Day +100

It’s been an incredibly long year. We’ve largely set aside our lives as we turned our focus to seeing our newborn through a terrible, rare and unforeseeable disease and all of it’s consequences. When we began this journey, I honestly didn’t think we’d be where we are today. I didn’t think Zoe would make it through this nearly unscathed. I feared for her very life based on the statistics, feared she would develop a neurological disability, feared she would suffer from any number of other permanent side effects to her treatment.

Today, Day +100, I can say that it appears we have made it. Zoe is bright and healthy, eating on her own, off most of her medications, and officially an HLH survivor. There continue to be worries, but increasingly they stem from having an infant and all of the peculiarities that each child has, and not from having a sick infant specifically.

On Monday she is scheduled to get her central lines removed, which is another very big day. She’s had lines in her chest now since she was about 2 1/2 months old, the majority of her life. We’re hopeful that, once they are out, she’ll feel much more spry and ready to work on her crawling. As of now, she doesn’t like being on her belly for very long, the lines and clips dig into her chest a bit.

It’s a little hard to say where we go from here. Both Michelle and I are looking at life changes as a result of this, and both of us, and Maya, feel irrevocably changed by the entire experience. I wouldn’t wish it on anyone, but it has been an amazing experience in both the highs and the lows. We’ve found new ways to bond as a family even as we’ve been forced to spend too much time apart. We’ve found ways to grow as people as anyone might, faced with unexpected hardship. We’ve watched our girls grow up even as the world seems out of control around them, and come out of it, well, just fine to our amazement.

I started out writing this blog without having much idea of where we were going. It was, and is, a way to cope with what we’re experiencing. I’ve always been better at coping by writing more than by talking or exercising, drinking, what have you. When I began, I decided that the best way to handle things would be with a bit of anonymity, so that if something bad did happen, I was not writing something that could become problematic for the doctors and others working hard to help us. We have made it through the gauntlet at this point, and so I’d like to offer a few thanks. There have been so many people we have encountered who have helped even in small but important ways, and I can’t thank everyone enough. Please know that we appreciate all of you and all of your help more than we could even convey.

We’d like to thank Dr. Dave Anderson of Forsyth Pediatrics, the doctor who first saw Zoe and trusted his gut to send her to the hospital earlier than expected or “required”, and in doing may have prevented major damage from the disease. Dr. Mike Kidder, who couldn’t sleep at night while he worried about Zoe and worked on her diagnosis tirelessly. The ER doctor who happened to have seen HLH before and, we’re told, first suggested it on rounds when her diagnosis continued to be elusive.

Dr. Thomas McLean and Dr. Marcia Wofford, Zoe’s primary doctors at Wake Forest University Hospital, who managed rapid and effective treatment of her disease and her recovery, putting her in a position to go into her transplant in the best possible condition.

Dr. Paul Martin and Jayne Cash, the primary transplant doctor and nurse coordinator at Duke, both of whom suffered my nearly endless questioning, pushing and challenges as we tried to be sure we were on the right course. Their patience with us was boundless. And, as silly as it sounds, the World Wide Web for existing and allowing me to give myself a crash course on this disease, ultimately helping us make good decisions where, even a decade before, we’d have been in the dark.

The entire nursing team at the Pediatric Blood and Marrow Transplant Unit at Duke University Hospital, particularly Jess, Luke, Courtney and Tameka. The transplant doctors who we saw on rotation, Drs. Parikh, Prasad, Szabolcs, Page-Chartrand, and Driscoll.

The transplant families who found us through this website, followed our progress, cheered us and advised us both publicly and privately, particularly David, Andrea, Shana and Michelle. Your counsel and support were invaluable.

Our friends and family, you know who you are, but particularly Sandra and Larry, who have set aside their lives just as we have ours, moved into our home, put thousands of miles on their cars, and taken on care of our dog, Zoe, Maya, Michelle and I whenever we needed them. We never could have done this without you.

Ups and Downs

One thing we’ve come to understand about this disease from reading others’ stories is, you must be prepared for the ups and downs. It causes a lot of havok, and can generate swings in the body’s natural function.

