Webinar DescriptionAn update on the diagnosis and treatment options of Cushing’s disease. Description of the endoscopic endonasal approach for the management of CD. Surgical videos demonstration and comparison with the microscopic transsphenoidal approach. Surgical outcomes and adjuvant treatment.
Presenter Bio
Dr. Mary Koutourousiou is an attending Neurosurgeon and Assistant Professor at the University of Louisville. She is the Director of the Pituitary and Skull Base Program. Dr. Koutourousiou received her M.D. from the Aristotle University of Thessaloniki, Greece and completed her neurosurgical residency at the General Hospital of Athens “G. Gennimatas”, in Greece. She underwent subspecialty fellowship training in Endoscopic Pituitary Surgery and Minimally Invasive Neurosurgery at the UMC St. Radboud, Nijmegen, in the Netherlands. She moved to the United States in 2010 and completed four years of research and a clinical fellowship in Endoscopic and Open Skull Base Surgery at UPMC Presbyterian in Pittsburgh, Pennsylvania.
Dr. Koutourousiou has published extensively in the field of endoscopic skull base surgery. Her studies have been presented in national and international neurosurgical meetings. Dr. Koutourousiou’s work in skull base surgery has been recognized by the European Skull Base Society and the World Federation of Skull Base Societies.
Summary: The objective of this study was to describe the management and outcomes of patients with Cushing’s disease (CD) in the Vancouver region over a 30-year period and to investigate the predictive factors of CD recurrence. Researchers found CD recurrence in 45.8% of patients who received initial transsphenoidal surgery (TSS), and that a post-operative serum cortisol level > 140nmol/L may be a positive predictor of recurrence in these patients.
Methods:
Researchers retrospectively reviewed the clinical charts from endocrinologists in Vancouver who provided consent to participate in this study.
Included in this study were 48 patients diagnosed with CD since 1985.
Results:
All 48 patients received initial TSS; the mean follow-up time was 11.73 (±6.98) years.
More than half of the patients (n=26, 54.2%) remained in remission, and 22 patients (45.8%) received subsequent interventions due to CD recurrence.
Second-line therapies included repeat TSS (40.9%), stereotactic radiotherapy (18.2%), and bilateral adrenalectomy (36.4%).
Among patients with disease recurrence, the average post-operative serum cortisol level was significantly higher (489.0 nmol/L vs 114.7nmol/L; P=0.003).
The positive predictive value for recurrence with post-operative serum cortisol > 140nmol/L was 76.5% (P=0.049), while serum cortisol < 140nmol/L had an 80% predictive value for non-recurrence (P=0.035).
Cushing’s and Addison’s Disease. An endocrine NCLEX review on how to differentiate between Cushing’s Syndrome/Disease vs Addison’s and Addisonian Crisis. In this video, I will discuss the pathophysiology, signs & symptoms, and nursing interventions for these endocrine disorders of the adrenal cortex and pituitary glands.
Addison’s Disease and Cushing’s Syndrome/Disease review notes for nursing school and NCLEX exam. In nursing school and for the NCLEX exam, you will need to know how to provide care to a patient with either Addison’s Disease or Cushing’s.
However, many students get these two endocrine disorders confused, but these review notes will help you differentiate between them.
These NCLEX review notes will cover:
Signs and Symptoms of Addison’s Disease vs Cushing’s
Causes of Addison’s Disease and Cushing’s
Nursing Management of Addison’s Disease and Cushing’s
Role of Adrenal Cortex: releases steroid hormones and sex hormones
Role of Aldosterone: regulates blood pressure through renin-angiotensin-aldosterone system, helps retain sodium and secretes potassium (balances sodium and potassium levels).
Role of Cortisol: “STRESS Hormone” helps the body deal with stress such as illness or injury, increases blood glucose though glucose metabolism, break downs fats, proteins, and carbs, regulates electrolytes.
