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Cystic Fibrosis: A useful guide

• Written by Josh

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Cystic fibrosis is a genetic condition which effects your lungs and digestive system. According to the Cystic Fibrosis Trust (CFT), the condition currently affects more than 10,400 people in the United Kingdom.

It is a medical condition which you are born with, meaning that if you are not born with cystic fibrosis you'll never have the condition. It is a condition that many of you may not have a clear understanding of, so today's article will explain what Cystic Fibrosis is, how it affects those diagnosed and the treatments available.

What is Cystic Fibrosis?

Cystic fibrosis is an inherited condition which is passed on from each parent to their child because of a faulty gene. To be born with cystic fibrosis, a child has to inherit two copies of this faulty gene – one from each of their parents. Their parents will not usually have the condition themselves, because they will only carry one faulty gene and one that works normally.

The gene in question means that some body cells struggle to move salt and water across the cell wall. This then leads to a build-up of thick mucus within the body's vital pathways and tubes - such as the lungs and digestive system.

If both parents carry the faulty gene, there's a 25% chance that each child they have will be born with cystic fibrosis. - NHS

According to the Cystic Fibrosis Trust there are five babies born each week with cystic fibrosis, whilst two people die each week. Cystic fibrosis is a life-limiting condition which means that the median survival rate for somebody with the condition is just 41 - depending on the severity of their condition. Some people may live beyond this due to treatment, whilst others who have a severe case may not make it past childhood.

Diagnosis

There are tests that can be carried out to diagnose cystic fibrosis at any age, however the majority of cases are detected soon after birth through a newborn blood spot test. This is offered to all babies in England to help detect any health problems as early as possible.

The blood spot test involves a doctor taking blood samples from the heel of the baby and sending them off to be checked. Most results come back by the time the baby reaches the age of eight weeks. Of course, if a problem is found the parents will be contacted much sooner than this.

The screening result isn't 100% accurate, so more tests will be carried out to confirm the diagnosis. This may involve both parents being tested alongside their baby. The two main tests used are:

  1. Sweat test - Measures the amount of salt in sweat. Somebody with the condition will have higher levels than normal.
  2. Genetic test - A sample of blood or saliva is checked for the faulty gene.

Anybody who fears that they might have the faulty gene can have a carrier test. This is particularly recommended if you have a close relative who is a known carrier or has cystic fibrosis, or if your partner is known to carry the gene.  This test can be done using a blood test, or sometimes by using a special type of mouthwash to collect cell samples.

Finding out whether you're a carrier of the faulty gene can help determine if you're at risk of having a child with cystic fibrosis.

Symptoms

As mentioned previously, cystic fibrosis affects the lungs and digestive system. Symptoms may begin in early childhood, although they can occasionally appear soon after birth or not until adulthood.

The condition causes your lungs and airways to become clogged with a sticky mucus. This will continually cause damage to the lungs and they may eventually stop working correctly. Other issues caused by your lung problems include:

  • Coughing.
  • Occasional wheezing and shortness of breath.
  • Recurring chest infections.
  • Persistent inflammation of the airways.
  • Being highly susceptible to lung infections.

This mucus also blocks part of the digestive system, which has a knock-on effect on the way food travels through the gut and how it's broken down or absorbed. Issues caused by this can include:

  • Problems putting on weight and growing, due to the body struggling to digest and absorb nutrients.
  • Serious bowel obstruction in the first few days of life.
  • Diarrhoea.
  • Jaundice (the yellowing of the skin and whites of the eyes).
  • Diabetes, if the pancreas become severely damaged.

Alongside these common affects and symptoms, having cystic fibrosis can cause a number of other problems as well. These can include the swelling and pain of your joints from late childhood (arthritis), thin and weakened bones (osteoporosis) and liver problems caused by the tiny bile ducts in the liver becoming blocked by mucus.

Treatment

Unfortunately there is currently no cure for cystic fibrosis. Through research funded through charities and other methods, there are certain treatments that can help to make the condition easier to live with.

