Wednesday, June 4, 2014

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.


  1. HI Laura.
    Could I check with you if there was any sign of abnormalities on the ultrasound in the NICU or did the ventrical enlargement only occur later? My little 23.5 weeker (now nearly 7 months corrected) had no brain bleeds and "mildly dilated ventricals" since 3 weeks old and they are still mildly enlarged. We have not had an MRI for any reason as she has been quite on track with her development, but this does make me worry that we could also have a PVL diagnosis in the future.

  2. Hi -- Sounds like our stories are similar. My son had maybe one tiny brain bleed in the NICU (only caught on the final MRI before departure and was at that point resolved) -- and his ventricles were shown to be on the large side of normal, so maybe it was already visible, but well within a normal range such that we weren't too concerned about brain health when he was discharged.

    If I were you I wouldn't worry much about it unless you find that she's taking a long time to acquire skills. We knew that there was an issue starting around the time he was 9 months actual, as he was behind on gross motor. We chalked it up to multiple gut surgeries at first (low core strength). We noticed then around 12 months actual that it took him multiple repetitions of the same action before he could do the action on his own, that he wouldn't naturally catch himself, that his core wouldn't rotate until after he'd had therapy to help him learn how to rotate, etc. So we started seeing visible signs of it. We knew it was a neuroprocessing issue, and that it resolved with practice long before we knew that it stemmed from his white brain matter.

    From the sounds of it, you have nothing to worry about at this point! She sounds like she's doing really really well. If you see outward signs of it, you could chat with your developmental pediatrician about seeing neurology or discussing CP or PVL -- but if there are no outward signs, then even if the MRI showed enlarged ventricles, they still wouldn't diagnose PVL. Some people's ventricles just are a little more dialated, with no outward signs of anything, and it's just their own normal.

    I hope that helps!


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