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Cystic fibrosis is a genetic disorder in which internal bodily secretions become thick and sticky, and hinder the function of certain organs, such as the lungs and digestive system.
The condition is caused by a faulty gene that controls the movement of salt and water in and out of cells in the body. When cystic fibrosis occurs, too much salt and not enough water passes into the cells and turns the body's secretions, which normally act as a lubricant, into a thick mucus. This mucus then clogs up the body's tubes, ducts and passageways, and makes them ineffective (not work properly) and prone to infection.
The faulty gene that causes cystic fibrosis is recessive, which means that two separate genes, one from each parent, are needed to inherit it. It is possible to carry the disease and not suffer from it; one in 25 people in the UK carry the condition. If two people who are both carriers have a baby, there is a 25% chance that the child will have cystic fibrosis.
Symptoms of cystic fibrosis include recurrent chest infections and poor growth, and the condition can also result in related health problems such as diabetes and infertility.
There is no cure for cystic fibrosis, but much research has been done on the faulty gene since it was identified, which continues today. There are also many treatments and therapies that can make the condition easier to live with.
Symptoms of cystic fibrosis
When a child is born with cystic fibrosis, symptoms usually appear in the first year of life, although occasionally they can develop later. The symptoms and related problems of cystic fibrosis may vary from person to person, and may be worse for some than for others.
The main symptoms are detailed below.
Troubling cough and wheeze - this is caused by the thick mucus in the lungs, which your body tries to shift by coughing it up.
Recurring chest and lung infections - these can be quite severe, such as bronchitis or pneumonia. They are caused by the continual build up of mucus in your lungs, which provides an ideal breeding ground for bacteria. To prevent the risk of cross infection, it is recommended that people with cystic fibrosis do not come into close contact with one another.
Malnutrition - this is due to the mucus clogging your digestive system. The mucus blocks the ducts in your pancreas that produce a food-digesting enzyme, so not enough of this enzyme reaches your intestines. As a result, your body cannot digest much of the essential nutrients in your food, and you may struggle to put and keep on weight. In children with cystic fibrosis, this can even result in delayed puberty if they are severely underweight.
Clubbed fingers and toes - 'clubbing' is where the ends of the fingers and toes become swollen and rounded. This deformity is commonly seen in congenital heart disease patients, but it is also sometimes evident in those who have certain lung conditions, such as cystic fibrosis, and appears as the condition progresses. However, the exact reason for it occurring is not fully understood.
Polyps inside the nostrils - these are small, soft growths in your nasal passages. They are caused by inflammation of your nose and sinuses due to a build up of mucus.
Large, odorous stools - this is a result of poor digestion caused by mucus build up in your digestive system. As much of the food you eat cannot be digested, stools tend to be greasy and bulky, and may contain undigested food stuffs.
Salty tasting skin - you may first notice your child's symptoms when you kiss them as their skin can have a salty taste. This is caused by high levels of salt in their sweat.
There are also several other health problems that can occur as a result of cystic fibrosis. These include:
- liver damage,
- infertility, and
- osteoporosis (weak and brittle bones).
Due to improvements in treatment, as many as 75% of children affected by cystic fibrosis live to young adulthood, and at present, the average life expectancy is between 30 and 40 years. This is expected to keep rising as research into gene therapy and treatment continues.
Causes of cystic fibrosis
Cystic fibrosis is an inherited condition caused by a faulty gene. The gene controls the amount of salt and water that can move between cells in your body, and in those with cystic fibrosis, the amounts are uneven.
The faulty gene allows too much salt and not enough water into your cells, which results in a build up of thick, sticky mucus in your body's tubes and passageways. These blockages damage your body's lungs and digestive system, resulting in inflammation and repeated infections.
In order for a child to be born with cystic fibrosis, they must inherit copies of the faulty gene from both their mother and their father. In the UK, it is thought that 1 person in every 25 carries the faulty gene for cystic fibrosis, which is why it is so common. A carrier can be completely healthy and have no symptoms of cystic fibrosis, as they only have one copy of the faulty gene required for the condition to exist.
