We came in To the ER on July 28th with what we thought was a fever and potential rejection. Since then, we’ve chased more questions and found each one met with more confusion. John and I know there are so many people who love our family and want an update, but this has been a very painful and confusing time, with little to share that felt concrete. But here’s our best attempt to share Hope’s recent challenges with friends and family.
She spent one week on a mechanical heart pump because her heart was in failure for what was assumed to be severe acute rejection in her heart. She came off the mechanical support and, instead of getting better, seemed to only plunge deeper into a confusing pool of troubling symptoms that didn’t add up to a diagnosis, the most concerning among them was kidney failure.
Transplant decided to reevaluate her for a new heart transplant, knowing we’d need to run a parallel path to figure out what was going on in the kidneys. We asked for second opinions. We were told it was unlikely that Hope would survive the next two weeks without mechanical support again, which they were unclear about offering based on the uncertainty with the kidneys. The message was subtle, but clear: these may be the last few weeks you have with her.
Finally, a piece of evidence emerged that brought serious doubt as to whether rejection was the wrong assumption and something much more dangerous was running amok. She has all the clinical signs of a condition called thrombotic Microangiopathy or TMA. If you want a laugh and to learn what this is, google CCHMC’s video with Como the dog that explains this condition. We’ve since learned Hope has a genetic mutation that makes her more prone to a specific type of TMA called atypical-HUS, triggered by the combination of her immunosuppressant therapy and/or the presence of paraflu, which she had in July.
The only definitive test for a-HUS is to biopsy the kidneys, but that was deemed to great a risk and we began treating as soon as TMA was suspected, since August 23rd. The bad news is that she only continues to get worse. Her kidney function completely deteriorated and she is on continual dialysis, pulling off 2.5 liters of fluid in the first week because of how dysfunctional her kidneys had become. Her liver plunged into failure and she turned jaundice. She couldn’t breathe well or control her fluid balance when changes were made to her dialysis plan. The team wanted to get her dialysis line switched and we thought it would be the prefect time to get biopsies of the liver and kidney and get some damn answers about what exactly was attacking these organs, to what extent, and whether they have been damaged beyond recovery.
The icing on the cake was the discovery of a clot in her SVC right near the atrium to her heart. Her head swelled up like a bobble head doll. We had to treat it, but at a large risk for internal bleeding and hemorrhagic stroke – so any thought of surgical procedures/biopsies was out the window. We started treatment and it wasn’t long before internal bleeding was suspected. This eventually led us to a CT scan that revealed three things. First, she had suffered three small hemorrhagic bleeds in her brain. Second, the clot had broken up enough to restore somewhat normal flow from her brain to her heart. Third, the suspected bleeding was actually a mass in her colon and no one is going to do an abdominal procedure on her with an unknown infection or source of inflammation going on. All our answers were put on hold, while we tried to convince GI to do a scope on her to get a clear idea of what it is.
So that catches everyone up to where we are. She went down to the OR for her scope and bone marrow biopsy this morning and with any luck we will be able to name one of the many culprits attacking her body by Friday when results are back. Once we’ve cleared that, we can once again turn our attention to the kidney and liver to help determine her long-term prognosis.
To be honest, Hope’s medical team is not really sure if she will be able to fully recover from this setback and ever return home again. Our hearts are broken and we have been grieving for the last few weeks as a family. All we can do at this point is pray for Hope’s path to be made clear to us and her medical team.
We thought we’d share one of our favorite videos of Hope as a parting gift for this rather dreary update. It captures her infectious laugh and determined spirit so perfectly. We promise to keep updates coming!
Love – John, Hope & Tess Sansbury