Photo: Prof. Sean Gaine with Kamil Hamidullah, immediate Past President of PAHSSc, the Turkish Pulmonary Hypertension Association.
This interview was originally published in Turkish on Nefes, the magazine of the Turkish Pulmonary Hypertension Association PAHSSc, on the March 2023 edition. Our sincerest thanks go to the PAHSSc for very generously sharing this interview with the wider pulmonary hypertension!
Click here for the Turkish version on the Nefes magazine at pages 26-29
Prof. Sean Gaine is Consultant Respiratory Physician at Mater Misericordiae University Hospital in Dublin, Ireland, and director of the Irish National Pulmonary Hypertension Unit.
Interview
Could you give some information about yourself? What triggered your interest in respiratory medicine even when nothing was known in a field that did not exist?
I was born in Dublin and went to medical school in Trinity College Dublin, in Ireland. Shortly after graduating I went to further my training and experience in the United States, at the Johns Hopkins Hospital. There I was surrounded by some wonderful physicians and scientists who became important role models in my life as a young doctor. I particularly liked the intensive care unit and the physiology of the cardiorespiratory system, and it was in the intensive care unit that I met my first patient with pulmonary hypertension. It was at a time when pulmonary hypertension was very much in the news, as calcium channel blockers had been discovered to be beneficial in some patients with the condition. Also, echocardiography was growing in popularity and this resulted in an increase in the number of patients being diagnosed with pulmonary hypertension. I decided I would do research to better understand the physiology of the pulmonary circulation. I worked in the laboratory studying the pharmacology of endothelin in the pulmonary circulation and it was this that ultimately led me to join famous PH specialist, Prof Lewis Rubin, at the University of Maryland.
Your dedication to your work is beyond limits – sans frontières. Could you give us some information on your cooperation with Professor Lewis Rubin and your involvement with patients in/from Turkey?
Prof Lewis Rubin had written a very important textbook on pulmonary hypertension and was a pioneer in developing prostacyclin, so I decided to go and work with him and to learn as much as I could. Working with Prof Rubin, I saw patients from all over the United States and from around the world. Indeed it was during this time that I first met Kamil and Fatima, both of whom had pulmonary hypertension and came from Turkey to begin on prostacyclin treatment. I learned enormous amount from Prof Rubin, we had a very busy practice and had many patients on prostacyclin. When Prof Rubin left the University of Maryland, I returned to Johns Hopkins and established a new Pulmonary Hypertension Center. However, after a few years I had the opportunity to come back to Ireland, to be closer to my family, and the opportunity to establish a new pulmonary hypertension service in Ireland.
In the past it was difficult to get diagnosis for pulmonary hypertension. Are there still delays in diagnosis and if so what is the reason?
Unfortunately, I believe there will always be some delays in the diagnosis of pulmonary hypertension. It’s a disease that can creep up quite slowly. Many people with PH initially consider that their breathlessness is due to a lack of fitness, and many doctors are not familiar with the disease or might find it difficult to access the investigations that are necessary to confirm the diagnosis. In more recent years, increased awareness about pulmonary hypertension has meant that many of the medical clinics have become so busy that there is a delay even after the diagnosis has been made to see the most appropriate specialist and begin on treatment. Hopefully, this is something that with patient advocacy it can be improved throughout the world,
In Ireland is there a registry system for PH patients?
Having a registry is very important in managing patients with a rare disease, such as pulmonary hypertension. However, you also need people to enter the necessary information into the registry, and to create reports, so that trends in the diagnosis and outcomes of patients can be readily reviewed. This is a weakness not only in in Ireland, but also in many other countries in the world and it continues to be something that is very important for patient support groups to advocate to increase government funding for registries.
You are the Head of the Pulmonary Hypertension Unit in Ireland. Are there a lot of multidisciplinary centres in Ireland? As for the more distant parts of the country can the first step centres cope with diagnostics, treatment and follow-up?
In Ireland, we have one main PH centre at the Mater Hospital in Dublin where we also have heart and lung transplantation. Ireland is a relatively small country and as a result there is good communication between the various specialists in Ireland and referral to the National centre. There are a number of centres in the other major cities in Ireland that are also very interested in PH with good access to the most important investigations, such as echocardiography and right heart catheterisation.
The home-based rehabilitation programme for pulmonary hypertension patients shown by Ms. Ciara McCormich is very impressive and extremely useful. Is a second phase being considered? Could you comment on the outcome of the ten-week programme please?
