Remote monitoring for patient with idiopathic pulmonary fibrosis

Pieter Van Der Wal first noticed something was wrong on a climbing trip with his wife Nelly in 2013. “I was out of breath, and struggling with the climb”, he tells The Lancet Respiratory Medicine. Pieter, now aged 74 years, believed the problem was with his heart, and that is what he told both the doctor where he was on holiday in Austria, and his local general practitioner (GP) when he returned to his home in Ridderkerk (The Netherlands). As he didn't do quite so much physical activity, the symptoms of breathlessness went away. And Pieter thought there was nothing seriously wrong. But, periodically, the symptoms returned, and appeared to be getting worse. “My GP recommended an x-ray of the chest, but this also found nothing wrong.” Pieter continued this way for around 1 year, increasingly struggling with breathlessness, until he could take no more and this time his GP referred him to a regional hospital in Zwijndrecht. He then had to do tests, including bloods, lung scans, and bronchoscopy. Another year after this, Pieter was referred to a specialist centre for treating lung disease in The Netherlands, The Erasmus MC in Rotterdam. Just one month after his first visit there, in January, 2018, he was diagnosed with idiopathic pulmonary fibrosis (IPF)—a progressive condition that causes irreversible scarring and loss of function of the lungs. IPF falls under the umbrella of interstitial lung disease (ILD), a collection of lung conditions that may cause similar symptoms and progression. Pieter was, of course, relieved to finally have a diagnosis. But, the overall outlook was not a positive one. “That day it was made clear to me that IPF is a life threatening and progressive illness and, depending on how well the medications work, it will slow down the progression of the disease, but never reverse it”, he explains. “When I asked for the worst case scenario, I was given 3 to 5 years, without treatment, and that was in January, 2018.” Peter was born and raised in Ridderkerk, near Rotterdam, and spent his early career working at a Dutch company that provides supplies for the offshore oil industry. This included a 4-year-stint in Aberdeen, Scotland, in the 1970s, where the couple's daughter Patricia was born. After this, he worked in the paint industry in various management roles for two different companies before his “retirement” in 2018. He then worked part time for a friend's company that manufactured devices to produce drinking water from sea water, travelling to many locations worldwide. There was nothing in his work history to suggest he would be at increased risk of IPF except smoking, but he had given up smoking in 1982. Upon his diagnosis, Pieter was offered a choice of two medications, pirfenidone, which modifies the action of various cytokines and growth factors, or nintedanib, a tyrosine kinase inhibitor with antifibrotic properties. Because he was being treated for dermal problems, it was explained that pirfenidone might be less suitable (due to skin related potential side effects), so he chose nintedanib. “Of course, both choices have side effects, but with nintedanib I am having to visit the toilet frequently due to diarrhoea”, he explains. “But otherwise, I generally tolerated it well.” Other supplementary treatments such as low dose prednisone help Pieter manage the regular coughing that comes with IPF. Pieter was also given some lifestyle advice and direct help to improve his physical activity. “The medical team advised me that strengthening my muscles and improving my general condition and endurance would help. So I went, and am still going once a week to a fitness school where, under the supervision of a physiotherapist, I do my exercises plus a bit extra”, he explains. Even during the COVID pandemic when the fitness school was closed, Pieter was given special permission to train with his therapist in the empty building, while social distancing. “And once a week since my diagnosis, no matter how good or bad the weather conditions, accompanied by my daughter, we go for a walk, as long as my condition allows me, with my Nordic walking sticks. At the moment we do between 4 and 5 km with the support of oxygen.” The unexpected thing he was offered at his diagnosis was a chance to join the IPF online Home monitoring programme—a pilot telemedicine programme by Catherina Moor and colleagues. Pieter was able to use a portable spirometer to take several lung measurements daily. “Using my tablet, the results are sent by a secure connection to the hospital so they can check my condition on a daily basis. Also, e-consultations and video consultations are part of IPF online.” During a summer holiday after his diagnosis in Mono Lake (Yosemite, CA, USA) spring 2019, Pieter suddenly got a cough attack, became very short of breath, and felt very sick. “I sent an e-consult to my doctor back in The Netherlands and despite the time difference, I got several questions (such as, what is the altitude?) and advice at very short notice. I followed the advice of course (mostly about not going to very high-altitude locations), but this incident, and the way we dealt with it, boosted my self-confidence that in critical situations I could turn to someone with knowledge of my medical background who could help me.” Later, he would also be given portable monitoring technology to measure step count and a pulse oximeter to keep track of his blood oxygen saturation. And, when the COVID pandemic exploded in March, 2020, Pieter remembers the interest in remote monitoring technology exploding with it. “Suddenly everyone was interested in this technology for all kinds of conditions. One of the most rewarding things I did was join several Zoom meetings with trainee doctors to talk them through the patient perspective on the technology. Moments like that were such a good experience during the boredom of the pandemic”, he explains. He also appeared on television with his doctor, Marlies Wijsenbeek, to discuss the benefits of monitoring technology. Pieter has co-authored a paper with Marlies Wijsenbeek and colleagues on home monitoring in interstitial lung diseases, again able to give his unique patient input. While periodic scans have revealed that the condition of Pieter's lungs is deteriorating as expected, it is functional capacity that matters most to him, and he continues to perform the 6-minute walking test to 570 m, and his spirometry readings are stable. Pieter has also been active in trials for new drugs. He started on a drug trial in 2021, using a new agent, ziritaxestat, from the pharmaceutical company Galapagos in addition to continuing with nintedanib. “I knew I was receiving the active form of the medication, not the placebo, because of the side effects I was experiencing”, explains Pieter. “But this new drug appeared to keep my condition stable, and I was happy with it.” However, late in 2021 this trial stopped. “I decided at this point with my doctor that I would switch to trying pirfenidone instead of nintedanib. Pirfenidone has a different side-effect profile, including sensitivity to strong sunlight, which unfortunately I suffer from.” Early in 2022, Pieter has begun a new study using monthly infusions of the new drug PRM-151, manufactured by Roche. This means he must do the 6-minute walking test monthly when he goes for his infusion, whereas before it was twice a year. There are, as Pieter and doctors expected, signs of progression of his disease. He continues with his physical therapy, but now must have supplemental oxygen while training, during the walking sessions with his daughter, and also while doing the walking test. And news has just arrived of a further complication—during a recent PET scan of his lungs, doctors also detected a 4·7 cm aortic aneurysm, for which Pieter is now receiving oral medication. When it reaches the threshold for treatment (5 cm) doctors might consider further intervention. However, he concludes, “All in all I am doing as well as can be expected, and getting on with enjoying my life”, he says. At the time of writing in June, 2022, he, Nelly, and Patricia are preparing to go on a holiday to Vancouver, Canada, for a month. (This writer has warned them that the city has many steep climbs!). “I'm confident if I have any issues, the remote monitoring system in place will be effective and I'll get any advice and support I need quickly. We are really looking forward to this trip.” Contact for Pieter Van Der Wal, Contact for Pieter Van Der Wal,