Putting pituitary disease on the radar in Irish healthcare

'The greatest part of our workload in the pituitary unit in Beaumont is pituitary tumours which have been referred for multidisciplinary work-up and consideration of neurosurgical intervention'

To mark National Pituitary Awareness Week, Prof Chris Thompson looks at this underdiagnosed and underfunded illness and the need to raise awareness of its complications.

 

The commencement of National Pituitary Awareness Week in Ireland provides a useful backdrop against which to write an article to highlight pituitary disease in this country.  To many doctors, pituitary disease is relegated to the status of a side show of the greater endocrinology speciality.

In Ireland, where, uniquely, endocrinology and diabetes are regarded as the same speciality — in most other Western countries they are entirely separate entities in large teaching hospitals — the development of services for patients with pituitary disease has been regarded as secondary to the provision of services to diabetes and other branches of ‘endocrinology’.

To a certain extent, this reflects the perception that pituitary disease is rare, whereas diabetes is common, affecting approximately 5 per cent of the population. But a patient with pituitary disease is not interested in the prevalence of their disease; they are, however, keenly enthusiastic that their condition is managed with services and expertise of an equivalent level of excellence to those provided by other European states.

With the information overload of the internet easily accessible, our well-educated patient population are quickly aware when services fall short.

In this article I will describe some of the more common pituitary conditions, broach the topic of diseases newly-recognised to cause pituitary dysfunction and address the need for well-supported, specialised units to provide effective management of pituitary conditions.

Presentation of pituitary disease
Pituitary disease is one of the greater mimickers of modern medicine with a multitude of presentations, which can make early detection difficult. The range of symptoms associated with hypopituitarism is so varied that there are very few ‘red flag’ symptoms that are pathognomonic of pituitary disease.
Diagnosis is dependent upon a high level of awareness of pituitary disease and a knowledge of conditions associated with hormone dysfunction.

Children with pituitary disease are often picked up early; the combination of headaches and excess growth, for instance, is almost pathognomonic of gigantism due to growth hormone excess, while failure to grow or progress through puberty will usually prompt early specialist referral. In adults, however, the wide variety of symptoms and slow nature of disease progression dictate that pituitary patients present to neurologists, neurosurgeons, ENT, ophthalmology, gynaecology and psychiatry, as well as to endocrinologists.

The greatest part of our workload in the pituitary unit in Beaumont is pituitary tumours which have been referred for multidisciplinary work-up and consideration of neurosurgical intervention.  We have approximately 1,400 patients with pituitary tumours on our books and many of these patients have deficiencies of all pituitary hormones.  Accurate diagnosis of the deficiencies and careful monitoring of treatment is at the complex end of the spectrum of pituitary disturbances.

The consensus document of the Royal Colleges of Physicians and Surgeons in the UK stresses that pituitary disease should not only be managed in units with multidisciplinary aspects with input from endocrinology, neurosurgery, neuroradiology, radiation therapy, neuro-ophthalmology and neuropathology, but that follow-up should be lifelong in view of the disease’s complexity.

The requirement for the new:return ratios demanded by the HSE are difficult to balance with the quality care recommendations of our professional bodies. Data from our own unit support the view of the consensus document.  There are well established data which show that survival in acromegaly is related directly to plasma growth hormone (GH) measurements.

In 2004, retrospective analyses of 92 patients previously operated on in Beaumont showed that 3 per cent of patients followed up in our own multidisciplinary unit had GH levels in the range associated with excessive mortality; the figure for patients followed up in non-pituitary units was close to 50 per cent.  Since then, we have provided follow-up care for all patients who have had their operations performed in Beaumont, unless geographical or other extenuating circumstances dictate otherwise.

Conditions newly associated with pituitary disease
Traumatic brain injury.  Following the observation that our register contained patients with hypopituitarism, our unit developed a research interest in pituitary dysfunction following brain injury, in conjunction with the Department of Neurosurgery. Our data showed that the prevalence of pituitary dysfunction in survivors of brain injury was 30 per cent.  The research programme has developed — with collaborative links with the University of Montpellier and Beaumont — and has become a key opinion leader and world leader in this field.  The research has translated seamlessly into patient care and we have now successfully treated many patients with this newly-recognised association.

