Improving quality of life in patients with pituitary tumours

Evaluation of health-related quality of life (QoL) in people with pituitary tumours has received much attention over the last 10-15 years. Most of them show impaired QoL, but little is known about how to prevent impairment or how to improve QoL. Our aim is to review what is known about QoL in pituitary tumours patients and to highlight the areas worth improving, for the patient's well being. The article has four sections: acromegaly, Cushing's syndrome, prolactinomas and non-functioning adenomas. Control of comorbidities is usually an important factor to prevent QoL impairment; however, each disease has specific characteristics that should be properly addressed in order to obtain full patient recovery after successful therapy

Quality of life in Cushing's disease: a long term issue?

The purpose of this review is to describe how quality of life (QoL) is impaired in patients with hypercortisolism due to Cushing's syndrome of any aetiology, including pituitary-dependent Cushing's disease. It is worse in active disease, but improvement after successful therapy is often incomplete, due to persistent physical and psychological co-morbidities, even years after endocrine "cure". Physical symptoms like extreme fatigability, central obesity with limb atrophy, hypertension, fractures, and different skin abnormalities severely impair the affected patients' everyday life. Psychological and cognitive problems like bad memory, difficulties to concentrate and emotional distress, often associated with anxiety and depression, make it difficult for many patients to overcome the aftermath of treated Cushing's syndrome. Recent studies have shown diffuse structural abnormalities in the central nervous system during active hyper-cortisolism, thought to be related to the wide distribution of glucocorticoid receptors throughout the brain. Even though they improve after treatment, normalization is often not complete. Shortening the exposure to active Cushing's syndrome by reducing the often long delay to diagnosis and promptly receiving effective treatment is highly desirable, together with preparing the patient for the difficult periods, especially after surgery. In this way they are prepared for the impairments they perceive in every day life, and live with the hope of later improvement, which can be therapeutic in many instances

Development of a new tool for assessing Health-Related Quality of Life in patients with primary hyperparathyroidism

Background: Several studies in recent years have evaluated Health Related Quality of Life (HRQoL) of patients with primary hyperparathyroidism (PHPT). No disease specific questionnaires are available to assess the impact of the disease. The aim of this research is to describe the development of a new disease specific Quality of Life (QoL) questionnaire for use specifically with PHPT patients. Methods: A conceptual model was developed describing the impact of the disease and its symptoms on QoL domains. A literature review was conducted to identify the most relevant domains. A focus group with experts was used to validate the domains; 24 patients were also interviewed to complement the information from the patient's perspective. A content analysis of the interviews was performed to identify items related with the impact of the disease, leading to PHPQoL-V.1 which was presented to a sample of 67 patients. Reliability was assessed by Cronbach's coefficient alpha and item-total score correlations. Validity was assessed by a factor analysis performed to determine the number of domains. Rasch analysis was carried out in order to refine the questionnaire items. Results: 259 items were extracted from the interviews that were subsequently reduced to 34 items. Cronbach's coefficient alpha was 0.92. The factor analysis extracted two domains (physical and emotional). After Rasch analysis the questionnaire PHPQoL-V.2 kept 16 items (9 physical and 7 emotional). The questionnaire was developed in a Spanish population and the final version was translated to English through translation and back-translation. Conclusion: The first disease specific HRQoL questionnaire for PHPT patients (PHPQoL-16) has been developed. Validation studies designed to assess measurement properties of this tool are currently underway

Patient-centred outcomes with pituitary and parasellar disease

Over the last 2 decades advances in the diagnosis and management of pituitary diseases have made it possible to attain an endocrine 'cure' in a large proportion of patients. In other words, tumors can be excised or controlled with drugs, and mass effect of the lesion on surrounding structures be solved, and pituitary deficiencies can be substituted with all relevant hormones. While this is considered a satisfactory outcome for health care providers, patients often suffer from an aftermath of prior endocrine dysfunction exposure, with irreversible effects both physically and psychologically, which have a great impact on their everyday life. Diagnostic delay, often of several years, adds a negative impact on health perception. This affects their social, professional and family domains and determines their future life. Understanding that this may occur is important and health care providers should offer information to prepare the patient for this difficult journey, especially in the case of acromegaly, Cushing disease or hypopituitarism. In order to maintain in the long-term a good quality of life, patients need to adapt to this new situation, something that may be difficult, since they often cannot continue with all the activities and rhythm they used to do. Depression is often the consequence of maladaptation to the new situation, leading to impaired quality of life

Percepción de la información recibida en los pacientes con síndrome de cushing y riesgo cardiovascular

