Lifelong 'disability' - PVL

This is a hard post to write, so like most hard posts, I've put it off for a good two or three weeks, mulling over whether or not I wanted to make this public and how. But sometimes it's the hardest things that are most important to put out there.

Last month a brain scan showed that our son would not outgrow his prematurity by age two. My response surprised me. We were not shocked. We took it the same way we'd take hearing he had an ear infection or strep. Perhaps because we realized that we already knew, and he has been receiving therapy for this for over a year without a name in hand. A full week later the implications set in, the finality of it all, and it took me another week to process this fully. In the end, though, my stomach has settled and my son is exactly the same person he was before the diagnosis.

To be fair, for the most part, the news came as a blessing. The MRI of his brain showed he did not have hydrocephalus. His ventricles are stable, not growing. He won't need brain surgery for a shunt. The neurosurgeon renamed his brain as exhibiting "mild ventriculomegaly," a close cousin to hydrocephalus, a term which (like "mild hydrocephalus") also means "mildly large ventricles." Doctors seem to give fancy terms for things that could be said in normal ways. Lovely. He suggested a reason for the enlarged ventricles, which was confirmed when we met with the neurologist a few days later.

The neurologist confirmed the neurosurgeon's suspicions and gave Jonathan a new sticker for his charts. His ventriculomegaly is caused by periventricular leukomalacia (PVL). DON'T look it up.  If you ran across this blog looking for info on PVL, then go ahead and look it up. But everyone else, here's what it means for JAM:

Nothing much new. Actually, yes, really. I'm not being sarcastic. It's not degenerative, it's not going to change for the worse over time, and we've already seen the signs of it and know he can overcome it. We just didn't know that it had a term or that we could see a picture of it in his brain.

Since that's not a very satisfactory answer, here's a better explanation. His brain MRI shows that some of his white matter (a part of the central nervous system, a neurotransmitter of sorts) is gone, making room for a larger than usual area for the spinal fluid (that's the ventricles - it's where the spinal fluid hangs out). Perhaps the white matter weakened and disappeared as a result of blood oxygen levels going low (desaturations). That happened a lot in his early life. Or perhaps the PVL is a result of the same intrauterine infection that caused him to be born 17 weeks early. Either way, the grey matter, the thinking part, is unchanged. His central processing unit is still intact.

Rather than raising questions, this explains everything we've observed for over a year. The best part is that now that we know more about the "why" for what we've seen, we can better address his particular issues.

Jonathan is a strong kid who shows none of the classic signs of cerebral palsy** -- no low muscle tone or spasticity. And yet he has had issues with motor development. Sometimes he did fine. He figured out a pincer grasp really early on,  he could feed himself cheerios, and he taught himself how to point and grab his feet and all sorts of things. That said, for some things he can't just do skills that other kids just "get." We had to explicitly teach him how to bend, how to catch himself, how to move his legs to walk and crawl, how to clap and hold a bottle.  For some of these things, we could see he was strong enough and that he wanted to do these things long before he could do them. The motor planning just wasn't there. He couldn't watch-and-repeat. He had to have us retrain his brain.

Think of it like learning to ride a bike or whistle. People show you and tell you how to do it, and yet you can't just do it. It takes practice. We show the brain what we want it to do until one day it just comes. A little more practice and it is perfected. Once the skill is perfected, it stopped taking so much thought. We can just do it. The same is true with JAM.

I've been telling friends for months about how I learned to vibrato or perform complex fingerings on my viola -- about how I knew how to do it, I was strong enough to do it, and yet getting the message from my brain to my fingers took so-much-effort. And then one day, it just all started to click, and after months and months of practice, it started coming more easily. A switch had turned on in my brain. Apparently that switch is in the white brain matter. Within another week, the task could be done without much thought at all. We all rewire. JAM just has to do this for more skills.

In a nut shell, this is what PVL means for Jonathan. It's not every skill that's affected, but it effects some skills unexpectedly, and as he grows and learns new skills, PVL will likely continue to trip him up. He couldn't clap his hands until just last month, at 22 months old. He could pat his legs, but he couldn't translate that to clapping until we worked with him on it for a solid one to two weeks.

