Cystic fibrosis is permanent inherited health condition that affects close to 4,000 people across Australia. This may not seem like such a high number. However, many more can be carriers of the gene. Living with cystic fibrosis can cause lung, digestive and at times, dexterity issues from conditions like finger clubbing.

In this article Blue Badge Insurance looks at some common cystic fibrosis questions to unpack more about this health condition.

What is cystic fibrosis?

Cystic fibrosis can affect several of a person’s organs, clogging them and leading to infection. It can affect the lungs, intestines and pancreas but can also lead to other health complications like finger clubbing (more on this further down).

The mucus (water and salt) that flow through the cells in our bodies gets thicker than is healthy. As you can imagine, this can lead to lung infections, blocked sinuses and all sorts of breathing difficulty. But it also causes a deeper issue.

Instead of flowing through the cells, the thick mucus (which is more salty and less watery) begins blocking organs like our lungs, pancreas and intestines. Cystic fibrosis can affect everyone to varying degrees and can be life threatening.

How is cystic fibrosis inherited?

Cystic fibrosis is a genetic disease which a person inherits if both parents carry a mutated genetic mutation that causes it. If just one parent has the gene the child won’t be affected, however, they will still carry the gene.

Each of us carries the cystic fibrosis transmembrane conductance regulator (CFTR) gene. That means each of us is born with two of these, since we inherit one from each parent. Genetic mutations to this gene can cause cystic fibrosis – but again, only if there’s a mutation on both inherited genes

One in 25 people are carriers of the genetic mutation that causes the disease.

This is why, when two people who are genetic carriers have a child, their child can be born with cystic fibrosis even if they don’t live with the condition themselves. There are over 2000 possible ways the CFTR gene can mutate.

This means that every one living with Cystic fibrosis may experience different symptoms and severity. For example, finger clubbing is a symptom some, but not all experience.

What is the life expectancy with cystic fibrosis?

Cystic fibrosis can shorten a person’s life since breathing problems can affect their ability to stay healthy and lung disease can eventually be life threatening.

Today there are many treatments available to help manage symptoms, although as yet, no cure has been discovered. While people of today have a better prognosis than in decades past, the average lifespan of a person living with cystic fibrosis is around 50 years.

Can people with cystic fibrosis have a normal life?

Some people grow live longer, reaching well into senior years. This can depend on the severity of the disease, lifestyle choices and treatment management plans.

Treatment and management

Here are some ways you can help manage cystic fibrosis:

• Medication. These include antibiotics for infection treatment, and / or prevention, inhalers and mucus thinning medications. There are also medicines that target the genetic mutation itself.
• Therapy. Oxygen therapy, chest physiotherapy, postural drainage and devices that help open or clear airways such as a nebuliser.
• Exercise. Activities that help you breathe deeply can help improve the circulation of oxygen. Flow sports like swimming and cycling are good examples. Exercising roughly three times weekly can be helpful.
• Diet. Since the bowel can be affected, it’s important to speak to your health practitioner about protein and calorie intake which should normally be higher as a child grows older and for adults. Also drink lots of water. Added to this, several vitamins are important. Speak to your health practitioner about doses and directions for vitamin supplements and pancreatic enzymes.
• Surgery. In some cases lung transplant has helped patients live healthier, fuller lives.

As with other health conditions that affect breathing, like asthma, it’s important to keep the home dust and smoke free to avoid unnecessary irritants.

A new treatment in Australia

In addition to the above treatments, there’s a newly developed inhaled antibiotic treatment called RSP-1502, which was developed in Western Australia. This innovative treatment was created by the Telethon Kids Institute and Perth Children’s Hospital.

This treatment is currently being tested in Australia and the U.S. and the results look promising.

Trials show 800 times reduced bacteria and 16% increase in lung function compared to 5% in standard care for chronic patients.

The next stage of trial will be looking at the safety of this new treatment option, which could help prevent lung infection and improve quality of life and life expectancy.

What are 5 symptoms of cystic fibrosis?

The symptoms of cystic fibrosis vary both in severity and in range. That’s because of how many variations on the genetic mutation to the CFTR gene exist.

Although the effects of cystic fibrosis van be varied, extreme and life threatening, they’re not always evidently visible. That’s why it’s sometimes considered an invisible disability.

Here are five symptoms cystic fibrosis can cause:

• Breathing problems
• Chronic lung disease
• Delayed growth
• Respiratory infections
• Malnutrition

However, the above is not by any means an exhaustive list and people can experiences changes in what and how they experience symptoms as they age. For example, finger clubbing and pancreatitis are more commonly experienced later in life.

Let’s look more closely at how cystic fibrosis can affect different areas of the body:

Respiratory system

People with cystic fibrosis often experience symptoms that affect their lungs and sinuses. This can affect their overall quality of life and can become life threatening. Symptoms can include persistent coughing and increased mucus production in the lungs and sinuses, leading to fatigue from pain and exhaustion.

Nasal polyps is another symptom which can cause symptoms like pressure, pain and congestion of the sinuses. Frequent episodes of pneumonia are also typical. These can be characterized by fever, intense coughing, shortness of breath and increased mucus production which also commonly leads to a loss of appetite.

Digestive system

Cystic fibrosis routinely affects the bowel causing symptoms like severe constipation, nausea, increased gas, bloating, or a noticeably swollen belly. Repeated bouts of constipation can lead to rectal prolapse.

Finger clubbing

Cystic fibrosis is commonly thought of as affecting the lungs and digestive system, but it can also affect movement. One way this can happen, often later in life, is finger clubbing.

This is when the oxygen circulation to the extremities (or ends or tips) of the body – those that are furthest from the heart – such as fingers and toes swell. They specifically swell at the tips affecting the nail and nailbed which can get soft and misshapen. This can be really painful.

While there’s no known treatment specifically for finger clubbing, the route is poor lung function caused by the disease. So in some cases, once the lung function improves the finger clubbing improves too.

Limited mobility

Although there’s often less focus on finger clubbing, joint pain, grip strength and muscular responsiveness – these are also symptoms people may experience.

Each of these can make it harder to move around with ease They can make it more difficult to open doors, food containers, medication and lots of everyday actions that are required at home, work or school.

Limited mobility affects thousands of Australians and for many, using a wheelchair, mobility scooter or other mobility aids like car modifications or Assistance Dogs can help. Blue Badge Insurance protects mobility equipment with quality, well-priced insurance.

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