Scleroderma and Pulmonary Hypertension
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 experience of an individual living with scleroderma and pulmonary hypertension. Through her story, we gain insight into the daily struggles, triumphs, and complexities of managing these interconnected conditions.
Living with Scleroderma and Pulmonary Arterial Hypertension: A Journey of Rare Resilience
Meet Isabelle Lynch from Laval QC, 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 arterial hypertension.
My name is Isabelle. I am 47 years old, soon turning 48, and I've had asthma since I was ten months old. I am a mother to Kierra, 21, stepmother to Alexandra, 30 and Jeremy, 28, and grandmother (Nana) to Sophia, who will be 5 years old in July and I am expecting two more grandchildren this summer.
The journey of my scleroderma started in June of 2014. I was having a problem with my asthma. The symptoms were shortness of breath and my fingertips turning blue. I also had white spots on my hands. So, I went to see my doctor to figure out what was going on. She changed my inhaler (for my asthma) to a stronger one. It didn't change anything. Also, during that period, I got 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, the shingles, and how thick my skin was. He suspected that I was suffering from scleroderma. Eventually, we did a lot of tests to make sure of the diagnosis, which included a blood test, x-rays, MRI, chest scans, and barium sip. In December 2014, the diagnosis confirmed that I had scleroderma.
I started to see other specialists in 2015: a gastroenterologist, rheumatologist, and pulmonologist. My pulmonologist strongly suggested 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 three years with a kinesiologist.
In June 2017, I was hospitalized for what I thought was a bad asthma attack, bronchitis, or pneumonia, issues I have dealt with all my life. After many tests (MRI, x-ray, barium meal, bronchoscopy), the doctor found out that I had aspiration pneumonia. I had to change my diet to tender, soft food and start a new medication so I wouldn't get gastric reflux. Had to stay away from work for three months.
In January of 2018, I had another case of bad pneumonia for which I got treatment. June 30, 2018, is when I began testing 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 that I went to the hospital by taxi. Just walking from the entrance of the E.R. to the nurse station, I felt like I was climbing Mount 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 thought I was having a heart attack, so they changed to a cardiac protocol. Once again, I had to do many tests to determine what was happening to 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 further, so they transferred me to the Jewish Hospital under the care of PH specialist Dr. David Langleben.
After many tests, Dr. Langleben diagnosed me as having pulmonary arterial hypertension (PAH). I had water in my lungs and on both sides of my heart. He put me on two oral medications: macitentan and tadalafil. The rheumatologist also gave me medication to help my skin become softer. In total, I've stayed five 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 physiotherapist and occupational therapist for nine months to be able to get back to work. Unfortunately, I was not able to get back to work. I've been on disability leave since then.
All my specialists still follow me, but I only see them every six months. My PAH is under control with the help of all medication. As for my scleroderma, it has started to get worse lately. I now have problems with my nose/upper airway 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. I'm able to go for walks (3,000 steps minimum per day) and spend time with my friends and family.
Thank you to Scleroderma Canada who partnered with PHA Canada to produce this blog post. Thank you also to Isabelle for sharing her story.