If two people who both carry the faulty gene have a baby, the chances of the baby having cystic fibrosis are as follows:
- 25% chance that the child will not inherit either copies of the faulty gene - this means the child does not have cystic fibrosis nor carries the condition,
- 50% chance that the child will inherit only one copy of the faulty gene from either their father or mother - this means the child does not have cystic fibrosis but is a carrier of the condition, and
- 25% chance that the child will inherit both copies of the faulty gene - this means the child has cystic fibrosis.
Diagnosing cystic fibrosis
Most cases of cystic fibrosis are diagnosed in very early life, but some children and young adults are diagnosed later through unexplained illness. If you or your child are displaying symptoms of cystic fibrosis, see your GP as soon as possible.
There are several different ways of screening and diagnosing the condition, which are detailed below.
Babies can be screened for cystic fibrosis at birth, as part of a blood test known as the Guthrie test. A small amount of your baby's blood is taken by a heel prick and transferred onto a card. The card is then analysed for cystic fibrosis, and other inherited conditions such as sickle cell anaemia and phenylketonuria. In Scotland, Wales, Northern Ireland and parts of England all babies are screened for cystic fibrosis when they are born. There has been much campaigning to make this screening process compulsory everywhere in the UK, and this should come into effect by the end of 2007. The sooner cystic fibrosis is diagnosed, the sooner treatment can begin and the better the outlook.
A test can be done on a woman when she is pregnant, if she has a high risk of having a child with cystic fibrosis. This is usually done around the 10th week of pregnancy, using the chorionic villus sampling (CVS) technique. CVS tests a small sample of your chorionic villi (placental tissues) for the presence of the faulty gene which causes cystic fibrosis. The sample is collected using a thin needle which is passed through the wall of your abdomen (stomach), or by passing a small tube through your vagina and cervix (neck of your womb). The process takes about 20 minutes and the test results should be back within 10 to 14 days.
There is a simple test, using a mouthwash, which can tell whether you or your partner is a carrier of cystic fibrosis. It is especially important to have this test if your partner is a known carrier, or if someone in your family has cystic fibrosis or knows that they carry the condition.
If you have cystic fibrosis, your sweat will have higher levels of salt than normal. In this test, a sweat-producing chemical is applied to a small area of skin on your arm or leg. Then an electrode creates a very weak and painless electric current which causes a warm sensation on the skin so that a sweat sample can be collected and analysed.
This test checks for the faulty gene by analyzing a saliva sample taken from inside your cheek using a swab.
If you or your child is diagnosed with cystic fibrosis, it can be extremely difficult to deal with. It can help to find out as much as you can about the condition. Call the Cystic Fibrosis Trust on 0845 859 1000 for information on its New Diagnosis Pack.
Treating cystic fibrosis
If you or your child has cystic fibrosis, you should treat the condition with help and advice from a team of healthcare professionals at a cystic fibrosis clinic. With regular visits, you can learn how to best manage the condition, and as each case is different, you can receive tailored care for you or your child's cystic fibrosis. See the Selected links section for details of how to find a clinic.
The aim of treatment for cystic fibrosis is to ease the symptoms and make the condition easier to live with. It can also prevent or reduce the long term damage caused by infections and other complications.
The different types of treatment and physiotherapy for cystic fibrosis are detailed below.
Medical treatments for cystic fibrosis can help to clear and control infections in your lungs and digestive system. They can also be used to treat some of the health problems related to cystic fibrosis. The main medications include:
- Antibiotics - these can be taken to fight infections in your lungs. They can be inhaled through a nebuliser (a device which turns drugs into a mist that can be breathed in), or they may be injected if your infection is more severe.
- Bronchodilator drugs - these are inhaled to help you breathe more easily. They work by relaxing the muscles that surround the airways in your lungs, helping them to open up.
- Steroids - these can be taken to reduce swelling of the airways in your lungs, which can help your breathing. Steroid nasal drops and sprays can be used to treat nasal polyps (small growths inside the nostrils).
- DNase - this treatment, which is usually inhaled, helps to thin and break down the sticky mucus in your lungs so that it is easier to cough up.
- Pancreatic enzymes - these should be taken before every meal to help your digestive system break down the food you eat, so that you can get the nutrients you need. Your pancreas cannot produce enough food-digesting enzymes due to the mucus clogging your digestive system, so these pills supply the enzymes instead.
