Draft respiratory care action plan: consultation

Annex C

Respiratory Conditions and outputs from the workstream groups

This chapter highlights the 5 respiratory conditions contained within the plan and includes the views of the condition specific workstream groups.

It is important to note, that these are the views of a number of clinicians rather than a factual picture of the care system, backed by official data and fact finding.

Asthma

Asthma is a common lung condition that causes intermittent breathing difficulties.

It affects people of all ages and often starts in childhood, although it can also develop for the first time in adults of any age.

Symptoms

Although there is currently no cure there are simple treatments that can control symptoms and reduce the impact on a person’s life.

The main symptoms of asthma are:

• a whistling sound when breathing out (wheezing)
• breathlessness
• a tight chest, which may feel like a band is tightening around it
• coughing

The symptoms can sometimes get temporarily worse. This is known as an asthma attack.

Causes

Asthma is caused by swelling (inflammation) of the breathing tubes that carry air in and out of the lungs. This makes the tubes highly sensitive, so they temporarily narrow.

It may occur randomly or after exposure to a trigger.

Common asthma triggers include:

• allergies (for example to house dust mites, animal fur or pollen)
• smoke, pollution and cold air
• exercise
• infections like colds or flu.

Identifying and avoiding asthma triggers can help keep symptoms under control.

Diagnostics

A carefully taken clinical history combined with peak flow measurements, spirometry, and sometimes more intensive testing including exhaled nitric oxide testing, and challenge testing.

Treatment

Asthma is usually treated by using an inhaler, a small device that lets the user breathe in medicines.

The main types are:

• reliever inhalers – used when needed to quickly relieve asthma symptoms for a short time
• preventer inhalers – used every day to prevent asthma symptoms occurring

Some people also need to take tablets, or injections.

The Asthma workstream raised the following issues with the current diagnostics, management and care.

Diagnostics – Spirometry

Diagnostic spirometry is a test which measures lung function in a controlled environment. It is carried out in primary and secondary care environments throughout Scotland.

Although spirometry is available both in primary and secondary care environments. The group thought that, in primary care, the test is not available throughout all Health Boards. However, those areas that do provide spirometry, access is generally more rapid, and closer to the person’s home.

Primary care spirometry training should be standardised, as well as have a clear quality assurance pathway for non-ARTP (Association for Respiratory Technology & Physiology) accredited practitioners. The group believed that waiting times for spirometry are variable, from few days to 6 weeks or more.

The workstream group members suggested that respiratory diagnostic hubs may be a solution to providing high quality, quality assured spirometry, with skilled interpretation.

Standards of care. National and international guidelines recommend person centred asthma action plans focussing on education and self-management. The group thought that access to asthma action plans is not standardised, and there is variation throughout Scotland.

Biologic therapies for asthma. It was felt that access to, and assessment for, biologic therapies varies across Scotland. There are no nationally agreed protocols for how to assess people for these therapies. Some areas do not have direct access to biologic therapies, or clinicians with expertise in this area. Standardising paperwork, data collection, and measurements of outcome measures should be a priority for difficult asthma services across Scotland.

Difficult asthma. It was highlighted that there is no uniform approach across the country for difficult asthma: some centres have a multidisciplinary team approach, whereas others have a single handed clinician. This is a particular problem for smaller centres, who need to refer to another centre for difficult cases.

Occupational asthma. Occupational asthma is one of a number of occupational lung diseases. It was the view of members of this workstream that there is significant variation in referral rates across Scotland, with no clear pathway in place to facilitate interaction with the Health and Safety Executive and occupational medicine teams.

British Thoracic Society guidelines on asthma suggest that up to 30% of adult onset asthma is related to occupation, and dedicated occupational asthma services should be in place to assess and treat such patients.

Bronchiectasis

Bronchiectasis is a long-term condition where the airways of the lungs become abnormally widened, leading to a build-up of excess mucus that can make the lungs more vulnerable to infection.

