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Rewriting the PH story

Pulmonary hypertension (PH) is a rare condition affecting the heart and lungs, with the main symptom being breathlessness.

It is rare, with no single obvious cause, incurable and often diagnosed relatively young. This means that it can be a difficult diagnosis to come to terms with. As our lead researcher Havi Carel often writes, a diagnosis like this can dramatically rewrite the life story of the person affected. A research team in Sheffield, funded by the Pulmonary Hypertension Association, recently explored how short story writing might help people come to terms with a diagnosis of PH and help healthcare professionals understand their experience.

This article was originally published in emPAHsis, the magazine of the Pulmonary Hypertension Association.

To finally get a diagnosis! Amanda has been waiting for it for months, for years even. She has been feeling ill, feeling exhausted, feeling unlike herself and not knowing why. The doctors told her there was nothing wrong, that she was fine, that had she thought about visiting a therapist? But now she has the answer! The answer may bring up more questions than it answers but it explains the fatigue. It explains why she wasn’t able to play with her kids, why she couldn’t take them to school, why she couldn’t help with their homework. The diagnosis doesn’t provide a cure, it doesn’t mean all her problems are solved. But it helps many of them. They say the medications may just be for the symptoms but this is what she has been looking for. She feels normal again. She no longer has to go out in a wheelchair, feeling like an old woman. She can walk up and down stairs unaided. She can drive again. She has got her independence back. The diagnosis is not an easy one and there are still many more challenges to face, but at least now she knows what she is facing. At least now she has some help. At least now she has a reason for it.

This story, based on patient testimony, is drawn from research published by the British Medical Journal Open Respiratory Research, and funded by PHA UK, which has delivered new insights into the impact of pulmonary hypertension for patients. The new approach focused on listening to the stories of people who have PH, and then finding new ways of communicating what was learnt. To do this, one of the researchers, Julia Goddard, wrote short stories inspired by each person of around 200 to 300 words long, with the aim of encouraging discussion about the illness and helping researchers develop a deeper understanding of the effects of PH. The research involved conducting interviews in a variety of locations including on the hospital ward and in patients’ homes. In total, 12 patients (six male and six female) from the Sheffield Pulmonary Vascular Disease Unit took part in the study.

The stories highlighted very powerfully how PH affects people’s lives. The discussions focused on time to diagnosis, impact of diagnosis, complexity of therapy, frequency of hospital visits, access to information and the reassurance of care delivered by an expert unit in a supportive ‘family’ environment. Many participants also spoke about changes in their illness day-to-day, which highlighted the need for healthcare workers to be sensitive to changing patient concerns.

Andy smiles to himself as he sees the confused look on the doctor’s face. He knows full well that this doctor,  and probably most of the people on the ward, haven’t heard of his disease, most people haven’t. And Andy knows roughly what’s about to happen next as well. There’s a few ways that it can go, but it tends to follow a certain pattern. The doctor may try and bluff his way through it. Entertaining, but obvious. They may look it up on their phone. Wikipedia has a lot to answer for. The preferred option is, however, that they ask. After living with the disease for half a decade now Andy has a range of explanations at his fingertips. He has the one for younger children. He has one for when he’s in a rush. He has one for his grandma’s friends who are being polite and enquiring after his health. And, of course, he has one for doctors who admit knowing little about this rare condition. It can become a little awkward if they don’t ask. If they demand that he listen to them, that they are the doctor and they know more, that they understand what the ECG means. Andy may not know what each of the little squiggles on the page mean but he knows his ECG isn’t going to look normal, isn’t going to look like everyone else’s. He could have told them this at the beginning as well, if only they had asked.

Professor Ian Sabroe at The Sheffield Pulmonary Vascular Disease Unit, who was part of the team that carried out the research, said: “Qualitative studies like this allow us to really understand the impacts of PH. Through story development we can communicate the effects of pulmonary hypertension and in turn, highlight the clinical challenges for healthcare workers in caring for patients with the illness.”

Iain Armstrong, chair of PHA UK, and also involved in the research programme, said: “This is very accessible research for patients and their families, healthcare workers and researchers alike. Story development is part of a holistic approach to understanding pulmonary hypertension and can help facilitate patient-focused discussion and interventions.”

A recent study of Parkinson’s disease revealed the value of using story analysis to illustrate the experience of receiving a life-changing diagnosis. The study reflected the need to adapt to changing circumstances in illnesses associated with nearly inevitable decline and with episodes of acute care and changes to therapy. Iain Armstrong said: “This research highlights that paying close attention to the qualitative aspects of pulmonary hypertension can help in the management of the illness. The impact of PH is not static and these stories indicate how patient and family concerns change over time. This has caused patients, family members, clinical practitioners and the public, to pause and reflect.”

The full study is available to download for free at

You can find out more about pulmonary hypertension and the work of the Pulmonary Hypertension Association on their website

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