Against the odds
The Bray-based couple hoped this for their first-born because the alternative was so much worse.
At Holles Street Hospital, where Seán was born, doctors suspected he had cystic fibrosis (CF). In Ireland, one in 19 people carries the gene for CF, which affects the glands and damages organs including the lungs, pancreas, digestive tract and reproductive system. It causes thick, sticky mucus to be produced, blocking the bronchial tubes and preventing the body’s natural enzymes from digesting food.
“Seán didn’t do that all-important green nappy after he was born. He had a blockage in his bowel and the doctor said it was CF. It was very frightening,” says Joe, who thought of his only other encounter with CF.
“My sister had pneumonia in the ’80s and she was tested for CF. When she was in hospital, I saw loads of kids with the condition who were in wheelchairs. They didn’t have enzymes then to aid digestion — many of those kids died from starvation because they couldn’t absorb food,” he says.
Transferred to Crumlin Children’s Hospital, where he spent a week in intensive care, Seán’s diagnosis seemed milder. “A doctor told us he was 99% sure Seán had Hirschsprung’s disease, where a part of the child’s intestine is dead. They take out the dead part and join up the remaining two pieces. Compared to CF, we thought this was good.” But their hopes were dashed when tests confirmed CF. “We felt our whole world had come to an end,” Joe says.
After six weeks, the Kavanaghs brought Seán home to live with the challenges of CF. “It was all new to us. He suffered from reflux and would puke up his bottle. We knew we needed to get as much food into him as possible to get his weight up. Every week, we’d take him to Crumlin Hospital to be weighed and he’d only have put on two ounces,” he says.
Within a few months, Seán outgrew the reflux and began holding down food. But his care meant other demands, such as twice-a-day physiotherapy. “We’d cup our hands and tap him on the chest, under the arm, and on the back 600 times a day to dis-lodge mucus in his lungs. We did this until he was three and got a little mask with which he could do breathing exercises,” he says.
Now 13 and a first-year student at Presentation College Bray, where he loves languages, Seán is irrepressible and lives life to the full. While his dad, who works in sales, wanted him to play golf rather than rugby (“Exercise helps dislodge mucus but too much exercise causes weight loss”), Seán had other ideas. “He wanted to play rugby and he gave up golf. He loves rugby. He trains twice a week and plays matches on some Wednesdays. With golf, I used to have to tell him to get his golf clubs. No such thing with rugby — he has all the gear,” he says.
For years, Joe wanted an activity he and Seán could do together. “I thought about a tandem bicycle but it had to wait until he was older.” When Seán was 10, the pair cycled 176 miles from Bray to Ballyhea, Seán’s granny’s home village in Cork. They raised €30,000, which went towards a new four-bed CF unit in Crumlin Children’s Hospital. Seán spent two weeks in the hospital prior to last Christmas and — though on intravenous antibiotics to rid him of a difficult infection — his good humour won the day. “He got one of the new rooms. He was in a motorised bed and was telling everyone he’d raised the money for this bed,” he says.
One of the biggest challenges continues to be weight-maintenance. “He’s very slight. Considering what he eats, he should be 20 stone.”
Seán takes vitamins, as well as enzymes, daily to help him break down fatty foods and to aid digestion. He’s on a nebuliser three times a day — this delivers antibiotic treat-ment to protect against infection. He has recently got a new type of nebuliser, which has reduced his sessions from 10 minutes each to three minutes.
Just into his teens, Seán’s entering a phase of life when — according to studies — many young people with CF rebel against taking their medication because it’s so inconvenient. For this reason, the CF world welcomed last November the launch of a new hand-held, portable inhaler — the Podhaler, which medics hope will result in better treat-ment-compliance, mainly because it has the potential to dramatically cut down the four and a half hours that patients spend each week connected to a mains-powered nebuliser. Adding to its user-friendly appeal is the fact that it doesn’t need to be washed but simply wiped with a dry cloth after use.
CF is a progressive, life-threatening disease. “Sometimes, you forget Seán has it. Other times, you remember and it’s depressing — knowing the outcome isn’t going to be so good. We’re hoping he’ll get a good old spin out of life,” Joe says.
The Kavanaghs’ eight-year-old daughter, Sarah, doesn’t have CF.
When Seán was born, a dad whose 10-year-old girl had CF gave the couple some much-valued advice. “He said, ‘you don’t have CF — Seán does. Don’t live CF’. We don’t. We have a little girl too and if we did live CF we’d be neglecting her.”
The couple also take their cue from Seán. “He knows all about what having CF means but he doesn’t say ‘I’m only going to live ’til whenever’. He’s a clever fellow and he sometimes uses CF to his advantage, like when he tells some girls he’s very ill so as to get a kiss off them,” Joe says.
. Visit www.cfireland.ie.
Ireland has highest proportion of CF sufferers
One of Ireland’s most common life-threatening inherited diseases, about one in 19 people is a carrier of the CF gene.
¦ Ireland has 1,100 people with CF — the highest proportion in any country in the world.
¦ Two carriers have a one in four chance of their baby being born with the condition.
¦ CF affects the glands, damaging organs including the lungs, pancreas, digestive tract and reproductive system. It mainly affects the lungs and digestive system. ¦ It causes the production of a thick, sticky mucus that blocks bronchial tubes. Build-up of mucus makes it difficult to clear bacteria, leaving the sufferer prone to cycles of lung infections and inflammation. These repeated attacks can damage the lungs.
¦ Mucus prevents the body’s natural enzymes from digesting food, so it’s difficult for the person to absorb sufficient nutrients. For this reason, people with CF must consume artificial enzymes with food, to help them get adequate nutrition.
¦ For most patients, symptoms appear in the first year of life, though they may appear later in the teens or in adulthood. First sign of the condition in the newborn is likely to be intestinal obstruction.
¦ It’s impossible to predict the outcome for a person with CF — for some people, lung disease can progress rapidly, despite early diagnosis and treatment. Others have a more favourable journey, reaching adulthood. Some factors considered important in determining prognosis include treatment, nutrition, lifestyle and compliance, as well as exposure to viral, bacterial and fungal infections.
