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HNI 464 – Pediatrics Fall 2011 By Hector Chavez ADDISON’S DISEASE
Why I Chose This Topic …
What is Addison’s Disease? <ul><li>Named for British physician Thomas Addison in the mid-1800’s. </li></ul><ul><li>An endo...
Pathophysiology <ul><li>Damage to the adrenal  cortex ; progressive atrophy of gland. </li></ul><ul><li>Deficiency in cort...
What Causes It? <ul><li>PRIMARY CAUSE : Immune system mistakenly attacking the gland. </li></ul><ul><li>Secondary causes :...
Signs & Symptoms <ul><li>Usually develops slowly; early signs often go unnoticed. </li></ul><ul><li>Weakness, fatigue, let...
Diagnostic Tests <ul><li>Laboratory Tests </li></ul><ul><ul><li>Low blood pressure </li></ul></ul><ul><ul><li>Increased po...
Treatment <ul><li>Lifetime hormone replacement therapy (oral or IM) </li></ul><ul><ul><li>Expensive </li></ul></ul><ul><ul...
Nursing Diagnoses <ul><li>Deficient Fluid Volume related to hypovolemia secondary to adrenal insufficiency. </li></ul><ul>...
Planning & Implementation <ul><li>The Hospitalized Patient </li></ul><ul><li>Encourage fluid intake and administer IV hydr...
Evaluation <ul><li>Expected outcomes in a pediatric population: </li></ul><ul><li>The child maintains hemodynamic stabilit...
References <ul><li>A.D.A.M., Inc. (2009, November 25).  Addison's disease . Retrieved November 23, </li></ul><ul><li>2011,...
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Addison's Disease

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A presentation made to my pediatrics clinical group at Good Samaritan Hospital. Stony Brook University School of Nursing, class of 2012.

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  • Abdominal x-ray and CT scans are usually useless in the initial stages or AI. Even when damage is severe, the adrenal cortex is so thin (5 mm) that adrenal atrophy is hard to detect.

    UFC is VERY UNRELIABLE in determining low cortisol as low cortisol and normal cortisol in urine have very similar ranges.

    DYNAMIC ENDOCRINE TESTING IS THE ONLY WAY TO DETERMINE IF THERE IS ADRENAL OR PITUITARY DAMAGE. The 1 ug or 250 ug ACTH stimulation test or Insulin Tolerance Test are the ONLY way to determine the adrenal's capability to respond to stress. Other tests are anecdotal.

    Other helpful testing prior to diagnosis and hormone replacement are aldosterone, renin, DHEA-S, testosterone and progesterone in the case of primary adrenal insufficiency. If secondary adrenal insufficiency is suspected, a pituitary MRI with contrast, full pit panel, dynamic GH testing and DHEA-s are helpful in determining the extent of the damage.

    IM injections of Solu-Cortef (the preferred emergency replacment steroid) should only be used when necessary because IM injections of corticosteroids can cause hip and shoulder ball necrosis.

    Oral hydrocortisone is NOT expensive.

    Low blood pressure treatment is IV fluid replacment.

    Prognosis for a normal life is ONLY possible if comorbid conditions are regularly evaluated and treated, if ALL possible adrenal horomones are replaced and if steroid replacement is physiological. CELIAC regularly accompanies AI and MUST be tested.

    Nursing Diagnoses: extreme weight loss and body image. Very important. Pigmentation in primary AI will resolve with proper cortisol replacement.

    You might want to read the Lancet's Adrenal Insufficiency by Arlt and Alliolo
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  • The cortex produces THREE types of hormones: The glucocorticoid hormones (such as cortisol) maintain sugar (glucose) control, decrease (suppress) immune response , and help the body respond to stress. The mineralocorticoid hormones (such as aldosterone) regulate sodium and potassium balance. The sex hormones, androgens (male) and estrogens (female), affect sexual development and sex drive.
  • Treatment with replacement corticosteroids will control the symptoms of this disease. However, you will usually need to take these drugs for life. People often receive a combination of glucocorticoids (cortisone or hydrocortisone) and mineralocorticoids (fludrocortisone).
  • Hemodynamic stability: Family education is very important:
  • Transcript of "Addison's Disease"

