Adult Primary Ciliary Dyskinesia Management Service (PCD) - Four Centres

The Service Specification for Primary Ciliary Dyskinesia management service (adults) has been developed to support improvement in services for patients with Primary Ciliary Dyskinesia which is genetic condition in which the microscopic cells in the respiratory system called cilia do not function normally. Ciliary dysfunction prevents the clearance of mucous from the lungs, paranasal sinuses and ears.

Recurring respiratory infections can lead to an irreversible scarring and obstruction in the bronchi (bronchiectasis) and severe lung damage. Cilia are also present in the ventricles of the brain and in the reproductive system so ciliary dysfunction can also affect other body systems.

The service model is described in the Service Specification 1637 for Primary Ciliary Dyskinesia management service (adults) approved by NHS England and published on the NHS England website https://www.england.nhs.uk/publication/adult-primary-cili... This service is provided by Highly Specialist Primary Ciliary Dyskinesia Management centres proving expert management advice to patients including outreach when delivered as part of a provider network.

This provision applies to adults. Some patients will transition into the service from the paediatric HSS PCD service.

Primary Ciliary Dyskinesia (PCD) is a rare hereditary disorder of ciliary dysfunction leading to multisystem abnormalities which include chronic respiratory, sinus and ear infections, cardiac abnormalities, infertility and ectopic pregnancies. Specialised care is important for this group of patients and has a direct impact on disease progression, morbidity and mortality.

Appropriate management of this patient group has been demonstrated to reduce disease progression and should therefore reduce premature death and enhance the quality of life for patients with PCD. The procurement will be split into four lots ((North, Midlands and East, South and London).