Families exam 5

What is Celiac Disease?

Chronic autoimmune disorder of small intestine when ingesting gluten leading to impaired absorption of fats & nutrients

Celiac disease clinical manifestations

Local

- Abdominal bloating, cramps

- Steatorrhea/Diarrhea

Systemic

- Anemia

- Small stature, delayed maturity

- Vitamin deficiencies

- Muscle wasting

- Rickets (vitamin B)

Celiac disease assessment

- Vitamin A deficiency: vision problems
- Anemic: vitamin B12 deficiency, RBC

Celiac disease Meds

· Vitamin & mineral supplements

· Iron, folic acid for anemia

· Vitamin k

· Corticosteroids

Celiac disease nutrition

Gluten-free diet

· NO wheat, rye, barley

· CAN have rice, corn, potatoes, eggs, fruits/veggies, dairy without malt, nuts, beans

· High protein, high calorie, low fat

· Decrease dairy/lactose intake

· IV nutrition for refractory disease

Meckel diverticulum clinical manifestations

· Rectal bleeding: usually painless
· Abdominal pain
· Bloody, mucus stools: monitor Hgb/Hct

Meckel diverticulum nursing interventions

· Meckel's scan: most effective dx test
· Prep child & family for surgery: surgical removal of diverticulum

Meckel diverticulum pre-post op care

Pre Op --> Blood transfusion, IVF to correct hypovolemia, IV abx, monitor blood loss in stools

Post op --> NPO, Assess bowel sounds, NG tube to low continuous suction

Meckel diverticulum teaching

Monitor for signs of infection

Rotavirus clinical manifestations

Viral infection transmitted via fecal-oral route & is most common cause of diarrhea in ages < 5 yrs (but affects all ages)

· Fever

· Diarrhea for 5-7 days: foul-smelling, watery stools

· Vomiting for 2 days

Rotavirus nursing interventions

· Stool sample

· Baseline height & weight

· Daily weights, monitor I/O

· Avoid rectal temp.

· IVF

Rotavirus nutritional interventions

Avoid: high carb, sugary, high-sodium, or caffeine and:

- Fruit juice, carbonated soda, gelatin

- Chicken/beef broth

- Undercooked or under-refrigerated foods

Rotavirus priority

1. Start with oral rehydration solution (ORS) of 75-90 mEq of sodium/L at 40-50 mL/kg over 4 hr

2. Initiate maintenance Oral Rehydration Solution (ORS) of 40-60 mL/kg/day

- Give ORS alternatively with intake of other fluids: breastmilk, formula, milk

- Give infants lactose free formula

- Older children may resume regular diets

Replace diarrhea stool: 10 mL/kg of ORS

Rotavirus teaching

- Transmission: fecal-oral

- Incubation: 48 hr

- Parents should inform child’s school: stay home during incubation period

- Use commercially prepared ORS when child experiences diarrhea

- Change bed linens & clothes/underwear daily: avoid shaking linens

- Clean toys & keep away from other children & do not share dishes/utensils

- Hand hygiene: after toilet or changing diaper

Mild dehydration clinical manifestations

· Weight loss: 3-5 % (infants) & 3-4% (children)
· Capillary refill > 2 sec
· Slight thirst

Moderate dehydration clinical manifestations

· Weight loss: 6-9% (infants) & 6-8% (children)
· Capillary refill between 2-4 sec
· Thirst & irritable
· Dry mucous membranes
· Slight increase pulse & RR
· Normal to sunken anterior fontanel: under 2

Severe dehydration clinical manifestations

· Weight loss: >10% (infants) & 10% (children)

· Capillary refill > 4 sec

· Tachycardia & orthostatic hypotension: can progress to shock

· Extreme thirst

· Oliguria or anuria

· Very dry mucous membranes & tented skin

· Sunken anterior fontanel

Mild Dehydration Nursing interventions

Oral rehydration is attempted first:

- Oral rehydration solution (ORS) 5 ml Q2-5 min

- Avoid fruit juice, sodas, & sports drinks

Moderation Dehydration Nursing Interventions

- ORS 5 ml Q2-5 min PLUS replace continuing losses rapidly over a 3-4 hr period

Severe Dehydration Nursing Intervetions

- IV therapy: 20 ml/kg NS bolus, may repeat x1

- Begin ORS when able to tolerate fluids

- Avoid fruit juice, sodas, & sports drinks

- Replace potassium losses AFTER the child has voided to ensure adequate kidney function (NEVER admin K+ as IVP)

Dehydration Nurses should Monitor

· Assess capillary refill
· Daily weight
· Monitor I/O, VS

Cleft lip: clinical manifestations

incomplete fusion of oral cavity

Cleft-palate: clinical manifestations

incomplete fusion of palatine plate (roof of mouth)

