infection or cardiac failure
- Much progress has been made in providing ventilatory methods to prolong & maintain the quality of life
Duchenne Muscular Dystrophy - Diagnostic Evaluation
Dx primarily established by blood polymerase chain reaction for dystrophin gene mutation

Prenatal diagnosis available as early as 12 weeks of gestation

Serum enzyme measurement, muscle biopsy, EMG also useful in establishing dx.

Serum creatinine kinase levels are extremely high in the first 2 years of life, before clinical weakness onset

If child demonstrates usual characteristics, has positive DMD family hx, and positive PCR: defer muscle biopsy
Duchenne Muscular Dystrophy - Therapeutic Management
No effective treatment

Corticosteroids prednisone and deflazacort has been evaluated as tx for DMD
- Several clinical trials demonstrated to inc. muscle strength and improve performance and pulm. function, with significant dec. in weakness progression when prednisone administered for 6 months
- Major side effects: weight gain (less in deflazacort), cushingoid facial appearance
- Deflazacort effective in dec. muscle wasting; available in Canada, not US

Primary goal: Maintaining optimal function in all muscles for as long as possible
- those who remain as active as possible are able to avoid wheelchair confinement for a longer time
Secondary goal: Contracture prevention
- Maintenance of function includes: stretching exercises, strength and muscle training, breathing exercises to inc. & maintain vital lung capacity, ROM exercises, surgery to release contracture deformities, bracing and ADL performance

Patients should always be involve in making decisions about child's care; teaching re: home safety and fall prevention is important
- Encourage parents to have child keep f/u appts. for medical care, pt and ot.
- Influenza and pneumococcal vaccines are encourage and avoid contact with resp. tract infections (Resp. tract infections most troublesome)

Eventually respiratory and cardiac problems become central focus
- Children with neuromuscular disease have abnormal breathing patterns (particularly during REM sleep) and hypoxia may occur as a result of inadequate oxygenation
- Children and parents should be involved in a discussion of long-term ventilation options

Cardiac & resp. assessment during wake-sleep cycles is imperative

Respiratory care for neuromuscular conditions in children include noninvasive ventilation (BiPAP) on a temporary or full-time basis, mechanically assisted coughing (MAC), or tracheotomy and relief of airway obstruction with coughing and suctioning devices
* Tracheotomy associated with more complications
- Home pulse ox. may be used to monitor o2 during sleep or to aid in deciding to use MAC

Several devices available for children with neuromuscular disease to assist in airway clearance when cough reflex is ineffective/diminished
- Mechanical cough inexsufflator (MIE) found safe for daily management
-- Delivers positive inspiratory pressures at a set rate, followed by negative pressure exsufflation coordinated with patient's own breathing rhythm; exsufflation designed to mimic cough reflex so mucus can be cleared
-- Airway suction after to clear airways
-- Survival may be prolonged with use of noninvasive ventilation and MAC as alternatives to tracheotomy and airway suctioning

Extensive cardiac evaluation recommended with child dx with DMD or Becker MD
- Neuromuscular conditions may not be seen with typical s/s of cardiac dysfunction
- Weight loss, n/v, cough, inc. fatigue on ADL performance and orthopnea should be carefully evaluated to detect early signs of cardiomyopathy

Genetic counseling recommended for parents, female siblings, maternal aunts and female offspring
- Long-term, end-of-life directives, palliative care options are issues to be discussed with the child and family
- Professional counseling may be required; referrals made as appropriate

Research of a number of tx for DMD in progress
- Clinical trials with glutamine and creatine monohydrate to preserve muscle strength; utrophin a protein similar to dystrophin and in large quantities can counteract dystrophin deficiency effects.
- Enzyme CT GalNAc transferase, which blocks muscle wasting in mice
Duchenne Muscular Dystrophy - Nursing Care
Major emphasis: help child and family cope with chronic, progressive, incapacitating disease; help design a program that will afford maximal independence and reduce associated predictable and preventable disabilities.; help child and family constructively deal with limitations that the disease imposes

Because of tech. advances: Children may live on to early adulthood
- Goals of care should also involve decisions re: quality of life, independence achievement, adulthood transition

Working closely with other team members, assist family in developing child's self-help skills to give satisfaction of being as independent as possible for as long as possible
- Requires continual evaluation of the child's capabilities (often difficult to assess).

