HEHI2 exam 4/final exam Spring 2017

Spastic CP

SPASTIC- most common (70-80%) characterized by *Hypertonicity, impaired motor skills (both fine and gross) and abnormal posturing* • *Hypertonicity*: characterized by persistent primitive reflexes (moro, babinski, grasp, rooting, trunkal incurvation, asymetric tonic neck [sword fighting]); increased muscle tone; development of contractures. • *Early manifestation is generalized hypOtonicity/decreased muscle tone lasting months-1 year*= floppy baby, don't have good muscle tone • *Impaired motor skills* both fine and gross motor Delayed gross motor development is the universal manifestation of CP. • *Abnormal posturing*: at rest or with position changes; poor control of balance and coordination

Folic Acid

AAP (American Academy of Pediatrics) pregnant women or women who may become pregnant -*rec. daily intake- 0.4 mg [400mcg]*- -Pre-conceptual folate supplement may decrease incidence of NTD by up to 50-70% *Previous history of NTD- Folic acid dose should increase to 4.0 mg*- under supervision of a provider - *1 month before pregnancy through 1st trimester* - Under provider supervision! (cereal is now supplemented with folic acid) FDA 1998- fortified cereals with folate - AAP guidelines: 400 mcg supplied in many MVI - Previous NTD: 4.0 mg under practitioner supervision. Don't do this thru lots of MVI b/c have too much of other vits too. For instance too much vit A can lead to fissure, impaired bone formation, defective tooth enamel

General Health Maintenance/ we advocate for with Down Syndrome:

Accurate information - Cannot predict the child's intellectual capacity at birth Frequent well child visits *Immunizations as scheduled* Monitoring physical growth - *special growth charts developed for Down's* Cognitive vs psychosocial intelligence

Tracheoesophageal Fistula (TE fistula)/Esophageal Atresia

Presentation: *TRIAD*: of *EXCESSIVE SECRETIONS, REFLUX, AND RESPIRATORY DISTRESS* (also common = FROTHY SALIVA) *Failure of trachea to differentiate and separate from esophagus into separate distinct structures* Prognosis 100% in otherwise healthy kids *Failure of espohagus to develop as a continuous passage* *diagnosed by x ray after passing radiopaque catheter into esophagus to take an xray and see the blind pouch* TEF/ Esophageal Atresia Incidence/Etiology (FYI): • 1 in 4,000 live births • 30-40% of affected individuals have *assoc. anomalie*s (look elsewhere for other anomolies) • *No definitive cause* identified • *Male= Female* • Increase in *low birth weight* • Increase in *prematurity* • Polyhydramnios Four different presentations/types: (middle = most common/ proximal esophagus is a blind pouch/dead end, lower esophagus is attached to trachea or bronchus via fistula. Not consistent with life, needs to be treated immediately) TEF/Esophageal Atresia Clinical Manifestations: • *Inability to swallow saliva* - increase in saliva/drooling • Oral gastric tube/suction catheter cannot be passed • *Choking or cyanosis with feedings*- if wasn't diagnosed prior to feeding • Recurrent Pneumonia • *TRIAD OF EXCESSIVE SECRETIONS, REFLUX, AND RESPIRATORY DISTRESS* • *FROTHY SALIVA in mouth and/or nose* • May become cyanotic or apneic secondary to aspiration TEF/ Esophageal Atresia Diagnosis: • Ideal to diagnosis before feeding - Clincal s/s: know these: *Triad of excessive secretions, reflux, and respiratory distress* - and frothy saliva *DIAGNOSED BY X RAY AFTER PASSING RADIOPAQUE CATHETER INTO ESOPHAGUS TO TAKE AN XRAY AND SEE THE BLIND POUCH* TEF/Esophageal Atresia Therapeutic Management: • *Surgical Correction*, must be stable to go to surgery • Sometimes requires staged repair • *Prognosis- 100% in otherwise healthy children* • Some post op complications can occur • Surgeries may be done in stages. Multiple TEF/Esophageal Atresia Nursing Interventions: preoperative: • Assessment- know s/s, what to look for • *Position: HOB elevated* to *decrease aspiration* risk • Frequent *suctioning*: to keep blind pouch empty • NPO (*DO NOT FEED pt's preop*eratively, *IVF for hydration* • I & O • Gastric decompression - Catheter in esophageal blind pouch to low wall suction, GTube inserted and open to drain TEF/Esophageal Atresia Nursing Interventions: postoperative: • *Airway management* • *NPO postop* • Thermoregulation • *Gastric decompression* even after the repair • *Suction*: prn *with extreme caution* • Chest tube • Pain control • Comfort • Feeds - *Gtube feeds* started slowly - *Oral feeds started slowly after healing from surgery* - Feeding difficulties are common: strictures/narrowing of trach. monitor Growth a d development Psychosocial Implications- expectations are not necessarily going to be what they once were for the child Family Support

Bones

• Function of MS system: Mobility, support/protection of soft tissues, *calcium and phosphate/phosphorus storage*, bone marrow storage • Density: mass of mineral in bone tissue = strength of bone (*DEXA scan*) • *Endocrine (estrogen) effects on bone*: increase and decrease resorption and formation (Low estrogen= weak bones/osteoporosis) (Adequate estrogen promotes osteoblast activity (bone building) • *Calcitonin* and *parathyroid hormone*- *control the release and uptake of calcium and phosphorus in the bone* (when there are problems with bones it will effect blood levels of calcium and phosphorus) • Muscles - movement, position maintenance • Tendons - *connect muscle to bone* • Ligaments - *connect bone to bone* Musculoskeletal Assessment (which population is *most at risk* for musculoskeletal system? *Old white ladies* (*post menopausal decrease in estrogen*) Nursing History • Nutrition- *calcium* and *vitamin d* to *promote good bone health*. Maintaining healthy weight • Family history/*Genetic risk* family hx of *autosomal dominant disease of the musculosceletal system*? Hx of *osteoporosis*? Ethnicity: *Chinese americans and european americans are more at risk* for fracture than black (women more than men) (*African americans have greater bone density* than chinese or european americans. Chinese and europeans more likely to have loss of bone, osteoproosis and fracture than black). *Obesity is risk for fractures. Greater strain* for longer periods of time. Athletes: trauma risk for MS system • Current musculoskeletal history- what is patients lifestyle? Too much activity, repetitive activity/stress/athlete increases risk for injury, *sedentary lifestyle can lead to loss of bone density*, obesity and loss of muscle (*atrophy*). Appropriate activity to *maintain bone health is weight baring exercise*. • Comorbidities that contribute to musculoskeletal system- obesity, *thyroid disorder (because endocrine involvement in bone structure/regulating calcium in the bone*), *diabetes*- at higher *risk of osteomylitis (bone infection) due to slow healing* of wounds caused by decreased blood flow/oxygenation/perfusion to wound and decreased nerve function/ peripheral neuropothy (may not feel wound) (slow healing wounds, especially around joints leave those wounds suseptible to bacteria/infection) • Physical Exam- if pt has abnormal posture of gait, this is clue that something is going on within musculoskeletal system • Posture and gait- clue something is going on with muscle or bones • Muscular structure- often see kyphosis, symetrical? Atrophy? Health Maintenance, and Promotion: • Protect and promote safe mobility • Understand how components of musculoskeletal system function • Active, but not over-active lifestyle • Nutrition • Non-*smoking*- impedes oxygenation because damage to blood vessels over time-> impact the musculoskeletal system. The bones are innervated with blood vessels, anything that effects blood vessels will effect bone • Appropriate assist devices- educate pt's about proper use of assist devices so they don't cause more harm than good

Musculoskeletal Trauma and Emergencies

• Sprains and Strains (in text) • Fractures (can be caused by trauma or pathologic) • Compartment Syndrome • Fat Embolism • DVT/PE • Carpal Tunnel Syndrome

*Classification of Intellectual Disability* --- KNOW

*Classification of Intellectual Disability* ---KNOW *Mild*: IQ of 50-70 Often *not noticed by the casual observer*; slow to walk and talk *Moderate*: IQ of 40-50 *Noticeable delays in motor development and skills* *Severe*: IQ of 20-40 pronounced delays with *few communication skills* *Profound*: IQ of less than 20 requires total care

Cleft Lip/Palate (the 2 most common cranial/facial deformations) Definition

*Cleft Lip- congenital fissure in the *upper lip*. Can be anything from a notch to through the nares/nostril. Can be unilateral or bilateral. *Can have isolated cleft palate, isolated cleft lip or have cleft palate and cleft lip* *Cleft Palate*- congenital fissure in either the soft palate alone or in both hard and soft palates *Cleft Lip* with or without cleft palate -*Most common* craniofacial malformation Incidence: *males*> females *Isolated Cleft Palate* -Incidence: *females*> males Cleft Lip/Palate *Pathophysiology*: incomplete fusion of the primitive oral cavity (*1st trimester*, often before a woman knows she's pregnant) -*Cleft lip - occurs at 6 wks gestation* when maxillary process fails to merge with the medial nasal elevation on one or both sides -*Cleft palate - occurs 7-12 wks gestation* when the lateral palatine processes fail to meet and fuse with each other, the primary palate, or the nasal septum Etiology: • *Multifactoral mode of inheritance* - 25% of CL/CP have *family history* of facial cleating • *Maternal nutrition*- esp. *folic acid* deficiency • *Maternal medication in 1st trimester* • Occurrence of *fever and influenza in 1st trimester* (give flu vaccine to pregnant clients) • *Maternal prenatal cigarette smoking* - Associated with 1.5 to 2 fold increase in risk for facial Clinical manifestations of cleft lip/palate: • Generally *evident at birth* • If not evident infant may *exhibit feeding difficulties*, chronic *upper airway congestion*/infection • Palpating palate part of newborn exam • 7-14% of infants with CL/CP have *other anomalies at birth* (many syndromes have cleft lip or pallate associated with them) Cleft lip/palate therapeutic management: • Requires *team approach* - Nursing, pediatrics, plastic surgery, dentist, orthodontist, speech therapy, audiology, social work, child psych, genetics counselor • Surgical repair priority with *goals of normal speech (repair before speech development)*, normal palate/facial growth, normal dentition(teeth), nutrition/feeding, and appearance to promote appearance that is pleasing to family and pt later on. - *Timing of repair: Cleft lip EARLY closure 10 wks or 10 lbs.*, and *6 months later for Cleft Palate* (before language) (cleft *palate repair is more complicated* procedure) Preoperative Nursing Interventions Nursing diagnsis: Nutrition less than body requirements r/t *inability to suck* s/t CL/CP *Elevate head of bed during feeds* Utilize *special nipples* (length of nipple is longer)(shorter nipples don't allow these babies to generate a suck/good latch) *Gentle steady pressure on base of the bottle* Frequent burping *Daily weight for nutritional evaluation* as well as *fluid volume status* *Breast feeding: position nipple well back into oral cavity* *Special equipment*: Breck feeder: puts formula/breast milk in mouth without sucking (sucking is important for oral muscular development) Cleft lip/palate nursing interventions: • Implementation: Preoperative - *Feeding difficulty*: grasping nipple and creating a suction. Requires patience and *special techniques/ equipment* used for sucking/nipples-> *higher flow and less suction needed to eat/longer nipples (breck feeder) raise HOB to (to decrease aspiration)* - (*sucking reflex* very *important*, important *for oral muscular development & speech*. Have to get suck needs met. Maybe use *football hold for breast feeding* Cleft lip/palate *postop*erative nursing interventions: • *Maintain patent airway*: elevate *HOB* (to decrease aspiration) • Assess frequently: infection, bleeding (airway compromise risk), resp. distress • *Protect suture line: put nothing in mouth after surgery/don't put anything in mouth (cant breast or bottle feed right after surgery)(oral syringe food in*= Breck feeder) • Keep infant calm- crying puts stress on surgical site • *Use elbow restraints: double no-no to protect the suture line* • Support parents by accepting and treating infant as normal (culture, spirituality beliefs about clefts) • *Fluid volume maintenance: IV Fluids and/or PO. Transfer to PO as soon as possible* • Diet: Start with clear liquids, advance as tolerated • Suture care • Discharge education- infection s/s, talk to dentist about future repairs, keep speech therapy involved, feeding goals. cleft lip/palate *prognosis*: • Language and speech - *Residual speech impairment* - Speech therapy- frequent visits • Hearing impairment - *Frequent otitis media*- dysfunction of eustachian tube (links nasopharynx to middle ear) inability to drain - Early *Hearing screens* because there may be residual hearing issues • Dental problems • Social adjustment and self image- help parents and child with image issues Cleft lip/palate psychosocial implications: • Parental Guilt/Grief - allow parents to trust us as nurse. Parents may feel they did something wrong or are afraid kid will be made fun of, maybe they smoked • Bonding can be an issue • Cultural Beliefs- some cultures see cranial facial deformity as a gift/honor or believed to based on parent sin • Acceptance/Reaction of Others

