PAM FOD #6 - Preemie complications: Retinopathy of Prematurity

Prematurity Awareness Month
Fact of the Day
Day 6

Q: How does prematurity impact one's eyes? (Part 2)
A: The world outside the womb is not ideal for eye growth. Inside the womb blood oxygen levels and blood pressure are more or less naturally kept at a good spot for the growth of the blood vessels in the eye. Outside the womb, other factors, including too much oxygen in one's blood cause the vessels to grow incorrectly out from the retina, leading to extra pulling on the retina and possible detachment of that thin but crucial film on the back of the eye. This, in turn, can lead to blindness.  (Retinopathy of Prematurity, likely from too much oxygen after birth, is what caused famous musician Stevie Wonder to go blind.)

Some factors that can lead to incorrect vessel growth in premature babies:

• Breathing issues - apnea, low or high blood oxygen levels, high carbon dioxide levels in the blood ("poor blood gasses")
• Heart issues - anything that makes blood flow inefficient or too slow.
• Transfusions - as I understand it this is because they create more blood pressure in a relatively short amount of time

Unfortunately, for a preterm baby, many of these are unavoidable. I stopped counting how many blood transfusions JAM had gotten after it reached half a dozen. He had heart issues. He had breathing issues. When you're born that early, your body isn't ready for all this baby stuff. The eyes took the fall.

Despite doctors efforts to control how much oxygen was in his blood and monitor blood gasses (the CO2 output or "good exhale" as seen by what remained in his blood), JAM developed retinopathy of prematurity.

There are five levels. You can read about them here. Level one is best, level five is blind (total retinal detachment).  If you hit level three, they will probably recommend laser eye surgery to stop you from getting to level four or five. The surgery zaps protein in the eye in the sections farthest from the retina to help the vessels grow into the outer section of the eye. Gnarly, thick vessels that don't grow into the full eye are cause for concern.

JAM not only got to stage three (in both eyes) - he got there alarmingly fast.  They give that a special name. It's something lovely that they call "Aggressive posterior ROP" or "Rush Disease," and best as I can tell, it's like Plus Disease (which is bad ROP), but a bit worse.

Do you like pictures?  Apparently they made one of JAM's primary NICU nurses a bit queasy, so I won't post any of his here, but you can find blood vessel pictures for ROP + and Rush Disease here.

In normal ROP that has gotten to stage three, laser therapy prevents blindness in 80 percent of patients.  For people like Jonathan, it works only about 65 percent of the time.

Thankfully, for Jonathan it worked, and his ROP slowly regressed until now he is not considered an ROP patient any more, but a CROP (or "has scarring from ROP") patient.

The surgery potentially saves the central vision, but at the expense of the periphery vision. Sorry, Jonathan will never be a hockey player. 

Often, for whatever reason, babies born severely premature also develop nearsightedness. In our case JAM looked good for the first nine months, and then at his appointment at just over a year tested severely nearsighted.  The doctor thinks it's because of all the scarring and abnormal development in our son's eyes. 

"He has A LOT of scars in here!" the doctor said at our last eye appointment, "a LOT."

But you know what?  That's the great thing. A year ago we were mentally preparing for a baby who would go blind. Prior to laser surgery, he would have gone blind.  We'll take the scarring. And a pair of glasses with that, too, if we can.

Yes, my mom totally made this the "FOD" so
that she could post this cute picture of me.

Today he got his first pair of glasses.  So as of about 11 am, the world began gushing over a really really cute baby in his brand new glasses. Jonathan is steadily getting used to being able to see more than two feet in front of himself. It wasn't love at first sight - it took him a bit of time to get used to the glasses. He celebrated by falling asleep ten minutes after we put them on. The visual stimulation was too much.

Sometimes it catches me off guard when he looks into my soul, connects, and smiles. "I didn't think you'd be able to do that, little buddy!"  And now he can do that from across the room.

