1. Providing Patient Centered Care for the Child With an Endocrine Disorder

2. Pediatric Differences in the Endocrine 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?

3. 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

4. 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

5. Phenylketonuria Galactosemia Maple syrup urine Tay-Sachs disease

6. Phenylketonuria (PKU)  Autosomal recessive disorder that may result in damage to the brain  Hepatic deficiency  Treatment/education  Counseling for future pregnancies

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

8. 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

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

10. 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

11. Treatment for PKU

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

13. 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

14. 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

15. 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)

16. Tay-Sachs Disease  Signs & Symptoms Deafness Blindness Decrease muscle tone Loss of motor skills Delayed mental & social skills Dementia Paralysis Seizures

17. Nursing Measures for Metabolic Disorders  Genetic counseling  Dietary teaching and compliance  Mixing special preparations  Family support

18. Diabetes – Type 1 How is diabetes in children different from diabetes in adults?

19. 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?

20. 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

21. 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

22. Failure to produce insulin leads to elevated blood glucose HYPERGLYCEMIA

23. Polyuria Polydipsia Polyphagia Three P’s Clinical Manifestations: how do you identify polyuria in a toddler or young child?

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

25. 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**

26. 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 Family’s resources

27. Goals of Diabetic Management  Maintain normal serum glucose for age  Facilitate normal growth  Maintain normal lifestyle  Prevent complications

28. Treatment:  Insulin therapy  Regulation of nutrition and exercise  Stress Management  Blood glucose and urine ketone monitoring

29. Insulin Therapy What are the goals of insulin therapy?

30. 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

31. Types of Insulin  Rapid (Lispro/Humalog)  Short acting (regular)  Intermediate acting (NPH, Lente)  Long acting (Lantus/Ultralente)

32. 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

33.  Insulin Injections – usually 3 or more per day  Continuous Subcutaneous Insulin Pump Infusion Route of Administration

34. 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

35. 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

36. 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%.

37. 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

38. 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

39. Treatment and Nursing Care of the Diabetic Child

40. 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

41. 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

42. 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

43. 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

44. 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

45. 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

46. Hyperglycemia Blood Sugar > 160mg/dl Symptoms:  Polyuria  Polydipsia  Fatigue  Weight Loss  Blurred vision  Emotional lability  Headache Treatment:  Insulin  Increase oral fluids

47. Home Teaching  Incorporate into the family lifestyle  “Honeymoon phase”  Community resources  Recognizing the cognitive levels at time of teaching

48. 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

49. Exercise for the Child with Type I Diabetes Mellitis  Exercise Avoid exercising during insulin peak Add an extra 15 to 30g carbohydrate snack for each 45-60 minutes of exercise

50. Acanthoseis nigricans

51. For clarification or questions regarding this content please contact: Marlene Meador RN, MSN, CNE Professor of Nursing mmeador@austincc.edu