Annette Duck, BSc, SCM, RN, is intertitial lung disease nurse specialist and Association of Respiratory Nurse Specialists committee member, University Hospital of South Manchester NHS Foundation Trust.
Duck, A. (2008) Nursing patients with interstitial lung disease. Nursing Times; 104: 9, 46–49. Annette Duck considers the important factors for supporting patients with a diagnostic label of interstitial lung disease.
The interstitial lung diseases (ILDs) are a group of over 150 lung disorders. They share common clinical, radiological and pulmonary function features. They usually present with shortness of breath and/or cough. If left untreated they can lead to lung fibrosis or scarring and, ultimately, death.
It is not necessary for nurses to understand the characteristics, pathology or medical management of all 150 types, in order to care and support patients with ILD. As with all illnesses, nurses treat the whole person and not the disease, holistically looking at their unique problems. Patients with ILD often have common problems that need further discussion. The diagnosis and investigation of ILD has been covered in previous NT articles (Duck, 2007a, b).
Diagnosis and providing information
Patients like to feel the clinicians looking after them are familiar with the characteristics of their disease. Sometimes it is not possible to give a definitive diagnosis of ILD and patients can find this distressing. It is vital to have an ‘open and honest’ discussion with the patient, explaining why it has been impossible to arrive at a conclusive diagnosis.
As ILDs are uncommon many GPs or general physicians may only care for a small number of patients with the disease. It is recommended that such patients are referred to a respiratory specialist for full diagnostic work up and management.
A significant number of patients will be given a diagnosis of one of the more common forms of ILD called idiopathic pulmonary fibrosis (IPF). This
form of ILD has a poor prognosis, similar to some types of lung cancer. Most patients will have an understanding of what it means to have a cancer, and this is a useful comparison to help explain their diagnosis. Many patients will not take in the implications of their diagnosis at first presentation and several explanations may be required.
It takes time to obtain a diagnosis of ILD and this can increase worry and anxiety for patients. Fallowfield et al (1995) in their study of patients with cancer identified a ‘strong absolute need for all general information, be it positive or negative’.
Breaking bad news is one of the most important skills a clinician can learn (Buckman, 1992). If patients perceive that information given to them is inadequate or poorly delivered, they may fail to adjust and exhibit signs of anxiety and depression (Fallowfield et al, 1995). If bad news is delivered appropriately, it can improve the therapeutic relationship between patient and clinician.
Patients with ILD experience breathlessness on exertion, which is usually associated with hypoxia, and are likely to need ambulatory oxygen before they need oxygen at rest. The British Thoracic Society (BTS, 2006) guidelines for oxygen assessment should be followed.
It is important to assess all patients with breathlessness on exertion for exercise-induced hypoxia, particularly those diagnosed with IPF. IPF progresses rapidly (Wells and du Bois, 1994) and patients are likely to experience exercise-induced hypoxia that can reduce their exercise performance significantly. They should be assessed with either a shuttle walk or six-minute walk test to evaluate whether they are experiencing breathlessness associated with hypoxia (BTS, 2006) (Box 1).
Most patients are reluctant to walk in public with supplementary oxygen due to embarrassment, difficulty transporting oxygen and anxiety about running out of oxygen. This therapy may also be symbolic of dependence and progressive disease.
Patients with an exercise capacity of over 300m may prefer to slow down or stop and rest in preference to using ambulatory oxygen. Patients usually have a threshold at which they will assess the benefit of wearing oxygen in public against social isolation, breathlessness and staying at home.
Using ambulatory oxygen may not stop oxygen desaturation on exercise, particularly in IPF, however, it will raise the baseline oxygen level, maintain higher capillary oxygen levels for longer and enable a faster recovery rate, thus improving exercise performance overall. Oxygen conserver devices attached to cylinders or liquid oxygen units deliver small pulsed doses of oxygen on inspiration, cutting off on expiration, thereby prolonging the life of the cylinder or liquid oxygen unit (Duck, 2006). Although these devices are useful for patients with COPD, who usually only require low flow rates of around 2l/min, they are not useful for patients who need higher flow rates that are equal to or greater than 4l/min with ambulatory oxygen. It is important, therefore, that patients are assessed with and without oxygen conservers before their use is recommended.
IPF is rapidly progressive with a mean survival of about three years from diagnosis (Wells and du Bois, 1994). Patients with IPF should be offered liquid oxygen for ambulatory use outside the home from an early stage, if they meet the BTS guidelines for ambulatory oxygen. Many of these patients generally have a good exercise capacity, but require substantial flow rates to maintain oxygen saturations during activity. As the disease is rapidly progressive, patients often do not have time to become physically deconditioned to exercise, but become limited by breathlessness associated with hypoxia. Correcting hypoxia during exercise means that patients can try and maintain their physical and usual social engagements, which is important to their sense of worth and well-being.