People with the condition can take various different types of medication to try and manage all of the symptoms that come with the condition. For example, medicines used to help the person's lungs include:

  • Antibiotics - To treat or control persistent infections.
  • Steroids - To reduce inflammation in the airways.
  • Mucolytics - To help break down mucus, making it easier to clear from the lungs.
  • Bronchodilator pills - To help open your airways by relaxing the surrounding muscles.

It is also important for those affected by the condition to try and perfect special breathing techniques to try and keep their airways and lungs as clear as possible. There are a few techniques that have been recommended by the NHS:

  • Active Cycle of Breathing Techniques - Involves a sequence of relaxed breathing, followed by deep breath exercises and then huffing.
  • Autogenic Drainage - A series of gentle breathing techniques which helps to clear mucus from the lungs.
  • Modified Postural Drainage - Involves changing your position to make it easier to remove mucus from the lungs.
  • Airway Clearance Devices - These are handheld devices with use vibration and air pressure to help remove mucus from your airways.

As mentioned, those who have cystic fibrosis have to stick to a special diet or, in some cases, be fed through a tube at night. For those who aren't reliant on a feeding tube, it is advised that they visit a dietitian to ensure they have a nutritional diet. The diet should be high in energy (calories) and rich in fat and protein to help compensate for the amount wasted as food is not fully digested.

A common suggestion is for people to take digestive enzyme capsules with all their meals and snacks to help support their digestive system. Vitamins and mineral supplements are also advised.

If a patient's condition becomes severe then it may be possible for he or she to have a lung transplant. This operation does have some risks, including rejection or infection, but it can lead to several improvements for the patient and can extend their life expectancy.

It's estimated that up to 9 out of 10 people will survive for at least a year after a transplant. - NHS

Currently there is a shortage of suitable donor organs, with a third of all those on the waiting list for a lung transplant likely to die before they receive a donor lung. You can register to donate on the Organ Donation website

Living with Cystic Fibrosis

Cystic fibrosis can make it extremely hard for a person to complete regular every day tasks. As the lungs are directly effected it means those with the condition can become breathless quite quickly when doing simple things such as walking up the stairs. A wheelchair and oxygen may be required on a daily basis.

Some people also have to follow a certain diet, with many having to use a food tube each night. Daily routines would also involve taking various antibiotics and other medication in order to reduce the risk of infection, make the mucus in the lungs thinner and to make it easier to cough up.

In order to remain as healthy and fit as possible, those with Cystic Fibrosis also need to do certain exercises every day. Physical exercise is also often used as part of an airway clearance regime. Exercise can help loosen mucus in the lungs and make airway clearance techniques quicker and easier.

According to the CFT: "People with cystic fibrosis often require more than 50 tablets a day, plus a couple of hours of physiotherapy at home and some nebulised treatments." Despite all of these set-backs, More than 50% of adults with cystic fibrosis are in education or employment.

Those with the condition should also not make contact with others who have cystic fibrosis due to having different bacteria in their lungs. The risk of infection is extremely high between two people with the condition if they meet each other.

Of course everybody with the condition is unique and will face their own challenges and symptoms.

Cystic Fibrosis Trust

The CFT is the only UK-wide charity dedicated to providing practical support, research and advice to those affected by cystic fibrosis. Their mission statement is as follows:

Our mission is to create a world where being born with CF no longer means a life-long struggle, when everyone living with the condition will be able to look forward to a long, healthy life.

The charity also operates a helpline – 0300 373 1000 – which is available from 9am and 5pm, Monday to Friday.

VAT Exemption

If you have Cystic Fibrosis then you qualify for VAT Exemption when you a personal alarm system. HMRC state that a product which has been “designed or adapted for a disability” qualifies for VAT exemption.

For a person to qualify they must meet certain criteria set by HMRC. This criteria says that the customer must have a long-term illness, a terminal illness or a disability in order to qualify.

Personal Alarm Information

A personal alarm could be very useful for somebody affected by cystic fibrosis. Should they have any breathing problems or any other symptoms they can simply press the red button on their pendant and our Response Team will respond, before arranging for help.

For more information about our life-saving personal alarm service, please get in touch with our friendly team on 0800 999 0400. Alternatively, complete our contact us form and we will get back to you as soon as possible.

Remember to use the discount code BLOG2018 when you order one a personal alarm on a Monthly or Annual Plan to receive £10 off.

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