Prognosis - Hydrocephaly

*Untreated- 50%-60% mortality* - survivors have high incidence of subnormal intellectual capacity (significant cognitive impairment) *Surgical treatment- 90% survival* - *2/3 are intellectually normal*

Anencephaly

*Both cerebral hemispheres absent* *Incompatible with life* - Many are born stillborn or die soon after birth due to respiratory failure - If born alive, given comfort measures and made DNR

system Concerns with Down Syndrome

*Cardiac*: *Altered tissue perfusion r/t cardiac issues (Present ~ 50%) Especially septal defects* (ventricular and/or atrial septal defects) *Respiratory concerns*: Impaired gas exchange r/t... - Susceptibility to *infections*- decreased respiratory expansion due to *poor muscle tone* so there is stagnant secretions - Compromised respiratory expansion - Secondary to decreased muscle tone - Acute and chronic obstructions - Secondary to underdeveloped nasal bridge leading to chronic problem (a lot of *upper respiratory infection*, decreased ability to clear infection mucus) of inadequate drainage of mucus. They can get a lower resp. infection from the upper resp. infection (it was the *cardiac issues and respiratory concerns that used to lead to shorter life spans*) GI Concerns: Alteration in nutrition r/t... • Postnatal - feeding intolerance, vomiting, failure to thrive - *Feeding difficulty secondary to large tongue and hypotonia* - *Tongue thrust does not indicate refusal to feed (tongue thrust is part of who downs babies are. Babies without downs with tongue thrust when they are done feeding. Downs babies tongue thrust all the time)* - With tongue thrust, refeed - Constipation is common - Obesity is common GU Concerns: Alteration in urinary elimination r/t... • 2% males have *hypospadias* - An abnormal opening of the male urethra upon the undersurface of the penis • 25-50% have *undescended testes* Hematologic Concerns: • Risk for *leukemia* is 10-30x general population common • Lower remission rate, higher early mortality than general population Alterations in visual/auditory perceptions: • *Visual Acuity*-50% (get visual screening early); refractive errors, 60% strabismus (eye deviation) - *initial VISUAL screening by 18 mos* • Hearing loss, mostly conductive - *initial HEARING screening by 6 mos* - Experience *frequent otitis media* (ear infection), have narrow canals, and impacted cerumen (ear wax) that contribute to the *hearing loss* Orthopedic concerns: • *Subluxations, dislocations of hips common* r/t muscle tone, ligamentous laxity • *Atlantoaxial instability* (17%) - Cervical spine has lax muscles and ligaments that can lead to spinal cord damage if forcefully flexed or extended - *Screen by 2nd birthday* (all kids that are going to participate in special olympics have to have screening for this) if screening comes back positive, they *can still be active, just monitor activities* - *Surgery may be required* - *Avoid activities that stress head and neck* (no head first tumbling in gymnastics, not diving in the pool) Endocrinologic Concerns:/ Altered growth and development • *Deficient growth in infancy, adolescence* - Utilize special growth charts • *Thyroid dysfunction* (hypothyroid) (high chance) - Periodic testing, esp. if growth is delayed • *Fertility in males is rare* (still have a clear code of conduct for behavior/ sexual interactions) - *Male genitalia is often underdeveloped* • *Ovulation present in most females* (40-70%) (still have a clear code of conduct for behavior/ sexual interactions) - Menstruation at average age - Reproduction is possible Neurologic Concerns: Risk for injury r/t... • Diminished muscle tone • Seizures (10%) • *Alzheimer's (by 51 yrs/early onset)* • Cognitive - mild to moderate (IQ from 40-70) - expressive language disabilities - *strength seen in social ability*

Compartment Syndrome

(muscles, bones, etc are in separate compartments, so if there is injury to one compartment the blood shouldn't flow to all compartments unless there is a rip between compartments)- *blood would fill a injured compartment until pressure too much, bleeding would stop, lead to necrosis/tissue death* *ORTHOPEDIC EMERGENCY!* Pressure within soft tissues in fixed body compartment Increases to level that exceeds venous pressure Results in compromised venous blood flow and limited capillary perfusion Causes muscle ischemia and necrosis Recognize/Assess/Respond: • *Long bone fractures, crush injuries highest risk* • *Pain out of proportion with injury* • With or without impaired sensation (tingling or numbness )(maybe have discoloration due to lack of blood flow) • *Pulselessness (late sign)- potential for lasting damage resulting from this* • Pt may complain of *muscle feeling tight* Compartment syndrome tx: Tx: *fasciotomy* - cutting down the compartment to relieve the pressure

Clinical Manifestations: of bacterial Meningitis *children/adolescents*

*Abrupt* or *gradual onset*: - *fever, chills headache, vomiting*, (ask good questions, history, presentation etc. because the prior s/s are common to many conditons) change in sensorium (*cognitive processing changing*), *irritable, agitated, photophobia (light sensitivity), confusion, hallucinations, drowsiness, stupor, coma* - *Seizure* can be the initial manifestation (if someone comes in with a seizure, we have to figure out if its menigitis) *Nuchal (neck) rigidity*- pain when putting chin to chest. Accompanied with other s/s of infection *Kernig sign*- lay child flat on the bed take the child's knee to a 90 degree angle, then straighten leg-> + sign = *hamstring pain* - Flex thigh on body and extend leg - Positive sign: Resistance and pain in hamstring muscles *Brudzinski sign*- positive - With pt supine/flat on bed, flex/raise head - Positive sign: pain - or hips and knees flex up involuntarily Meningococcal-*purpuric/petechial rash - classic sign, petichial rash*

An acute scrotum

*Acute painful swollen testicle in the child (emergency)* Tends to be *unilateral*, requires immediate diagnosis, typically requires *urgent surgery* Future *fertility is impacted*, needs to be treated ASAP, *sudden severe onset of pain without predisposing event*, usually unilateral, nausea and vomiting often present*. Causes: surgical emergencies included *torsion of the testicle* (twisting of the cord)(sudden onset of acute pain), *incarcerated hernia* This is an urgent finding. Requires immediate diagnosis, and possible surgical intervention. Mis- dx or delay can lead to loss of testicle. This was epidiymisis In the prepubertal male, the physician must differentiate among the following: torsion of the testicle, torsion of the appendage of the testicle, orchitis (sometimes due to Henoch-Schönlein purpura or Kawasaki disease), hydrocele with an incarcerated hernia, traumatic hydrocele or epididymitis. Of these, an incarcerated hernia and full torsion of the testicle, which is in many series the most common diagnosis of acute scrotum, are medical emergencies. And surgery must be performed immediately to salvage the torsed testicle or incarcerated bowel, respectively. - *Testicular torsion*: Testicular torsion is the twisting of the spermatic cord, which cuts off the blood supply to the testicle and surrounding structures within the scrotum. - Symptoms: Sudden onset of severe pain in one testicle, with or without a previous predisposing event (one testicle is red and painful) - Swelling within one side of the scrotum (scrotal swelling) - Nausea or vomiting - Testicular torsion is a twisting of the spermatic cord that cuts off the blood supply to the testicle. The signs are an enlarged, reddened and tender scrotum (the bag of skin hanging behind the penis)(does rogers not think we know what a scrotum is? and why does she have to put it this way "bag of skin hanging") and, in older boys and young men, abdominal or testicular pain and nausea/vomiting. This is a medical emergency

Clinical Manifestations of hydrocephaly in a Child (non infant)

*Clinical Manifestations in a Child* (not infant) (maybe peritoneal shunt isnt working as it is supposed to): -Look for s/s of increased intracranial pressure: ICP- irritable, lethargic, confused, apathetic, *cushings triad* increased BP, decreased heart rate, widening pulse pressure, RR decreased or irregular -Headache on awakening that improves after emesis of sitting up (relieves some of the pressure) -Change in cognitive/school performance -Ataxia- impaired gait/balance (cant feel fontinel on kid over 18 months as it is already closed) (s/s increased ICP- often assoc with space occupying lesion)

Neural tube

*Closes between 15-28 days post conception fetal development* Failure of the normal closure of the neural tube = neural tube defect (women often considers she may be pregnant ~15-28 days after conception. When a neural tube is closing= when a woman often doesn't know she is pregnant. In the 4th week the neural plate arises, it envaginates to form the neural groove, then we have complete closure of the neural tube. This is when neural tube defects occur (within the first 28 days) *90% of NTD are spina bifida and anencephaly* 1 in 5000 live births

Hydrocephalus (2 kinds)

*Communicating*- impaired absorption *Non-communicating*- impaired/ obstructed flow of CSF through ventricle system We care for them the same way -Communicating: impaired absorbtion of CSF within subarachnoid area -Non-communicating: cant get to the sub arachnoid space for absorption -If too much CSF- pressure compresses abnl parts of brain even further - any ↑pressure will damage brain Roger's notes: -Clinical manifestations: also s/s ↑ICP -Fontanel: ant fontanel tense and bulging, when does ant font close? 18 months -Dilated veins: esp when crying -Frontal bossing: see pic- frontal protrusion -Sunset eyes: eyes rotated downward, sclera visible above iris -Paradoxical crying: crying when picked up and quiet when left alone -Cushing's triad: ↑systolic BP, widened pulse pressure, bradycardia, abnl resp pattern *Cushing's triad* (not to be confused with the Cushing reflex) is a sign of increased intracranial pressure. It is the triad of: 1. *Hypertension* (progressively increasing systolic blood pressure) 2. *Bradycardia* 3. *Widening pulse pressure* (an increase in the difference between systolic and diastolic pressure over time) Cushing's triad suggests a cerebral hemorrhage in the setting of trauma or an space occupying lesion (e.g. brain tumor) that is growing and a possible impending fatal herniation of the brain Diagnosis: -Antenatal- fetal ultrasound- 14 weeks -Increasing Head circumference -Associated neurologic signs -Primary diagnostic tools- *CT and MRI* -Increasing HC: crosses 1 or more grid lines in 2-4 wks

Cushing's triad

*Cushing's Triad*: (can lead to ICP and brain bleed) 1) *Increased BP/HTN* (leads to hr down), 2) *bradycardia*, 3) *widened pulse pressure*, abnormal or decreased RR. Cushing's triad= sign of increased ICP, suggests a cerebral hemorrhage in the setting of trauma or an space occupying lesion (e.g. brain tumor) that is growing and a possible impending fatal herniation of the brain

Congenital Diaphragmatic Hernia

*Diaphragm not completely formed* Allowing *intestines and/or organs to migrate up and enter thoracic cavity* (*left chest cavity*) - *Restricted lung capacity due to lung having to compete for space* - *Abdomen will look scaphoid* because baby developed with abdominal contents in left chest. *Left lung will be underdeveloped* bc had to compete for space Manifestation: *acute respiratory distress at birth* *Pre-natal diagnosis ideal via ultrasound* - see loops of bowel outside of abdominal cavity *Post- natal diagnosis- can have restricted to arrested lung development on the effected side* (*left*)-> *acute resp distress at birth* -> *dyspneic* (trouble breathing) or *cyanotic*-> *raid progression of illness*/sick very quickly CDH- Management: *Post birth* • *Respiratory support*- *NO BAG MASK VENTILATION/ (NO AMBU)*(could lead to increased air into the abdomen, further decreasing lung capacity), *O2 by hood or by intubation* • *GI decompression via NGT* • *HOB elevated* • *Surgical repair*: early repair to return abdominal contents to abdomen and repair defect CDH- Nursing Interventions Newborn assessment: child will be in acute resp distress *that doesn't respond to typical resuscitation*. Needs to be intubated to prevent further intestinal compromise (don't want to get air in the loops of bowel/ don't want to ambu bag) Post-operative: sick babies, tpn and lipids for nutrition, iv fluids for fluid management.

