Fibrosing mediastinitis

Posted by tiss @tiss, Wed, Apr 17 12:51pm

Has anyone heard of this terrible lung disorder? My daughter’s life partner died from this January 11, 2019. He was only 35. It’s so devastating.

Hi @tiss that must be devasting for your daughter, and for you to watch your daughter be in so much pain from losing her life partner.

I wanted to share these few links so that you and others can learn more about this rare lung disorder.

NIH on Fibrosing Mediastinitis: https://rarediseases.info.nih.gov/diseases/8337/fibrosing-mediastinitis

Fibrosing mediastinitis: when to suspect and how to evaluate?: https://www.birpublications.org/doi/full/10.1259/bjrcr.20150274

Back to you @tiss, how is your daughter coping? Do you feel comfortable sharing your experience supporting your daughter and her life partner after he was diagnosed with this disease?

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Matt was diagnosed with this disorder when he was 22. Apparently he had had a histoplasmosis infection in years prior without ever knowing. He had a tremendous immune response in his lungs which they found out after a bicycle accident which resulted in a broken collar bone. The xrays showed lesions that were at first thought to be lung cancer. After seeing several doctors, it was determined he had FM. He didn't have symptoms at the time but 2 years later , at age 24, did have to have a stent put in the vena cava due to the scar tissue occluding it. Matt always had troubles with a cough, allergies, upper respiratory problems. All the pulmonologists, allergists told him this coughing was not due to his FM but asthma and allergies. To be fair, I doubt that any of his doctors had ever seen FM. It is very rare and extremely rare to have the most serious form, which we know now matt did have. I don't know why he was never sent to a specialist. January 2nd if this year, Matt started coughing up blood– copious amounts. He was rushed to the ER and an embolisation was attempted a few days later and put into a medically induced coma. But due to the extreme scarring in his lungs, the bleeding stopped for only a few hours. 5 days later, they attempted to remove the right lung, which they felt was where the bleeding was coming from. However, after only an hour in surgery, the thoracic surgeon told us that it was not possible to remove either lung. He said he had never seen such extensive scar tissue in lungs and it was rock hard and basically the lungs were rock hard. It was also determined that his Vera cava was again closed however due to the scar tissue and blood vessels that had grown in and around the scar tissue, it could not have a stent put back in to open it. After 10 days he had lost so much blood through his bleeding lungs that they gave him blood transfusions which caused everything to start shutting down– kidneys, blood pressure, heart, treatment . After 10 days of hell, our boy died of catastrophic organ failure. He had just turned 35 a week before. We are all heartbroken and his parents are beyond broken. Matt was their only child. My daughter is 31 and her world has turned upside down. She's in grief counseling and has her family and many, many friends. It is going to take a long, long time to work through this. Thanks for listening.

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@tiss

Matt was diagnosed with this disorder when he was 22. Apparently he had had a histoplasmosis infection in years prior without ever knowing. He had a tremendous immune response in his lungs which they found out after a bicycle accident which resulted in a broken collar bone. The xrays showed lesions that were at first thought to be lung cancer. After seeing several doctors, it was determined he had FM. He didn't have symptoms at the time but 2 years later , at age 24, did have to have a stent put in the vena cava due to the scar tissue occluding it. Matt always had troubles with a cough, allergies, upper respiratory problems. All the pulmonologists, allergists told him this coughing was not due to his FM but asthma and allergies. To be fair, I doubt that any of his doctors had ever seen FM. It is very rare and extremely rare to have the most serious form, which we know now matt did have. I don't know why he was never sent to a specialist. January 2nd if this year, Matt started coughing up blood– copious amounts. He was rushed to the ER and an embolisation was attempted a few days later and put into a medically induced coma. But due to the extreme scarring in his lungs, the bleeding stopped for only a few hours. 5 days later, they attempted to remove the right lung, which they felt was where the bleeding was coming from. However, after only an hour in surgery, the thoracic surgeon told us that it was not possible to remove either lung. He said he had never seen such extensive scar tissue in lungs and it was rock hard and basically the lungs were rock hard. It was also determined that his Vera cava was again closed however due to the scar tissue and blood vessels that had grown in and around the scar tissue, it could not have a stent put back in to open it. After 10 days he had lost so much blood through his bleeding lungs that they gave him blood transfusions which caused everything to start shutting down– kidneys, blood pressure, heart, treatment . After 10 days of hell, our boy died of catastrophic organ failure. He had just turned 35 a week before. We are all heartbroken and his parents are beyond broken. Matt was their only child. My daughter is 31 and her world has turned upside down. She's in grief counseling and has her family and many, many friends. It is going to take a long, long time to work through this. Thanks for listening.

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@tiss Am so sorry for your loss and for all Matt and all of you had to endure. Please accept my deepest sympathies fro this. Grief does get better over time, but it never really goes away. I"m glad there is grief counseling for your daughter and her family.