Symptoms, Causes, Diagnostics and Treatment

Symptoms

The most common symptoms of bronchiectasis include:

• a persistent cough that usually brings up phlegm (sputum)
• breathlessness
• recurrent lower respiratory tract infections

The severity of symptoms can vary widely. Some people have only a few symptoms that don't appear often, while others have debilitating daily symptoms.

The symptoms tend to get worse if a lung infection is developed.

The lungs are full of tiny branching airways known as bronchi. Oxygen travels through these airways, ends up in tiny sacs called alveoli, and from there is absorbed into the bloodstream.

The inside walls of the bronchi are coated with sticky mucus, which protects against damage from particles moving down into the lungs.

In bronchiectasis, one or more of the bronchi are abnormally widened. This means more mucus than usual gathers there, which makes the bronchi more vulnerable to infection.

If an infection does develop, the bronchi may be damaged again, so even more mucus gathers in them and the risk of infection increases further.

Over time, this cycle can cause gradually worsening damage to the lungs.

Causes

Bronchiectasis can develop if the tissue and muscles that surround the bronchi are damaged or destroyed.

There are many reasons why this may happen. The 3 most common causes in the UK are:

• having had a lung infection in the past, such as pneumonia or whooping cough, that damages the bronchi
• underlying problems with the immune system (the body's defence against infection) that make the bronchi more vulnerable to damage from an infection
• allergic bronchopulmonary aspergillosis (ABPA) – an allergy to a certain type of fungi that can cause the bronchi to become inflamed if spores from the fungi are inhaled

But in many cases, no obvious cause for the condition can be found. This is known as idiopathic bronchiectasis.

Treatment

The damage caused to the lungs by bronchiectasis is permanent, but treatment can help relieve symptoms and stop the damage getting worse.

The main treatments include:

• exercises and special devices to help clear mucus out of the lungs
• medication to help improve airflow within the lungs
• antibiotics to treat any lung infections that develop

Surgery is usually only considered for bronchiectasis in rare cases where other treatments haven't been effective, the damage to the bronchi is confined to a small area, and the person is in good general health.

Possible complications

Complications of bronchiectasis are rare, but they can be serious.

One of the most serious complications is coughing up large amounts of blood, caused by one of the blood vessels in the lungs splitting.

This can be life threatening and may require emergency surgery to treat it.

The Bronchiectasis workstream raised the following issues with the current diagnostics, management and care.

Diagnostics and case finding. The workstream highlighted that some people with bronchiectasis are either undiagnosed, or misdiagnosed with other respiratory illnesses, particularly COPD. Case finding studies have shown that people who receive multiple courses of antibiotics for lower respiratory tract infection have a high probability of underlying bronchiectasis. Case finding targeting these people will identify these patients, and should be introduced in a co-ordinated manner throughout Scotland.

The diagnostic test requires High Resolution CT (HRCT) scanning of the chest. Primary care clinician access to HRCT is not widespread across Scotland, therefore pathways necessarily include referral to secondary care for all suspected cases. The workstream group supported widespread access for primary care clinicians to HRCT scanning to assess people for bronchiectasis.

Other critical investigations include immunology testing, sputum cultures, and vaccination challenges. A pathway for assessment prior to secondary care review could be co-ordinated through respiratory diagnostic hubs.

Secondary care specialist clinics. It was suggested that each hospital should have provision for a specialist bronchiectasis clinic. Centres should have a complex infection multidisciplinary team, involving respiratory clinicians, specialist nurses, microbiologists, pharmacists, immunologists (where available), and ID clinicians. Difficult cases should be discussed at regional or national multidisciplinary team (MDT) meetings. It was the view of the group that a separate National Bronchiectasis Strategy may be given some consideration in the future. This would require investment in both leadership and administration, to facilitate audit and improvement work within the bronchiectasis community in Scotland. It was also highlighted that involvement in EMBARC (Bronchiectasis database) would support data collection.

Physiotherapy. The workstream group agreed that physiotherapy is an essential part of therapy for bronchiectasis. The workstream group felt that the provision of dedicated respiratory physiotherapy is variable around Scotland, with some areas only having access to physiotherapists through pulmonary rehabilitation services.

