Last night, Trey had emergency surgery to remove his new intrathecal port that was infected. Due to overwhelming support and love and requests for updates, here is the low down (it’s long because you all want updates in different areas and I needed to cover my bases J).
Three weeks ago, on October 7, Trey had an intrathecal port-a-cath placed as part of the MPS II Intrathecal Clinical trial at the University of North Carolina. One and a half weeks ago, on Tuesday, October 18, Trey received his first dose of intrathecal Elaprase through the port.
Thinking back, the incision sites never looked great. Even when the stitches were removed under general anesthetic during his first dosing, looking at pictures we took of the sites before he went back for the procedure, I can see the beginnings of what happened. To place the device, the neurosurgeon made one incision in Trey’s low back and one just under the right ribs in his abdomen. Through the incision on his back, the surgeon placed a catheter in the subarachnoid space in his spine. He then tunneled the catheter from his spine to his abdomen, where the port was placed. After surgery we were instructed to keep a binder on Trey, a wrap of sorts that goes around his abdomen, with the hopes of keeping pressure on the area so that while he was healing, no cerebrospinal fluid would leak out of the hole in his dura, pool, and cause an infection (and removal of the device). We were to keep this on for 2-3 weeks until we received a Benik wrap that would also wrap around his abdomen, but with a different purpose: to protect his new port from breaking. Many of these ports have broken, so the wrap was meant to minimize the chances of breakage.
While in North Carolina we were followed by a team of doctors and nurses who kept reassuring us everything was fine. 11 days post-op, when the stitches came out, Trey’s skin had already overgrown the stitches so you could barely see them and the site was red (Trey’s had numerous surgeries and his body had never done that before). The redness never really went away and looking back, his incisions never really looked great. We were seen in NC before we left, this past Monday, October 24 and were told everything was fine. We flew home Tuesday.
When I looked at the incision sites Wednesday, I was concerned. It was two and a half weeks post op and things weren’t healing as fast as they should. The port area was worst, as it seemed a bit ‘open’, but both areas were red. Doctors Wednesday prescribed an antibiotic cream for the sites and suggested leaving the site covered with cream, gauze and the binder during the day, but to leave the sites uncovered, to get air to the incision areas, over night. That should do it.
However, Thursday morning I was convinced I saw stitches in the sites, so we were back off to the hospital. They were in fact stitches, but they were deep stitches, not meant to be seen. The wound was opening up. Then, the plan was changed. The incisions sites were worse, so we stopped using the one cream, started another, and kept everything covered. I was nauseous just looking at the sites. We had an appointment to come back Friday afternoon to have things checked.
Friday morning, his port incision was basically wide open and I thought I could see the actual port. I paged Trey’s doctor and we headed back to the ER. By about 1:30/2pm on Friday, neurosurgery confirmed that what we were looking at was in fact the port, and that the device would need to come out now. It was infected.
Both the neurosurgeon here and I talked with Dr. Muenzer (and we sent Dr. Muenzer photos of the incision sites) and everyone agreed. We did not have time to travel back to North Carolina to have Trey seen. The device needed to be taken out as soon as possible.
Why did this happen? I’m still not sure. I’m not sure anyone is. Why was it an emergency? Long story as short as possible, the whole reason for this trial was to get Elaprase, the enzyme Trey is missing, into his brain. The weekly enzyme replacement therapy infusion he gets cannot cross the blood brain barrier because the protein in the Elaprase is too big. So, they crossed the blood brain barrier by putting a port-a-cath through the blood brain barrier (across the dura) into his central nervous system/brain. The reason the blood brain barrier is so protective and doesn’t let much cross it is because if the usual bacterial and viral infections we get like colds and flus get into the central nervous system, it’s SERIOUS. Like meningisits and death serious.
So, when Trey had his intrathecal port-a-cath placed, they were essentially making a clear path to get into the central nervous system (CNS)/brain. When the incision site at the port started opening up, there was now a path from the outside world (bacteria & virus’) into Trey’s CNS. Fever and an elevated white cell would indicate a systemic infection, which Trey fortunately never had, but the fact that this path from the outside to his CNS was wide open and infected, was not safe. If we didn’t get the device out now, the increased white count and fever was sure to come, and because we didn’t know when, we didn’t have time to fly back to UNC. And, because the local area was infected, they could not just re-suture up the site, because then the bacteria and infection would be sealed into his body, with a path still to his CNS.
So, Friday afternoon Trey was put on heavy duty antibiotics to prevent meningitis while we waited for the OR. Trey finally went back for surgery (after not eating all day, poor guy) at 9:30pm and was out by 11.
This morning he’s doing well. I’m just glad the device is out. I was so so scared and am just grateful this is behind us. Today we’re waiting to see cardiology and infectious disease to find out how long Trey needs to be on IV antibiotics before we can go home.
Then we figure out the next step. No one can go near his CNS until the infection is healed. So, we’ll be in touch with Dr. Muenzer and UNC as he heals. It sounds possible and it is my hope that as soon as Trey is healed, he can get his next dose (which should be on November 15) via lumbar puncture (like an epidural, just deeper: they just put a needle into the subarachnoid space, push in the dose and take the needle out). First priority is safety. Second is getting enzyme into his brain. Third, is discussing placement of a new device.
Because this device has had issues, I want to discuss risk versus benefit. Trey could get another port placed, get his monthly IT doses of enzyme wide awake, thus skipping all the risks associated with general anesthetic and sedatives. However, the device has broken or led to emergency removal more than once. Given what just happened, I’m also not sure I’m comfortable being this far away from the doctors who placed the device and know what’s going on with Trey, MPS II and the trial. For the past few days, it’s been a panic of who do I talk to, what do I ask, do we fly to NC or stay here, who’s overseeing Trey’s care, who’s making decisions, and getting all the doctors involved (UNC trial doctor who oversees Trey’s care in NC, UNC neurosurgeon, BC Children’s neurosurgeons whom we’ve never met before and who know NOTHING about the device or trial, and BC Children’s Biochemical Disease team who oversees Trey’s care here), to talk; to each other and to me.
On the other hand, Trey could get monthly lumbar punctures (LP’s) to deliver the drug, which would then have no port risks, but this would involve monthly sedatives and possible scarring of the spinal nerve roots. I have questions to ask, conversations to have.
But for now, Trey is well. And your support has helped me survive. My mom and grandma, Omi (who’s 86), took shifts yesterday looking after Avery and Sadie. Mom slept over. Omi took Sadie and Avery to dance class this morning. My cousin Krista and brother-in-law Clete are having a party for our nephew’s birthday. My mom is dropping Avery and Sadie off at the party. Someone at the party will drop them off at our other friend Madeleine’s house. Madeleine will watch the kids as long as we need. Tovah is making us dinner. Nadine stopped by last night to drop off gourmet snacks while we waited. Other people have stopped by the house to offer their time. Others yet have offered to shop for Halloween and Avery and Sadie’s birthday’s which are tomorrow and next Saturday.
At this point, we’re barely keeping our heads above water, so not only does your support mean so much emotionally to us, but we have really needed your help.
Thank you everyone, for keeping Trey and our family in your thoughts and prayers.
Love Deb
