I have been holding off an update because I really wanted more answers.
Or a plan.
Or for someone to say everything is going to be fine.
Or for Kyrie to not be purple.
Instead, Heavenly Father has sent me back to chaplaincy, where I am reminded that there are not always answers, and things don’t always go according to plan, and sometimes things are not fine.
For right now, that’s where Kyrie is holding: not-fine, but also not-in-crisis. Except she always is in crisis. But not. But she is, except she is fine, except for the crisis part. That’s our life with Kyrie.
By not in crisis, I mean to say, we are coming to terms with hypoxia and cyanosis just being part of her life, that no matter how badly we want a pink baby, we are having to come to terms with a tan/brown/purple/oh-it’s-a-good-day-so-she-is-peach-for-an-hour/nevermind-she-is-blue-again kind of baby.
She has just had her checkups with pulmonologist and ENT and neurologist.
The pulmonologist says that the physical therapist is right, that because she doesn’t breathe well enough, her chest cavity isn’t growing right, and that because she consistently works so hard to breathe, her back muscles are too tight and her shoulders stay too hunched. But her lungs themselves are doing great, other than being too small since they are never fully inflated, and so there is really nothing he can do to help except make sure medicaid is still approving oxygen and supplies (as much as they will).
She is to remain on oxygen when exerted, or her sats drop too low, or for physical therapy, or if she struggles when eating.
She is to remain on the keep-evaluating-for-a-trach-and-gtube list, but he’s not going to do it right now. She functions well enough without air for about a third of the time, so it’s not worth the extra risk to move forward yet, though we may still need to in the future depending on how she grows.
He is frustrated, like we are, that she wasn’t trached in the beginning, because she really needed one that first year, but now that she is almost 18 months, we can’t go back in time to fix it. But that wasn’t his fault, and he is doing his best with her now. He did order another swallow study to see how much she is aspirating these days, but that’s about all he can do.
She was sick for about five weeks with same thing the other kids had for only one week, but she did pull through it without any pneumonia or being hospitalized. That’s so big for her! He was willing to hospitalize her if we wanted, but felt it would be better for her not to if it could be avoided and we agreed. During that five weeks on oxygen, though, the same thing that has always happened did happen again: she grew. Every time she is on oxygen consistently for some time, she finally grows. In five weeks, she went up a shoe size and jumped out of 9 month clothes all the way up to busting out of her 12 month clothes. I know she is longer, and heavier, and I am excited for her 18 month checkup to get her new weight. She has chubby legs all the sudden, and her face is full again, and she was so happy and played so hard!
But then she pulled through, got better, and the oxygen went back to intermittent, and we are already losing some of the chub on her legs and she is fussier and doesn’t play as much so actively.
The ENT saw her, and pretty much said that it’s good she is talking so much, and that with her tubes she is hearing better than expected, even though it’s not perfect. The tubes are in place, and look perfect, and we have not had any more ear infections since they were placed, not even when she was just sick for all those weeks. This is a relief, that at least one thing is going better.
There is no new news structurally: her airway is too small and the wrong shape, her epiglottis is the wrong size and shape, removing tonsils and adenoids may open up her airway some but could also cause more speech and/or aspiration problems. He still thinks part of her random and unpredictable sudden desats may be because of the epiglottis and how it flaps down wrong sometimes, so he wants to shave it so her airway isn’t blocked from that, but if he does shave it then he can’t put it back so it’s a pretty permanent thing for something he’s just guessing at and we haven’t wanted to do that yet. She is outgrowing her trachea, which then gets more inflamed from trying too hard, and so especially when she eats and the esophagus pushes on it, she struggles and tears just pour down her face even though she isn’t upset or actually crying.
And, he doesn’t think she is a candidate for traching at all because she is still having central events rather than only peripheral events.
A “peripheral event” means her body is conserving oxygen, causing her hands and feet to change colors because her body is reserving all the oxygen for her brain and heart.
A “central event” means her brain and heart are not getting enough oxygen, which is when her lips turn blue and her face pale, etc.
If her body were struggling to breathe because of structural problems, which it is, but if that were the only issue, a trach could bypass this and solve the problem.
But they think part of the issue is her brain getting the messages to and from her heart and lungs, which a trach cannot solve.
That brings us to the latest MRI and CT scans:
The CT scans show that her mastoid air cells are not developing and still opacified, with complete opacification of the right middle ear and opacification of the epitympanum on the left. The opacification is bilateral, with her right ear worse than her left middle ear. They ruled out fluid and infection, but not all the bones are there so some of it floats around the wrong place. The tubes have helped hold things in place, and there may be some surgery they can do later, but right now it’s just because of the extra bones missing from when she was born.
The CT scan also shows disconjugate ocular gaze, which we already knew since last summer, and that’s why we patch her eye for part of the day. We go back to the pediatric eye specialist in a few more months to see if it needs to be patched for all day or not. It’s just part of her cerebral palsy, the left eye being weaker and not able to focus or move as it should.
The CT scan also confirmed a fistula, or a hole in her cleft palate. When we go to the 18 month well child appointment, we will get a referral to a pediatric dental specialist to get her an obturator if we need, but mostly once she got the hole we had liquids out her nose again for about two weeks but since then, it seems she has figured out how to manage it and seems to be doing fine and not be bothered by it. Her repaired palate, they said, was not repaired in the back, just the front half, which we know, so she that is why they are expecting her to need pflap surgery when she is four.
