Author: Pisana Ferrari, May 25, 2023, based on interview
Introduction
In the past few decades, significant strides have been made in the field of chronic thromboembolic pulmonary hypertension (CTEPH), encompassing enhanced disease comprehension, innovative therapies and surgical advancements. However, living with CTEPH continues to pose physical, practical, emotional, and social challenges for patients, families, and caregivers. Reaching a timely diagnosis remains a significant hurdle as CTEPH is a rare condition with symptoms that can mimic more common ailments. Patients often lack awareness of this potentially life-threatening disease and may not connect their initial symptoms to it. Insufficient knowledge among the general public and healthcare professionals adds to the concern. Evidence shows that early detection and diagnosis are crucial for enhancing patient outcomes.
Rishabh’s path from Symptoms to Diagnosis
Rishabh, an athletic 20-year-old university student from Singapore, experienced symptoms that persisted for several months, starting in May 2022, before seeking medical attention. Being young, fit, and actively involved in sports, he initially underestimated the significance of his symptoms. It was only after experiencing a sudden loss of consciousness, in the month of August 2022, that he realized the urgency of the situation and decided to take action. This underscores the challenges faced by individuals with less common or atypical symptoms, especially when their age and physical fitness may lead to initial assumptions, or overlooking, of potentially serious conditions. Despite initially being misdiagnosed me with asthma, the doctors in Singapore were comparatively very quick to diagnose him, as it commonly takes 12-18 months for CTEPH patients to receive an accurate diagnosis.
Addressing the Toll on Emotional Wellbeing
At the time of his diagnosis, Rishabh made a conscious effort to maintain a positive outlook, hoping that treatments would lead to improvements in his condition. With the passage of time and the ongoing deterioration of his health, Rishabh found himself increasingly grappling with emotional issues. Recognizing the need for support, he turned to psychological counseling and therapy. The impact of Rishabh’s diagnosis extended well beyond himself, reverberating through his family. His mother, in particular, was devastated by the news, describing the moment of diagnosis as if a bolt of thunder had struck her. The pain and worry she experienced are a testament to the profound effect such diagnoses can have on loved ones, emphasizing the need for comprehensive support systems.
Seeking Expert Medical Support Abroad
Rishabh’s condition took a rapid turn for the worse, and within a span of just six months, he became unable to perform even the simplest tasks of daily living. Additionally, he experienced several episodes of syncope, further compounding the challenges he faced. Due to the rare nature of the condition, and the deep experience required for the highly specialized procedure, Rishabh and his family decided to seek out one of the most experienced BPA centers globally based in Okayama Japan, led by Prof. Hiromi Matsubara. Rishabh and his parents are overwhelmed with relief and gratitude towards Prof. Matsubara and his team, as they reflect upon the remarkably positive medical outcomes he has achieved, and the exceptional care and support throughout their time there.
A Journey of Hope and Remarkable Outcomes
After undergoing a few rounds of BPAs, Rishabh’s hemodynamics have completely stabilized and returned to normal values – for example, his mPAP (mean pulmonary arterial pressure) was above 50mmHg at peak and reduced to 17mmHg at the end of his final procedure. Since February of this year, he has regained his health, confidence, and resumed his studies and activities, embracing life once again. Rishabh and his parents are overwhelmed with relief and gratitude as they reflect upon the remarkably positive medical outcomes he has achieved. They kindly accepted to be interviewed for this article because they believe it is of vital importance to encourage patients to seek expert medical support as soon as possible, and to spread awareness of CTEPH and the life-saving medical opportunities that are now available.
The Need for Holistic Care and Advocacy
Rishabh’s experience emphasizes the importance of holistic care that encompasses both physical and mental well-being. It serves as a reminder that a comprehensive support system is essential for individuals facing chronic illnesses. The imperative of ensuring access to potentially life-saving interventions and ensuring that all individuals with CTEPH have a fair chance at a better quality of life underscores the pressing need for heightened awareness, advocacy, and concerted efforts to expand available resources.
Many thanks to Rishabh and his parents for the interview and to PH Singapore for having put us in contact.