Supporting patients with ILD in Primary Care

'My diagnosis was made by a specialist multidisciplinary team'

Check that your patient understands their diagnosis and knows how to access specialist support. Offering information early can help patients feel more informed and confident about managing their condition.

Use our Living with Pulmonary Fibrosis leaflet and Pulmonary Fibrosis Passport to support these conversations. The questions below can help you identify unmet needs, understand how the condition is affecting daily life and ensure your patient receives the support they need.

Suggested questions:

• What symptoms are having the biggest impact on your daily life?

• How are breathlessness, cough or fatigue affecting your usual activities?

• How are you coping emotionally with your diagnosis? Do you have support from family or friends?

• Do you have any questions about your condition or treatment?

• Are there any activities, work commitments or goals that you are worried about managing?

• What additional support or information would be most helpful for you at the moment?

 'I have access to a specialist nurse for information and support. I know where to find more help and I have information about local patient support groups'

Specialist ILD nurses provide expert support from diagnosis onwards. They help patients understand their condition, manage symptoms, use treatments such as antifibrotic medications and oxygen therapy safely, and access appropriate services and support.

They also provide emotional and practical support to patients and their families, helping them navigate the challenges of living with a progressive lung disease. Specialist nurses play an important role in advance care planning, supporting informed decision making, patient autonomy and quality of life throughout the disease journey.

You can find the nearest specialist ILD service using this map if your patient does not have a specialist nurse.

Living with ILD can be a lonely and isolating experience for both patients and their families and carers. Action for Pulmonary Fibrosis has a telephone befriending service which provides peer support from a person with lived experience of ILD.

Action for Pulmonary Fibrosis also has a series of webinars on a range of issues that people with ILD face such as oxygen therapy, breathlessness and managing medication side effects.

A+LUK support groups for people with ILD run across the UK.  Share this link with your patient so that they can find their local group.  

The A+LUK Helpline provides an opportunity for patients to spend time talking to a specialist respiratory nurse or healthcare advisor about any fears, concerns, or difficulties they are experiencing. They can explore any social and practical issues that might be going on for your patient and help them get the support they need.

'I have been given information about how my diet can help my condition'

Both malnutrition and obesity can affect people with ILD and contribute to worsening symptoms, reduced exercise capacity and poorer quality of life.

Screen for malnutrition at diagnosis, following exacerbations and during annual reviews, communicating any concerns to the patient's ILD team. Advise patients to report any unintentional weight loss, as this may require further investigation.

Malnutrition can lead to muscle loss, including the muscles used for breathing, resulting in increased breathlessness, fatigue and susceptibility to infection. 

Obesity can increase the work of breathing and make physical activity more difficult. Encourage patients to remain as active as possible and consider referral to pulmonary rehabilitation where appropriate.

The Malnutrition in COPD Pathway can also be used in ILD and provides practical guidance on assessment and management.

Refer patients with nutritional concerns, significant weight loss or a BMI over 30 to a dietitian for specialist advice.

Our leaflet Eating Well for Healthier Lungs is full of information to support your patient.  Action for Pulmonary Fibrosis also have nutritional advice on their website.

'If I smoke, I am offered support and treatment to stop every time I see my doctor or nurse about my pulmonary fibrosis'

Quitting smoking is vitally important in ILD, as smoking is associated with poorer outcomes in ILD. Stopping smoking is particularly important for patients who require oxygen therapy because smoking while using oxygen poses a significant fire risk.

Very Brief Advice (VBA) is a simple and effective approach used in smoking cessation to help individuals quit smoking. It involves healthcare professionals delivering concise advice to patients, typically within 30 seconds. The VBA model follows a three-step process:

• Ask about smoking status.
• Advise on the benefits of quitting and the best ways to do so.
• Act by offering support and resources to help the patient quit.

This method aims to quickly and efficiently prompt smokers to consider quitting and take action toward cessation. Free online training is available from the National Centre for Smoking Cessation and Training (NCST) 

There are a variety of methods available to stop smoking, from nicotine replacement therapy, to fast acting oral and nasal products, to prescription only stop smoking medications and e-cigarettes. See NCST's guide for more information.

Make sure you know about your local smoking cessation services and the options available to help your patient quit.  Share the A+LUK booklet How to Stop Smoking with your patient.
Remember to revisit smoking habits every time you review your patient so they know that help is there when the time is right for them. 

COVID-19

People with ILDs are usually eligible for COVID-19 vaccination.  Find information for your patients here.

Respiratory Syncytial Virus

This vaccine is offered to pregnant people, adults aged 75 or people who live in an care home for older adults.  Programme documents, posters and patient group direction are available here.

Shingles

Vaccination for shingles is available for individuals turning 65 from 1st September each year.  Guidance and resources for HCPs can be found here. 

‘My oxygen levels are assessed at my clinic appointments. If appropriate, I have been referred for an oxygen assessment and given oxygen therapy.’

You may see your patient more frequently than their specialist team. Check for hypoxaemia by measuring oxygen saturations at rest and, where appropriate, following exertion.

If you are unable to perform a sit to stand test, consider recording oxygen saturations when the patient arrives (if they have walked to the appointment) and again after they have been sitting at rest.

Some patients may be advised by their specialist team to monitor their oxygen saturations at home. Encourage patients to report any significant or sustained reduction in oxygen saturations, or worsening breathlessness, to their specialist team promptly.

'If I need it, I have medication to help with my symptoms. I know what do if my condition gets worse or if my symptoms flare up '

An ILD flare up, or acute exacerbation, is a serious event that requires urgent medical attention. Flare ups are often life threatening and may be triggered by infection, environmental exposures or occur without an identifiable cause.

Symptoms may include:

• Rapidly worsening breathlessness
• Increased cough
• New or worsening hypoxaemia
• Reduced exercise tolerance or ability to carry out usual activities.

Ensure that your patient knows what to do if they become worse.  Record this in their self management plan, ensuring that they have the relevant phone numbers for their specialist team and know to seek help immediately they become unwell.

'My family, carers and I can access specialist services to help with and advise about controlling my symptoms and wellbeing support.'

Palliative care aims to improve quality of life by helping people manage symptoms and maintain independence. It is not the same as end of life care and should be introduced early alongside active treatment where appropriate.

Palliative care includes rehabilitation, to stay independent for as long as possible, with medication for symptoms (e.g. opioids for dyspnoea), and support from physiotherapists and occupational therapists, often at a hospice.  It is important that patients understand that hospices provide support for living well with a serious illness and are not solely for end of life care.

Do not assume that discussions about future care preferences or end of life care have taken place with the specialist team. Primary care professionals often have established relationships with patients and may be well placed to start or continue these conversations.

Family members and carers can experience a significant emotional and practical burden. Consider signposting them to support services and tools such as the Carer Support Needs Assessment Tool (CSNAT).

Advance statements and decisions can help ensure that a person's wishes and preferences are understood and respected.

Early access to palliative care can improve symptom control, quality of life and support for both patients and their families.

The A+LUK Helpline provides an opportunity for patients to spend time talking to a specialist respiratory nurse or healthcare advisor about any fears, concerns, or difficulties they are experiencing.