Children's primary ciliary dyskinesia service

Southampton University NHS Hospital provides dedicated follow up care for children with Primary Ciliary Dyskinesia (PCD).

For confirmation that children have PCD, they are first seen in the PCD diagnostic service (see Diagnosis| ).

We provide outpatient clinics every 3 months.

At these you will been seen by members of our team including:

• paediatric respiratory consultants,
• ENT (ears nose and throat) consultants,
• the PCD nurse specialists, and
• a respiratory physiotherapist.

Between clinic visits, the PCD team can be contacted via the secretary for additional advice and support.

Treatment of PCD

The mainstay of treatment for children with PCD is chest physiotherapy, antibiotic and ENT (ear, nose and throat) management.

Physiotherapy

The physiotherapist will teach you techniques to clear your child's chest to prevent a build up of secretions, which can lead to infections. If your child is old enough they will be taught how to do physio themselves, often using equipment such as a PEP mask (a positive expiratory pressure mask) to breathe into. People with PCD often need to do chest physiotherapy twice daily and more frequently during a cold or chest infections. The technique for chest physiotherapy is reassessed regularly at each clinic visit.

Antibiotics

Children with PCD are treated with antibiotics as soon as a chest infection is suspected. This is because the microscopic hairs (cilia) lining the breathing tubes are not working in a co-ordinated fashion to clear the increased secretions. A sputum test or a cough swab is taken by your GP before starting any antibiotics. The results of these tests will help your child to be treated with the right antibiotic to fight the infection. Some children with PCD are on permanent antibiotics to help prevent infections. At each clinic visit, the doctor will reassess your child's chest to check they are on the best treatment.

ENT management

Children with PCD can experience ENT (ear, nose and throat) problems. This is because the cilia are not working properly in the Eustachian tubes (connects the ears with the nose), sinuses and nose. As a result, children can experience hearing problems due to a build up of fluid in the middle ear, sinusitis and a constant runny or blocked nose. For these reasons, children have regular hearing assessments and are reviewed by the ENT doctor at clinic visits. Medications are available to help treat sinusitis and nasal problems. In some circumstances, children are given hearing aids to help overcome hearing impairment.

Outlook

If children with PCD are treated appropriately, children and adults with PCD can lead full, active lives.