Cushing’s Disease: What Every Diabetic Needs to Know

Cushing’s disease, (CD) it sounds as though it should be something soft or fluffy; something that would be okay. I’d never heard of CD until January 26, 2007. That’s the date my endocrinologist, recognized several physical characteristics in me that were typical of patients with excess production of the hormone cortisol, a constellation of symptoms refereed to as “Cushing’s syndrome”. By February 2, I was undergoing computed tomography (CT) and magnetic resonance imaging (MRI) scans for a possible tumor on the pituitary gland. I was scared and it didn’t take me long to realize that “Cushing’s” is not soft or fluffy.

According to Vivian Bonert, MD, clinical director of the Pituitary Center at Cedars-Sinai Medical Center, in Los Angeles, CA, the rare disease is caused by too much circulating cortisol in the bloodstream. The pituitary gland normally controls cortisol production from the adrenal gland.

Patients with CD develop a tumor of the pituitary gland, which causes a disturbance in the control of adrenal cortisol production and consequently too much cortisol secretion from the adrenal gland, Dr. Bonert explained. Patients with CD gain excessive weight, develop high blood pressure and diabetes mellitus, and complain of muscle weakness, poor concentration, and thinning of the bones. As these are common symptoms, patients frequently suffer from CD for many years before the diagnosis is made.

In order to make an early diagnosis, all health care professionals should have a high index of suspicion for CD, she said. The pituitary tumor is identified by performing hormone tests on blood and urine, and it is confirmed by an MRI of the pituitary gland. The treatment of choice is surgical removal of the pituitary tumor by an experienced pituitary surgeon. Cushing’s disease is most common in adults ages 20-50, although it can occur at any age. It affects women five times more often than men. An estimated 10 to 15 people per million are affected each year.1 I believe this low figure is due to an initial diagnosis not being made by most physicians.

Characteristics

(not all characteristics are found in any one person)

• Diabetes
• Abdominal weight gain
• Hypertension
• Poor concentration
• Poor short-term memory
• Irritability
• Acne
• Excessive hair growth (women)
• Red, ruddy face and neck
• Extra fat around neck (buffalo hump on back of neck)
• Round face (moon face)
• Fatigue/muscle weakness
• Menstrual irregularity
• Stretch marks on abdomen/thighs (purple)
• Insomnia
• Balding (women)
• Hip and shoulder weakness
• Swelling of feet/legs
• Excessive hunger
• Excessive thirst
• Frequent urination
• Depression

My experience with CD took me to The Pituitary Center at Cedars-Sinai Medical Center in Los Angeles, CA. Pituitary surgery is very delicate in nature and patients should find an experienced team of professionals if surgery is recommended. “The surgery most often recommended is known as transsphenoidal surgery. This is typically carried out by going through the nostrils or upper lip and through the sphenoid sinus to reach the tumor.” (The Pituitary Society).

According to Dr. Adam Mamelak, a neurosurgeon and co-director of the Pituitary Center at Cedars-Sinai, states, especially for patients with CD finding an experienced surgeon is critical to success. These tumors are often very small and not visible on MRI, so surgical cure depends heavily on the skill of the surgeon. Untreated, CD can be fatal, so it is crucial that patients be evaluated at a comprehensive center with experienced surgeons.

I had two surgeries within 6 weeks for removal of the pituitary tumor and eventually the entire pituitary gland. According to my laboratory tests, I am now cured, but I will also take Hydrocortisone, Synthroid, and Desmopressin Acetate for the rest of my life. I wear a medical alert bracelet to make sure I get supplement steroid hormones if I am in a medical emergency, since my body now lacks the ability to make its own cortisol. I want to inform people of CD as it can and does go undiagnosed for years at a time and can be fatal if left untreated. Three and one-half months, post surgery, I was back at work as a speech/language pathologist (SLP) in private practice, Good Speech, Inc., Las Vegas, NV.

As an SLP with over 25 years of experience I have found some language deficits in myself. These deficits are – but not found in the literature – word retrieval, memory, “language of confusion.” This confusion is worse on some days than on others. It is characterized by interchanging the beginning or ending consonants of single words such as saying, ford or chord and meaning board, or saying bed and meaning head. I recently used the word freight and meant straight. It’s weird and I recognize it as it is happening and able to self-correct it most of the time. My therapy model is self and family oriented. I am doing many word-retrieval exercises, I watch old movies (which I love) and identify actors, scenes, dialogue, etc., and I write.

Total recovery has been documented to take up to three years. I continue to experience muscle weakness, and extreme muscle aches due to “cortisol withdrawal.” Fatigue, dry skin, and getting very, very cold are also a part of my recovery. I lost 30 pounds in two months, lowered my insulin intake from 105 units per day to 8 units, cut blood pressure medication in half, lost the “moon face,” the ruddy red neck and the “buffalo hump.” My acne also subsided quickly and my concentration and word retrieval are getting better and the bonus is – I don’t have to shave my legs very often, for now anyway!

I hope that none of you or your family members experience CD. It is not readily identified by even experienced doctors. I was lucky; I went to only two doctors. (Many CD patients go to numerous physicians before CD is identified. That is because they tend to treat the symptoms rather than the origin of the disease.) The first things that were identified were my inability to loose weight and failure to lower my blood sugars regardless a myriad of medical interventions.

It is important that all diabetics are aware of the characteristics because CD can co-exist with other disabilities and it crosses cultures and age groups. CD is rarely diagnosed and it should be recognized as a disease that is not soft and fluffy but one that can be cured.