Skip to main content
Donate

FAQs

Diagnosis questions:

If your GP, paediatrician or other specialist doctor thinks you or your child has PCD they will refer them to a PCD diagnostic centre.  Diagnosis is only made in specialist units, of which there are three nationally funded centres in England located at Leicester, Southampton and in London. You or your child will either be seen at a clinic or an expert from the centre may visit a hospital nearer your home. It doesn’t matter where in the UK you live to be referred to one of the four PCD diagnostic centres, nor does it cost your GP to refer you for this. If you are suspected of having PCD, it is better to be diagnosed by a PCD diagnostic centre than to be sent for genetic testing, because genetic testing only identifies up to 70% of genes causing PCD. 

Sometimes a baby may be suspected of having PCD before birth, if situs inversus (organs in opposite position) or other situs abnormalities (where the heart, lung, stomach, liver and spleen are not in the normal position) are seen on the antenatal scan during pregnancy.  In many cases this may result in the obstetrician referring the mother for specialist foetal echo scans to check whether there are any problems with the heart.  After birth, the baby will be tested for PCD. 50% of patients with PCD have situs inversus but organ position abnormalities can occur for other reasons too.  

To be diagnosed, you or your child will be seen by a consultant who will talk to them about their symptoms, your family and if they feel it necessary, they will conduct several diagnostic tests.

These may include:

  • Measuring the level of nitric oxide (‘NO’) in the nose: this involves breathing into a small tube. It is a painless, ‘non-invasive’ test and is usually done in people aged 5+. 
  • A cilia biopsy: a small brush is used to ‘sweep’ cilia cells from high inside the nasal passages. This is slightly uncomfortable but is very quick. It might make your eyes water or make you sneeze. The cells are grown in a laboratory and then analysed using special microscopes to see how the cilia look and beat. 
  • A blood test: Sometimes your doctor will recommend a genetic blood test to identify the affected gene causing your PCD. Scientist have now identified 50 genes that cause PCD. It is important not to only have genetic blood test, as these only identify around 70% of PCD cases.  
  • Chest X-rays or a CT scan: These scans are important for looking at your lungs and are generally considered low risk. You should speak to your doctor if you are worried.
  • Lung function tests: you may be asked by a nurse, physio or technician to blow into a special machine to see how well your lungs work. This should be painless.

These tests will allow your doctors to look at the ‘ultrastructure’ (how your cilia are built and arranged). They will also tell your doctors whether your cilia are moving as they should. Most PCD cases can be diagnosed this way, however a small minority of people might not get a complete diagnosis from these tests. Research is ongoing to develop more advanced techniques to assist in these more difficult to diagnose cases. For these small number of patients, they will be treated in the same way as confirmed cases.

Most diagnostic centres will test you within 4-18 weeks of being referred by your GP, although the COVID-19 pandemic has meant there have been small delays to this service. You might find that the nasal oxide test and lung function test is done the same day of your clinic appointment, but the cilia biopsy will take around 4-6 weeks to complete. Sometimes the biopsy sample is not good enough and this test will need to be repeated. We know it can be frustrating whilst you wait for results, but you will be given some advice and contact information whilst you wait and the PCD specialist team will contact you with your results.

The symptoms of PCD can vary in severity from person to person. It is recommended that patients are tested for PCD if they have several of the following features: persistent wet cough; situs anomalies (where organs are not in the ‘normal’ position in the body); congenital cardiac defects; persistent rhinitis; chronic middle ear disease with or without hearing loss; being born at approx. 40 weeks with upper and lower respiratory symptoms or have been admitted to neonatal intensive care with breathing difficulties after birth. Having a sibling or close family member with PCD means that you may be more likely to have PCD – you should let your GP know this.

Doctors can use this diagnostic predictive tool to determine how likely it is you have PCD. 

 

Questions about what PCD is:

The European Union defines a disease or condition as rare if it affects fewer than 1 in 2000 people within the general population. Currently, there are over 6000 known rare diseases and new conditions are being described through medical literature on a regular basis.

PCD is classified as a rare as it thought to affect 1 in 15,000 people.

PCD Support UK supports Rare Disease UK – a national campaign for people with rare diseases and all who support them. They provide a united voice for the rare disease community by capturing the experiences of patients and families. They work with their supporters and the UK Government to raise the profile of rare diseases across the UK. They seek to bring about lasting change offering better health and quality of life for individuals and families affected by rare diseases.

