ADDISON DISEASE (AD)
Introduction
It is also called primary adrenal insufficiency. AD is a seldom disease of the endocrine as a result of the inadequacy of the adrenal cortical cells to churn out adequate aldosterone or cortisol hormones. Cortisol is one of the crucial hormones during body stress, thus essential in the maintenance of blood pressure, antiinflammation, and glucose metabolism in the liver (Smeltzer, Hinkle, Bare, & Cheever, 2014). The adrenal insufficiency may be due to the annihilation, absence, or dysfunction of the adrenal cortex of the gland. However, the secondary and tertiary causes are not involved, but the ‘Addison disease’ is directed explicitly to primary adrenal insufficiency (Allolio, 2015). The primary insufficiency only occurs when 90% of adrenal cortices was damaged. Besides, in childhood, the primary cause is congenital, but in adulthood, it is majorly due to autoimmune disease (Griffing, 2020). The identification of the disease is attributed to the literature works of Thomas Addison in 1855 when he made a clear connection of the clinical manifestations of the disease with the hormones affected (O’Connell & Siafarikas, 2016).
Epidemiology
In the United States of America, the prevalence of the malady cases is between 40 and 60 in one million of the overall population. Internationally, AD is a seldomly occurring disease. In Britain, the prevalent cases are about 39 in one million of the people, while Denmark has 60 in one million of its population (Griffing, 2020). According to the works of Sigurjonsdottir and Olafsson, Iceland has approximately 22.1 cases in one million people, and based on Hong et al. study, Korea has 4.17 cases in one million of the population. The deaths or morbidity of AD are due to hesitation or failure to make a prompt diagnosis or initiate the hormonal replacement therapy. Besides, the disease is not inclined to any race in particular, but in terms of gender, the unknown autoimmune cause has been dominant on children or women. The condition can attack anyone at any age, but mostly, it occurs in people age between 30 and 50 years (O’Connell & Siafarikas, 2016).
Causes/ risk factors
Often AD arises due to the damage of the entire or 90 % of the adrenal cortices leading to insufficient secretion of both the cortisol and androsterone hormones (O’Connell & Siafarikas, 2016). First, the autoimmune diseases in which the body’s immune system turns against the adrenal cells attacking and damaging them, leading to reduction or complete barring of cortisol or aldosterone production. Secondly, some infections, for example, tuberculosis has been known to cause calcification on the adrenal gland stopping the usual generation of the hormones mentioned above (Michels A, 2017). Thirdly, the surgical deletion of both adrenal glands. This procedure is termed as bilateral adrenalectomy. This means that there will be no cortex to produce either of the hormones.
Additionally, the congenital cause may occur, especially in the errors of genetic code reading. Therefore, this means the protein generation for cortisol or aldosterone production will be interrupted, thus insufficient cortisol and aldosterone (Griffing, 2020). Moreover, some medications such as prednisolone mimic the cortisol effects, thus impacting the pituitary gland to accept that there is sufficient generation of cortisol hormone by the adrenal gland, yet that is false. Thus, the pituitary gland will decrease the production of ACTH; hence the adrenal gland will hibernate by failing to produce too (Michels A, 2017).
Pathophysiology
Addison disease has its pathophysiology reliant on the causative factors. Since it is a primary adrenal insufficiency, the trigger is localized around the both of the adrenal glands []. In autoimmune induced Addison disease, the attack is directed toward the adrenal cortical cells, therefore, destroying the 21-hydroxylase enzyme. This is due to the auto-activity of T cells and autoantibodies of 21-hydroxylase. This auto-activity occurs due to idiopathic environmental causes in genetically vulnerable individuals. The majority of the weak genes identified are of major histocompatibility halo-types. These are DR4-DR8 and DR3-QDR (Allolio, 2015). Therefore, the above enzyme reacts with autoantibodies forming complexes, which causes 90% or more of the adrenal gland destruction. Thus, due to the massive damage, the cells with the capacity to produce cortisol or aldosterone will be destroyed, too, eventually terminating or reducing the secretion of the cortisol or aldosterone or both, due to this, signs and symptoms of Addison disease manifest, especially during stressful situation (Allolio, 2015). For the inborn case, the errors occur in genetic reading coding for the production of the protein responsible for initiating the production of cortisol. Therefore, due to these errors, cortisol production will not occur, causing Addison disease. Generally, the pathogenesis of Addison disease depends on the risk factors or the causative agent.
Clinical manifestations/ signs & symptoms
The clinical presentations always depend on an individual as they will vary from one person to another. Also, these symptoms progressively slow and are non-specific as they occur in various diseases. Firstly, the general feeling of fatigue is one of the most typical symptoms. Secondly, the dark hyperpigmentation of the skin may occur, leaving dark patches. In addition to that, vitiligo may occur, in which the white spots on the surface appear on the skin. The gastrointestinal tract presentations may happen, including nausea, abdominal pain, and occasional vomiting and diarrhea, which seldomly occur. Also, one may experience low or poor appetite. With time, the individual may have an unexpected loss of weight. Besides, myalgia, joint pain, dehydration, hypotension on standing, syncope, depression, irritability, poor concentration, craving for salt food, and hypoglycemia may also occur (Arlt, 2014). For women, they may experience irregular menses, reduced sex drive, and loss of body hair (Smeltzer et al., 2014). However, in case these occur abruptly, the Addison crisis may loom in. At this point, the patient will experience muscle weakness, pain at the lower back, legs, and joints. Besides, one may vomit and diarrhea-causing dehydration, hypotension, and syncope. This situation happens mostly during stressful occurrences such as surgery, accidents, severe infections, or trauma (Smeltzer et al., 2014).
