This was a really successful and fun event, held at Eureka, the Children’s Museum, in Halifax. It provided an informal setting for people to meet others with PCD.
Fiona Copeland, Chairman of the PCD Family Support Group, opened the meeting and thanked the Leeds team for organising the day. She encouraged the children to make their own wishes on the Rays of Sunshine form, and to decorate the physiotherapy adjuncts (like acapellas).
The day began with a personnel update as Andy Bush had retired from the PCD Medical Board and Mick Wilkin had retired as Treasurer of the PCD Family Support Group. She thanked them both for the fantastic help that they had given. Beatrice Redfern will be the new Treasurer and the new PCD email address is email@example.com Fiona asked for patient representatives to volunteer in each of the specialist centres as well.
Fiona went on to explain that the Family Support Group raises awareness, provides support to patients, their families, and the NHS, and promotes research. It also helps to manage the implementation of the adult service. It is a voluntary organisation that needs to fund raise in order to carry out these activities.
Fiona then introduced the first speaker, Dr Miles Denton, Consultant Microbiologist, at Leeds Teaching Hospital. He gave a fascinating talk on “Bugs and Stuff”. He explained how bad bugs make you cough and feel unwell, and how they need to be treated with antibiotics. The bugs are identified by analysing sputum which is grown in petri dishes. Once the bugs have been identified, they are tested to see which antibiotics they are sensitive to. PCD guidelines state that antibiotics should be given in two week courses to treat infections. If the oral antibiotics are ineffective, then intravenous antibiotics can be given. He outlined the common bugs in PCD and the antibiotics used to treat them. He emphasised the need to prevent bugs by staying healthy, doing physiotherapy to clear the chest, doing exercise and sports, eating healthily and having vaccinations, especially the flu jab.
He explained that Azithromycin is sometimes used as a long term antibiotic and anti-inflammatory to reduce exacerbations. Research has been carried out to assess the efficacy of this treatment, and the results will be available next year. Benefits and risks have to be assessed when using antibiotics. Common side effects can be nausea, diahorrea, etc. Some bugs affecting PCD patients can be more resistant and can be controlled but not cured.
After coffee, the Eureka team provided an entertaining and interactive insight into the history of medicine, from Ancient Egypt, the Roman Empire, Tudor England and the Victorian era.
Lynne Schofield, Physiotherapist at Leeds Children’s Hospital, then gave an interesting presentation on physiotherapy, including “lungs for life” plans. She emphasised that you only have two lungs for your whole life, and that it is really important to look after them. She discussed with the children what everyone has to do to keep their lungs healthy, and what extra things people with PCD have to do. She asked them to think about making a promise to their lungs, and to make a plan for how they will keep their promise to keep their lungs healthy. This was an interactive session, and some suggestions were “helping my sister to take her nebuliser every day” and “playing football every day, even it if it’s raining”. The children wrote their promises on the bear lungs, put them inside the bears along with a courage star (to help them keep their promise), and then stuffed them. Each bear received a birth certificate and a name, and a the delighted children were able to take them home.
After lunch Alison Truscott, Clinical Nurse Specialist and Katy Waller, Staff Nurse, did an entertaining and useful transition workshop, as well as an interactive quiz on PCD. This was followed by a “suitcase for life” detailing the need for children to become more independent in looking after themselves as they prepare for adulthood. The suitcase was packed with the things that the children needed to do in order to prepare for this, starting from the age of 11. Children need to understand about PCD, their medication, physiotherapy and how to look after themselves. They need to start to think about taking responsibility e.g going to the GP and pharmacy on their own sometimes. They need to tell their teachers about their condition and make them aware that stress affects the immune system. They may need to go out of an exam to cough and clear. They may need a doctor’s letter if they go on school holidays or trips. If they go to university they will need to look after themselves. They can request a disabled room that affords them privacy to do their physiotherapy, and a larger shower that dries out properly to cut down the risk of infection from this. Specialist teams can give advice on careers. It is important to disclose your medical condition when applying for a job. There are discrimination laws to protect you. A healthy lifestyle is important, including drinking 2 litres of water a day, having a good diet with “5 a day” and exercising for 30 minutes twice a day. Smoking as well as vaping (as this is chemicals being inhaled) are definitely not a good idea if you have PCD. By the age of 14/15 years it is recommended to go to some clinics on your own to prepare for adulthood and the transition to being seen by the adults’ teams.
Following this, Dr Eduardo Moya, Consultant Paediatrician, at Bradford Royal Infirmary, then gave a talk, “Why bother?” He explained about why it is important to treat PCD, and the effect it has on quality of life. He explained that the PCD cough never goes away and becomes “background noise”, but it is important not to ignore it. There is currently no cure for PCD, but there may be one day. In PCD the cilia don’t work properly and are unable to clear the lungs. This can result in permanent lung damage and breathing difficulties. In PCD there is a cough and a runny nose from birth, and colds can cause secondary bacterial infections. If weight loss occurs, then infections become more frequent and a downward spiral can result. Physiotherapy to clear the chest is very important. Hypertonic saline can help with this to make the secretions thinner and easier to move. He emphasised that physiotherapy should be part of your normal daily life like brushing your teeth.
He explained that the normal cilia work like escalators to remove debris from the lungs, but in PCD they don’t work properly. Bronchiectasis (lung damage) can result because the lungs can’t clean themselves properly. CT scans are used to assess the amount of bronchiectasis which causes permanent dilation of the airways. The air tubes become wider and become blocked and atelectasis (lung collapse) can result. He emphasised that you only have one pair of lungs and it is important to look after them! There is a new drug that can help some cystic fibrosis patients, and it is not known if it can help PCD patients, but it is very expensive.
Following these talks, Fiona then hosted a lively question and answer session with Dr Moya. Audience questions included the importance of keeping the school informed about PCD and its effects. It was also noted that a local respiratory nurse can go into the school to help with this. Dr Moya answered a question about appetite in PCD and explained that appetite can be reduced. Sputum in the stomach can reduce appetite and cause vomiting. Coughing causes pressure on the oesophagus and results in acid reflux.
Fiona closed the meeting and thanked everyone. She also reminded them about the T shirt competition. The children then left to explore the museum clutching their bears. This was a very successful, enjoyable and informative day, with many new families attending. Thank you to everyone involved in making the day a great success!
Written by Myra Tipping
10th May 2018