Practical difficulties: physical limitations of housing and mobility
- Some houses and apartments unsuited for wheelchairs
- Transportation also a barrier
- Assistance with these problems require team problem solving
- Diet, nutritional needs, nutrition modification discussed based on needs of child and family

Social activities restricted; continually modified to accommodate needs of affected child
- As child becomes more incapacitated, family may consider home-based care, assisted living facility, respite care
- Unless severely incapacitated, child should be involved in decisions re: such care
- Nurses assist with decision making by exploring all available options and resources and suppor the child and family in the decision

Older boys with MD may also need psychiatric or psychologic counseling to deal with depression, anger and quality of life
- Encourage parents to be involved in support groups because it is shown that adequate social support from family, community, and other parents is crucial to appropriate coping in families and children with chronic illness

Regardless of how successful the program or how the family adapts: superimposed on physical and emotional problems associated with a child with a long-term disability is the constant knowledge of the ultimate outcome of the disease

Nurses are valuable as they come to know the family and their problems
- Alert to family's problems and needs and make referrals when needed

Muscular Dystrophy Association: USA
Urinary Tract Infection - Overview
One of the most common conditions of childhood
- Up to 10% of children will have a febrile UTI during 1st 2 years of life
-- Among febrile males, circumcision determines UTI risk
* Uncircumcised males younger than 3 months have highest prevalance of UTI than any group

Circumcision status should be assessed in male infants with unexplained fever

UTI may involve urethra and bladder (lower urinary tract) or the ureters, renal pelvis, calyces, and renal parenchyma (upper urinary tract)

UTI applied to presence of significant numbers of microorganisms anywhere within the urinary tract, except the distal 1/3 of the urerthra, which is usually colonized with bacteria
Urinary Tract Infection - Classification
UTI may be present with or without clinical symptoms
- Makes site of infection difficult to pinpoint with any degree of accuracy

Various terms used to describe UTIs
- Bacteriuria: Presence of bacteria in urine
- Asymptomatic bacteriuria: Significant bacteriuria ( > 100,000 CFU with no evidence of clinical infection)
- Symptomatic bacteriuria: Bacteriuria accompanied by physical signs of UTI (dysuria, suprapubic discomfort, hematuria, fever)
- Recurrent UTI: repeated eps of bacteriuria or symptomatic UTI
- Persistent UTI: persistence of bacteriuria despite abx tx.
- Febrile UTI: bacteriuria accompanied by fever and other physical signs of UTI; presence of fever typically implies pyelonephritis
- Cystitis: bladder inflammation
- Urethritis: urethra inflammatioin
- Pyelonephritis: inflammation of the upper urinary tract and kidneys
- Urosepsis: Febrile UTI coexisting with systemic signs of bacterial illness; blood culture reveals urinary pathogens
Urinary Tract Infection - Etiology
80% of cases: E.Coli
- Other gram-negative enteric organisms
- Usually found in anal and perineal region

Other organisms: Proteus, Pseudomonas, Klebsiella, Staph aureus, Haemophilus and coagulase-negative Staph.

Several factors contribute to UTI development in childhood
- Anatomic/Physical factors
-- Structure of urinary tract believed to account for inc. incidence of bacteriuria in females
---short urethra: 2 cm (0.75 inches) in young girls and 4 cm (1.6 inches) in mature women
-- Urethra closure at end of micturition may return contaminated bacteria to the bladder
-- Longer male urethra (20cm or 8inches in adult) and antibacterial properties of prostatic secretions inhibit entry and growth of pathogens

* Difference between circumcised and noncircumcised not enough to recommend routine circumcision

Most important host factor: urinary stasis
- Urine normally sterile; culture medium at 98.6F
- Normal conditions: bladder is repeatedly and completely flushed before organisms can multiply and invade
-- Urine that remains allows bacteria from urethra to rapidly become established

Incomplete bladder emptying (stasis) may result from reflux, anatomic abnormalities, dysfunction of voiding mechanism, or extrinsic ureteral or bladder compression caused by constipation

Key to preventing UTI: maintain adequate blood supply to the bladder wall by avoding overdistention and high bladder pressure

Altered Urine & Bladder Chemistry
- Several mechanical and chemical characteristics of urine and bladder mucosa helps maintain sterility
-- inc. fluid intake promotes flushing of normal bladder and lowers concentration of organisms in infected bladder
-- diuresis also seems to enhance antibacterial properties of renal medulla

Most pathogens favor alkaline medium
- Urine normally slightly acidic with median pH of 6.0
* pH of 5.0 hampers but doesn't eliminate bacterial multiplication

