Seeking a Better Understanding of My Rare Disease

I am an advocate for my rare disease, but I still can’t describe it.

Several weeks ago, I went to the dentist with my husband. It was a hot and humid day, as it always is here in Texas. I was carrying “Cooper,” the name I gave to my portable oxygen machine, and I could tell the dentist wanted to ask me about it. I made a joke to sate his curiosity.

“This old thing? It’s one of my many appendages. I am known as the Modern Day Mutant,” I told him.

That put a smile on his face. He said, “May I ask why you use oxygen? Is it due to COVID-19 or another illness?”

I told him I have a rare disease known as lymphocytic interstitial pneumonia (LIP), which causes pulmonary fibrosis.

“What is that exactly?” he asked.

I realized I still didn’t know how to answer this question.

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I wrote about LIP in my very first column. At the time, I thought I had a good understanding of it. But I’ve been asked multiple times about my disease since then, and all I can muster is its name. So, I’m right back where I started.

I have joined several groups for people with pulmonary fibrosis in the hopes of finding others who have been diagnosed specifically with LIP. Where are they, I wonder, and what are their symptoms? I guess I’m looking for validation that I do indeed suffer from this condition.

A disease is considered rare because it affects so few people. I never paid much attention to rare diseases, because I didn’t know anyone who had one. I certainly never thought it would happen to me. I figured rare diseases are often genetic, and as far as I knew, no one in my family had one.

I have researched LIP, but I am still not clear on its progression. How did I get it? Is it autoimmune? Is it triggered by environmental factors? How does it relate to pulmonary fibrosis? Why do I have a constant cough? Why do my oxygen levels drop so suddenly during any activity?

Authors of a chapter published in “Reference Module in Biomedical Sciences” noted that, “Idiopathic LIP is a rare form of idiopathic interstitial pneumonia (IIP) that is categorized as part of a spectrum of pulmonary lymphoproliferative disorders.”

“Murray and Nadel’s Textbook of Respiratory Medicine” adds that, “Secondary causes [of LIP] include rheumatoid arthritis, Sjögren syndrome, lupus, Hashimoto thyroiditis, myasthenia gravis, common variable immunodeficiency, allogeneic bone marrow transplantation, and viral infections. Viruses implicated in LIP include human immunodeficiency virus 1 (HIV-1), human T-cell lymphotrophic virus 1 (HTLV-1), and Epstein-Barr virus (EBV) (especially in children).”

Secondary causes? That’s new to me.

Most of the research I’ve found about LIP cites symptoms of cough, fever, and dyspnea, all of which I’ve experienced. But don’t most people with interstitial lung disease experience these symptoms? How is my disease any different?

I hear other rare disease patients tell their stories, and they seem so confident in their understanding of their condition. I want that same confidence.

I’m not looking for a miracle cure, although that would be nice. I simply don’t want to be blindsided by this disease. Then again, that is how a rare disease works — it is never constant or predictable. Most likely, I will never be prepared.

I still have several other specialists to visit, including a rheumatologist, gastroenterologist, and cardiologist. My primary doctor and I are leaving no stones unturned. Given the potential secondary causes, it is best to take precautions.

I am willing to do all the necessary testing. But it would be nice to receive more information from my doctors about LIP.

I have been on oxygen therapy for two years and am responding well to the medications Ofev (nintedanib) and Cellcept (mycophenolate mofetil). My last CT scan revealed that my pulmonary fibrosis is progressing more slowly than expected.

I am still hoping to find someone else who has been diagnosed with this illness. I would love to compare notes.

For now, I’m off to my second visit with a new pulmonologist. I hope they can provide some answers. Either way, I am still up for the fight. I’m not willing to give in to this enemy.

The journey continues.

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Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.