Providing Patient Centered Care for the Child With an Endocrine Disorder
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?
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
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
Phenylketonuria Galactosemia
Maple syrup urine Tay-Sachs disease
Phenylketonuria (PKU)Phenylketonuria (PKU)
Autosomal recessive disorder that may result in damage to the brain
Hepatic deficiency
Treatment/education
Counseling for future pregnancies
Clinical Manifestations Musty or Mousey body and urine odor Irritability, hyperactivity Vomiting Hypertonia, hyperreflexivity
Complication Seizure disorder Untreatable mental retardation
Phenylketonuria (PKU)Phenylketonuria (PKU)
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
Goal of TherapyGoal of Therapy
MaintainMaintain
serum phenylalanine level serum phenylalanine level
between 2-6mg/dlbetween 2-6mg/dl
Treatment and Nursing Care for PKU
1. Special low-phenylalanine formula - Lofenalac, Minafen, and Albumaid XP
2. Diet low in phenylalanine – for life Avoid high-protein foods such as meats, fish,
eggs, cheese, milk, and legumes. Avoid aspartame
3. Take a phenylalanine-free protein supplement to maintain growth
4. Family support
Treatment for PKU
GalactosemiaGalactosemia
Carbohydrate metabolic dysfunctionRelated to a liver enzyme deficiency (GALT)Leads to accumulation of galactose metabolites in the eyes, liver, kidney and brain
Galactosemia: 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
Maple Syrup Urine DiseaseMaple 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
Tay-Sachs DiseaseTay-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)
Tay-Sachs DiseaseTay-Sachs Disease
Signs & Symptoms Deafness Blindness Decrease muscle tone Loss of motor skills Delayed mental & social skills Dementia Paralysis Seizures
Nursing Measures for Metabolic Nursing Measures for Metabolic DisordersDisorders
Genetic counseling
Dietary teaching and compliance
Mixing special preparations
Family support
Diabetes – Type 1Diabetes – Type 1How is diabetes in children different How is diabetes in children different
from diabetes in adults? from diabetes in adults?
What age is diabetes most likely detected in children?
Peak incidence is:5-7 years of agePuberty
What is significant about these ages? Can diabetes occur at any age?
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
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
Failure to produce insulin leads to elevated blood glucose
HYPERGLYCEMIA
Three P’s
Clinical Manifestations: how do you identify polyuria in a toddler or young child?
Other manifestations of hyperglycemia
Fatigue – unexplained Weight Loss (gradual, over several
weeks)Blurred visionHeadacheHunger
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**
Therapeutic Management
How does management of diabetes differ in the pediatric population from treatment in the adults? What factors influence treatment?
Age- developmental level- educationFamily’s resources
Goals of Diabetic Management
Maintain normal serum glucose for age
Facilitate normal growth
Maintain normal lifestyle
Prevent complications
Treatment:
Insulin therapy
Regulation of nutrition and exercise
Stress Management
Blood glucose and urine ketone monitoring
Insulin Therapy
What are the 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
Goals of Insulin Therapy
Types of Insulin
Rapid (Lispro/Humalog)
Short acting (regular)
Intermediate acting (NPH, Lente)
Long acting (Lantus/Ultralente)
Basal-bolus Therapy
ADA recommendations for childrenAdministrationAdministration
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
Insulin Injections – usually 3 or more per day
Continuous Subcutaneous Insulin Pump Infusion
Route of Administration
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
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
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%.
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 >CHO choices are consumed= adjust insulin dose
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
Treatment and Nursing Care of the Diabetic Child
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
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
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
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
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
Hypoglycemia:Blood Sugar < 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
HyperglycemiaBlood Sugar > 160mg/dl
Symptoms: Polyuria Polydipsia Fatigue Weight Loss Blurred vision Emotional
lability Headache
Treatment: Insulin Increase oral
fluids
Home Teaching
Incorporate into the family lifestyle “Honeymoon phase” Community resources Recognizing the cognitive levels at time of teaching
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
Exercise for the Child withType I Diabetes Mellitis
ExerciseAvoid exercising during insulin
peak
Add an extra 15 to 30g carbohydrate snack for each 45-60 minutes of exercise
Acanthoseis nigricans
For clarification or questions regarding this content please contact:
Marlene Meador RN, MSN, CNEProfessor of Nursing