Home

Personal experience of uveal melanoma

Dr Lesley Kirkpatrick, Doncaster, England, UK

picture0

Delivering my lecture (my first ever)

Introduction

I am grateful to Professor Damato and Dr Coupland for inviting me to write this article, which is based on a speech that I delivered at a meeting of the European Ophthalmic Oncology Group in Liverpool in March 2010.

 

picture10

The audience of around 100 delegates from all over Europe

 

I have worked in General Practice in the UK for 22 years and have seen 4 patients with metastatic choroidal melanoma – including me. This puts me in the rare position of looking at my disease from both sides of the doctor’s desk.

 

The aim of this article is to share my experience with patients and health providers so that the care of future patients might be improved.

Case History

Ophthalmic History

 

In 1983, I suffered from retinitis in my right eye, and since then my left eye has been my best eye.

 

In 1999, I found it difficult to read road traffic signs and went to Casualty. No abnormality was found and I saw an ophthalmologist. My symptoms resolved whilst waiting to be seen. My pupils were not dilated but perimetry was done. I was unaware that it showed a slight abnormality in the left eye.

 

 

 

Picture1

Left visual field in 1999 showing a subtle defect near the top edge

 

I was strongly reassured that my symptoms could be attributed to being female, aged 41 and with a stressful job. This embarrassed me.  I accepted the reassurance that my symptoms were of no consequence. They recurred once or twice a year but I ignored them I didn’t want to make a fuss.

 

In September 2006, my symptoms got worse and I started seeing pin pricks of blue iridescent lights in the morning. I went to an optometrist but no lesion was found. Again my pupils were not dilated. I requested perimetry, which showed a defect, and I was referred to an ophthalmologist.

 

 

Picture2

Left visual field in 2006 showing worsening of defect

 

 

I was found to have a small choroidal melanoma in the left eye. I saw a local ocular oncologist who offered plaque radiotherapy.  Due to logistical problems there was a long waiting list andI was referred to Liverpool. I was told that my tumour had a basal diameter of 8mm and a thickness of 2 mm and I was offered proton beam radiotherapy with TTT for exudation.

 

 

Picture3

My choroidal melanoma, close to the optic nerve and fovea

 

I was told I had a 30-40% chance of maculopathy, a 10% chance of optic neuropathy and a one-in-four chance of metastatic death in ten years. For any patient the moment of being told you have cancer is mind blowing. I was very fortunate to receive a recording of that conversation. It is invaluable to me.

 

In January 2007, I underwent Proton Beam radiotherapy followed by Trans Pupillary Thermo Therapy. I wanted a prognostic tumour biopsy but was told that my tumour was too small.

 

 

Picture4

My proton beam plan, showing the tumour (in red) and the beam

 

 

In November 2008, I suddenly found it difficult to drive and use a computer. I realised I had macular oedema.

 

 

Picture6

An OCT scan showing macular oedema

 

 

Fortunately, my recording of my first consultation with Professor Damato had mentioned Avastin. I contacted Liverpool immediately and had intra-ocular Avastin within two weeks. I have repeated the Avastin whenever I feel my vision deteriorate. I have had 15 extra months of normal vision. Two months ago, my vision was 6/5 although recently it deteriorated to 6/9. Just after my talk, I had another Avastin injection, which improved my vision to 6/5 again.

 

 

Picture5

My tumour after proton beam radiotherapy and TTT

 

 

Systemic history

At my first review, I learned that my tumour had shrunk to 0.8mm. I concluded it must be highly malignant. When I brought the topic up I found it isolating that my clinicians did not feel able to discuss this with me. I have have seen this problem with my own patients, when family members of one generation believed all they were told by their specialist and when those of a younger generation disagreed, possibly after researching the subject on the internet. After the patient dies, this can also cause distress to bereaved relatives, who may feel guilty if they feel their loved one might have lived longer if only they had challenged their doctor.

 

I accepted there were few options when metastatic disease developed, but liver resection looked beneficial to some patients. Early diagnosis seemed the key to success, but I found it impossible to find a doctor in the NHS who would perform liver imaging with high quality MRI with contrast and diffusion weighting. Incredibly, one oncologist even told me that MRI was too dangerous because of excessive exposure to ionising radiation. There seems to be a lack of understanding in the UK as to the relative sensitivities of various scanning techniques.

