Cushing’s Disease Stresses Your Heart and Your Mental Health

Written by Kathleen Doheny

With Oskar Ragnarsson, MD, PHD, and Tamara Wexler, MD, PhD

Adults with Cushing’s syndrome, also called hypercortisolism, have a three-fold higher risk of dying from heart disease compared to the general population,1 according to findings reported by a Swiss research team. Although the researchers found that the risk drops when patients are under care, receiving treatment, and are in remission, the risks don’t disappear completely.  For some perspective, heart disease is common in the United States, affectingone in four adults, regardless of health status.2

Patients with Cushing’s disease have excess mortality [risk],” says Oskar Ragnarsson, MD, PhD, associate professor and a senior consultant in internal medicine and endocrinology at Sahlgrenska University Hospital in Gothenburg, Sweden. He is the author of the study, which appears in the Journal of Clinical Endocrinology & Metabolism.

Having Cushing’s Requires Vigilance Beyond Disease Symptoms

Still, the news is not all bleak, he says. Simple awareness of the increased risks can help individuals reduce their risk, just as following your doctor’s treatment plan so remain in remission, Dr. Ragnarsson tells EndocrineWeb. In addition, patients who received growth hormone replacement appear to have better overall outcomes.1

Cushing’s syndrome occurs when your body is exposed to high levels of the hormone cortisol over a long period of time. This can be caused either by taking corticosteroid medicine orally, or if your body just makes too much cortisol. Common symptoms of this condition include: having a fatty hump between the shoulders, a rounded face, and stretch marks with pink or purple coloring on the skin. Complications, if Cushing’s disease goes untreated, may include bone loss (leading to increased risk of fractures and osteoporosis), high blood pressure, type 2 diabetes, and other problems. Usual treatment includes medication and surgery that are aimed to normalize cortisol levels.3

Increased Risks Are Cause for Concern in Cushing’s Disease

The researchers analyzed data from 502 men and women, all of whom were diagnosed with Cushing’s disease between 1987 and 2013 as indicated in a Swedish health database.1 The average age of these patients at diagnosis was 43 years, and, 83% of these individuals were in remission. During a median follow up of 13 years—half followed for longer, half followed for less time—the researchers noted 133 deaths, more than the 54 that had been anticipated in this patient population.

From this data,1 Dr. Ragnarsson and his team calculated that people with Cushing’s disease were about 2.5 times more likely to die than the general population. The most common reason, with more than a 3-fold increased risk, was attributed to events associated with cardiovascular disease, encompassing both heart disease and stroke. This group also appeared to have a higher risk of death from infectious and respiratory diseases, and conditions related to gastrointestinal problems.

Fortunately, just being in disease remission helps to reduce the risk of all-cause mortality,1 the researchers’ report, with both men and women whose Cushing’s disease is well-managed having a two-fold lower risk of death during the follow-up period.1 Those in remission who were receiving growth hormone had an even lower risk of death than those on other forms of treatment.

In addition, the researchers looked at the 55 patients with Cushing’s disease who were in remission and also had diabetes, finding that their risks remained the same. In other words, despite a strong relationship between diabetes and increased heart disease, the risks of death were not increased in this group of patients.1

In considering the impact that treatments may have, the researchers found:

  • 3 in 4 of these patients (75%) had undergone pituitary surgery
  • 28% had undergone radiotherapy
  • 1 in 4 (24%) had had both adrenal glands removed

Those who had their adrenal glands removal experienced a 2.7-fold higher risk of death, while those who were treated with radiotherapy or had pituitary surgery did not have an increased risk associated with cardiovascular events. When glucocorticoid therapy was added, it did not affect results, according to Dr. Ragnarsson and his research team.

Bottom line? “Even though patients in remission have a better prognosis than patients not in remission, they still have more than a 2-fold increased mortality [risk],” he says. The study, he says, is the first to uncover a high rate of death from suicide in Cushing’s patients. It has been reported before, but the numbers found in this study were higher than in others.

The findings, he says, emphasize the importance of treating Cushing’s with a goal of remission. Ongoing surveillance and management are crucial, he says. “Also, evaluation and active treatment of cardiovascular risk factors and mental health is of utmost importance,” Dr. Ragnarsson tells EndocrineWeb.

