Pedi Unit 5: Metabolic & Sensory Impairments Study Terms

Tay-sachs Disease

Deficiency of enzyme for fat metabolism causing fat to accumulate around nerves

S/S usually noticed at 5-6 months old

S/S include: Motor delays, blindness, brain damage, cherry red spot on the optic nerve, mental retardation

Mortality less than 5 yrs due to secondary infections

Palliative care, no cure

MR =

Mental retardation

Phenylketonuria (PKU) (Page 349)

Faulty metabolism of amino acid phenylalanine causing a buildup

S/S: Severe mental retardation

Dx: Mandatory blood test by heel stick 48-72 hours after birth

Lifelong dietary restriction of natural protein

-See nutritionist very 2 weeks because amount of protein recommended will change while growing

Cannot be given normal formula, need to have a specific type for up to 1 year

Maple Syrup Urine Disease (Page 350)

Unable to metabolize leucine, isoleucine and valine (amino acids) which causes acidosis

S/S: Feeding different, loss of moro reflex, hypotonia, convulsions

Urine and sweat have a sweet maple smell d/t ketoacidosis

Treatment: Hydration and peritoneal dialysis and diet low in leucine, valine and isoleucine

If untreated death can occur within 2 weeks

Galactosemia (Page 351)

Body can’t utilize carbohydrate galactose and lactose

S/S are abrupt and worsen gradually: lethargy, vomiting, FTT

If not treated can result in: cirrhosis, cataracts and mental retardation

Treatment: Lactose free diet, life long

(Soy-based formula, no breastfeeding or dairy)

PKU =

Phenylketonuria

Moro reflex

Infant reflex where a baby will startle in response to a loud sound or sudden movement

(Present until 5-6 months old)

Hypotonia

Reduced muscle tone or tension

ADH =

antidiuretic hormone

Galactose converts to __________

glucose

Hypothyroidism (Page 725)

Deficient activity of the thyroid gland and secretions

Worrisome if dx before age 3 because it indicates a severe deficiency

S/S: Thick protruding tongue, wt gain, lethargy, hypotonia, dry skin, cold hands & feet, constipation

Early treatment important: Levothyroxine, Thyroid replacement

Diabetes Insipidus (DI)

Posterior Pituitary hyposecretion of ADH and vasopressin due to head trauma or a tumor

S/S: Uncontrolled diuresis, polydipsia, dry skin, excessive thirst,

Treatment: DDAVP

DDAVP

Synthetic form of vasopressin

Used in kids with Diabetes Insipidus & to help with bedwetting

NIDDM =

non-insulin dependent diabetes mellitus

Diabetes Mellitus (DM) (Page 727)

Insulin deficiency and impairment of glucose transport

Type I: IDDM autoimmune destruction of beta cells

Type II: NIDDM increased incidence d/t obesity

Gestational DM: During pregnancy, dx by glucose tolerance test

Teaching children and eliciting cooperation is essential

Diabetes Mellitus Type I (Page 727)

Seen in ages 5-7 and 11-13 yrs old

3 P’s: Polydipsia, Polyuria, and Polyphagia

Labs: FBS > 126 twice with family hx & Hgb A1C test

Ketoacidosis d/t high BS resulting from metabolic rate, growth, infection or non-compliance (will see ketones in urine)

Somogyi phenomenon: Hyperglycemia that occurs in the morning due to hypoglycemia at night

Treatment of DKA

Types of stresses on families

Diabetic Teaching

Age appropriate Insulin Management

-SC at 90’ best for kids

-More prone to reactions

-Site rotation

-S/S hyperglycemia and response and hypoglycemia and response

-Insulin pumps (insulin is given on a schedule)

-Effects of illness and exercise on BS

-Mixing Insulins

Diet: Controlled sugar, high fiber

Hyperglycemia S/S

Frequent urination

Deep rapid labored respirations

Thirst

Hunger

Dry mucous membranes

Weakness

Malaise

Rapid and weak pulse

Hypotension

Hypoglycemia S/S

Shakiness

Confusion

Diaphoresis

Palpitations

Headache

Lack of coordination

Blurred vision

Seizures

Coma

Somogyi phenomenon

A rebound phenomenon that occurs in clients with type 1 diabetes mellitus.