Ciliopathy is a term used to describe a disease which causes defects in the function or structure of cilia. PCD is therefore a ciliopathy.

Dysfunctional cilia are known to cause several chronically disabling and sometimes life-threatening genetic conditions. They can affect multiple systems, causing blindness, deafness, chronic respiratory infections, kidney disease, heart disease, infertility, obesity and diabetes.

Over 20 ciliopathies (of which PCD is one) have been identified and more are suspected, with an estimated 1 in 1000 people affected. 

PCD Support UK is a member of The Ciliopathy Alliance which brings together patient support groups, researchers, doctors and allied health professionals representing patients and families living with and affected by ciliopathies.

Cystic fibrosis is a genetic condition which affects the movement of salt and water in and out of cells.  People with CF experience a build-up of thick sticky mucus in the lungs, digestive system, and other organs, causing a wide range of challenging symptoms affecting the entire body. 

PCD is a different genetic condition which affects the movement of cilia, tiny, microscopic hairs that line your airways and can be found in other areas of the body such as your ears and your reproductive organs. These tiny hairs help move mucus out of the lungs and if they do not work properly people get lung infections.  

One of the most important treatments for both conditions is physiotherapy to help remove the mucus from the lungs and the use of targeted antibiotics to treat any infections. PCD patients have in the past sometimes been treated by CF doctors because of the issues with the lungs and similar treatment. Nowadays, here in the UK, we now have specialist PCD centres where patients can see PCD specialists.

 CF is more common than PCD and affects 1 in 2500 babies born. Because of this, people are more likely to be tested for cystic fibrosis before PCD, and in the past this has meant that there has been more research into CF treatments and physio techniques, many of which have been recommended for PCD. There is increasingly more PCD-specific research, and we expect this to continue in the years to come. Recently there have been some new treatment breakthroughs in the CF world, including drugs called Trikafta (or Kaftrio), Orkambi, Symkevi and Kalydeco. These treatments target the genetic causes of CF, which are different to PCD. They are not therefore recommended for the treatment of PCD. 

Situs inversus is a congenital condition in which the major organs (heart, lungs, kidneys, spleen, stomach etc.) are reversed or mirrored from their normal positions. The normal arrangement of internal organs is known as situs solitus.  Many people with situs inversus have no medical symptoms or complications resulting from the condition. 

Situs inversus is found in about 0.01% of the population, or about 1 person in 10,000. In the most common situation, situs inversus totalis, it involves complete transposition (right to left reversal) of all the organs. The heart is not in its usual position in the left chest, but is on the right, a condition known as dextrocardia (literally, “right-hearted”). Because the relationship between the organs is not changed, most people with situs inversus have no medical symptoms or complications. 

If you have PCD, the cilia do not function properly from the early stages of embryonic development. Normally functioning cilia determine the position of the internal organs during early development, and so embryos with PCD have a 50% chance of developing situs inversus. When you have PCD and Situs Inversus it can be called Kartagener Syndrome (after the doctor who first discovered a connection between patients with situs inversus and respiratory disease in 1933).

Similarly, there is situs ambiguus. This is when the major organs are displaced, but not in a mirrored arrangement.

When only the heart is in a reversed position (on the right rather than the left), this is known as dextrocardia.

Both situs ambiguus and dextrocardia, as well as situs inversus can occur in PCD patients.

A lung function test is used to how efficient your breathing is.

When you arrive at the hospital, the technician will measure your weight and height. If they can’t measure your height (if you are in a wheelchair, for example), they will measure your arm span – this gives them a guide to your height.

For the tests, you will need to blow into a machine. With some machines, you blow into a tube; for others, you put a mouthpiece in your mouth, like a snorkel, and then blow. You will usually have to do this several times – but the tester will not rush you and it shouldn’t cause you any pain.

If you are breathing through your nose, and this is affecting your breathing test, you may be asked to wear a nose-clip.

If you wear dentures and they’re loose, then you might need to take them out when you blow into the machine.

Occasionally, the technician might ask you to sit in a booth, which looks a bit like a small see-through shower cubicle. The door will be closed and the test will be done while you sit inside. This will help your tester get more detailed results.

There should not be any side effects from carrying out these tests.

