Are you still able to do all the things you like? How often do you experience anxiety or stress? By answering questions like these you can get an idea of your quality of life. It is often stated that the quality of life of ILD patients is severely impaired. But according to Dr Mirjam van Manen, a medical student at the Erasmus University, a lot is unclear on this topic. For instance, how to reliably measure an ILD patient’s quality of life. Van Manen put medical school on hold to really devote herself to her postgraduate research (PhD) into the well-being of ILD patients. With this research, she hopes to shed some light on quality of life by generating better ways of measuring quality of life and ways to improve it in ILD patients.
‘Measuring a patient’s quality of life is quite challenging’, Van Manen says. ‘All kinds of different things are important. Not just if people are healthy or sick, but also the amount of support they get from their surroundings, if they are still working or not, etcetera. Also, it turns out you can’t measure quality of life with the standard ILD outcome measures such as lung function, lung capacity or blood values. These measures correlate very poorly with how a patient is feeling.’ In her PhD, on which she has worked the last couple of years, and which has culminated in her thesis defense at the Erasmus Medical Center, Van Manen and colleagues tried to find a reliable way of measuring quality of life in ILD patients. They also looked into ways to improve quality of life in these patients.
we have found a promising objective measurement for anxiety and depression.
‘One of the studies we did on measuring quality of life, focused on psychological distress in sarcoidosis patients’, she explains. ‘Most of the time sarcoidosis patients are tired and experience a lot of discomfort from their disease, which can cause psychological distress.’ The amount of stress a person experiences can be measured by a hormone called cortisol, also known as the stress hormone. Normally cortisol is measured in the blood, urine or saliva, but levels vary during the day, making it an unreliable parameter. At the Endocrinology Department of the Erasmus Medical Center, a unique way of measuring cortisol has been developed using people's hair. ‘By measuring the cortisol in the centimeter of hair closest to the scalp we could measure chronic cortisol levels. We investigated if there was a correlation between these chronic cortisol levels in sarcoidosis patients and quality of life measures like fatigue, stress, anxiety and depression. We found that the higher the cortisol levels in sarcoidosis patients, the more anxiety or depression patients experienced. With these findings, we have found a promising objective measurement for anxiety and depression.’
That research can lead to surprising findings, is apparent from Van Manen’s research into clubbing. Clubbing is a common complication in idiopathic pulmonary fibrosis (IPF), and it means that the fingers and toes grow into a rounder shape. Van Manen and colleagues wanted to know if clubbing could be measured objectively and if clubbing also correlated with disease severity and quality of life in IPF patients. Unfortunately, no relation between clubbing and disease severity could be found. ‘That surprised us’, Van Manen says. ‘But what was really surprising: it turned out we don’t even know how to measure clubbing reliably. We have no guidelines, no gold standard.’
It turns out patients cough about 520 times a day!
Measuring quality of life is one thing, but improving quality of life is also very important and necessary, according to Van Manen. One of the symptoms with a huge impact on the quality of life is cough. ‘Until recently, not much attention was paid to coughing in IPF. We measured how often IPF patients cough during 24 hours, using a cough monitor. It turns out patients cough about 520 times a day! This must have a huge impact.’ Her research also showed that an anti-fibrotic agent could alleviate the symptoms of cough, which in turn had a positive impact on quality of life.
But cough is just one symptom. Van Manen and her co-investigators also wanted to see if they could improve the overall quality of life of an IPF patient. ‘Patients expressed the need for better education, psychological and practical support’, Van Manen says. ‘Using these findings, we developed the Patient and Partner Empowerment Program in IPF (PPEPP). It actually is a kind of support group’, Van Manen explains. ‘It seems like kicking at an open door to say IPF patients need more information and support groups, but in IPF this concept is relatively new. So, someone actually had to kick that door open.’ And that is exactly what Van Manen and her colleagues did.
In PPEPP patients and partners meet in small groups and during three afternoon sessions a lot of information was provided by a psychologist, a pulmonologist, an ILD nurse, physiotherapists, a social worker and an oxygen supplier. And of course patients met other IPF patients and their partners. A lot of attention was paid to coping with the disease. ‘After these three meetings participating patients indicated their quality of life had improved. The program had a direct effect, which was fantastic to see. But the most important aspect of my research and the PPEPP’, Van Manen concludes, ‘is the insight to not just focus on treating the disease, but to focus on treating the patient as a whole, including his or her surroundings.’