- Bisphosphonates - these can be taken to treat osteoporosis (weak and brittle bones), that can occur as a result of cystic fibrosis. They help to maintain bone density and can reduce the number of fractures that can occur.
- Insulin - you will need to take this, and other appropriate treatments, if you develop diabetes as a result of cystic fibrosis.
- Immunisations and flu jabs - it is particularly important that people with cystic fibrosis are up to date with all required immunisations. If you have cystic fibrosis, you should also ensure that you have an annual flu jab, as you are more susceptible to complications as a result of any infection.
In advanced and severe cases of cystic fibrosis, a lung transplant may be recommended if there is respiratory failure, and all medical treatments have failed to aid breathing. Both lungs must be transplanted as they are both affected by the condition. A lung transplant is a serious operation which carries certain risks, but it can greatly improve the length and quality of life for people with severe cystic fibrosis. The longest surviving patients who had lung transplants as a result of cystic fibrosis had their operations over fifteen years ago.
Physiotherapy for cystic fibrosis helps to clear mucus build up in your lungs so that it can be coughed up. It is an important part of the treatment for cystic fibrosis as it helps to prevent infections and lung damage caused by the mucus.
Physiotherapy is carried out by 'clapping' on the patient's back and chest while they are lying down, which helps to shift the mucus in their lungs. If you or your child have been diagnosed with cystic fibrosis, you should start physiotherapy straight away. It should be done everyday, and the length of physiotherapy sessions may vary according to how clogged the lungs are. For example, if you do not have an infection and you lungs are no more clogged than usual, one or two sessions daily of 10-15 minutes should be enough. However, if you have an infection and your lungs are more clogged than usual, you may need as many as four daily sessions of up to 60 minutes a time.
Your physiotherapist will advise you on how much physiotherapy should be done. Adults can be shown how to carry out physiotherapy for children with cystic fibrosis, and adults with the condition can be taught how to do it for themselves.
As well as treating cystic fibrosis with medications and physiotherapy, there are things that you can do at home to improve the symptoms of the condition. Both diet and exercise are very important in treating cystic fibrosis.
What you can do
The symptoms of cystic fibrosis can be considerably relieved through diet and exercise, both of which can be managed at home, with guidance from your GP and cystic fibrosis clinicians. The following recommendations are intended to be advice, always follow the guidance of your GP and cystic fibrosis clinicians.
If you or your child has cystic fibrosis, getting the right nutrition is vital. A healthy body weight is necessary to help you fight off infections, and it is important that you have enough reserve energy to rely on if you do become ill. Mucus build up blocks the ducts in your pancreas that produce a food-digesting enzyme, and as a result, your body cannot break down all of the food you eat and you do not get all of the nutrients you need. Therefore, the diet of someone with cystic fibrosis should be high in calories, as they will inevitably eat more than they can digest.
The special diet for cystic fibrosis should start as soon as the condition is diagnosed, and it will need to adapt as the person with the condition gets older. The dietary requirements of babies, children, and adults with cystic fibrosis are detailed below.
Babies with cystic fibrosis
- If your baby has cystic fibrosis, they may be breast fed as usual, or most baby milks and formulas should be suitable. Ask your GP or dietician if you are unsure.
- Before your baby moves on to solid foods, they may need extra salt as both breast milk and baby milk are very low in it. This is because cystic fibrosis makes their sweat five times saltier and much of the salt in their bodies is lost through their skin. If your baby needs extra salt, your GP can prescribe a salt solution. You should never add salt to your baby's food or drink.
- You can feed your baby any normal solid baby foods, but they will need vitamin supplements to compensate for the loss of vitamins A, D and E in their stools. These are available in two liquid preparations; vitamins A and D are usually combined in one, and there is usually a separate one for vitamin E.
- Your baby will also need pancreatic enzymes, which take the place of the food-digesting enzyme missing from their digestive system, to help them digest food. These usually come in the form of granules, which can be mixed with fruit puree and spoon fed at the start of every meal. You should never give your baby dry granules as these can be a choking hazard. Your dietician or hospital can advise on the right amount of granules to use.
