IPF. Three fairly innocuous letters. It might be a firm of accountants, the model number on a printer, or a particularly dull tax form. It doesn’t sound exciting. It doesn’t sound like a problem. It certainly doesn’t sound deadly. But the truth could shock you.
IPF – Idiopathic Pulmonary Fibrosis – is a chronic and progressive lung disease. Around 15,000 people in the UK are living with a diagnosis of IPF and at present there are 5,000 new cases diagnosed every year. IPF patients can expect to survive an average of three years from diagnosis, with only one in five surviving for more than five years. Around 5,000 people will die of this disease in 2014, and that number is only going up. This means that more people die each year from IPF than from better known diseases such as ovarian cancer, kidney cancer and leukaemia yet most people – and even many doctors – have never heard of this devastating condition. That must change.
The term “idiopathic” means the origin and cause of the disease is not known, but we do understand much about its effects. It causes scarring of the lungs and hinders a person’s ability to breathe as the amount of oxygen that can pass from the lungs into the blood has been significantly reduced. The main symptoms are similar to other lung diseases, including breathlessness, a persistent dry cough, tiredness, loss of appetite and weight loss. Over time, its symptoms are associated with a decline in lung function, a reduced quality of life, and ultimately death. Short of a lung transplant, there is no cure for IPF yet known.
But we are starting to see the first signs of a possible light at the end of what has been a very dark tunnel. Although we can’t yet repair the damaged areas of the lung, emerging treatments are showing encouraging effects in slowing the progress of the disease. We’re also fortunate in Scotland to have a tight-knit and growing community of scientists, doctors, nurses and other professionals working in this field. They work across all corners of the country but come together regularly to share best practice and experience on nationwide basis.
One of these world-leading experts is Dr Nik Hirani of University of Edinburgh, who said: “Five years ago it seemed unlikely there would be a drug treatment for IPF that ‘worked’ and would be available in the foreseeable future. Now we have one drug licenced and approved for use and another which, based on new clinical trial data, may also be available to NHS patients within 12 months. No-one claims these drugs cure IPF, but in appropriately selected patients both drugs appear to slow the rate at which the disease progresses. As with all powerful drugs, there are side-effects and not all patients will benefit from treatment, so it is heartening to know there are on-going studies of novel drugs that carry with them the hope for a treatment that stops the disease and improves well-being and life expectancy.”
But much more needs to be doneand the British Lung Foundation has identified IPF as a priority. We’re funding programmes to study the causes the disease and find new and innovative ways to stop it. And we’ve launched an IPF Patients’ Charter to ensure that all people affected by this condition are able to access appropriate treatment and support consistently around the country.
A key element of this is early and accurate diagnosis and fast-track access to a dedicated multi-disciplinary team of expert medics working together. This level of support can transform the quality of care received by IPF patients and their carers. Getting there will require a modest level of investment and new capacity, but we know it’s worth it. It’s also an area where co-operation across Health Board boundaries can pay dividends in terms of patient experience and efficiency.
So far the signs are encouraging. Scotland’s respiratory clinicians are working together across the nation to maximise the expertise and support they can offer to patients. And the Scottish Government has signalled its support for a Scotland-wide response to this terrible disease.
We now need similar leadership and commitment from Health Boards around the country to identify innovative ways of working in partnership to maximise the services and support that can be offered, even while budgets and resources come under continual pressure. As we look ahead to a day when we have a cure for IPF, we all need to work together to provide support for those people affected by the daunting challenge of facing IPF ‘in the here and now’.
• Dr James Cant is head of BLF Scotland & Northern Ireland www.blf.org.uk/scotland