A Little Bit About Me
My name is Tricia Edgell (Lewandowsky) Since you have found my website, we may have something in common. I am a Fibrosing Mediastinitis survivor.
I was first diagnosed in October of 2004 when an histoplasmosis infection, which resulted in a secondary condition called Superior vena cava (SVC) Syndrome, had caused Fibrosing Mediastinitis.
I first noticed something wasn't right while stretching after a run. My symptoms started with fullness in my neck, face and arms, as well as severe headaches. As time went on, visible collateral veins began to show on my chest. I was having pressure in my head that was not normal. This sensation persisted for several weeks. I can best describe the feeling as comparable to hanging upside down on the monkey bars as a child. My head would fill with blood. This fullness was occurring all the time without hanging upside down!
My diagnosis path took me from my primary care physician to a vascular specialist. The initial thoughts were that there may be a tumor on or around my SVC. A catheterization was scheduled in an attempt to place a stent to achieve normal blood flow in the SVC. The procedure was done by an Interventional radiologist and was unsuccessful in opening up my SVC, but did ultimately lead to my diagnosis of Fibrosing Mediastinitis. I was referred to an Infectious disease doctor who, through an extremely thorough history and physical exam, confirmed Histoplasmosis and Fibrosis Mediastinitis. I was referred to the University of Michigan Infectious Disease office. After a very honest discussion about my diagnosis and treatment options I was referred to Dr James Loyd at Vanderbilt Medical Center.
My symptoms first started in July 2004 and by October I had my diagnosis. After hearing many stories of misdiagnosis and lengthy time to diagnose, I am grateful that my diagnosis was fairly direct and quick.
My first visits to Dr Lloyd included several different diagnostic tests to determine the severity and the location of the fibrosing. Results showed SVC syndrome, blocked right pulmonary artery and partial loss of function of my right lung. The stenosis in my SVC was the most critical concern as it had narrowed to around 1mm and had formed blood clots due to the narrowing. My body had been developing collateral veins as my SVC was narrowing. These collateral veins maintained blood flow to my heart. It was estimate that the fibrosis grows approximately 1mm per year. My SVC was around 16 mm wide, so it is assumed that over the past 10 -15 years, my body was creating its path for the blood to flow. The symptoms became severe when the blood clots in the SVC formed.
The treatment plan was to stent my SVC but first we had to remove the clots. I choose to go home for 6 weeks on blood thinners to see if they would dissolve themselves. Unfortunately, that option did not work, and my procedure at Vanderbilt University Hospital, was schedule for December 17, 2004. After a five hour surgery my clots were removed and two stents were put into my SVC. My SVC was opened and blood flow was restored! Relief was immediate! The swelling was reduced and my headaches went away due to removing the pressure in my head. I had taken measurements of my neck before the procedure and after a few weeks the diameter of my neck had decreased about three inches.
Other symptoms became noticeable that were initially masked by the SVC syndrome. My Doctors at Vanderbilt decided to place a stent my right pulmonary artery which was approximately 50% narrowed. Two stents were placed via catheritization.
The first few years after my procedure I saw my infectious disease doctor every three months. I continued to take sporonox (itraconazole antifungal) for approximately two years. I take Coumadin to prevent clotting, and will take it for life. My symptoms were reduced dramatically by the stenting. The symptoms that still remain have become a way of life. Anytime my head is lower than my heart the feeling of fullness is quick to occur, I have occasional chest pain and fatigue. After several years, of having follow up CT scans with angio (contrast); the progression of the scar tissue seems to have stopped.
In summary, I'll leave you with a few final thoughts?
- Be your own advocate. Fight for a doctor to listen to you and if they don't, find one that will.
- Find others who have the disease for support. The disease is so rare and it's a huge comfort to talk to others who know what you are feeling and thinking.
- Never lose your faith and hope. Many of us are told the worst possible outcomes but this disease is so unknown that there is always hope.
- Listen to your body. My lifestyle had to change. Some of my favorite activities had to end. Find others to replace them. When you need to take it easy, listen.
- Perspective. A rare disease with many unknowns gives us all perspective. Live your life in the present, yesterday has happened. Learn from it, but don't spend your days worrying about what tomorrow will bring; before you know it you've lost the present and you'll never get that back.