As you are usually asked to perform the breathing exercises a few times, you may feel a little tired after the tests have been completed – this is quite normal and you should not worry.

The tired feeling will quickly disappear. However, if you have any chest pain or other problems during the tests, please tell the person testing you.

Your lung function test results and a report, if needed, will usually be available almost immediately after the tests have been completed.

You can either take these results with you to the clinic or ward or they can be sent for you.

Do’s and don’ts

  • Do wear loose clothing which does not restrict your chest and abdominal movement.
  • Do not smoke for at least 24 hours before the tests.
  • Do not perform vigorous exercise for at least 30 minutes before the tests.
  • Do not eat a large meal during the two hours before the tests. Please do not drink alcohol during the four hours before the tests.

You may also be asked to do a spirometry test. It is a test that you can perform at home (if you have the equipment) and measures your FEV1 – which is how much air you can expel from your lungs in the first second of breathing out. It also measures your FVC – which is how much air you can get into your lungs and how long it takes for you to empty your lungs. If you have a confirmed diagnosis of PCD, your PCD team may send you a home spirometer to do these tests.

 

Genetics and research questions:

PCD is a recessive condition.  So, if your partner is not a carrier for PCD none of your children will have it, but they will be carriers. If your partner is a carrier for PCD (they will not show any symptoms) there is a one in two chance that each conception will result in a child affected by PCD.

More information can be found on our genetics page but if you are planning a family, you can ask your consultant to refer you to a genetic counsellor.

There are numerous ways you can get involved with research from giving samples of your cilia, to completing questionnaires.  See our research section for more info. 

If neither parent has a PCD diagnosis, there is a one in four chance at each conception of the child having PCD.

More information can be found on our genetics page but if you are planning a family you can ask your consultant to refer you to a genetic counsellor.

 

Help and support questions:

You may be entitled to a blue badge if you find walking difficult or are caring for a child who cannot walk far because of their lung condition. There is useful information here. In addition, you can review the eligibility criteria and process for applying for a blue badge on the government website here.

If you are referred to hospital or other NHS premises for specialist NHS treatment or diagnostic tests by a doctor, you may be able to claim a refund of reasonable travel costs under the Healthcare Travel Costs Scheme (HTCS).

You can find out if you are eligible for the scheme and how to make a claim here.

Before COVID-19 we held annual meetings for people with PCD to meet others. Currently we hold online meetings to help you learn more about PCD and to meet other people. Find out more.

We are a volunteer led organisation and are always looking for additional help. There are numerous ways you can help such as fundraising, helping at events, helping with updating the website, writing articles for our social media accounts, or you could tell your PCD story to raise awareness.  You may also have other suggestions. Please contact us if you are interested.

If you are an adult with PCD and are under state retirement pension age, you may be able to claim Personal Independence Payment (PIP).  Further details can be found here.

As a carer for a child with PCD, you may be entitled to claim Disability Living Allowance (DLA).  If the child is 16 or over, then you may be able to claim Personal Independence Payment (PIP). Further details can be found here.

DLA/PIP are social security benefits for people who have an illness or disability. They are tax-free and do not reduce the amount of any other benefits that you may get and may in fact increase them. They are not based on how much money you earn, or savings you may have.

PCD affects people in many different ways, and some people are more severely affected than others.  Some of our members claim the lower rates of PIP, some claim the higher rates, and some do not claim at all.

You may be able to claim a Council Tax Reduction if you use a room in the house to help with your health condition, e.g. for a physio bed/oxygen generator.  You will then pay at the rate of one council tax band lower than the one that you are currently paying. You would need to apply to your local County Council for this.  There is information on the individual Council websites about Disabled Band Reductions as well as the application form.  It is simple to apply for and has nothing to do with your income or savings.

We are PCD Support UK. We’re here for those affected by PCD, we talk about PCD as widely as possible and we champion research to improve its diagnosis, management and treatment. 