The CT scan also confirms her micrognathia, or the recessed chin, is getting worse. She will need another distraction, and that may be why her breathing problems are escalating again. We want to wait as long as possible, though, because each one starts the countdown on the next one, and we want her to have as few as possible over her lifetime.
The MRI showed a linear signal in the right side of cervical cord at c1, which may impact left side movement, which may be part of the cerebral palsy weakness on her left side. There is “significant blossoming” on the ride side of the medulla, which is where her stroke was, and the right side of the brain affects the left side of her body, so again the impact from the stroke and now cerebral palsy also both were left side affected. There is abnormality on the brain stem and lower cerebellum, and so they want another MRI to rule out a type of Chiari malformation as well? They could also see her palate repair, and that the micrognathia is improved from before distraction surgery last summer, but that it has gotten worse since after surgery, and that her tongue base is blocking her airway. They also found telangestasia, confirmed with the red “birthmark” on top of her head that we often see through her hair, which they said anywhere else it would be of little concern, but hers is obviously in her brain and leaves her high risk for a massive brain hemorrhage that would be life threatening. Because of this, they want us to go back to the geneticist again to be tested for HHT (I already forgot what that stands for) because that can also cause shortness of breath and seizures and small strokes, all of which she has had, as we well know. The neurologist is pretty concerned about what he saw in her brain, and kind of surprised her problems because of it haven’t been worse. He said there is a fair amount of artifact on the cerebellum, which means either it is even more serious or it is just a bad picture, so they are going to do another one.
All of this goes back to her midline facial features being consistent with PRS, but the root cause and other features really being other issues, and making her one complicated baby.
(In other news, a specialist I talked to at this conference, a doctor from Minnesota that was in my audience, said that this isn’t heroin that has done this to her. She said it had to be the alcohol and cocaine, which bio-mom did test positive for during pregnancy. Not that it changes anything, but at least it’s information.)
The simplest answer, though, is simply that sometimes she needs help breathing, supplemental oxygen seems to be sufficient, and she needs extra care when she is sick and a longer time to recover.
But otherwise, we have almost made it to 18 months, which is a miracle.
And on her good days, any stranger looking at her would not even know anything was wrong.
And on her worst days, we don’t know if this will be the morning she doesn’t wake up, or the time she loses her balance because she is having a massive stroke from which she will die, or the one time we try to resuscitate her and she doesn’t come back.
With Kyrie, it has always been the case that everything could change in only a moment.
But with Kyrie, it has also always been the case that she has constantly surprised (and charmed) everyone.
The pulmonologist says that as long as we can keep her alive, she is just going to be blue most of the time, and that the complications of that will continue to build up, but there isn’t much we can do about it.
The ENT says that we are going to run out of time avoiding surgeries, but even what he can do is just remove things that we can’t put back, and that this will absolutely impact quality of life.
The neurologist says that we need to understand that while obviously part of her breathing problems are structural, that she is not “only” a classic PRS baby, so a trach is not a magic wand in her case because a great deal of her breathing problems is from her brain causing central events and the inconsistent presentation and unpredictable desats. We need to be prepared, he said, that she could die suddenly from a hemorrhage, today or ten years from now, or just somehow carry that around with her for a lifetime. But he said, in an effort to have compassion on us, that dying from that would be far quicker and less painful that dying from suffocation, if that’s any comfort.
We need to understand, they all said, that we are lucky to still have her, that she is doing great – beyond expectations when you consider what is going on inside her – and that we should enjoy our time with her and know that we have done a good job caring for her.
We need to understand, they all said, that there is really nothing they can do, except monitor her and provide what we need for her to be comfortable, like oxygen and inhalers and ng tube food to keep her as beefed up as we can.
We need to understand, they all said, that her advanced cognitive experience may be part of her enjoying as much life as possible in as little time as possible, and just not miss a moment of it.
We need to understand, they all said, to let her good days be good days, and just be grateful, and embrace them, and not worry about what else we could do when there is nothing to do.
We need to understand, they all said, that blue really is her baseline, and this is just where we are.
It reminds me of the day when my mother was killed, when I thought we were grieving my father who had died of cancer, when I thought we were celebrating my new marriage and a pregnancy and our new life together. None of us know when or how we will die, and none of us know when. What they say is true for all of us, and I see it every day as a chaplain.
So yeah, she’s fine. Just fine. Sometimes blue, but fine.
Resigning ourselves to a blue baby doesn’t mean giving in. It just means we aren’t going to chase specialists all over the country anymore. She is too tired for that.
Accepting blue as a baseline after a year and a half of fighting against doesn’t mean settling. It just means our advocacy gets more focused. It means we fight from where we are, rather than where we had wanted to be.
Realizing you can’t make your baby any better doesn’t mean giving up. It means being present. It means dealing with your own emotional response to what your child is enduring, instead of fixing them by trying to fix the baby.
What is important is that we love her, and that she loves her brothers and sisters. What is important is that she is in best school for her, with teachers and a nurse who adore her. What is important is that she sings, talks in full paragraphs as of this week, and is learning what she likes to eat and doesn’t like to eat. What is important is that she loves stories, and baby dolls, and climbing the impossible slide in the backyard. What is important is that she knows how to pray, and how to ask for help, and how to laugh. What is important is that we are a family, and that we are sealed together for time and all eternity… even if we beg everyday in tearful prayers for more time.