21. For the purposes of this statement, we have looked at each attendance individually.
22. The following guidance is relevant here.
23. The NICE guidance (QS79) says on diagnosis pulmonary fibrosis:
‘Idiopathic pulmonary fibrosis is difficult to diagnose. The main symptom is shortness of breath that gradually becomes worse. It can only be diagnosed with confidence by a specialist multidisciplinary team with expertise in interstitial lung disease. Diagnosis is based on clinical features, lung function, radiological findings and pathology if indicated. Because of the severity of idiopathic pulmonary fibrosis and its poor prognosis, being incorrectly diagnosed with this disease can cause unnecessary distress for the person and their families or carers’.
24. The guidance describes the presence of clinical features of pulmonary fibrosis:
‘These are:
• age over 45 years • persistent breathlessness on exertion • persistent cough • bilateral inspiratory crackles in the chest • clubbing of the fingers • normal or impaired spirometry, usually with a restrictive pattern but sometimes with an obstructive pattern.’
25. This guidance also says on when a patient should be given a diagnosis:
‘People who might have idiopathic pulmonary fibrosis receive a diagnosis only after their symptoms and any test results have been discussed by a team of healthcare professionals who specialise in diagnosing and treating lung diseases. This will help to make sure that people are given the correct diagnosis.’
26. The NICE guidance (TA504) on treating pulmonary fibrosis says:
‘Pirfenidone is available on NHS as a possible treatment for idiopathic pulmonary fibrosis in adults, if:
• their forced vital capacity (FVC, a test of lung function) is between 50% and 80% of the value expected for them.’
27. Anti-fibrotic treatment can extend the life of a patient however is not a cure for pulmonary fibrosis.
28. Our physician advisers said pulmonary fibrosis is hard to diagnose on a chest X-ray. Patients with pulmonary fibrosis often do not exhibit any symptoms for long periods. If there is significant concern a patient is presenting with symptoms of pulmonary fibrosis the expectation is the Trust would arrange for a CT scan referral. The symptoms for pulmonary fibrosis often cannot be differentiated from other symptoms associated with heart problems (for example, breathlessness).
First attendance – March 2017 (Trust 1)
29. GMC GMP says ‘You must provide a good standard of practice and care. If you assess, diagnose or treat patients, you must: a. adequately assess the patient’s conditions, taking account of their history (including the symptoms and psychological, spiritual, social and cultural factors), their views and values; where necessary, examine the patient b. promptly provide or arrange suitable advice, investigations or treatment where necessary c. refer a patient to another practitioner when this serves the patient’s needs.’
30. In April 2017 the Trust performed a chest X-ray after Mr A presented with chest pain. The Trust radiologist noted the possibility of underlying pulmonary fibrosis or early pulmonary oedema (fluid in the lungs). The Trust radiologist report of this on 1 April 2017 said the X-ray findings were suggestive of pulmonary oedema. This is what the Trust treated Mr A for in 2017.
31. Symptoms commonly associated with pulmonary oedema include breathlessness, wheezing, coughing up foam and loose mucus, chest tightness and difficult breathing. It can be life threatening and requires immediate treatment.
32. Our radiology adviser said the chest X-ray was reported correctly. The X-ray showed changes that can be associated with both pulmonary fibrosis and pulmonary oedema.
33. Our physician adviser said that when considering the potential diagnoses, it was correct for the Trust to concentrate upon Mr A’s immediate and pressing problem. They said that in the context of his acute symptoms and cardiological presentation the Trust did not unreasonably attribute these to pulmonary oedema. Mr A did not present with symptoms of chronic lung disease such as breathlessness and cough which would have been suggestive of interstitial lung disease (pulmonary fibrosis).
34. We know pulmonary oedema can present similar to pulmonary fibrosis. In the absence of other clinical features to suggest pulmonary fibrosis over pulmonary oedema we are satisfied the Trust acted in line with GMC GMP in adequately assessing Mr A and promptly treating his heart problems.
35. There is no expectation or guidance to say patients should be told everything a Trust might consider during their attendance. This would be considered unnecessary and over informing the patient. It is neither practical nor reasonable to expect a Trust to disclose everything they may consider during an assessment of a patient’s condition.
36. At this time the Trust not unreasonably diagnosed and treated Mr A for pulmonary oedema. We have seen no indications it did anything wrong.
37. Second attendance – July 2020 (Trust 2)
38. In March 2020 Mr A attended for a pre-operation (triple heart bypass in July 2020) assessment at the Trust. The Trust performed a chest X-ray and reported it as showing ‘bilateral fibrotic lung changes most likely long standing’.
39. The Trust said pre-operative X-rays indicated some changes in his lungs from previous chest X-rays. It said these changes are reasonably common to heart patients and were maybe pulmonary congestion (fluid on the lungs) caused by Mr A’s underlying heart condition.
40. The Trust said at Mr A’s July 2020 pre-admission checks Mr A made no mention of breathlessness as a main symptom. When these changes (in lungs shown on chest) are not found with breathlessness the Trust tend not to investigate as investigations are based on symptoms and not just X-ray reports.
41. Our radiology adviser said the Trust correctly reported on its pre-admission chest X-ray and described fibrotic changes as long standing. They agreed these features are also suggestive of pulmonary oedema.