We help by:

  • Providing a source of information about PCD, its research and its treatments through our website and social media
  • Connecting our UK based PCD community via online forums, social media and live events
  • Helping people in the UK access specialist PCD services
  • Working alongside researchers, doctors and other organisations to encourage the future direction of PCD research and treatments
  • Putting people affected by PCD in touch with other organisations who might be of help (such as for benefits enquiries)
  • Providing small grants for specific needs (such as equipment) to individuals and families affected by PCD 
  • Raise awareness of PCD in the medical community, so that more healthcare professionals are aware of this condition 

 

Living with PCD questions:

Sputum (pronounced ‘sp-you-tum’), phlegm (pronounced ‘flem), catarrh (pronounced ‘cat-arr’) and mucus (pronounced ‘m-you-cuss’) are terms that you will come across if you have PCD. There are some differences in the meanings of these words, although lots of people use them for the same thing. Officially, ‘phlegm’ is the mucus (also known as sputum) you cough up from your lungs. 

Mucus is a slippery, gel-like substance that is produced by the body. In the respiratory tract (our lungs, sinuses, throat), it covers our airways to stop them from drying out. Mucus is made out of components found in the body, such as cells, enzymes and proteins and it helps to trap and destroy germs that may enter the respiratory system. 

For most people, cilia (the little hair-like structures lining our airways) beat mucus up from the lungs to the throat, where it is swallowed. As a result, germs trapped in the mucus will be destroyed by our stomach acid. In PCD, the cilia do not beat properly, and so mucus (and any germs it contains) gets trapped in the airways, causing infections. This is why physio is so important in PCD, as it helps our bodies move mucus out of the airways, so that germs do not have time to cause infections. 

Have you ever noticed that you produce more mucus when you are unwell? This is because when the linings of our airways become irritated, we produce more mucus. In addition to germs being trapped in the airways, irritation can also be caused by cigarette smoke, pollution and for some people, things like allergies. 

Being aware of changes in your mucus is an important part of managing PCD. If you notice that you are coughing up more than usual, or that your nose is more productive than usual, this might be a sign of having an infection. Any change of colour in your mucus should not be ignored. For example, if you usually cough up white or yellow mucus and this gets darker (such as green or brown), you should send off a sputum sample and consider starting your ‘rescue’ antibiotics. You might also want to contact your PCD centre for advice.  

More information can be found here.

In early 2020, COVID-19 was a new virus and doctors were learning about how it would affect patients on a daily basis. Our PCD specialists were cautious and recommended that all adults with PCD were classed as extremely clinically vulnerable and patients were asked to stay at home and shield.

In order to be able to learn more, a research team have been studying how COVID-19 infections affect people with PCD. Results are posted online and made available to people with PCD, health professionals and policy makers. Ultimately, the aim is to help improve the health of people with PCD. Every person with confirmed or suspected PCD can still take part – find out more here

PCD adults are recommended to have the COVID-19 vaccination when offered.  If you are concerned about this, please contact your PCD specialist.

PCD is diagnosed at one of three specialist centres which are funded by the NHS Specialised Services. Your paediatrician or adult respiratory physician can refer you for diagnosis.

Once diagnosed, you or your child will be seen in one of the four specialist centres.

Central England, Leicester/Birmingham
Prof Chris O’Callaghan, Dr Manjith Narayanan, Dr. Priti Kenia, Dr. Simon Range

Royal Brompton Hospital, London
Prof Claire Hogg, Dr. Siobhan Carr, Prof Michael Loebinger

Southampton General Hospital
Prof Jane Lucas, Dr. Woolf Walker, Dr. Mary Carroll

Northern Service, Leeds/Bradford
Dr. Evie Robson, Dr. Eduardo Moya, Dr. Daniel Peckham

It is important for you to choose a school that meets your child’s educational and welfare needs. If possible, find a school that is close to home or is easy for you to collect them if they are poorly or need to attend a hospital appointment. In some cases, having a medical condition such as PCD can mean that your child is considered a priority for attending a school close to where you live (e.g. in areas where there are waiting lists and limited school places). Speak to your local educational authority if this affects you. You might also want to ask your PCD team about this. 

Before your child starts at the school, you should arrange to meet the headteacher, class teacher and school nurse (if there is one) to explain how PCD affects your child and any needs they might have. In some cases, your PCD team might recommend your child has physio during the school day. If this is the case, your physiotherapist may visit the school to demonstrate how to do physio. These arrangements will need to be planned before your child starts. Further information can be found here.

In most cases, you are not legally required to tell your employer that you have PCD, however it could help. 

Sharing information about your PCD with your employer can help them understand how it affects you on a daily basis. This might make it easier if you need to have time off, attend regular hospital appointments or work from home. These things are called ‘reasonable adjustments’ which employers are required to make in order to enable a disabled person to work.