Managing with liquid oxygen outside the home is fundamental to learning how it can help in self-managing strategies with advancing disease and activities of daily living within the home.
Developing self-management strategies with the aid of liquid oxygen can help to avoid emergency admissions to hospital.
Ambulatory oxygen is the single most important supportive treatment we can offer patients with progressive ILD. Increased levels of ambulatory oxygen can mean that patients with advanced disease can maintain a near-normal quality of life. It has been shown that patients can double their exercise performance with adequate supplementary ambulatory oxygen (Hicks et al, 2007).
Box 1. Shuffle walk and six-minute walk test
Oxygen delivery devices
Oxygen devices that are useful for patients with ILD include the OxyArm device and high-flow nasal cannula (Box 2).
BOX 2. NEW DEVICES FOR DELIVERY OF OXYGEN
High-flow nasal cannula
Breathlessness with advanced disease
In the latter stages of disease patients become breathless at rest and, when the resting oxygen saturations is less than 93%, they should have arterial blood oxygen levels checked to assess whether they meet the criteria for long-term oxygen therapy (LTOT).
Some patients experience breathlessness without significant hypoxia. These are often patients with no history of smoking and the breathlessness is associated with the small airways becoming stiff as the disease advances. This stiffness makes inspiration difficult, and the patient takes rapid inspiratory breaths and speaks in short sentences with intermittent little coughs.
Breathlessness associated with ‘stiff lungs’ but not extreme hypoxia can sometimes be relieved by increasing the total gas flow of oxygen using the venturi mask system. It is essential that hypoxia as well as breathlessness associated with the ’stiff lungs’ syndrome is corrected. The patient may need to use the 40% or 60% oxygen concentration venturi mask. In the community these masks will require three and four oxygen concentrators, linked together to deliver sufficient oxygen to generate the pressure capable of delivering the required total gas flow.
Strategies for goal setting and positive thinking
Miller (2000) suggests that individuals who have a physical disability have access to multiple resources including social support, psychological stability, knowledge, self-esteem, energy and hope. These should be developed in order to relieve the feelings of powerlessness and loss of control that patients with ILD experience.
Many patients reflect on the past and will grieve about ‘what might have been’. Feeling supported and having opportunities to talk with nurses who are empathetic, acknowledging and understanding can help them to realise what is most important in their lives.
Occasionally, individuals find it difficult to motivate themselves. Nurses are well placed to encourage patients to believe in themselves, by reminding them of who they are and how they once were (Miller, 2000).
All individuals have things they like to do. Nurses can help patients work out ways to do activities that are most important to them and hold some meaning in their lives. If individuals want to do something badly enough they can usually work out ways in which they are most likely to achieve it. I call this model of achievement ‘the four ps’:
- Priming: Encourage and empower the patient, by asking them how they think they could do whatever it is that they would like to do.
- Prioritising: Help them to understand that they will not be able to do all the things that they used to do and that now they need to decide on those that are most important.
- Planning: Advise patients to think about how they are going to do something in light of their breathlessness and reduced exercise abilities.
- Pacing: Help patients to understand that they should not have an unrealistic expectation of themselves, that they will have good and bad days and will be able to do more on some days compared with others.
Involving a spouse or close friend in this model of care can help maintain a patient’s motivation at home. If the carer is present during an initial interview with the patient, they will usually learn how to manage the despondent moods that are initially experienced by the patient.
Helping patients plan and work out ways to do things that are important to them can help them regain some control over their lives. It can help relieve the feelings of powerlessness that lead to the emotional distress and maladjustment that is often seen in chronic illness. Patients with ILD have travelled independently with supplementary oxygen on public transport, continue to play golf despite being oxygen dependent and even managed to continue singing in a band. Nurses should ask patients what they would like to continue doing and help them work out how to achieve these goals.
Patients sometimes feel isolated and alone when dealing with advanced ILD. Support groups allow social comparison and exchange among members, encouraging reassurance about themselves and their reactions to their illness. Stewart et al (2001) note that support groups are most useful when they enhance role functioning, develop new skills, enable coping with stressful transition and ease social isolation. Box 3 shows how such support groups might work and their benefits.
Box 3. Starting a support group for patients with ILD
The author started a support group for patients with ILD and their carers. The format was agreed by the group and the author acted as facilitator and organiser. It runs every two months and provides an opportunity for patients and carers to exchange ideas, share concerns, discuss problems and provide support to each other. An invited speaker talks about a topic requested by the group and it usually finishes with a relaxation/visualisation session run by a hypnotherapist.
Some patients who attend enjoy ‘helping’ others and act as model experts on coping with the condition. Others gain strength from seeing how group members manage and ‘cope’.
Simply meeting other people who share similar symptoms and problems helps patients realise that they are not alone.
