What is Cystic Fibrosis?

Cystic fibrosis (CF) is a hereditary condition affecting around 11,000 people living in the UK. CF is genetic - this means people who have it were born with it, and you cannot simply catch it later in life. However, 1 in 25 of us carries the faulty gene that causes CF, often without knowing. If both parents carry the faulty gene there is a 25% chance that their child will have CF.

How are people diagnosed with Cystic Fibrosis?

These days, every newborn baby in the UK receives a number of tests after birth to help maternity staff identify any problems sooner rather than later. One such test is a heel-prick blood test, which allows healthcare professionals to spot the most common genetic faults that can cause cystic fibrosis.

Another sign that can help healthcare professionals to identify babies who might have cystic fibrosis is a bowel obstruction known as meconium ileus.

Despite these advances, it is still possible for children to be diagnosed with CF later in life. This is typical among those born earlier than 2007, those who weren’t screened at birth for any other reason, or those who present with less common mutations of the CF gene. Usually, these individuals receive a diagnosis much later, once they have begun to show symptoms of cystic fibrosis and become unwell.

How is Cystic Fibrosis treated?

It is vital that people with CF receive appropriate treatments to enable them to live longer, healthier lives. That treatment can take many different forms:            

Medication, physiotherapy, nutrition and exercise all play their part. There are also four precision medicines currently available that tackle the root cause of CF, known as CFTR modulators. Medicine to benefit the lungs can be taken by nebuliser (inhaled), orally or intravenously (into the veins).

When inhaling nebulised medicines it is important that the nebuliser used is efficient so that a high percentage of the drug is delivered into the lungs. The eFlow®rapid electronic nebuliser from PARI is not only efficient but also very quick, drastically reducing treatment times compared to conventional nebulisers. This nebuliser is used by most of the CF Centres in the UK.

Inhaled medicines include:

  • Bronchodilator medicines to open the airways by relaxing the surrounding muscles, relieving tightness and shortness of breath. Bronchodilators may also be used just before a physiotherapy session to open the airways and help with clearance. 
  • Antibiotics to treat or control persistent infection. People with CF tend to be more susceptible to an infection called Pseudomonas aeruginosa, which can badly affect their lung function over time. 
  • Inhaled Mucolytics such as DNase to break down mucus, making it easier to clear from the lungs. 
  • Inhaled Hypertonic Saline such as MucoClear 6% (another type of mucolytic). Hypertonic saline is available in different concentrations and can help to thin and loosen mucus. 

Physiotherapy can be performed daily to help clear the lungs of thick, sticky mucus. There are many different techniques and types of physio equipment, including: 

  • Active Cycle of Breathing Techniques, or ACBT
  • Autogenic drainage
  • Positive Expiratory Pressure (PEP) such as the PARI PEP S
  • Oscillating PEP therapy such as the PARI O-PEP

Often, people with cystic fibrosis require a diligent routine of various treatments all working together to help them stay healthy. This might include the taking of injected or inhaled medicines which aim to make the mucus thinner and help the immune system fight off infections; taking enzyme pills which help them to digest their food more easily; and continued physiotherapy sessions throughout the day.

It’s also the case that some people with CF may need to undergo an organ transplant. As it is a condition that primarily affects the lungs, people with CF are more likely to need a lung transplant than any other type. On the other hand, the condition can affect other organs such as the liver, kidneys or pancreas, and so these transplants may also be necessary.

There are a few charities in the UK offering support for those with CF, including:

Cystic Fibrosis Trust - in operation since 1964, funding vital research and publishing a wealth of information for those living with CF.

Cystic Fibrosis Care - providing grants, emotional support and raising funds to support CF units and their patients.




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