Acromegaly Support Group New Zealand Newsletter No 1 November 10

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1 Acromegaly Support Group New Zealand Newsletter No 1 November 10 Welcome to our first issue! Do you or a family member have acromegaly? Do you feel isolated? Do you feel no one understands? Do you want to meet other patients & families? Do you want to learn more about your pituitary condition? If you answer YES to any of the above, please fill in our membership form and return it back to us today. Website: pituitarynz@gmail.com Mobile: Phone: Fax: Address: Acromegaly Support Group NZ Endocrinology Department Greenlane Clinical Centre Private Bag Auckland Mail Centre Auckland 1142 Acromegaly is a rare condition, according to our endocrinologists, there are about 50 patients in the Auckland area. As an acromegaly patient myself, I have felt isolated and lonely at times, especially when I was newly diagnosed and undergoing treatment. There are many patient support groups overseas, including the US, UK and Australia. To my knowledge this is the first patient support group for pituitary patients in New Zealand. I am hoping to get enough members to introduce patient/family gatherings early next year, such as informal gatherings over coffee where we can meet others, and share our experiences and concerns. The next step after will be organising educational seminars involving relevant health professionals. My vision for us is to grow as a group, maybe expanding to include other patients with pituitary disorders. There is a very well organised group in Australia called The Australian Pituitary Foundation, I hope in the future we can offer similar support in New Zealand. You are receiving this because your endocrinologist has indicated that this maybe of interest to you. Simply fill in the membership form attached, or join online at Look forward to hearing from you, Dr Catherine Chan Founder Acromegaly support group NZ Can you help? I cannot keep a support group running without your support! Your ideas, skills and time will be invaluable. Things you maybe able to help with: Stories would you like to share your story & experiences with the group? We aim to publish a patient s story in each newsletter. Questions if you have any questions regarding your illness, post them in, I will get them answered! News if you have access to news articles/journals, please help contribute to the next newsletter by writing a brief news update. Organising do you want to join the committee, and play a part in organizing future newsletters, patient gatherings, and educational seminars? Computer skills If you can help with our website or newsletter, I want to hear from you. Photos/drawings anything graphical would make reading much more interesting. E.g. would you like to design a logo for the group? Money do you know of someone who maybe able to sponsor us? Anything else you can think of? Just give me a buzz, the more ideas the better! 1

2 Medical Column What is the Pituitary Gland? The pituitary is a small, pea-sized gland located at the base of the brain, this is behind the bridge of the nose and below the base of the brain, close to the optic nerves. The pituitary gland is an important gland and it is often referred to as the 'master gland', because it controls several of the other hormone glands. It consists of two parts (often called lobes) - a front part, called the anterior pituitary and a back part, called the posterior pituitary. The anterior pituitary makes several important hormones Growth hormone, puberty hormones (or Gonadotrophins) that stimulate the ovaries & testes, Thyroid stimulating hormones (TSH, which stimulates the Thyroid Gland to make Thyroxine), Prolactin and Adrenocortitrophic hormone (ACTH, which stimulates the adrenal stress hormone, Cortisol). The posterior pituitary makes the fluid balance hormone called Anti-diuretic Hormone (ADH). A pituitary tumour (or adenoma) is a benign growth on the pituitary gland. The tumour maybe non-functioning (does not affect hormone production), secretes excessive hormones, or the tumour can push on the pituitary gland leading to reduced hormone production (hypopituitarism). The Pituitary Foundation. There are several different forms of pituitary tumours depending on how it affects hormones in our body. In this article we are going to talk more about Acromegaly. What is Acromegaly? Acromegaly is a condition caused when a pituitary tumour produces too much Growth Hormone (GH). This is a rare condition with a prevalence of ~50 cases per a million people. With an average of 3-4 patients being diagnosed per year per a million people. Acromegaly usually develops between the ages of 30 and 50. If the condition develops before a person has stopped growing (before age 15-17yrs), it causes Gigantism, when a person grows far taller as GH causes continued bone growth. It is believed there is generally an 8 yr delay between symptom onset to diagnosis of Acromegaly, given the insidious onset of symptoms and its rarity making diagnosis difficult. Acromegaly means large extremities in Greek, as swelling of soft tissues commonly lead to swelling in hands & feet, with a subsequent increase in ring & shoe size over a number of years for many patients. Specific symptoms of acromegaly include: Enlarged hands and feet - often requiring larger rings or shoe sizes A change in the face, jaw or tongue: Cheekbones or forehead are more prominent Bite has changed (inability to bite) or teeth have become more widely spaced Lips and tongue have enlarged causing snoring or dribbling at night Symptoms that suggest acromegaly: Excessive sweating Heavy snoring or daytime sleepiness 2