 

It was a full year before I was able to arrange the sensitive scanning I wanted. It was a tough time. I continued my hazardous sports but was keenly aware that I was taking large risks without travel insurance. Eventually in September 08 I had a normal scan….. It was great!  ….My life changed. I got travel insurance again. The world was my oyster. For the first time since 2006 I started to plan things for the future. I know that many specialists believe there is not much point in screening for a disease that is only rarely treatable; however, psychological studies on patients with uveal melanoma like mine have shown that regular liver scans are very helpful because they reassure patients that they will continue to be well, at least for the next few months, so that life can be lived normally. My experience as a general practitioner has given me a broad perspective on many different cancers and my impression is that tests and treatments that are considered affordable for common cancers are sometimes regarded as being too expensive for rare cancers.

 

In May 2009, my second private scan showed a new 3mm lesion. Repeat scanning in August established growth. On the 9th September 2009, I had liver resection and 3 small tumours 9mm, 3mm and 4mm were removed. I was only able to have this operation so quickly because I was prepared to pay for it privately.  Recovery was straightforward.

 

 

Picture8

Here I am diving in Mauritius 8 weeks after my resection

 

 

Picture9

...and skiing in Colorado 12 weeks after my resection

 

 

Life… is now very good. In the last 3 months we have been diving in Mauritius, skiing in Colorado, with Christmas in Italy, diving in the Maldives and skiing again in Colorado. My husband and I have really made the most of every day together we have been able to do some of those things we had planned for our retirement. Last week’s liver scan was normal so training for my next half- marathon is ongoing.

 

My next challenge is to delay metastatic disease, or arrange things so that when the metastases come back I can get the best treatment without wasting time. I would love systemic therapy now. However, since my liver resection I have seen many oncologists- all caring professionals – who have offered me no treatment whilst clinically free of disease. I have been told that when I get metastatic disease, Dacarbazine is my only treatment. A few minutes on Google revealed that Dacarbazine has a less than 1% response rate in the treatment of my condition. Surely an unproven treatment is better than one proven not to work?

 

Discussion

Evidence- based medicine is seen as a great advance- but not to sufferers of rare cancers. In my lifetime …or yours, I don’t expect randomized trials to provide evidence for a beneficial treatment for metastatic uveal melanoma.  A different approach is needed for rare tumours like mine. How many of successful and wonderful ocular treatments are based on randomized trials?

 

In summary, I consider myself very fortunate. I have good vision and ..full… active… life. This may all have been different if I had not insisted on the optometrist performing perimetry when he found my eye healthy; if I hadn’t refused to wait for Brachytherapy; if I had settled for a less sensitive screening rather than pay for MRI with contrast, or if I had not insisted on immediate liver resection, again being prepared to pay. Having a medical education made this path easier for me. Who looks after the patients who are not doctors? I wonder.

 

My personal experience has made me realise how treatment is often given without informed consent because the patient is only informed about convenient treatments available locally. Nowadays, many patients are prepared to travel if only they are given proper information.

 

It is so important to empower patients and their relatives to help themselves as best they can. If knowledge is power, then patients need as much knowledge as possible.

 

Uveal melanoma patients are losing out because are not being told the truth (by some), and so that they led to believe they are cured until suddenly they find they have only a few weeks to live. I saw this with one of my own patients with ocular melanoma, who bought an old farmhouse to renovate only to develop metastatic disease only when this work was completed; if he had been told the truth about his prognosis, then he might have bought a different house, which he would have enjoyed during his final days.

 

Unlike other cancers, most uveal melanoma patients don’t have tumour typing or grading and are not staged properly, with the best tests available. Better grading and staging would give patients a better chance to plan their life and should also improve prospects for evaluating new treatments aimed at preventing or delaying metastatic disease.

 

Patients with rare cancers like metastatic uveal melanoma are let down by the excessive reliance on randomised studies, which are not possible with rare tumours. And so, they are given useless treatments like dacarbazine. With proper audit, it is better to have an unproven treatment than one proven not to work at all.

 

I hope my experience will help improve the care of future patients.

 

Acknowledgements

I would like to acknowledge all who have helped me with my disease, especially my husband for his unwavering support.

 

I have seen many wonderful doctors; however, the following deserve a special mention: Professor Damato, Consultant Ophthalmologist/Ocular Oncologist; Dr Guthrie, Consultant Radiologist; and Professor Lodge, Consultant in Hepatobiliary andTransplant Surgery.

 

Finally I would like to thank the Lance Armstrong Foundation for their ongoing encouragement in helping me make the best of a difficult situation.

 

I would welcome e-mails but regrettably I am unable to advise individuals.

 

This e-mail address is being protected from spambots. You need JavaScript enabled to view it

Doncaster,

UK