El síndrome de Cushing (SC) es una enfermedad rara caracterizada por una hipersecreción de cortisol. La principal causa de morbi y mortalidad en el SC es la enfermedad cardiovascular. Objetivos generales: el objetivo fue evaluar la percepción del paciente con SC sobre la información recibida en aspectos concretos de la patología y hábitos de vida en un seguimiento realizado entre 2008 al 2009. Material y método: estudio observacional descriptivo desarrollado en la Unidad de Enfermedades Hipofisarias del Centro de Investigación de enfermedades Raras (CIBERER 747) sobre 30 pacientes visitados en el dispensario de endocrinología por SC. La población era de 60 pacientes diagnosticados de SC. La recogida de datos se realizó a través de un cuestionario ad-hoc, contemplando aspectos referentes a la información recibida sobre su patología en las siguientes áreas: SC, síntomas, signos, tratamiento, pruebas complementarias, tratamiento, complicaciones, factores de riesgo, hábitos de vida nutricional, deseo sexual, actividad física y descanso. Se evaluó a través de la escala de Likert siendo 0 ninguna información y 10 la información máxima recibida. Resultados: la puntuación superior a 5 en la escala de Likert, suficientemente informados, hace referencia a aspectos generales de su patología (88%), complicaciones (60%) y tratamiento (80%), los valores inferiores a 5, bastante o totalmente desinformados, corresponden a áreas especificas de enfermería, factores de riesgo (76%), hábitos de vida (82%), nutricionales (78%), sexuales (97%), actividad física (93%) y descanso (100%). Conclusiones: la percepción del paciente sobre la información recibida en algunas áreas específicas de enfermería es insuficiente o inexistente

Factores de riesgo cardiovascular entre pacientes con síndrome de Cushing, curados y no curados con respecto a un grupo control

Objetivos: Describir los factores de riesgo cardiovascular que se presentan en el paciente con Síndrome de Cushing (SC), para establecer programas educativos adaptados a este grupo de pacientes. Método: El ámbito de estudio se desarrolló en el Hospital de Sant Pau, en el Servicio de Endocrinología. Se extrajeron datos de las Historias Clínicas de los pacientes que fueron visitados en consultas con diagnóstico de SC en el 2005, realizándose una recogida retrospectiva. No se excluyó ningún paciente, independientemente de los años de duración de la patología, de las opciones terapéuticas o del tratamiento farmacológico recibido, incluyendo todos los pacientes con SC. Elaboramos una hoja de recogida de datos demográficos, clínicos (peso, índice de masa corporal, tensión arterial, perímetro abdominal) y analíticos (lípidos, glicemia); para el tratamiento informático se utilizó el programa SPSS. Resultados: El perímetro abdominal fue mayor en pacientes no curados (100.8 ±13.9cm) que en controles (89±12.8cm) (p<0.05). Respecto al peso no se encontraron diferencias significativas entre controles (69,9±13,3Kg), curados (69,9±16,5Kg) y no curados (76,4±16,5Kg). El IMC tampoco reflejó diferencias en los tres grupos en estudio: control (26,5±5,2), curados (27,8±6,4) y no curados (29,3±3,8). El colesterol fue más alto en los pacientes curados (5.90±0.92mmol/L) que en controles (5.38±1.07mmol/L) (p<0.05), al igual que los triglicéridos (1.20±0.51mmol/L versus controles 1.05±0.56mmol/L; p<0.05). Ambos grupos de pacientes presentaron presión sistólica mayor que los controles (curados,128.3±16.7mmHg p<0.05; no curados,134.1±13mmHg, p<0.001). Los no curados (78.6±9.2mmHg) presentaron además presión diastólica mayor que los controles (72.3±8.6, p<0.05) Fig.3. No observamos diferencias en la glicemia basal entre los 3 grupos de pacientes. Conclusiones: Los pacientes con SC, curados de su hipercortisolismo y con enfermedad activa presentan mayor riesgo cardiovascular y metabólico, comparados con el grupo control. La enfermera educadora deberá contemplar e incluir en los programas de prevención secundaria a dichos pacientes, para promocionar hábitos saludables, prevenir y controlar sus factores de riesgo. Palabras Clave Síndrome Cushing, educación sanitaria, factores de riesgo cardiovascular

Body composition after endogenous (Cushing's syndrome) and exogenous (rheumatoid arthritis) exposure to glucocorticoids

Exposure to chronic glucocorticoid (GC) excess determines changes in body composition. The aim of the study was to compare body composition in women exposed to endogenous hypercortisolism (Cushing's syndrome, CS), exogenous glucocorticoid treatment (rheumatoid arthritis, RA) and controls. Fifty-one CS women, 26 RA women treated with low-dose prednisone (5 mg/day or 10 mg/2 days), and 78 female controls were included. Fourteen CS patients were hypercortisolemic, 37 in remission (10 required hydrocortisone substitution after surgery). Body composition parameters were measured by dual-energy X-ray absorptiometry scanning (DEXA). RA patients had a greater waist-hip ratio (WHR) (p<0.01), less lean body mass (LBM) (p<0.01), and lumbar bone mineral density (BMD) (p<0.01) than controls. CS patients, globally and those with cured disease, had more total fat (both percentage and kg) and trunk fat percentage, and less whole body-BMD than RA patients (p<0.05, p<0.01, p<0.05, respectively). Active CS patients had less whole body-BMD and more LBM than RA patients (p<0.05, p=0.01, respectively). Cured CS patients not taking hydrocortisone had more total fat [both percentage (p<0.05) and kg (p<0.05)], trunk fat percentage (p<0.05), lumbar BMD (p<0.01) than RA patients. Cured CS patients requiring hydrocortisone only differed from RA patients by smaller WHR (p<0.01). All the differences in BMD disappeared when the data were reanalyzed including only the estrogen-deficient groups. Hypercortisoliof CS determines an irreversible increase in body fat, greater than in RA. Endogenous and exogenous exposure to GC negatively affects body composition by increasing the WHR. There appears to be no additional effect on BMD in estrogen-deficient women

