Addisons disease and its management

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

24. 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.

25. •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.

26. 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.

27. 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.

29. 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.

30. 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

31. 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.

33. 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.

34. 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]

37. 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

38. 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

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