Children with cystic fibrosis
- Children who have cystic fibrosis should eat a varied diet, but they should also eat a large amount of protein and calories. Give them plenty of meat, fish and eggs, as well as starchy foods like bread and pasta. If your child's appetite is poor, try giving them food little and often, and offer snacks in between meals. Children who find it particularly hard to gain weight can be given dietary supplements in the form of milk shakes or fruit juices. Talk to your GP or dietician about these.
- Like babies, children with cystic fibrosis will need to continue taking vitamin supplements for vitamins A, D and E, in order to compensate for what they lose.
- Your child will also need to continue taking pancreatic enzymes at the start of every meal, which can be taken in easy to swallow capsules. Examples of pancreatic enzymes for children are Creon and Pancrease.
- Your GP may recommend salt supplements for your child to take in hot weather or on holiday, when they may lose salt through sweating.
- Your child will probably eat more sugary foods than normal due to their high calorie diet, so it is important to make sure they are brushing their teeth properly and visit the dentist regularly.
- If your child cannot gain enough weight and dietary supplements have not helped, they may need to be temporarily tube fed in hospital. This will usually be either nastrogastric (in which the tube is inserted through the nose and goes down to the stomach), or gastronomic (in which the tube is inserted directly into the stomach).
Adults with cystic fibrosis
- Adults who have cystic fibrosis do not need to avoid any particular food, but they do need twice the amount of protein than normal. Try to eat plenty of meat, fish and eggs, and eat three meals a day with several high calorie snacks in between, such as nuts and oily fish.
- If you have cystic fibrosis and you want to be a vegetarian, talk to your GP or dietician to about alternatives to meat. However, a vegan diet is not advised for people with cystic fibrosis.
- Most adults with cystic fibrosis need to continue taking pancreatic enzymes at the start of every meal to aid their digestion. There are many different kinds, so talk to your GP to find one that suits you. However, you should not stop taking pancreatic enzymes unless you are told to do so by your GP or dietician, as this can lead to a serious blockage in your bowels.
- You will also need to continue taking vitamin supplements for vitamins A, D and E. For adults, these come in the form of prescribed multivitamin supplements.
- If you lose a lot of weight due to an infection or illness, you may need to be tube fed in order to help you gain weight. As in tube feeding for children, this will be either nastrogastric or gastronomic, depending on how long the tube must stay in for. A gastronomy tube (which is inserted directly into the stomach) is best for longer periods of time as it cannot be dislodged during physiotherapy.
It is extremely beneficial for people with cystic fibrosis to exercise as it can help to prevent lung damage, and improves your body's strength and mass. Any sport or exercise that gets you moving and out of breath is good, such as football, running, tennis or swimming.
If your child has cystic fibrosis, let their school know that exercise is encouraged.
Young children can be encouraged to run around and play games.
Complications of cystic fibrosis
Cystic fibrosis can cause several health complications and related problems, which are detailed below.
Around ten percent of babies with cystic fibrosis are born with a serious bowel obstruction, known as meconium ileus, which can make them very ill in their first few days. All babies are born with meconium in their bowels, which is a thick, black material normally passed a day or two after birth. However, in babies with cystic fibrosis, the meconium is too thick to be passed through the bowels and causes a blockage. If your baby is born with meconium ileus, it is likely that they will need an urgent operation to remove it.
In older people with cystic fibrosis, the pancreas can become very damaged by the mucus that builds up in the digestive system. Diabetes can occur if your pancreas fails to produce enough insulin, a hormone that controls the level of sugar in your blood. The symptoms of diabetes include feeling constantly thirsty, frequently needing to pass urine, and feeling extremely tired. If you develop diabetes alongside cystic fibrosis, you may need to take regular injections of insulin to control it.
Osteoporosis (weak and brittle bones)
If you have cystic fibrosis, your bones can become weak and brittle if you do not get enough of the vitamins and minerals you need. Adults with cystic fibrosis are more at risk of developing osteoporosis if they are taking steroids to help their breathing. If you develop osteoporosis as a result of cystic fibrosis, you may experience joint pains, and your bones may fracture (break) more easily. You may need to take bisphosphonates, which help to maintain bone density.
