Cushing’s patients report high disease burden, despite treatment

Survey: Patients who had surgery scored better in health-related quality of life

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by Andrea Lobo |

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People with Cushing’s syndrome have a high disease burden linked to persistent symptoms of weight gain, pain, and anxiety, despite being treated, a recent study reported.

These observations highlight the need for interventions that address these persistent symptoms and “underscore the importance of the patient’s perspective in clinical decision making and the development of therapeutics for Cushing’s syndrome,” the researchers wrote in “Evaluating the burden of endogenous Cushing’s syndrome using a web-based questionnaire and validated patient-reported outcome measures,” which was published in Pituitary.

A feature of Cushing’s syndrome is an excess level of the hormone cortisol, or hypercortisolism. Cushing’s disease, one of the syndrome’s forms, is caused by tumors in the pituitary gland that lead to the excessive production and release of adrenocorticotropic hormone (ACTH), which is perceived by the adrenal glands as a signal to produce cortisol.

Cushing’s may also be caused by ACTH-producing tumors outside the pituitary gland or by adrenal tumors that produce cortisol. Regardless of the specific cause, this results in a variety of symptoms, including weight gain, fat accumulation in certain parts of the body, and skin changes.

To examine the ongoing burden of Cushing’s — from the appearance of the first symptoms through to treatment — researchers in the U.S. surveyed 55 Cushing’s syndrome patients who were recruited via patient panels, physician-assisted recruitment through third-party vendors, and by utilizing study flyers with relevant patient advocacy groups. To be eligible, patients had to have been diagnosed at least six months before entering the study and received at least one treatment at the time of the survey.

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Survey results about symptoms, treatments

The web-based survey, which was accessible via a computer, tablet, or smartphone, took about 30 minutes to complete. It evaluated Cushing’s symptoms, treatment history, and the impact of the disease on patients’ quality of life, utilization of healthcare resources, and productivity. The respondents were a mean age of 43.4 and the majority were women (85%).

Participants started having symptoms at a mean age of 30. A diagnosis was made a mean of 10 years later, however. This delay was mainly due to the inability of the primary care provider to diagnose the disease and to an initial misdiagnosis.

The majority (81%) had pituitary or adrenal tumors, whereas 20% had ectopic ACTH-producing tumors elsewhere in the body.

Most patients (80%) underwent surgery, mainly to remove adrenal (35%) or pituitary tumors (29%). Long-acting pasireotide (sold as Signifor LAR) was the most commonly used Cushing’s-specific medication (25%), followed by a short-acting release formulation of pasireotide (sold as Signifor) (18%).

Of the 46 patients who disclosed their height and weight, more than 95% were obese or overweight and most had coexistent disorders, such as high blood pressure, diabetes, or pre-diabetes.

About a third (36%) required assistance from a caregiver to manage their disease. Around half (49%) didn’t work, and 35% worked full time.

The burden associated with Cushing’s was significant, with 75% of participants reporting three to five symptoms at the time they first started to have Cushing’s-related symptoms and 64% having three to five symptoms at the time of the survey.

The most commonly reported symptoms were weight gain, muscle fatigue and weakness, and anxiety. Most eased over time with treatment, although anxiety and pain didn’t decrease significantly.

Even though the severity of weight gain, muscle weakness and fatigue, and menstrual changes was reduced after treatment, the symptoms were never fully eliminated. In contrast, the severity of anxiety and excessive hair growth didn’t change.

Based on the CushingQoL score, patients’ health-related quality of life was moderately impacted by Cushing’s. Most patients (69%) reported moderate or severe fatigue, as measured by the Brief Fatigue Inventory.

In a typical month, patients had symptoms on 16 days and those working reported missing two days of work (around 25 days a year) due to their condition. On average, they visited their physicians as outpatients six times a year.

Patients who underwent surgery had better scores on health-related quality of life, pain, fatigue, and anxiety measures than those who didn’t. These patients also had a higher number of symptom-free days a month and missed fewer workdays due to Cushing’s in the last 30 days.

“Overall, these data identify a continued high [disease burden] in treated … [Cushing’s syndrome] patients and highlight an unmet need for interventions that address their symptomatic burden,” the researchers wrote.