Dr Ciara McCormack recently published some very nice work on a home-based rehabilitation program for patients with pulmonary hypertension. Her initial research proposal had to be stopped because of Covid, however, she discovered that it is possible with modern technology to help patients at home to carry out exercises and have a significant impact on overall well-being and exercise tolerance. Dr McCormack recently received a grant to return to the pulmonary hypertension unit at the Mater Hospital and do further research on this topic, so I’m very excited to see if we can expand and grow this area of rehabilitation.
Regarding the progress achieved over the past three decades, do you believe a fourth pathway is close? Will there be a full cure for us the patients?
I believe we’ve made good progress over the past three decades with therapies, however, over the next decade will perhaps see more progress than we have seen over the past three. There is indeed a fourth pathway on its way with the recent discovery of sotatercept. Furthermore, there may even be a fifth pathway discovered soon, with very interesting preliminary data emerging about inhaled therapies that target the growth factor pathways. There are now a lot of pharmaceutical companies taking an interest in PH with new products they wish to pursue. I do believe there will ultimately be a cure for pulmonary hypertension. However, getting there will be incremental, but I’m still very hopeful that it will happen in my lifetime.
What was the impact of Covid-19 in general on patients suffering from pulmonary hypertension?
The Covid 19 pandemic was very difficult for everyone, particularly with the lockdowns and restrictions on family contact and reduced access to hospital follow-ups. In general, patients with pulmonary hypertension followed the public health guidelines, and avoided infection by the virus during the first few waves. Thankfully in Dublin, we saw very few cases of severe Covid in pulmonary hypertension patients. Now that the virus has mutated to the Omicron variety, we are not seeing many hospitalisations due to infection at the current time.
You say that you are fortunate to have the opportunity to engage patients in clinical trials. Are there many patients in Ireland, willing to take part in clinical trials?
There are many pharmaceutical companies now interested in developing drugs for pulmonary hypertension. However, carrying out clinical trials is extremely time consuming and must be done very carefully. In my experience, patients are generally very interested in participating in clinical trials. They understand that there is always a chance they may be on a placebo rather than new active drug, however, patients are very keen to help, knowing that while it may not help them, it could help others now and in the future.
What do think of the importance of patient associations and support groups? How about patient advocacy? The views of Ms. Ferrari have been taken into consideration as a patient in the 2022 European Society of Cardiology and European Respiratory Society guidelines for PH for the first time. Will this have a positive impact on the ideas of physicians?
Patient support and advocacy groups are essential in public hypertension. They are so important in helping new patients come to terms with their diagnosis and also to support those who have had the disease for some time. Equally important, is their ability to work with the clinical teams in speaking to politicians to increase awareness about the disease but also to help access funding to improve services. It was very encouraging to see the importance of advocacy in the new guidelines.
What recommendations would you give a patient who has recently been diagnosed with PH?
it’s always difficult to explain pulmonary hypertension to a new patient and to give the appropriate advice. Every patient is different. It is very important for people to accept and come to terms with the diagnosis and to understand that there are effective therapies available. It is also very important to explain how it is very possible to live a very fulfilling life with pulmonary hypertension and to become a real inspiration to those around them, especially to those who depend on them. New therapies are coming too so the future has never been brighter in PH.
Would you consider lung and lung-heart transplantation as a remedy for PH? In Turkey patients over a certain age are not considered as candidates. What about the situation in Ireland? How about finding donors, is it difficult in Ireland?
I have to say that over the past two decades, lung transplant has been one area that has been disappointing. It is not a readily available option for patients with pulmonary hypertension and the number of patients transplanted every year around the world has not increased sufficiently. Deciding on when is the best time to move from medical treatments to transplantation has been very difficult over the years and it’s not uncommon for someone to be turned down by a transplant program because it is felt they are too frail to undergo the rigours of a transplant. For this reason, I think we need to continue our efforts to maximise the medical therapies that are available rather than rely on lung transplant.
What advice would you give a young doctor who has just started his profession as a respiratory disease physician?
Respiratory medicine is perhaps the best specialty in medicine. We saw during Covid how important it was to have physicians trained in treating pneumonia, low oxygen and the use of ventilators that were absolutely crucial for patients with severe lung disease. It is one of the most fulfilling specialties and there is also the chance that some may subspecialise a little further and take an interest in the blood flow in the lung and increase the number of pulmonary hypertension specialists available for the future.