Both men and women with cystic fibrosis can have problems conceiving children. In men, this is due to a build up of mucus blocking the tubes that carry sperm, but this does not make them impotent. In women, the tendency to be underweight can affect their menstrual cycles, and can cause them to have irregular periods. However, the eggs are not affected so it is possible for women with cystic fibrosis to conceive, and there is still a need for contraception. Some oral contraceptives can aggravate symptoms of cystic fibrosis, so you should discuss this with your GP. You should also see your GP if you have cystic fibrosis and you want to become pregnant, as pregnancy can also make your symptoms worse.
In around 8% of people with cystic fibrosis, the tiny ducts in the liver can become blocked by mucus. This can be serious and may even lead to a liver transplant.
Future prospects of cystic fibrosis
Since the faulty gene that causes cystic fibrosis was identified, scientists have been carrying out research to try to correct the genetic defect. This is known as gene therapy, which replaces faulty genes with normal ones.
How gene therapy works
A complete set of genes are present in every cell of your body (except red blood cells), and these genes contain all the information needed to build a human being. It is your genes that determine factors like the colour of your eyes, and whether or not you can roll your tongue.
Each human cell contains 46 chromosomes, which are made up from 23 chromosomes from your mother, and 23 from your father. There are approximately 30,000 genes on each chromosome, and the faulty gene that causes cystic fibrosis is found on chromosome 7. For a child to be born with cystic fibrosis, they must inherit a copy of the faulty gene from both parents.
Gene therapy for cystic fibrosis involves adding a healthy copy of the faulty gene into the relevant cells in the body, such as the ones in the lungs and digestive system. This can be done using either viruses, which have been developed to enter the cells efficiently, or liposomes, which are fatty substances that naturally stick to the surface of cells and encourage genes to enter them.
So far, the benefits of gene therapy have only been made to last for a few days. However, with the knowledge that gene therapy works, scientists can continue to research how to make the effects permanent. The two main challenges for them are getting the healthy genes into the cells in a more efficient way, and making the benefits last as long as possible.
Paediatrician Dr Alan Day on the questions to ask
We asked Dr Alan Day, consultant paediatrician with a special interest in cystic fibrosis, at Cheltenham General Hospital, what he would want to know about the condition.
What questions should I ask if my child is diagnosed with cystic fibrosis?
After diagnosis, families can be shocked and be concerned about what it means for any future children.
The first thing many parents of a newly diagnosed child ask is, ‘Is my child going to survive?’ The survival rate has quadrupled from the age of 14 in 1977. Now, a child born in 2000 can expect to live into their mid-50s and beyond. The main cause of death in the past and, sadly, even now in children and young adults with cystic fibrosis is from long-term complications such as chronic chest infections. There are many treatments that aim to minimise these complications and parents will want to know what the treatment will involve. There have been many advances in the past 20 years and there will be many more.
How is cystic fibrosis detected?
A blood test called the Guthrie Test, carried out when a child is six days old, has resulted in a significant increase in the number of patients diagnosed. The test is now performed over the whole of the UK and it is proving to be effective in diagnosing cystic fibrosis very early. The sooner we diagnose patients as babies, the better the outcome should be. The test done thereafter is called a sweat test, which tests the salt content in bodily secretions.
What risk is there if I have more children?
Cystic fibrosis is the result of a simple genetic defect inherited because both parents are carriers of the defective gene, even if they themselves are not affected. There is a one-in-four chance of having another child similarly affected. There is also a two-in-three chance that siblings could be carriers, even if they're not affected. This can be detected through testing.
What happens if cystic fibrosis is not treated?
If left untreated, as happened 30 or 40 years ago, a child with cystic fibrosis would eventually develop a very bad chest infection and chronic diarrhoea. As the child wouldn't be able to absorb fat and protein, they would be very weak. Complications elsewhere would involve the gut, as the pancreatic gland doesn't work properly in 98% of cases, and some patients may develop diabetes. Because of the thick secretions, many will also suffer from liver problems. A number of patients will also develop joint problems such as arthritis. This is because the body is constantly battling against germs, and this can cause the joints to become irritated.
Can a child with cystic fibrosis enjoy a normal life?
As a child gets older there can be problems, such as rejection of the treatment, their physiotherapy being hard, and so on.