Fungal lung disease. There is a national level agreement that people with aspergillus lung disease be seen in the National (UK) Aspergillus Centre at the Wythenshawe Hospital in Manchester. The workstream discussed the perceived increase of prevalence of Aspergillus Lung Disease in Scotland and the long travel times from Scotland to Manchester for people from more rural and remote areas, and those who are most frail. Data is required to understand if fungal lung disease is now sufficiently prevalent in Scotland to require local expertise in each area, with fungal infection multidisciplinary teams including specialist radiologists, infectious diseases consultants, and immunologists (where available).

Chronic Obstructive Pulmonary Disease (COPD)

Symptoms, causes, diagnostics and treatment

Chronic obstructive pulmonary disease (COPD) is the name for a group of lung conditions that cause breathing difficulties, caused by inhalation of toxins, mainly from cigarette smoking.

Symptoms

The main symptoms of COPD are:

• increasing breathlessness, particularly when active
• a persistent chesty cough with phlegm; some people may dismiss this as just a "smoker's cough"
• frequent chest infections
• persistent wheezing

Causes

COPD is largely a preventable condition. The risk of developing it is significantly reduced by not smoking.

Stopping smoking can help prevent further damage to lungs before it starts to cause troublesome symptoms.

COPD includes:

• emphysema – damage to the air sacs in the lungs
• chronic bronchitis – long-term inflammation of the airways .

COPD is a common condition that mainly affects middle-aged or older adults who smoke. Many people don't realise they have it.

The breathing problems tend to get gradually worse over time and can limit normal activities, although treatment can help keep the condition under control.

Treatment

Without treatment, the symptoms usually get slowly worse. There may also be periods when they get suddenly worse, known as a flare-up or exacerbation.

COPD occurs when the lungs become inflamed, damaged and narrowed. The main cause is smoking, although the condition can sometimes affect people who have never smoked.

The likelihood of developing COPD increases the more the individual smokes and the longer they have smoked.

The damage to the lungs caused by COPD is permanent. Only stopping smoking can slow the progression of disease, but some other treatments can be effective in reducing symptoms, exacerbations, and mortality from COPD.

Treatments include:

• stopping smoking - the most important thing to do
• pulmonary rehabilitation – a specialised programme of exercise and education that has been shown to reduce mortality, reduce exacerbation rates, and improve quality of life
• vaccinations, particularly against influenza and pneumococcus
• inhalers and medications, including home oxygen to help make breathing easier, improve exercise capacity, reduce exacerbations
• surgery or a lung transplant, although this is only an option for a very small number of people, COPD remains the commonest indication for lung transplantation, and endobronchial valve/coil treatments are increasingly effective in selected patients.

The COPD workstream raised the following issues with the current diagnostics, management and care.

Pulmonary rehabilitation. The members of the COPD workstream were clear that equitable access to pulmonary rehabilitation across Scotland is the first priority in the management of COPD. Provision of pulmonary rehabilitation is variable across Scotland. There is variation in where it is held, who leads the classes (nurses, physiotherapists, or both), how access is granted (direct primary care access, or through secondary care), and provision of repeat rehabilitation. Accurate data on the supply of, and demand for, pulmonary rehabilitation are essential to improve the provision of pulmonary rehabilitation in Scotland. Collection of data from pulmonary rehabilitation should be standardised across Scotland.

Diagnostics – spirometry. Diagnostic spirometry is a test which measures lung function in a controlled environment. It is carried out in primary and secondary care environments throughout Scotland.

Although spirometry is available both in primary and secondary care environments. The group thought that, in primary care, the test is not available throughout all Health Boards. However, those areas that do provide spirometry, access is generally more rapid, and closer to the person’s home.

Primary care spirometry training should be standardised, as well as have a clear quality assurance pathway for non-ARTP (Association for Respiratory Technology & Physiology) accredited practitioners. The group believed that waiting times for spirometry are variable, from few days to 6 weeks or more.

