Jim's Journal

It's been almost a year since our last CT scan to monitor her abdominal aorta. She is still being treated for her autoimmune disease Giant Cell Aortitis. Low dose prednisone. We have decided to not try Biologics like Rinvoq as we prefer the devil we know to experimenting with another pharmaceutical with questionable efficacy.  We will have the CT scan in Oklahoma City with her new vascular surgeon consult via zoom. Dr Oderich is at Baylor Medical Cardiovascular Center in Houston. 

She was diagnosed with anemia this fall after being chronically fatigued short of breath. They believe it is caused by malabsorption due to her blood supply restrictions to her mid gut with her Celiac artery blocked by the EVAR stent placed last year. She has responded to iron supplements. However, my older cousin a retired Internal Medicine physician says we may need to explore Mechanical hemolytic anemia. It's caused by red blood cell shear and destruction by the valves themselves. It's a rare complication of mechanical valves. Yet another reason to reconsider mechanical valves. Debbie has had a difficult time with keeping her INR in range. 

Anemia causes inaccurate INR readings as well as interfering with the Warfarin dose, so her INR was extremely low until her anemia improved. Then when having a dental procedure and being given Amoxicillin as a prophylactic her INR shot up to dangerous level of 7.9. So she has had a difficult time scheduling a follow up procedure. The Corticosteroid has played havoc on her teeth.  

Dr Kapravinac at Cleveland Clinic thought she needed mechanical valves because she had porcine valves fail at 3 years and again at 5 years. He said her body doesn't appear to like them but in hindsight it could of been the autoimmune condition. Giant Cell Arteritis is a condition they don't fully understand. She has had a rough time with the chronic nature of her health problems and is really exhausted by the constant medical issues it just never seems to end. The doctors locally here just seem as baffled as I am by the inability to stabilize her INR and control her inflammation to keep any further damage to her aorta from causing failure of her aortic stents that would require intervention and the next EVAR procedure.   

Hopefully 2026 is a better year. 

Two years ago March 15th  Debbie underwent the first of many procedures to repair her diseased aorta. Twelve hour open arch repair called a frozen elephant trunk. They also replaced two tissue heart valves with mechanical. Cleveland Clinic in Cleveland Ohio the largest and highest rated cardio vascular surgery centers in the US. We have had a very tough journey three other vascular surgeries, a TEVAR extension in her descending aorta in July 2023 450 mm long extending the repair to her celiac artery. September 2024 another EVAR extension to the SMA artery to seal an endoleak. An Iliac artery repair, a complication of the EVAR procedure to fix a dissected artery. She had survived so much, but we just didn't think she'd make it through the next open procedure to take the repair below the kidney arteries. Cleveland Clinic advised that should be the next procedure when strong enough. In November, we reached out to a vascular surgeon in Houston, Texas at Baylor Medicine. We had a Zoom appointment in November that gave us hope and scheduled an in person visit in March. We had a CT scan to check her current status, and he told us the good news. Her stent grafts are holding up well with no leaks, he recommended we return in a year. Dr Oderich at Baylor Medicine is a vascular surgeon that has been a pioneer in the branched, sleeved endovascular stent graft procedure for noninvasive aneurysm repair. He practiced at Mayo Clinic for 20 years before moving to Texas. Not only was her current state good news, he assured us he could repair the next section endo vascularly.

One year post op of the TEVAR procedure and 18 mos prednisone to control her Giant Cell Aortitis, Debbie has been trying a couple different biologic medications to try to control inflammation and get her off of the corticosteroid therapy. Methotrexate after a few months only started her digestive problems that had been in remission without reducing her inflammation. She was put on Actemra monthly infusions that did knock out the inflammation but with a horrible side effect of hypertension. Not just elevated Blood pressure but isolated systolic hypertension with readings as high as 197 to 206 which sent her to the ER where she was scanned to reveal an endoleak of her thoracic aortic stent. 9 days in the hospital to control her spiking BP. The worst thing possible for an aortic aneurysm is high blood pressure. We travel to Cleveland next week for a follow-up and determine if the endoleak needs to be addressed.

Although this site is a Heart Valve Surgery site, My wife Debbie's journey began with a mitral valve failure in 2017. Sometimes in the shock and realization you're having to deal with life-threatening illness or major surgical procedures, it helps to put things in perspective. In some cases, the problem you are experiencing are minor in comparison to many others dealing with health problems. I have found it helpful to read other people's stories to realize we are not alone in this battle to return to a normal life some day.

