In 1986, I gave birth to my 30-week old son. It was determined, he was born with Neonatal Lupus with Complete Heart Block. Sadly, he died at 3 weeks old from V-fib. I was left with a broken heart and a diagnosis of Sjögren’s Syndrome and Systemic Lupus. I was not sure what my future would look like and I was devastated. Later, I did have two healthy sons, 1988 & 1990. (I was placed on Prednisone for both pregnancies). Afterwards, I started noticing, when I sat too long, or stood too long, that I would get severe leg pains with a bright red rash. Diagnosis? Small Vessel Vasculitis. It was horrible. The only thing I could do when the rash/pain came was lay down. It took a few days for the pain to subside and a few more days for the rash to disappear.
I suffered from small vessel vasculitis for years. No medication that I was put on worked, so I can tell you it impacted my life weekly with no relief. Starting in 1996, I started noticing chest pains, and esophageal spasms. I ended up in the ER several times, never a cardiac diagnosis. In fact, they did not know what the problem was. I had chest pain, but never related to cardiac issues, cardiac catheter was negative. In 2008, my jaw suddenly swelled up and via CT scan, I was diagnosed with Parotitis, an inflammation of one or both parotid glands. However, in my report, I happened to notice comments like ‘blebs and bullae in the apex and throughout both lungs’, which led me to a local pulmonologist.
This pulmonologist minimized the finding, and made me think it was congenital and no big deal. By 2013, I was sure something was wrong. I was experiencing some shortness of breath on exertion, and thought I should see another pulmonologist at Beaumont Health. After several appointments and tests, my Beaumont pulmonologist, sent me to the University of Michigan. At U of M, I met with Dr. Flaherty, another pulmonologist. I was then referred to Dr. Singer in rheumatology. I was placed on Imuran and was watched. By early 2015, I ended up with fevers, weight loss, and night sweats. Imuran was discontinued and I was referred to Dr. Phillip in hematology/oncology.
June 2015, I had a video-assisted thoracoscopic (VATS) pulmonary biopsy, and was diagnosed with Pulmonary Non-Hodgkin’s Lymphoma: subtype: Pulmonary Malt Lymphoma along with Amyloidosis with Progressive Cystic Lung disease – Stage 4 (both lungs). In October 2015, I was started on Rituxan (immunotherapy) infusions every 6 months. It was great for the vasculitis, but did nothing for the Malt lymphoma or the amyloidosis, which I later learned was creating the cystic lung disease. Chemotherapy was started in April 2018 and had 6 treatments until Aug 1, 2018.
Now I continue to get CT scans, labs, pulmonary function tests and I see the doctors every 6 months. I am now considered a ‘Wait and Watch’, but know that I will eventually end up with either more chemotherapy and/or a pill that I will have to take forever, according to Dr. Phillip. It is so frustrating. This has been a lifelong issue, that seems is never ending. I was told that the Malt would probably be there for years, but that the Amyloidosis was responsible for the progressive cystic lung disease. My life is a series of appointments and testing. It is all about ‘When and What’ instead of ‘Wait and Watch’. It’s so frustrating. To look at me, you would never know that I suffer from an illness. It’s a hidden illness and it is not only rare, but my case is considered ultra-rare.