Remission Reduces But Doesn’t Eliminate Serious Risks 

The study findings underscore the message that ”the priority for patients is to achieve biochemical remission,” says Tamara L. Wexler, MD, PhD, director of the NYU Langone Medical Center Pituitary Center, in reviewing the findings for EndocrineWeb.

“One question raised by the study findings is whether patients listed as being in remission were truly in (consistent) remission,” Dr. Wexler says. “One or more of several testing methods may have been used, and the data were based on medical record reviews so we can’t be certain about the status of these patients’ remission. In addition, we don’t know how much excess cortisol patients were exposed over time, which may change their risks.”

I have another concern about the findings, she says. While the method of analysis used in the study suggests that the length of time from diagnosis to remission is not associated with increased death risk, ”it may be that the total exposure to excess cortisol—the amplitude as well as duration—is related to morbidity [illness] and mortality [death] risk.” And, she adds, any negative effects experienced by patients with Cushing’s disease may be reduced further as remission status continues.

In addition, Dr. Wexler considers the authors’ comments that sustained high cortisol levels may impact the cardiovascular system in a way that is chronic and irreversible ”may be overly strong.” She believes that the total cortisol exposure and the duration of remission may both play important roles in patients’ ongoing health.

She does agree, however, with the researchers’ recommendation of the need to treat heart disease risk factors more aggressively in patients with a history of Cushing’s disease. Equally important, is for patients to be warned that there is an increased concern about suicide, she says, urging anyone with Cushing’s disease to raise all of these concerns with your health practitioner.

Overall, the study findings certainly suggest that it is important for you to know that if you have Cushing’s syndrome, you are at increased risk for not just heart disease but also mental health disorders and other ailments than the general population, she says, and that the best course of action is to work closely with your doctor to achieve remission and stick to your overall treatment plan.

Steps to Take to Reduce Your Risks for Heart Disease and Depression

Dr. Ragnarsson suggests those with Cushing’s disease make adjustments as needed to achieve the following risk-reducing strategies:

  • Be sure your food choices meet the parameters of a heart-healthy diet
  • You are getting some kind of physical activity most every day
  • You see your doctor at least once a year to have annual checks of your blood pressure, blood sugar, and other heart disease risk factors.

For those of you receiving cortisone replacement therapy, you should be mindful of the need to have a boost in your medication dose with your doctors’ supervision when you’re are sick or experiencing increased health stresses.

From https://www.endocrineweb.com/news/adrenal-disorders/61675-cushings-disease-stresses-your-heart-your-mental-health

Vision Loss The First Sign Of Adrenal Tumour In 42-Year-Old Patient

A 42-year-old woman who presented to hospital with acute vision loss in her right eye was diagnosed with a benign tumour in her adrenal gland.

Writing in BMJ Case Reports, clinicians described how the patient presented with a visual acuity of 6/36 in her right eye and 6/6 in her left eye.

Investigations revealed an exudative retinal detachment in her right eye as well as a pigment epithelial detachment.

The patient had multifocal central serous retinopathy in both eyes.

The woman, who had hypertension and diabetes, was diagnosed with Cushing syndrome and a right adrenal adenoma was also discovered.

During a treatment period that spanned several years, the patient received an adrenalectomy followed by a maintenance dose of steroids.

The patient subsequently developed central serous retinopathy again which the clinicians believe might be related to steroid use.

The authors advised “careful deliberation” in prescribing a maintenance dose of steroids following removal of the adrenal glands because of the potential link to retinopathy.

From https://www.aop.org.uk/ot/science-and-vision/research/2018/12/17/vision-loss-the-first-sign-of-adrenal-tumour-in-42-year-old-patient

Pituitary Tumors Affect Patients’ Ability to Work, Reduce Quality of Life

Pituitary tumor conditions, such as Cushing’s disease, have a substantial effect on patients’ work capabilities and health-related quality of life, researchers from The Netherlands reported.

The study, “Work disability and its determinants in patients with pituitary tumor-related disease,” was published in the journal Pituitary.

Pituitary tumors, like those that cause Cushing’s disease, have significant effects on a patient’s physical, mental, and social health, all of which influence their work status and health-related quality of life. However, the effects of the disease on work status is relatively under-investigated, investigators report.