Review- Meninges: protective membrane covering brain and skull

*Dura mater* - Tough outer layer- fibrous, stabilizes CNS within skull - External meninges which fuses with inner surface of vertebrae and inside of skull *Arachnoid* - Middle layer- contains CSF, arteries, veins, cranial nerve root. - Involved with normal flow of CSF (flow covers the brain and spinal cord in continuous pathway that is about protecting brain and spinal cord. In the absence of some defect within the CNS, CSF is about cushening and protecting these delicate structures. We produce and absorb csf in a balance right for out CNS. *Pia mater* - Inner delicate membrane on the brain. - Internal meninges- helps direct blood flow into brain

Epispadias and bladder exstrophy

*Epispadias: Defect on dorsal surface of penis (*chance of UTI and upper renal infections high*) *Bladder exstrophy*: severe defect with *externalization of the bladder* - Seen in males and females - In Males: almost always seen with epispadias - *Exstrophy almost always assoc with epispadias*. Can include other issues: undescended testes, inguinal hernias. Failue of abdominal wall to fuse in utero. Lower urinary tract is exposed - *NICU care, sterile non adherent dressings over the exposed bladder*, fluid volume management - Upper urinary tract is WNL. Fertility possible in females, complex issue in males. - Left untreated leads to kidney infection that migrates up to cause upper renal damage leading to failure - Genitals affected in males and females - *Immediate care*: NICU! - meticulous care of exposed bladder. *Sterile nonadherent moist dsg over bladder* - *Fluid volume management* Therapeutic management: • *Surgical correction early* - Can be performed in one stage repair, may require multiple stages - NICU care, sterile non adherent dressings over the exposed bladder, fluid volume management - Bladder closed during neonatal period •*Post op* - Monitor UOP - s/s UTI, wound infection Second stage: repair of epispadias and creation of urethral sphincter *Surgical correction With multiple stages: child wont have urinary control . Will have free flow of urine until second stage. Education involves parent education re: s/s UTI*

Spina bifida Cystica (has sac like protrusion) (meningocele and myelomeningocele)

*Visible defect with saclike protrusion* *Meningocele*= rare, *contains meninges and CSF only* *Myelomeningocele- most common SB, contains meninges, CSF (cerebral spinal fluid), spinal cord, and/or nerve roots* -Visible: protruding thru defect in vertebral arch -Meningocele: not associated with neurological defect -Myelomeningocele: most common SB Accounts for 90% of spinal cord lesions

Etiology of Down Syndrome

*Extra 21st chromosome* (cause unknown) Translocation or mosaicism *Statistically there is an increased incidence in Down's born to older women, but 80% of Down's are born to women < 35 years of age* - Mom who is 30 yrs of age: 1 in 952 live births - Mom who is 35 yrs of age: 1 in 350 live births - Mom who is 40 yrs of age: 1 in 100 live births Incidence ~ 1 in 800 live births 1965 - 50% died by age 5 1995 - 85% live longer than 30 years *The life expectancy has improved, but remains lower than the general population. Current lifespan average is 55 years.* ~ 5,000 children born each year in US Bright prospects for downs: - Family life - Better access to quality health care - Better early intervention programs (sometimes when a family chooses faith based preK they give up rights to PT, OT - Growing awareness and acceptance - All children with disability have the right to free and appropriate education in the least restrictive environment and an individualized education plan - By age 3- there are guaranteed educational services available under the Individuals with Disabilities Education Act. (preschool, OT, PT etc) - Many states have free early intervention from birth to 3 yrs of age

Cryptorchidism

*Failure of one or both testes to descend normally into scrotum* (non painful, not emergency) *watch for ~1 year to see if it resolves itself* Incidence: up to 45% in *preterm* boys, less than 5% in term boys (*fetal development: testes are abdominal organs until ~ 25-30 weeks gestation when they descend into the scrotum. If the baby is born early, this may not have happened yet*) Vs. Retractile testes: cremasteric reflex is active in infancy (where the testicles, when they are cold will retract up higher) WNL for infants thru 5 yrs Rogers' notes from last slide - Remember descent of the testes occurs beginnign 25-30 weeks (descent is complete ~36 weeks ega usually) - In term boys, can have endocrine abnl too - Assessment tech to differentiate cryptorchidism and cremasteric : apply firm finger pressure on the external ring before palpating genitalia - Have to determine if crytporchid, anorchid, ectopic - Retractile testes: WNL for infants thru 5 yrs. Overactive cremasteric reflex that causes withdrawl of testes above the scrotum and into inguinal canal in response to stimuli- cold. Can be gently moved into scrotum. Doesn't require treatment. If retracitle, not intervention needed. By 1 yr, 75% of retractile descend (both term and preterm) *Therapeutic management of Cryptorchidism* • *Orchiopexy between 1 and 2 years of age if the testis doesn't descend spontaneously* - Surgery to move an undescended testicle into the scrotum and permanently fix it there (increased risk of testicular cancer and decreased fertility IF NOT DONE) - This prevents overexposure to body heat that can damage undescended testicle. - Orchipexy: *maintains future fertility* - *Decrease incidence of cancer/malignancy* formation which is higher in undescended testicles (due to exposure to body heat when not in the scrotum). Avoid trauma and torsion. Psych issues of empty scrotum - Higher fertility with earlier surgery Nursing Care/ post op/ maintaining future fertility: - Prevention of infection- incisions made in inguinal folds, closed with dermabond (surgical super glue) - *Pain management- often Nsaids* - Parent education- prevention of infection because baby is usually still in diapers - hygiene - Try to Avoid rigorous activity for 2 weeks., limit pressure on incision

Nursing Care- *pre-op*erative shunt malfunction

*Freq assess for increased ICP*- irritable (*DO NOT SEDATE THIS CHILD/ DONT MASK IRRITABILITY*), vital sign changes, lethargic, seizures, decreased feeding, headache (if old enough), change in level of consciousness, vomiting-> feeling better *Feedings- small freq feeds if they are PO because we don't want them vomiting, because if they are vomiting we don't know if its ICP or something else* Ventricular tap if needed - Advanced practice required: MD or NP- to relieve excess pressure of CSF. Done by advanced -Daily HC, assess fontanel- bulging?, assess suture lines- separation? -Irritable- would you sedate this child? NO -Don't mask irritability!

Cerebral Palsy

*Group of nonprogressive* (not degenerative) *disorders of movement and posture* caused by *abnormal development of, or damage to, motor control centers of the brain*. (symptoms/manifestations don't all present at once, they are progressive, but the disorder/whatever caused the CP is not progressive) (*CP is non progressive although the manifestation may be progressive*) -This is *not a cognitive diagnosis* CP- Description: *Impaired Movement* -*Caused by abnormal functioning of cerebral cortex* - Non- progressive: though all the physical manifestations may not be present immediately - 1 in 500 live births - *The leading cause of disability in children* Etiology: - *Prenatal*: 70-80% of CP caused by unknown prenatal factors. *Majority are prenatal* with *unknown cause*. - Perinatal (during birth): *low % attributed to birth trauma* - Postnatal: infection (possibly meningitis), toxins (eg. increased bilirubin-> encephalopothy-> CP), traumatic brain injury, kernicterus - Prematurity: 50% of all cases of CP are in children born *PREMATURELY* = *single most important risk factor* (more premature you are, higher the chances) - More common in Multiple Births

Abdominal Wall Defects

*Hernias: A protrusion of organ through an abnormal opening*. Become *dangerous when constriction leads to impaired circulation* -Diaphragmatic -Umbilical -Gastroschisis -Omphalocele • *Occur in 3rd week gestation* • Approximately 2 per 10,000 live births • *Omphalacele*- herniation of abdominal contents *thru umbilical ring; with intact peritoneal sac*- more often associated w other anomolies. They vary in size. Reduction back into abdomen= surgery.the baby grew with intestinal contents outside of the intestinal cavity, so there isnt lot of room to put back in) Prognosis is ~70% due to other anomolies • *Gastroschisis*- herniation of abdominal contents *lateral to umbilical ring; no peritoneal sac*- vary in size, rarely associated to oter anomolies. Reduction back into abdomen= surgery. Prognosis 85% survival with repair UMBILICAL HERNIA • *Incomplete fusion of umbilical ring where umbilical vessels leave abdominal wall*: *Most common type of hernia*. (open defect, *not incarcerated*)Usually *resolve on their own* • Incidence: *African american*> Caucasians - Incidence: *low birth weight/premature*> full term - Usually an isolated defect • Complications: rare - *Surgery if still present at 2-3 yrs*, after that age it wouldn't resolve on own • Nursing interventions: reassure parents (don't tape the umbilical hernia down w coin, it can cause skin integrity issues/bowel issues). Reduce stress on surgical site if corrected with surgery Therapeutic Management: • *Ideally diagnosis was made in prenatal period, deliver via c section* as to avoid putting undue stress on the abdominal contents outside of the body • Cover sac with *sterile saline wrap* • *Supine posion* as not to put pressure on abdominal contents • IVF's and antibiotics - Prevent fluid loss (risk because organs are outside of body) • Surgery closure: contents reduced into abdomen and abdominal wall closed. May or may not be completes in one surgery • *Silo*: used when a staged repair is needed, putting abdominal contents back in slowly, *may effect respiratory status because pushing ondiaphram/lungs* • Thermoregulation Nursing Interventions: • Pre- operative - Infection risk - *Fluid management* - *NG tube to decompress* - Family support • Post- operative - Assess: - *Resp: secondary to increased abdominal pressure* - *Bowel function* (anticipate this child may have ilius/ decreased peristalsis) - TPN/ IL secondary to prolonged ileus

Hydrocephalus (hydrocephaly): Clinical Manifestations in Infancy:

*Hydrocephalus (hydrocephaly): Clinical Manifestations in Infancy:* -Bulging fontanel (bouncy/above the skull level) (due to ^ ICP) -Dilated scalp veins -Head growth at abnormal (fast) rate (must be plotted on growth curve) -Frontal bossing -*Setting sun eyes* (eyes that go down) -Irritable (more than fussy) (don't mask irritability with morphine/meds) -Lethargic (inability to arouse. more than sleepy/ beyond somnolescence) -*Poor feeder* (poor appetite) -Change in Level Of Consciousness -*Paradoxical crying* (crying when picked up) -High pitched cry (= neural cry)

Hirschsprung's Disease (Congenital Aganglionic Megacolon)

