``Naturally it makes everyone think of their own situation,'' says Nicola Wakerley, chairperson of the Cystic Fibrosis Association Bay of Plenty Branch.
Mrs Wakerley, who has two children with cystic fibrosis (CF) is talking about the recent passing of one of their member's sons, nine-and-a-half-year-old William Leitch. William died at Tauranga Hospital on January 26.
For a branch comprising just 12 families, it is a difficult time.
``We're a close group, and it's pretty hard. Our core group were all at the funeral, along with all the hospital staff we deal with. It's difficult.
``We all knew William's journey. How well he was up to a certain time, and you do, you look at your own kids.
``William was really well until he was 7 years old, and then things went downhill for him. So you do worry about it. But you just have to do what you do and keep going.''
Mrs Wakerley and husband Todd, a youth pastor at Otumoetai Baptist Church, have three children: Jemma, 8, and Matthew, 5, both of whom have CF and 10-year-old Sam, who does not.
She is keen to accentuate the positives of ever improving life expectancies for CF sufferers.
``It's a life-limiting disease. We haven't got New Zealand data yet but in America the average age is 38. It used to be a childhood disease but now we're at the point where in New Zealand we've got more adults, over 18s, with CF than children for the first time ever. That happened last year.
``That's really encouraging. They are growing up and having kids of their own now. They're living full lives, they're really well and they're doing very well.
``We hope one day they're going to come up with a cure, of course. There are some very good drugs which have been developed in the US and England, and are in their phase-three trials, and it's looking really promising.
``However, in New Zealand it will probably be something like 10-plus years away before we can even think about it.''
CF is a genetic disorder whereby the body makes thick mucus which congests the lungs. Sufferers get more infections, which narrow airways and can block the pancreas, preventing enzymes breaking down food. It affects other organs to a lesser extent, but over time the effects build up.
Mrs Wakerley is stoic, but the strain of coping with the additional daily routines and pressures necessitated by the disorder shows in her face.
``Life's busy,'' says the Bethlehem pharmacist without an ounce of self-pity in her voice. ``They have to do things every day to keep themselves well. Both my children, their pancreas doesn't work. So that means everything they eat, they have to take enzymes with it so they can break down the food.
``The enzymes are worked out based on the percentage of fat in the food. So you have to calculate it every time you eat. It becomes second nature. I can't look at food now without knowing the percentage of fat in it.
``They have high fat, high salt diets because they lose a lot of salt. The disorder is based around the chloride transfer in and out of cells. And high fat because they require more energy due to not always breaking their food down as well.''
That is just the start.
``They do physiotherapy in an ideal world twice a day.
``When they were younger it used to be percussion on their back. Thumping on their back in time to remove the mucus. Now they're older we do it with a nebuliser and they blow against a little device called a `pep'. You have to count the amount of breaths in and out. We do that twice a day, morning and night.
``Exercise is very important because it keeps their lungs clearer. So they do swimming lessons, and Jemma does dance and volleyball.''
CF kids pick up bugs more easily and the effects are more severe.
``They get infections in their lungs faster than we will because the mucus in there grabs the bacteria, keeps it there and grows it.
``It makes you a lot more wary about hygiene. A lot of the kids carry round hand sanitisers and we try to encourage ours to stay away from kids who are sick. You can't hide them from everything, though.
``If they're unwell we'll go up to Tauranga Hospital to do a cough suction, see what they're growing and then treat them. So they do antibiotics a lot, more in winter. If it's bad enough they will do intravenous antibiotics, which is a two-week stay in hospital.
``We've just finished a three-month course of antibiotics for them, which is nebulised antibiotics. That takes 20 minutes, twice a day. That's tagged on to their physio as well, of course, so we have some arguments about that,'' she says raising her eyebrows.
``Physio is often a fight. It's doing something which is boring and it's not what they want to be doing. It takes time. We can get it over and done with in 10 minutes but for them it feels like forever.''
The routines leave the children more tired than others their age.
``They're exhausted when they come home, but you just have to look at it that that's what life is. That's what we have to do. It just means routines.''
Jemma and Matthew go to a clinic at Tauranga Hospital every three months for blood and lung function tests, chest X-rays, and to see the physio, dietician and paediatrician. They attend Starship Hospital once a year for similar tests and any others deemed necessary.
As if parents with CF children did not have enough to contend with, there is the added burden of fundraising.
``One of the hardest things is that you've got this condition you have to deal with and all these extra time factors, and then you've got to be part of a branch and go out and fundraise for it. Because nobody else is going to do it for you.
``You don't have big organisations behind it.''
It is the reason events such as the BOP Times/Bethlehem Foundation auction, on Saturday June 8 at the Tauranga Racecourse, are so important, said CFANZ CEO Kate Russell.
``We are so thrilled to be a part of this iconic Tauranga event. The great thing is we are partnering with a much loved Bay of Plenty charity but also benefiting those living with CF... ``This money will go a long way to assisting us to maintain our national Breath4CF fund.''
Facts on Cystic Fibrosis (CF)
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The Cystic Fibrosis Association of New Zealand (CFANZ) provides support, counselling, education, lobbying for new medications and financial assistance and has been working since 1968 to provide a better quality of life for all people with CF. -
It means lots of pills, needles, time in hospital and isolation for the people who have it and their families. -
Affects the lungs, pancreas and other organs of the body, leading to frequent and serious chest infections, an inability to digest food and an abbreviated life. -
o get CF a child must receive a copy of the gene from both mother and father. -
Caused by a recessive gene which is carried by one in 25 people. T -
A hereditary (genetic) disease that affects 450 New Zealanders. -
It also supports research into CF both here in New Zealand and overseas, through funding and participation in medications trials.
Source: CFANZ
Charity auction
The seventh BOP Times/Bethlehem Foundation Auction will be held on Saturday June 8 at the Tauranga Racecourse.
Bookings for tables of 10 at $1,500+GST for the black-tie dinner can be made by calling (07) 579 1816 or visiting bethlehemfoundation.org.nz. Event proceeds will be shared equally between the Cystic Fibrosis and Bethlehem Foundations.
