Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis is defined as an autosomal recessive genetic disorder. Cystic fibrosis is a progressive disease that causes ongoing infection to the lungs which limits the ability to breathe over time.

What causes cystic fibrosis?

Cystic Fibrosis is caused by a mutation to the cystic fibrosis transmembrane regulator gene. The protein of the function cystic fibrosis transmembrane regulator gene is involved in regulating sodium and chloride transport across epithelial cells. Therefore, mutations in this gene affect the transportation of chloride across cell walls of the epithelial cells on the surface of the mucosa throughout the body. This results in thick secretions in the gastrointestinal tract, respiratory tract, pancreas and other glands which give rise to difficulties transporting nutrients and gasses across membranes i.e. eating and breathing difficulties are often present.

How can Bites Dietitians help?

A diet high in energy, fat and supplementation of vitamins and minerals is usually put together for you based on the foods you enjoy. High energy diets are usually recommended for clients with cystic fibrosis due to the high energy demand caused by chronic inflammation and high nutrient demands caused by chronic malabsorption. Bites dietitians will work to ensure that your diet is meeting all your nutritional needs to maintain your optimal health. We will also work with your GP and specialists to ensure that micronutrients are at sufficient levels and give you guidance on how to improve your intake of these if necessary. Some key nutrients that are poorly absorbed include calcium, iron and magnesium and if not corrected, can result in low bone density, iron deficiency and hypomagnesemia. Bites Dietitians will be able to ensure that all nutrient requirements are being met through diet or supplementation to delay and lower the risk of these deficiencies. If you have an enteral feeding tube or nasogastric feeding tube inserted we can also calculate and guide you on feeding regimes.

An interesting fact about Diet and Cystic Fibrosis

Even though eating may be the last thing you feel like doing when you’ve got a respiratory infection, not consuming adequate energy can mean your body finds it harder to fight infection and takes a longer time to recover.

How can Bites Exercise Physiologists help?

Exercise is an important aspect for overall health and wellbeing. It is important for individuals including people with cystic fibrosis to stay active. Physical activity has many benefits including increased pulmonary function and overall higher quality of life compared to individuals who do not exercise. Individuals that exercise are more likely to cope better with the impact of cystic fibrosis as well as having fewer infections. The benefits of exercise include:

improvements in lung function and capacity
increased oxygen capacity
improved clearance of airways
reduced risk of cardiovascular disease
increased muscle mass
improved overall quality of life.

Our exercise physiologists are trained in monitoring respiratory and cardiovascular symptoms during exercise and can prescribe you a safe, fun and effective exercise routine for you.

How will you know if it’s working/you’re improving?

As a result of following the exercise plans prescribed to you, improvements in lung capacity/lung function will likely be evident within 6-12 weeks of consistently performing your exercises. Increased muscle mass can be observed within 6-8 weeks of following Bites dietary and exercise plans. Following a structured diet and exercise plan will also likely mean that the frequency of hospital admissions may reduce as your body is clearing secretions, fuelling better day to day and is less susceptible to infections. Improvements in weight will be evident within 2-6 weeks, if weight gain is a point of concern.

Helpful things to bring/remember before your first appointment

Referral
Pathology test results eg. blood tests revealing iron, glucose and status of other minerals
Respiratory measurements from your respiratory physician such as FEV1 and other lung function tests
Any discharge paperwork from your last hospital admission.

Here’s A Cystic Fibrosis Friendly
Recipe That You Might Like To Try

CHECK OUT OUR FREE RECIPES

Contact Details

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305/29–31 Lexington Drive, Bella Vista NSW 2153

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0452 458 146

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