I FOUND out recently that the health problem I was diagnosed with aged eight, a rare lung condition called Hyper IgE Syndrome, is so rare it affects around only 20 people in the UK – it is so uncommon, I’m literally one in a million! I suppose I should be flattered.

Even I’ve never met anyone else who has the same thing as me - I guess I’d just be worried that we’d having nothing else in common.

Hyper IgE is one of a cluster of conditions known as primary immunodeficiencies, which makes me very vulnerable to infection, especially in my lungs.

As a child, I was in and out of hospital, and spent two birthdays in Great Ormond Street – a couple of days before turning nine I was in an oxygen tent.

I was always “the sick one” at school and didn’t take part in sports, although my surgery scars always impressed my classmates.

Given that doctors don’t see many people who have what I have, I feel lucky to have been diagnosed at all – I flummoxed medics until the problem was eventually identified at Great Ormond Street. I feel fortunate we have the NHS, and have generally been looked after extremely well.

At times, the condition has restricted what I can do – I have to take 15 tablets a day which keep me alive. But you learn to live within the limitations, and I don’t feel as though I have missed out on much.

Over the years I’ve had maybe 20 operations, the first at just 22 months, which left me with scars over much of my body. Abscesses of the lungs and elsewhere are particular problems, hence the surgery.

In the last couple of years, I’ve felt myself start to slow down. It takes me longer to get over late nights, to the extent that I no longer even take being able to breathe for granted.

I have always known I would have a shorter than average life, and it certainly focuses the mind – I am aware that if I don’t do the things I want to do now, I probably never will.

I’m lucky to have the support of my husband Richard, 45, an accountant. In 1997, when I woke up in the early hours with a collapsed lung, he asked me what I should do, giving me back control of the situation. I knew then that he was the one for me!

Probably one of my lowest recent times was in 2011 when, from early summer and into the autumn, I had three kinds of pseudomonas or superbug infections, and a fungal infection, penicillium. I was virtually living downstairs and permanently exhausted. It would take me two hours just to do a supermarket shop. My lips would often turn blue, and I felt as though there was no breath left in my lungs.

But I kept going and took no time off work as an admin manager for a Twyford publishing and marketing company, although it would take me up to 15 minutes to recover from walking across the office car park and up one flight of steps. It was easier to just carry on. I admit I don’t like being cared for and can be stubborn at times!

That spell made me think of the Swiss assisted suicide organisation Dignitas – and I contacted their website; I was not sure how much longer I could go on. For me, it is good to know they are there. I would not want to be cared for full-time, and hospitals aren’t always geared up to dealing with conditions like mine.

A year ago, a dear friend died at 50 from breast cancer, in hospital with just a curtain pulled round her for privacy. I know I couldn’t face that.

I don’t think we talk enough about death in this country, and we find it hard to accept that it will happen to all of us. With so many medical advances, people don’t expect to become unwell, and so are perhaps more floored by illness than they should be.

Realistically, the risk of infection is so great I know I won’t reach 50. But I have every intention of celebrating my 40th next spring, not least because some doctors didn’t think I’d make 20, never mind 40.

I was told not to work, but have always worked. I was told not to travel but have been to Australia four times, among many other places – next on the calendar is a trip to Canada in May. I would like to travel more, particularly in Europe and to see more live music – Band of Skulls is top of my list.

That’s why I’ve just had my first tattoo done, bearing the words, 'The scar remains, reminds me that I’m still living’, from the song Walk Away by Green Day, a favourite band. I just wanted to mark this birthday and the fact that I’ve reached it against the odds.

For me, for now, it’s all about life, not death.


- HIES affects around one in a million.

- It is estimated about 20 people have it in the UK and around 100 across the world.

- Also called Job’s syndrome and Buckley syndrome, the condition is known medically as a heterogeneous group of immune disorders.

- The syndrome is characterised by recurrent “cold” staphylococcal infections, unusual eczema-like skin rashes, severe lung infections that result in pneumatoceles (balloon-like lesions that may be filled with air or pus or scar tissue) and high concentrations of the serum antibody IgE.

- Most patients with Hyper IgE syndrome are treated with chronic antibiotics to help protect them from staphylococcal infections. Good skincare is also important in patients with hyper IgE syndrome.