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This content, which is intended for UK healthcare professionals only, has been developed and funded by Boehringer Ingelheim. Dr Steve Holmes, GP, received payment for involvement in the development and accuracy review of this content. Mr Ron Flewett also received payment for involvement in the development of this content as a trustee and spokesperson of the Pulmonary Fibrosis Trust.

Managing patients with IPF

An early diagnosis of idiopathic pulmonary fibrosis (IPF) will provide patients with time to consider their condition and its effect on their life and plan accordingly, as well as provide more options for treatment and management, which could relieve the burden of symptoms, as well as potentially slow down disease progression.1

Care in IPF should include:2

  • Access to an interstitial lung disease (ILD) specialist nurse
  • An assessment for home and ambulatory oxygen therapy at each follow-up appointment with their specialist and before the patient leaves hospital following an exacerbation of the disease
  • Access to pulmonary rehabilitation programmes which provide services designed specifically for IPF
  • Access to services that meet their palliative care needs and the needs of their family or carers

The National Institute for Health and Care Excellence (NICE) also recommends that ‘best supportive care’ is made available to all patients on diagnosis including information and support, symptom relief, management of comorbidities, withdrawal of ineffective therapies, and end-of-life care. It advises doctors to consider psychosocial needs and referral to relevant services as appropriate.3

As a GP, you can help continue to assess your patient for comorbidities, which may include anxiety, bronchiectasis, depression, diabetes, dyspepsia, ischaemic heart disease, lung cancer and pulmonary hypertension.3

“Post-diagnosis, we can play an important role in helping patients manage their condition effectively. This could include signposting to support groups, helping them access care and support at home and empowering them to take steps to manage their condition to reduce unwanted emergency hospital admissions.

It is important to ensure that advice has been provided on the potential benefits and, if appropriate, supporting a Blue Badge application. Local social services and occupational therapy teams may be able to maintain independence with adaptation to the home environment. We also know that the condition does not only affect the patient but family and carers.

A holistic approach is important. Try to look beyond the condition itself and check for possible related health and wellbeing challenges such as depression and pain. Don’t forget that the patient’s family will be under a lot of pressure to care for their loved one – they might need help and support too; always try and make time to check in with them to ensure they are coping as best as they can.”

Dr Steve Holmes, GP

Treatment options

Patients may benefit from a combination of different approaches to help manage their condition, including medication, pulmonary rehabilitation and oxygen therapy. Lung transplant may also be a viable option for some patients, though this requires careful assessment. Patients and their families may need support with palliative and end-of-life care.3

Antifibrotic medications

Antifibrotic drugs are available for patients with IPF.4 They are only available via specialist ILD centres, rather than general hospitals. In 2016, there were 30 hospitals providing specialised care across the UK.5

Oxygen therapy

Oxygen therapy can often be useful in the care of people with advanced IPF.6 Oxygen therapy can relieve symptoms of breathlessness and helps patients to be more active, increasing their independence.2,6 It is not uncommon for patients with IPF to need a higher level of oxygen than you may be familiar with in chronic obstructive pulmonary disease (COPD).7

Oxygen therapy also makes it possible to take part in pulmonary rehabilitation classes. Where a patient has been hospitalised after an exacerbation, a specialist should review their oxygen requirements. This may also prevent a further exacerbation.2

Pulmonary rehabilitation therapy

Pulmonary rehabilitation (PR) is a short programme of classes which provides education and exercise for patients, so they are better able to manage their condition.6 PR may improve their exercise tolerance, pulmonary and ventilatory capacities, dyspnoea and quality of life.8 NICE advises that patients with IPF should be assessed for PR at the time of diagnosis.3

Lung transplant

On average, patients with IPF can survive 4.5 years after a lung transplant and not all patients are eligible.3,9 The patient’s management team will discuss the possibility of lung transplant with them if they wish to explore this option and they are eligible.3

Palliative care

The patient and their family may benefit from palliative care at any stage, and not just at the end of their life. This could include help with symptom management and control, psychological and spiritual support, and giving practical information.2

In palliative care, a multidisciplinary approach is important, including input from primary care, the ILD specialist nurse and, if needed, specialists in palliative care.2

“While as GPs we would not be involved in most of the treatment decisions outlined in this section, palliative care is an area where we would be extensively involved, so it is important that we follow up with our patients to help them manage their symptoms the best they can, also providing advice if they find some of the therapies to be cumbersome, such as oxygen therapy, which is key to protect their organs from the damage caused by low levels of oxygen in their blood. Keeping in contact during the illness, if it does progress, makes for easier care in the community.”