Cranberry juice to inc. urine acidity
- Limited evidence for value in promoting urinary tract health
- Further research required to clarify unanswered questions before recommendations can be made for using this supplement, especially in peds
Urinary Tract Infection - Diagnostic Evaluation
Depends on age

Confirmed by detection of bacteriuria in urine culture
- Collection difficult, especially in infants and very small children
- Several factors may alter urine specimen, contamination of a specimen from organisms from other sources than the urine (perineal and perianal flora in bag specimen) is most frequent cause of false-positives
- Unless specimen is morning sample, recent high fluid intake may indicate falsely low organism count
-- Children shouldn't drink large volumes of water to quickly obtain specimen

** Evaluate a child for UTI that is incontinent despite being toilet-trained, urine smelling strong, frequency & urgency

More accurate estimates of bacterial content from: suprapubic aspiration (children younger than 2 years) and proper bladder cath. (exclude first few mL)
- Take directly to lab for immediate culture

Tests to detect bacteriuria being used with increased frequency in UTI screening
- Dipstick tests for leukocyte esterase or nitrite are quick and inexpensive methods for detecting infection before final culture results

Localization of infection site may involve more specific tests
- Percutaneous kidney taps and bladder washout
- Also: ultrasonography, voiding cystourethrogram, intravenous pyelogram, dimercaptosuccinic acid scan after the infection subsides to identify anatomic abnormalities contributing to infection development and existing kidney changes
Urinary Tract Infection - Therapeutic Management
Objectives
1) Eliminate current infection
2) Identify contributing factors to reduce risk of recurrence
3) Prevent systemic spread of infection
4) Preserve renal function

Abx therapy initiated on basis of identification of pathogen, hx of abx use and location of infection

Several antimicrobial drugs available for UTI tx, but can be ineffective due to resistance

Common antiinfective agents: Penicillins, sulfonamide (incl. trimethoprim and sulfisoxazole in combination), cephalosporins, and nitrofurantoin

If anatomic defects (primary reflux or bladder neck obstruction): surgical correction to prevent recurrent infection
- Follow up study important, since relapse rate is high and infection tends to recur 1-2 months after tx termination
- Aim of therapy and careful f/u: reduce chance of renal scarring
Urinary Tract Infection - Therapeutic Management: Vesicouretal Reflux
Recurrent infection of urinary bladder predisposes individual to transient eps of VUR

Abnormal retrograde flow of bladder urine to ureters
- During voiding, urine swept up the ureters and flows back into the empty bladder, acting as a reservoir for bacterial growth until next void
- Primary reflux: congenitally abnormal insertion of ureters into the bladder
- Secondary: result of acquired condition

Not clear that reflux necessarily causes infections
- More likely associated with recurring kidney infections than simple bladder infections (cystitis)
- In the presence of reflux: infected urine from bladder has access to kidney, resulting in pyelonephritis
- Symptoms: high fever, vomiting, chills

Reflux (when associated with UTI) most common cause of renal scarring
- may occur with first ep of febrile UTI
- No renal damage in presence of sterile urine

* Most important concept: preventing bacteria from reaching kidneys
- managed conservatively with daily low-dose abx therapy.
- urine culture every 2-3 months and any time child has a fever
- This method requires motivated, reliable and cooperative family.

Many children outgrow reflux over a period of years
- Annual VCUG done to assess status

For children with mild-moderate reflux: minimally invasive endoscopic option (subtrigonal injection/STING) is an alternative to daily abx or open surgical intervention
- Bulking agent (dextranomer-hyaluronic acid polymer [Deflux]) injected into mucous membrane of ureter, making retrograde flow more difficult
- Overall cure rates relate to degree of reflux and range from 72% to 84%

Indications for open surgical intervention: significant anatomic abnormality at the ureterovesical junction, recurrent UTIs, severe VUR, noncompliance with med. therapy, abx intolerance, VUR after puberty in females
Urinary Tract Infection - Therapeutic Management: Prognosis
Excellent for long-term with prompt & adequate tx at the time of dx.

