Causes, Incidence, and Risk Factors
1. Cushing’s syndrome is a syndrome or disease which is caused by either excess production of cortisol or excess use of high dose corticosteroids. Cortisol is a hormone which is produced in outer portion of adrenal glands of kidney. The regulation of the production of cortisol is done by a hormone named adrenocorticotrophic (ACTH). It helps the body in condition of changes and stress like swelling or high blood sugar. In this case study Ms Maureen smith took high dose of corticosteroids during her treatment of Rheumatoid Arthritis which leads to her Cushing’s syndrome. This form is called iatrogenic and is a common side effect of high dose of steroid hormones which is used for life threatening disease like Rheumatoid Arthritis (RA) (Lacroix et al., (2015)..
This disease was named after Harvey Cushing who identified the disease first in women in 1912. This type of disease is common in nature as the corticosteroids use is found worldwide for diseases like RA. In adults it is more common in women than men. The age limit is normally from 25- 45 years. The total incidence is about 5 to 25 case per million people per year. In Australia this disease is rare and probably affects 300 to 1200 Australians per year. Ectopic ACTH which causes this Cushing’s syndrome is more common than the normal form (almost 660 per million per year)
The risk factors included in this disease are type 2 diabetes, obesity, high blood pressure and low control of blood sugar. All these symptoms are found in the patient of case study 2.
The indications and deficiencies of person with Cushion’s Syndrome is based on the amount of cortisol in the patient, disease time period and normal health of the patient. If proper treatment is provided then the disease can be cured within 2 to 18 months. The patient and their family get frustrated because the treatment takes long time and slow improvement occurs. The patient feels weakness and fatigue due to which visit do doctor becomes in huge number which is problem for the patient and family. If this Cushing’s syndrome is not getting cured then the Patient will suffer more from weakness of muscle, weight gain in face and abdomen depression and mood swing. The family of patient feels serious burden because they have to take the patient to doctor several times for blood test and treatments of the symptoms and complications of the disease.
Common Signs and Symptoms and Their Underlying Pathophysiology
2.
Symptoms of Cushing’s Syndrome |
Pathophysiology linked with it |
Type 2 diabetes |
The patients of Cushing’s syndrome suffer from diabetes mellitus. They suffer from disfunctioning of glucose metabolism This condition arises due to excess use of glucocorticoids. These drugs induce gluconeogenesis which increase the level of glucose and also degrades the insulin production. Thus the glucose produced is not degraded and diabetic condition occurs (Mazziotti et al., 2017).. |
Obesity |
Cortisol acts as a stress remover and helps in reduction of stress conditions in human body like reducing swelling. It also helps in regulation of carbohydrates, fats and protein. In case of this disease the level of cortisol increases due to excess use of corticosteroid drugs. Excess cortisol content in body sometimes gives false alarms and does food metabolization and absorption of body even though if body does not require it. Thus due to this factor the patient keeps on eating and the body starts accumulation of excess fats in various parts of the body. Specially fat accumulation occurs in abdomen region and face (Lee et al., 2014).. |
Hypertension |
Hypertension is common symptom of Cushing’s syndrome. About 80 % of the patients suffer from hypertension. Glucocorticoids cause hypertension by their inborn mineralocorticoid action; through enactment of the renin-angiotensin framework; by upgrade of vasoactive substances, and by bringing about concealment of the vasodilatory systems. Glucocorticoids also have some effect on cardiovascular regulation by CNS (Isidori et al., 2015).. |
Muscle weakness |
Around 60 % of patients of Cushing’s syndrome suffer muscle weakness. The muscles get weak and they feel tiredness and pains in muscle. The high dose of corticosteroids changes the protein metabolism. These drugs decrease the rate of protein synthesis, thus the protein breakdown is more which leads to the degradation of muscles. These drugs induce catabolism of muscle protein (Fry et al., (2016).. |
Thinning of skin |
The thinning of skin and other mucous membranes is one of the symptoms of the Cushing’s syndrome. The skin of the patient becomes dry and gets injured very easily. This is due to the fact that the cortisol causes degradation of some dermal proteins and also causes thinning of some blood vessels. Due to this the skin becomes very weak and formation of shiny, quality of paper thin which gets torn easily (Raff, Sharma & Nieman, 2014).. |
3. The two classes of drugs used in the treatment of Cushing’s syndrome are anti steroid drugs (Aminoglutethimide) and Adrenal corticosteroid synthesis inhibitor (Metyrapone) (Eckstein et al., (2014).
For treatment for this disease this type of drug is used. One example is Aminoglutethimide which is a type of anti steroid drug and marketed under the name of Cytadren. This type of drugs blocks the production of steroids which are cholesterol derived and used in Cushing’s syndrome. These drugs are used with combination of other drugs and are used for the inhibiting the function of adrenal gland of patients of this disease. The Aminoglutethimide has two mechanism of action in case of this disease. Firstly it helps in blocking of aromatase in generation of estrogens from androstenedione and testosterone. Secondly it helps in blocking of conversion of cholesterol to pregnenolone by blocking the enzyme P450scc. The effects of these types of drugs are formation of rash of skin, cortisol inhibition in human and hepatoxicity. (Niema et al., 2015).
These types of drugs like Metyrapone are generally used for the diagnosis of insufficiency of adrenalin and for treatment of Case disease. These types of drugs block the synthesis of cortisol by inhibiting reversibly steroid 11 β hydroxylase. This thus turns the stimulation of secretion of ACTH and increases the plasma 11-deoxycortisol levels. This type of drugs is used for the control of hypercortisolism in case of Cushing’s syndrome (Daniel et al., (2015). It is used for controlling of hypercortisolism. It also acts and stops adrenal steroidogenesis. This is not used in long time treatment of this disease but acts as short term relief facility. Metyrapone is also used for testing the patient with Cushing’s syndrome. If the patient has not function of pituitary will have increasing ACTH level with the reaction of metyrapone (Gadelha & Vieira (2014). In addition with the needed effects some other effects is also caused by them. Some of the effects of this type of medication is dizziness headache and nausea. But if its used in high dose then serious case will happen like vomiting and sudden weakness. It also causes skin rash sore throat and fever.