The workstream group members suggested that respiratory diagnostic hubs may be a solution to providing high quality, quality assured spirometry, with skilled interpretation.

Smoking cessation services. The group felt that there is a need for a cohesive national smoking cessation program. Opportunistic smoking cessation advice programs, including targeting people who are staying in hospital or attending out patient appointments have proven to improve quit rates.

Vaccines. Vaccinations are an crucial part of reducing hospitalisations and exacerbations for people with COPD. It was thought that uptake of vaccines is poor in this patient group. The vaccination transformation program, yet to be fully enacted, instructs regional health boards to provide a co-ordinated Plan to ensure all people who are eligible for influenza and pneumococcal vaccines are given access to those vaccines. A clear Plan of where people can receive vaccinations, including during routine primary and secondary care encounters, and acute admissions, in a more comprehensive and opportunistic manner is a priority.

Specialist Nurses. The workstream group thought that specialised nursing services is varied across the Country and are provided by secondary or primary care. People with COPD highly value contact with specialist COPD nurses. The workstream group thought that it is important that people with COPD have access to specialist nurses. They also thought that it would be beneficial if nurse specialty qualification were clearly defined, as well as a set number of specialist nurses per population of people with COPD.

Idiopathic pulmonary fibrosis, and other forms of pulmonary fibrosis

Symptoms, causes, diagnostics and treatment

Pulmonary fibrosis is a condition in which the lungs become scarred, or thickened: breathing becomes increasingly difficult, then, as the disease progresses, oxygen levels within the blood begin to fall. Idiopathic pulmonary fibrosis (IPF) is the most common of these diseases, but there are a range of others. When this Plan refers to IPF, other forms of pulmonary fibrosis are implied.

Symptoms

The main symptom of IPF is breathlessness. People living with IPF may also experience a persistent cough and may feel tired all the time. Finger clubbing might also be a symptom for some.

Causes

The cause is not clear, but it usually affects people in their 6^th and 7^th decade and is rare in people under 50. It is more common in men than women, and more common in current or ex-smokers.

Antifibrotic treatments can help reduce the rate at which IPF progresses, but there is currently no treatment that can stop or reverse the fibrosis of the lungs.

In people with IPF, the tiny air sacs in the lungs (alveoli) become damaged and increasingly scarred resulting in stiff, less compliant lungs. The person must work much harder to breathe, perceived as breathlessness. As the lungs become increasingly scarred there is a reduction in the amount of oxygen absorbed into the blood. Over time, this causes damage to the heart, kidneys, and brain. Breathlessness can become very severe, greatly limiting people’ exercise capacity and ultimately making them house bound.

The reason this happens is not clear. Idiopathic means the cause is unknown.

IPF has been linked to:

• exposure to certain types of dust, such as metal or wood dust
• viral infections
• a family history of IPF – around 1 in 20 people with IPF has another family member with the condition
• gastro-oesophageal reflux disease (GORD)
• smoking

Diagnostics

If a GP thinks a person could have a lung condition such as IPF, they can refer to a hospital specialist for tests such as:

• breathing (lung function) tests
• blood tests
• a chest X-ray and CT scan
• a lung biopsy, where a small piece of lung tissue is removed during either keyhole surgery, or bronchoscopy (a telescopic camera test into the lungs) so it can be analysed under a microscope

Treatment for IPF

There is currently no cure for IPF, but there are treatments that can help relieve the symptoms and slow down its progression.