Many people with PCD consider themselves disabled under the Equality Act 2010. The Equality Act 2010 defines disability as “a physical or mental impairment, and the impairment has a substantial and long-term adverse effect on the ability to carry out normal day-to-day activities”.  The Equality Act 2010 states that disabled people have the right to ‘reasonable adjustments’, which can make jobs and services accessible to them. This applies to most areas of public life, including public and private services and employers. 

The charity, Scope, has some useful guidance about work and careers for people with disabilities and long-term health conditions.

Some patients can have upset stomachs from taking antibiotics.  

Antibiotics may be effective at eliminating bacterial infections in the body, but they also disrupt the balance of desirable and undesirable bacteria in the gut.

It is advisable to try and introduce some friendly bacteria into your diet in the form of live yoghurt, cottage cheese, miso, sauerkraut, tofu, tamari, and kefir. However, these may not be enough, and a friendly bacteria supplement known as a probiotic may be beneficial. You can research lots of information on probiotic supplements on the internet or you may choose to discuss with a dietician or nutritionist. 

It is important to regularly test your sputum so that you and your PCD team can be aware of what might be growing in your lungs. Sending in a sputum sample approximately 4 times a year is a useful way of monitoring this and will help you manage your condition in the short and long term. You can send a sputum sample via your GP or PCD centre. It is advisable to keep a note of any bacteria you grow and to share this with your GP or PCD centre for their records. Sending off regular sputum samples will make sure you are on the right type of antibiotics (both for prophylaxis and rescue antibiotics), as not all bacteria respond to certain types of antibiotic.

You should also send a sputum sample for testing every time you think you have a chest infection. Often when you have an infection, you will feel unwell and your sputum may change colour, but this is not always the case. Some common signs of becoming unwell with a chest infection include: sputum colour change, producing more sputum, noticing a change in taste or smell to your sputum, chest pain, shortness of breath, temperatures, feeling more tired than usual, joint aches. There may be other symptoms too. You will get to know these symptoms over time.

You can expect your sputum results to take about 1 week from the point of sending off the test. Normally you will call up your GP (or hospital – if that’s who sent it off) for these results, and sometimes they will contact you. Some people with PCD regularly grow bacteria (often called ‘bugs’) in their lungs, without feeling unwell. If this happens, your PCD team will work with you to decide whether to treat this or not. If you are feeling unwell, your GP or hospital will normally recommend 2 weeks of antibiotics, commonly referred to as ‘rescue antibiotics’. If you are still feeling unwell after 2 weeks of antibiotics, you should speak to your doctor about whether to send off another sputum sample to see if the infection is still there.

Some bacteria that are commonly present in the natural environment can be harmful to those with PCD and it is important to make sure that you regularly get your sputum checked for this. The Bronchiectasis guidelines recommend that you ask your GP to check your sputum under cystic fibrosis protocol. This means they will check for bacteria that don’t usually cause problems in healthy lungs but can cause infections in PCD. 

Most GPs will put some pots and forms together for you to take home, so that if you decide you need to test your sputum, you can drop in a sample without needing to arrange a doctor’s appointment. Most doctors recommend your sputum sample being the first cough of the morning (as this is more likely to have bacteria in it). You should drop off your sample the same day that you do it. Some people find it helpful to ask their doctor’s surgery what time the sputum sample collections are, so that they can ensure their test is collected the same day. 

Many patients keep a note of the bacteria that they grow in their sputum samples. This can be helpful to know, particularly if you regularly see different doctors. The most common bacteria grown in people with PCD are: staphylococcus aureus, streptococcus pneumoniae and haemophilus influenzae, however this varies from person to person. There are an increasing number of patients growing a bug called pseudomonas aeruginosa, which can be difficult to treat. Take a look at a previous talk we had about this, here.

Vitamin D is an essential nutrient in protecting the immune system.  Our Vitamin D intake comes from our diet and is made via the skin exposed to the sun.  Vitamin D helps fights against infections, regulate the immune system, reduce inflammation in the body after a viral infection.

Many people with PCD have low levels of Vitamin D.

