What is Cystic Fibrosis?

What is cystic fibrosis?

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What is cystic fibrosis?

  • There is currently no cure for CF.

  • Currently, the average life expectancy of a person with CF at birth is 38 years.

  • CF is a genetically inherited condition that primarily affects the respiratory, digestive and reproductive systems.

  • Cystic fibrosis or CF is the most common life threatening condition affecting young Australians.

    What causes CF?

    What causes CF?

  • As a result of this mucus in the pancreas, some people with CF can develop CF related diabetes over their lifespan.

  • Some people with CF also have a build up of mucus in their pancreas and need to take enzymes (capsules) to help with digestion.

  • The build up of this thick, sticky mucus causes irreversible damage to the lungs.

  • CF is caused by an imbalance of salt and water in the cells, causing mucus to be thick and sticky.

    How common is CF?

  • 30 % live in regional areas.

  • As of December 2014, there are approximately 3,300 people living with CF in Australia.

  • In Australia, 1 in 2,500 babies are born with CF each year. That is 1 every 4 days.

    How do you get CF?

  • CF carrier screening is used to help identify whether a person is a carrier of the CF gene.

  • If both parents are carriers then there is a 1 in 4 (25%) chance of having a child with CF with each pregnancy.

  • To have a child with CF both parents must be carriers of the CF gene.

  • 1 in 25 (over 1 million) Australians are carriers of the CF gene, most are unaware.

    How do you get CF?

    How is CF diagnosed?

  • However, some people with CF are diagnosed during adolescence and adulthood.

  • CF is primarily diagnosed in the first few weeks of birth via newborn screening (Heal prick/Guthrie test).

    How does CF affect a person?

  • CF can affect a person’s mental and social health as well as their physical health.

  • CF requires daily and ongoing treatment to ensure the person maintains optimal health, including spending extended periods of time in hospital.

  • CF is an invisible condition, many children and adults with CF often look the same as others their age.

  • CF affects everyone very differently. Some people with CF are more severely affected by the condition than others.

    How do you treat CF?

    Parts of the body affected by CF

  • vas deferens (in males).
  • sweat glands
  • pancreas
  • lungs
  • CF mainly affects the:

    The effects of CF on the lungs

  • Due to ongoing damage to the lungs, some people with CF will need a lung transplant.

  • People with CF can become increasingly unwell from colds, flu and even gastro due to their compromised lungs.

  • The build up of thick, sticky mucus in the lungs of a person with CF can result in difficulty breathing and increased lung infections resulting in irreversible lung damage.

    Lung health and treatment

    Lung health and treatment

    Exercise

    Antibiotics

    Airway clearance or Physiotherapy

    For a person with CF to maintain their lung health the following treatments are required daily:

    The effects on the pancreas

  • Most people with CF have difficulty putting on weight and some are smaller in stature.

  • Because of a build up of mucus, some people with CF can develop CF related diabetes.

  • Approximately 85% of people with CF are not able to digest food properly, due to mucus blocking the pancreas.

    Pancreas health and treatment

    High calorie/fat/salty diet
    Enzyme replacement capsules (Creon)
    Vitamins

    For a person with CF to maintain their lung health the following treatments are required daily:

    How does CF affect the sweat glands?

  • Losing too much salt can cause dehydration and make the mucus in the lungs thicker and more difficult to clear, therefore harder to breathe.

  • People with CF lose 2 - 5 times more salt than the average person when sweating.

    Salt replacement for people with CF

  • Salt tablets or liquid: help replace salt lost and avoid dehydration.
  • Diet: often need 3 - 4 times more salt in their diet.
  • It is important for people with CF to ensure they are maintaining enough salt in their bodies and replace any salt lost.

    Other CF related complications

  • arthritis
  • reflux
  • sinusitis/nasal polyps
  • infertility for males
  • bowel obstructions
  • liver disease
  • CF related diabetes (CFRD)
  • osteoporosis and arthritis
  • People with CF are at risk of developing the following complications as they get older:

    CF and the environment

  • Other people with CF
  • Germs and bacteria
  • Infections
  • For people with CF there are potentially harmful factors in everyday environments that can lead to poor health and even hospitalisation.

    Hospital admissions for managing CF

    Hospital admissions for managing CF

  • Some people with CF can have a few admissions a year, while others may have no admissions.

  • Sometimes people with CF may need to be admitted to hospital for 1 - 3 weeks for a ‘tune-up’.

  • People with CF are required to attend regular clinic appointments (every 3 months) at their local CF treatment centre.

    Hospital admissions for managing CF

  • Such lines and ports can be left in place for weeks or months.

  • They may also need to undergo a procedure to insert a line or port to assist with the absorption of antibiotics and food.

  • During hospital admissions the person with CF will undergo a series of intensive treatments to clear the bugs in their lungs and increase their overall health.

    PICC lines, Ports and PEGS

  • PEGS: are used during cases of severe malnutrition or the inability to gain weight. It involves high calorie liquids being administered directly into the stomach. This usually will be done at home.

  • Port: is a small device placed beneath the skin and used for easy access for needles.

  • PICC line: inserted into the arm to assist with the delivery of antibiotics into the blood stream, reducing the need for needles.

    Some children with CF may have the following:

    Having a PEG

    The impacts of CF on mental health

    This could be due to reasons such as:

  • physical burden of care
  • decline in health and fear of uncertainty
  • phobias: swallowing tablets, needles and hospitalisation
  • body image
  • medical costs
  • Research indicates that people with CF are 2-3 times more likely to experience a mental health problem (depression or anxiety) than the normal population.

    The outcomes of mental health

    It is important for a person with CF to have good support systems in place to reduce the risk or impacts of mental illness.

  • poor adherence to medications and treatment, leading to more hospitalisation

  • risky behaviour

  • low motivation

  • lack of engagement in education

  • poor body image

  • lower self esteem

  • poor relationships

    Mental health problems can impact a person’s quality of life and result in :

    The outcomes of mental health

    The future for CF

  • There are now as many adults with CF as children.

  • Due to improvements in treatments and breakthroughs in research, people with CF are living much longer than ever before.

    The future for CF

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