Providing Patient Centered Care for the Child With an Endocrine Disorder

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Providing Patient Centered Care for the Child
With an Endocrine Disorder

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Pediatric Differences in theEndocrine System

• Is the endocrine system fully developed at birth?
• When does hormonal control of body functions
  begin to take place?
• How do infants manifest imbalance in hormonal
  function?

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Understanding the Endocrine System in Children

• Puberty brings many changes
• ?GH released
• ? production of LH and FSH in females
• Development of sexual characteristics
• Feedback mechanism in place

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Collecting data during an Endocrine Assessment

• Percentiles on weight and height
• Distinguishing facial features, abdominal fat
• Onset of puberty
• Routine NB screening
• Blood glucose levels
• Detection of chromosomal disorders

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Inborn Errors Of Metabolism

• Phenylketonuria Galactosemia
• Maple syrup urine
• Tay-Sachs disease

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Phenylketonuria (PKU)

• Autosomal recessive disorder that may result in
  damage to the brain
• Hepatic deficiency
• Treatment/education
• Counseling for future pregnancies

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Phenylketonuria (PKU)

• Clinical Manifestations
• Musty or Mousey body and urine odor
• Irritability, hyperactivity
• Vomiting
• Hypertonia, hyperreflexivity
• Complication
• Seizure disorder
• Untreatable mental retardation

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Newborn Screening

• Required by state law
• Should not be done until feeding on breast milk
  or formula
• Should be done 48 hrs. after birth
• Repeat testing x2 and begin treatment as early as
  possible

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• Goal of Therapy
• Maintain
• serum phenylalanine level
• between 2-6mg/dl

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Treatment and Nursing Care for PKU

• Special low-phenylalanine formula - Lofenalac,
  Minafen, and Albumaid XP
• Diet low in phenylalanine for life
• Avoid high-protein foods such as meats, fish,
  eggs, cheese, milk, and legumes.
• Avoid aspartame
• Take a phenylalanine-free protein supplement to
  maintain growth
• Family support

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Treatment for PKU
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Galactosemia

• Carbohydrate metabolic dysfunction
• Related to a liver enzyme deficiency (GALT)
• Leads to accumulation of galactose metabolites in
  the eyes, liver, kidney and brain

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Galactosemia Clinical Manifestations Treatment

• Clinical Manifestations
• Poor sucking
• Failure to gain weight / IUGR
• Vomiting and diarrhea
• Hypotonia
• Cataracts
• Infections
• Treatment
• Lactose-free formula and diet

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Maple Syrup Urine Disease

• Disorder of amino acid metabolism
• Diagnosis made by UA
• Signs and Symptoms
• Poor appetite
• Lethargy
• Vomiting
• High-pitched cry
• Seizures
• Sweet odor of maple syrup in body fluids
• Treatment/management
• Removal of the amino acids and metabolites
• Diet low in proteins and amino acids

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Tay-Sachs Disease

• Deadly inherited disease
• No treatment
• Death by age 4
• Eastern European Ashkenazi Jews
• Absence of hexosaminidase A, a protein in nerve
  tissue which breaks down gangliosides (fatty
  substances that build up in nerve cells of the
  brain)

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Tay-Sachs Disease

• Signs Symptoms
• Deafness
• Blindness
• Decrease muscle tone
• Loss of motor skills
• Delayed mental social skills
• Dementia
• Paralysis
• Seizures

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Nursing Measures for Metabolic Disorders

• Genetic counseling
• Dietary teaching and compliance
• Mixing special preparations
• Family support

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Diabetes Type 1

• How is diabetes in children different from
  diabetes in adults?

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What age is diabetes most likely detected in
children?

• Peak incidence is
• 5-7 years of age
• Puberty
• What is significant about these ages?
• Can diabetes occur at any age?

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Emerging Trends of Diabetes

• Incidence of Type 1 diabetes increasing, the
  etiology is unknown. This trend is most apparent
  in very young children
• Obesity is causing increased incidence of Type 2
  diabetes in children and teens
• As children with chronic illness survive
  longer(i.e. cystic fibrosis)with more extreme
  measures and (i.e. transplants), diabetes becomes
  another side effect of their illness

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Etiology

• Autoimmune process
• Inflammatory process in the insulin secreting
  islet cells of the pancreas
• Destruction of the islet cells
• Failure to produce or excrete insulin

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Pathophysiology
Failure to produce insulin leads to elevated
blood glucose HYPERGLYCEMIA
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Clinical Manifestations how do you identify
polyuria in a toddler or young child?
Three Ps
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Other manifestations of hyperglycemia

• Fatigue unexplained
• Weight Loss (gradual, over several weeks)
• Blurred vision
• Headache
• Hunger

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Diagnosis Symptoms of diabetes plus Plasma
Glucose Levels (any of the following)

• Fasting plasma glucose 126 mg/dl
• Two-hour plasma glucose 200 mg/dl
  
• Random serum glucose concentration 200 mg/dl
  Ketonuria is a frequent finding

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Therapeutic Management

• How does management of diabetes differ in the
  pediatric population from treatment in the
  adults?
• What factors influence treatment?
• Age- developmental level- education
• Familys resources

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Goals of Diabetic Management

• Maintain normal serum glucose for age
• Facilitate normal growth
• Maintain normal lifestyle
• Prevent complications

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Treatment

• Insulin therapy
• Regulation of nutrition and exercise
• Stress Management
• Blood glucose and urine ketone monitoring

