Download this leaflet is available as a PDF file (1.35 MB)

What is Acromegaly?

Acromegaly is a disease of the Pituitary gland. Your pituitary gland is a small but very important gland at the base of your brain behind the bridge of your nose. Acromegaly is caused by a Benign tumour of the pituitary gland which causes the production of too much Growth hormone (GH). Pituitary tumours are almost invariably benign rather than malignant (cancerous) and therefore do not spread anywhere else.

What are the symptoms of acromegaly?

The symptoms of acromegaly can be very distinctive and unpleasant, but you can be assured that they can almost always be improved by treatment.

• Some of the first symptoms might involve tiredness and sleep disturbance.
• High levels of growth hormone can cause your hands and feet to enlarge, so that you may notice rings becoming tight and they may even need to be cut off. You may find that your shoe size has increased. T wo processes are at work here – firstly, the hands get bigger in the sense of increase in soft tissue (sausage fingers) but also the hands and feet actually undergo bone growth, making them physically larger).
• Your facial features may have changed, perhaps making your jaw more prominent, or spaces appear between your teeth, or your lips become enlarged.
• Perspiration can be a problem.
• Your skin may become oily and have enlarged pores.
• About a third of patients develop diabetes mellitus (sugar diabetes).
• Some patients suffer from a tingling feeling or pains in their hands, you may hear this referred to as Carpal Tunnel Syndrome. This symptom usually disappears once treatment has lowered your level of growth hormone.
• Enlargement of your pituitary gland may cause headaches.
• The enlarged gland may also press on the optic nerves (the nerves that connect your eyes to your brain) which pass just above it, and this may cause problems with your eyesight, especially with peripheral (side) vision
• In addition, the rest of the pituitary gland may become underactive. Because this gland controls the function of your ovaries or testes, as well as the thyroid and Adrenal glands, some symptoms may relate to these. The commonest problems here are impotence, infertility, and irregular periods. Your thyroid and adrenal glands are less likely to be affected.

All of these symptoms tend to develop gradually and the changes may not be noticed for some time either by you or by your family.

How is acromegaly diagnosed?

You will be referred to an Endocrinologist (a doctor who specialises in the Endocrine system) who will examine you, perform some blood tests and may give you a glucose tolerance test. This involves drinking a sugary liquid such as Lucozade, after which your blood will be measured for sugar and growth hormone over a period of about two hours. If you have acromegaly your growth hormone (GH) levels will remain high throughout the test and similarly a high blood sugar indicates you have diabetes mellitus. GH regulates the amount of the hormone IGF-1 (Insulin-like Growth Factor-1) in your blood. IGF-1 is responsible for many of the actions of GH, and measuring the levels of IGF-1 in your blood can be of value both in diagnosing acromegaly and in monitoring treatment.

It will usually be about a week before you get the results of this test. If the diagnosis is confirmed, you will have other tests to see if any other pituitary hormones are missing. If so, you will be started on replacement therapy for these hormones (see our leaflet entitled Hypopituitarism and Replacement Therapy for further details). In addition, you will have a special scan of your head to find the exact size and position of your Pituitary tumour. This scan will be either an MRI scan or alternatively a CT scan. You may be given an injection before the scan to improve the results. A minority of patients are allergic to this injection; so do tell the specialist if you have asthma or any allergies. The scan is not painful, but it does involve being in the scanner, which is noisy, for around half an hour. If you think this will make you claustrophobic or nervous, tell the doctor who will give you something to keep you calm.

How is acromegaly treated?

Acromegaly may be treated by surgery to the pituitary gland to remove or reduce the size of the tumour, by Radiotherapy, by drug treatment, or a combination of these. Nowadays for many patients the first treatment is surgery, although in some centres drug treatment to reduce the size of the tumour is given first. The aim of all treatments is to reduce growth hormone and IGF-1 levels to normal as this leads to the disappearance of many of your symptoms and the improvement of your general well-being.