Yet again science at work, saving babies. Thanks, scientists and doctors everywhere!

The Philosophy of Trees -- Retinopathy of Prematurity and Perception

I don't remember much about my undergraduate philosophy course, but I remember that my instructor had been dreadful.  He had assigned a half a dozen books, and I had been excited for the class. I loved philosophy. I loved thinking about what was real or false or concepts of how the world worked.

My classmates didn't feel the same way. They were taking the class because they had to. And they quickly discovered that our instructor was very easily brought along with them on unnecessary tangents. And tangents meant less material to learn and less would be on the test, because our instructor did not live by his own syllabus.

So we started with Rene Descarte's Meditations. A good place to start. And it was the only book we really read.  By the second or third meditation, our class had discovered the aforementioned secret, and they were all about continuing class discussions on topics long after they were dead.

Don't have a picture to illustrate this, so here's a picture
of JAM with a famous philosopher.
Bonus points if you can recognize the philosopher.
(Don't worry, not the philosopher who taught me.)

And so we spent somewhere between three and five class periods -- that's OVER A WEEK -- on whether the color I see as red is the same as the color you see as red, or if what I see as red you would perceive as green or blue or purple.

It was dumb.

I would not be majoring in philosophy.

But a year ago I decided I knew the answer to this ridiculous question from a decade earlier.

Yes. Yes. The color orange you see is different from the color orange I see. I know this, because the color orange I see is different from the color orange I see. At least, it was that afternoon. I was driving home from the NICU. The trees had all turned into a fantastic display of reds, oranges, greens and deep purples and browns. The landscape was a fireworks display. And the display had gotten brighter.  It wasn't because things were sunny.  On the contrary, things were back to uncertain. But these colors -- they were so alive. More alive because I was noticing them. I didn't take sight for granted any more. I wondered if my son would ever be able to indulge in the autumnal feast of colors.

Jonathan a year ago, recovering from eye surgery.

My perception of the world had changed.  And it wasn't just the leaves on the trees.  When the leaves fell off, the very trunks of the trees became not wood, but living vessels.  Some were ROP trees, like the thick gnarly wood of crab apple and oak trees.  Others, like the tall slender lines of the elm trees or the young maple tree, represented the vessels in the eye of normal babies, full term babies.  Those trees knew how to grow into the sky.  They grew straight, with many new twigs coming off each branch. The apple trees tried hard but couldn't find the right path. I pictured them pulling the weight of the earth up with them, detaching themselves because they couldn't grow, couldn't push to the sun.  They'd never be able to see.  You could tell by the lower branches. Dead, if not trimmed. Dying because they saw only shade.

Jonathan was now post-op from his eye surgery. The surgeon had said he'd done a very thorough job, zapping as much of the protein in the sky of Jonathan's eyes so that the branches of the vessels could grow tall and healthy, filling his eye and keeping his retina firmly planted at the base of it all.  They'd be dilating his eyes at least two times a week for a few weeks to see how those vessels grew, to look out for stage four or five of ROP+.

I walked across our beautifully wooded campus at work. I again admired the bright colors of the leaves. Maple leaves are my favorite. But I also loved the leaves that turned a deep purple. My foot crunched on a dried brown oak leaf, and I smiled. This. This was a gift. The trees don't just flame, they crunch.

My son may never see a burning bush. He may never see a hill side dotted with reds and greens and yellows and browns as an entire forest erupts into a symphony of color.

But he will crunch. He will feel the leaf under foot or in his hand. And he will wave that hand and crunch those leaves, and maybe even toss them into his sister's hair.  He's going to live now, you see. I was becoming more and more sure of it. And if he lived, he was going to live a GOOD life. I would see to it.  It would be colorful, even if he would not see color.

The first eye exam post-op was neither positive nor negative. The ROP had not reversed. But it hadn't gotten worse either.  We kept waiting.

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

Spoiler alert.

