Leo is running around in the garden playing tree monsters with his brother and daddy. He runs up to me as I sit enjoying the autumn sun. "Mummy, I'm happy," he shouts. My heart bursts and breaks at the same time. I know it seems morbid but I sometimes wonder how many autumns he will have. I also wonder whether he will still be happy when he realises what is wrong with him.
Leo was diagnosed with cystic fibrosis at the age of six months, after being very ill since birth. As CF results in too much mucus being produced in the body, mainly the lungs and digestive system, he needs help to combat this. He is now three and just about tolerates his daily regime of 12-14 digestive enzymes, four doses of inhaler, physiotherapy morning and evening, antibiotics, laxatives and vitamins. This treatment plan seems to keep him well and provides us with a near-normal family life.
He now accepts that he has to have his digestive enzymes with all meals. These replace those that normally make their way from the pancreas to the stomach, but Leo's gut is too clogged up with mucus. He does, however, get very distressed about his bowels, which seem to swing from diarrhoea to constipation. "Do poo Mummy... got tummy ache... lie on mat," often means it will take him about half an hour to empty his bowels, aided by lying (and crying) on the changing mat and large doses of TLC. When I am stroking his tummy I find myself trying to predict the future and wondering how he will cope with this when he is 16.
The prospect of him taking more control of his own health as he grows up is both frightening and liberating. I am sometimes horrified by how much his life expectancy depends on my ability and motivation to carry out his daily regime. If he didn't get his enzymes, antibiotics or physiotherapy, his life expectancy could be cut in half. Consequently, I seem to spend a lot of energy remembering which medicine comes next, or how to persuade him to take it.
Leo's treatment was seriously interrupted last year. I thought I would be ultra-efficient and get a nagging little job out of the way. I knew the stool wasn't high enough but thought I could reach to install a new battery into the smoke alarm. I hadn't taken account of my reaction to the sudden beeping it set off and fell. There I was with two toddlers downstairs, six weeks before Christmas, with a broken wrist. Grandparents came to the rescue but this couldn't be expected of them every day. I thought I would try social services. This proved to be quite amusing as the social worker seemed to think the health visitor should sort something out, and the health visitor seemed to think the social worker should do it. I got by with scrounging lifts, but my main concern was coping with Leo's treatment.
I was amazed by how quickly I learnt to open his Creon capsules with my teeth. Fortunately, the plaster cast was only on for four weeks, but Leo did develop a cough and missed out on a lot of physiotherapy.
Holidays have been a bit of a problem. I have been reluctant to go abroad with Leo since he was diagnosed, and this anxiety was heightened by the Sars scare. (If he caught the virus he would be unlikely to survive.) After a rather disappointing caravan holiday in Cleethorpes, mainly due to cigarette smoke throughout the "family-friendly" facilities, we plucked up the courage to go to France in August.
The ferry company said that Leo would not be covered by its usual travel insurance but recommended a specialist. After completing a lengthy telephone questionnaire for this specialist, I was speechless to be quoted £789 for one week in France. I later managed to obtain insurance from a company recommended by the Cystic Fibrosis Trust for £60.
We would love to visit relatives in Australia but the flight could present problems as Leo might need oxygen while travelling in a pressurised cabin. It has recently emerged, through a survey done by the CF Trust, that airlines vary in their approach to this special need. Some carry extra oxygen at no charge while others can charge up to £500, although a four-litre canister of oxygen costs less than £15.
As parents of a child with a life-threatening illness we are aware of the need to educate Leo and his brother, Oskar, about the condition. We always call the medicines by their correct name, we refer to CF regularly, and we honestly point out to Leo that if he doesn't have his medicines he could become very ill and have to stay in hospital. But we sometimes slip into denial and tell ourselves that Leo is very well now and will always be very well. Unfortunately, that probably won't be the case. Having read about CF sufferers in the CF Trust newsletters, it seems that their health tends to start to deteriorate around 30 years of age, resulting in a heart and lung transplant or retirement on medical grounds.
We are also aware of the impact of Leo's condition on family dynamics. Oskar's justified jealousy at the amount of extra attention Leo gets was recently crystallised into a single statement: "Leo hasn't got cystic fibrosis, he's better now. I've got it now." In other words, "It's my turn to have all the attention." We try to compensate in various ways, but sometimes I feel like saying to Oskar: "When you understand what this is you won't be jealous of Leo."
One ray of hope that kept us going for quite a while was the prospect of gene therapy. Leo has been given a life expectancy of 40, but we were under the impression that gene therapy would be sorted out long before that, effectively giving Leo a "cure". Our understanding of how it works is that healthy gene cells are somehow introduced into the lungs to counteract the unhealthy genes, thus preventing over-production of mucus. However, our hopes have been dashed since watching a Horizon programme about gene therapy, which featured the case of a young adult dying as a result of being involved in research into gene therapy. The problem seems to be the "vector", the agent that carries the gene into the body. At present, viruses are used as vectors, with obvious problems emerging - infection from the virus.
At the end of the programme, my partner and I looked at each other and said: "It isn't going to happen, is it?" We were both quite down for a few days, and subsequently felt stupid for having pinned our hopes on a cure. More realistically, medical treatments are improving at a great rate - 10 years ago Leo would not have had access to the effective regimen he is on now. And the Department of Health has allocated £2.5m over the next five years for CF gene therapy research.
Leo has recently had his third birthday party, an event which I found emotive. Unaware of the implications of what is happening inside his body, Leo is acting like any normal toddler. He has a tantrum if he cannot wear his Bob the Builder shirt. He is rather put out by the fact that his older brother has started "big school" but he can't go. He tries to boss me about and often succeeds. Mealtimes are a nightmare as he doesn't stuff himself as much as he used to. Combine this with the fact that he is supposed to eat 30% more food than a non-CF child (to balance out the malabsorption) and you have a recipe for mealtime stress. Parental anxiety about feeding CF children is quite common. I recently spoke to Leo's dietician about the fact that he seems to want to be fed rather than feeding himself. I confessed that I tend to follow him around with spoonfuls of food in an effort to keep his weight up. It was tactfully pointed out to me that the problem seems to be mine.
Finally, I discovered something mind-boggling recently. Leo has CF because my partner and I are both carriers of CF; my partner, Leo's father is my second husband. I have discovered that my first husband is also a carrier of CF. I find this oddly comforting - almost as though I was destined to have a child with CF.
