Supporting patients with ILD in Primary Care

Although a patient with ILD's care will mostly take place in specialist care, they will need support and monitoring in primary care for ILD and any other comorbidities they have.  This section will help you to know what will make a difference to your patient with ILD's quality of life.

You can use our Pulmonary Fibrosis Passport to structure your conversation with your patient.  You will find some suggestions below of how you can use elements of the patient passport to support your patient in primary care.

'My diagnosis was made by a specialist multidisciplinary team'

Check that your patient understands their diagnosis.  Offer them our Living with Pulmonary Fibrosis leaflet to help explain it.

Here are some suggestions of questions to guide future conversations:

Understanding and education:
•    What have you been told about IPF so far?
•    Do you have any questions about what IPF is and how it might affect you?
•    Have you heard about the treatment options available for IPF?

Symptoms and daily impact:
•    What symptoms are you experiencing currently, and how do they impact your daily activities?
•    Do you experience breathlessness, and if so, when does it feel most challenging?
•    Are you having issues with coughing, and does it interfere with your quality of life?

Lifestyle and support:
•    How is your diagnosis impacting you emotionally? Do you have support from family or friends?
•    Are there specific activities or hobbies you’re worried about managing?
•    Do you currently work, and would you like help with your work-life balance?

Health and treatment history:
•    Do you have any other medical conditions or take other medications that we should consider?

Planning and goals:
•    Are there any short- or long-term goals you have that we can help you achieve despite this diagnosis?
•    Would you like to discuss advance care planning, or would you prefer to revisit this later?

Education and follow-up preferences:
•    How would you like to receive information about your condition (e.g., written materials, online resources, or support groups)?
•    What follow-up or additional support do you think would be most helpful for you at this time?

 '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 guidance from diagnosis onwards, educating patients on managing symptoms, understanding medications (such as antifibrotics), and using oxygen therapy safely. 

ILD nurses offer vital emotional and psychological assistance to patients and their families, helping them navigate the practical and emotional challenges of living with a progressive lung disease. They also play a key role in discussing advance care planning sensitively, supporting patients’ autonomy, and improving quality of life.  

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 the patient and the people who live with and care for them. Action for Pulmonary Fibrosis have a telephone befriending service which provides peer support from a person with lived experience of ILD.

Action for Pulmonary Fibrosis also have 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'

Malnutrition and obesity are both common among ILD patients.

Malnutrition often starts in the community, so it is essential that it is identified and treated early.  This involves screening for malnutrition using a validated tool at diagnosis, and at post-exacerbation and annual reviews and communicating any changes to your patient's ILD team

Patients need to be aware that any unintentional weight loss should be reported in case it needs further investigation such an a chest x-ray to exclude lung cancer.

Malnutrition depletes skeletal muscles, including the muscles that support breathing. This in turn causes increased breathlessness and fatigue, both of which make eating and the preparation of food challenging. Malnutrition decreases immunity, which can make your patient more susceptible to infections, which also decrease appetite.  

The Malnutrition in COPD Pathway can also be used in ILD, and recommends the LEARN acronym for assessment:

Look at the individual – what do you see? Have they got muscle wasting, are they thin or frail looking?
Eating – ask about what they are eating, are they consuming foods from all food groups, missing meals?
Appetite – ask about appetite, has it changed?
Relatives – family members can be a source of information and support; do they have any concerns?
Nutrition – give appropriate nutritional advice. If you are not skilled or confident to do so, seek advice from someone qualified e.g. your local dietitian.

Patients aged over 65 should take vitamin D supplements.

Obesity in ILD patients often results from inactivity and an increased appetite due to oral steroid use. Even minor obesity can lead to increased respiratory effort in those with ILDs, causing:

• Greater breathlessness during physical activity
• Decreased exercise capacity and activity levels
• Worsening of overall symptoms

Significant weight loss can be difficult to achieve and should be approached with sensitivity. Exercise programs like pulmonary rehabilitation can be beneficial. Patients with a BMI over 30 should be referred to a dietitian for expert guidance.

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 increases disease progression. Many patients will need home oxygen, which makes stopping smoking essential.

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 usually eligible for COVID-10 vaccination.  Find information for your patients here.

Respiratory Syncytial Virus

All adults aged over 75 years are eligible for RSV vaccination, with an additional catch up campaign for those already aged 75 to 79 from 1st September 2024.  Programme documents, posters and patient group direction are available 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 often than their specialist team do.  Ensure that you  identify any hypoxemia early by checking their resting and exertional oxygen saturations whenever you see them.

If you can't perform a sit to stand test, then you can record their saturations when they first come into your room (if they have walked to do so) and then again when they are sitting at rest.

You can also encourage your patient to monitor their levels at home and report any significant drop. 

'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 very serious event.  ILD. Flare-upsare frequently life-threatening and may occur due to factors like infections, environmental exposures, or unknown triggers. Key symptoms  include:

•    Rapidly worsening breathlessness
•    Increased cough
•    New or worsening hypoxemia

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 in interstitial lung disease aims to provide symptom relief, comfort, and support, distinguishing it from end-of-life care, which is specific to the final months or days of life. 

Palliative care should be integrated early to the patient's care, focusing on symptom management. This includes rehabilitation, to stay independent for as long as possible, with medication for symptoms (e.g., opioids for dyspnea), and support from physiotherapists and occupational therapists, often at a hospice.  It's important that your patient understands that hospices are not there solely for end of life care. 

Do not assume that conversations about end of life has been discussed with their specialist team and give space to your patient to be able to talk freely if they want to.  It may be that they feel more comfortable talking to an HCP in primary care that they have an established relationship with than their specialist team.

Informal carers bear a significant burden and benefit from tools like the Carer Support Needs Assessment Tool which helps to identify, express and prioritise areas of their lives in which they need more support and express their concerns for their loved one. 

Planning ahead with documents like advance statements and decisions (e.g., DNACPR) allows individuals to state their care preferences. 

Ultimately, earlier provision and better understanding of palliative care options, such as oxygen therapy, can enhance quality of life for ILD patients and their families.