This picture was taken a few weeks ago.  Today Jonathan can both see the splendor and feel the crunch of the leaves. He's nearsighted and he has limited peripheral vision from the scarring of laser eye surgery, but his central vision was preserved.

Day 76: An unexpected surgery - Rush ROP+

October 1, 2012.  I started the morning pumping. While I pumped, I posted a few cute pictures of JAM on his care page site. I talked about how great he'd done the night before with breathing on his own and how very proud we were of him.

I then went off to work. I walked in so that Steve could take Mimi to daycare and then take the car in to the hospital and spend the morning with Jonathan. I hoped he'd be able to see his face free of CPAP, but doubted they'd remove the breathing support except for during the night cares.

I'd only been back to work for about three weeks. I was still working hard to get through the paperwork that had piled up from the summer of work I'd lost, but overall work was going well. I'd settled into a new routine, and we were making it work.

About a half an hour after I sat down at my desk, the NICU called.

"Your son has Rush ROP" the doctor said, "It's a fast progressing version of ROP, and so we want to do surgery now, today, before it gets worse."

I found out later that it's a rare form of Plus Disease, and that in this type of ROP, a child can go from stage two to stage four or five very quickly, skipping the in-between phases and becoming legally blind overnight.  Jonathan had stage two ROP in one eye, and stage three in the other.

"Steve's on his way in," I said, "He'll be there within the half hour."

I called Steve and filled him in, and then sat back at my desk and stared mindlessly at my monitor.

Steve was already driving in. If I had him pick me up, I'd be delaying the time between now and when a parent would be there. Jonathan needed us, but he only needed one of us.

And then there were the statistics. The doctor had talked us through ROP a few days earlier, he'd said that if it progressed they could do laser surgery on the eyes, and that in 80% of cases that would prevent retinal detachment.  This morning he'd changed the statistics. In babies with Rush ROP, the chances went down. Only two thirds had successfully avoided blindness with surgery. In one third of the cases, surgery was not enough.

If surgery didn't take, he'd be on his way to a bigger city, a different NICU. There he'd spend two to six weeks undergoing retinal reattachment procedures to preserve some sense of sight, even if it was only the ability to see some movement or color. He'd be blind, but some vision would be retained.

We wouldn't know right away what path he'd take.

I stared at my monitor and tried to image it.  Maybe I could work Tuesday through Thursday and take my unpaid maternity leave throughout the next two months, taking Monday and Friday off and spending the weekend in the NICU far away. I couldn't leave him alone, but I couldn't quit, and I couldn't be that far away from my girls for so long.

I calculated the hours. How many weeks would I be able to make this work?

There was no way I could take the day off to sit in a room while he had surgery. I didn't know how many surgeries were in his future.  This was the fourth in just over two months of life. Steve could be here this time. I'd have to save my days for a time when Steve wasn't available.

I contacted the care network at my church.  They'd reached out to me several times asking how they could help, and I'd never been able to find a good way.  "If this happens," I asked, "Would the church consider helping pay for the hotel or hostel stay in the city far away?" I had to figure it out. How could we make it work, with the extra gas costs and the extra expense of hotel stays every weekend?  They responded quickly. "Of course," they said. And they were praying.

I had to figure it out, because one more thing -- one more dreadful thing -- and I thought I would snap.

"If I prepare for the worse," I told a friend, "and his eyesight is preserved, then I haven't lost anything.  If I don't prepare, and he ends up being rushed to the city far away, I don't think I could take it."

And I wasn't kidding.

I considered packing a bag, just so I was ready to go.

I did all this to keep myself moving, because frankly, for the first time, I was ready to throw in the towel. This was hard. Too hard. I wanted to crumble into a ball on the floor and not move. I wanted the world to go away.

But there was no towel to throw in. Just a baby. And he needed me. He needed us. So we kept running.  The race was long and hard and every bone in our soul hurt, but there was no other choice.