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Insulin Therapy
What are the goals of insulin therapy?
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Goals of Insulin Therapy

• Maintain serum glucose levels from
• Toddlers and preschoolers
• 100 180 before meals
• 110-200 at bedtime
• School-age
• 90- 180 before meals
• 100 180 at bedtime
• Adolescents
• 90 130 before meals
• 90 150 at bedtime

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Types of Insulin

• Rapid (Lispro/Humalog)
• Short acting (regular)
• Intermediate acting (NPH, Lente)
• Long acting (Lantus/Ultralente)

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Basal-bolus Therapy

• ADA recommendations for children
• Administration
• Basal insulin administered once a day Glargine
  (Lantus) or twice daily (Humulin or Ultralente)
• Bolus of rapid-acting insulin (Lispro or Aspart)
  given with each meal and snack or consumes
  carbohydrates

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Route of Administration

• Insulin Injections usually 3 or more per day
• Continuous Subcutaneous Insulin Pump Infusion

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External Insulin Infusion Pumpin Children

• Disadvantages
• Requires motivation
• Requires willingness to be connected to device
• Change sites every 2-4 days
• More time/energy to monitor BS
• Syringe, cath changes every 2-3 days

• Advantages
• Delivers continuous infusion
• Maintain better control
• ? of injection sites
• ?hypo/hyper episodes
• More flexible lifestyle
• Eat with more flexibility
• Improves growth in child

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Factors which may affect insulin dosage in
children

• Stress
• Infection
• Illness
• Growth spurts (such as puberty)
• Meal coverage for finicky toddlers
• Adolescents concerned about weight gain not
  wanting to eat AM snack

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Evaluation of Insulin Therapy

• Monitored every 3 months
• Draw glycosylated hemoglobin value (A1c)
• Want the glycosylated hemoglobin value (A1c) to
  be no higher than 7.5-8.

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Nutrition and Insulin Needs

• Children use carbohydrate counting
• 1 CHO choice 15 gm CHO
• Young children consume 2-4 choices /meal
• Older children and adolescents consume 6-8
  choices /meal
• 1 unit of insulin covers 8 Gm of CHO. Insulin
  dosing is based upon meal consumption and number
  of CHO choices
• If gtCHO choices are consumed adjust insulin dose

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About Insulin

• Store insulin in a cool, dry place do not freeze
  or expose to heat or agitation
• Check the expiration date on the vial before
  using
• Once opened, date the vial and discard as
  recommended
• When mixing two different types of insulin,
  inject the appropriate amount of air into both
  vials, then withdraw the short-acting (clear)
  insulin first

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Treatment and Nursing Care of the Diabetic Child
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Newly Diagnosed

• Many times ketoacidosis (DKA) is the cause of
  admission and the initial diagnosis occurs
• Signs of DKA
• Signs of hyperglycemia plus
• Abdominal pain / Stomachache
• Nausea and vomiting
• Acetone (fruity)breath odor
• Dehydration
• Increasing lethargy
• Kussmaul respirations
• Coma

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Treatment for DKA

• IV Fluids (boluses)
• IV insulin - Wean off IV insulin when clinically
  stable
• Electrolyte replacement
• Oral feedings introduced when alert
• Prevention of future episodes

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Nursing Management at the time of diagnosis

• Child is admitted to hospital
• Nursing assessments
• Vital Signs
• LOC
• Hydration
• Hourly monitoring of BS
• ____________________________________
• Dietary and caloric intake
• Family coping/adaptations

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Focus of Child and Parent Education

• Signs and symptoms of hypoglycemia and
  hyperglycemia and related treatment
• Blood-glucose monitoring / urine ketone
  monitoring
• Administration of insulin
• Sick day guidelines
• Nutrition

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Sick Day Guidelines

• Monitor serum glucose levels more often
• Test urine ketones when blood glucose is high
• Do NOT skip doses of insulin
• Adjust dose of insulin to serum glucose
• Encourage increased fluid intake

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HypoglycemiaBlood Sugar lt 70mg/dl

• Symptoms
• Trembling
• Sweating, clammy skin
• Tachycardia
• Pallor
• Personality change/ irritable
• Slurred speech

• Treatment
• 15g carbohydrate
• Glucagon sub-q
• IV glucose
• OJ, sweet beverage, raisins,
• cheese and crackers, candy

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HyperglycemiaBlood Sugar gt 160mg/dl

• Symptoms
• Polyuria
• Polydipsia
• Fatigue
• Weight Loss
• Blurred vision
• Emotional lability
• Headache

• Treatment
• Insulin
• Increase oral fluids

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Home Teaching

• Incorporate into the family lifestyle
• Honeymoon phase
• Community resources
• Recognizing the
• cognitive levels
• at time of teaching

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Nutrition for the Child withType I Diabetes
Mellitis

• Meals and snacks are balanced with insulin action
• Both the timing of the meal or snack and the
  amount of food are important in avoiding
  hyperglycemia or hypoglycemia
• Adherence to a daily schedule that maintains a
  consistent food intake combined with consistent
  insulin injections aids in achieving metabolic
  control

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Exercise for the Child withType I Diabetes
Mellitis

• Exercise
• Avoid exercising during insulin peak
• Add an extra 15 to 30g carbohydrate snack for
  each 45-60 minutes of exercise

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Acanthoseis nigricans
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• For clarification or questions regarding this
  content please contact
• Marlene Meador RN, MSN, CNE
• Professor of Nursing
• mmeador_at_austincc.edu