There has been some research to suggest that PCD patients (especially those with a low BMI) should check their Vitamin D levels. Most people in the UK have low vitamin D levels, particularly if you have darker skin or if you don’t get much sunlight on your skin. Vitamin D supplements are cheaply available from pharmacies, such as boots. To find out more, visit here

 

Questions about travelling:

You must advise your travel insurance company that you have PCD. If you do not, then if you make a claim on the travel insurance for anything (even if not PCD related) you could find that your insurance is not valid.

For more information see here.

If you are travelling abroad check whether your nebuliser will work:

  • In Europe (except for Cyprus, Gibraltar & Malta) the nebuliser will work with a standard travel plug adapter.
  • If travelling to the USA, South America, the Caribbean, Cyprus, Gibraltar & Malta, you will need a 110v nebuliser. A plug adapter is not enough and will damage your nebuliser.

Discuss this with your physiotherapy department well in advance of the holiday – they may be able to lend you one or recommend a portable one you can purchase.  Alternatively, they are hireable in the USA from medical equipment companies. If you think that you will need to use a nebuliser whilst travelling (particularly when flying), make sure you check with your airline or travel company ahead of your journey.
 

 

Treatment questions:

It is helpful to think of the nose as ‘one airway’, connecting to your lungs. Runny or congested noses are a common cause of PCD, and it is as important to manage these symptoms as it is to manage your lung health. This is because mucus can build up in your sinuses, causing pain, sinus pressure and infections. It can also run down your throat into your lungs (called a post-nasal drip), which can contribute to lung infections.

Blowing your nose regularly help keeps your airways clear, making it easier to breathe and reducing the risk of getting sinus infections from bacteria building up in the nasal passages. Many people with PCD also use saline to douche their nose and sinuses. As the nasal passages connect to the ears, this can help with ear symptoms too. Your ear, nose and throat (ENT) doctor may give you a leaflet about how to mix your own saline solution, however some PCD patients prefer to buy sachets and a nasal rinse bottle, such as those produced by Neilmed. Others use a saline spray, such as Sterimar. 

If your nose symptoms are not controlled, you might be prescribed a steroid nasal spray by your ENT doctor. However, it is important to avoid buying over the counter medicines (such as decongestant sprays or tablets to reduce mucus), as these might make your symptoms worse. Talk to your ENT doctor about which products might be most effective for you.

It is also very important to drink lots of water throughout the day, so that your airways are hydrated. This can help thin the mucus, making it easier to clear from the lungs and nose. Some people are concerned that consuming dairy products makes them produce more mucus. This has been proven to be incorrect. What’s more, dairy is rich in calcium, which is very important for growing and bone health. Please speak to your consultant if you are concerned about consuming dairy – they may refer you to a dietician. 

Our UK PCD specialists work in collaboration with their EU and US counterparts to agree best treatments for PCD. At PCD Support UK, we also are in regular contact with our charity counterparts in other countries, such as Australia, the USA, Germany, Italy and France, as part of a growing global PCD community. This community of researchers, doctors and patient groups often collaborate, such as the BEAT-PCD Project.

There are a few differences in the treatment and management of PCD across the world. This is thought to be because of the way healthcare is funded in different countries, such as in the USA. For example, many USA-based PCD patients use a vibrating vest to do their physiotherapy treatment. This is not usually recommended in the UK, where it is believed that PCD patients can get more effective lung clearance by doing other techniques. In rare cases, expensive ‘vest machines’ might be recommended in PCD for patients with severely limited mobility. 

If you see something on the internet or social media platforms that is different to the treatment advice you have received from your PCD specialist, we recommend you ask them at your next clinic appointment or alternatively give your PCD specialist nurse a call.

One of the main problems with PCD is that children get regular chest infections. These will be treated with oral antibiotics but for more stubborn infections they may be required to have intravenous antibiotics, where antibiotics are given through a vein. These are normally administered in hospital for a minimum of two weeks. Whilst in hospital, your child will also have more targeted physiotherapy treatment. Some children may require further investigations to work out why they are getting stubborn chest infections. They might have a procedure called a bronchoscopy (‘bronk-oscop-ee’), where they are anaesthetised or sedated and a camera is inserted via the throat into the lungs.

Before your child reaches adulthood, they will begin the process of preparing, planning and moving from children’s to adult services.  This process is called ‘transition’ and can start in their early teens.  

Moving away from a team of doctors and nurses that you have been with for many years can be scary but hopefully, by getting involved in the transition process, you will feel more confident and happier about the move.

