Transcript Sickle Cell Disease

Endocrine Stressors
and Adaptation
Common Pediatric
Endocrine Disorders
 Type I Diabetes
Congenital Hypothyroidism
Acquired Hypothyroidism
(Hashimoto’s Disease)
Hyperthyroidism (Graves disease)
Growth Hormone Deficiency
The Endocrine System
GlandsHormonesEndocrine Disorders are either:
Primary
Secondary
Hypofunction
Hyperfunction
Pediatric Differences in the
Endocrine System
The endocrine system is less developed
at birth than any other body system
Hormonal control of many body functions
is lacking until 12-18 months of age
Infants might manifest imbalances in
concentration of fluids, electrolytes, amino
acids, glucose, and trace substances
Type I Diabetes
Most common endocrine disorder
in children
Pancreas becomes unable to
produce and secrete insulin
Peak age: 5-7, or at puberty
Abrupt onset
Genetic link
Type 1 Diabetes
Beta cells- type of cell found in the Islets of
Langerhans within the pancreas that make
and release insulin.
Insulin is a hormone required to move the
glucose into cells throughout the body.
If no insulin can be produced, the glucose
stays in the blood instead, where it can
cause serious damage to all the organ
systems of the body.
Etiology
Autoimmune
process causes
destruction on
insulin-secreting
cells in the
pancreas
At dx 90% of beta
cells are destroyed
Type 1 Diabetes
No cure, but JDRF is funding studies
that perfect pancreas transplantation
and regeneration the body’s own beta
cells without islet transplantation
Serum Glucose
Levels
Normal:
70-110mg/dl
Glycosated Hemoglobin Hgb A1C
1.8 to 4.0 is normal
> 6.0 = DM
Diagnosis:
fasting: >126mg/dl
Random (non-fasting): ≥ 200mg/dl
with classic signs (next slide)
Signs & Symptoms
Polyuria
Polydipsia
Polyphagia
Fatigue
Blurred vision
Headache
Shortened
attention span
Mood changes
Diabetic
Ketoacidosis(DKA)
Medical Emergency
As glucose levels rise, child will
progress into DKA if not treated
Blood glucose levels > 300
Cellular starvation leads to ketone production
Nausea, vomiting, abdominal pain
Acetone (fruity) breath odor
Dehydration
Kussmaul respirations
Coma if untreated
When to Monitor for DKA
Abdominal pain
Nausea and vomiting that persists for over
6 hours
More than five diarrheal stools in 1 day
 A 1- or 2-day history of polyuria and
polydipsia
Has illness (e.g., viral or other) and is
unable to eat
Juvenile Diabetes
Treatment
Multidiscipline
Goal: Normal G & D, optimal glucose
control, minimal complications,
adjustment to disease
Treatment consists of:
Insulin replacement
Diet
BG monitoring
Exercise
Diet Therapy
Well-balanced, enough caloric
intake to support growth and
development
Three meals, snacks spaced
throughout the day
No diet foods
Don’t omit meals
Diet Therapy
No foods excluded, encourage
good nutritional choices
Learn dietary allowances
outside of home
Need to have consistent intake
& timing of food to correspond
to the time & effect of insulin
prescribed
Exercise
Encouraged, never restricted
Lowers blood glucose levels,
by aiding the body’s use of
food
Decreases insulin requirements
Proper snack before
Add an extra 15- to 30-g
carbohydrate snack for each 45-60
minutes of exercise
BG Monitoring
Glucose monitoring
Urine testing for ketones
Record keeping
Self-management at age appropriate
level
2-6 choose food, clean finger for BG
4-6 dip own urine
6-8 BGM
8-10 insulin injections, diary
10-14 nutritional decisions
12-18 full management
Insulin
Precise dose cannot be predicted
Amount is based upon average
capillary or serum blood glucose
levels
Will change based of G & D
Can be administered BID SQ by
needle/syringe, pen or by
insulin/portable pump
Types of Insulin
Synthetic Human Insulin
 Rapid acting
 Lispro (Humalog)
 Aspart (Novolog)
 Fast acting
 Regular ®
 Intermediate
 NPH (N)
 Mixed (70/30)
 Long Acting
 Glargine (Lantus)
 Ultra Lente
Typical Management
The peak of the insulin should
occur Post-Prandial (after meal) to
avoid hypoglycemia
Insulin
Alternate sites
Don’t inject
extremity to be
used in sports
Give at room
temperature
Always draw
regular up first if
mixing
Pumps
Delivers fixed amounts of short-acting
insulin continuously
Worn on a belt, the tubing & catheter
are changed Q48 hours and taped in
place
Should not be removed for > 1-2 hours
Subject to minimal malfunction
Self-motivated
Pumps
Advantages
Less scar tissue
No daily injections
Less to carry
Private
Sense of control
Disadvantages
Must wear continuously
Need to carry extra battery
Good BGM
If insurance dose not cover
$$$$$
Still need emergency
needles, insulin, and
remember how to inject
ILLNESS
Alters diabetic management
Dosage requirements may increase, decrease,
or remain unchanged depending on the
severity of the illness & the child’s appetite
Rapid-acting insulin used to manage
hyperglycemia associated with illness
Monitor fluids, may require extra oral fluids
while ill
“Sick Day” Guidelines
 Seek medical attention for fever or other signs of infection.