3 Tingling/numbness in both hands (carpal tunnel syndrome) Skin becoming more oily, thicker and hairier Other conditions associated with acromegaly: Arthritis Diabetes High blood pressure How is Acromegaly diagnosed? The diagnosis is through a series of blood tests to check the hormone levels including GH (growth hormone), and IGF-1 (insulin-like growth factor 1) a hormone produced by GH stimulation, and is the main mediator of the effects of GH. A special test maybe done by drinking a bottle of sugary liquid and measuring GH changes for 2 hours after. Other blood tests to check other hormones produced by the pituitary gland will also be done. And imaging of the pituitary gland via a CT or MRI scan. Treatments for Acromegaly by Professor IM Holdaway These are exciting times for those with acromegaly, with many developments in the field of acromegaly treatment. Many, although not all, of these treatments are available in New Zealand. Pituitary surgery: Surgical removal of growth hormone secreting pituitary adenomas causing acromegaly remains the most important form of treatment. Studies have shown that it is essential that the operation is done by an experienced neurosurgeon doing an appreciable number of similar operations each year. We are lucky in New Zealand that each major centre has at least one neurosurgeon with appropriate experience. There have been major technical advances such as the use of endoscopy at surgery, which has improved surgical outcomes. The surgical cure rate for those with small adenomas (microadenomas) can be as high as 80-90%. Larger adenomas (macroadenomas) can extend into regions outside the pituitary cavity and may be more difficult to completely excise at operation, although cure can still be achieved in 40-50% of cases. Figures for surgical cure by the principal pituitary surgeon in Auckland, Mr Andrew Law, are at least as good as these international results. For those not cured by surgery, or in the rare situation where surgery is for some reason contraindicated, circulating levels of growth hormone and the growth factor produced by growth hormone, IGF-I, can usually be controlled by other treatments. These include: 1. Octreotide, an analogue of the natural hormone somatostatin, inhibits secretion of growth hormone by pituitary adenomas. It is not active taken by mouth, so is usually given as a depot injection each month (LAR Octreotide). 2. Cabergoline, a medication similar to the natural hormone dopamine, can act to suppress growth hormone production in a proportion of patients. It can be taken orally. 3. Pituitary radiotherapy, given either as external beam radiotherapy using standard radiotherapy machines, or as highly focussed stereotactic radiotherapy, is an effective method to control growth hormone over-production and reduce the size and activity of any adenoma remnants. However, the effect of such radiotherapy is gradual, so ultimate cure of the condition by radiotherapy is achieved only slowly. 4. Overseas units can treat pituitary adenomas with gamma knife radiotherapy using a focussed beam of gammarays from a cobalt 60 source. This has a rapid curative effect on the pituitary adenoma. The equipment for this type of therapy is not available in New Zealand. 5. Pegvisomant, an inhibitor of growth hormone binding to target cells in the liver, can lower serum levels of IGF-I, and is another effective method to reverse the symptoms of acromegaly. It is given by self-administered daily injection. At present the agent is not funded by Pharmac in New Zealand, and is very expensive for self-purchase. Individuals with acromegaly are thus now in the fortunate position of having a range of therapies for their condition, and research on further methods of treatment is under way around the world. Did you know? "ONE IN FIVE individuals may have an abnormal growth on their pituitary gland, causing significant health complications that, if left undiagnosed and untreated, can impair normal hormone function and result in a reduced lifespan." Shereen Ezzat, M.D., Professor of Medicine, University of Toronto 3

