Report from Vilnius meeting

The Lithuanian patient association invited associations from the Baltic countries plus some neighboring countries to a meeting in their capital Vilnius on Friday October 19th. Representatives from six associations accepted the invitation: Latvia, Estonia, Belarus, Ukraine (two associations) and Russia.       

Doctors and other health personnel ran a parallel meeting in an adjacent conference room at the hotel. Ignas Rudys, the president of the Lithuania association, had chosen to postpone the startup of the patient association conference until after lunch so that the participants could have the chance to join the first half of the conference to the professionals.

As with the patient association conference, this was the second time for a Baltic Pulmonary Hypertension Conference for the professionals. In addition to health professional speakers from the Baltic region, professor Adam Torbicki (Poland), professor Marcin Kurzyna (Poland), professor Ekkehard Grünig (Germany) and professor David Montani (France) were speakers at the conference.

Here is a short review of the sessions I attended for the health personnel:

Professor Adam Torbicki presentation was named «What is new in PH – insights from Nice 2018»

Prof. Torbicki started his talk by giving a review of the history of the PH symposium. It started in Geneva in 1973 at the time when PH was classified as either primary or secondary PH. A more granular classification was made and we now have the five classes of PH with PAH as class one. The PH symposium was later held every five years starting in Evian, France in 1998, Venice, Italy in 2003, Dana Point, California in 2008, and Nice, France in 2013 and 2018.

In 2018 symposium reports from 13 task forces were presented. This will lead to papers published by each task force.

One of the major news from the symposium, was that one of the task forces suggested to change the definition of PAH! A healthy person has a mean pulmonary arterial pressure (mPAP) of 13 and a Pulmonary Vascular Resistance (PVR) of 54 (+/- 29). A person is categorized as having PAH if the mPAP is greater or equal to 25 mmHg and the pulmonary arterial wedge pressure (PAWP) is less or equal to 15 mmHg. The new definition of PAH will in the future be mPAH greater or equal to 20 mmHg while the PAWP has the same definition. Prof. Torbicki pointed out the potensial risk of over diagnosing PAH patients due to this change. He also stressed that there were no approved drugs for patients with mPAP between 20 and 25 mmHg. However, the upside is that patients might be diagnosed earlier than before and be put on medication at an earlier stage of their disease.

Task force nine worked with problems related to PH due to left heart disease. Prof. Torbicki pointed out two methods to uncover PH HFpEF (Heart Failure with preserved Ejection Fraction): fluid loading and exercise testing. He also pointed out that there are still no PH drugs for PH patients with left heart failure.

Task force six worked with risk stratifications. The task force suggested a new way to define PH patients with low, intermediate and high risk. They also pointed out the importance of starting the vast majority of patients on initial combination therapy. What’s also new, is that some PAH patients should be considered for referral of lung transplant already at the point of diagnosis.

Task force eleven worked with issues related to CTEPH patients. Ten years ago the only possible treatment for this group of patients was an operation to remove the blood cloths (called Pulmonary Endarterectomy or abbreviated PEA). While this is still the first choice of action for patients with blood cloths that are operable, new therapies now exist. (PEA is the only way to cure patients of PH in addition to lung transplant.) One is balloon angioplasty. Japan is the leading country on these kinds of procedures in the world. And as a third option, there is now medication available. Riociguate from Bayer has been tested and proven to work for CTEPH patients. Prof Torbicki also pointed out that other types of PH medication seem to have an effect on CTEPH patients although not yet proven through clinical trials.