Here, researchers evaluated the work disability among patients who were treated for pituitary tumors in an attempt to understand the impact of disease diagnosis and treatment on their social participation and ability to maintain a paying job.

In their study, researchers examined 241 patients (61% women) with a median age of 53 years. The majority (27%) had non-functioning pituitary tumors, which do not produce excess hormones, but patients with acromegaly, Cushing’s disease, prolactinomas, and Rathke’s cleft cyst also were included.

Participants were asked to complete questionnaires to evaluate their health-related quality of life and disease-specific impact on their work capabilities. Each participant completed a set of five questionnaires.

Participants also reported their hormonal status and demographic data, including gender, age, education, and marital status. Specific information, such as disease diagnosis, treatment, and tumor type was obtained from their medical records.

Work status and productivity were assessed using two surveys, the Short-Form-Health and Labour Questionnaire (SF-HLQ) and the work role functioning questionnaire 2.0 (WRFQ).

SF-HLQ was used to obtain information on the participants’ employment and their work attendance. Employment was either paid or unpaid. (Participation in household chores was considered not having a paid job.)

WRFQ is a 27-question survey that determines work disability regarding being able to meet the productivity, physical, emotional, social, and flexible demands. A higher score indicates low self-perceived work disability.

Disease-specific mood problems, social and sexual functioning issues, negative perceptions due to illness, physical and cognitive difficulties, were assessed using a 26-item survey called Leiden Bother and Needs for Support Questionnaire for pituitary patients(LBNQ-Pituitary).

Overall, 28% of patients did not have a paid job, but the rates increased to 47% among those with Cushing’s disease. Low education, hormonal deficits, and being single were identified as the most common determinants of not having a paid job among this population.

Further analysis revealed that more patients with Cushing’s disease and acromegaly had undergone radiotherapy. They also had more hormonal deficits than others with different tumor types.

Overall, patients with a paid job reported working a median of 36 hours in one week and 41% of those patients missed work an average of 27 days during the previous year. Health-related problems during work also were reported by 39% with a paid job.

Finally, health-related quality of life was determined using two questionnaires: SF-36 and EQ-5D. The physical, mental, and emotional well being was measured with SF-36, while ED-5D measured the health outcome based on the impact of pain, mobility, self-care, usual activities, discomfort, and anxiety or depression. In both SF-36 and EQ-5D, a higher score indicates a better health status.

Statistical analysis revealed that the quality of life was significantly higher in patients with a job. Overall, patients with a paid job reported better health status and higher quality of life than those without a paid job.

Although 40% of the patients reported being bothered by health-related problems in the past year, only 12% sought the help of an occupational physician, the researchers reported.

“Work disability among patients with a pituitary tumor is substantial,” investigators said.

“The determinants and difficulties at work found in this study could potentially be used for further research, and we advise healthcare professionals to take these results into consideration in the clinical guidance of patients,” they concluded.

From https://cushingsdiseasenews.com/

Rare Case of Cushing’s Disease Diagnosed in 7-year-old Boy

A recent case report describes a 7-year-old boy with Cushing’s disease who had an unusual clinical presentation, which significantly delayed his diagnosis.

The study, “A variable course of Cushing’s disease in a 7 year old: diagnostic dilemma,” was published in the Journal of Pediatric Endocrinology and Metabolism.

Rare in children and adolescents, Cushing’s disease refers to overproduction of cortisol caused by excessive adrenocorticotropic hormone (ACTH) secretion from a pituitary tumor. In pediatrics, early symptoms of excess cortisol include weight gain and delayed growth.

Despite being extremely unlikely in children younger than 7, some cases of Cushing’s disease in infancy have been reported.

“If undiagnosed or untreated it can lead to considerable morbidity and mortality, and the inability to detect a microadenoma [tumors smaller than 10 mm in diameter] on magnetic resonance imaging (MRI) can lead to a diagnostic dilemma leading to unnecessary delays in treatment initiation,” the researchers wrote.

Researchers from the Indraprastha Apollo Hospital in New Delhi, India, described a 7-year-old boy who complained of excessive appetite and weight gain in the previous five months. The child weighed 46.8 kg, was 127 cm tall, and had a body mass index (BMI) of 29, indicating he was overweight.