Cushing’s (Syndrome & Disease)
Cushing’s: hyper-secretion of CORTISOL (watch video for clever ways to remember this)
Cushing’s Syndrome vs Cushing’s Disease
Cushing’s Syndrome: caused by an outside cause or medical treatment such as glucocorticoid therapy
Cushing’s Disease: caused from an inside source due to the pituitary gland producing too much ACTH (Adrenocorticotropic hormone) which causes the adrenal cortex to release too much cortisol.
Signs & Symptoms of Cushing’s
Remember the mnemonic: “STRESSED” (remember there is too much of the STRESS hormone CORTISOL)
Skin fragile
Truncal obesity with small arms
Rounded face (appears like moon), Reproductive issues amennorhea and ED in male(due to adrenal cortex’s role in secreting sex hormones)
Ecchymosis, Elevated blood pressure
Striae on the extremities and abdomen (Purplish)
Sugar extremely high (hyperglycemia)
Excessive body hair especially in women…and Hirsutism (women starting to have male characteristics), Electrolytes imbalance: hypokalemia
Dorsocervical fat pad (Buffalo hump), Depression
Causes of Cushing’s
Glucocorticoid drug therapy ex: Prednisone
Body causing it: due to tumors and cancer on the *pituitary glands or adrenal cortex, or genetic predisposition
Nursing Management for Cushing’s Syndrome
Prep patient for Hypophysectomy to remove the pituitary tumor
Prep patient for Adrenalectomy:
If this is done educate pt about cortisol replacement therapy after surgery
Risk for infection and skin breakdown
Monitor electrolytes blood sugar, potassium, sodium, and calcium levels
Addison’s Disease
Addison’s: Hyposecretion of Aldosterone & Cortisol (watch the video for a clever way on how to remember this and not get it confused with Cushing’s)
Signs & Symptoms of Addison’s Disease
Remember the phrase: “Low STEROID Hormones” (remember you have low production of aldosterone & cortisol which are STEROID hormones)
Sodium & Sugar low (due to low levels of cortisol which is responsible for retention sodium and increases blood glucose), Salt cravings
Tired and muscle weakness
Electrolyte imbalance of high Potassium and high Calcium
Reproductive changes…irregular menstrual cycle and ED in men
lOw blood pressure (at risk for vascular collapse)….aldosterone plays a role in regulating BP
Increased pigmentation of the skin (hyperpigmentation of the skin)
Diarrhea and nausea, Depression
Causes of Addison’s Disease
Autoimmune due to the adrenal cortex becoming damaged due to the body attacking itself:
Tuberculosis/infections
Cancer
Hemorrhaging of the adrenal cortex due to a trauma
Nursing Management of Addison’s Disease
Watching glucose and K+ level
Administer medications to replace the low hormone levels of cortisol and aldosterone
For replacing cortisol:
ex: Prednisone, Hydrocortisone
Education: Patient needs to report if they are having stress such as illness, surgery, or extra stress in life ( will need to increase dosage), take medication exactly as prescribed….don’t stop abruptly without consulting with MD.
For replacing aldosterone:
ex: Fludrocortisone aka Florinef
Education: consume enough salt..may need extra salt
Wearing a medical alert bracelet
Eat diet high in proteins and carbs, and make sure to consume enough sodium
Avoid illnesses, stress, strenuous exercise
Watch for Addisonian Crisis
This develops when Addison’s Disease isn’t treated.
In addisonian crisis, the patient has extremely LOW CORTISOL levels (life threatening).
Remember the 5 S’s
Sudden pain in stomach, back, and legs
Syncope (going unconscious)
Shock
Super low blood pressure
Severe vomiting, diarrhea and headache
NEED IV Cortisol STAT:
Solu-Cortef and IV fluids (D5NS to keep blood sugar and sodium levels good and fluid status)
Watch for risk for infection, neuro status (confusion, agitation), electrolyte levels (sodium and potassium, glucose)
Pasireotide is the first pituitary-directed drug approved for treating patients with Cushing’s disease (CD). Our 10-year experience with pasireotide in CD is reported here.
