Monday, July 19, 2010

In-patient, Doernbecher Children’s Hospital.

Ethan, Sarah and Grandma Pat and Grandpa Bob came in today for Ethan’s enzyme infusion.  To which they added an additional 4 hour IV-IG (immune system booster) infusion.

During Sarah’s meeting with Dr. Nemecek today, they discussed two concerns about Ethan’s central line (a double lumen Hickman).  The first was that a loop of suture thread that had been placed on the line just outside the skin was now a couple of centimeters down the line.  This indicates that the line’s been pulled in the 8 or so months that he’s had it.  The second is that there was a small gap at the end of the line before the Y connector that we were worried might mean the two lumens at the end of the line might eventually break off for the line itself.

They sent Ethan for chest x-rays in-between infusions.  Then after the second infusion, they attempted to remove the Y connector and the lumens and glue on a replacement.  Evidently that didn’t go to well and/or the chest x-ray showed that the internal end of the hickman had in-fact moved.   They did not reattach the Y connector and lumens, but clamped the line and secured it.

Tomorrow, Ethan will have surgery to remove the Hickman, and replace it with a PICC line.  The PICC is similar in function to the Hickman, but will come out of his arm instead of his chest and only has one port, instead of two.  

No one has said as much, but I’m guessing that Ethan will have another surgery at some point within the next couple of months to place what will be his third Hickman.


The other news that we got today, was about Ethan’s July chimerism tests.  I haven’t seen the actual results, but evidently the numbers had dropped from 30% and 28% to 26% and 20%.  

Those numbers aren’t good.    While we were a decline from the June tests, it’s a faster decline than we’ve seen in recent months.  It was a shockingly unpleasant surprise.

No one has officially called this transplant a failure, but we’re all thinking it.  I’d say it’s probably more than 90% likely that this transplant will fail and Ethan will need a second transplant.

We also saw a note today indicating that Dr. Nemecek may be thinking of tapering Ethan off of the immunosuppresents.  In Sarah’s meeting with Dr. Nemecek today, Sarah asked about the current status and long term impact of Ethan’s current med regime.  One of the things that came out of that conversation is that Dr. Nemecek will taper Ethan off of the immunosuppresents a month or more before transplant in preparation for the chemo regime.  So, if the brief note that we saw, is in fact an indication that Dr. Nemecek is planning to taper his meds, she may be thinking it’s time to call this.  I think we’ll know more tomorrow.

If Ethan needs to go through a second transplant, the risks remain largely the same as the first transplant--30% risk of mortality, significant risks of graft versus host disease, et al.
In addition, if he needs a second transplant, it’s more likely that they will use a full toxicity chemo regime as opposed to the reduced toxcity regime they used this time.

On the plus side, Ethan has recovered well from the first transplant.  He’s strong.  His liver function is in good shape.  He’s in good shape for a second transplant.

 But, I suppose I'm getting a head of myself. We're not there yet. We're starting to make preparations in that direction, but we'll keep trying to keep this transplant going as long as we can.

Surgeries for MPS kids are always risky. Please keep Ethan in your thoughts and prayers tomorrow. I'll try to post an update tomorrow evening after we see how the day goes.

Thanks for being there for us.