Primary Ciliary Dyskinesia Service

Welcome to the Primary Ciliary Dyskinesia Service

We have an experienced multidisciplinary team made up of specialist doctors, specialist physiotherapists and nurses. We hold regular outpatient clinics for patients with PCD within the cystic fibrosis department at Wythenshawe Hospital. At each appointment, you can expect to be seen by a doctor, physiotherapist and nurse. We will do breathing tests and take sputum samples to find out what bacteria are present. We will then recommend treatments tailored to each individual patient, based on factors including symptoms and severity of the condition.

For those patients requiring inpatient therapy, we have a dedicated ward for patients with cystic fibrosis that also treats patients with PCD. Each patient has their own ensuite room. Inpatients are managed by a specialist multidisciplinary team comprising doctors, physiotherapists and nurses.

The team also works with other professionals, including ENT surgeons, clinical genetics and fertility services.

What is Primary Ciliary Dyskinesia?

PCD is an inherited genetic disorder of the structure and/or function of the cilia, which are the tiny, microscopic moving structures lining the airways, ears, sinuses and some other areas of the body. The sweeping, wave-like motion of cilia is important for keeping these areas clean and free from infection.

In patients with PCD, this wave-like motion does not happen in the normal way, and in some, it does not happen at all. Without properly functioning cilia, people with PCD are unable to protect their respiratory system. The build-up of mucus in the chest increases the likelihood of infections and possibly lung damage (bronchiectasis).

It can lead to hearing loss and a constantly blocked or runny nose. Some patients with PCD also have other conditions such as dextrocardia (heart on the right side of the body) and situs inversus (a mirror image arrangement of their internal organs). It can also predispose to infertility.

What tests might I have?

We use tests to help monitor patients with PCD, including:

• Breathing tests to monitor your lung function
• Sputum cultures to look for infection

We may occasionally also arrange for you to have other tests such as blood tests, chest X-rays or CT scans.

Possible treatment options

Treatments include airway clearance techniques to help move and clear sputum from your lungs. Our physiotherapist will assess and teach you the most appropriate airway clearance techniques for you. They may also provide you with additional handheld devices and medications to help move your sputum.

We will also advise about using antibiotics to treat any airway infection. We will also assess if we think you may benefit from using inhalers if there is narrowing of the airways, and other interventions such as exercise, pulmonary rehabilitation and supplemental oxygen. We will also advise on treatments to help any nasal and sinus symptoms.

Research

We may ask you if you are interested in participating in any research studies, although there is no obligation to take part.

Our Team

Our multidisciplinary team is made up of specialist doctors, specialist physiotherapists and nurses.