Treatments include:

• self-care measures, such as stopping smoking, eating healthily and exercising regularly
• medicines to reduce the rate at which scarring worsens, such as pirfenidone and nintedanib
• breathing oxygen through a mask – this can be done whilst a person is at home or while they’re out and about
• exercises and advice to help breathe more easily (pulmonary rehabilitation)
• a lung transplant – this is suitable in a few cases, although donor lungs are rare

The Pulmonary Fibrosis workstream raised the following issues with the current diagnostics, management and care of pulmonary fibrosis

Diagnostics. IPF and other forms of pulmonary fibrosis are rare: a typically sized general practice in Scotland may have only 2 people with IPF, and fewer than 5 with pulmonary fibrosis of any form. Given its rarity, the group believed that there is a need for better awareness of this condition to avoid possible delay in diagnosis. It was highlighted that the work undertaken by the Scottish Access Collaborative shows that primary care access to diagnostic tests, notably high-resolution computed tomography (HRCT) scanning, shortens the person’s journeys without increasing demand on investigations. Diagnostic algorithms for IPF should include primary care access to HRCT, and describe pathways for ongoing referral in the case of a positive results. Key to this process is engagement with colleagues in radiology and primary care.

Access to secondary care. There should be a clearly defined referral pathway in place for every centre in Scotland. Larger centres may have pulmonary fibrosis specific clinic and a lead clinician for pulmonary fibrosis; other smaller centres may need to collaborate with other local centres to pool resources and expertise. There should be access to pulmonary fibrosis multidisciplinary meetings (including experts in respiratory medicine, radiology, and specialist nursing), either locally or via remote access to a bigger centre.

Access to anti-fibrotic agents. There is clear evidence that the uptake of anti-fibrotic agents for IPF (specifically) is very variable across the country, with no clear understanding of why this is the case. This warrants further investigation as a priority in this disease area.

Specialist nurses. NICE clinical guideline CG163 recommendation 1.3.3 states

that an interstitial lung disease specialist nurse should be available at all stages of the care pathway to provide information and support to people with idiopathic pulmonary fibrosis and their families and carers with the person's consent.

Specialist nurses may be primary or secondary care based, but they must be sufficiently trained to deal with the specific and generic challenges presented by people with IPF.

There is a masters level nursing module focussed on interstitial lung disease (ILD) available, although there is some uncertainty on how this can be accessed equitably across the country.

Research by the British Lung Foundation in England and Wales shows that people with IPF highly value the input of a specialist IPF nurse, as they provide holistic, person centred, personalised care. This includes self-management plans, review of symptoms and lung function, cognitive behavioural therapy, and palliative care.

Palliative care. There is a perception that there is variation in provision of palliative care for non-malignant respiratory illness in Scotland. The newly formed palliative care Managed Clinical Network (MCN) is closely aligned with respiratory MCNs throughout Scotland. Palliative care for people with IPF includes management of breathlessness, anxiety, depression, psychological isolation and desperation, and the provision of palliative oxygen therapy.

Long term oxygen therapy. Oxygen therapy is key in the management of later stage pulmonary fibrosis. The national service agreement for oxygen provision is an example of excellent practice.

Pulmonary Rehabilitation (PR). Pulmonary fibrosis specific PR differs from COPD pulmonary rehabilitation. It is currently unknown how many pulmonary fibrosis specific PR services exist in Scotland and intelligence is required to determine the need for specific

Obstructive Sleep Apnoea Syndrome

Symptoms, causes, diagnostics and treatment

Obstructive sleep apnoea syndrome (OSA) is a relatively common condition where the walls of the pharynx and larynx relax and narrow during sleep, interrupting normal breathing.

This may lead to regularly interrupted sleep, which can have a significant impact on quality of life and increases the risk of developing certain conditions.

Apnoea and hypopnoea

There are 2 types of breathing interruption characteristic of OSA:

• apnoea – where the muscles and soft tissues in the throat relax and collapse sufficiently to cause a total blockage of the airway; it's called an apnoea when the airflow is blocked for 10 seconds or more
• hypopnoea – a partial blockage of the airway that results in an airflow reduction of greater than 50% for 10 seconds or more

People with OSA may experience repeated episodes of apnoea and hypopnoea throughout the night. These events may occur around once every 1 or 2 minutes in severe cases. As many people with OSA experience episodes of both apnoea and hypopnoea, doctors sometimes refer to the condition as obstructive sleep apnoea-hypopnoea syndrome, or OSAHS.

