1. Endocrine Disorders in the Pediatric Client Susan Beggs, MSN, CPN Susan Beggs, MSN, CPN

2. 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

3. Collecting data during an endocrine assessment  Percentiles on weight and height  Distinguishing facial features, abd. fat  Onset of puberty  Routine NB screening  Blood glucose levels  Detection of chromosomal disorders

4. Pancreatic dysfunctions  Etiology  Preclinical stage  Manifestations  Diagnosis

5. Therapy for diabetes in children  Diagnosis:  Under 18?  Type I diabetes  Clinical therapy combines:  insulin  nutrition  exercise regimen  psychosocial support

6. Insulin therapy

7. Insulin review  Rapid (Lispro/Humalog)  Short acting (regular)  Intermediate acting (NPH, Lente)  Long acting (Lantus/Ultralente)

8. Basal-bolus therapy  ADA recommendations for children  Basal insulin administered 1-2x day; bolus of rapid acting with each meal and snack  Method of this therapy:  Lower glucose levels  Stabilize glucose levels  Eliminate ketones  Insulin dose adjusted to BS readings 4x day

9. Basal bolus, cont.  BS monitored 4-8x day; 1x a week at midnight and 3AM  Therapy can be achieved with 3+ insulin injections a day or by pump  There must be consistent carb counts  Routine exercise

10. 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

11. External insulin infusion pump in children  Advantages  Delivers continuous infusion  Maintain better control  # of injection sites  hypo/hyper episodes  More flexible lifestyle  Eat with more flexibility  Improves growth in child  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  Infection may occur at site  Wt gain common when BS is controlled

12. Insulin therapy, cont.  Monitored every 3 months by hemoglobin A1c  Represents amt of glucose attached to hemoglb over period of time  Roughly 120 days  Good predictor of levels over 6-8 wks

13. Nursing Management at the time of diagnosis  Child is admitted to hospital  Nsg assessments directed toward:  Hydration  LOC  Hourly monitoring of BS  Dietary and caloric intake  Ability of family to manage

14. “Sick Day guidelines”  Days that child is ill  Attention to glycemic control  BS levels more often than routine  DO NOT SKIP INSULIN!  Factors key to preventing DKA

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

16. Diabetic Ketoacidosis  Review of patho  Causes  Criteria for diagnosis of DKA  BS levels> 300  Serum ketones  ↓ bicarbonates  Acidosis (pH <7.3)

17. Treatment for DKA  Fluids (boluses)  Wean off IV insulin when clinical stable  Oral feedings introduced when alert  Prevention of future episodes

18. Type II diabetes in children  There is insulin resistance  Fatty tissue produces hormone  Hormone desensitized to insulin  Can result in hyperinsulinism  Signs and symptoms

19. Acanthosis nigricans

20. Inborn errors of metabolism   Phenylketonuria   Galactosemia   Defects in Fatty Acid Oxidation   Maple syrup urine disease

21. Phenylketonuria (PKU)   Autosomal recessive   Liver deficiency   Treatment/education   Counseling for future pregnancies

23. Galactosemia  Carbohydrate metabolic dysfuntion  Autosomal recessive  Liver enzyme deficiency  Implications/symptoms  Treatment/management

24. Defects in fatty acid oxidation   Defects result in fatty acid oxidation   Most common of inborn errors   Most common presentation   Diagnosis/treatment

25. Maple syrup urine disease   (MSUD)   Disorder of amino acid metabolism   Diagnosis made by UA   Treatment/management

26. Nursing measures for metabolic disorders   Genetic counseling   Dietary teaching.compliance   Mixing special preparations   Mainly supportive