 Monitor the blood glucose levels more often than routine (1 to 4 hours).
 Test urine ketones when the blood glucose level is greater than 200 mg/dL.
 Do not skip doses of insulin.
 Large fluid intake (drinks with carbohydrates) is essential if the child cannot
eat as usual.
 If the child cannot consume adequate amounts of fluids, seek medical
attention.
Hyperglycemia BG > 160
Gradual onset
Lethargic
Polyuria
Polydypsia
Dulled sensorium, confused
Weakness, fatigue, lethargy
 Glucose 250 mg/dl
 Large ketones in blood & urine
 Blurred vision
 Ketoacidosis
 Coma
Hypoglycemia BG < 70
Commonly occurs before meals
when the insulin effect is
peaking
 burst of physical activity
without additional food,
delayed, omitted, or
incompletely consumed means
of snacks
Too much insulin-wrong dose
Signs & Symptoms
of Hypoglycemia
Rapid onset
Irritable, nervousness
Difficulty concentrating
Shaky feeling, tremors, hunger
Diplopia
Pallor
Weakness
Headache, dizziness
Sweating
Unconsciousness and convulsions
Treatment of
Hypoglycemia
Give simple concentrated sugar
Glucose gel or SL tablets
Hard candy
Sugar cubes
Low-fat milk or OJ
Followed by a complex CHO & Protein
Slice of bread or cracker with peanut butter
Glucagon SQ for severe hypoglycemia (may
cause vomiting, prevent aspiration)
Nursing Diagnosis
Risk for injury R/T
hypoglycemia or hyperglycemia
Fear R/T
diagnosis, insulin injection, negative effect
on life style
Risk for ineffective coping R/T
complex self-care regimen and uncertain
future
Imbalanced nutrition: more than body
requirements R/T
intake in excess of activity expenditures
Nursing Diagnosis
Risk for non-compliance R/T
complexity of regimen
Risk for ineffective therapeutic
management R/T
insufficient knowledge of condition
Knowledge deficit R/T
new health condition AEB questions being
asked
Altered family processes R/T
situational crises AEB uncertainty of chronic
disease/disability
Nursing
Considerations
Begins with survival education
Educate child & family regarding
Nature of disease, hypo/hyperglycemia
Meal planning (3 spaced meals, 3 snacks)
Wearing ID bracelet
Effective duration, onset & peak action of
insulin
Injection procedure, rotate sites
Glucose monitoring, urine testing, record
keeping
Exercise regime
Nursing
Considerations
Provide emotional support
Encourage growth and development
Identify home care needs
Disorders of the Thyroid
Congenital Hypothyroidism
Acquired Hypothyroidism
Hyperthyroidism
CONGENITAL
HYPOTHYROIDISM
Disorder at birth
Body is producing insufficient thyroid
hormone to meet metabolic needs
caused by absent or underdeveloped
thyroid gland
If not treated can lead to severe CI
Detected in Newborn Screen
Incidence and Etiology
Caused by defect in the embryonic period
in thyroid glad production
Also caused by inborn error of thyroid
hormone synthesis (an inherited
autosomal recessive trait)
 Can be secondary to pituitary dysfunction
Thyroid gland is unable to produce T3 and
T4
CONGENITAL
HYPOTHYROIDISM
Mottled skin
Large fontanel
Large tongue
Hypotonia/slow reflexes
Distended abdomen
Low T4 < 6, High TSH > 40
CONGENITAL
HYPOTHYROIDISM
An infant with a low T4 <6 and a
TSH value exceeding 40 mU/ml is
considered to have primary
hypothyroidism until proven otherwise
CONGENITAL
HYPOTHYROIDISM
 Treated with lifelong thyroid replacement therapy
 Synthroid 10-15 mcg/kg/day
Administration of increasing amounts over 4-8
weeks to avoid symptoms of hyperthyroidism
Taken 30-60 minutes before meals for optimal
absorption
 Monitor G&D and Thermoregulation
 Labs q 2 wks then q 3 mos *** look for upper range of
normal
 Medication compliance
 Teach parents to monitor for hyperthyroidism
Signs of Medication
induced Hyperthyroidism
Nervousness/anxiety
Diarrhea
Heat intolerance
Weight loss