Twenty patients with de novo, persistent, or recurrent CD after pituitary surgery were treated with pasireotide from December 2003 to December 2014. Twelve patients were treated with pasireotide in randomized trials and 8 patients with pasireotide sc (Signifor®; Novartis AG, Basel, Switzerland) in clinical practice. The mean treatment duration was 20.5 months (median 9 months; range, 3-72 months).
Urinary free cortisol (UFC) levels mean percentage change (± SD) at last follow-up was-40.4% (± 35.1; range, 2-92%; median reduction 33.3%) with a normalization rate of 50% (10/20). Ten patients achieved sustained normalized late night salivary cortisol (LNSC) levels during treatment. LNSC normalization was associated with UFC normalization in 7/10 patients. Serum cortisol and plasma ACTH significantly decreased from baseline to last follow-up. Body weight decrease and blood pressure improvement during pasireotide treatment were independent from UFC response. Glucose profile worsening was observed in all patients except one. The frequency of diabetes mellitus increased from 40% (8/20) at baseline to 85% (17/20) at last follow-up requiring initiation of medical treatment only in 44% of patients.
Pasireotide treatment was associated with sustained biochemical and clinical benefit in about 60% of CD patients. Glucose profile alteration is a frequent complication of pasireotide treatment; however, it seems to be easy to manage with diet and lifestyle intervention in almost half of the patients.
People sometimes ask me how I found out I had Cushing’s Disease. Theoretically, it was easy. In practice, it was very difficult.
In 1983 I came across a little article in the Ladies Home Journal which said “If you have these symptoms…”
I found the row with my symptoms and the answer read “…ask your doctor about Cushing’s”.
After that article, I started reading everything I could on Cushing’s, I bought books that mentioned Cushing’s. I asked and asked my doctors for many years and all of them said that I couldn’t have it. It was too rare. I was rejected each time.
Due to all my reading at the library, I was sure I had Cushing’s but no one would believe me. My doctors would say that Cushing’s Disease is too rare, that I was making this up and that I couldn’t have it.
In med school, student doctors are told “When you hear hoofbeats, think horses, not zebras“.
According to Wikipedia: “Zebra is a medical slang term for a surprising diagnosis. Although rare diseases are, in general, surprising when they are encountered, other diseases can be surprising in a particular person and time, and so “zebra” is the broader concept.
The term derives from the aphorism “When you hear hoofbeats behind you, don’t expect to see a zebra”, which was coined in a slightly modified form in the late 1940s by Dr. Theodore Woodward, a former professor at the University of Maryland School of Medicine in Baltimore. Since horses are the most commonly encountered hoofed animal and zebras are very rare, logically you could confidently guess that the animal making the hoofbeats is probably a horse. By 1960, the aphorism was widely known in medical circles.”
So, doctors typically go for the easily diagnosed, common diseases. Just because something is rare doesn’t mean that no one gets it. We shouldn’t be dismissed because we’re too hard to diagnose.
When I was finally diagnosed in 1987, 4 years later, it was only because I started bleeding under the skin. My husband made circles around the outside perimeter each hour with a marker so my leg looked like a cut log with rings.
When I went to my Internist the next day he was shocked at the size of the rings. He now thought I had a blood disorder so he sent me to a Hematologist/Oncologist.
Fortunately, that new doctor ran a twenty-four hour urine test and really looked at me and listened to me. Both he and his partner recognized that I had Cushing’s but, of course, couldn’t do anything further with me. They packed me off to an endo where the process started again.
My final diagnosis was in October, 1987. Quite a long time to simply “…ask your doctor about Cushing’s”.
Looking back, I can see Cushing’s symptoms much earlier than 1983. But, that ‘s for a different post.
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