The term "obstructive" distinguishes OSA from rarer forms of sleep apnoea, such as central sleep apnoea, which is caused by the brain not sending signals to the breathing muscles during sleep.

Symptoms

The primary symptom of OSA is daytime somnolence: falling asleep easily during the day.

During an apnoeic episode when oxygen saturations fall sufficiently, the brain awakens the person, bringing them from deep, restorative sleep, into a lighter sleep or wakefulness, during which the airway reopens, and normal breathing, and oxygen saturations, are restored. These repeated sleep interruptions can make the individual feel very tired during the day. The person usually has no memory of their interrupted breathing, so they may be unaware they have a problem.

It's normal for the muscles and soft tissues in the throat to relax and collapse to some degree while sleeping: most people will experience some apnoeas every night, however in most cases this does not cause significant problems, and the person do not have daytime somnolence.

Causes

In people with OSA, the airway narrows as the result of a number of factors, including:

• being overweight
• OSA is more common in men than in women
• being over 40 years of age
• taking medicines with a sedative effect – such as sleeping tablets or tranquillisers
• alcohol
• smoking
• the menopause (in women)
• having a family history of OSA
• nasal congestion

Treatment

Daytime somnolence due to OSA can be treated effectively:

• lifestyle changes – such as losing excess weight, cutting down on alcohol and sleeping on the side
• using a continuous positive airway pressure (CPAP) device – these devices prevent airway closure during sleep by delivering a continuous supply of pressurised air through a mask
• wearing a mandibular advancement device (MAD) – this gum shield-like device fits around the teeth, holding the jaw and tongue forward to increase the space at the back of the throat during sleep

Surgery may rarely be an option if OSA is thought to be the result of a physical problem that can be corrected surgically, such as an unusual inner neck structure.

The Obstructive Sleep Apnoea Syndrome Workstream Group raised the following issues with the current diagnostics, management and care.

Diagnostics. The group thought that there is variation in practice across Scotland.

A number of Health Boards have a dedicated OSA assessment and treatment units, designed to deal with the prevalence and incidence of OSA within their catchment area. Other areas have Service Level Agreements with larger centres to deal with either the treatment, or assessment and treatment, of people with OSA. Pathways are variable, particularly in centres where diagnostics are carried out locally, but treatment is initiated at a larger centre.

An example of good practice exists where people are seen rapidly by a specialist nursing team for assessment, diagnostics, interpretation of results, and initiation of therapy within 48 hours, with a waiting time within 6 weeks. The workstream felt this pathway may be repeated across Scotland.

Vetting. Referrals for assessment and treatment of OSA make up nearly half of the referrals to respiratory medicine in most centres. The probability of a positive diagnosis varies widely, and most centres have a single pathway for assessment. Many people have a very high probability of OSA, based on their clinical history, whereas others have a very low probability, usually due to lack of significant daytime somnolence, or risk factors. A pathway that allows for variation in assessment due to probability of OSA would be welcomed.

Diagnostic hubs and locality based assessment. The acceptable diagnostic test is agreed to be Limited Polysomnography (LPSG), a sleep test. This technology is now more affordable, and test interpretation more automated, allowing testing to be carried out nearer to the patient, rather than in large hospital centres.

Diagnostic Respiratory Hubs have been suggested as an ideal location for OSA assessment centres, with LPSG available at a local level. Negative studies will reduce the referral to secondary care; positive studies will streamline the access to therapeutics for OSA. A nurse led model of OSA assessment and treatment could be integrated into a diagnostic respiratory hub, negating the need for secondary care involvement.

Machines and consumables. It was highlighted that specialist nurses spend a lot of time dealing with problems caused by machinery, and the consumables associated with the Continuous Positive Airway Pressure (CPAP) therapy. It was suggested that a solution would be to engage with industry that could provide a service where they provide all the equipment, the consumables, etc. A national service level agreement, such as that made for Oxygen therapy, would be beneficial to streamline the delivery of machinery and consumables directly to the patient, freeing up specialist nursing time to focus on assessment and treatment of people with OSA.