Cranial irradiation. Our unit, in association with the Departments of Neurosurgery and Radiotherapy, published the index paper on pituitary disease following cranial irradiation for non-pituitary tumours; up to 50 per cent of patients showed abnormal function. This has led to a surveillance programme in conjunction with the radiotherapists, which has unearthed many patients who require hormonal treatment, with consequent improvement in quality of life. Our hope is that the surveillance programme provides further data on the natural history of the development of hypopituitarism after irradiation.

Sultan Kosen holds the Guinness World Record for being the tallest man in the world. His growth has resulted from a tumour affecting his pituitary gland.

Subarachnoid haemorrhage. Our current research programme is devoted to the quantification of the incidence and predictors of hypopituitarism following subarachnoid haemorrhage and the hormonal determinants of post-subarachnoid haemorrhage hyponatraemia. Preliminary data reveal significant pituitary abnormalities and the translational aim of the research is the identification of clinical, radiological and biochemical features that predict hypopituitarism in this condition.

Haemochromatosis. Dr Howell Walsh, recently retired from the South Infirmary, Cork, was at the forefront of the international drive to quantify the prevalence of hypopituitarism in the important condition of hereditary haemochromatosis.  His work is another example of the dynamism of the pituitary research programme in Ireland and its ability to directly affect patient care and subsequent quality of life.

Organisation of pituitary services
Having worked in endocrinology in Ireland, Scotland, England and the USA, I have no hesitation in declaring that our doctors and nurses compare favourably with anywhere in the world. With the research dynamism that matches the quality of the consultant staff, we have the raw material to provide outstanding pituitary care. The greatest threat to our ability to do so lies in the absence of an infrastructure to deliver such care.

Current government policy is aimed at concentrating clinical care into centres of excellence; this is entirely in line with the recommendations of the UK consensus document, which is directly applicable to Irish practice.  However, although the multidisciplinary pituitary units in Cork University Hospital and Beaumont Hospital provide a de facto national service, neither are recognised units for this work. Seventy per cent of my patients come from outside of my hospital’s catchment area and 40 per cent are referred from other endocrine specialists.  The Cork unit — with which we work in close collaborative collegiality — would have a similar experience.  However, the lack of recognition of the de facto national nature of the work of the two units leaves them vulnerable to bed closures in the current economic climate.

Recent bed closures in Beaumont include the unit in which the pituitary investigation beds were sited. Over a year on, the unit has not been re-established, with significant detriment to staff morale and our ability to deliver our part of the national pituitary workload.  Loss of either the Cork or the Beaumont units would significantly compromise our ability as a nation to deliver the high-quality care for pituitary patients that our people deserve, and to which the HSE should aspire.

The Irish Endocrine Society has written on four occasions to the HSE to seek the establishment of a Clinical Care Programme for Endocrinology, similar to those in existence for other specialities, in order to plan a National Strategy for pituitary care and other endocrine issues, but at the time of writing this article, the Irish Endocrine Society has yet to receive the courtesy of a response.

The establishment of a National Pituitary Unit on two sites — Beaumont and Cork — is entirely in keeping with the Government and the HSE’s commitment to concentrating care in high-quality, specialised units and would be almost cost neutral in terms of provision of infrastructure.
The high quality professionals are raring to go. The intellectual dynamism is waiting to be unleashed. Is the political will and the funding?

• Professor Thompson is Professor of Endocrinology at Beaumont Hospital/RCSI and Secretary of the Irish Endocrine Society.  He is chairman of the European Hyponatraemia Network, a member of the international TBI/hypopituitarism working group and the European Endocrine Society delegate to the American Endocrine Society Hyponatraemia Guidelines Committee. He is also doctor to the Dublin senior Hurling Team.

From http://www.imt.ie/clinical/2011/10/putting-pituitary-disease-on-the-radar-in-irish-healthcare.html