I’m very reassured to be occasionally contacted by new readers of this blog, mostly patients with MEN themselves, because they find the information useful and want to seek some more advice. However, it’s also clear that I haven’t updated the blog for a considerable period of time and – with staggering university commitments in my final year, as well as lots of other personal, time-consuming projects – I think it’s unlikely that I will be able to prioritise writing about health in the foreseeable future. I want to make it clear, though, that anyone who finds themselves reading this should feel immediately free to contact me about anything. This blog will remain here as it is now, with information both about MEN in general and about my own experience with the condition, so I hope you will find some of that useful, but if you need a little more, don’t hesitate to get in touch.
Advanced MEN2 and Faith Healing
There are many families around the world who have relatives, often children, affected by devastating illnesses that cannot be cured. Those with a belief in a god may turn to faith healing, which, though ineffective, is an understandable measure given that these families are largely bereft of hope.
Much has been made of cases where treatments are available, and where faith healing has been used in place of standard medical practice, often leading to unnecessary deaths. But I came across a more intriguing case today, of a family with a genetic disorder for which a treatment exists, but which they apparently didn’t know exists.
On Mary Velez’s blog – a blog called The Way of Love, which is devoted to her career as a spirit healer – she wrote about a young boy, Marcel, whose parents came to her for help because he had been diagnosed with Multiple Endocrine Neoplasia (MEN) and was told he has less than a year to live.
As a patient with MEN myself, I know the condition very well, and, importantly, there are two types, distinguished by the different glands that they damage. I have type 1, and I suspect Marcel and his family have type 2, as type 2 is far more likely to spread as a cancer, especially when young.
Both types of MEN are well-understood by educated endocrinologists, and both of them are treatable. The standard practice is to simply remove tumours wherever they occur, which sounds simplistic and can necessitate life-long medication, but it works. Now, thanks to amazing genetics research in the past few decades, patients with both MEN1 and MEN2 can expect to live as long as anyone else, so long as they have a good doctor who diagnoses it early.
For children with MEN2, and therefore with Marcel, the most important procedure is to remove the thyroids as early in life as possible in order to stop all chance of metastasis. Ordinarily, if a child’s parents already know that they have the disorder in the family, the child will have an operation very soon after birth. Sadly, even though it seems Marcel’s family was aware of the genetic condition, they and their doctors cannot have known that Marcel ought to have had his thyroids removed immediately. If they had known this, Marcel could have lived a much longer life.
Multiple Endocrine Neoplasia is not a fatal condition when well-managed. It is absolutely heart-breaking that Marcel has been given a death sentence, but what this case demonstrates is that information, education and learning about health conditions are what saves people. If you fall back onto faith healing, you are accepting that things are beyond your understanding and control. You therefore accept defeat. Although many individuals will have conditions so advanced that no new treatment can help them, anyone affected by MEN or other horrible illnesses should push for more consciousness raising and medical research, so that awareness is raised amongst doctors, and other families realise that it doesn’t have to be this way. There is hope, and it’s in science.
The Annual MRI Scan
I returned to university on Monday, and it turns out that I was just in time because, waiting in the post-room, I had a letter from the hospital here in Oxford saying that I had an appointment for an abdominal MRI scan on Wednesday (all of my treatment takes place in Oxford where I am at university, but outside of term I live in Norfolk).
Having a multiple endocrine neoplasia disorder requires life-long surveillance unless you reach the unfortunate stage of having all your potentially affected endocrine glands removed (which, in itself, would necessitate life-long regimes of medicinal supplements). So, for me, although the period from October 2008 – May 2011 was my major period of diagnosis, medical and surgical treatment, and general personal upheaval, my calmer period nevertheless features regular trips to the hospital.
Probably the most important surveillance technique in managing MEN1 is an annual MRI scan, with mine happening to fall in January. Although an MRI can occasionally detect a parathyroid tumour (other nuclear medicine scanning techniques are more effective), MRI scans are used in MEN patients to keep track of pituitary and pancreas tumour development. This could mean either detecting a tumour in the first instance, or making sure that a tumour already detected isn’t growing or spreading. Whether you have any tumours on these glands or not, if you have the MEN1 gene, it is suggested that you have these annual scans.