To support you through transition, our specialist teams use the Ready Steady Go transition programme.

This programme helps children gain the knowledge and skills to manage their condition to improve their long-term outcomes.

Early Teens Independence with Professor Hogg

Your physiotherapist will work with you to work out the most effective way to do your physio – which may vary depending on your age and personal circumstances. It is important to have regular reviews with your physiotherapist to check that your physiotherapy is effective, fits with your current lifestyle and is keeping you well. They may suggest the use of physio devices which are the most appropriate for you. If you see a device that you haven’t used but would like to try, speak to your physiotherapist about it at your next clinic, as they may be able to provide you with one.  For more information see here.

A ‘huff’ (also known as the forced expiratory technique – FET) is used to help force secretions (phlegm) up the throat so it can be removed via the mouth. The technique is quite simple and involves you taking a breath in and then forcing the breath out through your open mouth using the muscles in your chest and stomach.

The technique is sometimes taught separately, however its most common use is as one of the stages of the Active Cycle Breathing Technique (ACBT).  For more information see here.

Antibiotics are sometimes given to prevent, rather than treat, an infection. This is called prophylaxis (or maintenance therapy).  

There are lots of antibiotics that are used as prophylaxis antibiotics, rather than ‘rescue’ antibiotics (what you take when you’re unwell). Your PCD team should tell you whether you need to be on prophylaxis antibiotics and which ones to take, if so. They will also tell you which antibiotics to change to if you become unwell. From time to time, your antibiotics may be changed, depending on what bacteria you grow in your sputum tests. This is normal. 

Azithromycin is a common prophylaxis antibiotic used to reduce infections in PCD. More information can be found in this research paper.

 

COVID-19 questions:

We are aware that hospitals are identifying ways of avoiding unnecessary exposure to hospitals, where possible. This may mean your clinic is done virtually using video-conference software, by telephone or that your clinic is postponed. If your appointment is in the next week, you should contact the clinic to check whether it is still going ahead. If not, then you should wait a while to see if the clinic contacts you, so that the clinic staff do not become overloaded with requests.

You should also think about whether you feel you need to be seen – if you are not managing your PCD very well, do not delay your appointment. Urgent face to face appointments will still occur when urgently needed or you are starting IV antibiotics at home. Hospitals will maintain strict hygiene standards and will be careful to minimise the risk to you/your child if you need to physically go in.

It is very important not to delay seeking advice or seeing your doctor if you are unwell. Firstly, you should make sure that you have a two week ‘rescue pack’ of antibiotics on hand, and you should start these if you think you are getting a chest infection or symptoms such as cold symptoms, flu-like symptoms, fever, or a new/worsening cough. You may also want to ask your GP to prepare you some pre-filled sputum testing forms and pots, so that you can send off a sputum sample as soon as you begin feeling unwell. It may be worth discussing with your GP whether there are changes to the sputum sample drop off system during this time.

As a high-risk group, those with PCD should make sure they are especially careful to manage their lung health at this time. If you think you are not managing an exacerbation, do not put off getting further advice or treatment because of concerns about COVID-19.

If you do develop any of the symptoms, you need to go to the NHS 111 website for advice as to whether testing for COVID-19 is required. Testing for COVID-19 is accessed via the NHS website not via your GP or your specialist hospital. After you have sought advice, please talk to your specialist hospital straight away, as you may require further treatment, such as a face-to-face consultation, an arranged admission or home IVs.

Advice for this situation is constantly changing. Please follow the latest government advice on what to do by reading this link.

If you think your employer may have unfairly dismissed you, or otherwise treated you unlawfully following the outbreak of COVID-19 you might be able to access advice via your local Citizens Advice Bureau or Law Centre. Though many advice centres may be struggling they will do their best to answer your queries. There are changes to eligibility for claiming benefits. You can find out more information about this on the government website. You can also speak to the Citizens Advice Bureau about what you may be entitled to, or look at the Money Advice Service. Please note that there are many sources of advice and these are just some examples:

Law Centres
Citizens Advice
Government benefits
Money Advice Service

There are some places where you must wear a face covering by law unless you are exempt or have a reasonable excuse. See latest government guidelines here.

If you find wearing a mask difficult, we advise you talk to your clinical nurse specialist.