Lung health in children and young people with neuromuscular disease: The Importance of good lung health

Introduction: Who is this leaflet for and what is its aim?

This patient information leaflet is aimed at children, young people and their families who have a diagnosis of a neuromuscular disease, who are attending a Paediatric Respiratory clinic at Addenbrooke's Hospital.

How does having a neuromuscular disease effect lung health?

Lung health may be affected by a neuromuscular disease especially if you are no longer able to walk. This is due to many factors that such as a weaker cough or problems with breathing when you are asleep.

It is important to have regular respiratory follow up and lung function testing to ensure ongoing monitoring.

How to keep your lungs healthy?

Keeping your lungs healthy is important in preventing lung infections and slowing down respiratory decline.

• Avoid smoking, vaping and second-hand smoke fumes at home and in the car.
• Remain as active as physically possible.
• Regular compliance with prescribed therapies (medications, physiotherapy, nebulisers etc.)
• Notify clinical team of changes with your child’s condition to ensure timely monitoring and intervention

What to expect from respiratory clinic appointments

It is especially important to see a respiratory team when you can no longer walk. During clinic visits your lung health can be monitored by seeing a physiologist who will ask you to complete a range of blowing tests. You will be asked to attend 30 minutes before your scheduled appointment time to allow time for these tests to be completed before you see the doctor. There is often a physiotherapist and a specialist nurse present during your consultation with the doctor.

Blowing test will include

Spirometry

Spirometry consists of asking you to breathe normally through a mouthpiece and then take in as big a breath as you can. When we see that you are completely full, we will then ask you to blow the air out like a blast as quick as you can until your lungs are empty. Once they are empty, we will ask you to breathe back in quickly until your lungs are full.

Respiratory muscle assessment

This test assesses whether the muscles which control your breathing are working correctly.

Sniff nasal inspiratory pressure (SNIP)

The 'SNIP' involves placing a small bung into the opening of your nostril and sniffing in as hard as possible. We ask you to do this in both nostrils and may repeat the test several times. The bung does not hurt.

Mouth inspiratory pressure (MIP)

The 'MIP' involves wearing a nose peg and breathing through a tube attached to a computer. We will ask you to blow out until your lungs are empty and then suck in as hard as you can through your mouth and against a slight blockage. The nose peg does not hurt.

Mouth expiratory pressure (MEP)

The 'MEP' also involves wearing a nose peg and breathing through a tube attached to a computer. For this test we will also ask you to support your lips and cheeks with your hands. We will ask you to take a big breath in and then blow out as hard as you can against a slight blockage.

Peak Expiratory Cough Flow (PECF)

The PECF involved you breathing normally through a mouthpiece. We will instruct you to take as big a breath as you can. When we see that you are completely full, we will ask you to cough as hard and sharp as possible with your lips tight around the mouthpiece.

You will be required to repeat all tests multiple times with the physiologist for accuracy purposes.

Test results

After your lung function, the consultant, will discuss the results and the next steps in your treatment.

Risks

Due to the nature of the tests and the exertion required, some patients may feel dizzy or slightly faint during testing. Coughing is also common. If you have any of these symptoms let the person doing the tests know so you can have a rest.

Treatments that could also be helpful

Physiotherapy

Regular physiotherapy techniques that will be taught by the physiotherapist in clinic visits are important to practice regularly as advised to clear mucus from your chest.

Other Equipment

Cough assist

If your cough becomes weaker and less effective you will be assessed by a physiotherapist to see if a Cough-Assist might be a useful device to help you clear secretions from your chest If this is felt to be a useful device, the physiotherapist will talk through with you what this looks like, how and when to use as well as arranging the equipment to be available to you.

When unwell

If your child develops a cough or cold and has not improved by day 4-5 please call the clinical nurse specialist using the contact details supplied to discuss if any different or increased treatments are required.

Flu and C0VID vaccines

We would recommend routine vaccinations.

Further information

Please feel free to email the team if you have any further questions.

MyChart

If you have not already done so, we would encourage you to sign up for MyChart. This is the electronic patient portal at Cambridge University Hospitals which allows patients to securely access parts of their health record held within the hospitals’ electronic patient record system (Epic). It is available via your home computer or smart phone.

If you are interested in this please let us know and we can provide information, or look on our website: MyChart