Cleft lip-palate priority assessment findings

· Ear infections & hearing loss: at risk for recurrent otitis media
· Speech & language impairment
· Dental problems: teeth might not erupt normally

Cleft lip-palate pre/post op care

Pre-op:

- Inspect lip & palate using gloved finger, assess ability to suck & feed

- Baseline weight

Post-op:

- Pain management

- Suture line care: petroleum jelly

- Protect site

* Nothing in mouth

* Elbow restraints: may be used to keep infant from damaging the repair site: remove periodically to assess skin & allow movement

* Logans' bow

* No brushing 1-2 weeks

- Back to sleep on back or upright on side

Cleft lip-palate nursing plan of care

· Cleft lip: repair done 2-3 mo
· Cleft palate: repair done 6-12 mo (most require 2nd surgery)
· Monitor for ear infections
· Refer parents to speech therapist

Cleft lip-palate nutritional interventions

Isolated cleft lip

- Encourage breastfeeding & use wide-based nipple for bottle feeding

- Squeeze infant cheeks together during feeding to decrease gap

Cleft palate or cleft lip & palate

- Position upright while cradling head & use specialized bottle with one-way valve & specifically cut nipple

- Burp frequently

- Syringe feeding if necessary

GERD clinical manifestations: Infants

· Spitting up or forceful vomiting, irritability, excessive crying, blood in vomit

· Respiratory issues, apnea

GERD clinical manifestations: Children

· Heartburn

· Abdominal pain

· Difficulty swallowing

· Chronic cough

· Non-cardiac chest pain

GERD nursing interventions

· Sit them upright with HOB elevated to 30 degrees after meals
· Small frequent meals
· Thicken infant's formula
· Avoid foods that cause reflux: citrus, peppermint, spicy or fried foods
· PPI's

GERD Education

may outgrow GERD

Prevention

- Avoid eating close to bedtime

- Avoid tight-fitting clothing, smoking, alcohol

GERD findings to report

· Pneumonia
· Failure to thrive
· Weight loss

Appendicitis clinical manifestations

- RLQ pain: check for rebound tenderness

- N/V, anorexia

- Diarrhea or constipation

- Fever/chills

Perforation

- Temporary relief from pain, worsening S/Sx

- High fever

- Dehydration

- Shock

Appendicitis nursing plan of care

· Prep child & family surgery
· Avoid enema & laxatives
· IVF & IV ABX (avoid peritonitis)
· Avoid applying heat to abdomen

Appendicitis pre-op care: Non-ruptured

· IVF & ABX

· No pain meds

Appendicitis pre-op care: Ruptured

· Admin electrolyte & fluid replacement

· NGT for decompression

· IV ABX

Appendicitis post-op care: Non-ruptured

· Assess respiratory status, O2 if needed

· Obtain VS, admin analgesics

· Assess surgical site: bleeding

· Assess bowel sounds: if absent --> NPO

Appendicitis post-op care: Ruptured

· Assess respiratory status, O2 if needed

· Obtain VS, admin analgesics

· Assess surgical site: bleeding

· Assess bowel sounds & function

· IVF & IV ABX

· Maintain NPO, NGT to low continuous suction

· Assess for peritonitis

Appendicitis findings to report

- Peritonitis: fever, sudden relief from pain, then increase in pain, rigid abdomen/distention, tachycardia, pallor

- Rupture: NO PAIN!!

- Infection around incision site

Pyloric stenosis clinical manifestations

thickening of pyloric sphincter
- Vomiting following a feeding: can be projectile vomiting
- Blood-tinged vomit
- Constant hunger, irritable
- Hyperactive bowel sounds
- Olive-shaped mass on RUQ

Pyloric stenosis pre/post op care

Pre-op

- Prepare for surgery: pylorotomy

- NG tube for decompression, IVF

- NPO & monitor I/O

Post-op

- Antiemetics, analgesics (Tylenol)

- Advanced diet as indicated

* Breastfeed = straight to breast milk

* Bottle-feed: start with Pedialyte first

Intussusception clinical manifestations

one part of the intestine telescopes into another --> obstruction --> impairs blood flow
· Sudden episodic pain
· Screaming with drawing legs to chest
· Sausage-shaped mass in RUQ
· Stools that are mixed with blood & mucus - "currant jelly" consistency
· Vomiting, fever

Intussusception nursing plan of care

· Air enema with or without contrast: pushes bowel back into normal position
· Surgery for reoccurring cases: remove part of the bowel (emergency condition)

