PEDIATRIC ENDOCRINE DISORDERS

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 months of age  Infants might manifest imbalances in concentration of fluids, electrolytes, amino acids, glucose, and trace substances

Understanding the Endocrine System in Children  Puberty brings many changes ↑ GH released ↑ production of LH and FSH in girls  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 (PKU)  Genetic metabolic disorder characterized by absence of enzyme  Phenylalanine hydroxylase to breakdown phenylalanine.  As a result, excessive phenylalanine builds up in the blood stream causing permanent damage to brain

 Clinical Manifestations Musty or Mousey body and urine odor Irritability, hyperactivity Vomiting Hypertonia, hyperreflexivity  Complication Seizure disorder Untreatable mental retardation Phenylketonuria (PKU)

Goal of Therapy Keep serum phenylalanine level at 2-6mg/dl

Newborn Screening  Required by state law  Should not be done until feeding on breast milk or formula  Should be done ~48 hrs. after birth  If test shows elevated levels of plasma phenylalanine, repeat test done and if that is elevated, treatment started.

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

Galactosemia Maple syrup urine Tay-Sachs disease

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

 Signs and Symptoms Poor sucking Failure to gain weight / IUGR Vomiting and diarrhea Hypotonia Cataracts Infections  Treatment Lactose-free formula and diet

 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

Nursing Measures for Metabolic Disorders  Genetic counseling  Dietary teaching and compliance  Mixing special preparations  Mainly supportive

Diabetes Inability of the body to produce or excrete insulin

When are Children most likely to be diagnosed with Diabetes?  Peak incidence is: 5-7 years of age Puberty It can 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

Polyuria Polydipsia Polyphagia Three P’s Clinical Manifestations

polyuria How would you tell polyuria in a toddler? Answer: Enuresis in a toilet-trained child

Other manifestations of hyperglycemia  Fatigue – unexplained Weight Loss (gradual, over several weeks) Blurred vision Headache Hunger

Diagnosis Symptoms of diabetes plus Plasma Glucose Levels of: Fasting plasma glucose ≥ 126 mg/dl or Two-hour plasma glucose ≥200 mg/dl or Random serum glucose concentration ≥200 mg/dl **Ketonuria is a frequent finding**

Therapeutic Management Managed and educated by a multidisciplinary team of experts in pediatric diabetes

Goals of Diabetic Management

These Goals are Met by:  Insulin Administration  Regulations of nutrition and exercise  Stress Management  Blood glucose and urine ketone monitoring

Insulin Therapy Goal of Insulin Therapy is to replace the insulin the child is no longer able to make thereby: Lower blood glucose levels Stabilize glucose levels Eliminate ketones

Maintain serum glucose levels from:  Toddlers and preschoolers 100 – 180 before meals at bedtime  School-age 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 childrenAdministration  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 Pump in 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 (A 1c )  Want the glycosylated hemoglobin value (A 1c ) 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. So 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

Newly Diagnosed  Many times the newly diagnosed child is admitted to the hospital in ketoacidosis (DKA) 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 directed toward: Vital Signs LOC Hydration Hourly monitoring of BS ____________________________________ Dietary and caloric intake Ability of family to manage

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 blood glucose levels more often  Test urine ketones when blood glucose is high  Do NOT skip doses of insulin  Usual doses of insulin may be increased  Encourage large fluid intake,

Hypoglycemia: Blood Sugar < 70mg/dl Symptoms:  Trembling  Sweating, clammy skin  Tachycardia  Pallor  Personality change/ irritable  Slurred speech Treatment:  15g carbohydrate  Glucogon sub-q  IV glucose  OJ, sweet beverage, raisins, cheese and crackers, candy

Hyperglycemia Blood 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 with Type 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 with Type I Diabetes Mellitis  Exercise Avoid exercising during insulin peak Add an extra 15- to 30-g carbohydrate snack for each minutes of exercise