Surgery

The operation is usually carried out by making a small cut in front of the upper teeth behind the upper lip, or through the nose. This is called Transsphenoidal surgery. By going behind the nose like this, the surgeon can see your pituitary gland without having to operate on the main part of your head. In some centres an endoscopic approach is used - fine tubes called endoscopes are pushed through the back of the nose, involving a small incision.

Most patients can be treated very successfully this way, although results are usually better if your tumour is small. The operation takes about an hour and a half. You will normally be in hospital for about five days. For the best results it is important to be referred to an experienced pituitary surgeon and your endocrinologist will be able to advise whom you should see. See our leaflet entitled Pituitary Surgery for further details about the operation.

Surgery will normally lower your growth hormone levels considerably, but in some instances the acromegaly is not cured. In such cases, consideration will be given to treating you further with radiotherapy and/or drug therapy.

Radiotherapy

Radiotherapy (treatment with radiation) might be needed if your surgery has not been completely successful (not all the tumour could be removed) or if it was not possible for you to have an operation. If you are given radiotherapy, it will be planned and carried out with extreme care. Using the images from your MRI or CT scans; the radiotherapy team will spend time planning exactly where the X-rays will go. You will attend the clinic on two or three occasions to have a special mask or fixation device made. This is a clear plastic mask or device which is used to hold your head still and ensure the radiation beams are correctly aligned. Treatment - very low doses of radiation - is usually given through three parts of the head - one on top of your scalp, and one area just beside each ear. The treatment itself usually comprises five-six weeks of daily treatment (Monday to Friday). It may take several months, or even years, after the treatment for the effects of radiotherapy to be complete. While you are waiting for this, you may be given drug treatment.

Improvements in pituitary scanning and computerised control of radiotherapy mean that new forms of finely focused, high dose radiotherapy (stereotactic) are being developed. This treatment is not suitable for all pituitary tumours and its benefit is being evaluated. Stereotactic radiotherapy allows much higher doses of radiation to be given to a part, or occasionally the whole, of the tumour often in a single session. The larger dose of radiation means that your GH levels come under control much more quickly, maybe months rather than years. The Gamma Knife Ò, the best-known form of stereotactic radiotherapy, is currently not widely available. For more information about radiotherapy, see our leaflet entitled Pituitary Radiotherapy.

Drug Treatment

There are three types of drugs for treating acromegaly - each acts by different mechanisms:

• Somatostatin analogues work on specialist markers (somatostatin receptors) to inhibit GH release from the tumour.
• Dopamine agonists work on alternative markers (dopamine receptors) on the surface of the tumour to inhibit GH release from the tumour by a different mechanism.

A new drug, pegvisomant, works differently and blocks the action of GH.

There are two somatostatin analogues available for the treatment of acromegaly:

• Octreotide (brand name Sandostatin, manufactured by Novartis) and
• Lanreotide (brand name Somatuline, manufactured by Ipsen).

Somatostatin analogues require injections. Octreotide can be given once a month deep into muscle (intramuscularly) for the long-acting preparation (Sandostatin LAR, Novartis), fortnightly or, occasionally, three times a day under the skin (subcutaneously). The short-acting preparation of octreotide is provided as ampoules or vials, which can be used for several doses. It is normally kept in the fridge, but must be removed and come naturally to room temperature before use. The long-acting preparation is usually given by a nurse at your GP surgery.

Lanreotide (Somatuline LA, Ipsen) is available both as an injection deep into muscle (intramuscular) which needs to be given every seven to 14 days or in a newer formulation which is given once a month under the skin.

Octreotide and lanreotide help the symptoms of acromegaly by reducing growth hormone levels into the target range of less than 5 mU/l in more than half of patients. When you first start taking somatostatin analogues, it may give you stomach colic or diarrhoea, but these effects usually wear off within a few days. Some patients find that stomach problems improve if they do not inject for about two hours after eating. In the longer term octreotide may cause gallstones, but these very seldom cause problems.

