Transcript Sickle Cell Disease

Endocrine
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
• Hypofunction
• Primary
• Secondary
• Hyperfunction
• Primary
• Secondary
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 1 Diabetes
Type 1 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 fasting glucose 70-110mg/dl
• Glycosated Hemoglobin (Hgb A1C) < 6.0%
Initial Clinical Manifestations
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
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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
Type 1 Diabetes Treatment
• Multidiscipline
• Promote normal growth and development
• Optimal glucose control
• Minimal complications
• Adjustment to disease
• Treatment consists of
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Insulin replacement
Diet
Blood glucose monitoring
Exercise
Diet Therapy
• Consistent intake & timing of food to
correspond to insulin prescribed
• Well-balanced, enough caloric intake
to support growth and development
• Three meals, snacks spaced
throughout the day
• No diet, sugar-free foods
• Don’t omit meals
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
Blood Glucose Monitoring
Finger stick glucose monitoring several times a
day
Urine testing for glucose and ketones
Teach good record keeping
Self-management at age appropriate level
2-6 choose food, clean finger for stick
4-6 dip own urine
6-8 Blood glucose management
8-10 insulin injections, keep 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 growth
Can be administered twice daily
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
• Must be self-motivated
Pumps
• Advantages
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Less scar tissue
No daily injections
Less to carry
Private
Sense of control
• Disadvantages
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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 only is 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 > 110
Gradual onset
Lethargic, fatigue
Confused
Weakness
Polyuria
Polydypsia
Polyphagia
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 meals or snacks
Too much insulin-wrong dose
Hypoglycemia BG < 70
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 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
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 cognitive impairment
• Detected in Newborn Screen
CONGENITAL HYPOTHYROIDISM
• 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
Clinical Manifestations
• 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 is
considered to have primary
hypothyroidism until proven
otherwise
Treatment
• Treated with lifelong thyroid replacement
therapy
• Synthroid 10-15 mcg/kg/day
• Taken 30-60 minutes before meals for
optimal absorption
• Crush pill and mix with formula or breast
milk
Parental Education
• Monitor Growth and Development and
Thermoregulation
• Labs every 2 weeks then every 3 months
look for upper range of normal
• Stress medication compliance
• Teach parents to monitor for medication
induced 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)
Acquired Hypothyroidism
• Autoimune disorder
• Antibodies and developed against
thyroid gland
• Gland becomes inflamed, infiltrated by
antibodies and destroyed
• Thyroid produces inadequate levels of
thyroid hormone > age 2
• T4 decreases, TSH rises
Types of Acquired
Hypothyroidism
• 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
Clinical Manifestations
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Decelerated
• Goiter
growth
• Dry, thick skin
• Edema around
eyes, face and
• Coarse hair
hands
• Fatigue
• Constipation
• Cold intolerance
• Sleepiness
• Delayed puberty and • Mental declinenot permanent
menses
cognitive
impairment
Acquired Hypothyroidism
Treatment
• Thyroid hormone replacement-Synthroid
• Starting dose 10 -15 mcg/kg/day
• Taken 30-60 minutes before meals for optimal
absorption
• Teach child to swallow pill or crush
• 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)
Acquired Hyperthyroidism
• 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
Clinical Manifestations
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Emotional liability
Physical restlessness at rest
Decreased school performance
Excessive appetite without weight gain
Fatigue
Hair fine, unable to curl
Diarrhea
Poor attention span
Insomnia
Increased perspiration/heat intolerance
Clinical Manifestations
• Increased HR
• Palpitations
• Widened pulse pressure
• Exothalmos
• Wide-eyed expression with lid lag
• Fine tremors
• Systolic murmurs
Thyroid Storm
• Acute Onset
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Severe irritability & restlessness
Vomiting and diarrhea
Hyperthermia
Hypertension
Severe tachycardia
Prostration
May progress to death
Treatment
• To suppress thyroxine
• PTU - propythioracil
• MTZ – methimazole
• Subtotal thyroidectomy
• Ablation with radioiodine
Nursing Care
• Needs 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
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
• If taking PTU or MTZ observe for side effects of
medications
• Neutropenia and Hepatotoxicity
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
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 with type 1 diabetes tells the
school nurse that he has some early signs of
hypoglycemia. The nurse recommends that
the child:
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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:
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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 four times a day and HgA1C every 3
months
Check glucose daily as long as you feel well
A 10-year-old girl with type 1 diabetes 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 feeling
drowsy. The nurse suspects:
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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?
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Weight loss
Polyphagia
Lethargy
Difficulty with school work
A child with type 1 diabetes 7:00 am blood
glucose is 189. The previous evening at 5:30 pm
the child injected rapid insulin and NPH, 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 when injecting at 6:45 am, be
sure the child does not miss:
1. 7:00 AM Breakfast
2. 12:30 PM Lunch
3. 6:30 PM Dinner
4. 10:00 AM Snack