Trepidation

Today is Eli-Grace's 3 month birthday.







The past week has been full of anxiety as Eli of course steps a little outside of her normal and has been exceptionally sleepy. It's hard to read these signs and know what they mean. It seems to have settled out again, and likely isn't anything of great concern. That being said, what's on everyone else's mind, and on ours is, that Elianna died just 8 days after her 3 month birthday...we only had about a week of warning with her, where we felt like something was up, but didn't know how to interpret it. In hindsight, we of course can now see what was going on. It's hard to not to hold our breaths as we live these days leading up to that landmark. We've done this before and we know what's coming, but anticipating your child's death is still a brutal experience.







Milena is becoming more and more of a big helper in our home. She certainly loves to be a part of caring for her sister.

It's hard to say what will happen with Eli-Grace. For some context, I know of a family who had identical twins with the same condition and they died at 4 and 8 years old. Doctors aren't saying anything prognosis wise, and they really can't, it's just too hard to predict anything. She's definitely got her own personality and way of being in the world. Her issues are different that Elianna's, but we seem to be managing them reasonably well.

Today I was watching her, and marvelling at the fact that she can move her arms (more than her sister could). I can't always tell if the movements are seizure activity or not, but I try to enjoy them regardless. So, it seems that she is doing a bit better than her sister at this age, but that's not to say that disease isn't still present and progressing.






My gut says we will get to spend at least part of the summer making memories as a family with Eli. We've managed to be adventurous a few times, and will hopefully do a few more outings. It's a bit of a challenge with the pumping and feeding schedules, but I'm more flexible now than last time (I have a much greater trust in my milk supply), and feeding feels more flexible too, because Eli-Grace lets us know if she's hungry, though it's much easier to stick to the schedule.







As a family, we are managing (sort of). Trying to developed a schedule that accommodates both girls' needs is tricky. Meal times and bedtime seem to often coincide with feeding and pumping. So, sometimes I get creative.







Walking this journey certainly has taken it's toll on us all emotionally. We are short with each other, much more than I would like to be. Michael and I struggle to have any time for each other, and feel like we are ships passing each other. Milena feels starved for time with me. There are days where I feel like we get it right. But there are many other days where it just feels awful. Please continue to pray for us, we need God's peace and strength to fill our home, and the gaps in our resources when it comes to our relationships with each other, and heaps of grace for the each other in the hard moments.

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Cold hands

We are back from our trip to BC. It was intense but worthwhile. I'm glad we got to go on a meet the baby tour. We saw friends and spent time with family. Getting ready to leave and travelling was exhausting. Staying at the MCC Guesthouse was brilliant. Turns out that I have a tribe out west too, people came by for visits, shared toys with M and brought food when they stayed for mealtimes. Abbotsford was GREEN and warm and very springlike which was good for my soul.
Eli seems to be improving when it comes to things like motor skills, or at least is a little more capable than her sister. She can turn her head from side to side when she lays on my chest. She can move her arms and bring her hands to her face. She also cries when she's hungry and when she has a dirty diaper.
It's easy to want to evaluate these things as signs that things are good, that she is doing good. The thing is, this disease is unpredictable and things can shift suddenly. I don't like to evaluate these things, but simply observe them.
Other things are also going on. She had some pretty big apnea episodes at the beginning of our trip. The other day during a belly rub to ease some gas pains, I realized I could feel her liver (this was something that was very enlarged and could be felt in Elianna's belly in the week leading up to her death).
Many times at night I reach over and feel her cold hands and have a moment of panic. I then move my hand to her body to see if I can feel her breathing. Her hands get cold because she can move them out from under her blanket. I wonder if she is uncomfortable? Tonight I will try swaddling her, see if that helps keep her hands warm.


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2 weeks

Eli-Grace Mahala Schultz
Born March 14, 2016 at 7:57pm weighing 7lbs 15 oz and 22 inches long.  