We just returned from Cleveland for her post TEVAR procedure in July. It was to check on the state of her 450mm stent extending from her FET arch repair in March continuing through the diagram landing just above the celiac artery. We've been given an all clear and a 9mos return to monitor her AAA that is at this time dilated but stable, no growth noted.  Her heart function is good after the double valve replacement also in March. The last echo showed a moderate tricuspid regurgitation, but the most recent showed only mild. She is suffering from unilateral diaphragmatic paralysis as a complication from the major open procedure in March but it is improving. It is such a relief to have such good news after going through a year of medical procedures following a porcine valve failure in December of 22, and an incidental finding of aortic disease. We have spent 9 weeks in Cleveland since March where they determined her aortic and mitral valves both having failed and repaired with TAVR 2020, and TCMVR December 2022 the surgical team in Cleveland led by Marijan Koprivanac would perform the Frozen Elephant Trunk arch repair and replace her aortic and mitral valve with mechanical valves followed by TEVAR to address her aneurysmal aorta caused by a vasculitis called Giant Cell Aortitis. 

We live in the largest metropolitan area in our state and have access to cardiovascular specialists but sometime they are just not equipped to deal with complex medical conditions. We were fortunate to be able to travel to Cleveland Clinic for her care. 

Traveled to Cleveland June 26th for 3 month followup. Part of the FET B-Safer clinical trial she is a part of. The mitral and aortic valve 3 month echo showed the new mechanical valves are working well. She still has shortness of breath while laying flat and borderline pulmonary hypertension with moderate tricuspid regurgitation. Our cardiologist is very good there and exploring possible causes as we are being referred to pulmonology. The endovascular procedure is delayed a bit while we explore. Back to Cleveland in July. Fortunate to be there and able to travel for her care. She continues to defy the odds and doing well in spite of her complex problems. 

We have been home for two weeks. Surgery was March 15th at Cleveland Clinic. Mitral and aortic valve replacement with mechanical valves along with the ascending, arch, descending aorta using the frozen elephant trunk procedure to the descending thoracic aorta in preparation for an endovascular repair of the descending thoracic aneurysm. Refer to My Story for how we got here. Pathology of the diseased aorta, Giant Cell Aortitis was the diagnosis given. Seems to be a rare condition and Cleveland Clinic has experience with treating it, so we are certainly lucky to have found our way there. We go back in June for the next procedure. 

Debbie has showed remarkable strength and resilience through all her many procedures, but the most recent one was more than either of had bargained for. Entering into this latest procedure, her endurance and physical shape was so affected by the two valve in valve procedures leading up to this surgery having undergone the TAVR in January 2020 and Covid hitting with the March shut down made it impossible to fully rehabilitate post op. Then the transcatheter mitral valve in valve procedure in December 2022 left her with a stenotic valve by virtue of the size limitation of the failed tissue valve caused her shortness of breath with minimal exertion. The procedure itself she was able to bounce back from, but the concerns of the valve being too small and the aneurysm discovered on the diagnostic pre op CT gave us concerns to completing a cardiac rehab program post op. 

Lessons learned

1) seek not only 2nd opinions but 3rd opinions if possible 2) Not every facility is able to handle every situation but more importantly they aren't always willing to admit it. 3) Travel isn't easy, but sometimes necessary to achieve the best results. 4) Don't assume the doctors are infallible, and never be afraid to ask hard questions. 5) Everyone is important in life and death situations, but sometimes the details can fall through the cracks. If you feel a detail has been missed, speak up. 

Day 15 post op in step down unit. Debbie was cleared to start solid food after two weeks tube feeding. Hopefully released in a couple of days. What a roller coaster ride of emotion to be where we are now. We give a more detailed update as time permits. Possibly, on reflection, as we seemed to have turned a major corner in her recovery. 

The Cleveland Clinic is an impressive place. Only regrets is we didn't come sooner. Being an Oklahoma Sooner and living 1,050 miles away, it wasn't an easy trip. One day post op after AVR and MVR along with the thoracic, ascending, arch and descending aorta frozen elephant trunk procedure. 11-hour surgery Debbie is doing amazingly well. First she's tough as nails and second Dr. Marijan Koprivanac is an amazing surgeon along with the entire team. A long recovery ahead. If you want to know the story of how we got here it is in My story which is really Debbie's I just accompany her. Day one post op was tough, but she's a fighter. 

We are making plans for our journey to Cleveland. 16 hours by car from OKC. Stop over in Indy to see our kids. Not sure about return trip 14 days post op best case scenario. She's been through two open chest procedures, Debbie is tough but not sure which would be worse all day flying with potential layovers on the return trip or 16 hour drive in our Sequoia possible to lay down if needed. Any experienced heart surgery travelers have any advice?