Increased HR
Outcome
Prevention of cognitive impairment–
newborn screening on all babies
Early treatment has had significant impact
on morbidity
Most children progress to within normal
ranges on developmental assessment
Poor prognosis in more severe cases
Acquired Hypothyroidism
(Hashimoto’s disease)
Thyroid produces inadequate
levels of thyroid hormone > age 2
T4 decreases, TSH rises
Autoimune disorder
Antibodies and developed against thyroid
gland
Gland becomes inflamed, infiltrated by
antibodies and destroyed
Etiology
Primary (Hashimoto’s thyroiditis)
Most common
Autoimmune
Childhood, adolescents, females>males
Secondary
associated with other conditions that
affect the thyroid
Pituitary and hypothalmic dysfunction
Tertiary
Radiation, surgery, trauma
Acquired Hypothyroidism
Goiter
Dry, thick skin
Coarse but thinning
hair
Fatigue
Cold intolerance
Delayed puberty
and menses
Decelerated growth
Edema around eyes,
face and hands
Constipation
Sleepiness
Mental decline-not
permanent cognitive
impairment
Acquired Hypothyroidism
Treatment
 Thyroid hormone replacement-Synthroid
Starting dose 10 -15 mcg/kg/day
Administration of increasing amounts over 4-8 weeks to
avoid symptoms of hyperthyroidism
Taken 30-60 minutes before meals for optimal
absorption
Repeat thyroid function test one month should
see normalization of TSH
Requires lifetime follow up
Dose and adjustments based on clinical
evaluation & TSH
Prognosis is good if kept euthyroid (normal)
Acquired Hyperthyroidism
(Grave’s Disease)
A hyperfunction of the thyroid gland
Produces excessive circulating thyroid
hormone (T3 and T4)
Four times more common in girls
Occurs between the ages of
12 – 14 yrs. (puberty)
Manifestations develop gradually with
an interval between onset & diagnosis
of 6 to 12 months
Genetics involved
Follows a viral illness or period of stress
Grave’s Disease
Subjective Signs & Symptoms:
Emotional liability
Physical restlessness at rest
Decreased school performance
Excessive appetite without weight gain
Fatigue
Grave’s Disease
Physical Signs & Symptoms:
Increased HR
Palpitations
Widened pulse pressure
Exothalmos
Hair fine, unable to curl
Diarrhea
Poor attention span
Grave’s Disease
Physical Signs & Symptoms:
Wide-eyed expression with lid lag
Fine tremors
Increased perspiration/heat intolerance
Systolic murmurs
Emotional liability
Insomnia
Grave’s Disease
Thyroid Storm
Acute Onset
Severe irritability & restlessness
Vomiting and diarrhea
Hyperthermia
Hypertension
Severe tachycardia
Prostration
May progress to death
Grave’s Disease
Diagnosis:
Elevated thyroid function studies,
low TSH, high T4
Management:
To suppress thyroxine
PTU - propythioracil
MTZ – methimazole
Subtotal thyroidectomy
Ablation with radioiodine
Grave’s Disease
Nursing Care:
Quiet un-stimulating environment
conducive to rest
Maintain a regular routine to minimizing
stress of coping with unexpected demands
Physical activity is restricted
Tire easily, experience muscle
weakness and are unable to relax to
recoup their strength
Grave’s Disease
Nursing Care
Increased need for calories to meet their
metabolic rate
Offer 5-6 moderate meals throughout
the day, and vitamin supplements
Stress good hygiene because of excessive
sweating
Once therapy is instituted observe for side
effects of medications
Monitor for: Neutropenia, Hepatotoxicity,
Bone density
Grave’s Disease
Nursing Care:
If surgery is planned administer iodine a
few weeks before the procedure
Mixed in a strong-tasting fruit juice
given through a straw
Fear of having throat cut is real
Post-op position neck slightly flexed and
observe for bleeding
Supplemental thyroid hormone then for life
Hypothyroidism
Hyperthyroidism
Tiredness/fatigue
Nervousness/anxiety
Constipation
Diarrhea
Cold intolerance
Heat intolerance