Diagnosis of Addison disease
During the diagnosis of AD, a thorough history taking of the patient and picking out of findings are essential to include Past medical history; medications are taken at home, Assess vital signs, Health perception/health management, Nutritional and metabolic, Elimination, Perception of pain, Integumentary, Respiratory, Gastrointestinal, Neurologic and Reproductive data. However, the above clinical manifestations are non-specific; thus, they are unreliable (Griffing, 2020). Besides, the diagnostic tests that may be undertaken include Blood tests. This will indicate elevated serum potassium and a reduced amount of serum sodium (Griffing, 2020). To confirm the diagnosis, ACTH stimulation tests are conducted to check the level of cortisol hormone in serum. To check if the problem lies in the pituitary gland, and insulin-induced hypoglycemia test is done. Moreover, antibody tests should be done to investigate for the presence of autoantibodies directed toward the adrenal cells. The imaging studies, such as the CT scan, may be done on the abdomen to check on the adrenal gland size and calcification or tumor size (Arlt, 2014).
Differential diagnoses
Some of the conditions that may present similarly to Addison disease are adrenal hemorrhage, adrenal crisis, eosinophilia, hyperkalemia, histoplasmosis, sarcoidosis, and adrenal crisis (Griffing, 2020).
Complications
If Addison’s disease goes undetected and untreated, this shifts to the Addison crisis, which is life-threatening. These are associated with orthostatic hypotension, hypoglycemia, hyperkalemia, hyponatremia, and syncope(O’Connell & Siafarikas, 2016).
Medical management
The treatment of Addison disease focuses on the specific symptoms. Therefore, generally, the procedure is majorly the replacement of steroid hormones produced by the adrenal cortex portion. Those are cortisol and aldosterone. Hydrocortisone (Cortef) /prednisone/ methylprednisolone is administered as per the cortisol production cycle to mimic the usual daily fluctuation of cortisol levels in a bid to restore the body levels of cortisol. In contrast, the administration of fludrocortisone acetate restores the aldosterone levels (Griffing, 2020). Besides, advise the patient to take food rich in sodium, majorly on vigorous exercise, hot weather, or if in the presence of gastrointestinal problems like diarrhea (Arlt, 2014). Additionally, the patient should always have a medical-alert card or bracelet for easy identification that you are diagnosed with Addison disease.
However, in the case of the Addisonian crisis, a medical emergency, immediate treatment involves the intravenous infusion of normal saline, corticosteroids, and dextrose. For women, androgen replacement may be conducted to enhance their sexual drive (O’Connell & Siafarikas, 2016).
Nursing management
A nurse conducts observation and examination of the patient by making a thorough history of the patient and looking for presentations of stress, changes fluid body volume, blood pressure changes, skin color, and turgor changes. Besides, a nurse observes for the indications and controls of the Addison crisis. Therefore, a nurse looks forward and satisfies the requirements of the patient in a bid to restore a previous healthy state. In case of a fluid disequilibrium, a nurse takes part to restore the fluid volume by advising the patient to consume fluids and foods rich in sodium and the ability to restore adequate fluid state(Smeltzer et al., 2014). Also, he or she directs the involved in the home care of the patient to give the hormonal replacement medication as per guideline and modify as required. Additionally, due to poor tolerance of activity by the patient, a nurse ought to educate and direct on the significance of reducing stressful events in crisis time as this will help the patient to resume daily activities well progressively. As a fundamental role, a nurse promotes home-based care integrated with adequate and quality family and patient education on self-care, medication and their modification, medical alert bracelets, and need to take medication regularly without an abrupt quitting (Smeltzer et al., 2014).
A case of Addison’s disease
A 35-year-old woman presented with dark skin pigmentation, dizziness, and fatigue, nausea, vomiting, and diarrhea with an unexpected loss of weight in six months before she came in. Physical examination revealed pallor, dark skin hyperpigmentation on the face, palmar, knuckles, and oral membranes. On CT scanning, the abdominal findings suggested extrapulmonary tuberculosis of the adrenal gland. She got treated with anti-tuberculous medications. Fifteen days following her admission, she experienced nausea and vomiting, low-grade fever, and loss of muscle strength. The assessment of cardiovascular implied an elevated heart rate, low weak pulse, and BP of 60/50 mmHg. The patient was put on dextrose running in saline and hydrocortisone. On the management, she recovered gradually well and was placed on a maintenance dose of oral prednisolone and fludrocortisone.
References
Allolio, B. (2015). ( ADDISON ’ S DISEASE ). TUE Physician Guidelines Medical Information to Support the Decisions of TUECs ADRENAL INSUFFICIENCY, 1(June), 1–11.
Arlt, W. (2014). Adrenal Insufficiency Patient Booklet. Society for Endocrinology, 01(1), 8. Retrieved from https://www.endocrinology.org/media/1767/16-04_adrenal-insufficiency.pdf
Griffing, G. (2020). Addison disease. Revue de l’Infirmiere, d(129), 16–17.
Michels A, M. N. (2017). Addison Disease: Early Detection and Treatment Principles. Addison Disease, 89(1), 563–568.
O’Connell, S., & Siafarikas, A. (2016). Addison disease: Diagnosis and initial management. Australian Family Physician, 39(11), 834–837.
Smeltzer, S. C., Hinkle, J. L., Bare, B. G., & Cheever, K. H. (2014). Brunner & Suddarth’s TEXTBOOK OF Medical-Surgical Nursing. (D. Sherry, Ed.) (13th ed.). Philadelphia: Lisa McAllister.