Normal or elevated blood glucose levels are present at bedtime; hypoglycemia occurs at about 2 to 3 am causing counter-regulatory hormones to be produced to prevent further hypoglycemia, which results in hyperglycemia

(evident in the pre-breakfast blood glucose level).

Treatment includes: decreasing the evening (predinner or bedtime) dose of intermediate acting insulin or increasing the bedtime snack

Otitis externa (Ch. 22)

Inflammation or infection externally (swimmers ear) tympanic membrane remains unaffected

S/S: Rubbing/pulling ear, hearing loss, loud speech. Inattentive behavior, fever, pain, drainage

Treatments: Irrigation, topical antibiotic, antiviral, ear drops, tubes in ears

Complications: Hearing Impairments

Dx: Brazelton Neonatal Behavioral Assessment, Tympanometry, Rinne test, Webber test

Otitis media (Ch. 22)

Inflammation or infection in the middle of the ear, common after a respiratory infection

S/S: Rubbing/pulling ear, hearing loss, loud speech. Inattentive behavior, fever, pain, drainage

Tx: Tubes in ears to help fluid drain

Complications: Hearing Impairments

Sensory Conditions Dx tests (Ch.22)

Brazelton Neonatal Behavioral Assessment

Tympanometry

Rinne test

Webber test

Hearing impairments (Page 542-543)

Classified according to where the problem is

Conductive: When tympanic membrane prevents sound from entering the middle ear, can be caused my impacted earwax, environmental toxins

Sensorineural: When hair cells along the cochlear and acoustic nerve may be damaged from toxins and/or loud noises

Combined: Both types of hearing loss due to loud noises and environmental toxins

Congenital: Hereditary as a result of a utero infection or meds taking

Dx: BAER assessment, Rinne test, Webber test

Treatments: Hearing Aids, Cochlear Implants, Sign language

Vision (Page 544)

Eyes develop at 4 weeks in utero

Newborn vision 20/400 (need to be right in their face for them to see you)

Eyes are crossed at birth d/t coordination weakness

-Improves by 3-6 months

Tears flow 1-3 months

Depth perception develops at 9 months

Amblyopia (Page 545)

“Lazy eye”

Loss of vision that occurs in children who strongly favor 1 eye

More common in 1 eye but can be bilateral

Tx: Eye glasses and patching the good eye to allow the bad eye to strengthen

Tx goal: obtain normal vision

Strabismus

“Crossed eyes”

The child is not able to look in both directions at the same object so the brain disables 1 eye to allow clear vision

Tx: Surgery, post-op elbow restraints are required to prevent the child from touching the eye, good lighting, larger print, and resting of the eye

Hyperopia:

Farsighted

Myopia:

Nearsighted

Conjunctivitis

Inflammation of the conjunctiva can be bacterial or viral

Bacterial is treated with antibiotics and is not contagious after 24 hours on antibiotics

Causes: Allergens, toxins, irritants

S/S: Thick drainage and redness or clear drainage

Tx: Antibiotics, warm water with baby shampoo

Hyphema

Pressure from blood in the anterior chamber in the eye

Cause: Blunt trauma

S/S: Bright red area on the lower portion of the iris

Tx: Bed rest with the HOB elevated 30-45 degrees to decrease intraocular pressure

NSAIDs are contraindicated because of bleeding

Retinoblastoma (Page 547)

Malignant tumor of the retina of the eye, can be hereditary or spontaneous

Average diagnosis: about 15-17 months for unilateral

S/S: "cat eye reflection" white reflex in the pupil due to a bubble behind the lens

Complications: vision loss, hyphema, pain

Tx: Unilateral removal of the eye, post-op care requires elbow restraints to reduce touching of the eye and than an eye patch

-When the socket is healed the pt will be measured for a prosthetic eye

Retinopathy of Prematurity (ROP) (Page 327)

Disorder of the developing retina in premature infants

Can lead to blindness due to receiving high levels of oxygen causing fibrous tissue to develop behind the lens of the eye

Tx: Retinal abrasion

Legal blindness

20/200 vision or less

ROP =

Retinopathy of Prematurity

Nursing considerations for a child with a visual impairment

Create safe environment: Use a clock system to tell them where things are, don't move their stuff w/o them knowing

