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Managing acromegaly in the community: the PN's role

Jean Berrigan
BSc(Hons) RGN
Practice Nurse
Oakfield Surgery
Newmarket
E:jeanne_berrigan@hotmail.com

Acromegaly is caused by the secretion of excess growth hormone (GH) from a pituitary tumour, usually a macroadenoma, although there are, rarely, other causes.(1)

Acromegaly is prevalent in both men and women between the ages of 30 and 50 years old. Because onset is insidious, diagnosis may be undetected for several years. In the UK, 4-6 cases per million population are diagnosed each year.(2)

Without treatment, people with acromegaly are at risk of developing a number of long-term health complications (see Table 1), leading to a two- to threefold increase in mortality.(5) The practice nurse plays a pivotal role in the care of the patient with acromegaly, and integrative nursing pathways are key to good practice.

[[NIP10_table1_14]]

Signs and symptoms
GH excess leads to a chronic debilitating disease associated with bony and soft tissue overgrowth.(1) The main symptoms of acromegaly are:

  • Enlargement of hands and feet and changes of facial features (see Figures 1 and 2).
  • Excessive sweating.
  • Snoring.
  • Sleep apnoea.
  • Headaches.(4)

[[NIP10_fig1_14]]

[[NIP10_fig2_14]]

The main clinical features are: hypertension; dental problems (eg, malocclusion and wide spacing of the teeth); depression or feeling "low"; general tiredness; loss of libido; aches and pains in the joints; tongue enlargement; carpal tunnel syndrome; and visual disturbances due to tumour growth.(4)

Other findings, as suggested by Bilner et al,(1) are increased left ventricular mass or left ventricular thickness. Amenorrhoea may occur with or without hyperprolactinaemia. The patient may have an enlarged kidney or liver without impaired function. There may also be coexisting diabetes.

The patient may begin to suspect that there is a problem when comparing photographs over the years.

Diagnosis
Making a diagnosis without overt hypertrophic osseous changes is not always straightforward. Patients may have been polysymptomatic for years. Symptoms such as paraesthesia, headaches, muscle weakness, sweating, arthralgias, fatigue, weight gain, kidney stones and arthritic complaints make precision diagnosis difficult.

Once a diagnosis of acromegaly is suspected, the patient should be referred to an endocrinologist. After initial consultation a number of tests are performed. One of the most definitive is the glucose tolerance test. Ingested glucose normally suppresses GH concentrations in healthy people. In acromegaly this does not occur.

Other tests include measurement of prolactin levels and insulin-like growth factor (IGF-1) concentrations and pituitary function tests.

On diagnosis, the patient will undergo a magnetic resonance imaging (MRI) scan to identify the location and size of the tumour. These tests are undertaken by a specialist endocrine nurse and managed in Outpatients.

Treatment
Five clear aims have been set by the Society for Endocrinology (2000) for acromegaly treatment: reverse the signs and symptoms of acromegaly; restore GH and IGF-1 to safe levels; remove the pituitary tumour while preserving ­pituitary function; prevent recurrence; and restore normal life expectancy.(6)

Surgery
Depending on the size and location of the tumour, trans-sphenoidal surgery can provide one of the most rapid responses in reducing GH to safe levels. Surgical resection of tumours 1cm diameter is 60% effective.(7)

If GH levels postsurgery are persistently raised, the endocrinologist and specialist endocrine nurse should discuss further treatment options, such as radiotherapy and medical treatment, with the patient.

Radiotherapy
Radiotherapy can be successful in treating acromegaly if a patient is considered unsuitable for surgery. However, its curative effect is slow, taking up to 10 years.(3)

Medical treatment
Medical treatment is given either as an adjunct to surgery and radiotherapy or as a primary treatment method.

Dopamine agonists
Oral dopamine agonists (eg, bromocriptine), which are known to increase GH levels in normal individuals, paradoxically decrease levels of GH in acromegalics.(8) At high doses they can cause unpleasant side-effects.(4) They are best used as an adjunct to other therapy.

Somatostatin analogues
Octreotide and lanreotide are analogues of somatostatin, a peptide inhibitor that regulates the release of GH and IGF-1, and they are used frequently in the long-term management of acromegaly.

Octreotide has a short half-life and can be self-administered by the patient. It is given three times a day subcutaneously. This is more suited to the patient who needs to be independent of the surgery and is able to self-inject. Although effective in reducing GH secretion, the potential for poor patient compliance exists. In addition, there is the issue of safety of sharps disposal in the patient's own home - it is important for the practice nurse to ensure regular distribution, collection and disposal of sharps containers.

Alternatively, in the surgery, a long-acting micro­particle octreotide (Sandostatin LAR; Novartis) is available in doses of 10mg, 20mg and 30mg. It can be given by the practice nurse. It requires reconstitution and gentle mixing. Sedimentation is sometimes a problem, causing needle blockage. It is administered by deep intramuscular injection every 28 days.

