Fibrosing Mediastinitis or FM (sometimes called mediastinal fibrosis) is a rare, progressive auto-immune disease that is often a complication of a fungal infection called histoplasmosis. Histoplasmosis is prevalent in areas of the Ohio and Mississippi Valleys. I grew up in the Ohio Valley, where I apparently contracted the disease. My histoplasmosis infection went untreated and was often contributed to a bronchitis or other respiratory infection. I was even mis-diagnosed with asthma and placed on steroid inhalers, which may have contributed to my fibrosing mediastinitis.
I had never heard of fibrosing mediastinitis before I was diagnosed with it. I have been a nurse for nearly 25 years and have never taken care of a patient with my disease. This is part of the frustration of living with a rare disease.
This is a photo of me, recovering my last angioplasty
Read the Whole Long Story if you want the details
Mediastinal fibrosis causes debilitation by interfering with or obstructing vital structures within the chest cavity, known as the mediastinum. These structures include, major veins and arteries, lung structures, airways, heart and esophagus. Many patients I've met have either pulmonary artery or superior vena cava blockage.
How FM Affects Me
My FM is localized around my left pulmonary artery, which is the major blood vessel to my left lung. Blood flows to the lung from the heart to become oxygenated through the pulmonary artery. Blockage causes critical oxygen deprivation and shunting with overload to the heart. It is not possible to remove the fibrotic mass surgically. Most patients are treated palliatively. I have two stents in my pulmonary artery which allow blood flow to my lung. I have had several angiograms and two angioplasties to keep these stents open. You can read more about that on my blog: Fibrosing Mediastinitis
Prognosis
I am living a full life today because of my physician, Abraham Rothman, a pediatric cardiologist, practicing in Las Vegas. I am his only FM patient. He is the one who restored my function after I was told by the Cleveland Clinic that there was no hope for me and that my life expectancy would probably not exceed forty. I have lived over 10 years with FM. Dr. Rothman can be contacted at the Children's Heart Center in Las Vegas, NV. He is willing to see patients with this disease.
If you have questions, comments, or are a person living with FM, please contact me at tjaskren@gmail.com
Radiographic journal article about FM. .pdf file. Good info.
Medical Article with detailed information about FM
Chest journal article on FM .pdf file
FM Foundation Merchandise Buy stuff: support FM research
Pulmonary artery stenosis and fibrosing mediastinitis
Facebook Group: People with Fibrosing Mediastinitis
A Rare Lung Disease Gina Stark's page
Stent Placement for treatment of pulmonary artery stenosis secondary to FM pdf file
Careplace:
A support network for people with FM
Click to join Fibrosing_Mediastinitis