Addisons disease and its management

Addisons disease and its management

1. ADDISON’S DISEASE AND ITS MANAGEMENT -BY SHWETA SHARMA M.SC. NURSING 1 ST YEAR AIIMS, JODHPUR

2. INTRODUCTION Addison’s disease or adrenocortical insufficiency, occurs when adrenal cortex function is inadequate to meet the patient’s need for cortical hormones. Also known as Adrenocortical hypofunction; Chronic adrenocortical insufficiency; Primary adrenal insufficiency. Addison’s disease occurs when more than 90% of adrenal gland tissue is destroyed (primary Addison’s disease).

3. John F. Kennedy 35th president of the United States (1961-1963)

4. EPIDEMIOLOGY The frequency rate of Addison's disease is roughly one in 1,00,000. 40–144 cases per million population (1/25,000–1/7,000). This disease can affect persons of any age, sex, or ethnicity. It typically presents in adults between 30 and 50 years of age. More common in females and children.

5. ADRENAL GLAND

6. RISK FACTORS •History of other endocrine disorders •Taking glucocorticoids for more than 3 weeks with sudden cessation •Taking glucocorticoids more than once every other day •Adrenalectomy •Tuberculosis

7. ETIOLOGY •Autoimmune •Idiopathic atrophy of adrenal glands •Surgical removal of both adrenal glands ( Bilateral adrenalectomy ) •Infection of adrenal glands ( Tuberculosis [20%] and histoplasmosis are the most common infections that destroy adrenal gland tissue) •Fungal infections

8. •Loss of blood flow to the pituitary •Removal of pituitary gland •Inadequate secretion of ACTH from the pituitary gland •Therapeutic use of corticosteroids • Haemorrhage into adrenal glands • Tumours

9. PATHOPHYSIOLOGY Primary adrenal insufficiency. This is known as Addison's disease. It occurs when the adrenal glands are damaged. They don’t make enough of the hormone’s cortisol and aldosterone . This condition is rare. It may occur at any age. Secondary adrenal insufficiency. This starts when the pituitary gland doesn’t make enough of the hormone ACTH (adrenocorticotropin). As a result, the adrenal glands don’t make enough cortisol.

10. CLINICAL MANIFESTATIONS

11. •Hyponatremia •Hyperkalaemia •Depression, emotional lability, apathy, and confusion are present in 20% to 40% of patients •Chronic dehydration •Chronic diarrhoea, nausea, and vomiting •Dizziness when standing up •Paleness •Mouth lesions on the inside of a cheek (buccal mucosa) •Salt craving

12. COMPLICATIONS 1.Addisonian crisis- An acute adrenal insufficiency, a life-threatening emergency caused by insufficient adrenocortical hormones or a sudden sharp decrease in these hormones. This condition is characterised by- Hypotension; rapid, weak pulse; rapid respiratory rate Cyanosis, pallor Fever Nausea and vomiting Shock Headache, abdominal pain, diarrhoea and weakness Confusion, restlessness

13. •Even slight overexertion, exposure to cold, acute infection, or a decrease in salt intake may lead to circulatory collapse, shock, and death if untreated. •The stress of surgery or dehydration resulting from preparation for diagnostic tests or surgery may precipitate an Addisonian or hypotensive crisis. 2.Osteoporosis- Due to excessive use of glucocorticoids as the protein matrix in the bones is broken down and therefore calcium cannot be retained.

14. DIAGNOSTIC EVALUATION History collection- -History of recent infection, steroid use, or adrenal or pituitary surgery. -History of poor tolerance for stress, weakness, fatigue, and activity intolerance. - Anorexia, nausea, vomiting, or diarrhea as a result of altered metabolism. - Dizziness due to orthostatic hypotension. -History of craving for salt or intolerance to cold. -Presence of altered menses in females and impotence in males.

15. Physical examination- Signs of dehydration such as tachycardia, altered level of consciousness, dry skin with poor turgor, dry mucous membranes, weight loss, and weak peripheral pulses. Postural hypotension Inspect the skin for pigmentation changes Inspect the patient's gums and oral mucous membranes to see if they are bluish-black. Temperature Any loss of axillary and pubic hair that could be caused by decreased androgen levels.