Now, to the post title. This new diagnosis means J won't outgrow his prematurity by next month. It means he has a life-long disability as a result of his prematurity. Sort of. Let me explain.

We made a new friend a week before the diagnosis. Her elementary school aged child also has PVL. His response to J's diagnosis echoed our response, "That's so.. AWES... ... I mean, that's really sad."  No, little boy, that IS so awesome. We are not alone. In fact, right in front of us is a thriving boy who is showing us life down the road. His parents call PVL a hidden disability. He still gets help for his PVL, still has some therapy, but you don't know when you meet him that there's anything different about the way his brain works. His parents have no doubt that he will be able to do nearly anything he sets his mind to - it just may take a little more effort than the average person.

All in all, this is fantastic news. No brain surgery needed. We will just work hard,  and we will overcome. This is not a disability, this is him daily proving his ability.

Here's a song that JAM jammed out to today (he is quite a good dancer). It echos my thoughts on all this.

--------------------------


**Side note on the CP comment above: Cerebral palsy often comes with PVL, the two are not mutually exclusive. A child with PVL is at higher risk for both CP and seizures. My comments here are not meant to confuse. It just appears at this point that J's issues are nearly all PVL related, and not CP related. We doubt he will be diagnosed with CP, but he is still too young to know for sure.

Yesterday was fun, tomorrow is big

This has been a crazy month -- full of packing and cleaning one house, unpacking in a new home, packing up my office at work and preparing to train my replacement, all peppered with neurology, ophthalmology, and neurosurgery appointments. In the midst of all this, J battled an ear infection, we coordinated new early intervention services, I spent hours on the phone with various specialists, we talked about possible seizures and sleep apnea, J lost weight (again), we transferred records, and I've been attending weekly training sessions at the NICU learning how to be a good parent-to-parent volunteer.

Yesterday was a fun break from it all. We got to say "thank you" to all the anonymous blood donors that helped save J's life. See? We got to be on TV.

As exciting as that is, tomorrow will be a bigger day. Tomorrow is a day I've been waiting for for months.

Tomorrow J is finally scoped. From both ends. Biopsies will be taken. His gut will be analyzed. In the end, this means that we might start to have answers. 

Jonathan hasn't gained a pound since I stopped giving him breast milk in late October/early November. He hasn't kept even a half a pound extra on him since then. About six weeks ago, after a few fantastic weeks where he consumed more calories than necessary for a kid his size and age, wasn't sick (for a change) and was on a new med to help with bacterial overgrowth in his gut, I was actually excited for his weigh-in. I was sure he'd FINALLY be 17 pounds. But instead? He'd lost weight. Back to 16 pounds 2 ounces. And I crumpled. A few weeks later, after the aforementioned ear infection and accompanying nausea, he was back down to 16 pounds even.

He's gained many inches, but no weight. His energy level during the day bounces about and causes me worry. I can count his ribs. We don't want this for him any more. We want answers. Tomorrow we might get a few. We want our kiddo to be a thriving child.

I am holding my breath in part because I know how this goes, and I know that despite the best intentions of everyone on Team JAM, this month may come and go and we may be no closer to actionable answers. And I know that if that happens, I will crumple into a heap in the corner. It's so hard to see him like this.

Jonathan 4 months ago.
(We won't share a picture of now, it's worse.)

Grow Johnny Grow

We met with the developmental pediatrician's nurse again today.  The one we normally see wasn't in, so this nurse was filling in. JAM had lost half a pound since his last weigh-in. He still hasn't gained since November.

She sat and looked at us for a long time, thought out loud, and was in essence fantastic.  We re-explored together all sorts of options. Pancreatic insufficiency. Celiacs. Nephrological issues. During the visit Jonathan lustily ate 8 oz of high calorie toddler formula, proving to her that he really WAS taking in food and really DID have hunger cues (something that isn't a given for micropreemies).

She is as baffled as our previous nurse. She pulled in the developmental doctor. They discussed JAM's case. She discussed what GI was doing with him. We rejoiced that he continues to grow vertically and his head is on the curve, but we mourned at how his BMI has utterly tanked since last fall.  At this rate he'll be at olympic athlete status for BMI by next fall -- only without the muscles.  Not right for a toddler.