*absence of ganglion in a portion of the large intestines that leads to mega colon* (*usually near the end of the descending colon*). Condition-> *no ganglions/nerves, no peristalsis, cant sense all the crap, colon enlarges due to inadequate motility.* (this is an *OBSTRUCTIVE DISORDER*). 1st defined by Harold Hirschsprung in 1887 Definition- congenital anomaly that results in *mechanical obstruction* from *inadequate motility* in part of the intestine. Pathophysiology: Absence of autonomic parasympathetic ganglion cells (of the submucosal plexus of Meissner and the myenteric Auerbauch plexus) in one or more segments of the colon. Lack of innervation (no peristalsis) results in absence of propulsive movements *Lack of innervation also leads to decreased relaxation of the internal rectal sphincter. It stays tight, doesn't sense need to poop* Incidence: - Accounts for about ¼ of all cases of neonatal obstruction (1:5000 live births) - *Males*> females - *Only way to diagnose*= *biopsy* - you would *see absence of ganglion* Clinical manifestations: • Vary according to the age when symptoms present. • *Newborn* (41 to 64%)- *abdominal distension, vomiting, no meconium in 48 hours* (very sick baby) (*most present with symptoms during newborn period*) • *Older Infancy* (21-35%), *Childhood* ( 15-26%)- *Chronic constipation, failure to thrive, stools- foul smelling "ribbon like"/thin, abdominal distention* Therapeutic Management: *Stage I* : get *biopsies* from different parts of bowel, *determine if it is Hirschsprungs*, TX Primarily involves *surgical removal of the aganglionic bowel*. (if bowel has necrosized in parts, may be removing more than the aganglionic portions). Then give *bowe rest/ colostomy bag*. after about *8 months to year of bowel rest go to stage 2* *Stage II* : (*8 months-1 year or approximately 20 lbs)- Involves "pulling" the end of the intact bowel down to a part of the rectum for reanastomosis* *Stage III* : *closure of colostomy* (if performed) usually *3 months after stage II*. Baby will be able to *stool from below* as normal • Conservative Therapy (very small population with *mild symptoms*) (present with chronic constipation) - Occasional enemas - Develop regular pattern of defecation - At continued risk for development of enterocolitis because have a small portion of aganglionic bowel Nursing interventions: • Neonate- *observe for passage of meconium* (often delayed), these are sick babies • Beyond the neonatal period - get comfortable with getting good bowel history • Preoperative care - NG placement - Strict I & O - *Measurement of abdominal girth- good indicator of bowel health* - Vital signs • Postoperative care: - IV fluid maintenance - NGT - Dressing changes - Foley care - I&O's - Vital signs - Discharge education

Complications of VP shunt:

*Infection* *Most serious complicaton: Infection/sepsis* greatest risk= *1-2 months after placement*, may be sepsis or wound infection. (would be caused from contamination during the procedure) sick, vomiting (may be a GI bug but we need to make sure we don't have a shunt malfunction)(*anything that looks like increased ICP we have to assume its Icp until we rule it out*. Have to get a *"shunt series"* to check function of shunt.) *Signs and symptoms of shunt not working*: ICP -shunt malfunction (*increased ICP*), *fever* (= infection), *wound/shunt inflammation*, *abdominal pain*, (infection can look like a shunt malfunction) Risk for infection: but can be anytime! May be sepsis or wound infection *Treatment for VP shunt infection*: - *Ideal*: IV antibiotics for 7-10 days • *If infection not relieved with IV antibiotics then Often requires removal of shunt and placement of EVD-(extra ventricular drainage system* [managed on picu] device placed outside the body while infected internal shunt area heals from infection) - *Externalizes the shunt until infection is resolved* - Allows for removal of CSF to external collection device - Without this, when shunt is removed, child will experience increased ICP - *Chance of contamination and further infection are significant with EVD* - *Must keep baby and drainage system at the same level all the time.* - Once infection is clear, go back to surgery to put vp or va shunt back in EVD: allows for removal of CSF from a tube into the ventricle that goes to a collection device

Inflamatory response (Osteomyelitis)

*Inflamatory response (Osteomyelitis)* *Bacteria invades* (can be fungus or virus as well) triggers *inflamatory response* -> vascular leak-> edema-> initial site of infection. It becomes larger, pus develops -> absess. Blood flow becomes blocked and can lead to necrosis*. Initial infection can cause pressure and pain inside of the bone, maybe before any other symptoms. Fever may be the first symptom or fever + localized infection-> swelling, erythema, edema* Osteomyelitis (Infectious Bone Disorder) Definition/Diagnosis/Incidence • Infection of bone (s/s= *swelling, erythema, edema*) • Classified by- - *DURATION: ACUTE OR CHRONIC*- (*acute*- maybe someone who had an injury, someone had a cut close to their joint, someone. Or someone who was immobilized suddenly and develops pressure injury on their coxis) (*chronic* seen more in pt's with diabetes who have slow healing wounds and constant infectious cycle that it taking time to heal and is more chronic) - *LOCATION*, status of host, local environment: Stage 1-4 ( staged based on level of involvement/severity. *Stage 1 less severe than stage 4*. stage 1 patient may be able to go home with oral antibiotics and follow up xray. Stage 4 may need drainage of the site, IV antibiotics , etc.) - osteomyelitis seen in children: *vertebrae* (due to long term compression, reduced blood flow and necrosis of tissue-> inflamation) most common in adults (*in diabetes patient the infection will be in the joint closest to the nonhealing wound*) Etiology/Risk Factors: - *Direct (monomycrobial) or indirect (polymicrobial) inoculation of infection into bone* - Adults: trauma, surgery, vascular insufficiency r/t diabetes - Older adults: s/t pressure injury, total joint arthroplasty

Meningitis

*Inflammatory process of meninges and CSF*- issue because when brain cells die they don't regenerate *3 main types:* -bacterial (pyogenic) -viral (aseptic) -Tuberculous -*Potentially fatal*

Kidney Changes Associated with Aging

*Kidney Changes Associated with Aging* ● Reduced blood flow to kidneys ● Thickened glomerular and tubular basement membranes ● Decreased tubule length ● Decreased glomerular filtration rate ● Nocturnal polyuria ● Risk for dehydration

Diagnosis-CSF- Bacterial Meningitis

*Lumbar puncture- Definitive diagnostic test of menigitis* - *Spinal fluid pressure*: increased - *WBC*: elevated - *Protein concentration*: increased - *Glucose level*: decreased - *Color*: cloudy - *Gram stain*: positive

Prenatal Fetal Surgery Procedure pioneered at Vanderbilt in 1997

*MOMS trial- Management of Myelomeningocele Study (in utero surgery) (trying to repair myelomeningocele in utero: goal: decrease infection*) - Decreased need for shunting - At 30 months, children have increased motor functioning, increased ambulation, increased mental development Prognosis Care of this child = Multi- disciplinary approach (PT, OT, urology, ortho) *Normal intellect should be assumed in the absence of significant hydrocephalus* Secondary issues: psychological, social development, low self-esteem, lack of social skills

management of Hydrocephaly

*Management of hydrocephaly- Surgery:* • Shunt procedure (sterile)(surgically placed inside the body) -provides primary drainage of CSF from ventricles in the brain to a compartment outside of the brain (extracranial compartment)- usually peritoneum (but can be to heart) (shunts are performed when there is obstructed flow of CSF or impaired absorption) • *Ventriculoperitoneal shunt* (shunt to peritoneum)- VP shunt. In the body goes from behind the ear behind the neck and down to the peritoneal cavity to drain. There are valves in this that are sensitive to ICP, when the pressure gets too high the valves open and it drains, when ICP not too high the valves shut so it doesn't drain(CAN allow additional tubing for growth) •*Ventriculoatrial shunt* (shunt to right atrium of heart)- VA shunt (cant allow extra tubing for growth, comes displaced more frequently)

What is the only structure in the kidney that does not change with aging?

*Medulla*- the one kidney structure that does not change with aging process.

Hypospadias

*Urethral opening is located below the glans penis or anywhere along the ventral surface* Mild: meatus just below the tip of the penis (*more severe when it is farther away from where it is supposed to be*) Most severe: meatus on perineum *Chordee*- ventral curvature of penis accompanies severe form With severe, the foreskin is often absent on ventral , with chordee looks crooked. Can look something like female anatomy If we see a baby born with a hypospadias don't do a circumcision, *foreskin will be needed for correction* Care of Hypospadias: • Careful newborn assessment - Foreskin may be used in the repair. Often no circumcision • Surgical correction - Goals: Improve physical appearance of genitalia; Preserve sexually adequate organ; enhance ability to void standing with straight stream. Use foreskin for surgical correction. Could require multiple surgeries • Parental support and education *Can be accomplished with 1 or multiple surgeries depending on severity* (*surgery wont be immediate most of the time, unless it is severe or increased risk of UTI, constant dribbling*)

Myelomengocele

*Myelomeningocele- most common SB, contains meninges, CSF (cerebral spinal fluid), spinal cord, and/or nerve roots* *Degree of neuro impairment- at defect and below* (usually @ lumbar and sacral). *Usually loss of sensation and complete* (usually)/partial paralysis* - Neuro: Location and extent of defect determines nature and extent of neuro deficit. - L2 and above wheelchair, L2-5 crutches, lower than S3 no motor impairment, may need ankle bracing. *Hydrocephalus- most frequent anomaly associated with spina bifida*-monitor for signs and symptoms of *increased intra cranial pressure* (ICP) (ICP *can result from closing/ surgical correction of the neural tube defect*) (*80-85% of kinds with spina bifida will develop hydrocephalus*) - May not be apparent until after birth and after primary closure of the defect/opening - *Bladder: decrease nerve supply to bladder affects both sphincter and depressor muscles. Can cause constant dribbling or lead to overflow incontinence.* (cant feel when bladder is full, bladder will fill to capacity and leak, constant dribbling) (maybe tx= metrofinoff= urinary catheter (in and out)diversion for patient with no awareness or no tone to bladder) *Anal sphincter tone: lack of bowel control (rectal prolapse, chronic constipation)- (teach them to have routine bowel habits) (MACE tx= irrigate from above and stool from below)* - Anal: lack of bowel control, and rectal prolapse *Orthopedic abnormalities such as dislocated hips and club feet more likely.* - Ortho" b/c muscles to LE are denervated, produce joint deformities in utero. Dislocated hips and club feet common *Spinal deformities such as scoliosis or kyphosis* (often repaired at an earlier because impacts respiratory excursion, pain or ability to be in their chair appropriately

Diagnostic Evaluation for CP

*NEURO EXAM AND HISTORY*: primary modality of diagnosis/ best way to diagnose *Motor dysfunction*: often not apparent until 2-4 months of age (floppy baby) *Persistence of primitive reflexes* Differential diagnoses

Pathologic fracture

*Pathologic Fracture* (fragile bones due to condition. Understand the risk. If a patient has a bone structure condition they may be at higher risk of fractures) (normal movement during pt's day lead to fracture because the bone was so weak) • *Due to weakened bone structure/ condition* - Osteogenesis imperfecta - Rickets - *Hyperparathyroidism* - *Cancer* Clinical Manifestations or pathologic fracture- same as any other fracture. Pain, bone displacement Nursing Management- same for any other fracture

Phimosis and paraphimosis

*Phimosis*: Narrowing or stenosis of the opening of the foreskin that prevents retraction of the foreskin (mild or severe) - Normal finding in infants and young boys - Severe phimosis (impairs urinary elimination) is treated by circumcision (rare) - Teach parents to retract foreskin as part of routine care - Never force foreskin down/retraction as it can cause paraphimosis (foreskin that cant be replaced in the normal position- neurological emergncy ) *Paraphimosis*: retracted foreskin that cannot be replaced in normal position over glans (*emergency*) - Urological emergency! - Kid will be screaming and crying - Tx = circumcision Rogers notes: Teach parents to gently retract foreskin. Never force as it can cause paraphimosis- retracted foreskin cannot be replaced in normal position over glans. Edema venous congestion and is a urologic emergency. The boy will be crying/screaming and in a lot of pain.