The child’s excess fat was mainly in his abdomen plus he had a round, red, puffy face, which are both common features of Cushing’s disease. He had no history of acute or chronic steroid intake, mood swings, sleep disorders, or issues with eyesight.

Given his clinical presentation, the investigators suspected the boy had Cushing’s disease or pseudo-Cushing’s disease, which refers to situations where the overproduction of cortisol is caused by something unrelated to the disease, such as stress or uncontrolled diabetes mellitus.

Biochemical testing showed the patient had high levels of cortisol, which remained unchanged after a dexamethasone suppression test. In addition, his levels of “bad” cholesterol, referring to low-density lipoprotein, were extremely elevated at 194 mg/dL, where a normal range is defined as less than 110 mg/dL.

Imaging revealed no lesions in the pituitary gland.

The boy was sent home with dietary recommendations. Eight weeks later, he had lost 4 kg, while his height remained the same; he also complained of headaches and various episodes of double vision.

This confused the clinical team as hallmarks of Cushing’s disease include short stature and weight loss triggered by pharmacological therapy. Despite having lost weight, he did not take any medications to help him with it, plus the boy’s height was normal for his age.

Nonetheless, the patient was complaining of neurological symptoms, suggesting progression of Cushing’s disease.

An ophthalmologist did not observe anything abnormal with the child’s eyes that could explain his double vision episodes.

A new series of tests revealed slightly elevated 24-hour urinary cortisol levels, decreased concentration of ACTH, and mildly increased cortisol levels after a two-day dexamethasone suppression test.

Magnetic resonance imaging (MRI) showed a small microadenoma in the right lobe of the pituitary gland.

Using Gamma Knife radiation therapy, a kind of high-precision radiation therapy, and surgery, doctors successfully removed the boy’s microadenoma. Six weeks post-procedure, his cortisol and ACTH concentrations returned to normal.

“MRI findings of the pituitary may be inconclusive in the beginning of the disease process and should be borne in mind during further follow-up. In cases where a clear-cut diagnosis may be difficult, a diligent follow-up is required to ascertain the course of the disease and to make timely diagnosis,” the investigators concluded.

From https://cushingsdiseasenews.com/2018/10/25/rare-case-cushings-disease-diagnosed-7-year-old-boy-case-study/

Finding Ways to Deal with Post-surgery Anxieties

Post-traumatic stress disorder (PTSD) following Cushing’s disease is a real issue many of us face. However, we don’t have to let it control our lives — there are ways to cope.

Cushing’s changes us both mentally and physically. We become forgetful. We lose strength. We become someone we don’t recognize in the mirror. We lose hair on our heads and gain it everywhere else. We’re always in pain, and we’re always sick, with no end in sight (or at least it feels that way).

Some days will be trying and seem as if nothing seems to work, no matter what you do. I promise that you’re not alone, and you will make it through those days.

Following are a list of ways to deal with post-surgery scares:

  • Therapy/counseling: If you can afford it, talk with a professional about your health worries and how your anxiety affects you. It takes the burden off your caregivers who don’t like to see you suffer because they care so much.
  • Journaling: Journaling is a therapeutic and inexpensive way to let out your worries. Documenting your anxieties can help you keep track of how your thought processes are changing. Writing out your stresses is cathartic. Give it a try — if you haven’t already.
  • Yoga or any light exercise: If you’re in the early stages of recovery, you shouldn’t go straight back to the gym — working out is a stressor on the body. Light yoga, such as restorative yoga, in which you practice stretching, deep breathing, and relaxation, will help your mind and body to recover. Light walks are amazing for the brain and body post-surgery.
  • Delve into things you enjoy: Read, cook, go for walks, sit outside, etc. Do whatever feeds your soul and keeps your mind free from negative thoughts. Feeding your soul is one sure way to keep your mind and body happy and healthy.
  • Other ideas from the CushieWiki
  • Please share your ideas in the comments on this post or on the message boards

Adapted from https://cushingsdiseasenews.com/2018/10/19/cushings-post-surgery-anxieties-ptsd-post-traumatic-stress-disorder-journaling-yoga-therapy/

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