Every breath she takes

Just a taste of Charlotte Taylor's medication and equipment. Photo by Mikaela Turner
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Mikaela Turner reports on a brave teenager's endurance with a disease for which there is no cure and the average lifespan is just 37 years.

While most pre-schoolers can barely dress themselves, Charlotte Taylor was already independently managing her medications, swallowing pills before every meal.

Charlotte, now 17, has a terminal genetic illness called Cystic Fibrosis (CF).

Cystic Fibrosis is a disease which affects a persons mucus function. Missing or broken cells cause mucus to become dehydrated, resulting in thicker, stickier and immobile mucus.

For sufferers, the disease is without a community. People with CF must keep a three meter distance from each other in order to reduce the spreading of any viruses or bacteria.

This means Charlotte cannot have cannot have any friends with her condition. Unlike other illnesses, she cannot go through life in solidarity with others in her situation.

CF organisations run camps for siblings and events for parents, but CF sufferers must go it alone.

Charlotte Taylor begins her day with physical therapy, breathing in and out hypertonic saline through a nebuliser.

Due to the immobility of her mucus, anything she breathes in lives and grows in her lungs.

Therefore, physical therapy is needed to thin the mucus so it can travel into her throat and be coughed out, along with it, what’s been breathed in.

After physical therapy, she takes four to five tablets with breakfast. Without these, she would be unable to digest her food.

For a healthy person, enzymes produced in the pancreas move through mucus into the intestines. But because Charlotte’s mucus is immobile, the enzymes are trapped.

The tablets therefore replace enzymes missing from the intestines.

Even as a baby still being breastfed, Charlotte had to ingest enzymes through pureed apple.

“CF babies start eating solid food from the day they are born”, said her mother, Robyn.

After school, she goes through physical therapy again.

Then overnight, she is fed through a tube.

CF sufferers burn up food much faster due to the extra work their body does to simply breathe. Therefore, they must eat considerably more than an average person.

However, eating more means coughing more and increased coughing often leads to vomiting.

To negate this problem, at just six years old Charlotte went through a percutaneous endoscopic gastrostomy procedure so that she could be tube fed.

“It has been a lifesaver, metaphorically and literally,” said her mother.

Cystic Fibrosis greatly affects a person’s quality of life.

“I can barely run 10 meters without coughing.

“I get tired all the time because I don’t get enough oxygen to my brain”, said Charlotte.

While coaching gymnastics, Charlotte needs assistance because she cannot simply talk for two hours.

But as her mother said “you have to put your health first above school, work, anything else. It has to be the main focus”.

Charlotte spends at least two weeks of every school holidays in hospital.

“I’m there for most Easters”, said Charlotte.

Hospital is the Royal Children’s, a place where Charlotte finds community despite the stronger drugs making her feel “sick in the head”.

She is friends with the nurses, many of whom have watched Charlotte grow up.

However, Charlotte’s time at the Royal Children’s is coming to an end.

At 17, she is in transition clinic, preparing her for the move to an adult hospital.

“I don’t want to leave, I like the Children’s. I wish we could choose when we left, until we were 21 or something” Charlotte said.

However, the choice is out of her hands.

Cystic Fibrosis is a relatively rare disease. One in 2500 births produces a child with CF.

However, one in 25 are carriers of the gene, or as Mrs Taylor put it “one child in every classroom”.

If two people carry the gene and have a child, each pregnancy will have a one in four chance that the child will have CF.

A baby is born with CF every four days.

There is no cure for CF. Treatment only slows the progression of the disease. The average lifespan is 37 years.

Some sufferers are lucky enough to receive transplants, but not even a triple transplant (heart, lungs and liver) will cure CF.

Anywhere there is mucus, there is a problem. Therefore the nasal system, gastrointestinal system, reproductive system, liver, pancreas, lungs and more are all affected.

Transplants do however improve a sufferer’s quality of life.

Despite having to take anti-rejection tablets for the rest of your life, “a few pills are nothing compared to being unable to walk from your front door to your letterbox”, said Mrs Taylor.

At 13 Charlotte Taylor found out she also has diabetes. But not type 1 or type 2.

Interlinked with CF, she has a third type where her pancreas still produces insulin, but not enough.

She must therefore take long acting insulin to get enough for the whole day, as well check her blood sugar levels by finger pricking.

Many CF sufferers have been given hope in the form of a new revolutionary drug called Orkambi which claims to add 20 years to a patient’s lifespan.

Orkambi is yet to become available via the Pharmaceutical Benefits Scheme, much to the Cystic Fibrosis Australia’s frustration.

Charlotte, however, cannot even hope for this drug as it will not help her. It only assists those with the most common form of CF, a form she does not have.

The Taylor family instead hopes they may be able to create a more generic form of Orkambi which may assist sufferers with other rarer forms of CF.