Association for Multiple Endocrine Neoplasia Disorders

Jo (Cameron’s mum; age – ancient according to Cameron)

Cameron had no symptoms of medullary thyroid cancer at all since his total thyroidectomy was done prophylactically (to prevent cancer).  When it was discovered that I had the rare genetic disorder, multiple endocrine neoplasia type 2 (formerly MEN2a), both my children underwent genetic testing which identified that only Cameron had inherited the faulty gene.  All patients with MEN2a develop medullary thyroid cancer (MTC) and so we made plans for Cameron to undergo a prophylactic total thyroidectomy when he was just 3 ½ years old in the hope that this would ensure he never develops MTC. 

Cameron had a few blood tests before surgery to see if this would tell us whether or not he already had MTC, but they were not entirely conclusive and one, the pentagastrin stimulation test, was somewhat barbaric for such a young child (and is no longer done in children).  The histology later showed that Cameron was beginning to develop the cancer since there were areas of what is known as C-cell hyperplasia (the precursor to MTC).

Back in 2002 there was a difference of opinion as to when to operate to remove Cameron’s thyroid.  A paediatrician said to do so before the age of 5 and a surgeon thought it could be left until after puberty.  Since then things have moved on in the way of knowledge and there is now agreement as to when to perform surgery (asap or before age 5 in our family’s case).  At that time, which was pre-AMEND, it helped me most to talk to another family in the USA with MEN2a, and whose daughter had undergone surgery at age 4.  She did already have MTC cells in her thyroid and this gave me the determination to insist on early prophylactic surgery for Cameron.

Once the right surgeon was found it was relatively plain sailing.  Kids are generally amazing at recovering from surgery and Cameron was up and about finger-painting and demanding jam sandwiches within an hour of waking from the anaesthetic.  He stayed in hospital for just 2 nights after surgery mainly because he needed regular blood tests to check his calcium levels which had dropped quite a bit due to parathyroid gland injury.  He had had a parathyroid auto-transplant in his neck but it took a few weeks after surgery for this to start working again properly.  Once home the blood tests continued for a couple of weeks at our local hospital and he found this rather traumatic as it seemed every ‘Tom, Dick and Harry’ wanted to practice on him. Due to this Cameron developed an acute fear of needles which has taken years and plenty of encouragement for him to overcome, but he’s got there now.

Being so young it was hard to get Cameron to take his levothyroxine at first.  He couldn’t manage to simply swallow a pill as we adults do and so I began by crushing the tablet and either putting it in a spoonful of apple sauce or by dissolving it in a syringe of juice.  The latter caused tooth decay very quickly and so, full of guilt, I encouraged him to take his pills ‘like mummy does’ instead.  He quickly caught on and now happily pops his pills with breakfast in the morning, but generally only if he’s reminded to do so!

At the time of his surgery Cameron was too young to understand much about what was going on.  At that time I told him that he had a ‘bad butterfly-shaped thing’ in his neck and that it needed to be taken out before it made him very poorly and he seemed to accept this.  He has to learn considerably more about the other aspects of his condition as he grows older and I am totally honest with him when answering any questions he has.  He knows the signs of hypothyroidism already and once insisted that he needed a dose increase because he was feeling so lethargic, had bags under his eyes and was getting chubby around the middle – he was proved correct with a blood test!

To other parents I would say, get informed and don’t be afraid to question your child’s healthcare professionals.  Also, when they are to face a lifetime of monitoring blood tests it is essential to insist from day one that these are done by someone experienced in order to avoid repeated jabs and the increased stress and anxiety that go with this.  Finally, be very honest with your child; they know if you’re not!

 

Cameron (Jo’s son; aged 13)

I can’t remember much about my operation because I was only 3!  I do remember the hospital a bit because it was fun, the nurses were really nice and they had an aquarium with loads of different fish in it.

I take my pills every day with my breakfast but sometimes I forget.  I now take two pills, a 100mcg and a 25mcg.  I was on 100mcg but I was feeling tired all the time so that’s when mum and I thought that I needed more.  I had a blood test and the doctor wrote a letter to say that I needed another pill.  I now have much more energy and enjoy playing lots of football and I also love to ski.  I think I feel like a normal boy!  Some of my friends know a little bit about my condition and I wear a MedicAlert bracelet, but other people don’t know that there is anything different about me.

To other children like me I would say don’t panic about your blood tests.  Try to watch the TV or something while they do it.  I began to think the blood looked like blackcurrant juice when it goes along the tube and that made me laugh!  Nowadays I am much calmer about it!

[2013]