42. Our physician adviser said it was reasonable for the Trust to give precedence here to treating Mr A’s immediate and most pressing life-threatening cardiac problems.
43. In the absence of Mr A presenting with other symptoms suggestive of pulmonary fibrosis we have no compelling evidence to say the Trust should have initiated further investigations into this at the time or consider. We are satisfied the Trust was correct in identifying and giving precedence to treating Mr A’s cardiac problems as required in line with GMC GMP (paragraph 34).
44. On 21 August 2021 the Trust said follow up documentation recorded Mr A walking without limitations. He denied chest pain or breathlessness, and he was therefore discharged from service with regular cardiology outpatient follow up until December 2022.
45. Mr A seems to have made a good recovery and symptomatically was quite well with no breathlessness or chest pain on walking as mentioned in discharge summaries of the time. The Trust treated Mr A’s immediate presenting cardiac issues.
46. We have no evidence of the Trust acting in line with GMC GMP in referring patient to another practitioner when this serves the patient’s needs.
47. The Trust radiology report references bilateral fibrotic lung changes. We have seen no evidence of the Trust informing Mr A’s GP of his April 2020 chest X-ray findings and suggesting further follow-up or referral to respiratory services for an assessment. This was not in line with NICE QS79 on diagnosis.
48. But we have seen no indications that if this happened Mr A would have been diagnosed with pulmonary fibrosis or received anti-scarring medication. As we have already explained, pulmonary fibrosis shares symptoms with other conditions Mr A had been treated for. Even as late as August 2021 the evidence shows Mr A was not presenting with symptoms of pulmonary fibrosis or showing any indications of progressive illness. We cannot say there would have been anything to tell Mr and Mrs A in 2020, or that any treatment would have been initiated, given his lack of symptoms. That being the case we will take noy investigate any further.
49. Mrs A may like to know that, even if a diagnosis was possible in 2020, it is unlikely Mr A would have been eligible for any treatment at that time. In 2022 Mr A had an FVC of 65% (likely due to exacerbation of his acute infection at that time). This is within bounds for anti-scarring medication. Our physician adviser said it is quite likely that Mr A would not have met the criteria for anti-fibrotic treatment in 2020 as he was not presenting with features to suggest persistent and progressively worsening breathing.
Third attendance – October 2022 (Trust 1)
50. In October 2022 the Trust admitted Mr A for three days suffering breathlessness and exhaustion.
51. The Trust recorded a good history. Mr A is documented as being able to walk daily however had noticed changes over the previous three months. This suggested Mr A’s symptoms had become more acute recently. The Trust conducted a chest X-ray which showed extensive changes from his previous chest X-ray in March 2017. Trust 1 did not have access to Trust 2’s scans from 2020.
52. The Trust diagnosed Mr A with COVID-19, and discharged him home after three days when he required no further treatment. COVID-19 can present in a similar way to pulmonary fibrosis and the Trust could not provide a diagnosis of pulmonary fibrosis at this time despite evidence of extensive changes in his lung scarring. The Trust ordered a follow up chest X-ray for six to eight weeks later and a plan for further investigations if it showed persistent changes.
53. Our radiology adviser said the Trust reported correctly on the chest X-ray. There was a significant change for the worse in Mr A’s chest in October 2022 compared with that October 2020. The Trust correctly treated the immediate problem (COVID—19) and initiated further investigations (high resolution CT scan, full breathing function test and walk test) on its suspicions of pulmonary fibrosis.
54. The Trusts actions here are in line with GMC GMP on promptly arranging investigations. It could not yet give a diagnosis of pulmonary fibrosis however it started process for doing this in line with NICE QS79.
55. In November 2022 Mr A was readmitted to the Trust for seven days with further difficulties. The Trust informed Mr A he had terminal interstitial lung disease (pulmonary fibrosis). The Trust said he could have anti-scarring medication after follow-up tests with the specialist service (chest clinic) who could prescribe this. The Trust treated Mr A with anti-inflammatory steroids then discharged Mr A home.
56. The Trust actions here are in line NICE QS79. We are satisfied the Trust told Mr A and Mrs A of his terminal illness when it had sufficient evidence to do so. It could not have done this in 2017 or 2020.
57. Its actions after this are then in line the BTS guidance which says ‘if there is evidence of clinically significant interstitial lung disease (ILD) such as organising pneumonia or pulmonary fibrosis, patients should be considered for referral to Regional Specialist ILD service’ (i.e. respiratory service). This is what the Trust initiated as well as assessment for eligibility for anti-scarring medication.
58. We have seen no indications the Trust did anything wrong in late 2022.
59. Sadly, Mr A deteriorated rapidly and was readmitted in December 2022 before the CT scan and specialist service involvement could take place. The steroids had not been effective, and he required more oxygen. Mr A died ten days following his admittance.
60. Our physician advisers said a rapid decline can happen to patients who have pulmonary fibrosis especially after acute infections such as COVID-19. It is unlikely anti-scarring medication if given to Mr A in October 2022 would have significantly affected this.
61. That concludes our statement. We are very sorry to hear about what happened to Mr A’s health and about the sad circumstances of his death. We know this has been a very difficult time for Mrs A.