I know it seems silly.  Compared to everything else he'd undergone, this was easy. This was blindness, not death. But it's like when you turn a corner, expecting to see the finish line, and only see five more miles before the next turn, without knowing what is beyond that. We were exhausted.

My mom suggested that JAM always took the road less traveled, and so she'd pray that he'd do the same in this case.

I told her she was wrong in her math. Two-thirds chance he'd be okay. Pray for the road MORE traveled.  Pray that he'd be okay.  We were ready to be done beating the odds.  We wanted to move into just being.

This post's content has not yet been edited. If there are errors, they will likely be corrected (and pictures added) in the next day or so.

Retinopathy of Prematurity & believing he would live.

A year ago, on September 25, Jonathan was diagnosed with stage two and three Retnopothy of Prematurity (ROP). Suddenly all his care centers around preserving his eyesight. His red blood cells were low. Normally they'd give him more blood. Now they hope he's strong enough to make his own red blood cells because more blood parts means a change in blood pressure. A change in blood pressure might make worse his ROP.  Without the red blood cells, Jonathan looked pale and weak. Since red blood carries oxygen, he required more assistance with his CPAP.  Whereas his pressure and oxygenation needs had been steadily declining, they now began to rise again.

At home Steve and I both battle infections. Mine is so severe that they put me back on an antibiotic that Jonathan can't have, which means I was back to "pumping and dumping" again. On top of this, I'd broken out in stress-induced eczema. I had no idea such a thing existed! It seems unfair, doesn't it? Just as I'd thought I'd gotten my stuff together, we'd made a "new normal" and weren't even accepting food any more -- just as I thought things were getting better, my body decides to tell me differently. Hives covered my legs and arms and torso. Thick, raised hives.  I was put on predinsone to calm it. To me this particular steroid feels a bit like a freight train running through my veins. I was jittery. I was itchy. My body was rebelling. And then, on top of my skin deciding I'd had enough, I got sick. And then on top of that, Steve got whatever I was fighting.

Neither of us could go visit Jonathan. Our "every day he'll see one of us" rule was broken. Thankfully not for more than a day or two, and then I started feeling better, but it was broken nonetheless. We didn't want to chance him getting our bug. Not when he was finally doing okay. I didn't want to see a vent on him again. Never. Never again.

On our caring bridge site I write "In more than one way, we are all investing in the future, even if it means temporary discouragement." On my facebook page I wrote "So tired. Bone tired."

But in a way, it was okay. The retinopathy of prematurity and our illnesses. We were only worried about Jonathan's eyesight. We could tweak the blood parts he received to help preserve his eyes. We were only worried about his eyesight. And, while I missed him, I knew I'd be seeing him again soon. As soon as I kicked the nasty cold. We'd have many more days.

I believed he would live.

At home I started to think about nannies. If he came home, what would we do while I was at work? The daycare we'd planned to use, the one that had saved a spot for him, it was out of the question. Too many germs for a baby like Jonathan. If Jonathan came home...

In a fit of spontaneous optimism, the girls and I set up a baby registry. Not that we had need of one, I wouldn't be having a shower - this was the third child after all, and that's not how things are done - but I thought it'd be nice to put one together, because that's what people do in their third trimester. And here I was, third trimester.

I became frustrated that I couldn't register for a baby who had already been born. I put in his due date, but only one friend could find him by that date. So, in the end, our registry may have been not-so-useful for gift giving, but it was TERRIBLY useful for our sense of hope. I'd be having this baby. Really, truly, having and holding my two month old. He might be six months old or more before I could do it, but I'd be bringing him home. The giraffe prints and cuddly pajamas and mahogany cribs on the floor of the baby store told us so. And at home, he'd teethe. He'd need bottles. He'd need a cozy blanket or cloth carrier. He'd need a high chair, since I'd given away the girls'. Ella and Mimi loved the gun that they could point at things they thought Jonathan would want. (I limited them to two baby toys.) I enjoyed the hope, the chance to finally dream.

He'd be coming home.