There are difficult stages in life, particularly adolescence. They might question the diagnosis and ask, ‘Why me?’ The treatments can also, at times, be socially embarrassing. We try to normalise things as much as possible and address lifestyle issues, such as finding a suitable job. There are statistics to suggest that cystic fibrosis patients have marginally better state exam results and they are often very sporty. People with cystic fibrosis really can live life to the full. We aim to have no limitations on what a person with cystic fibrosis can do.
What does the future hold?
We can be very upbeat. We're getting earlier diagnoses through neo-natal screening, and the treatments are improving through lots of research run by the Cystic Fibrosis Trust, which helps to minimise the complications. Sadly, for reasons we can't clearly understand, there are still complications. But there has been a huge increase in the life expectancy for this lifelong condition.
Just 20% of babies in four areas of the UK were routinely screened for cystic fibrosis before the launch of the new screening programme. The programme will soon be available in all areas of the UK, so the CF test will be offered to all newborns.
Dr Kevin Southern, reader in paediatrics and honorary consultant at Alder Hey Hospital, Liverpool, where routine screening was introduced in February 2007, answers some of your questions.
What happens during a screening test?
The test is very simple to perform. Your midwife will prick your baby’s heel when he or she is five days to a week old, and collect the few drops of blood that come out on a special card known as a Guthrie card. The card is then sent to a laboratory.
Exactly how is the blood screened for cystic fibrosis?
The lab will screen the blood for levels of a protein known as IRP. If your baby has high levels of IRP, that may indicate he or she has cystic fibrosis. But it’s not a very specific test. Some children who don’t have CF may also have high IRP. If the IRP is high, a DNA analysis of the blood spot is done to look at the baby’s genes. This will show whether the genes contain the four common mutations associated with cystic fibrosis. If these show up in the blood, the diagnosis is confirmed.
How accurate is blood screening?
Screening isn’t always this straightforward. There are many other rare mutations. If the baby has a very high level of IRP and doesn’t show the common mutations, the midwife will do another heel-prick test when the child is between three and four weeks of age. If the IRP is still raised, there’s a very good chance the child has CF.
Are there any other tests to check my child for cystic fibrosis?
Your consultant might also recommend a ‘sweat test’. The sweat glands of cystic fibrosis sufferers can’t remove the salt from their sweat, so their sweat has a very high level of salt, which we can test for.
More frequently asked questions on cystic fibrosis screening are answered on the UK Newborn Screening Centre website (links to external site).
Cystic fibrosis and young people
Living with cystic fibrosis is a real challenge for young people. However, there are things that you can do to try and make your life easier.
Cystic fibrosis specialist nurse Jenny Cottrell offers some advice:
“As you are born with CF, the chances are that you’ll have grown up with its problems. The main problems are usually chest infections and recurrent abdominal problems. Both of these can be embarrassing for young people, as they have to cope with the coughing in public and the need to go to the lavatory quickly.”
Ways to cope
There’s no escaping the fact that Cystic Fibrosis is a serious condition and sometimes a stay in hospital for treatment will be necessary. It is very important for your child to keep up with all their physio and treatment, even when they feel unwell. It will lead to a healthier, fuller life.
Some children are happy to take their enzyme tablets in front of their friends. Others can get embarrassed so the school should provide a quiet place. Speak to the school and your child’s teachers so that they know about the CF. Good schools will strive to ensure that all children are treated the same and your child is not made to feel awkward or embarrassed. Teachers should help by letting the child visit the lavatory when needed or providing a key if the facilities are locked.
Exercise is a good way for young people with CF to keep healthy. It causes coughing which moves the sputum that sits on the lungs. Encourage your children to find a sport they like, as they won’t see it as a treatment. Many take to football and swimming.
The family as a whole can exercise together by going for a bike ride. In fact, there is no exercise that your child should not do and they should join in with everyone else at school. However, they should stop if they become short of breath.
Children with CF should eat often and regularly because being underweight is a big problem. They should eat calorie-packed foods like crisps and sweets as well as a healthy diet and the enzymes that help them absorb the food.
Young people with CF should learn breathing techniques so they can clear their chest rather than relying on parents or carers to perform physiotherapy. This allows them an independence as well as control over their life and health.