4. As a registered nurse I would take the following strategy for the patient who is admitted in hospital with Cushing’s syndrome. Firstly I will closely monitor the patient just to avoid complications. My assessment will be based on the history of health including the level of activities which he can do daily and his self care activities. Then I will do physical examination of skin to check for trauma injury or infection and finally check for mental stability of the patient which is their moods, response and awareness. After this assessment I will check for their disease assessment which includes the 1) risk for injury- checking for weakness, 2) infection risk- swelling response, 3) deficit of self care- weakness of muscle, fatigue and disrupted sleeping pattern, 4) checking for skin injury, 5) checking for problem in body like less activity level and different physical appearance. 6) Disturbances in mood swings and depression. After checking for the disease I will try to provide care for the above assessment (Gulanick & Myers, 2013).
Common Classes of Drugs for Treating Patients with Cushing’s Syndrome
For decreasing the risk of injury I will try to provide a suitable and protective environment so that the patient does not fall and injure their bone and soft tissues. I will try to give good diet of rich protein and calcium to reduce their muscle reduction. For decreasing infections in patient I will minimize the contact of patient with other people and check for inflammation infection. I will check the glucose level of the patient and give medications for its reduction. I will try to engage the patient with moderate activities and ample rest. I will try to chalk out a plan for schedule of rest and activity (Llahana & Thomas, 2016). For reducing chances of infection I will try to use medications and equipments including glassware’s in sterile condition. Will try to give meticulous skin care to avoid the injury of the fragile skin. The body weight of the patient needs to be reduced by giving them diets with low carbohydrate and low sodium and high protein. To improve the patient’s mood swings and behavior I will try to explain the patient’s family with the cause of the disease and ways of treatment. After consulting the doctor I will try to give the necessary first line medications to reduce the immediate symptoms of the disease.
I also need to do constant monitoring of some crucial factors like hypotension weak nerve impulse respiratory rate and check for factors which causes crisis in patients like trauma and surgery. I will provide the patient with fluids and electrolytes if required and check for laboratory values and daily weight. The blood test has to be done to check for diabetes level. I will encourage the patient to deep breathing coughing and incentive spirometry every 2 to 4 hrs. The main important work is to provide information to patient and family about the self care. Information has to be provided to family of patient that use of corticosteroid use should be minimized as this will cause increase in symptoms of Cushing’s syndrome.
References:-
Daniel, E., Aylwin, S., Mustafa, O., Ball, S., Munir, A., Boelaert, K., … & Davis, J. (2015). Effectiveness of metyrapone in treating Cushing’s Syndrome: a retrospective multicenter study in 195 patients. The Journal of Clinical Endocrinology & Metabolism, 100(11), 4146-4154.
Eckstein, N., Haas, B., Hass, M. D. S., & Pfeifer, V. (2014). Systemic therapy of Cushing’s syndrome. Orphanet journal of rare diseases, 9(1), 122.
Fry, C. S., Nayeem, S. Z., Dillon, E. L., Sarkar, P. S., Tumurbaatar, B., Urban, R. J., … & Choudhary, S. (2016). Glucocorticoids increase skeletal muscle NF?κB inducing kinase (NIK): links to muscle atrophy. Physiological Reports, 4(21), e13014.
Gadelha, M. R., & Vieira Neto, L. (2014). Efficacy of medical treatment in Cushing’s disease: a systematic review. Clinical endocrinology, 80(1), 1-12.
Gulanick, M., & Myers, J. L. (2013). Nursing care plans: nursing diagnosis and intervention. Elsevier Health Sciences.
Isidori, A. M., Graziadio, C., Paragliola, R. M., Cozzolino, A., Ambrogio, A. G., Colao, A., … & ABC Study Group. (2015). The hypertension of Cushing’s syndrome: controversies in the pathophysiology and focus on cardiovascular complications. Journal of hypertension, 33(1), 44-60.
Lacroix, A., Feelders, R. A., Stratakis, C. A., & Nieman, L. K. (2015). Cushing’s syndrome. The Lancet, 386(9996), 913-927.
Lee, M. J., Pramyothin, P., Karastergiou, K., & Fried, S. K. (2014). Deconstructing the roles of glucocorticoids in adipose tissue biology and the development of central obesity. Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 1842(3), 473-481.
Llahana, S., & Thomas, N. (2016). Structured nursing educational programs improve quality of life outcomes in patients with Cushing’s syndrome. Endocrine, 53(1), 1-3.
Mazziotti, G., Formenti, A. M., Frara, S., Maffezzoni, F., Doga, M., & Giustina, A. (2017). Diabetes in Cushing Disease. Current Diabetes Reports, 17(5), 32.
Nieman, L. K., Biller, B. M., Findling, J. W., Murad, M. H., Newell-Price, J., Savage, M. O., & Tabarin, A. (2015). Treatment of Cushing’s syndrome: an endocrine society clinical practice guideline. The Journal of Clinical Endocrinology & Metabolism, 100(8), 2807-2831.
Raff, H., Sharma, S. T., & Nieman, L. K. (2014). Physiological basis for the etiology, diagnosis, and treatment of adrenal disorders: Cushing’s syndrome, adrenal insufficiency, and congenital adrenal hyperplasia. Comprehensive Physiology.