If channels of communication with patients have been kept open and the health professionals involved have been honest about their prognosis from the beginning, patients often realise when they are approaching the end of their lives as breathlessness and oxygen requirements increase. Some patients will start a conversation about dying overtly, others may skirt around it and active listening skills will be required to understand what might be behind a question.
It is important that patients are assessed for physical aids to enable them to continue with activities of daily living. An occupational therapy and social review will help to identify individual physical and financial needs.
As the disease progresses, oxygen requirements will steadily increase, so that at the end stage patients will be oxygen dependent and probably use a high-flow nasal cannula, usually at 8–10 l/min, alternating with a high oxygen concentration venturi mask (typically 60%). A non-rebreathing mask, which can deliver 80–90% oxygen (Duck, 2006), may be used to complete activities of daily living around the home. A liquid oxygen domiciliary unit kept near to patients and used with a non-rebreathing mask can help them remain in their home.
Patients may describe having ‘panic attacks’ as well as difficulty getting up from a chair and going to the toilet because of breathlessness. These events are probably associated with extreme hypoxia related to minimal exertion and nurses must be aware that a review of management and treatment must be considered at this stage. Patients can be referred to the local palliative care teams at any time, depending on their coping strategies, but should definitely be referred at this stage in the disease trajectory. Referrals can also be made to the local Macmillan or district nursing services, which can review patients in their homes.
Opiates should be used when required to relieve the symptoms of distressing breathlessness even though they have a respiratory depressant effect. Palliative care teams can offer advise and support.
As with all other terminal illnesses, end-of-life care should be managed with local palliative care services. Patients and their families need to be asked their ‘preferred place of death’ and these preferences should be documented.
Palliative care teams have the knowledge and expertise to relieve distressing symptoms and should work with ILD specialists to ensure that the most distressing symptom of breathlessness is minimised for the patient. The Liverpool Care Pathway (Ellershaw and Wilkinson, 2003) is a useful protocol that can be used to manage end-of-life care either in hospital or in the community.
High-flow oxygen circuit systems are useful for managing symptoms of extreme breathlessness by ensuring patients can have high flow rates and high concentrations of oxygen. They are available in hospital but not in the community and discussions should take place about the preferred place of death before a patient is started on high-flow oxygen systems in hospital. In my experience, it is difficult to remove high-flow oxygen from a patient.
It is imperative that this treatment is used in conjunction with a dying pathway and opiates, so that when maximum flow and oxygen concentrations are reached, breathlessness can be treated by pharmacological means. Midazolam and diamorphine subcutaneous pumps are sometimes needed to control breathlessness, particularly with younger patients who have IPF.
There are now recommendations for the way services for patients diagnosed with ILD are managed (Johnston et al, 2006) and nurses must learn to understand the specific needs of these patients. With better understanding, nurses can develop models of care that will improve patients’ quality of life and benefit those who find it difficult to cope with these lung disorders.
British Thoracic Society (2006) Clinical Component for the Home Oxygen Service in England and Waleswww.brit-thoracic.org.uk/Portals/0/Clinical Information/Home Oxygen Service/clinical adultoxygenjan06.pdf
Buckman, R. (1992) How to Break Bad News: A Guide for Healthcare Professionals. Baltimore, MD: John Hopkins University Press.
Duck, A. (2007a) Interstitial lung disease 1: diagnosis and investigation. Nursing Times; 103: 43, 28–29.
Duck, A. (2007b) Interstitial lung disease 2: management strategies. Nursing Times; 103: 44, 26–27.
Duck, A. (2006) Cost-effectiveness and efficacy in long-term oxygen therapy. Nursing Times;102: 7, 46.
Ellershaw, J., Wilkinson, S. (2003) Care of the Dying. A Pathway to Excellence. Oxford: Oxford University Press.
Fallowfield, L. et al (1995) No news is not good news: information preferences of patients with cancer. Psycho-oncology; 4: 3, 197–202.
Hicks, S. et al (2007) Ambulatory oxygen in idiopathic pulmonary fibrosis: of what benefit? Thorax; 62: 12 (Supplement 111), A1–A168.
Johnston, I. et al (2006) Connecting for Health – Do Once and Share Interstitial Lung Diseasenwww.informatics.nhs.uk/cgi-bin/library.cgi?action=detail&id=2704&dir_publisher_varid=2
Miller, J. (2000) Coping with Chronic Illness: Overcoming Powerlessness (2nd edn). Philadelphia, PA: F.A. Davis Company.
Stewart, M. et al (2001) Group support for couples coping with a cardiac condition. Journal of Advanced Nursing; 33: 2, 190–199.
Wells, A., du Bois, R. (1994) Prediction of disease progression in idiopathic pulmonary fibrosis. European Respiratory Journal; 7: 4, 637–639.