Baby Hayden Hyper IgM Syndrome – Day 4

Today was the meeting of the minds, the final powwow before the fun happens tomorrow. The day started with the 4th injection of Neupogen, followed by sitting in the waiting room until the final meeting started. Once the main doctors were able to come down, Stephanie and the medical team went through the final instructions for the next 48 hours. Since many of the doctors are still managing other patients, not everyone was in the meeting for the whole time. There was a lot of coming and going in stages as each department said what they were planning on doing when it was their turn, and then they would head out to help other people.

To jump to the first day blog post, click here.

Day 2 and 3 blog post can be found here.

For the most part, everything is still going as planned, and the tentative schedule that we were provided with at the start of this process is still the plan for tomorrow. As a recap, this is what we are expecting schedule wise over the next 48 hours:

• Tuesday:
  • 6:15 am – Stephanie gets to the Fetal Diagnosis and Treatment and the Fetal Heart Program center for her last injection. Dr. Bill or Jessie will meet her there, perform the injection, and then walk her to the Interventional Radiology Department of the Children’s Hospital of Philadelphia (CHOP).
  • 6:30 am – She should be at Interventional Radiology by this point. She gets checked in, and then the Apheresis Team and Anesthesia Team will come in to get her prepped for her central line. It takes about 20 minutes to set up the central line. The type of central line they are using (since there are various placements, and names for each procedure) is going to be Internal Jugular Central Line (going into the jugular vein, and not the superior vena cava like was mentioned before). The anesthesia is not going to completely knock her out, but the goal is to get her woozy enough to wake back up if they had to shake her around to do so. Using ultrasound to identify the proper placement, they will then inject the needle, with a guide wire into the jugular, and will then stick the line over the wire and prepare the process. Sounds scary, but once again, these happen all of the time, and are very safe. After receiving the central line, they will then bring her back upstairs to have the apheresis done.
  • Once she is ready for apheresis – As mentioned earlier, they are estimating about 2 blood volumes (all of her blood, twice) will pass through the machine to collect stem cells. Interesting note, while everyone agrees that this is a safe procedure, and it happens all of the time, it has technically never been done with a pregnant female for the purpose of stem cell collection. Dr. Haewon Kim, the medical director for the Apheresis department, started making calls to her colleagues throughout the country to ask about it, and apparently there is no experience with pregnant women and apheresis. There is one patient that was written about in a medical case study that was pregnant, and had to receive aphoresis for cancer treatment (no complications to mom or baby from apheresis), but that is about it. Due to this, there is now a fetal monitoring team that will be watching over everything during the procedure. The number one focus is Stephanie during the apheresis, so they will monitor ionized calcium, magnesium, etc during the process. Similar to when someone donates plasma, tingling, cold mouth/hands, etc can be an indication of lowered electrolyte levels in the blood. There should not be any problems, but they will monitor nonetheless. Additionally, blood pressure could go down (hypotension), so they will include calcium in the return lines back to the jugular vein. As long as blood pressure and calcium levels are normal, no problem with the baby. Estimated time 2-2.5 hours. (Side note, she was told to take Tums in order to increase her calcium before, and during the procedure… which temporarily made me question the intelligence of who we were working with, and what we were doing again… but I digress, and we continued on…)
  • Once the number of stem cells needed is collected, the central line comes out. They will monitor her for a few hours after the apheresis, and as long as she can walk around, eat, etc. without issues, she will be free to leave the hospital, and would not need to stay for further monitoring.
  • They will move the stem cells to the special machine that they had to get emergency/temporary FDA approval to use in our situation, where the cells will processed until ready to use. They are guessing that the cells will be ready for use by 3:00 pm Wednesday.
• Wednesday:
  • 1:00 pm – Stephanie gets back to CHOP a few hours before the cells are expected to be ready, just in case they are ready to go before their scheduled time. She will then prep for an outpatient procedure of injecting the prepared stem cells into the fetus’ heart. Since Stephanie is crazy, and likes to watch things like this, they are not planning on using anesthesia during this part. If for some reason they need to use an anesthesiologist though, they would need to move her from outpatient to the operating room for the injection. This helps since they may need to have Stephanie walk or move around to get the baby in the correct position for the injection. If the baby is “quiet” and not willing to move around, they sometimes give orange juice (or other sugary drink) to increase glucose to the baby. It is common knowledge that sugar makes people go crazy, and it would be a way to encourage movement to the baby in order to get where they need him to be. Dr. Mark Johnson will then decide if the baby is in the correct position, and begin with the injection of stem cells into the heart. There is a possibility that they could use a local anesthetic (and not involve the OR) on the baby if he is a “whirling dervish” and they need to calm him down from moving too much. They will then take a transducer and under ultrasound guided imaging, they will take a “very long” 22 gauge needle, preferably to the right side of the heart, and then inject the stem cells, followed by a little bit of fluid to make sure it all gets into the heart. There could be some slowed heart rate during this process, but it returns back to normal most of the time in only a few heart beats. They may have to do more than one needle stick if the baby moves before the injection happens, but the goal would be one stick. This procedure would be similar to the CVS that Stephanie has performed every pregnancy in order to get genetic samples, with the exception that the CVS uses a much larger needle, so it shouldn’t be as uncomfortable for Stephanie. Risks of course include unsuccessful procedure via bleeding from the heart, or stopping the heart all together. This is uncommon. There is also a potential of infection, so Stephanie will be prepped for surgery as a precaution. Once they have injected everything into the correct place, they will monitor her for a few hours to see how everyone is holding up. After monitoring she will be released from the hospital with instructions to not overexert, or be too active for about 24 hours. Following that she is able return to normal activities. They will do follow up ultrasounds after a day or two, and another ultrasound about a week later before she returns to Houston.

They encouraged her to relax and take it easy during this process, and some how Gilmore Girls, binging on Netflix came up. The male doctors appeared confused and disoriented, while the females in the room giggled with glee and disappointment as to the how the new Gilmore Girls episodes turned out. I guess we can all look forward to Stephanie’s thoughts Gilmore Girls…

More details as they come throughout the various procedures, etc.

To see the Day 5 blog post, CLICK HERE.