We discussed absorption issues -- our best guess at this point. He's borderline in some areas. She confirmed that he didn't lose enough of his gut during his bout of NEC to qualify for short gut issues.  She also confirmed that he was adorable.  We knew that one.  We left with a new formula and no real answers. She is on the case, though, and really wants to get to the bottom of this. We're grateful. We want this mystery solved, too.

--------------------------------------------

In other news, changes are in the air. The weather is warmer, I begin training for NICU volunteering tomorrow, and we move within the week. So much is happening. I cannot wait until we are settled and I can start writing in a more disciplined fashion again.

-------------------------------------------

In other-other news, even if I haven't managed writing, I have kept reading, and now that I've gotten feedly to work on my phone, much of that reading has been other blogs.. I guess I've used commenting on other people's blogs as a stand-in for writing on my own during this busy season, especially when the post hits home, like this one. Jack's mom liked my comment on this blog post so much that she turned it into a stand-alone blog post. Thank you. (Life with Jack is one of my favorite micro-preemie blogs. If you haven't checked it out, do. He's a few years older than JAM which has always helped me see the path forward.)

------------------------------------------

Speaking of following people, if you're not following me on facebook yet, "like" my author's page, here. (www.facebook.com/momofa23weeker)

Welcome April? - A peek at life now.

I cannot believe March is over. In many ways this year it was nasty and gross, the sort of month you are ready to shake off. The showers of spring have finally hit and are washing away the ugly grime.

We are moving. We think we've sold our house. We were sick with bronchitis and pneumonia basically all month. This was scarier for JAM than for the rest of us. We were close to hospitalization and oxygen, but we dodged it, gratefully. His albuterol inhaler was well-used this past month. And then, as if the weather were commiserating with us, winter seemed to refuse to end.

April has made a huge difference. We are all a bit more optimistic. Days have been spent out of doors, and while we still have sniffles and are still in transition, the world just looks brighter.

In March we visited the GI doctor. She gave us some magic juice that was specially designed to realign the delicate flora of his GI. She called the juice an antibiotic. She also asked us to give him probiotic. We did so faithfully. Some of the pain went away. The overall problems abated for about a week and then returned. Less pain, but same symptoms.

So this week I contacted his GI doctor and his nutritionist again. Since hitting the screw with a hammer didn't seem to nail anything in last time, we're going to try again, only hit harder. More antibiotic - but for a full month instead of a few weeks. His gut will heal, gosh darn it. We will make it so.

The nutritionist offered a variety of really yummy looking high-calorie smoothies. I told her I'd be sure to try the recipes "to make sure they tasted okay" before handing them over to him. She has been in conversation with an NP and a developmental pediatrician, all of whom are stumped about my little guy. Keep up with the zinc supplements. Keep up with the high calorie foods. And maybe, maybe he might someday grow.

Warm weather finally allowed us to get out. Here's JAM
with his sister at a sculpture garden, shoeing a gigantic horse
with great effort.
See, I told ya he was still so small.

He's been hanging out at 15-16 pounds since October.  He's still comfortable in his 9 month clothing. (Yep, he's 20 months old. Yes, that means kids a full year younger than him are in the same size clothing as him.)

Just because he's little, though, doesn't mean he doesn't LOOK his age. Over the past month he's decided that he's a toddler now.

He's crawling and climbing and giggling and exploring. He talks, though we don't often understand him, and he has a will that is showing more and more each day with insistent whines that we don't always know how to interpret. He loves to hold our hands and walk around the living room. We did this in a waiting room one day, and someone commented that he looked too little to be walking around like that. He's actually behind in gross-motor by about six months. Had he been a "normal" size for his age, they'd be wondering why he wasn't walking on his own yet.  I guess there are benefits to being little. You look brilliant for your size. Brilliance. That's what April will be about. Sunshine.

Welcome, spring.

Paradox.

Imperfect. That’s what we are. That’s how we were born.

But then also in the image of God.

Redeemed, but still sinning. Already, but not yet.

This is the tension we live with, the seeming contradiction of our souls, our bodies, our beings.

And it applies even to the smallest baby.

I know, because I had the smallest baby. Okay, not THE smallest, but certainly the smallest my doctor had delivered.

