What is Primary Ciliary Dyskinesia?
PCD is a rare, inherited disease resulting in abnormal ciliary motility. Cilia can be dyskinetic, immotile or even absent completely. This prevents
effective mucociliary clearance from the lungs, paranasal sinuses and ears and can result in repeated infections.
Some signs and symptoms of PCD may include;
• Productive, wet cough (even when well)
• Breathlessness/ wheeze
• Hearing problem/ recurrent ear infections
• Occasionally organ reversal known as Situs Inversus or dextrocardia
Repeated respiratory infections can lead to chronic airways infection and progressive lung disease and bronchiectasis.
The European Respiratory Society (ERS) and NHS England recommend highly specialised management is needed for patients with a suspected, or known, diagnosis of PCD. Early diagnosis and appropriate treatment can reduce morbidity, mortality and disease progression. Currently, there is little research into treatment for PCD and practice varies from hospital to hospital.
Prof Daniel Peckham (Consultant)
Dr Ian Clifton (Consultant)
Dr Giulia Spoletini (Consultant)
Niccola Burke (PCD Nurse Specialists)
Alison York (CF Nurse Specialists)
Rita Mele (CF Nurse Specialists)
Emma Farrell (Dietitian)
The Leeds Primary ciliary dyskinesia service was set up in 1998 and provides secondary and tertiary care for people with PCD living in Leeds and west Yorkshire region.
The consultant team
The clinics are run by Prof Daniel Peckham, Dr Giulia Spoletini and Dr Ian Clifton who both have specialist interest and training in the management of bronchiectasis and PCD. The clinics are supported by specialist nurses and a new Physio who were appointed in 2020.
A dedicated bronchiectasis clinic is held every other Thursday afternoon at Seacroft Hospital. At each outpatient visit, a patient undergoes sputum analysis, respiratory function tests, oxygen saturation monitoring, weight and height measurements. The service is supported by a specialist nurse. Patients can review their results in graph format and a clinic letter/report is sent to both the patient and their GP.
Port flushes, end of home IV antibiotics and annual assessment are undertaken in the outpatients.
Emergency drop-in arrangements are in place both in the outpatient clinic and on the ward for those who are registered on the home IV program. Telephone advice is available to both GP and patient.
The Clinical Nurse Specialist team, run the home IV service and aims to encourage self-care and responsibility. A formal process of assessment is undertaken to ensure suitability and safety.
Links to key services
The PCD team works closely with a large number of specialist clinics, including microbiology, vascular radiology, immunology, ENT and rheumatology.
Patients undergo a formal nebuliser trial with pre and post lung function before starting new nebulised medication. A clear guideline for the indication and dosing schedule for nebulised antibiotics has been developed.
The Leeds unit submits national audit data. The service is run using e records.
Primary Ciliary Dyskinesia (PCD) is an inherited condition associated with the abnormality of cilia.
PCD Family Support Group >>
British Lung Foundation >>