    1. 1. HNI 464 – Pediatrics Fall 2011 By Hector Chavez ADDISON’S DISEASE
    2. 2. Why I Chose This Topic …
    3. 3. What is Addison’s Disease? <ul><li>Named for British physician Thomas Addison in the mid-1800’s. </li></ul><ul><li>An endocrine disorder characterized by the inability of the adrenal glands to produce enough of its hormones. </li></ul><ul><li>Relatively rare, yet affects men and women equally. </li></ul><ul><li>Also known as adrenal insufficiency </li></ul>
    4. 4. Pathophysiology <ul><li>Damage to the adrenal cortex ; progressive atrophy of gland. </li></ul><ul><li>Deficiency in cortisone and aldosterone </li></ul><ul><li>Imbalance of these hormones causes deregulation of important body functions that are potentially life-threatening. </li></ul><ul><li>Disease manifests itself when 90% of the adrenal gland is destroyed. </li></ul><ul><li>80% of cases have an autoimmune origin, 20% result from infection. </li></ul>
    5. 5. What Causes It? <ul><li>PRIMARY CAUSE : Immune system mistakenly attacking the gland. </li></ul><ul><li>Secondary causes : Infections such as tuberculosis, HIV, fungal infections, tumors, use of blood-thinning drugs (anticoagulants). </li></ul><ul><li>Comorbidity with other autoimmune diseases, such as: </li></ul><ul><li>Chronic thyroiditis </li></ul><ul><li>Graves' disease </li></ul><ul><li>Hypoparathyroidism </li></ul><ul><li>Hypopituitarism </li></ul><ul><li>Myasthenia gravis </li></ul><ul><li>Pernicious anemia </li></ul><ul><li>Testicular dysfunction </li></ul><ul><li>Type I diabetes </li></ul><ul><li>Vitiligo </li></ul>
    6. 6. Signs & Symptoms <ul><li>Usually develops slowly; early signs often go unnoticed. </li></ul><ul><li>Weakness, fatigue, lethargy, anorexia & salt craving, poor </li></ul><ul><li>weight gain or weight loss. </li></ul><ul><li>Hyper-pigmentation of skin at “skin fold” sites; sometimes accompanied by a generalized bronzing of skin. </li></ul><ul><li>Abdominal pain, n/v/d, symptomatic hypoglycemia. </li></ul><ul><li>Addisonian Crisis </li></ul><ul><li>A life-threatening condition ! Need medical attention ASAP! </li></ul><ul><li>Severe hypotension, weakness, pallor, fever, abdominal pain, continuous vomiting, dehydration, hypoglycemia seizures, circulatory collapse, shock, possible coma. </li></ul>
    7. 7. Diagnostic Tests <ul><li>Laboratory Tests </li></ul><ul><ul><li>Low blood pressure </li></ul></ul><ul><ul><li>Increased potassium levels </li></ul></ul><ul><ul><li>Low serum sodium </li></ul></ul><ul><ul><li>Low cortisol levels </li></ul></ul><ul><ul><li>Normal sex hormone levels </li></ul></ul><ul><li>Abdominal x-ray and CT scans </li></ul><ul><li>Blood eosinophil count, urine cortisol, and other tests may be used as confirmatory tools. </li></ul>
    8. 8. Treatment <ul><li>Lifetime hormone replacement therapy (oral or IM) </li></ul><ul><ul><li>Expensive </li></ul></ul><ul><ul><li>Cannot skip doses or crisis may occur </li></ul></ul><ul><ul><li>The health care provider may increase the medication dose in times of infection, injury or stress. </li></ul></ul><ul><li>During an Adissonian crisis, hydrocortisone injections are required immediately, along with low blood pressure treatment. </li></ul><ul><li>Similar to individuals with severe allergies that carry “epi” pens, some people with Addison's disease are taught to give themselves an emergency injection of hydrocortisone during stressful situations. </li></ul><ul><li>Prognosis </li></ul><ul><li>With hormone replacement therapy, most people with Addison's disease are able to lead normal lives. </li></ul>
    9. 9. Nursing Diagnoses <ul><li>Deficient Fluid Volume related to hypovolemia secondary to adrenal insufficiency. </li></ul><ul><li>Ineffective Tissue Perfusion: Peripheral related to dehydration. </li></ul><ul><li>Anxiety related to lack of knowledge about the effects and treatment of adrenal insufficiency. </li></ul><ul><li>Disturbed Body Image related to changes in skin pigmentation. </li></ul>
    10. 10. Planning & Implementation <ul><li>The Hospitalized Patient </li></ul><ul><li>Encourage fluid intake and administer IV hydration </li></ul><ul><li>Monitor intake/output and serum electrolytes </li></ul><ul><li>Monitor blood pressure, pulses and skin turgor q2h </li></ul><ul><li>Fall risk precautions in place </li></ul><ul><li>Help patient identify appropriate stress-reducing techniques </li></ul><ul><li>The Discharged Patient </li></ul><ul><li>Educate patient and family on adjusting for lifelong treatment </li></ul><ul><ul><li>Understanding of crisis symptoms; special needs before medical procedures </li></ul></ul><ul><li>Discuss a diet high in Na+ and low in K+ </li></ul><ul><li>Arrange for a medical alert bracelet or pendant </li></ul><ul><li>Older children & adolescents: discuss skin pigment changes </li></ul><ul><li>Mid/late adolescents: entrust a friend to know in case of emergency </li></ul>
    11. 11. Evaluation <ul><li>Expected outcomes in a pediatric population: </li></ul><ul><li>The child maintains hemodynamic stability. </li></ul><ul><li>The child achieves fluid balance. </li></ul><ul><li>The child and family demonstrate understanding of the disease and treatment regimen. </li></ul><ul><li>The child demonstrates a positive body image. </li></ul>
    12. 12. References <ul><li>A.D.A.M., Inc. (2009, November 25). Addison's disease . Retrieved November 23, </li></ul><ul><li>2011, from PubMed Health - U.S. National Library of Medicine: </li></ul><ul><li>http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001416/ </li></ul><ul><li>  </li></ul><ul><li>Ball, J. W., Bindler, R. C., & Cowen, K. J. (2010). Child Health Nursing: Partnering </li></ul><ul><li>with Children & Families. Saddle River, NJ: Pearson Education. </li></ul><ul><li>  </li></ul><ul><li>Pearson Education. (n.d.). Nursing Care Plan: A Client with Addison's Disease. </li></ul><ul><li>Retrieved November 27, 2011, from Pearson Education: </li></ul><ul><li>http://wps.prenhall.com/wps/media/objects/737/755395/addisons_disease.pdf </li></ul><ul><li>  </li></ul><ul><li>Sabol, V. K. (2001, July). Addisonian Crisis. American Journal of Nursing , pp. </li></ul><ul><li>24AAA-24DDD. </li></ul>

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