In my own case, I have a pituitary adenoma already detected, and a collection of pancreatic tumours, half of my pancreas having already been removed. In my case, as the pituitary tumour is a micro- (small) rather than macro- (large) adenoma, it is unlikely to grow and cause physical problems near my eyes and brain, so surveillance is of low importance, while medicinal treatment with testosterone replacement to offset the increased prolactin production is vital. Instead, my pancreas is the major focus of my annual trip inside the scanner, as there is roughly a 50% chance of pancreatic tumours turning malignant in patients with MEN1.
There are two key features to look out for in patients with pancreatic tumours:
- Tumours which are greater than 20mm (2cm) in size, as these are most likely to spread soon, and many surgeons will operate on them pre-emptively.
- Evidence of tumours already having spread, usually to the liver in the first instance (often accompanied by jaundice).
An MRI scan can be very unpleasant, as the machines are claustrophobic, uncomfortable, and, for abdominal scans, require occasionally difficult breathing exercises. Of course, they are also extremely loud, but competent technicians should give you sufficient ear protection. Given this, I was pleased to be told at the hospital today that their ‘new’ and ‘slightly more advanced’ scanners have halved the time it takes for the particular scan I needed – I was in and out in 20 minutes.
It will be about a week before my results are passed on to my endocrinology consultant, though I don’t have an appointment scheduled with her until May. I may well chase the results up in a couple of weeks, but I think it will be safe to assume that no news is good news – I think even my forgetful doctors would get in touch with me as soon as possible if my tumours had grown significantly or had metastasised to another organ! With any luck, the scan will be fine and, physical symptoms being well, I’ll have the all-clear for the next year and can get on with my degree uninterrupted.
Should we be scared of cancer?
‘Yes and no’ is the short answer. Perhaps ‘much less than we should have been a few decades ago’ is more accurate.
Over at nature.com, on their blog Soapbox Science, David Ropeik has written about ‘cancer phobia’. From the point of view of a consultant in risk perception and communication, he astutely demonstrates that cancer is not necessarily the horrifying monster it would once have been, but the word ‘cancer’ nevertheless retains its frightening punch. And because the word still carries such psychological weight, many people are driven to great anxiety and even to unnecessary medical treatments though their condition is easily treatable and manageable.
Take Ropeik’s example of prostate cancer, where he quotes the U.S. National Institutes of Health (NIH):
Although most prostate cancers are slow growing and unlikely to spread, most men receive immediate treatment with surgery or radiation. These therapeutic strategies are associated with short- and long-term complications including impotence and urinary incontinence.
Approximately 10 percent of men who are eligible for observational strategies (keep an eye on it but no immediate need for surgery or radiation) choose this approach.
Early results demonstrate disease-free and survival rates that compare favorably (between observation and) curative therapy.
Because of the very favorable prognosis of low-risk prostate cancer, strong consideration should be given to removing the anxiety-provoking term ‘cancer’ for this condition.
In other words, prostate cancer is a condition that we can deal with much better than we could just a few decades ago, but the very word ‘cancer’ sets people in a frame of mind that leads them to tackle the problem more aggressively than is necessary. This is all thanks to a fear of cancer having successfully seeped into the public consciousness more than with any other diseases. A Harris poll discovered that 41% of people in the U.S. fear cancer more than any other medical condition, compared with 8% who fear heart disease most even though heart disease is actually the bigger killer. ‘Cancer’ is more viscerally loaded with connotations of fear, pain, and death.
Of course, our advancement in some cancer treatments does not detract from the fact that many cancers are still inoperable and incurable. But cancers are killing less and less, and people are starting to question the psychological effect that a ‘cancer’ diagnosis can have on patients who have very good prognoses. So let’s not take our eye off the ball in the ongoing effort to cure cancer, but let’s also not let ourselves get carried away with visions of unstoppable trauma if we hear the ‘c’ word.