Somatuline Autogel (Ipsen UK) is a new aqueous formulation of lanreotide available in doses of 60mg, 90mg and 120mg in prefilled syringes. It is administered by deep subcutaneous injection every 28 days. Dosage is adjusted according to response of levels of GH or IGF-1. The advantage of this preparation is that it eliminates sedimentation and needle blockage. Injection technique is specific. A deep subcutaneous injection is given into the upper outer quadrant of the buttocks. The skin is stretched and the injection given at 90(°).

Postsurgery and/or radiotherapy, a hormonal assay and dynamic pituitary function test will determine the need for adjuvant oral or intramuscular therapy.

Community management - the practice nurse's role
Integrative nursing is the focus of acromegaly management through shared care. This not only ensures continuity of care, it enhances it. It is important that effective communication between the endocrinologist, specialist nurses, GP and practice nurse take place. For best practice, a meeting to discuss the patient's history, treatment requirements and management plan should occur before the patient's discharge to the community.

Following training from the endocrine nurse, the practice nurse can be responsible for the following:

  • Administration of a long-acting somatostatin ­analogue on a monthly basis (if appropriate).
  • Observation, troubleshooting and reporting of side-effects to the GP and endocrine nurse.
  • Patient compliance with long-term ­management strategies and shared-care ­recommendations.
  • Providing ongoing advice and information to the patient as required.
  • Monitoring the transmission and reception of ­information between primary and secondary care.

Key considerations
Because of the rarity of the condition, it is important to have access to up-to-date information. It is also essential to implement a written, shared-care agreement to ensure continuity of care in the community.

It is paramount to good practice that the practice nurse has the facility to access "specialty expertise" for help with problem-solving, basic queries or expanding their theoretical knowledge of the disease. Ease of contact between the medical and surgical endocrinology team enhances and supports patient care in the community.

Treatment is long-term. Endocrinologists usually review patients every 6-12 months. The practice nurse can stress the importance to patients of attending these appointments.

In the surgery, it is important to share information with other practice staff. In the event of absence through holidays or sickness, other staff should be confident in maintaining patient care.

Patient support
The first stage for the patient is learning to fit the injections into their lifestyle. Forward planning between patient and nurse promotes confidence and compliance with treatment. Drug costs and the rarity of acromegaly mean that somatostatin analogues are not stock drugs, so forward planning is vital to ensure that the treatment is never interrupted due to failure of obtaining the ­analogue from the pharmaceutical suppliers.

By providing the patient with information about the possible side-effects they may experience, such as transitory pain, redness and swelling at the injection site, the practice nurse can alleviate some of their anxiety.

If the patient misses an injection appointment, it is important that they make a new one to ensure the injection is administered as soon as possible.

Other issues, such as negative body image and physical wellbeing (eg, loss of libido), need careful management. Reduction of symptoms such as headaches and joint pain is encouraging for the patient. Because of the intensity of their medical care, they may feel they have no right to voice what they perceive as a "grumble".

Being in partnership with the patient is constructive. It is equally important that the patient is fully educated on all aspects of their care. Given the complexity of their condition and their long-term medical needs, the patient also needs to be fully informed at all stages, both pre- and postoperatively.

Further guidance
A panel of endocrine and practice nurses have developed the document Managing acromegaly in the community: a shared care approach. This outlines a best practice approach to managing acromegaly based on evidence and clinical experience. It provides guidance on the shared-care approach to ensure continuity of care and improve patient outcome. The document has been produced with the assistance of an educational grant from Ipsen Ltd and is available by calling Harvard Health on 01932 350006.

References

  1. Bilner Beverley MK, Daniels GH. In: Harrison T, editor. Harrison's Principles of ­internal medicine. 14th ed. New York: McGraw-Hill; 1998.
  2. Alexander L, et al. Epidemiology of acromegaly in the Newcastle region. Clin Endocrinol 1980;12:71-9.
  3. Guistina A, et al. The ­pharma-cological aspects of the treatment of acromegaly. Pharmacol Res 1996;34(516): 247-68.
  4. Weekes LM, et al. Treatment options in acromegaly. Pharmaco-economics 1996;10(5):453-9.
  5. Bates AS, et al. An audit of outcome of ­treatment in acromegaly. Q J Med 1993;86:293-9.
  6. Society for Endocrinology. The use of somatostatin analogues (Position Statement). Bristol: Society for Endocrinology; 2000.
  7. Clayton RN, et al. Pituitary surgery for acromegaly. BMJ 1999;319;588-9.
  8. Davies IJT. Postgraduate medicine. 5th ed. London: Chapman and Hall; 1991.

Resources
The Society for Endocrinology holds an annual study day in which nurses are encouraged to learn about updates in the treatment and management of acromegaly
W:www.endocrinology.org

The Pituitary Foundation
PO Box 1944
Bristol BS99 2UB
T:0870 7743355
W:www.pituitary.org.uk