Next was professor Ekkehard Grünig that talked about the task force related to exercise. The aim of the task force was to develop standards for exercise for PH patients. 24 studies had been investigated by the task force. These studies included a total of 784 PH patients. 517 of them were «inpatients» (rehab center), 176 were «outpatients» (supervised by health personal but did not stay at any clinic) and 25 patients were at home. Prof. Grünig pointed out that exercise for PH patients was defined as daily exercise of 15 minutes at low intensity with a heart rate lower than 120 and an O2 greater than 90 %. (If the saturation fell lower, oxygen supply was recommended.) The results of the studies showed that the most effective results were found for those that participated in a rehab program (inpatients). The Quality of Life (QoL) and functional ability were significantly improved. On average one observed a 15 % increase in peak VO2 and a 72 meter increase in six minutes walk! This is equal to or better than any medication. (Exercise is not suggested to replace medication, however, but to be used as a supplement.) The interesting thing was that the one could also measure an increase in hemodynamics of about 20 % after a period of exercise (e.g. a rehab program). However, 9.5 % of the patients experiences some sort of adverse events and there was a possible risk of possible worsening of the NT-proBNP value. (Maybe due to training too hard?)

I talked to Prof. Grünig after his talk and we discussed the possibility of educating more health personnel in order to start up rehab programs in more European countries. He said that he was in the process of starting up a new trial and would welcome new participating countries. He gave me his email and asked me to contact him in order to discuss a collaboration with PHA Europe and national associations.

Next Prof. David Montani talked about Pulmonary Veno-Occlusive disease. He pointed out that one could suffer from this due to mutation of a gene called EIF2AK4 (codes from GCN2 protein). One could also get PVOD/PCH from exposure to Trichloroethylene. Prof. Montani pointed out that PVOD/PCH patients often were responders to Calcium Channel Blockers. However, these were «false» responders and would not have long term effect from CCB therapy. Around 20 % of these patients also developed pulmonary edema and he also pointed out that PH drugs are not effective for this group of patients.

Prof. Marcin Kurzyna then talked about Balloon Pulmonary Angioplasty (BPA). As mentioned before, this method is used when the blood cloths are not operable. He talked about the suggested efficacy of PH drugs:

–     Bosentan: 24 %

–     Riociguat: 31 %

–     Macitentan: 16 %

–     Treprostinil: 25 %

One question from the floor (Andrew from the PHURDA association) was related to the expected efficacy of Sildenafil and Ventavis. Prof Kurzyna pointed out that this had not been tested yet, but the efficacy was probably comparable to the other PH drugs. He also pointed out that one should give medication first and then perform BPA. New methods of printing a 3D image of the vessels of the lungs had also proven helpful prior to BPA. He also talked about new methods of applying the balloons that made the procedures more safe than before. (They now pinpoint different areas prior to covering larger areas. Before they worked on the lungs parts by parts.) Prof. Kurzyna also pointed out the impressive survival rate of CTEPH patients of 95 % three years after a BPA. It was also recommended to stop or reduce anticoagulation prior to a BPA as one was more concerned about bleeding during the procedure than cloths being formed prior to the procedure.

Professor Elena Jureviciene from Lithuania then talked about PH management in the Baltic States. She pointed out that the three countries had five PH centers. However, none of them were part of ERN Lung at present time. She said that the prevalens of PH seemed to be similar in all Baltic countries, but there were different medicine reimbursement schemes. (Estonia seemed to have the best reimbursement scheme.) Prof. Torbicki pointed out that one should use examples from neighboring countries when trying to convince health officials about changing the reimbursement scheme. Prof. Jureviciene replied that they would welcome the help of the patient associations as the politicians seemed to listen more to them than to the health professionals. Prof. Grünig also pointed out the importance of initial combination therapy. Another chair person talked about patients being treated abroad. She felt that it might be better to bring the specialists to the Baltic States than to send the patients abroad. The reason was that in order to send the patients abroad, one first had to solve big logistical, economical and political problems.

Professor Alan Altraja from Estonia then presented the Estonian registry. They had divided the patients from the registry into three groups: low, intermediate and high risk patients. Not surprisingly, the registry showed that the high risk group of patients had the worst survival rate. Prof Altraja pointed out that NT-proBNP seemed to show strong correlation for survival, while the cardiac index (CI) showed no statistical significance. However, the six minutes walk showed a slight statistical correlation for survival.