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@alamogal635

@tiss Am so sorry for your loss and for all Matt and all of you had to endure. Please accept my deepest sympathies fro this. Grief does get better over time, but it never really goes away. I"m glad there is grief counseling for your daughter and her family.

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Thank you for your kind words.

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Thank you for sharing your story @tiss. I wanted to thank and second the response from @alamogal635

I also wanted to share a link to our Loss & Grief group so that you can connect with others who have experienced similar losses.
https://connect.mayoclinic.org/group/loss-grief/
Please keep us updated with how you and your daughter are doing.

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@ethanmcconkey

Thank you for sharing your story @tiss. I wanted to thank and second the response from @alamogal635

I also wanted to share a link to our Loss & Grief group so that you can connect with others who have experienced similar losses.
https://connect.mayoclinic.org/group/loss-grief/
Please keep us updated with how you and your daughter are doing.

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Ethan, can you copy this story onto the grief site? I don't know how to do that. And thank you for your kind words.

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Does anyone have Fibrosing Mediastinitis?

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@msvicki

Does anyone have Fibrosing Mediastinitis?

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Hi @msvicki, welcome to Mayo Clinic Connect. I moved your message to this existing discussion about fibrosing mediastinitis in the Lung Health group https://connect.mayoclinic.org/group/lung-conditions/ Here you will meet @tiss.

Vicki, do you have fibrosing mediastinitis or are you taking care of someone with FM?

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@msvicki

Does anyone have Fibrosing Mediastinitis?

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I do- was officially diagnosed in April of this year.

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Hi @notallwhowanderarelost and welcome to Connect. Thank you for sharing your diagnosis, I'm sure it something that @msvicki would like to hear. How have you been doing? How was it diagnosed?

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@ethanmcconkey

Hi @notallwhowanderarelost and welcome to Connect. Thank you for sharing your diagnosis, I'm sure it something that @msvicki would like to hear. How have you been doing? How was it diagnosed?

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I’ve been doing okay, a lot better than I was prior to coming to Mayo.

I was diagnosed in April. Last October I had to have an emergency pericardial window due to excess fluid around my heart. I developed blood clots in my left leg that went to my lungs- while in the hospital they discovered the mass in my chest. It wasn’t until late March that we ended up going to Mayo, as we thought the mass wasn’t anything to be concerned about. (We had done biopsies and another surgery to try and remove some of it, and everything came back negative) But in February/March, my breathing started getting bad again. We finally discovered that the mass was growing around my airways, so we came to Mayo.

They put a stent in my left airway to open it up since it was almost completely closed. The stent was in for about a week because it got filled with mucus/tissue from my body rejecting it, and my left lung had partially collapsed. They took the stent out, and I did 2 IV treatments of rituximab, as well as I’ve been on prednisone.

Overall I’m doing a lot better than I was. My breathing is better. I think my lungs were functioning at 63% when I came to Mayo the first time, and this last visit I was up to 74%. Certainly not great, but a whole lot better than where I was.

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@notallwhowanderarelost

I’ve been doing okay, a lot better than I was prior to coming to Mayo.

I was diagnosed in April. Last October I had to have an emergency pericardial window due to excess fluid around my heart. I developed blood clots in my left leg that went to my lungs- while in the hospital they discovered the mass in my chest. It wasn’t until late March that we ended up going to Mayo, as we thought the mass wasn’t anything to be concerned about. (We had done biopsies and another surgery to try and remove some of it, and everything came back negative) But in February/March, my breathing started getting bad again. We finally discovered that the mass was growing around my airways, so we came to Mayo.

They put a stent in my left airway to open it up since it was almost completely closed. The stent was in for about a week because it got filled with mucus/tissue from my body rejecting it, and my left lung had partially collapsed. They took the stent out, and I did 2 IV treatments of rituximab, as well as I’ve been on prednisone.

Overall I’m doing a lot better than I was. My breathing is better. I think my lungs were functioning at 63% when I came to Mayo the first time, and this last visit I was up to 74%. Certainly not great, but a whole lot better than where I was.

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I'm glad you are doing better. There is a closed Facebook group on FM and there is good information. Did you see Dr Piekart at Mayo? That's who we were hoping Matt might be able to see. I hope the infusions work for you.

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@msvicki

Does anyone have Fibrosing Mediastinitis?

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Yes I have FM which now my pulmonary vein have been blocked due to it only one remains open

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@loveme88

Yes I have FM which now my pulmonary vein have been blocked due to it only one remains open

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@loveme88
Please meet @ notallwhowanderarelost and @msvicki. Welcome to Mayo Clinic Connect. Good morning, I'm glad that you found us. Are you terrible uncomfortable? How long ago were you diagnosed? Are there any treatments planned to open one of the pulmonary veins?

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i have pain in my chest often and trouble to breathe at times. it's been about 2-3 years I had a stent put in before 3 months later it had close they tried to reopen it ut that didn't work now they say its nothing they can do about It currently have just one pulmonary vein open

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