Comorbidities, the simultaneous presence of two or more medical conditions in a patient, present unique challenges in
diagnosis and management. Often, these conditions can exacerbate one another, leading to complex treatment pathways and outcomes. One example is the relationship between scleroderma and pulmonary arterial hypertension (PAH).
Scleroderma, a chronic autoimmune disease characterized by the hardening and tightening of the skin and connective tissues, can affect various organs within the body, including the lungs. Pulmonary hypertension, on the other hand, is a serious condition marked by high blood pressure in the arteries of the lungs, leading to strain on the heart and potentially life-threatening complications.
The intersection of scleroderma and pulmonary arterial hypertension is rare but may occur in approximately 14% of people with scleroderma. Scleroderma can lead to pulmonary fibrosis and vascular changes in the lungs, contributing to the development of pulmonary arterial hypertension. Conversely, pulmonary arterial hypertension can worsen the symptoms and prognosis of scleroderma, creating a challenging scenario for patients and healthcare providers alike.
In this blog post, we delve into the experiences of individuals living with both scleroderma and pulmonary hypertension. Through their stories, we gain insight into the daily struggles, triumphs, and complexities of managing these interconnected conditions. Let us explore how these patients navigate their journey, finding strength, support, and hope along the way.
Living with Scleroderma and Pulmonary Hypertension: A Journey of Resilience
Meet Isabelle, a 47-year-old woman who was diagnosed with scleroderma a decade ago. Despite navigating the complexities of her condition, Isabelle has approached each day with resilience and unwavering determination. In 2018, however, she faced a new challenge with the diagnosis of pulmonary hypertension.
“My name is Isabelle I am 47 years old soon turning 48 and I've had asthma since I was 10 months old. I'm the mother of Kierra, 21 years old in May and the step-mom of Alexandra 30 years old in August and Jerremy 28 years old in March. I'm also the grandmother (Nana) of Sophia who will be 5 years old in July. I will have the honor to be a grandmother (Nana) twice this coming summer (June and July).
The journey of my scleroderma started in June of 2014. I was having a problem with my asthma. The syndrome was shortness of breath and the tip of my fingers turning blue. I also had white spot on my hands. So , I went to see my GP to figure out what was going on with me. So she changed my inhaler (for my asthma) for a stronger one. It didn't change anything. Also during that period a got the shingles. I received treatment for it. I asked her if it was possible to meet with a pulmonologist. Which I did in September 2014. I had to tell him all about my background with asthma history. and the shingles, and how thick my skin was/he suspected that I was suffering of Scleroderma.
Eventually we did a lot of tests to make sure of the diagnosis. Which included a blood test, x-rays, MRI, chest scans, barium sip. In December of 2014 the diagnosis was: I have scleroderma.
Then I started to see other specialists in 2015: gastroenterologist, rheumatologist, and my pulmonologist. My pulmonologist strongly suggests that I start to go to the gym to help with the stiffness of my body. Because it was really hard for me just to get up from a chair. So I trained for 3 years with a kinesiologist.
In June 2017 I was hospitalized for what I thought was a bad asthma attack, bronchitis, or pneumonia. Because I've dealt with those issues all my life. After a lot of tests (MRI, x-ray, barium meal, bronchoscopy). The doctor found out that I had aspiration pneumonia. So I had to change my diet to tender soft food and start a new medication so I won't get gastric reflux. Had to stay away from work for three months.
In January of 2018 I had another bout of pneumonia for which I got a treatment. June 30 of 2018 is when I all began for Pulmonary Hypertension without knowing it. Once again I was not feeling well due to shortness of breath. I was also thinking I had asthma. The temperature outside was so hot in Montréal (+40 degrees Celsius and added humidex on top = +45 degrees).
So I went to the hospital by taxi. Just walking from the entrance of the ER to the nurse station I felt like I was climbing the Kilimanjaro. I was swollen like Popeye the sailor man. Because I was short of breath and having chest pain they put me on the asthma protocol. They took x-rays and found out it was my heart that was the problem. They were thinking I was having a heart attack.