Prof. Egle Ernimiene from Lithuania then talked about tradition and innovative echo parameters for analyzing of right ventricle function and pulmonary circulation in PH patients. The discussion was too medically sophisticated to repeat in this report, but the conclusion was that there now seem to be many innovated ways of detecting signs of PH and also PH status by using echo.

Then it was time for the patient associations workshop:

Ignas Rudys started by welcoming all the delegates. All together 14 people from eight different countries participated. (I represented PHA Europe at the meeting.) Next I talked about the White Spot Program and the Capacity Building Program and how it could be to help to the associations. I presented a standard image brochure that could be given to patients and doctors (who will give it to patients) that has been proven to be effective in getting patients to join the patient association. I also presented a fundraising leaflet that could be used for fundraising purposes. The image and fundraising brochures can be translated to different languages and the CBP can help pay for translation and printing.

In addition, I talked to each association individually. We discussed the outcome of the CBP workshop in Berlin and the notes that I had taken. Then we discussed any possible adjustments to them and how I will continue to work on an adjusted action plan for each association.

Ramunas Ciuciulka then presented the Lithuanian association and the PH treatment in Lithuania. They have two PH centers and this is also where PH medication can be prescribed. Ramunas also talked about he rehab program that had started in Lithuania as a result of the collaboration with Prof. Grünig. There seemed to be a lot of interest in the rehab program and we decided that I would later give a presentation of my stay at the rehab center in Heidelberg, Germany. 

Ieva Plume from Latvia then presented some booklets that they had made in the Latvian association. One booklet was meant for the patients and carers as a description of the disease and medications and treatment available. Ieva was the main author of this together with some PH doctors in Latvia. 

Next she presented a booklet she had made demonstrating some physical exercises for PH patients. Exercise for PH patients is a hot topic and after some discussion the conclusion was that it would be great if one could make a video version of the booklet that could be shared with the patients. Hall will discuss this further with Gerry and the board.

The group was then split into smaller groups of two people. Ieva wanted each person in each group to list three ways of helping newly diagnosed patients with information about the disease. Each group would then choose one of the alternatives and elaborate more on it and present it to the rest of the groups. The workshop seemed to give a lot of interesting and innovative ideas that could be beneficial to newly diagnosed patients. I will ask Ieva to share the results.

I then presented my experience with my rehabilitation stay in Heidelberg, Germany in 2011. In short, the three week period consisted of daily interval training on a bicycle. The training was 16 minutes of length with two minutes of warm up and two minutes of cool down. The effective 12 minutes of biking was divided into 30 seconds of high intensity training and 30 seconds of low intensity training. The high intensity was, however, not very hard at all. I had, for instance, never a heart rate over 100 during the interval training. In addition, we had some very light strength training using rubber bands. The focus was on the upper body. In addition, knee lifts and bringing the feet up to the buttocks provided some light exercises for the lower body. We performed this type of training 2-3 times a week. We also did some breathing exercises in order to strengthen the diaphragm. In addition, daily walks were encouraged. Healthy eating was also a big issue at the clinic. Meals consisting of lots of vegetables and little processed food were commonplace.

Extensive blood work and peak VO2 testing were performed at day two of the rehabilitation and day 20. The idea was to see if there had been any improvements. I was very confident that no improvement could be detected in my case since I felt that the training had been so light. To my big surprise, I had 15-20 % better results when it came to peak VO2, etc. I have continued daily exercise and continued to eat healthy food after my rehab stay in 2011, and my latest tests show an exercise level on par with health individuals my age!

It was a great and fruitful meeting that seemed to be enjoyed by the participants. I have made action plans that will be shared with each association (in addition to the board members) and in this way follow up the discussion we had at the meeting. We also concluded that it was too little time for discussions and presentations, so that the meeting next year will be at least a full day meeting.

Hall Skåra

Egersund, Norway

Oct 22nd 2018

Photos: Ignas Rudys