Screening Detection of SB

*Prenatal Detection* - Detected on *Ultrasound- 16-18 weeks gestation* (2nd trimester) - Alpha-fetoprotein (*AFP*) - Fetal *MRI* - *Amniocentesis- 14-17 weeks (can do amniocentesis if neural tube defect is suspected following Afp, let parents know risks to amniocentesis*) Rogers' Notes : From mayo clinic: alpha-fetoprotein (AFP) a protein that's produced by the baby. It's normal for a small amount of AFP to cross the placenta and enter the mother's bloodstream, but abnormally high levels of AFP suggest that the baby has a neural tube defect, most commonly spina bifida or anencephaly, a condition characterized by an underdeveloped brain and an incomplete skull. Prenatal detection: can determine presence of some major open NTD prenatally AFP: reliable except when defect if closed Some spina bifida cases don't produce a high level of AFP. On the other hand, when a high level of AFP is found, a neural tube defect is present only a small percentage of the time. Varying levels of AFP can be caused by other factors — including a miscalculation in fetal age or multiple babies — so your doctor may order a follow-up blood test for confirmation. If the results are still high, you'll need further evaluation, including an ultrasound examination. Ultrasound Many obstetricians rely on ultrasonography to screen for spina bifida. If blood tests indicate high AFP levels, your doctor will suggest an ultrasound exam to help determine why. The most common ultrasound exams bounce high-frequency sound waves off tissues in your body to form black-and-white images on a video monitor. Amniocentesis If a blood test shows high levels of AFP in your blood but the ultrasound is normal, your doctor may offer amniocentesis. During amniocentesis, your doctor uses a needle to remove a sample of fluid from the amniotic sac that surrounds the baby. An analysis indicates the level of AFP present in the amniotic fluid. A small amount of AFP is normally found in amniotic fluid. However, when an open neural tube defect is present, the amniotic fluid contains an elevated amount of AFP because the skin surrounding the baby's spine is gone and AFP leaks into the amniotic sac. A second test can be done on the same sample to reliably confirm that a neural tube defect is present.

Etiology of Spina Bifida- Multifactoral Risks

*Previous pregnancies with neural tube defect*- most significant risk factor *Folic acid deficiency*- second most important risk factor (neural tube closes ~ day 28, if think may be pregnant, take folic acid supplements *girls* > boys *Caucasian* > African American Diabetes in Mom- good control of diabetes in mom important Obesity Anticonvulsants Maternal *heat exposure* (heat directly on the uterus. No *hot tubs*, no *hot baths* [showers ok], no concentrated heat to the uterus) -Previous preg: greatest risk! -Folic acid: 50% occur as result of nutritional deficiency: green leafy veg, cabbage, asparagus, liver, kidney, nuts, egg -Race: 3 X higher in white than AA -Anticonvulsants: valproic acid -Maternal heat: hot tubs

Initial Care of SB

*Radiant warmer- because baby cant be dressed or swaddled. Can possibly dry out sac* *Prone position*-decrease tension on sac and decrease risk for trauma *Sterile moist dressing over defect immediately to avoid cracks or lesions-non-adherent with sterile N.S. every 2 hrs* Inspect sac closely for ss of infection or skin breakdown Observe for *s/s of Hydrocephalus*- *↑ ICP- bulging fontanel, dilated scalp veins, high pitched cry, restless, ↑BP, ↓HR, Resp rate ↓or irregular* -Radiant warmer: temp maintained without clothing. Watch sac does not dry out -Prone: -Sterile dressing: -Inspect sac: look for leaks, abrasions, s/s infection -Observer for hydrocephalus: s/s

Diagnosis of Down Syndrome

*Suspect (probability of downs based on appearance)*: - Flattened hypoplastic midface with depressed nasal bridge, inner epicanthal folds, upward obliquity of palpebral fissures, small external ears, small mouth and narrow palate (causes all kinds of respiratory problems), prominent palatine ridges * post birth Confirm*: - Karyotype of chromosomes *Prenatal Screening* (many ways to get a definitive diagnosis) - Nuchal translucency test- ultrasound at 11-14 wks Chorionic villus sample at 8-12 wks - Amniocentesis at 15-20 wks - Percutaneous umbilical blood sample at 20 wks

Therapeutic management of Cerebral Palsy:

*early diagnosis & early intervention* is key • *Goals: Establish locomotion (movement), communication, self help skills* (care for self) • Gain *integration of motor functions* • Correct associated defects • Provide educational opportunities • Promote socialization experiences Therapeutic Interventions: multidisciplinary approach - Physical Therapy- engage in early - Occupational Therapy- engage in early - Speech Therapy- *want involved before a child can speak, involved with feeding, to develop oral musculature* (remember, CP is a movement disorder) - Feeding Therapy (major part of therapeutic regime) *speech/feeding therapsits are involved early involved with feeding and oral musculature development for best possible language outcomes in the future*. - *Self feeding*: typically the 1st major self help skill that children learn. Requires integration of fine and gross motor skills and visual perception - Team approach- there is a large *risk for aspiration*, - Complications: aspiration Education- want *least restrictive educational environment* that is effective for this child's education Recreation

Hydrocephalus

*hydro=water, cephly= head* *Increased accumulation of CSF within ventricles of the brain* *Congenital - spina bifida* (result of mal-development or intrauterine infection) or *Acquired- infection, neoplasm(brain lesion), hemorrhage*

Osteomalacia

*osteomalacia* = *problem with mineralization of the bone (undermineralized, softer bone)* (often *misdiagnosed* as osteoporosis in the US because osteomalacia isnt common in US and osteoporosis is) Definition/Diagnosis/Incidence: • Decreased mineralization of turnover sites • *Bone biopsy* • Clinical features, lab, radiology - Often misdiagnosed • Low incidence in U.S. • *Higher in populations with suboptimal nutrition and inadequate sun exposure* Etiology/Risk Factors: • *Genetic, Prolonged and severe Vitamin D deficiency or resistance resulting in hypocalcemia* (not really dietary in nature) • Hereditary *hypophosphatemia* • In conjunction with rickets syndromes persisting into adulthood Clinical Manifestations - Osteomalacia: • *Bone pain*(osteoprosis patients don't generally have pain, osteomalacia patients do)/ patient may describe as musculature(checking for bone pain- press over tibia, if it causes pain it is likely bone pain) • Fracture • Muscle weakness/cramps • Waddling gait/difficulty ambulating (deformities: *bowing legs due to weight baring*) • Labs - *Decreased serum Vitamin D, calcium, phosphate* - Elevated PTH Nursing Management - Osteomalacia:(depends on underlying etiology) (can happen to all ages and ethnicities) • Non-pharmacologic Intervention - Treatment depends on underlying etiology - Sun exposure/Nutrition - Bracing - Surgical intervention • Pharmacologic Intervention - Vitamin D supplementation - Calcium intake > 1000mg/day - Phosphate supplementation - Estrogen (post-menopausal women) - Thyroid hormone

Diagnostic Criteria for Intellectual Disability

-*3 areas* of diagnostic criteria for Intellectual Disability (formerly known as Mental Retardation) 1) *Intellectual functioning*: typically IQ under 70 2) *Functional strengths/weaknesses* (not IQ alone) - *Impairment in 2 of 10 adaptive skill domains*: Communication, self care, home living, social, community, self direction, health/safety, functional academics, leisure, work 3) *Onset before 18 years of age* (i.e. if you are over this age and get in MVA and have head injury you are not classified in the same way) Diagnosis: *Routine developmental screening* assists in early identification (can't do developmental screen on sick kid, do them on well baby/child check) *4 domains of developmental screen* • social/personal • fine motor • language • gross motor Standardized tests: - *Bayley Scale* of Infant Development - *Denver Developmental* Screening Test - Looks at personal social, fine motor, language, gross motor Etiology of Intellectual Disability: - Infection and intoxication - Trauma or physical agent - Metabolism or nutrition - Chromosome abnormalities - Gestational disorders - Environmental influences Nursing Care: Goal to promote optimum development - Education: right to free and unobstructed education that best serves the child - Hospitalization: know where they are based on their developmental disability - Discipline- *(structure) can be difficult but should still be in place. Should be rule oriented* because may not get to abstract level of thinking - Social skills- play, exercise, work - Play/exercise - Sexuality: May be easily persuaded or lack judgment. *Need a well defined code of conduct*. "you need to keep your shirt on"

Pyloric Stenosis

A *narrowing of the pyloric sphincter* (sphincter between the stomach and the small intestines) at the outlet of the stomach, *OBSTRUCTING the flow of food into the small intestines* Incidence/Etiology: • *1st born caucasian male* • Pathophysiology - *Muscle of the pyloric sphincter becomes thick and elongated, narrowing the pyloric channel* • Clinical manifestations - *progressive emisis- Early nonbilious vomiting beginning around 3 wks of age, progressing to projectile*- baby is losing weight bc of the vomiting, but is *crazy hungry* - *Olive shaped mass* in epigastrum- right where you expect the pyloric sphincter to be Diagnosis: • Diagnosis often made by *history and Physical exam* • *Ultrasound* • Possible *metabolic alkalosis and severe volume depletion* if watched too long and not treated quickly (base is lost through booty. fewer poops + vomiting -> metabolic alkalosis) *Surgical repair required* - pyloromyotomy- response is quick and good, they may still vomit some but will regain weight quickly and should recover well Nursing Interventions: • Pre-operative - NPO - Rehydrate - Correct metabolic alkalosis • Post-operative - Start *feed 4-6 hrs post op* - *Vomiting post up not uncommon* - Pain management

GU disorder assessment

Assessment: Nursing History • Demographics- age, race, gender • Socioeconomic status- elderly pt's on fixed income, having to get up in the middle of the night with urgency, may fall on way to bathroom and may need walker. processed foods with a lot of sodium/lower income areas. • Nutrition History- what foods are being consumed, what foods help or hurt their problems. High calcium can lead to stone • Medications (prescriptions and OTC)- *pt's with kidney issues shouldn't take NSAIDS* • Environmental Exposures- what is work/home like? Exposed to chemicals? • Previous Kidney or Urologic Problems- • Current Health Problems/Comorbid Conditions- anything diagnosed? • Family History/Genetic Risk- cancer, polycystic ovary disease • Travel- what have they eaten/drank/been exposed to? • Trauma- trauma can hurt kidneys bruise/laceration. Severe dehydration. If they get hit in the bladder can cause scarring. Ureter can get lysed. • Sexual Health- impacts pt's, sex impacts • Patterns of Urination- overdistension of bladder/ stasis/uti • Usual Weight- have they gined weight? Watch bc of fluid/electrolyte balance *Physical Assessment* Neurocognitive: memory level of consciousness, alertness. May be altered in Geriatric Populations with Urinary Tract Infections (UTIs) • Integumentary: Reflection of fluid volume status; rashes, bruising,Yellowing • Respiratory: Reflection of fluid volume status • Cardiovascular: *Electrolytes and fluid volume status; peripheral edema* • Renal/GU: Costovertebral angle tenderness, Intake & Urinary Output (UOP) comparison (I's and O's), Bladder distension, Urinary symptoms including: frequency, urgency, painful urination (dysuria) • Musculoskeletal: Electrolytesi kidneys help with electrolytes but muscles work with potassium. May see spasms • Psychosocial: Coping and support

Dyskinetic/nonspastic CP

Athetoid: • *Wormlike writhing* movements involving extremities, trunk, neck, facial muscles, tongue. Associated with hyperbilirubinemia • *associated with HYPERBILLIRUBINEMIA* Can be caused by kernicterus (staining of brain cells) Involvement of oral muscles causes drooling, dysarthria ( impaired speech)

Paget disease

Bone Disorders - Paget Disease of bone Definition/Diagnosis/Incidence • *Disorder of bone metabolism: accelerated remodeling, overgrowth, and impaired integrity of bone*. Classic= *overgrowth of bone in the skull*. Caused by mismatch of osteoclast and osteoblast activity. *Too much bone building* (can also happen is spine, pelvis, extremities) • 2-9% of older patients of European descent • Diagnosis with radiography, laboratory analysis *Etiology*/Risk Factors: • Genetic • Environmental • Viral Clinical Manifestations - Paget Disease (disorder of bone metabalism, mismatch of osteoblast[over active] and osteoclast activity[underactive])(continuous layering of bone that causes deformities) can *present with pain before deformitiy or just with deformity before pain* • Arthritis • *Pain*; chronic back pain, hip and pelvic pain • *Bone deformity* - enlarged skull, abnormal spinal curvature • Fracture • Labs - *Elevated alkaline phosphatase* • Imaging- *xrays will show overgrowth of bone but it can take a long time for bone to manifest. Pain may set in sooner* Nursing Management - Paget Disease: • Non-pharmacologic Intervention - Goal: ease pain and correct imbalance bw osteoclast and osteoblast (maybe assisted with right hormonal balance) - Education - Nutrition - Medications - Fall prevention- *pain leads to compensation in walking, gait is changed leading to higher risk for falls* - Physical therapy - Non-impact exercise - Surgical intervention Pharmacologic Intervention: • Antipagetic medication - Nitrogen-containing bisphosphonates - Simple bisphosphonates - Calcitonin • Analgesics

