Hyper IgM Syndrome with Baby Hayden – Days 2-3

So far things are chugging along as expected. We are still in Philadelphia, and continue to meet with the doctors. There was a good response from everyone after the last post regarding getting the whole situation fleshed out, so that those that were interested could see what we were doing, why we were doing it, and what the potential options are. If you are wondering why I am doing “family” stuff on my “office” blog, well, that’s how we do things in our office. We treat our patients like family, and most of our patients treat me (us) like family as well. We know about each others families, vacations, struggles, and goals… In fact, it is due to the many patient and friend requests that I posted the last update. So thanks for reading along, and your support, prayers, and thoughts during this time in our lives.

If you would like to go back and read the original blog post that talks about Hyper IgM Syndrome, and the current endeavor that we are going through, feel free to check out this blog post: Baby Hayden Medical Journey – Background and Day 1.

As for updates, there is nothing too crazy to update as of now. On the 17th of February, Stephanie started her daily G-CSF injections. These will go on for five continuous days. The medication is actually called Neupogen, which in Latin means “crazy expensive.” Originally we thought the medication would be around $1000, but there was some discrepancy regarding the dosing instructions based on weight. One doctor thought we would only need a certain dose per weight, while another doctor felt differently. Throughout the day, there was a lot of phone calls, and discussion among the medical team, and it was decided that she would need more medication according to her weight in order to get the desired outcome. So the dosing tripled. Due to the back and forth between the various doctors, the first injection didn’t happen until nearly 5:00 pm.

The time of the first injection creates a minor problem, but everyone is confident that we can adjust the time table accordingly to get back on schedule. So Day 2’s injection happened around 3:00 pm, Day 3’s injection around 12:00 pm, Day 4’s injection around 10:oo am, and Day 5’s injection will happen around 7:15 am. It is a little earlier than the original schedule of “every 24 hours” but everyone is confident that it will still do what we need it to, without creating complications. After the first 2 injections, Stephanie reported headaches, and deep bone pain in the hips that affected her sleep. Luckily, the medical team consists of an excellent chiropractor that was flown in from Houston in order to oversee complications, and manage pain. Stephanie is in good hands.

As mentioned in the last blog update, the apheresis blood collection will take place after 5 days of injections. The goal is for Stephanie to get in early for her last injection, and then for them to immediately perform the apheresis. I also mentioned that in “normal” situations, the arms would be the supply and return lines for the stem cell collection process. In Stephanie’s situation, a central line handle this process instead. In order to accomplish this, she will go to the radiology trauma department, and under guided imaging, they will place a needle into the neck, in order to access the superior vena cava. If you have ever heard of a PICC Line, or a Hickman Line, it would be similar to that. The central line, or central venus catheter, is really safe, and research shows pretty much zero complications. Once the central line is in, all of Stephanie’s blood will pass through the collection machine an estimated 2 times (over 2 hours) until the machine has collected enough stem cells. Once her blood goes through the machine, it will be cycled back into her superior vena cava through the same line (different tube). If more stem cells are needed after the 2 hours, they will keep running the machine until they have enough.

The long and short of days 2 and 3 have included going to the hospital in order to get the injection of the medication. Two of the residents (fellows) that are helping out with the overall process have met us at the hospital in order to provide the shot. It is a subcutaneous injection, and uneventful. The running joke has been that Stephanie has probably performed more subcutaneous injections that all of the doctors combined since she used to work back office in dermatology. After all, how many times have you see the trauma surgeons on Grey’s Anatomy or House take time to do a small injection into someones arm? Since days 2 and 3 were over the weekend (Saturday and Sunday) everyone felt it would be easier to supply Stephanie with all of the supplies needed for the injection too. Apparently the doctors do not know where anything in in the hospital, and the nurses get everything for them. (I can relate… sometimes I feel like my office assistant knows more about the office than I do.) With medications, syringes, band-aids, etc in hand, we bring what we need to each injection.

Since we were only in the hospital for 15 minutes or so for the injections, we had a chance to do some sight seeing together. We went to church on Sunday which was close to the Children’s Hospital. They were nice and receptive, and welcomed us in like family. We saw the steps and statue from Rocky, Liberty Bell, played disc golf (of course!), the Philadelphia LDS Temple (just opened in 2016) and went to a fun restaurant where Stephanie’s friend’s nephew is the main cook. It was pretty awesome, and the friend called ahead to make sure we were taken care of. If you are ever in Philly, go check out Hawthornes Cafe, it is legit.

Tomorrow is injection number 4, and the big powwow with the whole medical team. The head of each department that is working with this case will come together, and talk about the schedule, procedures, etc. Unfortunately, I will not be there, but Stephanie’s mother will be arriving tomorrow, and should be there to help out. More updates as we find out.

For the third blog post, CLICK HERE.