We have been fairly lucky so far with Zoe, she has weathered it well all things considered. Here’s hoping that will continue.

Last week Zoe’s immune count marker (SEG Neutrophil) was up to 2.0 or 2000, this week it’s back down to .2, or 200. Not a good swing for us.

So this week we’re back in huddle mode, windows closed, no going outside for her to enjoy the beautiful weather until she has rebounded again. Dr. W felt it was likely due to the chemotherapy regimen, but we are wondering if she is not also struggling with her first pollen season.

In retrospect, it was probably a bad week to spend much time out, pollen in our region is still fairly heavy.

Vitals this week:

  • WBC: 3.1 (5.0+ is normal)
  • RBC: 2.83 (3.1+)
  • Hemo: 8.6 (9.5+)
  • SEG Neutrophil: .2 (1.0+)
  • Platelets: 250 (150+)

Rolling with the punches here.

Hopefully next week will be back to where we were last. We’ll have more to report about the outlook for her Stem Cell Transplant soon.

The Horrible Very Bad “NPO”

Nil Per Os: A Latin phrase which means, “nothing through the mouth”

Outside of the truly bad things, those three little letters have come to foreshadow more anguish and suffering around our household than most any other.

Don’t feed your baby. Don’t feed your baby.

It’s really hard to get that through the head, and even harder after a few hours of listening to her fuss about it. With an older child, I’d imagine you can explain somewhat, but Zoe only wants one thing at this point, and she just doesn’t understand when she doesn’t get it.

We’re due for Zoe’s new line to be installed today. We spoke to Dr. W and had the actual VP-16 moved back a day to our and her relief, so that she is not getting it right on the heels of the surgery and well into the night. In order to have the surgery though, Zoe has to have her anesthesia, and of course this means that little card appears with the dreaded NPO on it.

We have some confusion as to when the NPO is to start, and a little frustration as a result. Michelle was told initially 12am on the phone, with a surgery scheduled for 12pm. That’s 12 hours without food for a child who eats every 2 hours normally. A little harsh? You would think so. The nurse specifically said no milk or formula, even though the 12am time is typically for solid foods.

The point of confusion is that we were told 4 hours on Monday by the surgeon doing the procedure, and we tend to want to trust that number more.

So to try and resolve it, I call in, explain who I’m calling for, and that I need clarification on the NPO. “How long before the surgery do we need to wait to feed her?” 12am. I explain that Zoe is 3 months old, and immediately the response is “oh, well the NPO is more for solid foods she can have fluids up to five hours before.”

I feel sure that whatever information is available to whoever is making that decision must indicate her age. Surely. If it is, I wish that they would give us the 5 hour number up front.

After a little more back and forth, we clarify that it’s technically 4 hours, but they tack on an extra hour in case they want to move up her surgery, and the NPO is pretty much always set for 12am the day before surgery. Does it matter if it’s an infant? Technically yes, but in practice, no. They put what they put and it’s again up to the parent to push back and get the exact information, lest they suffer through needless additional hours of crying inconsolable baby stress.

Early in our stay at the hospital, for her first Broviac, this same thing happened exactly — one person told us 4 hours but someone else said midnight, we played it safe and went with midnight, and the result was a horrible experience for Zoe and Michelle and I. At that time Zoe’s blood pressure and heart rate were a concern, since she was still fighting back from her initial fever, yet still she was put on a 10-hour NPO crying and amping up her stress all night.

This time, we knew better. Progress!  And, now Zoe and her parents will get to sleep tonight instead of living the horror movie that is an unfed baby.

Update: Zoe is done with surgery, all went well. She was very very hungry by the time of it, and let everyone know.  As feared however, we did not actually get in there until 2pm.

I completely understand the difficulty in lining up multiple departments at one time to get the Surgeon, Anesthesiologist and Oncology NP in at the same time, but it feels like there must be a way that doesn’t involve infants going unfed for 7-12 hours routinely. At the very least making sure to distinguish between solid fed and breast/formula fed children seems like it would be a good idea.