Carpal Tunnel Syndrome

Carpal Tunnel Syndrome- *not an emergency*, is an injury, *caused by overuse of the wrist and compression* of *median nerve* Definition/Diagnosis/Incidence: • *Focal mononeuropathy of hand*. In the thumb and first 2 fingers in the hand and halfish of the 3rd finger • *Compression of the median nerve through the carpal tunnel* • Diagnosed with symptom presence and nerve conduction studies • Usually unilateral, can be bilateral *Etiology/Risk Factors*: • Most common compressive focal mononeuropothy; 1-5%, *women* > men - Obesity - Female - Diabetes - RA - Genetic predisposition - Workplace factors Clinical Manifestations: • *Pain* or *paresthesia*/numbness in first three digits and radial half of fourth digit • Symptoms *worse at night*; *awaken patients from sleep* Treatment: - Workplace modifiction - Splinting - Glucocorticoid injections or oral - Surgery treatment of choice for ongoing symptoms without a reversible etiology (surgery = releasing ligament thats compressing the carpal tunnel and median nerve

Celiac Disease

Celiac Disease= *Malabsorption syndrome*: *second most common cause of malabsorption in children* (#1 = CF) *Permanent intestinal intolerance to dietary wheat and related proteins that produce mucosal lesions in GENETICALLY susceptible individuals* Incidence: 1 in 3000, *females* more than males, rarely in Asians or AA See Box 33-13: Clinical Manifestations of Celiac Disease, p. 1326 - *Impaired fat absorption* - Impaired nutrition absorption - *Behavioral changes* (*NO respiratory manifestations, CF DOES have resp manifestations*) Symptoms- Diarrhea, failure to thrive, abd distention, anorexia, irritability Diagnosis- *withdraw gluten from diet- have full remission of symptoms* Treatment- *Dietary management* These kids just don't want to eat wheat, they want to feel better so are ok with not eating wheat. Now there are a lot of good gluten free options

Gastroesophageal Reflux

Clinical manifestations: *lower* esophogeal sphincter not effective -> a lot of reflux/ spit-up Gastroesophageal Reflux (GER) vs GERD (disease) *Disease*=: 1) *FTT* (failure to thrive)/weight issues 2) *Anorexia* (loss of appetite), irritability 3) *recurrent pneumonia* (aspiration pnemonia develops from spit-up aspiration) Diagnostic Evaluation- lower esophagus sphincter open allowing reflux Therapeutic Management: *elevate for feeds, small frequent feeds, burping , sitting up for 30 mins after feeds, meds/proton pump inhibitors, Surgery: Nissen Fundoplication* Surgery: *Nissen Fundoplication* Wrap top of stomach/fundus of stomach around the lower esophagus to act as another lower esophageal sphincter because it contracts, sutured in place. *Common procedure for kid with GERD, failure to thrive, aspiration pnemonia, anorexia*

Complications/management/ Nursing care of - Bacterial Meningitis

Complications: - Obstructive hydrocephalus - Deafness, blindness, paralysis of facial muscles - Cerebral palsy (CP) - Intellectual disability - Learning disorder - *ADHD* - *Seizures* - *Death*- 10-15% fatality Management- Bacterial Meningitis - *Isolation* - Antimicrobial therapy - *Maintain hydration* - Ventilation - *managed in PICU* - Decrease increased ICP - Manage systemic shock - Control seizures - Control temp - Treat complications Nursing care: - Environment- *quiet with decreased stimulation* - Position- *allow position of choice/comfort* - *Frequent Pain evaluation (not masking signs of irritability but managing pain/menigitis is very painful*) - Safety- *seizure precautions* (chance of menigitis -> seazures is high) - *Frequent assessment*: - VS, neuro checks, LOC, I & O - Evaluate for s/s increased ICP - Family support - Prevention- Menectra vaccine for ages 11-55 (Meningococcal Meningitis Vaccine)

Inguinal Hernia

Definition-*protrusion of small intestines/fluid into groin through a weakness or tear in the abdominal wall* Incidence: *males*> females, *premature*> term (testes are abdominal organs until ~25 weeks, descent complete ~36 weeks, *this is a defect in the processus vaginalis [tube that testes travel down when they drop] where the proximal portion doesn't atrophy as it should and the dystal portion doesn't surround the testes as the tunica vaginalis*. what happens is the sack doesn't close, no atrophy of the proximal processus vaginalis and the *abdominal contents can go up and down* ) (*may develop at 2-3 months of age*. Earlier *babies may not have the capacity to create interthoracic/interabdominal pressure to create hernia before this point* Manifestation: *mass in groin*, *can present as painless inguinal swelling* Sac is present at birth, but may not manifest until 2-3 months (this isnt some trauma that happened, the non closure was always present, the baby just developed the ability to generate interthoracic pressure enough to cause hernia) *Management: surgical repair required, usually bilateral (not an urgent/emergent* issue but does have to be repaired with surgery, just don't have to rush/immediate)

Rheumatoid arthritis

Definition/Diagnosis/Incidence • *Chronic, progressive, systemic* (unlike osteoarthritis) inflammatory *autoimmune process* (will *progress even if athlete stops sport*, some meds will slow progression) • Affects synovial joints • 1.3 million people, primarily Euro-Americans, women > men Etiology/Risk Factors • Unclear etiology; environmental and genetic, maybe female reproductive hormones • *Women*> men • *European americans* at greater risk Clinical Manifestations - Rheumatoid Arthritis • *Acute/severe or slow/progressive pain onset* • *With or without Joint inflammation*(without would be early stages) • *systemic* • Generalized *weakness* • Persistent low-grade *fever* • Joint inflamation that is symetrical or not • Usually caught early: *goal becomes slowing progression*, preventing flare ups • *Late*: - *Frequent morning stiffness, decreased ROM, joint deformity* - *Systemic manifestations: vasculitis, respiratory/cardiac complications, syndromes* Nursing Management - Rheumatoid Arthritis: • Non-pharmacologic Intervention • Education - *Rest* - Proper positioning - Stress management - Self-management- heling patients *understand meds and know what to use when to prevent flare ups. More flare up's -> faster progressing disease* • Cold/hot applications • Plasmapheresis • CAM therapies: acupuncture, hypnosis, imagery, music therapy Pharmacologic Intervention • Disease-modifying antirheumatic drugs (DMARDs) • NSAIDs • Biological Response Modifiers (BRMs) *Not as common as osteoarthritis. Osteoarthritis patients will be older with repetitive use of joint*

Viral Meningitis (aseptic) - Diagnosis

Diagnosis: must test CSF with lumbar puncture Lumbar puncture: -WBC: *slightly elevated* -Protein: *within normal limits (WNL) or slight increase* -Glucose: *WNL* -Color: *clear/slightly cloudy* -Gram stain: *negative* Clinical manifestations: -Headache, fever, malaise, GI symptoms *Treatment*- symptomatic: (treat the symptoms untill their body can recover) - *Hydration, pain management, positioning*

Meningitis- Bacterial (pyogenic) (etiology/pathophysiology)

Epidemiology-*HIB vaccine*- 1990 1987- 41 cases per 100K 1993- 2 cases per 100K 1996- *near elimination* Etiology: • *Over 2 months of age* - H flu, strep pneumoniae, nisseria meningitidis (menningococcus) • *Neonates* - group B strep, e coli, listeria (*where has baby most recently been*) *Pathophysiology-Bacterial Meningitis:* • Most common route of infection - *vascular dissemination from a focus of infection elsewhere* (because of more permeable blood brain barrier) =* most common cause of bacterial meningitis. Most often by direct extension of nasopharyngeal infection and inner ear infection* • *Direct entry of organisms * - Post LP (lumbar puncture) or surgical procedures (may be caused by entry of orgnaisms, even when performed under sterile procedure) • *Anatomic abnormalities*- spina bifida (sac could get dry and cracked and get infection that goes straight to meninges) • *Foreign body*- VP shunt • *Infective process* - On the brain - Inflammation, exudation, WBC accumulation, tissue damage • Brain- becomes hyperemic, edematous, covered with purulent exudate, can lead to obstructive hydrocephalus(would be signs of ICP) Roger's notes: - *Most often by direct extension of nasopharyngeal infection and inner ear infection* - r/t altered permeability of the blood brain barrier - Orgs from nasopharyngeal Invade the underlying blood vessel, enter cerebral blood supply. Less common to have direct extension of infection from paranasal/mastoid sinuses - Direct entry: organism entering by direct implantattion during procedure.

Fat embolism

Fat Embolism-*emergency* chunk of fat comes out of the long bone fracture, travels through the vasculature and ends up in the lungs causing fat embolism (same as DVT due to thrombosis/pulmonary embolism but this is caused by fat and not clot *Respiratory Distress!* *Long bone and pelvic fractures; hip most at risk*; bilateral femoral fractures high risk Closed > open- happens *more commonly in closed fractures than open fractures because theres not an outset for all these cells to go* in a fracture so they go into the vasculature *Trauma* > non-trauma- occurs more in trauma fractures than non trauma (pathologic fractures) Clinical Manifestations: *Classic triad* 1) *HYPOXIA, SHORTNESS OF BREATH* 2) *NEUROLOGIC ABNORMALITIES*- confusion disorientation 3) *PETECHIAL RASH*- sign that it's a Fat embolus and not a DVT (rash is not localized, its systemic/can be anywhere) • Patient with a bone injury, petechial rash, hypoxia and neurological abnormalities, you know its probably a fat embolus rather than a pulmonary thrombus (because the petechial rash and bone injury)

Hydrocele

Fluid in the *processus vaginalis* Same developmental process as inguinal hernia *Asymptomatic except for a palpable bulge in inguinal or scrotal area* Presents as soft *PAINLESS swelling* of the scrotum (*not* an emergency) (common) - Doesn't effect future fertility - *Unlike hernia, it cannot be reduced*. And may not by produced by sudden increase in intra abd pressure - Soft painless swelling of scrotum. *Hydrocele (2 kinds)* 1) *Non-communicating* - Upper segment of processus vaginalis is closed - Common in newborns - *Often subsides spontaneously*- okay to watch and see if it resolves 2) *Communicating* - Processus vaginalis remains open (can have increase in fluid especially after standing up) - More likely Requires surgery if not spontaneously resolved at *1 year old* (surgery= simple) - *Morning it is smaller, after a active day will appear larger* - Non communicating: tunica vaginalis still has some peritoneal fluid - Communicating: because it is open, peritoneal fluid may be forced by pressure and gravity into scrotum. Scrotum appears larger after active day, smaller in morning. b/c this represents a patent processus vag, can lead to strangulation. If doesn't spontaneously resolve at a year, surgery

Fracture

Fracture (common injury, different types) • *Interrupts blood supply to bone*, local hypoxia, furthered by inflammation (consider the level of impairment that happens to the musculoskeletal structure/function, blood flor or nerve innervation? Is it compound (skin open/ infection risk) all fractures are painful. All fractures can occur and cause no nerve or blood vessel damage or all can cause nerve and blood vessel damage • Closed vs open • Comminuted • Displaced • Oblique • Spiral • Impacted • Greenstick Fracture - Clinical Manifestations: • Pain, bruising • Loss of function • Deformity • Crepitus • Edema • Erythema and/or ecchymosis • Impaired sensation Fracture - Nursing Management • Confirmed by X-ray • *Pain control= #1 priority for nurse* • Stabilization- if it's a compound facture and very unstable and you are 1st on scene you will want to stabilize it to get pt to treatment (can occur with patient falls) • Infection control and Tetanus vaccination if open • Splint/Cast • Reduction / reduction and fixation • Surgical intervention • Traction fracture Fixation (Pain is mainly around the insertion site, bone is stabilized by the fixation) • Internal Fixation Surgical repair of bone with rods, plates, prosthetics • Immobilization device • Removable/non-removable External Fixation •External placement of rods/pins through skin and bone; attachment to rigid external frame. Often used for lower extremities but also Pelvis, neck, and upper extremities Neurovascular Compromise- assess for this if there is a fracture (assess for cap refill, sensation, color, pulses) - Recognize - Prevent- good assessment, repeated and ongoing to recognize change - Respond- when change in assessment is recognized/ neurovascular compromise is recognized, respond appropriately (loosen the splint if too tight, notify provider if it is not caused by something you as a nurse can manage)

