Wednesday, August 24, 2011

Cystic Fibrosis steals simple joys

By John Maslin Wanganui Chronicle, New Zealand
Cystic fibrosis (also known as CF or mucoviscidosis) is a genetic disease which affects the entire body, causing progressive disability and often early death. However, the prognosis has improved. In 1970, children with CF were lucky to reach 19. In the US, the life expectancy for infants born in 2008 with CF was 37.4 years. John Maslin meets three people with an intimate knowledge of cystic fibrosis
Whenever they hold a cystic fibrosis conference, any of those with the disease who want to attend have to get a medical clearance before they can go.

And meeting old friends is usually restricted to an "air hug" for fear of transmitting an infection.

Welcome to the world of the cystic fibrosis sufferer.

Erin Bartrum is a bright, ebullient woman who celebrated her 42nd birthday on August 19. Born with CF, her life expectancy was dismal, but she has beaten the odds and received a double lung transplant in January last year.

From living like an invalid, where every action was a struggle because her breathing couldn't cope, Mrs Bartrum is doing the normal things a house-proud woman does.

CF is an insidious killer, with the average carrier having a life expectancy of about 40 years. When Mrs Bartrum was born, 41 years ago, that life expectancy rarely exceeded 19 years.

Her younger brother had CF too, but he died aged 10, so Mrs Bartrum knows her transplant was a lifesaver.

For those with CF, hospitals can become second homes, while physiotherapy and medication are daily necessities.

Before her lung transplant, Mrs Bartrum's life was a living hell. Breathing was such a struggle that she had problems doing something as simple as eating.

"Think of trying to eat while you're running and you get the idea," she said.

Her transplant was not without complications, and instead of being out of hospital in two or three weeks, she was there for about four months.

"The lung transplant was good from the start, but my stomach packed up and then they found a bowel cancer and that meant another six operations."

One in 25 New Zealanders carry the CF gene, but it's often not until they have children that they know that.

Both parents have to carry the gene before it can be transmitted to their offspring.

Mrs Bartrum's mother, Madeline McCrea, said being born with CF meant her daughter didn't gain weight until she was 9 weeks old.

"She just couldn't absorb any food," she said.

It was after Erin's birth that she and her late husband discovered they both carried the CF gene, which was being transmitted to their children.

That's why Mrs McCrea joined her daughter and another mother, Anna Scoullar-Jones, in helping with CF Awareness Week in Wanganui from August 15-21.

The theme of the week was bubbles.

"It takes breath to blow bubbles and some of these CF kids just don't have the breath to blow bubbles," Mrs Bartrum said.

Ms Scoullar-Jones' son Lockie, 11 - like most other sufferers - was diagnosed with CF at birth.

For Lockie and others afflicted with this debilitating disease, diagnoses and treatment have come a long way.

"The lifespan is increasing, largely through medical research and treatment," Ms Scoullar-Jones said.

Lockie has physiotherapy about 20 minutes every day to ease his congested lungs, but other than that he lives life as most 11-year-old boys do.

"He occasionally has problems with his digestion and the occasional chest infection and that will require hospitalisation."

But Ms Scoullar-Jones said the disease could still claim people at a very young age. "When Lockie was born, they told us that time would tell how long he would live."

After her transplant, Mrs Bartrum was shown her old lungs. Only about 20 per cent of both lungs was functioning properly.

With that sort of incapacity, day-to-day routines were a real struggle. Mrs Bartrum was on oxygen 16 hours a day.

Now, with her new lungs, she's working part-time, her appetite is back (it's "impressive", according to her mum) and she does usual around-the-home tasks like the laundry and vacuuming.

"To see her do her own housework, gardening, things like that, is a joy to watch from my point of view," Mrs McCrea said.

"It's about being normal," her daughter agreed.

Cystic fibrosis is a progressive, life-long condition in which the glands that produce mucus, sweat and intestinal secretions do not function properly.

Thick mucus accumulates in the lungs, leading to breathing difficulties and infections. The severity of the disease varies.

There is no cure for CF, but treatments are available to help improve quality of life.
Although some people with CF still die in infancy, many live into their 30s or older.

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