Pituitary Disorders, Giants, and Burial Rights
Skeleton of Charles Byrne, the 'Irish Giant', in the Hunterian Museum
In Multiple Endocrine Neoplasia type 1, pituitary tumours occur in around a third of patients. Often, these secrete excessive amounts of hormones, with the most common type being a prolactinoma (the type I have). A rarer kind, somatotropinomas, overproduce a growth hormone which in turn causes a condition known as acromegaly.
In recent years, it has been determined that ‘Irish Giant’ Charles Byrne (1761-1783) had such a tumour, causing the immense growth of his skeleton. In his case, the pituitary tumour was caused by a different condition called Familial Isolated Pituitary Adenoma (FIPA), as his condition was hereditary (familial), but no other glands in his body were affected (isolated) as you would expect in MEN (I am currently writing an information booklet on FIPA for AMEND, as the Pituitary Foundation has apparently shown little interest in the genetic disorder. Hopefully I’ll have some news on that in the coming weeks).
Byrne, from County Londonderry, stood a little taller than 7ft 7in, and found fame exhibiting himself as a “freak” in London. At the age of 22, he died at his home after having taken to drink. Intriguingly, he requested to be sealed in a lead coffin and buried at sea, but John Hunter – the renowned Scottish surgeon after whom the Hunterian Museum at the Royal College of Surgeons is named – bribed one of Byrne’s friends to acquire the body and boiled it down to the skeleton.
Since a few years after Hunter died and his collection was acquired by the RCS, the skeleton has been on display at the Hunterian Museum. However, as an article in the Guardian reports, experts in law and medical ethics have suggested that the skeleton finally be laid to rest at sea:
What has been done cannot be undone but it can be morally rectified. Surely it is time to respect the memory and reputation of Byrne: the narrative of his life, including the circumstances surrounding his death.
The Museum has refused to do so, stating that the skeleton remains important both culturally and in terms of research, especially as the skeleton has been key in recent discoveries about FIPA. I can’t understand this reasoning, though. As the ethicists have pointed out, the DNA has already been extracted and can be used again without the original skeleton; it is likely that some current patients with acromegaly would participate in research while living and leave their bodies to science in death; and, for the sake of a cultural display, a synthetic copy could be made, which is not unusual. Although they seem to have no rational defence for it, the Museum’s response is simply this:
The Royal College of Surgeons believes that the value of Charles Byrne’s remains, to living and future communities, currently outweighs the benefits of carrying out Byrne’s apparent request to dispose of his remains at sea … At the present time, the museum’s Trustees consider that the educational and research benefits merit retaining the remains.
Steve Jobs’s Pancreatic Cancer
Steve Jobs
I found out just today that Steve Jobs had a particular type of pancreatic cancer that is rare and behaves atypically, but will be familiar to many MEN patients. The tumours are called insulinomas (which happen to be the kind that I had), and, as the name suggests, as well as being abnormal growths they also produce excessive amounts of insulin. This can lead to all sorts of problems with low blood sugar, but it is their potential to spread to the liver and other organs that is of most concern. Still, strangely enough, it’s a kind of pancreatic cancer that is curable.
Usually, pancreatic cancers are very aggressive and hard to treat, with one of the worst prognosis rates of any cancer. However, pancreatic cancers that secrete hormones – such as those found in MEN and other rare cancers such as Steve Jobs’s – are classed as NeuroEndocrine Tumours (NETs). Although these can also be malignant, they are typically slower growing and less aggressive. An early surgical removal (though possibly leading to diabetes and other digestive issues) can usually cure you, but Steve Jobs sadly didn’t take this treatment early enough, even though it was available to him. An interesting article by the BBC explores why Steve Jobs didn’t opt for surgery when it could have saved his life.
Finding a Parathyroid Surgeon in the UK
Most people with a MEN disorder will develop one or more parathyroid tumours at some point in their lives. The treatment for this, as is usually the case with MEN tumours, is surgical removal, but it’s important to know what kind of surgery to have. Many surgeons carry out a small number of open parathyroid surgeries in a year, but you ought to look for a surgeon doing a large number of operations per year who offers minimally invasive (laparoscopic) surgery.
Simon Doherty, who writes the blog Do I Have Primary Hyperparathyroidism? has put together a useful list of such surgeons in the UK.