Without getting into a lot of details, we had a slow start to labour, not really a big surprise, since this has been my mode of operation with both of the other girls.  Once things were really going, around noon on the 14th, the reality of our circumstances really hit me hard.  It was by far the hardest thing I've ever done, labouring with such an unknown waiting for me at the end of it.  Apparently I kept saying out loud between contractions, but I'm going to get to hold her, this was some kind of strange motivation, but it was the one thing I knew for certain, that no matter what happened, I would hold our baby Eli that day.  
Once Eli was delivered, I was able to pick her up.  Turns out God heard our prayers, I wanted so very much to have some time with Eli, to be able to bring her home.  As I held her, I realized she was breathing, her colour was much better than Elianna's, everyone in the room was amazed.  The doctors tried suctioning her, but there wasn't really anything to suction.  A bit of oxygen was used, just to encourage her to keep breathing.  Realizing things were stable, we had Milena brought in to meet her sister, and shortly thereafter made the call to go home as planned if she was stable.  Milena got the privilege of cutting the cord and was totally grossed out by vernix.  She went and got a wash cloth to wipe it off my shoulders and chest, so sweet!  We got ourselves together and headed home a few hours after Eli-Grace was born.

The first few days at home were a little chaotic, as we arranged our living space to accommodate our newest family member.  The key things were out of storage or still nearby from Elianna's life, but we hadn't officially made a space for Eli in our home.  Milena is the most doting big sister a baby could ask for, but needs some help channelling her love in ways that are helpful.  

I inserted a feeding tube when Eli-Grace was about 16 hours old and gave her her first feed shortly thereafter.  It went smoothly, even though it's a skill I hadn't used in over a year.  This time around, tube feeding was much less stressful and I had the confidence to follow my maternal gut instincts, rather than some feeding chart and pressure from doctors to meet calorie requirements.  I am pumping breastmilk again to feed Eli-Grace, and it is going well.  Again, much more relaxed this time, as I have confidence in my milk supply.  Our doctors and midwife have been amazing, checking in with us regularly, smoothing the road for us and coming for home visits.  

Eli-Grace's condition seems similar to Elianna in some ways, and in other ways feels different.  The prenatal diagnosis still stands, she does have Zellweger Spectrum Disorder.  The list of symptoms is long and varied, but here's what we know is going on with Eli-Grace.  She is having seizures, she has low muscle tone, potential vision and hearing impairment, and poor reflexes, particularly her suck/rooting reflex seems to be non existent (which is why she has a feeding tube), and she has had some apnea episodes and has silent reflux.  She does have some sensitivity to pain, based on the fact that she cries when I insert the tube, and even fights it a bit, or remove the tape that holds it in place.  She seems to cry to let us know she's hungry, which has meant the schedule has been held to more loosely, since it feels like we can rely on her to tell us she's hungry.  Most of these issues can be traced back to neurological problems caused by the disease, and as such are not curable, though some symptoms can be managed with medication, like the seizures.  

Overall, we are doing reasonably well.  With a prenatal diagnosis, we have been able to better prepare ourselves for this journey, and yet in many ways still feel totally unprepared.  Emotionally we are not in such a state of shock.  We have more flexibility in our work lives and our support people have been ready to jump in and help when we need it.  This is not to say we aren't stressed or tired or anxious.  We are.  We are caring for a dying infant.  That is hard.  Nothing can change that, but it feels just a tiny bit less awful this time.  Maybe it's because we are somehow a little bit accustomed to living in this kind of tension, I don't know.  

The famous question of how is Eli-Grace doing is a tough one to answer.  We have had our share of ups and downs in these first two weeks.  Every little thing leaves us questioning if it's an indicator that death is imminent.  That being said, as we get used to her normal, we have less of these freak outs and feel comfortable that today isn't going to be the day she dies.  So, when we say she's doing well, it just means we don't think she's going to die today, or even this week.  The things she is doing are within what's expected for her condition and are part of her normal, but it certainly doesn't mean there isn't things going on, apnea and seizures are scary things to witness.  It also doesn't mean she isn't going to die on some day in the future.  We don't know when that will be, so for the moment, we say she is doing good because that is what we know.  

We appreciate your love and prayers and practical support.