I watched him struggle with his imperfections. His lungs could not breath, his kidneys failed twice, his heart failed to recognize that it was out of the womb and change the way the blood flowed. He needed heart surgery. Then he needed surgery again as his gut perforated. It had begun to die off, unable to digest food. A month later his retinas were on the verge of detachment. This was not a perfect being, it was a being slowly unraveling, held to this earth only by a thin thread and the wires and tubes of medical machinery.

But yet he was strikingly perfect, even at just over one pound. His hands, his sliver-sized translucent knuckles. When he was born he had perfect little fingers with fingernails. FINGERNAILS! How is that possible on so tiny a being?

Imperfect, but in the image of God.

A paradox. Like the world we live in. I don't understand.

How do I explain sin? I cannot. I wish it didn’t exist. How do I explain why my baby lived when others died? I cannot. I wish children did not die.

But I read this – the death? It’s not how God intended. Just as we were not created to give in to our selfish desires, or be victims of others’ sin, we were also not created to die tragic early deaths. We’re in the already – Christ has come and we are saved – and we are in the not-yet, living in a fallen and broken world where sin and death still plague us.

“See, I will create new heavens and a new earth. The former things will not be remembered, nor will they come to mind. But be glad and rejoice forever in what I will create, for I will create Jerusalem to be a delight and its people a joy. I will rejoice over Jerusalem and take delight in my people; the sound of weeping and of crying will be heard in it no more. Never again will there be in it an infant who lives but a few days.”  - Isaiah 65:17-20

Never again.

I cannot tell you how much comfort verse 20 gives me. God knows that infants die, and he also thinks it isn't right.It isn't fair.

My son lives. This is by the amazing work of a team of medical professionals. This is also by God’s grace, giving life to kidneys that had failed when the nephrologist told us nothing more could be done, giving wisdom to doctors on when to perform PDA surgery, giving skilled steady hands to JAM’s surgeon for the many surgeries performed. Miraculous. The "already."

But there is still death. And sorrow.

We are there, with a good God who works miracles in small beings. We have a savior who has conquered death by death.

And we are not there, sin still reigns. It still impacts us and rocks us to the core. But somehow grace is still poured down, even in the midst of the scorching heat of sin and death, there are drops of rain, and dew collects on grasses.

I do not understand. It is a contradiction, a paradox I cannot unravel.

Already, but not yet.

This is what a one (and a quarter) pound baby taught me.

What is a miracle?

We are still in the throws of preparing to move. It's just enough of a messy process that we hope this is it - both that our home has finally sold (we have an offer on the table) AND that we don't ever.have.to.do.this.again. [Punctuation placed liberally to tell you just.how.done.with.this.I.feel.]

Jonathan is doing very well. He is a trickster and today's trick was standing, by himself, for one whole second. He was so proud.  Maybe he'll be walking by himself before this last season of quarantine is over. Maybe we'll surprise everyone when we reemerge from our winter hibernation.

In the mean time, take a look at my post on the Hand to Hold blog, where I discuss coming to term with the term "miracle" that is used liberally by doctors and friends alike to describe my son. You might be surprised, but I don't like the term. Read here to find out why.

It doesn't take a preemie to be given "a miracle."
You're just more aware of what a miracle life is when you've got a preemie.

GI doc

Tomorrow we add a GI specialist to JAM's team. It's about time. We hope to find out why he has vitamin deficiencies, isn't growing well, and has severe bowel pain almost daily.  More to come, I'm sure.

In the mean time, JAM is enjoying our house. In addition to adding a GI specialist, tomorrow is also the day our house goes on the market. Since the floors are swept and polished to perfection, we've taken down anything to bar his way and are allowing him to explore almost the whole house.  He's enjoyed it immensely and is so proud of his crawling ability. He's getting really good at getting everywhere. He even managed to wedge himself under our bed, a feat that was just traumatic enough for him that it is something I hope he won't repeat.

We've even practiced going up and down stairs. He doesn't quite have the strength to do it alone yet, but he's getting the hang of it and is so proud of himself.

His lungs suffered for a few days from either a cold or our cleaning spree (likely both) but he's back on track now.