In general the effectiveness and side-effects of long-acting somatostatin preparations are similar to the three times daily octreotide but you may prefer the convenience of only having to have an injection once a fortnight or once a month. The injection into muscle needs to be given by a nurse.

There are several dopamine agonists available, all are taken by mouth but only two are widely used:

• cabergoline (brand name Dostinex, manufactured by Pfizer)
• Bromocriptine (brand name Parlodel, manufactured by Novartis)

Cabergoline, a newer long-acting dopamine agonist that causes fewer side-effects than bromocriptine, appears to be more effective in lowering GH and IGF-I levels, and needs to be taken only twice weekly. It is not licensed for the treatment of acromegaly but is an accepted and widely used treatment.

Bromocriptine usually needs to be taken two or three times a day.

Unfortunately, although dopamine agonists are taken by mouth, they are often less effective than somatostatin analogues which have to be injected. They may cause you to be constipated, although this can be alleviated by increasing the fibre in your diet. At the beginning of treatment, you may also suffer nausea or dizziness when you first stand up. These effects also tend to wear off with time.

Pegvisomant (Somavert, Pfizer) is a completely new way of treating acromegaly. All current forms of treatment attempt to lower the amount of GH released by the pituitary gland. Pegvisomant is a blocker of the action of GH. It does not try to inhibit the release of GH from the pituitary into the blood but instead stops the GH leaving the blood to stick to cells throughout the body. This should block all the unwanted effects of GH and studies in patients with acromegaly suggest it is very effective. It is given as a daily subcutaneous (under the skin) injection. It is currently used for patients in whom the more traditional treatments have not been successful.

Some patients experience difficulty in obtaining funding for these drugs. Advice can be obtained at The Pituitary Foundation.

What effects will I see from this treatment?

Once treatment is successful, you will notice that the soft tissues of your hands and feet decrease in size and patients often notice that their facial features gradually return towards normal. This may take some time, but do not despair - improvements will happen once the treatment is successful. If you were suffering from excess sweating this should decrease and if you had sugar diabetes, it should improve or disappear altogether. Your headaches will usually improve and so will any visual disturbance that was present beforehand. If you snored, it should be cured. Any increase in bones will revert very slowly with the strong possibility that reversal may be incomplete.

How is my progress monitored?

All acromegalic patients should be looked after by a specialist in this condition (an endocrinologist). Ideally your endocrinologist will have access to specialist nurses and radiology plus access to a neurosurgeon specialising in pituitary disease.

The success of the treatment of your acromegaly needs to be monitored. The aim of treatment is to lower your average GH level to less than 5 mU/l and have your IGF-1 level in the normal range for your age.

Your endocrinologist will ask you how your symptoms have changed, in particular whether there has been any change in your ring size and facial appearance. It is also important for you to have regular blood tests to check your GH and IGF-1 levels, as well as the function of the rest of the pituitary gland. GH & IGF-1 can be measured either during a glucose tolerance test (see above) or by means of tests known as a ‘day curve’ when blood samples are taken several times during a single day. In order to determine its effect, these blood tests will need repeating when there has been any change in your treatment.

Aftercare

Your condition will require long-term monitoring and this will be shared by your endocrinologist and GP. Because pituitary conditions are relatively rare, you might find that you will be the only patient with acromegaly your GP is treating and (s)he might find it helpful to have a copy of our Pituitary Disease Factfile for General Practitioners.

Longer-term implications include an increased risk of osteoarthritis and heart problems in the elderly. The risk of colon cancer may be slightly raised and people should have a colonoscopy around the age of 50. These risks all depend on when diagnosis was made and how treatable the acromegaly.

Very occasionally people develop severe problems caused by growth of the jawbone. They may be eligible for chin reduction surgery or free dental treatment.

Loss of Libido, Infertility and Relationships

You may suffer from a low sex drive, impotence or lack of self-esteem due to the imbalance of hormones and, in some cases, physical changes. This, in turn, may cause a strain on your relationship and your partner may feel neglected or hurt. There is also a possibility that you may have problems conceiving. It may help to talk to your partner about how you are both feeling and to consult your GP/endocrinologist. You may also wish to request a copy of our leaflet Psychological Issues in Pituitary Disease.