Assistive devices: Magnifying glasses, dog, blind stick

Computer screen readers: Reads for you

Emotional/Behavioral Conditions

Role of stress in society

Parenting skills

Early childhood experiences linked to personality formation

Identifying kids at risk and early intervention is key

School psychologist and pediatricians are resources

Autism (Page 768)

Neurochemical imbalance that causes a child to have no interest in interactive play, no eye contact, no pretend play, poor attention span, lack response to name, stimming and tip-toe walking

Txs include: Structured environment, behavior modification and meds M-CHAT screening

Stimming: self-stimulation, repetitive motor movements

ADHD (Page 769)

Pts present with impulsivity, inattentive, and hyperactivity

More common in boys> girls

Learning disabilities can occur

Class room management is key: Sit in front of class, re-focus, breaks

Multi-disciplinary (don't know how to control their emotions)

Medications may alter alertness

ASD =

autism spectrum disorder

Stimming

Self-stimulation

Behavior consisting of repetitive actions or movements of a type that may be displayed by people with developmental disorders, most typically autistic spectrum disorders

M-CHAT screening

Stands for "Modified Checklist for autism in Toddlers"

This autism assessment that is a 20-question test that looks for multiple signs of ASD and assesses the risk in toddlers aged 16-30 months

Obsessive-Compulsive Disorder (Page 768)

Genetic origin that causes persistent, repetitive thoughts or actions (ritual) which become an obsession

-Children can often control

-Cognition and intellect not impaired

If not treated can lead to: Depression, psychiatric problems and suicide

Treatment

-Treated with Behavior modifications

-Medications that alter brain chemicals

-Determine if suicidal

Tardive dyskinesia

Involuntary movements of the facial muscles, tongue, and limbs

A possible neurotoxic side effect of long-term use of antipsychotic drugs that target certain dopamine receptors

Schizophrenia

Psychological disorder that is complicated to diagnose in children and is characterized by delusions, disordered thinking and/or speech, hallucinations, and impaired functioning

Interventions:

-Neuroleptic medications (side effects!)

-Therapy

-Involve the family in treatment

Nursing Considerations:

-Monitor compliance

-Watch for side effects **tardive dyskinesia

Neuroleptic medications are given to pts with Schizophrenia, what is a side effect?

Tardive dyskinesia

Bipolar Disorder

Marked by alternating mania and depression

Rapid cycling of mood changes

Children are highly emotional, or can be extremely irritated, distracted or extremely active, especially at bedtime.

Treated with Lithium **side effects

Nursing considerations:

-Monitor Lithium blood levels (0.5-0.8 Meq/L)

-Monitor adherance and response to treatment

-May take up to 7 days for therapeutic effect of Lithium

-Involve family

Lithium side effects

Severe nausea and vomiting

Hand tremors

Confusion

Vision changes

Serum TSH levels and renal function should be monitored

Anxiety and Depression

Prolonged behavior change affecting family, friends or school

S/S: Withdrawal, insomnia, truancy, lying, wt loss

Untreated can lead to: Substance abuse and possible suicide

Watch for: Physical and emotional S/S of bullying

Suicide

Third leading cause of death in teens

Check if they have a plan, means to do it and if they have support

Anxiety

The condition of feeling uneasy or worried about what may happen

Depression

A prolonged feeling of helplessness, hopelessness, and sadness

Truancy

skipping school/failure to attend school

Substance Abuse

Using Stimulants, Marijuana, Opiates, Hypnotics unprescribed

Alcohol and cigarettes precursor to taking more harmful agents

Children of alcoholics at greater risk

Children of alcoholics cope with flight, fight, perfect child or family savior

Stimulants: cocaine (crack, charlie, coke)

Marijuana: (joint, pot, hash, weed)

Opiates: Heroin (Horse, crap, smack)

Hypnotics: Barbiturates (goofballs, downers, barbs)

Anorexia Nervosa

Often over achievers, with high intelligence that have a fear of gaining weight, and altered self eval.

-Preoccupied with food

50% cured

30% improve

15% remain ill

Complications: Gastritis, cardiac arrhythmias, kidney and bowel problems

Bulimia

Uncontrolled binging followed by induced vomiting

Family dysfunctional

Often leads to electrolyte imbalance

Educate, identify, prevent and refer