My Family, Our Needs has teamed up with The Cystic Fibrosis Trust to find out more about the condition and what you need to know.
What is cystic fibrosis?
Cystic fibrosis (CF) is a life-shortening genetic condition, which causes the internal organs, especially the lungs and digestive system, to become clogged with thick sticky mucus, resulting in chronic infections and inflammation of the lungs.
People with cystic fibrosis often look perfectly healthy. But it’s a lifelong challenge involving a vast daily intake of drugs, time-consuming physiotherapy and isolation from others with the condition. It can place pressure on those around them and the condition can critically escalate at any moment. Currently half of those who die from CF are aged 28 or younger.
Cystic fibrosis diagnosis
Two million people in the UK are carrying the recessive gene that causes cystic fibrosis without realising it. You could be one of them.
If two carriers have children, there’s a one in four chance their child will have the condition. Babies are screened through the Newborn Screening programme for cystic fibrosis and this is usually diagnosed within three months of birth through the heel prick test.
It can be a very stressful time for parents finding out their newborn baby has cystic fibrosis and, as with any diagnosis, there is a lot to consider including managing medications and treatment regimes.
Characteristics of cystic fibrosis
Because CF affects so many areas of the body it may show up in different ways. Some babies are born with bowel problems or meconium Ileus (a blocked bowel at birth) which can be a sign of CF and will need urgent surgical care.
As a child with CF gets older, they often have a characteristic cough and may have clubbing of their fingers and toes. Otherwise they will look just like any other child.
How should I treat my child?
Your child’s paediatrician will be able to give you the specific treatment regime for your child as everyone’s Cystic Fibrosis is different. However, you and your child may have to work through daily physiotherapy to bring up mucus. This is important as the mucus often harbours bacteria that can lead to infection.
Other treatments include taking oral and nebulised medications and digestive enzymes to ensure that the child is able to digest their food. The Cystic Fibrosis Clinic will give you access to CF Physiotherapists and Dieticians. Although it all seems very scary at first, it soon becomes part of a daily routine.
Will my baby be healthy?
Every baby is different but a child with CF may be more susceptible to bugs and infection.
General development of children with cystic fibrosis
It is usual for children with CF to grow in the same way as other children providing treatments are going well. There is no learning disability associated with cystic fibrosis, but some children may appear smaller in stature. However, this is by no means always the case.
Can you catch CF?
No, it is inherited, you cannot catch the condition.
Isn’t CF the condition where parents have to pat their children on the back?
Many people see CF as only affecting the lungs and patients needing percussion (or chest and back patting). There are many new techniques to help people with CF to cough up their mucus, including air flow treatments, breathing techniques, bubble blowing and CF Vests. Percussion is an older technique but it is still used. However, CF doesn’t only affect the lungs; it affects nearly every part of the body.
Aren’t people with CF always sickly looking?
Most people with CF look healthy on the outside. Thanks to improvements in medication people with CF can live as they wish without it having too much of an impact.
Will my child go to school?
Yes, your child will go to school but may need to be visited by healthcare professionals whilst there. Parents usually work closely with the school to ensure that during meals and snack times the correct medicines are given and any other medications necessary are taken during the day.
You or other carers may have to come into school to help your child with daily physiotherapy. Nebuliser treatment may also be necessary.
The number of physiotherapy sessions that take place each day will vary according to your child’s current state of health. The length of each session may vary from 15 minutes to an hour.
Schools must be careful that your child does not come into contact with any other child with CF as this may cause cross-infection. You should check with the school to find out if there is another child in the school who has CF. You may need to explain to the school that they must avoid them coming into contact with each other.
Children with CF are as academically able as their peers, so teachers should expect similar standards, but hospitalisation or chest infections can result in prolonged absence from school. As such, extra help may be needed to catch up with the rest of the class. Examining boards can make certain allowances for pupils with CF.
Some older children take advantage of portable intravenous antibiotic equipment, which enables them to attend lessons more regularly.
Physical exercise is beneficial for children with CF so they should be encouraged to participate in games and PE lessons. Illness can result in a loss of energy which should be taken into consideration.
What is cross-infection with cystic fibrosis?
Cross-infection occurs when one person spreads an infection to another, either directly or indirectly. For people with cystic fibrosis, cross-infection can be very harmful and poses a particular threat. This is why people with cystic fibrosis should not meet face to face.
People with cystic fibrosis are vulnerable to different bacteria or ‘bugs’, which grow in their lungs. While these bugs are usually harmless to people who don’t have cystic fibrosis, they can settle in the lungs (colonise) and be harmful for those who do. These bugs can be easily transmitted from one person with cystic fibrosis to another.
There is less risk of transmission of ‘bugs’ in an outdoor environment, but meeting indoors, travelling with other people with cystic fibrosis, or spending time with them socially has a high level of risk. The risk of cross infection increases the longer people with cystic fibrosis are in close proximity to one another.
As a result of this, those with cystic fibrosis may be very involved in on-line communities, in forums and across social media platforms as this is the ideal way to connect and share stories and experiences.
Will my child make friends?
Yes, many children find that their friends accept their condition as most of the medication to control it is taken in the morning and evening. Some older children with CF have a noticeable and productive cough, which they may find embarrassing in front of others.
Often people living with CF choose to make the most of their life as they are aware it could be shorter than their peers. For this reason, many CF children embrace life and make lots of friends.
Will my child get a job?
Yes, they have the same chances as any other child. As people with CF are living longer, healthier and more independent lives, many more are taking the opportunity to work part-time or full-time.
Is it my fault?
Absolutely not, most people are unaware that they carry the CF recessive gene and will have had no idea that their partner has either.
Can it be cured?
There is currently no cure for CF but medicines and treatments are coming out all the time which seek to correct the genetic fault that could help children with CF to live longer with less medication.
People with cystic fibrosis today
People with CF today have a much better outlook than 30 or even 20 years ago. Advances in treatments and identification of genetic type mean that a baby born today could live to see retirement. Thanks to newborn screening, babies are being treated from birth, which means any additional or extra damage to the lungs is stopped as early as possible.
Many people living with CF take it in their stride and children who know nothing else cope very well with the treatments. But, CF is unpredictable and as a person gets older, infections and lung damage become inevitable. A person with CF could end up taking about 40-50 different tablets a day and two to three nebulised and inhaled treatments up to three times a day.
Because people with CF are living longer, they sometimes develop other related complications such as, diabetes, osteoporosis, liver and kidney damage, stomach problems and arthritis. But this does not affect everyone.
Many men with CF go onto have families but may need IVF or other fertility help as CF can cause fertility issues. In women, the pressure placed on the body by pregnancy can be too much and adversely affect their lung function. Women with CF who are planning to have children should discuss this closely with their CF consultant.
Is there any help available?
The Cystic Fibrosis Trust can point parents in the right direction to find out about financial support. For those in need, welfare grants are also available from the Cystic Fibrosis Trust, including provision of equipment and small grants for essential household items.
The hospital will be in charge of your child’s general healthcare needs and you will have access to a specialist CF Centre or receive shared care. Parents should be given access to a range of support from CF nurses, showing you how to administer medicines, physiotherapists, dieticians, social workers and psychologists.
Cystic Fibrosis Trust
The Cystic Fibrosis Trust is a registered charity that provides help, advice, guidance and welfare support for families affected by CF. The Cystic Fibrosis Trust also works closely with specialist Cystic Fibrosis Centres and health workers to ensure they are providing the best possible care for patients.
Helpline: 0300 373 1000.
A factsheet all about the condition, including symptoms, diagnosis and treatment.