Adrenal Insufficiency in Children

Transcription

1 Adrenal Insufficiency in Children Stephanie Hsu, MD, PhD Assistant Professor Director of Quality Improvement and Patient Safety Pediatric Endocrinology Children s Hospital Colorado and the Barbara Davis Center for Diabetes 1 Conflicts of Interest I have no commercial ties and no conflicts of interest 2 Outline and Objectives Adrenal physiology Adrenal insufficiency Stress dosing 3 1

2 Adrenal Glands Vital organs Located above the kidneys Each layer has a specific job 4 Layers of the Adrenal Gland Unique enzymes to make unique hormones Different signaling pathways zg zf zr SALT SUGAR SEX Aldosterone Cortisol Testosterone 5 Cortisol Pathway Increase blood pressure Release sugar stores Suppress inflammation Critical in times of STRESS 6 2

3 Adrenal Insufficiency: Primary The problem is in the adrenal gland itself Severe, as there is no way to make the hormones Requires daily replacement of missing hormones REQUIRES increased replacement during times of stress zg zf zr X X SALT SUGAR SEX Aldosterone Cortisol Testosterone 7 Examples of Primary Adrenal Insufficiency Autoimmune destruction (Addison s disease) Congenital adrenal hyperplasia (CAH) Other pathology (infections, hemorrhage, trauma, etc.) 8 Adrenal Insufficiency: Secondary The problem is in the signaling pathways (ACTH) May require daily replacement of missing hormones REQUIRES replacement during times of stress X zg zf zr SALT SUGAR SEX Aldosterone Cortisol Testosterone 9 3

4 Examples of Secondary Adrenal Insufficiency Iatrogenic (chronic steroid exposure) Congenital abnormality of the brain Septo-Optic Dysplasia Ectopic posterior pituitary Tumor or surgery near pituitary Radiation to the brain 10 Symptoms of Untreated Adrenal Insufficiency Primary or Secondary: Weakness Weight loss Abdominal pain Diarrhea or constipation Syncope Primary only: Salt craving Pigmentation of skin 11 Diagnosis of Adrenal insufficiency -Symptoms can be subtle -Must have high index of suspicion -Secondary adrenal insufficiency often presents milder unless there is a stress situation 12 4

5 Daily Replacement Cortisol replacement: Hydrocortisone 2-3x per day Prednisone/Dexamethasone daily Harder to titrate to not affect growth Aldosterone replacement: Salt Fludrocortisone Balance enough cortisol without impairing growth 13 Adrenal Crisis Occurs in times of physical stress Lethargy, dehydration Low Blood pressure not responsive to fluid Lab Findings Low BG Low Na High K Shock Death 14 Goal : Prevent Adrenal Crisis! Recognizing risks (stress) Promptly giving steroids 15 5

6 Stress Dosing Indications Vomiting (even once) Diarrhea Fever >100.4 degrees F Dehydration Lethargy Fainting Surgical procedure involving anesthesia Significant injury (i.e. broken bone, concussion, etc.) 16 Stress Dosing Instructions Mild/moderate stress AND able to take oral medications Oral steroids based on size Acetaminophen or Ibuprofen OK Does not usually need EMS >Moderate stress OR unable to take oral medications IM Solu-Cortef (Hydrocortisone) 5-10 years: 50 mg IM >10 years: 100 mg IM lasts 4-6 hours Call EMS Can t overdose 17 How fast do you have to give the stress doses? As soon as the stress is recognized Must travel with kids just like an Epi-Pen Likely up to minute delay is ok, but the sooner the better Check with local EMS, as some districts do not have IM solu-cortef in their approved medication list Some districts will allow Methylprednisolone but not hydrocortisone Contact Endo Doc to get stress dose changed to Methylpred 18 6

7 Example of a Student Health Plan for Adrenal Insufficiency Adrenal insufficiency is caused when adrenal glands can not make enough cortisol. Student takes daily maintenance dose of Cortisol to replace the hormone. If the child is getting sick, fever, mild injury, they will need a STRESS dose of Cortisol and parent MUST pick child up from school. In the case of severe injury or illness, the child could experience an adrenal crisis which could lead to shock. If severe illness or injury occurs, the child will require an intramuscular (IM) injection of Solu-Cortef and emergency support from 911 personnel as soon as possible. 19 Interventions on Health Plan Adrenal Stress mild-moderate symptoms: abdominal pain, acute illness, broken bone suspected, dehydration, dizziness, diarrhea, fatigue or severe weakness, fever, headache, head trauma, unusual and excessive sweating on face or palms, vomiting -Give oral stress dose of hydrocortisone 15 mg (3 tabs) immediately -Call parent to notify of illness or injury immediately and request pick up from school -Monitor student in clinic until parent picks up -If student vomits within 20 minutes of oral stress dose, give another oral stress dose 20 Interventions on Health Plan Adrenal Crisis symptoms: bone broken through skin or multiple broken bones, child appears to be in shock, extremely pale, loss of consciousness, severe dehydration, severe head injury, repeated vomiting -Call 911 immediately, notify them of Adrenal Crisi -Give IM dose of Solu-Cortef (50 mg, 100 mg) some doses may be partial as the vial contains 100 mg/2ml -Have student lie down with feet elevated if able -Keep student warm with blankets until EMS arrives -Give Solu-Cortef vial, dosage and time of dosage to paramedics that respond to the call 21 7

8 Field Trips Current HCP should accompany student on all field trips. Supervising staff will review this Student Health Plan. Trained and delegated staff will provide necessary health interventions as identified by the Student Health Plan. Parents will be notified prior to field trips. 22 Health Problem: MAJOR illness or stress Goal: Prompt recognition of symptoms indicating need for Solu-Cortef Action: If unable to take oral medication, severe dehydration, illness with continuous vomiting or diarrhea, accidents with multiple broken bones, internal injuries or unconsciousness. 1. Administer 100 mg of Solu-cortef intramuscularly. Solu-Cortef is stored in health office and coach fanny pack. Instructions for the Solu-Cortef Act-O-Vial (100mg/2ml) a. Press down the plastic activator on the Solu-Cortef Act-O-Vial so that the liquid enters the powder section. b. Mix gently by shaking or rolling until the mixture is clear. c. Remove plastic tab covering center of stopper d. Clean the rubber stopper with alcohol or water. e. Stick the needle through center of the rubber stopper until tip is just visible. Then turn the bottle upside down (with the needle still in it) and draw up 100 mg/2ml of the mixture into the syringe. f. Inject into a muscular part of the body, such as the thigh, buttocks or upper arm. Individual Health Care Plan Template Commonly asked questions It is never wrong to start stress dose steroids, always start when unsure Parents should not be told to double/triple doses should always be provided a with specific stress dose plan 24 8

9 Caveats for Iatrogenic Adrenal Insufficiency They don t always look Cushingoid May have signs of glucocorticoid excess as well as adrenal insufficiency depending on dosing scheme and ½ life of exogenous glucocorticoid For example, may have sx of adrenal insufficiency on alternate days when dose is dropped from daily to qod May be adrenally suppressed enough to stop growth but not receiving enough to handle a major stress 25 TAKE HOME POINTS Multiple reasons for adrenal insufficiency Adrenal crisis is life threatening Giving steroids is the treatment (everything else is secondary) One high dose cannot hurt and can save a life 26 HOW TO CONTACT US Phone to speak with Endocrine nurses Fax for school forms of patients cared for by our group 27 9

10 Thank You For Caring for Our Students! 28 10