Madison Brenton - Since 2016
First off, my name is Madison. As of March 2017, I am 23 years old with two beautiful children, Adilene who is almost 5 and Brenton who is 2. My support system is incredible, starting with my amazing parents who have fought with me every step of the way, to my best friends who at a moments notice fly out when they know I need them, to my awesome community who all volunteer their time and talents to assist with whatever my family needs.
I was diagnosed with PAH June 1, 2016. My PH is consequential due to my PVOD (pulmonary veno occlusive disease) which is an extremely rare disease. About an estimated 1-2 people per 10 million have this worldwide. It is guessed that my onset happened in the last trimester of my son’s pregnancy (June 2014) because I was passing out quite a bit and had a difficulty time breathing. However, when carrying a 9 lb 3 oz baby you don't really think anything of it. After having my son I noticed I was extremely short of breath upon exertion. I was trying to get back to “normal” after baby. When I expressed my concern to my family practice naval doctors they told me I just needed to get back in shape and that it was in my head. Months went by and I continued to keep pushing myself but my shortness of breath continued to worsen.
Again, I went to my doctors at our Naval Hospital (for insurance purposes we were not allowed to go anywhere else without referral) and again, told I needed to just work harder. This wasn't good enough for me. So, I pushed. They sent me to an allergy and asthma specialist. Of course, that came back clear. Finally, with a lot of pushing, I got referred out to an off base pulmonologist and he insisted on a thoracotomy biopsy. That was completed on July 29, 2015. In one day I was diagnosed with blood cancer, LAMs disease, to “we don't know it’s inconclusive”, to well since we don’t know what it is, must be sarcoidosis. For about a year I was treated for sarcoidosis. Placed on extreme high doses of Prednisone and chemotherapy treatments that were brutal on my body.
In November of 2015 I went to visit home. While there, I decided a trip to Cleveland Clinic’s sarcoid center would be smart. I went in for my first appointment and only by reviewing my file the doctor said I did not have sarcoidosis. I don't fit any of the criteria for sarcoidosis. However, he was also puzzled by my case. I went back to California without diagnosis and my pulmonologist there withdrew care and treatments. Not until I visited home again in March 2016 did I have any further medical care. I was in limbo. Due to furthering complications I had to be admitted to the Cleveland Clinic where they continued to work hard and try to diagnose me.
After presenting me to conferences and holding large meetings in the clinic with other specialties they still couldn’t quite figure out what was wrong with me. All blood work always came back clean, scans showed them scarring and abnormalities in the lungs but nothing specific, biopsies came back clean or inconclusive. Not until another admission in mid April 2016 did PH finally present itself. The right side of my heart was now severely enlarged. From there, my PH specialist Dr. Joseph Parambil searched deeper into my case to explain why my PH came on so aggressively and so suddenly . On June 1, 2016 Dr. Parambil diagnosed me. After reviewing my biopsy taken back in 2015, he discovered by looking at the vessels rather than looking at the lung that I had an extremely rare disease called PVOD. PVOD, essentially, is progressive blockage of the small veins in the lungs.
I have been treated now for about a year but, unfortunately, PAH medications work against PVOD and make it worse. I am now listed at the Cleveland Clinic for double lung transplant. This will give me quality of life back. As of now, I barely get out of bed and when I do, it’s to the couch or bathroom. Anything more, stresses my heart out or, occasionally, causes me to pass out. The doctors at the clinic have said we have exhausted all medicinal treatment. They’ve given me about 6 months to 2 years WITHOUT transplant. But, more likely, I'm lucky if I make a year. I am so blessed to be so close to these incredible physicians at the Cleveland Clinic. Even though this journey has been so hard and very trying they still manage to make me hopeful for a better life. My family and I truly feel like they have held our hands through every “up and down.” Their care extends past the medicine, which is refreshing. They are always very concerned about how I am doing in my personal life, how my kids are doing and managing, and how my parents are doing as well. I’ve got a long road ahead of me yet, but, I’m ready!