Dr Steve Holmes, GP

What can patients do?

You can remind and empower patients with IPF to maintain their health and emotional wellbeing:

  • Stop smoking – NICE recommends offering smoking cessation advice and treatment3
  • Take regular, supervised exercise, following your advice about level and type of activity that could benefit them the most10
  • Get vaccinated against flu11
  • Talk to someone about what they are going through – support groups can be a lifeline; family members and close friends can also help them practically and emotionally11

A list of resources can be found on the ‘Living with IPF’ tab

“Living with IPF can take its toll on your mental and emotional wellbeing as well as your physical health. Unfortunately, this is not only true for patients, but also their families, who often become full-time carers. Knowing that things are never going to get better, and they are likely going to get worse is difficult to accept. It’s common for people with life-threatening conditions to just give up, so encouraging people with IPF to continue to live their lives the best way they can is important.

Doing everything we can to give our bodies a chance to fight off infections and to boost our immunity is especially important – eating well, gentle exercise if it’s possible for the patient, getting vaccinated in the winter, and speaking about what we are going through are all important steps in staying as healthy as we can. Hospice care is a great option, especially when you are going through a particularly difficult time and can offer your carers a brief respite from looking after you.

Organisations like the Pulmonary Fibrosis Trust offer help and support to people with IPF and their families. Knowing that you’re not alone and there’s someone to speak to can be a real lifeline.”

Ron Flewett, trustee and spokesperson of the Pulmonary Fibrosis Trust,
who is living with IPF

 

NP-GB-100108
December 2019

References

1du Bois R M. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev 2012;21(124):141-146

2National Institute for Health and Clinical Excellence (NICE). Idiopathic pulmonary fibrosis in adults. Quality standard [QS79]. Available at: www.nice.org.uk/guidance/qs79/chapter/introduction. Last accessed November 2019

3National Institute for Health and Care Excellence (NICE). Idiopathic pulmonary fibrosis in adults: diagnosis and management. CG163. Available at: https://www.nice.org.uk/guidance/cg163/evidence/full-guideline-pdf-190086877. Last accessed November 2019

4Maher TM, Strek ME. Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat. Respiratory Research 2019;20(1):205

5British Lung Foundation. Pulmonary Fibrosis Services in the UK. Available at: >https://www.blf.org.uk/sites/default/files/Table%20of%20UK%20PF%20services%20in%20the%20UK%20v2.1.pdf. Last accessed November 2019

6Action for Pulmonary Fibrosis. Patient survey report 2018: Giving patients a voice. Available at: www.actionpulmonaryfibrosis.org/wp-content/uploads/2019/03/APF-Report-Final-070319-crop.pdf. Last accessed November 2019

7O’Driscoll BR, Howard LS, Earis J et al. British Thoracic Society Guideline for oxygen use in adults in healthcare an emergency settings. BMJ Open Resp Res 2017;4:e000170. Doi:10.1136/bmjresp-2016-000170

8Vainshelboim B et al. The effect of pulmonary rehabilitation on exercise tolerance pulmonary function dyspnea and quality of life in patients with idiopathic pulmonary fibrosis. European Respiratory Journal 2013;42: P3569

9Kistler KD et al. Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature. BMC Pulm Med. 2014;14:139

10Vainshelboim B. Exercise training in idiopathic pulmonary fibrosis: is it of benefit? Breathe 2016;12:130–138

11IPF - Idiopathic Pulmonary Fibrosis. Breathe (Sheff) 2019 15(2):153–160



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