General Care of SB

GU functioning - *Goal is to ↑ bladder function, ↓ subsequent risk of urinary system distress, ↓ need for reconstructive surgery to lower urinary tract* - *Often need to be intermittently catheterized on a regular schedule . Parents are taught to do this early* - *Child is taught to do this ASAP- usually begin at 6 yrs age!* Bowel control- *regular habits* Diet modification and regular toilet habits can ↓ constipation, ↓ impaction use of laxatives, suppositories, enemas -*Mace procedure*- irrigate from above and stool from below -Incontinence threatens social acceptability -Tactile: in abnormal state sceondary to positioning, important to touch, stroke, caress

Gout

Gout (deposit of urate crystals) • Definition/Diagnosis/Incidence Systemic disease of *urate crystal deposit in joints and other tissues, causing inflammation. Has to do with how the body metabolizes purine* (deposit of urate crystals, most often seen at the great toe) • 6.1 million people • Primary or Secondary • Diagnosis by *arthrocentesis*, synovial fluid cell counts and examination for crystals Etiology/Risk Factors • Primary - *error of purine metabolism* • Secondary - *excessive uric acid* (deposited in select joints) r/t renal insufficiency, diuretic therapy, crash diets, chemotherapy agents

Hip fracture

Hip Fracture- one of the most frequent musculoskeletal injuries. *Highest morbidity and mortality rate of MS injuries*. Often leads to rapid decline in health of elderly person. Occurs more in elderly due to muscle atrophy, decreased mobility, bone loss/ osteoporosis *Intracapsular* • Femoral neck fracture. *The head of the femur is very vasculated so necrosis* can occur more often in the femoral head due to impeded vessels. *More likely to lead to total hip replacement* • *Extracapsular* Below femoral neck- *less risk for necrosis*

GI Obstructive Disorders

Hypertrophic Pyloric Stenosis Hirschsprung's Disease Intussusception

Clinical manifestation of bacterial meningitis *Infants/young children- 3mos to 2 yr*

Infants/young children- 3mos to 2 yr - *bulging fontanel= MOST significant sign*; fever, poor feeding, vomiting, irritable, restless, high pitched cry Nuchal rigidity, Brudzinski, Kernig - *not consistently present in young child less than 18months old* (children less than 18 months of age can have meningitis and still have a supple easy to manipulate neck, *don't use Nuchal rigitity for diagnostic purposes under 18mos*)

Management of SB

Initial care- *Prevention of infection* (the *sac is exposed after birth, cover with sterile moist dressing*) - Monitor closely for signs/symptoms of infection (baby will be sleeping prone pre closure of defect)(*look for ss of increased cranial pressure*, baby can be hyper/hypo thermic) Neuro assessment: - observe for associated anomalies. - *Daily head circumference, look to see if crossing growth lines (increased head circumference = ICP sign and hydrocephalus*) - *Assess fontanels frequently for tension or bulging (icp sign*) *Early closure of defect: within 24-72 after birth for most favorable outcome to decrease morbidity and mortality esp from serious infection. Closure doesn't impact sensory or motor/Physical disability isnt effected when surgery post birth* Basic needs of infant- trust v mistrust- sound/lights/physical touch -Prevention of infection: monitor closely for s/s infection: increased or decreased temp (ss of sepsis), irritability, lethargy, nuchal rigidity -Neuro assessment: observe for associated anomalies. Daily HC, fontanels examined daily for tension or bulging. Assesss anal tone- helps determine motor involvement, Urine output? -Early closure: within 24-72 hrs. for most favorable outcome to decrease morbidity and mortality esp from serious infection. -Why? Prevents infection, decreases trauma to exposed tissue, decreases stretching of other nerve roots when sac expands after birth -Surgery doesn't change physical disability~

General Care of Spina Bifida: Latex

Latex Allergy-(these kids are *on latex precautions to prevent allergy*) (*more you are exposed to latex= increased risk of allergy*) watch out for allergy - Latex: repeated exposure to latex products, ↑ surgeries, ↑ procedures - Goal: prevention of latex allergy, provide latex free environment

Connective Tissue Disease

MOST classified as autoimmune disorders (osteoarthritis is not) Characterized by chronic pain, progressive joint deterioration, impaired function/mobility Interdisciplinary approach to care Arthritis: - Non-inflammatory (localized, non-autoimmune) - Osteoarthritis (non auto immune) - Inflammatory (systemic) - Rheumatoid Arthritis; Lupus erythematosus

Acute Intestinal Obstruction

Manifestation: • *Abdominal pain* (patient may be *guarding*) (pain is usually *superior and proximal to the obstruction* [above and behind]) • *Distension*- due to accumulation of gas and fluids above the obstruction • *Nausea and vomiting*- because lack of passage of abdominal contents • *Change in stool pattern*

Pediatric Urinary tract infections

Most UTIs are uncomplicated and respond to outpatient antibiotic treatment (*febrile neonates <60 days with fever 100.4+ need to investigate, need to do in and out cath for urine culture because high possibilty that this is UTI*) • *Pediatric UTI Requiring Hospitalization:* - Toxic or septic patient - Signs of urinary tract obstruction or significant underlying disease - Patient unable to tolerate PO fluids or medications / vomiting - *Infants and children <2 years old with febrile UTI* - *All infants <3 months old* • Causes of Pediatric UTI - Vesicoureteral reflux - Urinary tract obstruction - Incomplete or infrequent voiding - Neurogenic bladder - Sexual abuse *Pediatrics Radiologic Studies:* • The studies ordered will evaluate for reflux, obstruction, or anatomic abnormalities. • *Indications for imaging include the following*: - Pyelonephritis - kidney infetion - First UTI in a boy of any age (boys UTI not so common) - First UTI in a girl less than 3 years - Second UTI in a girl older than 3 - First UTI in a child with family history of urinary abnormality

Clinical manifestations of bacterial meningitis *Neonate*

Neonate- Difficult to diagnose- *vague* like all neonatal sepsis (*going to have to do a lumbar puncture to diagnose*) - *poor feeder, poor suck, poor muscle tone*, hypothermia/hyperthermia (fever), irregular respirations, *apnea* (cessation of breathing can be presenting symptom of menigitis), wt loss, irritable, drowsy, bulging fontanel- may or may not appear Neck is usually supple Going to have to do a lumbar puncture, look at cerebral spinal fluid to diagnose (CSF usually colorless, clear. *Bacterial meningitis CSF can be cloudy or discolored*) *If you are treating an infant/neonate and they come in with these symptoms, you will want to elevate care to hosptial/ maybe life flight*

Nursing care- *post-op*erative shunt malfunction

Observe for s/s shunt *infection and malfunction* *Position flat on un-operated side (taking pressure off the operated side*) *Inspect operative site freq-using aseptic technique- leaks, redness, edema* Skin care Family support through procedure: families often scared, do family teaching, model Family/patient teaching: *educate s/s ↑ICP + s/s infection* Rogers' notes: -*Infection: greatest hazard post-op. s/s: febrile, poor feed, vomit, ↓responsiveness, seizure* -Malfunction: s/s ↑ICP, fontanels, pupil dilation, VS, abd distension -Position flat: to avoid too rapid decrease on ICP -Skin care: esp since decreased mobility secondary to prone -*Strict I & O, NPO x 24 hrs* -Family support: Fear! Brain!

Osteoarthritis

Osteoarthritis (*not an autoimmune disorder, not systemic, may not progress if aggravating activity is stopped*) *classified as a connective tissue disease because it's a deterioration of cartilage in the joints* Definition/Diagnosis/Incidence: • *Progressive deterioration/loss of cartilage and bone * • One or more joints often accompanied by secondary *inflammation* • 27 million individuals in US Etiology/Risk Factors: • *Primary: normal aging >60, genetics- most common* • Secondary: joint injury, obesity, caused by repetitive overuse - less common (common reason for total knee replacement) Clinical Manifestations - Osteoarthritis (also see in hands and knees): • *Chronic joint stiffness, pain; worse with activity, better with rest* (*some joints effected, some not*) • *Tenderness or crepitus on palpitation* • Bony hypertrophy, nodules • Inflammation (synovitis) • *NOT SYSTEMIC*, but can manifest bilaterally/syemtrically (maybe symetrical, maybe not) Nursing Management - Osteoarthritis Non-pharmacologic Intervention • Education - *Rest versus exercise- determine what is activity that is causing the problem (in early osteoarthritis rest usually helps, in later osteoarthristis rest contributes to the settling of the fluid in the joints in this case exercise can help)* - Functional joint positions- keeping joints in their anatomical positions in later stages - Heat/cold applications - Nutrition - weight control - Medications- otc meds common • Physical therapy • CAM therapies - capsaicin, glucosamine and chondroitin • Surgical management - TJA total joint artheroplasty (replacement of effected joint)(last option) Pharmacologic Intervention • Topical and oral analgesics/NSAIDs- pain management is key COX-2 inhibitors first choice • Cortisone, Hyalgan injection (used when half or more of the cartilage is lost) (if an athlete recognizes this is occurring and stops sports it may not get worse)*Osteoarthritis patients will be older with repetitive use of joint* More common than Rheumatoid arthritis

osteoporosis

Osteoporosis- *causes damage to bone structure before you actually start seeing symptoms* Definition/Diagnosis/Incidence: • *Excessive resorption by osteoclasts (bone loss)* • *Deficit of new formation by osteoblasts* (bone building) • Causes decreased bone density and risk for fracture • *Fragility fracture or Bone Mineral Density assessment T-score < -2.5* • 44 million at risk • 10 million with the disease • *Postmenopausal women > men or premenopausal women Etiology/Risk Factors *Risk factors* men and women; • Advanced age • Smoking • Immobility • Vitamin D deficiency • *Low impact fracture after age 50* • Estrogen deficiency in women • *HypERparathyroidism* Clinical Manifestations of Osteoporosis: (often vague symptoms, *typically not diagnosed unless there is a fracture* first. *Bone mineral density screening done for affirmative diagnoses*, shows you density of bone. Osteoporosis =bone more porous ) (*bone is strong and hard, just full of pores*) • Kyphosis • Height loss • *Low impact fracture- fall from standing height or lower* • Voluntary mobility restriction: spine. Maybe they stretch their spine a lot or are not as mobile as they used to be • *Spinal tenderness* Nursing Management Non-pharmacologic: • Education - Nutrition, Exercise, *build strong bone by a young adult* • *Bracing, heat, massage* • Surgical intervention (only reason to have is for injury such as hip fracture, etc) nurse management Pharmacologic Intervention: • *Calcium/Vitamin D supplements* • *Bisphosphonates* • *Estrogen* (post-menopausal women) • *Thyroid hormone*

Cerebral spinal fluid (CSF)

Our bodies make the correct amount ot CSF, it goes between the hemospheres and we absorb it in a balance that is right for the correct intracranial pressure of our bodies *Cushion* protecting the brain and spinal cord from physical impact. Supplies nutrients for brain cells CSF is *usually clear and colorless* Amount and pressure typically kept within narrow range

cerebral palsy Pharm

Pharmacotherapy (*if we have hypertonicity we want to have pharmacotherapy that decreases tonicity*) *Centrally acting anti-spasmodics* (baclofen) (decreases tonicity) - *High doses of oral baclofen are not effective and have significant side effects* (high doses of oral are ineffective) (if patient cant be managed on therapeutic doses of oral baclofen then we need to find another route) - *Baclofen pump*: (solution to when oral doses are not effective) surgically implanted device that is externally loaded with Rx and has slow release of Rx *Skeletal muscle relaxant* (dantrolene) *Local nerve block to reduce spasticity* - *Botox*: safe and well tolerated, effective to ↓ spasticity in children *Anticonvulsants* (phenobarbital, dilantin) (to reduce seizures)

Spina Bifida occulta

Spina Bifida- Type One SB *Occulta = hidden*- wont see a sack when you turn the baby over and look at back - failure of vertebrae to close without protrusion (no protrusion) of spinal cord or meninges/ *Not visible externally* - Cutaneous manifestations- skin depression, tuft of hair: may or may not occur - Neuromuscular disturbances- *most often asymptomatic/ no consequence/no interference w life, may have foot weakness, bowel/bladder sphincter issues* Not visible externally. May be as much as 10-15% of general population Cutaneous: skin depression, dimple, dark tufts of hair, port wine angiomatous nevi, soft subq lipoma (benign tissue/fatty lump under skin) Neuromuscular: can have foot weakness, bowel/bladder sphincter issues, may not be evident until child walks.

