Let’s pick up where we left off in our last update: We were admitted for a fever and infection last week, putting the viability of her procedure in question. We were able to come home late Friday and we spent the weekend resting and recovering from a lovely two-day stay at Hotel Children’s last week. We came home on a regimen of immune boosting shots, orders to keep her well protected from sources of infection and to check in with labs Monday to find out whether her immune system had rebounded enough to allow us to proceed with the planned procedure for her pulmonary vein obstruction on Thursday. Her labs were better in some ways and just strange in others, which meant they decided to wait to decide regarding proceeding until another set of labs on Wednesday were done. Wednesday was chaotic. Her labs were strong enough to proceed, but then an emergency situation bumped Hope to Case #2 and then a bed shortage put her procedure in jeopardy all together and they couldn’t even give us a green light until Thursday morning. Talk about just-in-time-delivery! Hope ended up going ahead on the schedule, but the challenging thing about being the second case is that you have no idea when you will start. They took her back at 11:15 a.m. to get the ball rolling. Thus began a waiting game worse than dial-up internet.
Around 5:00 p.m. Dr. Goldstein came out and delivered the outcome: despite valiant efforts, they were unable to reach the obstruction and thus were not able to clear the blockage in her left pulmonary vein. We peppered him with questions. Would we try again? Are there other avenues or ways to clear the blockage? The answers were obvious: No to both. There is no other way short of open heart surgery and we aren’t willing to risk that right now. And every time we try causes damage to her liver and it’s just not worth the risk. As we let the reality of no alternative sink in, our thoughts turned to the plan they’d shared with us if this procedure was unsuccessful: keeping a very close watch to assess whether it was progressing and, if so, how aggressively. Our questions sought to understand how they will gauge progression. Even though we are at the #2 Children’s hospital in the country, they still are not equipped with a crystal ball to tell us exactly what to expect in this situation. John and Goldstein had quite the humorous banter where John found numerous ways to ask the same basic question, “What’s going to happen next?”, and Goldstein found numerous ways to restate that he can’t predict the future.
In the face of so much uncertainty, there are only a few things we know. First, Hope will effectively live with one lung (and has been doing so for an undetermined period of time since we don’t know precisely when this blockage occurred). Second, you can live with only one lung, albeit with some significant physical limitations that she will find as she starts to exert herself more. Third, only time can reveal the full impact of this blockage.
We thought Friday was going to be a quiet, easy day of observation followed by discharge before/by noon, but like so many things in Hope’s path, we diverged from the expected. We knew that Transplant asked for some unusual labs earlier in the week and even more new labs were pulled while she was under on Thursday. Friday morning we learned that another team of specialists would be following Hope’s case: Immunology. We bounced back and forth between visits from Transplant and Immunology all day. The concern from Immunology was two-fold: our battles with neutropenia over the last few months as well as a low level of Immunoglobulin in Hope’s blood. Both of these may have contributed to the number of infections Hope has been troubled with lately. We have known about the neutropenia for some time, but the lab for the Immunoglobulin was one we hadn’t run before and, thus, was new information. After 3 -4 visits from Immunology throughout the day, they were exhausted from our relentless questions and we felt like our heads were spinning.
We aren’t sure exactly what’s going on yet, as we need to see some test results over time before parts of the picture can become clear. For now, they have added a monthly immune-boosting treatment (IVIG – a solution of highly purified immunoglobulin G, derived from large pools of human plasma that contain antibodies against a broad spectrum of bacterial and viral agents) and will be following her case closely until a diagnosis is more clear. Ironically, IVIG is sometimes referred to as ‘liquid gold’ because it is actually more costly than actual gold ($1981/oz IVIG vs. $1249/oz gold). They suspect that some of this could be the long-term impact of the treatments she received in conjunction with plasmapheresis to get her to transplant, but again, it will take time to confirm this, too.
Suffice it to say this weekend, like last, was spent recuperating from another stay in Hotel Children’s wonderful parent accommodations. If only they had a loyalty program for those “frequent fliers”. Ya know, the same perks as a normal hotel: faster check-in, more comfortable beds/rooms, nothing crazy! No luck…yet. John and I have found a good rhythm for our in-patient stays, with a nicely equipped bag of supplies at the ready and a schedule of me on nights and him on days. Sometimes I sleep there and it’s no problem – hell I slept there for 6 months and found a way to be relatively rested. Now, it seems hit or miss as to whether I leave and feel fine or leave and feel like my entire body is in pain. I think the image above adequately sums up how this most recent stay felt. A pinched nerve with a 3-day headache are enough to have me praying for Christmas break to come sooner rather than later. On the plus side, Hope’s spirits remain good and she has rebounded, slowly, but surely, from all the hubbub from last week, as shown in this video where she is showing off her ability to bounce right back to cuteness!