Jenny Cottrell says: “Focus on who you are as a person, not on your CF. Try to fit CF into your life rather than letting it take over your life and enjoy yourself as much as possible!”
The Cystic Fibrosis Trust (links to external site) offers practical help and advice on all aspects of living with cystic fibrosis
Staying in hospital
Hospital stays can be a regular feature of life with cystic fibrosis. But they can be scary places for children. Cystic fibrosis nurse specialist Jenny Cottrell answers parents’ concerns.
Why does my child need to go to hospital?
The most common reason for a hospital stay is a chest infection, which needs to be treated with intravenous antibiotics every day for two weeks. That means your child will need to have a ‘line’ inserted, usually in a vein in their arm, for the antibiotics to go into their bloodstream. Your child will be given a local anaesthetic beforehand so it won’t hurt. Some children who require frequent intravenous antibiotics may be offered a portacath, a device surgically implanted in the upper chest to allow easier access to the bloodstream.
Where will my child stay?
If your child is under 16, he or she will probably be on a children’s ward. If they are over that age, your child may be put on a special adult ward for people with CF.
Can I stay with my son while he’s in hospital?
Children with CF are usually given a room to themselves and many parents stay for as long as they like, sleeping on a Z-bed. Some hospitals also have special accommodation for parents.
My child’s very scared about going into hospital for the first time. How can I help reassure her?
Going into hospital can be very overwhelming for children, as they don’t know what to expect. But play leaders, who work on children’s wards, will explain to children exactly what’s going on, and help keep them occupied. You’ll also be allowed to stay with your child while they are having their treatment. There are lots of distractions around, like music, mobiles and toys and you can bring in your child’s favourite games. If there’s anything at all that you’re worried about, talk to the nurses - they're there to help.
'I was 12 years old when I learnt how to do the physio by myself'
Lauren, 14, talks about living with cystic fibrosis and how she doesn’t let it stop her from doing the things she loves
"Cystic fibrosis stops me from going out with friends sometimes and from doing sports because it makes me cough a lot.
"I also have to go to doctors’ appointments and to hospital when I’d rather be with my friends and doing social things. It can get a bit annoying at times.
"My personal symptoms are things such as coughing, wheezing and being unable to do things that I normally could do if I wasn’t ill. For example, if I was coughing a lot, then I couldn’t do any sports or dancing because it would make me more productive, which means bringing up lots of phlegm and isn't very nice. Sometimes it makes me sick in front of my friends, which can be embarrassing.
"My treatments are things like medication, or I have IV antibiotics through a needle in my hand or portacaf, which is a piece of metal under the skin. I also have inhalers and physio twice day. The physio is basically percussion, tapping on the chest to make me bring up phlegm. It helps because it can stop me from getting bugs and brings up all the nasty stuff that can do my body harm.
"I was 12 years old when I learned how to do my physio by myself properly. I also learned how to use my inhalers, the PEP (Positive Expiratory Pressure) mask and the flutter. My mum taught me how to do it and I think it was easier for her to teach me than a doctor because she has been with me the whole way through. I wouldn’t say there was a proper age for people with cystic fibrosis to start learning their own treatments, just when they feel confident. It’s quite important that people learn to manage their own treatment because it makes you feel more independent and happy that you’re not relying on somebody else.
"Having CF doesn’t stop me from doing everything I want to do, but when I’m unwell, it can stop me from swimming and dancing. When I’m well, I can do anything that someone who doesn’t have CF can do. I love to dance and sing, and I want to be an actress on Broadway or dance in musicals.
"When I’m unwell I always plan something and try to have something to aim for. When I’m in hospital and can’t go out for a few days, I always plan something nice so that I have something to look forward to. So you can fight anything bad that comes along because there’s something positive at the end."
NHS Choices links
- Health A-Z: chorionic villus sampling
- Health A-Z: bronchodilator drugs
- Live Well: child health 0-1
- News: CF drug
- News: CF in mice
- Live Well: antenatal screening
- NHS Guide: diabetes
- Cystic Fibrosis Trust
- Genetic Interest Group
- Cystic Fibrosis Screening
- Gene Therapy Consortium
- Find a cystic fibrosis clinic
- Healthtalkonline: real stories