A specific nursing educational program in patients with Cushing's syndrome

Cushing's syndrome (CS) is a rare endocrine disease, due to cortisol hypersecretion. CS patients have comorbidities, often still present after biochemical cure. Specific nursing healthcare programs to address this disease and achieve improved health related quality of life (HRQoL) are lacking. Thus, an educational nursing intervention, through the development and promotion of specific educational tools, appears to be justified. The objective of this study is to assess the effectiveness of an educational nursing program in CS patients on HRQoL, clinical parameters, level of pain and physical activity, patterns of rest, and use of health resources. A prospective, randomized study was conducted in two reference hospitals for CS. Sixty-one patients (mean age 47 ± 12.7 years, 83.6 % females) were enrolled and divided into 2 groups: an 'intervention' group where educational sessions were performed over 9 months and a 'control' group, without these sessions. Specific questionnaires were used at the beginning and end of the study. After educational sessions, the intervention group had a better score in the CushingQoL questionnaire (p < 0.01), reduced level of pain (p < 0.05), improved physical activity (p < 0.01) and healthy lifestyle (p < 0.001) compared to the control group. A correlation between the CushingQoL score and reduced pain (r = 0.46, p < 0.05), improved physical activity (r = 0.89, p < 0.01), and sleep (r = 0.53, p = 0.01) was observed. This educational nursing program improved physical activity, healthy lifestyle, better sleep patterns, and reduced pain in CS patients, influencing HRQoL and reducing consumption of health resources. Moreover, the brief nature of the program suggests it as a good candidate to be used in CS patients

Cardiovascular risk and white matter lesions after endocrine control of Cushing's syndrome

Objective: Cushing's syndrome (CS) is associated with high cardiovascular risk. White matter lesions (WML) are common on brain magnetic resonance imaging (MRI) in patients with increased cardiovascular risk. AIM: To investigate the relationship between cardiovascular risk, WML, neuropsychological performance and brain volume in CS. Design/methods: Thirty-eight patients with CS (23 in remission, 15 active) and 38 controls sex-, age- and education-level matched underwent a neuropsychological and clinical evaluation, blood and urine tests and 3Tesla brain MRI. WML were analysed with the Scheltens scale. Ten-year cardiovascular risk (10CVR) and vascular age (VA) were calculated according to an algorithm based on the Framingham heart study. Results: Patients in remission had a higher degree of WML than controls and active patients (P<0.001 and P=0.008 respectively), which did not correlate with cognitive performance in any group. WML severity positively correlated with diastolic blood pressure (r=0.659, P=0.001) and duration of hypertension (r=0.478, P=0.021) in patients in remission. Both patient groups (active and in remission) had higher 10CVR (P=0.030, P=0.041) and VA than controls (P=0.013, P=0.039). Neither the 10CVR nor the VA correlated with WML, although both negatively correlated with cognitive function and brain volume in patients in remission (P<0.05). Total brain volume and grey matter volume in both CS patient groups were reduced compared to controls (total volume: active P=0.006, in remission P=0.012; grey matter: active P=0.001, in remission P=0.003), with no differences in white matter volume between groups. Conclusions: Patients in remission of Cushing's syndrome (but not active patients) have more severe white matter lesions than controls, positively correlated with diastolic pressure and duration of hypertension. Ten-year cardiovascular risk and vascular age appear to be negatively correlated with the cognitive function and brain volume in patients in remission of Cushing's syndrome

Quality of life in Cushing's disease: a long term issue?

The purpose of this review is to describe how quality of life (QoL) is impaired in patients with hypercortisolism due to Cushing's syndrome of any aetiology, including pituitary-dependent Cushing's disease. It is worse in active disease, but improvement after successful therapy is often incomplete, due to persistent physical and psychological co-morbidities, even years after endocrine "cure". Physical symptoms like extreme fatigability, central obesity with limb atrophy, hypertension, fractures, and different skin abnormalities severely impair the affected patients' everyday life. Psychological and cognitive problems like bad memory, difficulties to concentrate and emotional distress, often associated with anxiety and depression, make it difficult for many patients to overcome the aftermath of treated Cushing's syndrome. Recent studies have shown diffuse structural abnormalities in the central nervous system during active hyper-cortisolism, thought to be related to the wide distribution of glucocorticoid receptors throughout the brain. Even though they improve after treatment, normalization is often not complete. Shortening the exposure to active Cushing's syndrome by reducing the often long delay to diagnosis and promptly receiving effective treatment is highly desirable, together with preparing the patient for the difficult periods, especially after surgery. In this way they are prepared for the impairments they perceive in every day life, and live with the hope of later improvement, which can be therapeutic in many instances