Developing a new pulmonary rehabilitation program tailored for interstitial lung disease with Newcastle upon Tyne Hospitals' Interstitial Lung Disease service

The main aims are:

• To ensure patients living in Newcastle upon Tyne and surrounding areas have access to a dedicated ILD specific PR as per the NICE guidelines and recommendations.
• To try and increase overall activity levels and participation in activities of daily living.
• To improve overall experience of ILD patients through a condition specific PR.
• To provide a full and thorough education timetable appropriate for ILD patients and their carers.
• To develop a service that could be evaluated and modified depending on outcome measures that could be transferable to other ILD centres if successful.
• To publish our findings as to whether an inspiratory breathing device and pedometers aid outcomes with conventional PR.

All the above would be measured by completing questionnaires and using quantified ILD specific outcome measures throughout the PR programme. It was agreed that we would modify and alter aspects of the group as they arose.

Pulmonary Rehabilitation (PR) is a multidisciplinary service that improves symptoms, function and overall quality of life (QoL) for patients. After a detailed assessment an individual program of exercises is devised alongside educational talks. It is group therapy which requires twice weekly attendance for 6-12 weeks. Both aerobic and strengthening exercises are instructed. Progression is led by the physiotherapist with a view of optimising function and overall QoL.

Interstitial Lung Disease (ILD) is a group of chronic respiratory conditions including Idiopathic Pulmonary Fibrosis (IPF). The main symptoms are distressing breathlessness, cough, fatigue, anxiety and depression. Mean survival in the UK is approximately 3 years from diagnosis with only 20% of patients surviving past 5 years. Management of IPF is challenging with treatments focusing on improving physical and mental health of patients. Patients with ILD in and around Newcastle upon Tyne have access to a regional service which provides treatment and support services. Access to ILD specific pulmonary rehabilitation is one of five standards of care for IPF (NICE 2015).

There is extensive evidence for PR in patients suffering from chronic obstructive pulmonary disease (COPD) but much less for ILD. Historically patients with ILD struggling with symptoms were referred for PR in the acute hospitals alongside patients with COPD. In the Quality standard by NICE in 2013 (QS79) it specifically states that PR programmes [should] provide services that are designed specifically for idiopathic pulmonary fibrosis.

Despite being a regional centre for ILD patients we were not meeting this standard of care. As a result we were unable to optimise the care delivered to this patient group. Whilst accessing our established PR, our ILD patients recognised themselves being surrounded by patients with COPD whose needs differed greatly from theirs.

An interest group was set up with a Specialist Respiratory Physiotherapist from the PR team, the ILD medical team, Newcastle University Partners and palliative care teams to develop a new service. As a collaborative team we recognised the potential to deliver PR more effectively, using best evidence and quality standards. A Clinical Research Associate joined the team in 2016 with the aim to assist in the development, delivery and evaluation of the new service.

The team evaluated and reflected on what we previously did well but also what we could improve in designing a new ILD specific PR service. Through meetings and discussions over several months a plan was made on how the service would run with a view of evaluating it and modifying as required. Practical aspects such as location had to be considered. Through collaboration with Marie Curie there was opportunity to run the programme from their day unit. The small gym is very well equipped with aerobic and strengthening machines. With the number of patients with ILD referred to PR much lower than COPD patients, we felt cohort programmes would be sufficient to meet demand and keep waiting lists at acceptable levels.

Our Clinical Research Associate reviewed research and guidelines specific to PR in ILD. Current guidelines for PR recommend 6-12 weeks in duration but this is not specific to ILD. We felt 8 weeks would allow us to deliver a good quota of education sessions and see a meaningful improvement in patients’ overall QoL. A comprehensive framework was developed which included the patients’ assessment to include lung function, muscles strength, six minute walk testing and maximum inspiratory pressure. This would be repeated at the end of the 8 weeks. Exercise training includes where possible endurance and resistance training in the gym with a comprehensive home exercise program using therabands and pedals. Each week the physiotherapist progresses the exercises. 