Hazard for progressive renal injury is greatest when infection occurs in young children (especially younger than 2 years of age) and is associated with congenital renal malformation and reflux
* Early dx is particularly important
Urinary Tract Infection - Nursing Care
Instruct parents to observe regularly for clues suggesting UTI
- signs not as evident as those of upper resp. tract infection
- many cases go undetected because no one thought to investigate

Unable to detect discomfort experienced from dysuria (due to difficulty expressing feelings and sensations)

Hx re: voiding habits, stool pattern, eps of unexplained irritability may assist in detecting less obvious UTI cases
- Parents should be cautioned to observe for specific clues of UTI in suspected cases

** Check diaper every half hour
- Inc. opportunity for observing stream for straining, fretting before voiding begins, discomfort signs before and during urination, starting and stopping stream intermittently, frequent dripping of small amounts of urine

When infection suspected: collect appropriate amount of specimen
- Nurse must take every precaution to obtain acceptable clean-voided specimens to avoid the use of other, more invasive collecting procedures expect when absolutely indicated
- Because of unreliability of urine collection bag: suprapubic aspiration or sterile cath should be done for infant or young child with fever

Additional tests for anatomic defects; prepare children as age appropriate
- Explain procedure, purpose and what child will experience
- Simple description of urinary system
- Especially for preschool children: clarify urinary tract is separate from any sexual function and test is for a problem they didn't cause
* Children may associate blame for perceived wrongdoing (masturbation) or unacceptable thoughts with the reason for illness or tests
- Children younger than 3-4 years: explained on a doll
- Older children: simple drawing of bladder, urethra, ureters and kidneys helps

Handling actual equipment also helps in allaying anxiety in children
- Anticipatory instruction on distraction to help relax: deep breathing, storytelling, imaging

If surgery indicated: facts and understanding procedure helps to dec. fear and anxiety concerning more extensive intervention

Advise parents of proper abx dose and admin.

Antiseptics (nitrofurantoin) used for prolonged therapy to maintain urine sterility: explain continued necessity when no signs of infection present

Encourage adequate or inc. fluid intake
Urinary Tract Infection - Nursing Care: Prevention
Most important goal in primary and recurrent infections
- Most preventive: simple hygienic habits that should be routine
-- Teach parents to cleanse infant's genital areas from front to back to avoid contaminating urethral area with fecal organisms
-- Girls taught to wipe from front to back after void or defecate
-- Children should void as soon as they feel the urge

Sexually active adolescents advised to urinate as soon as possible after intercourse to flush out bacteria introduced during sex.

Children with recurrent UTIs or neurogenic bladder are frequently maintained on daily low-dose abx
- Give dose at bedtime allows drug to remain in bladder overnight

Reinforce importance of compliance to parents and older children
Urinary Tract Infection - Prevention of UTI
Factors predisposing to development
- Short female urethra close to vagina or anus
- Incomplete emptying (reflux) and bladder overdistension
- Concentrated urine
- Constipation

Measures of prevention
- Practice perineal hygiene: wipe front to back
- Avoid tight clothing or diapers; wear cotton panties than nylon
- Check for vaginitis or pinworms (especially if child scratches between legs)
- Avoid holding urine; encourage child to void frequently, especially before long trips or other circumstances in which toilet facilities are not available
- Empty bladder completely with each void; have child "double void (void, wait a few minutes, then void again)"; severe cases may require CIC or biofeedback instruction

Avoid straining during defecation, avoid constipation

Encourage generous fluid intake
Urinary Tract Disorders/Disease - Clinical Manifestations
Neonatal (Birth to 1 month)
- Poor feeding
- Vomiting
- Failure to gain weight
- Rapid respiration (acidosis)
- Resp. distress
- Spontaneous pneumothorax or pneumomediastinum
- Frequent urination
- Screaming on urination
- Poor urine stream
- Jaundice
- Seizures
- Dehydration
- Other anomalies/stigmata
- Enlarged kidneys or bladder

Infancy (1-24 months)
- Poor feeding
- Vomiting
- Failure to gain weight
- Excessive thirst
- Frequent urination
- Straining or screaming on urination
- Foul-smelling urine
- Pallor
- Fever
- Persistent diaper rash
- Seizures (with or without fever)
- Dehydration
- Enlarged kidneys or bladder

Childhood (2-14 years)
- Poor appetitie
- Vomiting
- Growth failure
- Excessive thirst
- Enuresis, incontinence, frequent urination
- Painful urination
- Face swelling
- Seizures
- Pallor
- Fatigue
- Blood in urine
- Abd or back pain
- Edema
- HTN
- Tetany
Lab Values - CBC
RBC: reflects bone marrow function; indirectly estimates hemoglobin content
- Average: 4.5 - 5.5 million/mm3

Hemoglobin
- Average: 11.5-15.5 g/dL

Hematocrit: approx 3x Hgb, also indirectly measures Hgb
- Average: 35-45%

Mean Corpuscular Volume: average size of single RBC
- Average: 77-95 femtoliters