Sunday, April 27, 2014

Days +239 to +248: Headed in the Right Direction

We are happy to report that this past week was a good one! The doctors repeated the antibody tests last week. The good news is that Ezra is no longer testing positive for red blood cell antibodies. The bad news is that he still has antibodies against his own platelets. BUT, even though he still has platelet antibodies, his platelets increased this week and he has not needed IVIG. Tomorrow will be four weeks from his last high dose IVIG treatment. This is a huge improvement considering that last month he couldn't make it a few days without needing treatment. 

When we asked the doctor if the red blood cell antibodies can return, she said "not usually," but we could sense the hesitation in her voice, as things with Ezra often don't follow the "usual" path. She is certainly proceeding with caution on the steroid wean and going very slowly. Ezra remains on a significant steroid dose and we remain very concerned about the side effects. 

The other good news is that we are doing a trial run of an oral medication that can replace the medication that Ezra was receiving by IV three times a week at the hospital. This means that if all goes well we get to move down to clinic visits twice a week. Ezra is really excited about this change - we just hope the oral med works out. When we were explaining that for this week, Ezra would not need to go to the bed area at the hospital unless his bloodwork shows he needs medicine, Mr. Comedian responded with "ah, back to the good 'ole days of waiting in the waiting room." This kid needs to stop spending so much time with grown-ups in so many ways! 

It was exactly two months ago that this crisis began. At the time, we felt like we were on the ledge, with a child in serious danger and at risk of losing his transplant again. We are grateful for his doctors who thought outside the box to get Ezra to a better place in a way that was best for him. While we know he is by no means in the clear yet, we are grateful for where he is at this moment and that we still have our happy, energetic boy keeping us on our toes. 

Thursday, April 17, 2014

Days +228 to +238: A Better Week

Overall, this past week has been a better one for Ezra. Instead of dropping again at the one week post-IVIG mark, his platelets actually increased. He is now past the two week mark from the last dose of IVIG. Ezra's hemoglobin has also remained in the normal range. Despite these good numbers, several indicators of cell destruction are showing that he is still destroying his own cells. As long as these indicators show that destruction is still occurring, we are not in the clear. There was, however, a small amount of improvement with these numbers - enough that the doctor allowed another small steroid dose decrease last week. 

The most important point is that Ezra's numbers have improved (at least for now) even with decreasing the steroid dose. They have also been tinkering with the dosing of Ezra's immunosuppressant because his blood levels had been showing that it was undetectable. The last test showed that the level of the drug in his blood is at the right point, so we are hopeful that this is also helping his counts to improve. 

The take away from this past week is that the situation is improving, but we know that we can show up any day for clinic and find that the destruction has ramped up. Things remain day to day. 

Ezra has been handling the three times a week hospital visits for meds and bloodwork better than we could ever expect. He regularly asks for confirmation that "we are home more than we are at the hospital," and for now, as long as the answer is yes, he seems satisfied. The days are long and hard, but he remains happy and energetic. We sometimes feel bad for the other patients in neighboring beds because Ezra gets so excited (and loud!) while he's playing. He keeps us all busy with the creative projects that he comes up with - never ceases to amaze with what we can accomplish in a tiny hospital cubicle. Our focus as always remains on making sure he continues to develop and learn despite his restricted life - no small feat, especially with this latest turn of events.

We gladly accept a better week while we remain always on guard for the possibility that tomorrow everything may change.

Sunday, April 6, 2014

Days +218 to +227: Birthday Do-Over and The Latest

Last Sunday, we had the joy of holding a "do over" party to celebrate Ezra's 5th birthday. We had a small grown ups only party with a visit from Mad Science, as requested by the birthday boy. Ezra was bursting with excitement waiting for his party to begin, making all of us smile at his happiness. He had a blast with the experiments and projects, and especially the insane model rocket launches from our backyard (still waiting for a visit from the cops!). Ezra was lucky to have an amazing moon cake made with love once again by Cake & Co. in Teaneck, NJ through Icing Smiles. Ezra was surrounded by love as we all sang Happy Birthday, all of us wishing that this will be the year Ezra is finally granted good health (except for the birthday boy who was probably wishing to launch more rockets!). 

For a few precious hours, we got to forget our worries. Everything came crashing back to reality on Monday when Ezra's platelets dropped again. He is responding to the high dose IVIG, but the steroids do not seem to be helping the platelets. This is ok, as long as he continues to respond to the IVIG. The hope is that he will be able to go longer between doses of IVIG. For the past six weeks, he has been able to last only one week between doses. Tomorrow is the one week mark again, so we will see if his platelets hold and he is able to go longer. 

Ezra's hemoglobin has been good, so we have been able to do another small decrease in the steroid dose. The goal is to get him as low as possible on the steroids and let the immunosuppressant take over. This process is going to take months and is only possible if his counts remain stable. Now that we are out of emergency mode, we are focused on the reality of the damage that the high dose steroids are doing to his body. The list of side effects is long and frightening, especially the increased risk of infection and particularly invasive fungal infections. For the side effects that are possible to mitigate - bone deterioration and muscle weakness - we are determined to do what we can to counteract the steroids with increased PT time and making sure Ezra gets plenty of exercise. For the other side effects, all we can do is hope that he can get to a lower dose of steroids as fast as possible. 

On to another week of challenges, unknowns, and rocket launches.