Health questionnaires specific to this group iPOS and K BILD are completed. With the Clinical Research Associate in the team we want to write a paper on what we find with our service and so need to ensure there were measurable outcomes. We use an inspiratory muscle training device (Powerbreathe) and pedometer as well as a physical activity monitor for patients to wear a week prior to starting and a week post program to detect changes in physical daily activity.

Appropriate staffing was discussed at length. The same specialist physiotherapist attends all sessions. A specialist nurse is present and a doctor from the Marie Curie inpatient unit is available should the need arise. The Clinical Research Associate attends every session to carry out the assessment of the Powerbreathe and analyse pedometer results. We have oxygen on site and appropriate monitoring devices such an oxygen saturation and blood pressure machines.

Results are based on the first 4 cohorts to complete the programme which gives data for 13 patients. All but 1 completed the program suggesting very high adherence to the program for this patient group. Most patients brought a carer (often a family member) with them to every session.

With regards to outcome measures specifically, the six minute walk tests demonstrated a majority improvement after attending PR with results for 12 patients available, 9 patients improved. The improvement ranged from 6m to 189m-with the mean 41.4m. The general improvement does meet the minimum clinical importance difference for ILD which is around 30m but as the results are based on the first 4 cohorts of 13 patients’ further results would help strengthen the results.

Patient’s activity was assessed one week prior to starting the PR and one week post completion. Of the 12 patients, 5 had improvements one week completion, despite the usual trend of IPF with more progression of the disease and worsening of exercise capacity.

As we have been hoping to improve the patients overall QoL, depression scores have been closely monitored and it is rewarding to see some improvement in the results. The improvement of depression scores were strongly associated with the improvement of their physical activity, demonstrating possibly that patients are coping better with their condition and participating in ADLs.  

General feedback has been extremely positive. The environment and the duration of each session have allowed the patients and carers to enjoy informal chats and feel able to ask questions during the education sessions. The pedometer and Powerbreathe have been well tolerated and all the patients have been happy to complete the home exercise diaries. Feedback on the education topics has also been positive. The range of topics is varied and the literature given out has been sufficient.  

All our patients responded differently to the tailored exercises, while some of them improved in their physical measures, others showed improvement in different outcomes. All of them felt the program was really beneficial and it helped them be more active with a greater understanding about their conditions. We believe the personalised approach to the rehabilitation is essential for good outcomes for such conditions.

In the past our physiotherapists have assessed the patients and completed the outcome measures. By setting up the service alongside research aims, the assessments were initially completed in clinic with the Research Associate. However it became apparent early on the importance of the physiotherapist being involved with the assessment of the patients to understand their needs and goals for the exercise element of the PR. This also allowed for a greater understanding of the starting point for each patient in the gym.

• During their first session, a patient became unwell during the exercise session and the safety precautions we had set up proved effective and the patient had timely medical treatment, cementing the reason why such precautions need to be in place when setting up a PR program off the acute hospital site.
• Because some of the equipment we are using for the research is not feasible or practical in everyday PR groups due to financial limitations, we will need to look at each element and how we can sustain the quality of PR programs on a long term basis.
• Despite historically PR being twice weekly we have found that once weekly with a comprehensive home exercise program has been successful. This patient group can have many hospital appointments and being once weekly may have contributed to their adherence to the program. We also felt starting home exercises early in the rehab process patients might be more likely to adhere long term. Patients have asked re-equipment and more exercise sheets on completion of the group.
• As discussed in the AHPs in Action paper, the physiotherapists are well positioned to be an integral part in the setting up and running of the new program. Their specialist skills and experience helped address the short falls of the current services and help shape how the new service could be developed alongside the guidance form the NICE guidelines. We have achieved a new service that has been well received by patients and carers. Having reflected on our old service we acknowledge the sub optimal service we have previously been offering this patient group by supporting them in programs with other differing respiratory conditions.