Enuresis (bedwetting) clinical manifestations

Primary: child has never been free of bed-wetting for any extended periods of time

Secondary: child who started bed-wetting after development of urinary control

Enuresis (bedwetting) assessment

· Family hx of enuresis
· Male
· Bladder dysfunction
· Emotional events(new sibling/divorce)
· Behavior disorders

Enuresis (bedwetting) Education

- evaluate self-esteem

- Void prior to bed; avoid caffeine

- Avoid fluids 2 hrs before bed

- Awaken child at regular intervals to void

Enuresis (bedwetting) complications

Emotional problems: low self-esteem, altered body image, social isolation

- Support child & family by listening to concerns & offer referrals

UTI clinical manifestations

- pain w urination (dysuria)

- abdominal or back pain

- increased thirst

- frequent urination

Seen from: bubble baths, tight underwear & more common in females than males

UTI nursing interventions/plan of care

· Encourage frequent voiding, empty completely at each void
· Monitor urine output & encourage fluids
· Mild analgesics, antipyretics PRN
· ABX

UTI complications

· Progressive kidney injury
· Pyelonephritis
· Urosepsis

Hypospadias clinical manifestations

· Urethral opening on ventral (underside) surface

· Meatus opening below glans penis

Hypospadias & epispadias nursing interventions

Pre-op

- Assist with maintaining self-image

- Focus on education & support

- NPO if needed

Post-op

- Assess pain, admin analgesics

- Anticholinergic to decrease spasm of bladder

- Monitor I/O

- Wound/dressing care

- Monitor for infection

Hypospadias & epispadias parent teaching

· Repairs done at 6-12 months, no later than 3 years

· No circumcision, foreskin used for surgical repair

· No tub baths for at least 1 week

· Limit activity

· HYGIENE!!!!

· Monitor for UTI’s

Epispadias clinical manifestations

surgery will be needed w/in 1st year of life

Male

- Widened pubic symphysis, urethral opening on dorsal side of surface

Female

- Wide urethra bifid clitoris

Both sexes

- Possible exstrophy of bladder

What is Nephrotic syndrome?

Alterations in the Glomerular alterations let proteins pass into urine

Nephrotic syndrome: clinical manifestations

- proteinuria, hyperlipidemia, hypoalbuminemia, edema
· Facial & periorbital (eyes) edema: decreased throughout day
· Weight gain over a period of days or weeks: ascites & edema to lower extremities/genitalia
· Muehrcke lines on fingernails
· Pallor, anorexia, diarrhea, irritability, vomiting
· Dark frothy urine
· BP within expected range or slightly below: HTN is rare

Nephrotic syndrome lab findings

UA/ 24 hr urine collection:

- Proteinuria > 2+ on dipstick: frothy urine d/t protein

- Few RBC;s

Serum chemistry:

- Hypoalbuminemia

- Hyperlipidemia

- Hemoconcentration: elevated H&H and PLT

- Possible hyponatremia

- GFR: normal OR high

Nephrotic syndrome nursing interventions

· Steroids: 80-90% respond

· IV albumin followed by furosemide: DO NOT give furosemide first

· Monitor edema and measure abdominal growth daily

· Restrict fluid & sodium

· Turn, cough, deep breathe

· Manage pain, promote rest and protect skin

Nephrotic syndrome pharm interventions

· Prednisone: decreases inflammation in kidneys & reduces the amount of protein lost in urine(take w meals and avoid crowds)
· Furosemide(increase potassium)
· 25% albumin: increases plasma volume & decreases edema
· Cyclophosphamide: for children who cannot tolerate prednisone or have repeated relapses

Nephrotic syndrome findings to report

· Sepsis/infection
· Manifestations worsen: relapse

Acute glomerulonephritis clinical manifestations

Inflammation & obstruction on filter of kidneys (glomeruli)

- smoky or cloudy, tea colored urine

- Mild-severe HTN

- Periorbital edema, facial edema: worse in morning and spreads to extremities and abdomen during the day

- Recent strep infection: most common cause

- Decreased urine output

- Irritability, lethargy, anorexia, ill appearance

Acute glomerulonephritis lab findings

· Throat culture: usually come back negative in strep

· Urinalysis: protein & hematuria

· Increased BUN/Cr: kidneys are impaired(REPORT a HIGH CREATININE!!)

