I’m Aware That I’m Rare: Maria Morais (490)
the phaware® interview
Maria Morias, a registered nurse from Canada, shares her journey of managing multiple autoimmune diseases, including her recent diagnosis of pulmonary arterial hypertension. Previously dealing with scleroderma, Raynaud’s phenomenon, and a rare liver disease leading to a liver transplant, Maria’s ongoing symptoms of shortness of breath were eventually linked to pulmonary hypertension. Her story reflects on the psychological impact of chronic illness and the empowerment gained from community support and self-advocacy.
My name is Maria Morias and I live just outside of Toronto Ontario in Canada. I’m 54 years old and I’m newly diagnosed with pulmonary arterial hypertension. I have other autoimmune diseases. So, to sort have another disease added to my list has been overwhelming to understand, but also it’s given me an answer to my shortness of breath.
My pulmonary hypertension is considered Group 1, associated with scleroderma. I do have mixed connective tissue disease. I’ve been seeing a rheumatologist for a number of years. In my early 20s, I was first diagnosed with Raynaud’s phenomenon and circulation issues of my hands and feet that have been severe throughout my life course.
Then, in 2018, got diagnosed with a major rare liver disease, autoimmune rare liver disease called primary biliary cholangitis. I developed several severe symptoms of fatigue, itchiness, brain fog, and shortness of breath. So, the shortness of breath I sort of figured, at that time, was due to ascites and a massive fluid buildup in my abdomen, where towards the end of my liver disease, just pre transplant, my liver became cirrhotic and not effective in what it was needing to do. So, fluid is backing up into my abdomen. I’d gain about 20 pounds a week, go in on a Friday morning and have fluid drained, 10 liters of fluid drained. I thought there was a lot of compression on the diaphragm causing a lot of shortness of breath.
Pulmonary hypertension was never on the radar of any of my physicians, my hepatologist, my rheumatologist. Post liver transplant in 2021, I started to recover. That was a pretty intense recovery of regaining my energy and short walks, but I was still having severe shortness of breath. Slowly along the way, I had the respiratory sort of check out with a six-minute walk. I’ve been doing that every six months with a respirologist and pulmonary function test.
It wasn’t until last probably December of 2023, my rheumatologist said, “Let’s do some more testing.” I did have interstitial lung disease as a general diagnosis from the respirologist, but this rheumatologist thought, “Let’s check some other things.” So he sent me for an echocardiogram, which I thought, “Well, how does this work?” The rheumatologist looking after respirologists sort of work. It was a good thing, right? So, never discount somebody’s professional opinion as an option. Of course, I wanted to inform my respirologist and he said, “Sure, no problem.” So good thing that there wasn’t any territorial or issues there.
My pressures came back pretty high on echo. So, that led to sort of the next investigation. Then, I was referred to a respirologist that actually deals more, and specifically with pulmonary hypertension. That was earlier this year in 2024, to then go in for the gamut of testing. So, I started off with a sleep study and I thought, “Well, what does a sleep study really have to do with my shortness of breath?”
I am a registered nurse. I work in public health. I’ve been a nurse for over 32 years, but more from a health promotion angle. So, it’s kind of ironic that I’m on the other end lately as that patient and trying to advocate for my own health. It’s really important, right, to navigate through the system, to understand the various healthcare provider roles and where we sit as a patient, how you’re feeling, what you’re feeling, all your appointments and putting everything together. It’s quite overwhelming even with someone that can have ownership over your own health and control. However, you sort of lose that control when you leave it to others to kind of put your story together of coming up with what you really have.
I did go for the sleep apnea test, and it was about, well, if you feel more rested then you’re better able to cope with your day. So, there was some mild apnea with about seven episodes per hour. But then when I went into REM, it was 24 episodes per hour. I thought, “Okay, so there might be something there.” I have not progressed yet to use a PAP machine, so that’s kind of on my radar of consulting with others to see if I really need to invest in that or not. Then, I went for a bubble echocardiogram, which was pretty interesting to see these little effervescent bubbles go through my heart. I was watching the screen just out of curiosity. It did show that I did have a small hole in my heart. Apparently, that’s been there since birth. I said, “Well, is that the problem of my shortness of breath?” The respirologist said, “Well, you’ve lived with it for 54 years, so that probably is not the problem.”
Then, the next sort of definitive exam was the heart catheterization, which sounded pretty scary, right? Having a tube put in right through to your heart can be very scary. The image of something touching your heart, what could happen. So again, went in. It was very simple. I think I was on a relaxation sort of medication. I wasn’t put under. It was quick. There we had it, I think it was 30 milligrams of mercury. Certainly I think the cutoff is in the 20s. I’ve now started on a couple of medications. We had to go through a lot of insurance hoops to get that approved. Thankfully, for the Toronto UHN Hospital, they have patient navigation mechanisms that made it very easy. On the back end, they did all the paperwork. Then following that, it was kind of a leveling up, see if I had any major side effects that they listed off, swelling, some discomfort, some cramps in the legs. I had a little bit of that, but then tolerated it. Now, just a couple of weeks ago, have added an additional medication. I still have yet to feel any sort of changes. I understand that it could be six months to a year with a repeat of the heart catheterization.