4 Patient Story Catherine In 2003, when I was 20 years old, I went to my GP after my periods stopped for 6 months. My GP initially thought I had PCOS (polycystic ovarian syndrome), given I had very oily skin with acne, increasing hair growth on my arms & upper lip. She did some blood tests, which weren t consistent with her diagnosis of PCOS, and I am eternally grateful for what she did next, she referred me to an endocrinologist. And there began my journey of tests, surgeries, radiotherapy, and self-discovery. I was promptly seen at endocrine clinic, followed by more blood tests, and eventually a MRI scan of my pituitary. Suddenly I found out I had a macroadenoma in my pituitary. As a young & naïve medical student at that stage, I realised what that meant to a certain extent. All of a sudden my life halted. Being a young & fit person previously, I believed I was bulletproof, being ill was the last thing I expected. This was something I was not prepared for. Looking back I had been experiencing subtle changes over the preceding 3-4 years. Like my nose getting bigger, my hands and fingers, my shoe size went from 6.5 to 8, and a few people commented that I was taller. Other changes I also noticed were tiredness, lack of concentration, not coping with work. I was feeling cold all the time, wearing my polar fleece when my flatmates were in singlets. All of these symptoms were subtle and gradual, and I always put them down to another cause. I do not waste time doing things that are not meaningful. Life is too short for that During that period of hardship I really found out who my true friends were, those who stuck by me and send me get well wishes even when I was too unwell to reply! I am now symptom free, and well controlled on a cocktail of pituitary replacement hormones including oestrogen, thyroxine, and desmopressin nasal drops for diabetes insipidus. I finished my medical training at Auckland medical school in 2007, and I thank the support of all the staff at the medical school that supported me through treatment, fitting in training amongst all my appointments at hospitals. My illness experience has changed my life, my goals, and my attitudes. I see myself as a much more mature person, and I do not waste time doing things that are not meaningful. Life is too short for that. The main problems I still have to conquer include my energy levels, poor concentration, back pain/stiffness, for which I do stretching & yoga with regular walking. I still need an afternoon nap more often than not, and get yawning attacks. My weight - I have gained 30 kgs in the last 5 years which is a very scary thing. Also I get mood swings, going from excitement, happiness, to feeling depressed, to feeling angry very quickly. I try to manage by having a regular routine, a regular wake-up & sleep time everyday, regular exercise, and having plenty of metime to reflect on my day. My social life has definitely suffered as a result, but my health is my priority. I went back to my endocrinologist a week later with a collection of the above symptoms, and my suspicion that I had acromegaly & hypothyroidism. I don t know what went through his mind, but his fingers were twice the size of mine even though I claimed mine had grown! Nevertheless, further blood tests confirmed I indeed did have high GH/IGF-1 consistent with the diagnosis of acromegaly. Since then, I have undergone 2x transphenoidal surgeries. Followed by monthly injections with sandostatin LAR. Despite the above my IGF-1 went up again, therefore eventually I underwent a 6-weeks course of Stereotactic radiotherapy at Dunedin Hospital. Those times were very stressful, for my family & myself. Especially when my vision deteriorated to 6/36 (I could see at 6 meters what a normal person could see at 36 meters). The support from the endocrinology team was fantastic. Day before surgery A few mths later The last few years working had been a real struggle for me, having had to work 65 hour/week rotating shiftwork just did not agree with my body. I recognised I need to look after myself as my health was deteriorating. I am very happy with my current job, which I landed in July this year. This allows me to work part-time with regular hours. Having a lot more free time now than I did before, I have seized this opportunity to tick off a task which has been on my To-Do list for many years, that is, to set up an Acromegaly patient support group in New Zealand! I would love to have the opportunity to meet you all. If you would like to comment on the above, or would like to share your story with others, please write to me at pituitarynz@gmail.com Upcoming Issues: Future newsletter topics will include: - Update on surgical interventions - Radiotherapy - Medications updates - Joint problems - Eye problems - Diabetes in acromegalics - Sleep apnoea - Psychological effects If you have any ideas or would like to contribute, let us know! 4

5 Membership Form By joining us you will receive regular newsletters, news, invites to patient gatherings and educational seminars. You & your family will benefit by helping us to form a communication network with other patients with this disorder. Name: Title: (Mr/Mrs/Ms/Miss/Dr/Prof) Age: Gender: Postal Address: City: Postcode: Phone: (Home/work) Mobile: Do you prefer to receive future correspondence by Post Are you a (please tick) Patient Family Member/Friend/Carer? Health Professional Other: What pituitary condition(s) do you have? When were you originally diagnosed? (e.g. Acromegaly, diabetes insipidus, hypopituitarism) Treatment: (Specify type/date/hospital/doctor if you wish) Surgery: Radiotherapy: Medications: Others: Patient Contact Register this enables members to be able to contact fellow members with their permission. If you would like to be part of this Register, please tick your preference below. Please include my name and contact details on the Register: my phone my Both Depending on response, I am aiming to organize patient gatherings for patients & families to meet & network. Please indicate if you are interested in attending: social informal gatherings e.g. Coffee seminars/educational sessions other I am happy in helping Acromegaly NZ with: Please note all information on this form is voluntary and will not be divulged without your permission. Signature: Date: You can also fill in this form online at Or fax this back to us Fax: Address: Acromegaly Support Group NZ Endocrinology Department Greenlane Clinical Centre Private Bag Auckland Mail Centre Auckland 1142 Look forward to Receiving regular newsletters (we aim to publish 4 newsletters annually) Invites to informal patient gatherings to meet & network Invites to future educational seminars If you have any questions or contributions Website: pituitarynz@gmail.com Mobile: Phone: Fax: