Cystic Fibrosis

Facts based on the information given on  http://www.cftrust.org.uk/aboutcf/whatiscf/

What is Cystic Fibrosis? Basic Facts

· Cystic Fibrosis (CF) is one of the UK's most common life-threatening inherited diseases.

· Cystic Fibrosis affects over 9,000 people in the UK.

· Over 95% of the UK CF population is Caucasian, but CF affects many ethnic groups.

· Over two million people in the UK carry the faulty gene that causes Cystic Fibrosis - around 1 in 25 of the population.

· If two carriers have a child, the baby has a 1 in 4 chance of having Cystic Fibrosis.

· Cystic Fibrosis affects the internal organs, especially the lungs and digestive system, by clogging them with thick sticky mucus. This makes it hard to breathe and digest food.

· Each week, five babies are born with Cystic Fibrosis.

· Each week, two young lives are lost to Cystic Fibrosis.

Currently half of the CF population will live past 41 years of age, and improvements in treatments mean a baby born today is expected to live even longer.

What is the cause?

Cystic Fibrosis is caused by a singular faulty gene that controls the movement of salt within the body. Cystic Fibrosis causes the internal organs to become clogged with thick, sticky mucus resulting in infections and inflammation making it difficult to breathe and digest food. Both parents must be carriers of the faulty CF gene for the baby were to be born with it. There is a 1 in 4 chance that the child will not contain the virus, 2 in 4 chance of being a carrier and 1 in 4 chance of having CF genes when both parents carry the gene.

Symptoms

The symptoms of Cystic Fibrosis can include a troublesome cough, regular chest infections, prolonged diarrhoea and poor weight gain. The organs affected by CF are the lungs, digestive system, other already affected organs.

Treatments

Treatments for Cystic Fibrosis includes physiotherapy which is important as it helps prevent the thick mucus from blocking the air tubes which helps reduce infection and prevent lung damage.  Physio is often needed daily for around 15 however if there are many secretions then I can take up to 45mins to 1 hour.

Nutrition is important as with a damaged digestive the wrong things should not be eaten. The diet must be suitable, gaining a good body weight can help fight chest infections.

Exercising well is an important part of the treatment process. Exercise prevents deterioration of the lungs and improves physical bulk and strength. The ideal exercise would be making you out of breath, it helps the spine, ribcage and shoulders remain fully flexible and a good posture should be maintained.

Medication for cystic fibrosis includes bronchodilator which can be administered through the lungs using nebulisers taken orally or injected, this treats the lungs open the airways by relaxing the muscles relieving tightness and shortness of breath. Antibiotics helps prevent infection, steroids reduce inflammation in the airways and DNase breaks down mucus making it easier to clear.


Double lung transplant
A double lung transplant can cost up to and more than £300,000. It can be a lot more affective than a single lung transplant as its possible there will still be cystic fibrosis in other lung however it is more risky as it must be 100% successful to work. The lungs will come from a body that has recently died therefore it can be at any time of day in which the sufferer of Cystic Fibrosis will be called to come into hospital. The lung transplant is a daunting thought as they are vital organs which help you breathe and it is hard to imagine what it would be like not able to breathe correctly. This is a main focus and most interesting area of the Cystic Fibrosis process i believe, therefore discussing it with a survive of the transplant will be very real and interesting to hear their story.