Having a diagnosis is important because then you ask, “Well, how did this happen? Why did it happen?” Leading it all back to, in my case, the autoimmune and scleroderma. Scleroderma was always something that was kind of query on my medical record. I said, “Well, how does that express itself? What am I going to see over the years?” Here it is, the starts of that as well as the interstitial lung disease. I have some start of kidney disease, as well. Also, autoimmune. I’m at stage 3A, which there are five stages, so I still have room. But knowing that how quickly my liver disease progressed to be needing a liver transplant, of course one of the things that goes through my mind is lung transplant. I try to sort of keep in check with that and take one day at a time.
Right now, I’m very, very early in the diagnosis, second, third month in. I’m reading a lot, I connected with the Pulmonary Hypertension Association of Canada to get credible information and to start to connect with people that are also experiencing it. I’m in Facebook groups and just learning. One of the things that I do with my liver disease is I belong to the Canadian PBC Society, and I do a lot of patient advocacy there. I’m actually the VP patient advocacy. It is a totally volunteer organization across Canada. I have met some really wonderful people, lots of great peer patients. I do a lot of mentoring. I’m hoping maybe I can explore some opportunities into the pulmonary hypertension world and learn for myself and help each other sort of cope with all our uniqueness of all these rare diseases.
As a nurse, one of the things that I always keep in mind is this psychological component of disease and healthcare challenges. Being in the nursing role, you have the resources, you have the referrals, you have the connections. But then finding myself in a patient role, it’s very much different, because it’s being done to you and upon you. What I found is that I had to bring my voice forward and have my symptoms be heard. A symptom like fatigue is something that is easily described by a mother, a working mother, children, balancing work life care provider of parents, multiple things. We’re always tired. We’re a society that’s tired because we’re doing it to ourselves. Technology makes things easier. Therefore, we do more.
To really be as a patient describing what that tiredness is and how that affects the quality of life, but also then having it tied to an actual diagnosis and understanding that, I think it is a totally different situation being a patient than actually nursing that patient.
Having a liver transplant for my situation, I still have PBC, primary biliary cholangitis, which is a rare autoimmune disease that affects the small bile ducts of the liver, that can lead to cirrhosis. It’s rare in this population group of PBC patients. There are several stages. I unfortunately was part of the less than 30% of patients that ended up with end-stage liver disease. Having said that, it does not take away my PBC. I still live with PBC. It just says that I now have a new liver that my body will live with and eventually may, could develop cirrhosis again. My autoimmune could attack this new liver.
However, I’m now 54, so by the time it attacks my liver again, if it does at all, I’ll be that much older or not here. It doesn’t preclude that I need to be taking care of myself, watching what I eat, my quality of life. I have a lot of medications to maintain the quality of my liver and to prevent that cirrhosis from happening. Whereas I probably lived with the disease without knowing, because that’s a lot of patients do. By the time you get diagnosed, it’s because something is off. Typically, your liver enzymes, but that’s something we don’t test daily. The fact is, I still have PBC as that autoimmune liver disease, which may or may not complicate other autoimmune disorders that are evolving in my body, which pulmonary hypertension being that example.
Having the lived experience of several autoimmune diseases, and a lot of them sort of rare, it’s really helped me to be a better and broader healthcare practitioner. I always understood that we’re complex human beings, that factors influence the way we are and how we take care of ourselves. But the added bonus or blessing out of my health issues in the last three years is that I’m now better positioned to speak forward for patients, to bring their voices, to amplify their voices, to listen to what the community of these diseases, those that are living with these autoimmune diseases, in particular, the rare ones, where to give voice, whether it’s advocating for better treatment options, like in my PBC situation, being in situations with industry and pharmaceuticals and impacting on the kinds of science and treatments that are being thought about.
It’s actually a really interesting time where a lot of pharmaceutical companies and physicians are really trying to hear and receive the end user or the patient perspective into their treatment. I’m fortunate enough to find myself in that healthcare system or the credibility of being a nurse. And having that lived experience, the doubling up effect, fortunate, unfortunate, has made me that much more confident. I feel like there’s a calling in me to do this. That’s why when I was diagnosed with pulmonary hypertension, I thought, “Well, I’ve done this in the liver world and the living organ donation world, why not?” It’s the same sort of mechanisms, just a different disease.
My name is Maria Morais, and I’m aware that I’m rare.
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