A daily battle for young CF sufferer

ON Tuesday, November 23, 2004, Billy Burke lost his battle with lung disease.
The 29-year-old Killorglin man, who also suffered from cystic fibrosis, had been on a waiting list for a double lung transplant for almost four years, died without having the life-saving operation.

In the absence of a transplant facility in Ireland, the Freeman Hospital in Newcastle, England had the exclusive contract with the Department of Health to perform all Irish lung transplant operations.
Mr Burke was removed from Freeman Hospital’s waiting list in November 2003 because he was considered too high a risk after he had contracted an infection.
Mr Burke sought a second opinion and was put on the waiting list at Wythenshawe Hospital in Manchester.
That hospital sought priority access for Mr Burke to suitable Irish lungs but this was allegedly refused because of Newcastle’s first call on all lungs donated in Ireland.
Mr Burke had appealed directly to the then Taoiseach, Bertie Ahern and to former Health Minister, Micheál Martin, to ensure that he got the operation. More than 55,000 people signed a petition supporting his call, while 5,000 people attended a rally in Killorglin in April 2004.
Deborah and Patsy Kett, who live in Rathclooney a few miles from Clooney village, had more reasons than most to take a special interest in the Burke case.
On April 6, 2004, Deborah received the bombshell that their first child, Hannah had cystic fibrosis. It was a huge shock because there was no history of the disease in the family.
Patsy’s grandmother, Annie, from Killaloe, who is 104 this year, had no recollection of anything resembling the disease and similar research into Deborah’s own family also drew a blank.
Hannah was born in January 2003 following a normal delivery and didn’t experience any serious problems until she was about eight months old. Between eight months and 14 months, Hannah was very sick with chest infections and ended up with a partially collapsed left lung.
An X-ray at the Mid-Western Regional Hospital, Ennis revealed an infection, which her parents thought would be cleared with antibiotics but this didn’t happen. A third X-ray showed pneumonia in March 2004 and this required a transfer to the Mid-Western Regional Hospital, Limerick.
At the time, Deborah was pregnant with her second child, Michael. On April 5, Hannah had a very high temperature and pneumonia and Deborah remembered her paediatrician asking about the possibility of cystic fibrosis. She couldn’t understand these questions because of the lack of any previous history.
In the meantime, Billy Burke’s desperate plight was being highlighted in the media at the time, which made the shocking diagnosis even harder to come to terms with.
Deborah recalled being moved into a single room, which seemed to indicate the arrival of bad news. Later that evening, the paediatrician informed her that Hannah had CF and stated, “Mrs Kett, you are aiming for quality of life, not quantity”.
“When you bring your daughter into hospital with pneumonia, you don’t expect to be told she has cystic fibrosis. A CF nurse gave me information about the condition and I was also referred to a CF specialist,” she explained.
“The case involving Billy Burke was on the radio earlier that morning. Patsy came in and sat beside me. Hannah was asleep at the time. He got upset and I got upset. I just remember saying that we would be fine.
“My mother and sister came into the hospital to give us some support. Hannah stayed in hospital for two weeks, her pneumonia was sorted out and she was put on medication. With cystic fibrosis, you take one day at a time. You have to make things work and make the best out of situations,” she recalled.
Following the birth of their son, Michael, he was given the all clear from the disease.
The diagnosis has completely changed their lives but they continue to make the best out of a very difficult situation. It’s impossible to plan holidays or breaks away because Hannah can get sick at any time.
Over the last 12 months, Hannah has only been able to attend 104 out of the 183 days during the school year because of sickness.
“We do our best to make life as normal as possible. We don’t want to wrap her in cotton wool. Hannah loves school, playing with her friends and engaging in all types of activities.
“She loves swimming and is a member of the pool in the Auburn Lodge Hotel, Ennis. We get private lessons at a quiet time. Hannah is like any girl her age. She loves handbags, make-up and dressing up. She is very socially adept with people.
“Hannah has accepted her condition and deals with it very well. She displays a lot of maturity for a six-year-old. When she was nearly four, she asked me ‘why am I always sick Mammy?’ which was hard. We are very open with her and tell her what we are doing, we have never hidden anything.
“All the teachers and staff in the Gaelscoil have been hugely supportive of her condition and have been quick to volunteer for home tuition.
“All her friends know she has to take medication and I remember Michael saying last year ‘Hannah is very sick, she has a shadow on her lung’,” she said.
Commenting on Hannah and CF, Deborah says, “Cystic fibrosis has a very serious impact on her life and she knows she will have it until she is dead”.
Deborah decided to speak publicly about Hannah’s illness to raise public awareness about the condition and to help the regional drive to raise €3.5 million for a specialised Cystic Fibrosis Outpatient and InPatient Unit at the Mid-Western Regional Hospital, Limerick.
“We have done a lot of fundraising privately to raise money for the charity and now we are involved in the branch. A lot of good things are being done to improve the plight of cystic fibrosis sufferers. For the first time, 56% of the cystic fibrosis population are over 16 and they will require adult services,” she said.
Hannah’s diagnosis meant that Deborah effectively had to become a nurse and learned to administer oral and intravenous antibiotics and other medicines in an effort to keep Hannah out of hospital as much as possible.
While there are benefits for Hannah to be treated in hospital, Deborah feels her quality of life is very important and tries to treat her at home as much as possible.
Hannah’s normal day begins with several nebulised drugs, then physio to clear the lungs. She then takes her enzymes with her breakfast, her vitamins after her food and also medicine for her liver. Throughout the day, it’s enzymes any time she eats, then it’s more nebulisers and physio before bed.
When she becomes sick, there are oral antibiotics to be taken, extra nebulisers, extra physio sessions.
It is only when the oral medicines do not work, that the IVs start. They last for approximately two to three weeks at a time. Then it is back to enjoying the weeks when she is well until the next round starts.
Deborah is very quick to stress that, while it is hard, the family know that this is the way their life is and they are okay with that.
Deborah and her family thanked everyone who has ever helped with their fundraising efforts and would encourage future support for the branch’s fundraising group TLC4CF.
Hannah will also be featured in a documentary called Donation Nation, which will be aired on November 5, 12 and 19. This programme will highlight the importance of organ donors.