16. ACTH stimulation test- Short test- compares blood cortisol levels before and after 250 mcg of tetracosactide (IM/IV) is given. Long test- uses 1 mg tetracosactide (IM). Blood is taken 1, 4, 8, and 24 hours later. Increased ACTH level: Primary insufficiency Decreased ACTH level: Secondary insufficiency

17. CRH stimulation test The doctor will draw some blood and measure the cortisol level. Next, synthetic CRH is injected into your bloodstream. Blood cortisol is measured every 30 minutes for about an hour and a half after the injection. If CRH injection causes an ACTH response, but no cortisol response, the pituitary is functioning but the adrenal glands are not. Such results are consistent with the diagnosis of primary adrenal insufficiency or Addison’s disease. If CRH injection does not generate ACTH response, the problem is the pituitary gland (secondary adrenal insufficiency). CRH injection produces a delayed ACTH response, the problem is the hypothalamus.

18. Complete blood count (CBC)- Anaemia Blood urea nitrogen (BUN)- Increased Electrocardiography (ECG)- Shows low voltage and peaked T waves caused by hyperkalaemia Computed Tomography (CT) scan and Magnetic Resonance Imaging (MRI) Urine cortisol and aldosterone- Decreased Hypoglycaemia, hyponatremia, hyperkalaemia, leucocytosis

19. Combined measurements of early-morning serum cortisol and plasma ACTH are performed to differentiate primary adrenal insufficiency from secondary adrenal insufficiency and from normal adrenal function. -Patients with primary insufficiency have a greatly increased plasma ACTH level and a serum cortisol concentration lower than the normal range or in the low-normal range. - ACTH levels are decreased in secondary adrenal insufficiency.

20. MEDICAL MANAGEMENT Treat the circulatory shock : restore blood circulation, administer fluids and corticosteroids, monitor vital signs, and place the patient in a recumbent position with legs elevated. Glucocorticoid (hydrocortisone-15 mg on waking and 5 mg at 6p.m.) and mineralocorticoid (fludrocortisone 0.05 to 0.1mg daily) Antibiotics - If infection has precipitated adrenal crisis in a patient with chronic adrenal insufficiency. Monitor the patient closely to identify other stressors, factors or illnesses that led to the acute episode.

21. Initiate oral intake as soon as tolerated. If the adrenal gland does not regain function, the patient needs lifelong replacement of corticosteroids and mineralocorticoids to prevent recurrence of adrenal insufficiency. Supplement dietary intake with salt during GI losses of fluids through vomiting and diarrhoea . The patient with Addisonian crisis should avoid physical and psychological stressors such as cold exposure, overexertion, infection, and emotional distress.

22. Treatment of patient with Addisonian crisis -Administration of fluid, glucose, and electrolytes especially sodium. -Replacement of missing steroid hormones; and vasopressors. -Large volumes of 0.9% saline solution and 5% dextrose are administered to reverse hypotension and electrolyte imbalances until blood pressure returns to normal.

23. NURSING ASSESSMENT Assess for fluid and electrolyte imbalance and patient’s level of stress. Monitor the blood pressure and pulse rate as the patient moves from a lying, sitting, and standing position to assess for inadequate fluid volume. A decrease in systolic pressure (20 mm of Hg or more) may indicate depletion of fluid volume, especially if accompanied by symptoms. Assess skin for changes in colour and turgor , which could indicate chronic adrenal insufficiency and hypovolemia. Assess for change in weight, muscle weakness, fatigue, and any illness or stress that may have precipitated the acute crisis.

24. Establish baseline data regarding mental status, vital signs and weight. Obtain a complete medication history to determine drugs that can potentially interact with corticosteroids. These drugs include oral hyperglycaemics , cardiac glycosides, oral contraceptives, anticoagulants and NSAIDs. Protect against exposure to infection and assist with daily hygiene. Protect from noise, light, and environmental temperature extremes.

25. NURSING DIAGNOSIS 1.Imbalanced nutrition: less than body requirements related to insufficient dietary intake as evidenced by anorexia, nausea, vomiting, and/or diarrhea. 2.Risk for deficient fluid volume related to increase in sodium and water excretion with potassium retention . 3.Risk for decreased cardiac output related to increased use of corticosteroids. 4.Anxiety related to treatment as evidenced by facial expressions. 5.Deficient knowledge related to disease process and its management as evidenced by frequent questioning.

26. PATIENT EDUCATION Notify the health care provider if vomiting or diarrhea occurs. Teach the patient about the signs and symptoms of corticosteroid deficiency and excess (Cushing syndrome) and to report these signs to their health care provider so the dose can be adjusted. It is critical that the patient wear an identification bracelet and carry a wallet card stating the patient has Addison’s disease so that appropriate therapy can be initiated in case of an emergency. Instruct patients using mineralocorticoid therapy how to take their blood pressure, increase salt intake, and report any significant changes to their health care provider. The patient should carry an emergency kit at all times with 100 mg of IM hydrocortisone , syringes, and instructions for use.