· Decreased GFR

Strep antibodies

· ASO titer: positive

· Antihyaluronidase (Ahase)

· Antideoxyribunuclease B (ADNase-B)

Acute glomerulonephritis nursing interventions

· Monitor I/O, daily weight, neuro status, behavior changes (esp. with edema, HTN, & gross hematuria)
· Seizure precautions if needed
· Manage fluid restriction
· Monitor for skin breakdown: reposition, pad bony prominences, specialty mattresses, elevate edematous body parts
· Promote rest: cluster care
· Monitor & prevent infection: hand hygiene, ABX

Acute glomerulonephritis nutrition

· Possible restriction of sodium & fluid
· Restrict foods high in potassium during periods of oliguria
· Provide small, frequent meals of favorite foods d/t a decrease in appetite
· Managed at home if BP & urine output is normal

Acute glomerulonephritis pharm

Diuretics (Furosemide) & antihypertensives (Labetalol)

- Remove fluid & manage HTN

- Monitor VS & signs of hypokalemia

ABX: step infection

Phosphate binders: decreases absorption of phosphate

Sodium polystyrene sulfonate: corrects hyperkalemia

Acute glomerulonephritis findings to report

· Infection

· High creatinine

· Hypertensive encephalopathy: HA, seizures

· Circulatory overload

· Acute renal failure

Hemolytic uremic syndrome clinical manifestations

acute renal disease characterized by acute renal failure, hemolytic anemia and thrombocytopenia
· Loss of appetite, irritable, lethargic
· Hallucinations, stupor
· Edema, pallor
· Bruising, rectal bleeding
· Anuric or HTN = severe

Hemolytic uremic syndrome assessment

· Breakdown of RBC’s clog kidneys

· MED EMERGENCY

· 90% caused by E coli 0157:H7 (diarrhea & HUS)

· HUS usually follows a period of diarrhea & vomiting

Hemolytic uremic syndrome lab findings

· CBC: decreased Hgb/Hct & PLT
· Elevated reticulocyte count (baby/immature RBC's): body is trying to compensate
· Hematuria & proteinuria
· Elevated BUN/Cr
· Fibrin split products in serum & urine (thrombocytopenia)

Hemolytic uremic syndrome dietary/patient teaching

AVOID
· Undercooked meat
· Unpasteurized milk, juice & unwashed veggies
· Alfalfa sprouts
· Public pools

Vulvovaginitis clinical manifestations

· D/C
· Itching
· Pain
· Fishy smell

Vulvovaginitis nursing interventions/plan of care

· Admin metronidazole gel: intravaginally

· Avoid douching & tight-fitting clothing

Hyperglycemia clinical manifestations

HOT AND DRY BLOOD GLUCOSE TOO HIGH

· Blood sugar > 250 mg/dL

· Polyuria(excessive urine)

· Polydipsia (excessive thirst)

· Polyphagia (excessive hunger)

· Kussmal respirations

· Confusion, weakness, lethargy

· Mental changes: seizures &/or coma (extreme)

Hyperglycemia priority interventions

· Check blood sugar: admin insulin if needed
· Encourage fluid intake: correct hydration & acidosis
· Test urine for ketones & report if high

Hypoglycemia clinical manifestations

COLD AND CLAMMY GET SOME CANDY

- Blood sugar < 60 mg/dL

- Hunger, lightheadedness, shakiness, anxiety

- Difficulty thinking, slurred speech

Hypoglycemia priority interventions

- ABC

- tx with 15 g simple carb: 3-6 oz OJ or 8 oz milk

- Then recheck in 15 min:

* Still low? Carb again

* Normal? Give another complex carb and protein (PB, crackers)

- Unconscious or unable to swallow – admin glucagon IM or SQ

Type 1 diabetes patient teaching

- Trim toenails straight across & be careful when walking barefoot
- Examine eyes yearly & regular dental health
- Exercise plan:
* Team sports: snack 30 min prior
* Prolonged activities: food intake q45-60 min

Type 1 diabetes management

- Check blood sugar and urine test for ketones (risk for DKA) q 3hrs

- Continue taking insulin or oral antidiabetics

- Oral hygiene to prevent dehydration(Sugar-free, non-caffeinated)

Type 1 diabetes when to call provider

- BS > 240

- Positive ketones in urine

- Vomiting occurs more than once & liquids cannot be tolerated

Type 1 diabetes pharm: insulin

Rapid-acting (Lispro)

· Onset: 15 min

· Peak: 30-90 min

· Duration: 3-6 hr

Short-acting (Regular-Clear)

· Onset: 30 min-1 hr

· Peak: 1-5 hr

· Duration: 6-10 hr

Intermediate-acting (NPH-Cloudy)

· Onset: 1-2 hr

· Peak: 6-14 hr (if you give at 0800, worry about hypoglycemia around lunch to dinner time)