So they change it to the cardio protocol. Once again I had to do lots of tests to know what was going on with me. They gave me a diuretic to help with the swelling. After a week at Cité de la Santé hospital. They couldn't help me more so they transferred to the Jewish Hospital into the care of Dr. David Langleben (cardiologist/specialist in Scleroderma and P.H.)
Once again after a lot of tests he found out I was having Pulmonary Hypertension. I had water on my lungs and on both sides of my heart. So he put me on the meds: Macitentan and Tadalafil. The rheumatologist had also given me a med to help with my skin to be more soft. In total I've stayed 5 weeks in the hospital.
After my hospitalization I went to the Jewish Rehab Center to relearn how to walk. I was under the care of a Physio and OT for 9 months to be able to get back to work. Unfortunately I was not able to get to work. I've been on disability leave since then. After a year and half of treatment on Tadalafil and Macitaten Dr. Langleben told me that there was a new medication I could take. Because I was really responding well to it. So I've switched to Opsinvy in 2020.
I'm still followed by all my specialists, but I only see them every 6 months. My Pulmonary Hypertension is under control with the help of all medication. As for my Scleroderma, it has started to get worse lately. I now had problems with my nose as well as my lungs.
But I stay positive because I have great support from my friends and family. I call them my Pearl Necklace. I always see the glass half full. And I'm able to go for walks (3000 steps minimum per day) and spend time with my friends and family."
As we witness Isabelle's journey, we are reminded of the strength within each individual facing the complexities of scleroderma and pulmonary hypertension. Now, let's delve into another perspective, that of an anonymous patient living with scleroderma and PH, shedding light on the diverse experiences within this community.
While Isabelle's story highlights one individual's resilience, the experience of living with scleroderma and pulmonary hypertension varies for each person. Through the anonymous account of another patient, we gain further insight into the multifaceted nature of these conditions..
Living with Scleroderma: A Personal Journey
“For those undergoing the diagnostic process or grappling with a recent Scleroderma diagnosis, the road ahead may seem daunting. Personally; the pursuit of a diagnosis proved to be the most arduous aspect of my journey. It required a year and a half of relentless self-advocacy. The process was a rollercoaster of dismissal from medical professionals, misdiagnoses, and urgent medical procedures.
Eventually, I received my diagnosis; albeit six months after self-diagnosing through meticulous symptom observation and blood work analysis. This process, though anxiety-inducing, emphasizes the necessity of self-advocacy in a healthcare system that often focuses more on quantity than quality of care.
Oddly, there is a sense of relief upon receiving a diagnosis. It signifies the end of uncertainty yet marks the beginning of a journey filled with new challenges—navigating treatment protocols, conducting research, and adapting to the disease's unpredictable nature. Acceptance of this new reality involves grieving the life you once knew and embracing flexibility in the face of an illness with diverse manifestations among individuals.
Personally, the support of loved ones and connecting with those facing similar challenges have proven invaluable. Living with Scleroderma demands adaptation. My primary symptom, lung involvement has necessitated lifestyle adjustments, but through dedication to my medical plan, stress management, dietary changes, and regular exercise, I've reclaimed a semblance of control. With time, I've found my balance, which I know will be ever changing. While I still live with the disease, it no longer dictates every aspect of my life.
For those with Scleroderma, take it one step at a time. Cultivate a supportive medical and social network; prioritize holistic well-being, and maintain meticulous records to effectively communicate with healthcare providers. Advocacy for oneself is paramount—ask questions, seek clarification, and stay informed about treatment options, and potential side effects of medications. Knowledge is empowering, and staying informed enables you to make informed decisions about your health. While Scleroderma presents serious challenges, it does not define you. With perseverance and support, you can live a fulfilling life despite the challenges.”
In the intertwining narratives of Isabelle and our anonymous patient, we find common threads of resilience, determination, and the power of community in facing the challenges of scleroderma and pulmonary hypertension. Their stories serve as a reminder that while these conditions may present obstacles, they also inspire hope and strength in the face of adversity.
As we continue to raise awareness and support for individuals living with scleroderma and pulmonary hypertension, let us strive to amplify their voices and champion their journeys towards health and well-being. For more information on pulmonary hypertension and available resources, visit www.SometimesItsPH.ca.
If you enjoyed this post, consider reading our other blogs, such as, Community Impact: Scleroderma Ontario's 2024 Grants