27. RESEARCH ARTICLES 1.Bone mineral density in patients with Addison disease on replacement therapy with prednisolone. Chandy DD, Bhatia E conducted a cross-sectional study to determine BMD and its relation with therapy in patients on physiologic doses of prednisolone replacement. Forty-one consecutive patients, receiving prednisolone were studied. BMD was evaluated by dual-energy X-ray absorptiometry and compared with an age- and sex-matched reference group of healthy Indian subjects. Among males, BMD Z-scores at lumbar spine, femoral neck and total hip were significantly lower than the reference population. Z-scores in female patients did not differ from controls. Among postmenopausal females and males >50 years, 43% had osteoporosis, as compared with 25% in the reference group. A high proportion of males had low serum testosterone, but there was no correlation between testosterone and BMD. It concluded that male patients receiving physiologic prednisolone replacement had a small but significant diminution in BMD at all sites.

28. 2.Addison’s Disease Symptoms – A Cross Sectional Study in Urban South Africa Ian Louis Ross, Naomi S. Levitt conducted a cross-sectional study in 2013. 148 patients were enrolled. Demographic and clinical data were elicited using questionnaires. Biochemical data were obtained from folder reviews and laboratory archived results. Hyperpigmentation was observed in 76% , nausea and vomiting occurred in more than 40% , and weight loss was noted in 25%. Loss of consciousness as a presenting feature was recorded in 20%. with a 95% confidence interval [CI] of (14–28%) and shock occurred in 5% CI (1.5–8.5%). 5

29. NEW ADVANCES Addison's Disease Information System (ADIS) Addison disease patients now have a way to get quick and easy access to potentially life-saving treatment using a Quick Response (QR) code, which can be carried at all times and scanned to access vital medical support. The innovative device comes in the form of a bracelet, which has a printed QR code on a plastic card. With the use of a smartphone, the device syncs to the Addison's Disease Information System (ADIS), which provides comprehensive clinical management advice specific to the patient. Conveniently, QR codes are also free; they can be easily generated and printed with the use of free web-based software.

30. SUMMARY AND CONCLUSION •As discussed throughout the presentation, learning about Addison’s disease and its management will help nurses to care for patients with Addison’s disease. •Nurses can do assessment of patients with Addison’s disease, observe the sign and symptoms, provide the necessary nursing care and support the patient psychologically. •Nurses can also counsel the patients and their family for various options available in treatment for Addison’s disease.

31. REFERENCES 1.Lewis. Medical Surgical Nursing Assessment and Management of clinical problems.2015. New Delhi. Elsevier. 2nd Edition. Volume II. Pg. no.983-985, 1264-1266. 2.Janice L. Hinkle, Kerry H. Cheever. Brunner and Suddarth’s Textbook of Medical Surgical Nursing. 2015. New Delhi. Wolters Kluwer.13th Edition. Volume 2. Pg. no. 1494-1496. 3.Joyce M. Black, Jane Hokanson Hawks. Medical Surgical Nursing Clinical Management of Positive Outcomes.2015. New Delhi. Reed Elsevier India Private Limited. Volume II. Pg. No.1040- 1044. 4. Chandy DD, Bhatia E. Bone mineral density in patients with addison disease on replacement therapy with prednisolone. Endocr Pract . 2016 Apr;22(4):434-9. doi : 10.4158/EP151014.OR. Epub 2015 Dec 18. 5. Ross IL, Levitt NS (2013) Addison’s Disease Symptoms – A Cross Sectional Study in Urban South Africa. PLoS ONE 8(1): e53526. https://doi.org/10.1371/journal.pone.0053526 6. Krista Rossi. New Device Helps Addison Disease Patients Access Life-Saving Treatment. JULY 02, 2018. Available from https://www.mdmag.com/medical-news/new-device-helps-addison-disease-patients-access-lifesaving-treatment [cited 29 Jan 2020]

32. Q1. Which of the following electrolyte imbalance is seen in Addison’s disease? a) Hypernatremia and hyperkalemia b) Hyponatremia and hyperkalemia c) Hyponatremia and hypokalemia d) Hypernatremia and hypokalemia Answer- b

33. Q2. What is primary adrenal insufficiency? a) Impaired function of pituitary to produce ACTH b) Impaired function of hypothalamus to produce CRH c) Impaired function of adrenal glands to produce cortisol d) Impaired function of pituitary and adrenal glands Answer- c

34. Q3. What ECG changes will be noted in a patient with Addison’s disease? Peaked T waves Small ST segment Wide QRS comples Depressed T waves Answer- a