Dry, thick skin
Smooth, velvety skin
Edema of face, eyes,
hands
Prominent eyes
Decreased growth
Accelerated linear growth
Decreased activity/energy Emotional liability
Muscle hypertrophy
Muscle weakness
Decreased heart rate
Increased heart rate
Growth Hormone
Deficiency
Failure of the pituitary to produce growth
hormone
Affected boys=girls
Boys tend to be evaluated more
75% cause is idiopathic
Can be a result of injury and destruction of
anterior pituitary gland from
Brain tumor
Infection
radiation
Symptoms
Normal size and weight at birth
Within first few years child will fall
below the 3rd percentile on growth
chart
Late onset of puberty
Delayed dentition
High-pitched voice
Child-like face with large forehead
Criteria for Suspecting
Growth Hormone (GH)
Deficiency
Consistently poor growth (<5
cm/yr)
Growth rate more than two
standard deviations below the
mean for age
Downward deviation from the
previous growth curve
Assessment and
Diagnosis
Evaluate family history
Prenatal/birth history R/O pituitary
tumor
Growth charts
Diagnosis
X ray, MRI to study bone age
Pituitary function tests
Management
IM recombinant human growth hormone
2-3 times per week
Given at bedtime when GH usually peaks
GH is a powder that needs to be mixed
with diluent
Parents/child need teaching
Rapid growth is often painful, pain
management is needed
Nursing Considerations
Speak to child in age appropriate
manner (be careful not to address as a
younger child)
Be discrete when providing step
stools, etc
Provide with anticipatory guidance for
adolescence
Dress in clothing that reflects age not size
Choose sports that height is not a requirement
Practice Questions!
A 10-year old type 1 diabetic client tells
the school nurse that he has some
early signs of hypoglycemia. The
nurse recommends that the child:
1.
2.
3.
4.
Take an extra injection of regular insulin
Drink a glass of orange juice
Skip the next dose of insulin
Start exercising
An adolescent with Type I diabetes has had
several episodes demonstrating lack of
diabetic control. The nurse teaches the
client by stating: “The best way to
maintain control of your disease is to:
1.
2.
3.
4.
Check your urine glucose three times a week
Check the HgA1C every 3 months and every 6
months when stable
Check your BG QID and HgA1C every 3 months
Check glucose daily as long as you feel well
A 10-year-old diabetic girl comes to the office of the
school nurse after recess. She was just out of
school for an extended illness and reports that
she returned to her usual insulin dosing schedule
today. The nurse notices she is nervous with hand
tremors, pale, sweaty, and complaining of
sleepiness. The nurse suspects:
1.
2.
3.
4.
Exercise-induced hypoglycemia
Hyperglycemia caused by increased intake at lunch
Ketoacidosis caused by infection
The child is avoiding returning to class
After being diagnosed with Hyperthyroidism, a
teenager begins taking PTU for treatment
of the disease. What symptom would
indicate to the nurse that the dose may be
too high?
1.
2.
3.
4.
Weight loss
Polyphagia
Lethargy
Difficulty with school work
The child’s 7:00 am blood glucose the following
morning is 189. At 5:30 pm: the child injected
rapid and NPH, then ate dinner and had a 10 pm
snack. The nurse concludes (select all that apply)
1. Rapid insulin dose may be to low
2. NPH insulin dose may be to low
3. NPH insulin dose may be to high
4. The child ate too little dinner
5. The child ate too much snack
 The nurse is teaching a parent of a child with type 1
diabetes about the different types of insulin. The nurse
assumes the parent understands rapid insulin peak
times if the parent states that after a 7:00 am injection,
be sure the child does not miss:
1. 6:45 AM Breakfast
2. 12:30 PM Lunch
3. 6:30 PM Dinner
4. 10:00 AM Snack