Other Factors to Consider

Returning to Work

The nurses will sort out a certificate to cover your stay in hospital and you will be advised how long you will be expected to remain off work. This usually depends on what your job is and whether you are self-employed or not.

Prescriptions

If you will have to take Hydrocortisone, Thyroxine or Desmopressin permanently you will get free prescriptions for all medicines. Ask at your GP’s surgery, hospital or pharmacist for form FP92 (EC92A in Scotland). The form (which will need to be signed by your doctor) tells you what to do. You will then receive an exemption certificate. These certificates only last for a finite period after which they must be renewed. Your health authority may automatically send out an application for renewal.

Information about free prescriptions and the full list of medical conditions which qualify for exemption from prescription charges can be found in leaflet HC11, available from pharmacies and main Post Offices or on www.dh.gov.uk. If you are not sure whether you are entitled to free prescriptions, you must pay for your prescription and ask for a NHS receipt (form FP57 in England, EC57 in Scotland) when you pay; you can’t get one at a later date. This form tells you how to get your money back. You must claim within three months.

If you don’t qualify for free prescriptions and need more than five prescription items in four months, or more than 14 in a year, ask your pharmacist about pre-payment certificates. This will be more economical way of paying for a large number of prescription items.

Driving

You have a legal obligation to advise the Driver and Vehicle Licensing Agency (DVLA) if there is any reason why you should not drive. Many patients with pituitary conditions will find there are no restrictions on their driving, but you should check with your GP. The only condition likely to affect you is problems with your eyesight. Transsphenoidal surgery does not in itself limit your entitlement to drive. Your doctor or specialist will give you full advice. You may also seek extra advice from the DVLA by contacting the Medical Adviser, The Drivers’ Medical Branch, 2 Sandringham Park, Swansea Vale, Llansamlet, Swansea SA6 8QD. 0870 0600 0301. There is a 24 hours answering machine.

However, it is not a good idea to rush back to driving after any major operation, regardless of whether it is legal or not. Try to leave driving for a week after returning home and only drive for short journeys to start with.

Alcohol and Replacement Hormones

There is no interaction between alcohol and these drugs, and you are allowed to drink in moderation. You should restrict yourself to one to two units of alcohol a day.

Insurance and Pensions

Each case will need to be assessed individually. As a guide, if a pituitary tumour has been completely removed, you will probably be accepted at normal rates. Of course, each insurance company will have its own practices. You need to persevere and be specific about your condition, as the people you speak to initially may not have any medical knowledge themselves. It is not unheard of, for instance, for Diabetes Insipidus to be confused with diabetes mellitus (sugar diabetes). If you would like more information, please email us on helpline@pituitary.org.uk or telephone 0845 450 0375.

Employment Issues

If your pituitary condition is causing you difficulties in retaining, seeking, or returning to employment, contact the HelpLine or your local Citizens Advice Bureau for the most up to date information about employment rights and where to get advice about benefits.

Personal Medical Identification

If you are taking hormone replacement medication, it is a good idea to wear a medical information bracelet or equivalent, as the information will help the doctors if you have an accident and are unconscious.

Members have tried:

• MedicAlert® The MedicAlert Foundation, 1 Bridgewharf, 156 Caledonian Road, London N1 9UU. Tel: 0800 581420. Website: www.medicalert.org.uk
• MediTag, 37 Northampton Street, Hockley, Birmingham B18 6DU. Tel: 0121 200 1616. Website: www.medi-tag.co.uk .
• Doctag, 31-33 West Pilton Drive, Edinburgh, EH4 4HS. Email: info@doctag.net. Website: www.doctag.net.

Useful Addresses

The Pituitary Foundation has a listing of options for clothing taller people and shoes in bigger sizes. Please contact the Foundation for details or go to www.pituitary.org.uk.