· Duration: 16-24 hr

Insulin admin

- DRAW up SHORTER first and then LONGER acting
- Cloudy to clear, clear to cloudy
- Rotate injection sites, inject at 90-degree angle
- Do not mix glargine
- Peak times: highest chance of hypoglycemia

DKA clinical manifestations

· Blood sugar > 330 mg/dL

· Fruity breath

· Mental confusion, dyspnea, dehydration

· Metabolic acidosis

· Kussmal respirations

Somatropin admin

· SQ injection 6-7 days a week
· Use cautiously in children receiving insulin: can cause hyperglycemia
· Gently mix, DO NOT shake

Somatropin patient teaching

continued until bone maturation takes place --> evidenced by epiphyseal closure

Wilms tumor (nephroblastoma) clinical manifestations

malignancy that occurs in kidneys or abdomen; metastasis rare

- dx before 5 years: around 2-3 years

- firm, painless abdominal mass/swelling: DO NOT PALPATE (can break up tumor)

Wilms tumor (nephroblastoma) plan of care

- surgical removal of tumor

- may need pre-op chemo &/or radiation

Post-op: closely monitor GI/GU, monitor for infection

Neuroblastoma clinical manifestations

malignancy of adrenal gland, head, neck, pelvis, or chest

- Occurs in toddler years & more common in males

- 1/2 of all cases have few findings and have been metastasized before dx (can be like a silent tumor)

Metastasis: S/Sx depend on location/stage of tumor:

- Periorbital ecchymosis

- Bone pain

- Proptosis: bulging eyes

Neuroblastoma plan of care

Surgical removal of tumor; chemo &/or radiation for metastasis

Neuroblastoma lab findings

· CBC, UA, coagulation studies
· Urine catecholamines (epi/norepei, vanillylmandelic acid, homovianilic acid
· CT of skull, neck, chest, abdomen, bone to locate tumor
· Bone marrow aspiration to r/o metastasis
· Scans of metalodobenzylguanidine (MIBG) to note bone marrow & soft tissue involvement
· Tumor biopsy

Leukemia assessment

· Malignancies that affect bone marrow & lymphatic system: peak onset 2-5 years
Two groups:
- Acute lymphoid leukemia (ALL) & acute myelogenous
- Acute Nonlymphoid leukemia (ANLL); ALL most common

Leukemia clinical manifestations: Early

· Low grade fever, pallor, HA

· Increased bruising & petechiae

· Enlarged liver/lymph nodes/joints

· Abdominal, leg, & joint pain

· Constipation, vomiting, anorexia

· Unsteady gait

Leukemia clinical manifestations: Late

· Hematuria

· Enlarged kidneys & testicles

· Manifestations of increased ICP

· Ulcerations in mouth

· Pain

Leukemia plan of care

CSF: determine CNS involvement

- Have child empty bladder

- Monitor for increased ICP

- Around the clock pain management

Bone marrow aspiration =MOST DEFINITIVE DIAGNOSTIC

Chemo:

· Common agents: vincristine, doxorubicin

· Central line or port

· Steroids: minimize A/E of Tx

Leukemia lab findings

· Immature WBC (blasts): elevated

· RBC, PLT, WBC (neutrophils): decreased

(RISK FOR INFECTION)

Chemotherapy nursing interventions

· Admin stem cells via IV infusion
· Ensure protective isolation: private & positive pressure room, 12 air exchanges/hr, HEPA filtration of incoming air
· Wear respiratory mask, gloves, & gown
· No plants/flowers

Chemotherapy pharm interventions

Ondansetron: antiemetic – given before chemo for N/V

Filgrastim: granulocyte colony-stimulating factor – stimulates WBC production by SQ admin daily

Chemotherapy lab findings

Pancytopenia: decreased RBC, WBC, & PLT

Leukocytopenia: decreased WBC

- Increased risk for infection

Erythrocytopenia: decreased RBCs

- Decreased oxygenation ability/fatigue

Thrombocytopenia: decreased PLT

- Bleeding/bruising

Chemotherapy patient teaching

· Avoid crowds, fresh fruits/veggies, & invasive procedures
· No aspirin or NSAIDs
· Watch for skin breakdown, infection, nutritional deficits
· Visit dentist before chemo
· Chlorhexidine mouthwash/salt rinse: for mucositis & dry mouth
· Scalp hygiene: hair grows back in 3-6 mo

Radiation post-procedure assessment

· Avoid invasive procedures
· Private room during nadir (lowest point)
· Provide comfort measures: pain management
· Monitor for infection, WBC, PLT counts

Radiation plan of care

· Dose calculated is delivered in divided treatments over several weeks
· Affects rapidly growing cells in body - cells that normally have fast turnover can be affected in addition to cancer cells
· Wear lead apron