Congenital and Pediatric Neurological Disorders Review

Spinal cord: 44 cm long from medulla to lumbar vertebrae Nerves: all nerves to trunk and limbs issued from spinal cord Reflex: center of reflex action containing conducting pathways to and from the brain Encased in protective sheath of bone and meninges Meninges: thin protective layer covering spinal cord and brain Neural tube defects: largest group of congenital anomalies

structure of kidney

Structural & Functional Units of the Kidney Structural: • Capsule-fibrous tissue covering outer surface of kidney except for the Hilum (opening) which is the opening where vessels, nerves, and ureters exit • 2 layers of functional kidney tissue: Cortex & Medulla • Medulla fans into pyramid, Renal Columns separate pyramids • Papillae -tip of each pyramid. Papillae drain into the major and minor calyx • Major & Minor Calyx- Collect urine at end of each papillae, form together which narrows to make Renal Pelvis then becomes the Ureter Functional • Nephron - *Forms urine from blood (get waste products out)* - 1 million/kidney 2 types of nephron: Cortical & Juxtamedullary • *Cortical= shorter, [shorter word and shorter nephron]* • *Juxtamedullary-make up 20% of nephrons, longer, and extend deep in the Medulla & function to concentrate urine in times of low volume* Nephrons located in Renal Cortex Papillae-drain urine into collection side Ureter-pathway for urine to travel to bladder

Delivery of SB baby

Tertiary care facility: with access to neurologic surgical services for the newborn Elective pre-labor C Section(puts decreased stress on the defect)(laboring can put undue stress on the defect and cause additional damage)(will have to change birthplan*) - Prelabor c section May result in better motor function in baby than with SVD (spontaneus vaginal delivery), or CSxn after labor failed - Prevents injury to defect - Tertiary: with access to neuro surgical services - Elective CSxn: may result in better motor function than with SVD. Or with CSxn after labor failed. - Also prevents injury to sac - Fetal surgery: has significant decrease in hydrocephalus (fluid on brain) in SB

Urinary Tract Changes with Aging

Urinary Tract Changes with Aging • Bladder- decreased capacity, weakness, and loss of urinary sphincters' tone - *Urinary stasis- incomplete bladder emptying- risk of UTI* - MEN-*enlarged prostate. Puts pressure one and decreases draining of urine* - WOMEN-*Cystocele & Prolapse (decreased elasticity & tone of pelvic floor muscles)* shorten the urethra-pessary (instead of surgical repair, gives extra support) - Pregnancy causes bladder displacement as fetus grows • Urethra- risk for urethritis *Hypo-estrogenic states in older women adversely effects vagina & urethra due to atrophy, making them more susceptible to infections* • Prostate gland- Enlargement causes urinary retention & difficult initiating urinary stream

Urinary Tract Infection (UTI)

Urinary Tract Infection (UTI) • Occurs various sites of the urinary tract ‐ Urethritis, cystitis, prostatitis, pyelonephritis (wherever the -itis is look for inflammation or infection, that's where they will call the infection) Most common in women r/t urethral proximity to rectum • *90% of UTIs are cause Escherichia coli. Women are more susceptible* - Less common cause Staphylococcus saprophyticus, Klebsiella pneumoniae, Proteus and Enterobacter species UTI Symptoms: Painful, burning post micturition (dysuria) • *Frequency and urgency* • *Pelvic pain* • *Fever* - *Elderly presentation -Confusion*/AMS - *Pregnant +/- Symptoms. More at risk for UTI*, *may be asymptomatic* • Urine Assessed • Olfactory-Malodorous Visual-Concentrated, Cloudy, Hematuria (blood) UTI Presentations: *Older population* • Behavioral symptoms: - Altered mental status (may or not be able to tell you about the pain) - Confusion - Dizziness - Falls - Hallucinations - Fatigue - Lethargy Pregnant • +/- S/S (may or may not have s/s)

Down Syndrome presentation

When we look at *growth and development/denver* scale : Looks at: - *personal social*- downs kids excel socially - *fine motor*- delayed- - *language*- probably delayed - *gross motor*- delayed in Downs, hypotonia (flobby baby) *Brushfield Spots* (white specs in eyes, speckling of the iris) *Epicanthal Fold* - skin of the upper eyelid covers the inner corner of the eye *Hypotonia* (and delay of gross motor skills) and *Hyperflexibility*(like Cerebral palsy, has hypotonia) *Simian Crease* (1 line all the way across palm of hand) *all downs have simian crease*. If you have a simian crease you may/may not have downs also *wide spacing between thunb and 1st finger*. Same with toes -Small square head -Slant to the eyes -Epicanthal folds/ skin of the upper eye lid folds over the corner of the eye -Flat nasal bridge -Protruding tongue -Modeling skin -Hypotonia -Hands: Short stubby fingers -Maybe: short broad neck -shortened rib cage -Increased risk of pectus excavatum- sunken chest (doesn't cause any cardiac or other issues)

care planning for infants with unrepaired myelomeningocele

cluster care, be aware of hospital environment, voices, lights, sound, etc General- At Risk For *sensory deficit* r/t positioning (also risk for sensory overload bc in ICU) s/t myelomeningeal (MM) sac Skin- ARF infection r/t exposed non-epithelialized meningeal sac s/t MM (*if it gets infected would get menigitis*) (cover skin with sterile moist dressing)(monitor for s/s of infection , looking for anything that looks like meningitis GI- Nursing diagnosis : Nutrition less than body requirement r/t decreased intake s/t prone positioning or increased ICP (baby has to be belly down with an unrepaired sac, harder to eat in prone position), or placed NPO as they are preparing for for surgery, (if baby is eating, probably small frequent feeds)(if baby is NPO get mom to pump to keep milk supply up) GU- ARF urinary retention/ UTI r/t neurogenic bladder s/t MM (teach how to have good urinary hygeine, teach parents how to *in-and-out cath* [not foley]) GU: clean intermittent cath, meticulous perineal hygiene MS- ARF Falls always! ARF injury r/t neuromuscular impairment s/t MM MS: ROM exercises Rogers' notes: -Importance of POC always! -Remember *priority diagnosis- prevention of infection*! Even as you care for the whole child and family -General: also ARF for sensory overload, r/t ↑ stimulation secondary to hosp environment: -Cluster care, ↓envrionmental stimulation during times of rest. Older: self care deficit Skin: talked about covering sac, of course monitor closely for s/s infection, ↑or↓temp, irritability, lethargy, maybe nuchal rigidity -ARF: altered skin integrity r/t ↓mobility secondary to neuro impairment -Turn head freq., turn q 2 hrs after surgery, assess pressure areas -GI: feeding- small freq feeds every 2 hrs while awake -ARF: constipation r/t impaired bowel fxn secondary to MM -Diet, toilet habits

Spina Bifida

failure of closure of neural tube (failure of the neural tube to close, most likely in the lumbar and sacral areas) *Spina bifida: Failure of bony spine to close*: defect in wall of spinal cord caused by lack of union between laminae (flat part of either side of arch of vertebrae). Faiure of closure of neural tube *Occurs most often in lumbar and sacral regions (low)* Vertebrae_ 33 bony segments of spinal column that surround spinal cord Show WNL, occulta, meningocele, myelomeningocele

DVT/PE

hip fracture pt there for repair is at risk for DVT due to clot and fat embolism due to fracture *DVT Clinical Manifestations* • *Swelling/edema/erythema/heat/likely in lower extremity/calf*. Nursing Management for DVT: *notify provider, rest, medication* administration *PE Clinical Manifestations* • *Sense of impending doom, sharp pain, shortness of breath, hypoxia, rapid onset* Nursing Management: *notify provider, support respiratory function with O2, position* (supportive nursing response is the same for PE due to thrombus or fat embolism)

2 types of Cerebral Palsy

spastic & diskinetic based on type of movement disorder

Surgery: Nissen Fundoplication

surgery for gastroesophogeal reflux

Fracture Healing Process

takes time. *4-6 weeks* for young healthy person = healing time. Longer for older people or poor health *Hematoma Formation- happens in the first few days* *Granulation tissue formation- following weeks* Soft callus formation Hard callus formation- Remodeling

Complication: Shunt malfunctions

• *Usually mechanical obstruction* (most common malfunction)- junk gets in it, it cracks, etc. • *Displacement for growth* (presents with s/s of ICP) - *Most common reason for revision = displacement for growth* (not infection)- kid is growing and runs out of that extra tube in the peritoneum. Ss= ^ ICP, Irritable, vomiting, head hurts, felt better when vomited and sitting up, not thinking clearly, loss of milestones/skills, change in LOC - Displacement for growth: Most common reason for revision! - The peds nurses see a lot of infection-> revision, but #1 reason for revision is displacement for growth - Displaced either at vent end or distal end

Intussusception

• Definition: The *telescoping of the bowel upon itself/ invaginates* in on itself like a sock-> GI *OBSTRUCTION* • Incidence/Etiology - 2.4/1000 live births - Most common: *3 months-3 years*, M>F, recurrence 10% • Clinical manifestations: *current jellylike stools (because bleeding), obstruction, inflamation* - Early - Late Clinical Manifestations *Acute- Triad* 1) *Pain*: sudden onset of crampy abdominal pain in age range 3 mo-3years) 2) *Sausage shaped abdominal mass* (typically on right side) 3) *Currant jelly stool* So, this is an acute abdomen so pt will present with *N/V, distension, abdominal pain* Chronic: *diarrhea, anorexia, weight loss, vomiting, periodic pain* Diagnosis and Therapeutic Management: • Reduction by barium- hydrostatic reduction- when go from below/the rectum to see what it is, it actually untelescopes it • *Reduction by surgical manipulation- if the intussesception reoccurs* • *Surgical resection- if the bowel is severely effected* Nursing Interventions: • H & P- get good history and physcial, many conditions can be diagnosed or ruled out with good history and physical • Family support- education, support

Anatomy Review. GU = kidneys, ureters, bladder

• Urine formation - glomerular filtration • Electrolyte Balance - tubular reabsorption by diffusion, Na+ • Acid‐Base Balance - tubular secretion, K+, H+ • Filtration, diffusion, active transport, and osmosis • *Renin production (renin= vasoconstrictor)* • Prostaglandins production (prostaglandins respond to injury site) • *Bradykinin production (vasodilator)* • Erythropoietin (RBC) • Vitamin D activation (calcium uptake)

PVS and Nervous System Influences on bone/muscle health

• Vascular Compromise - atherosclerosis (thick arterial wall) and HTN can cause vascular compromise and bone issues • Oxygenation compromise • Reduced Vessel Elasticity • Nerve Impulse Compromise- *anything that decreases sensation increases risk of injury* (failure to recognize when bodily harm occurs) *Older Adult*- common changes in bone structure: • *Osteopenia*- bone loss • *Cartilage loss* • *Muscle atrophy*- may see boney prominences (not more bone, its just more apparent because of lack of muscle) because of muscle atrophy. • Comorbidities- diabetes, HTN, long term steroid use, obesity