Nathan is a Rock Star! - Part 2

Nathan was delivered by emergency c-section at 3lbs 1oz and a mere 14 inches. He was rushed off to the NICU to be tested and observed. The doctors had to make sure that his brain was able to sustain his body functioning...for a normal baby, that would mean vital organs functioning properly, but for a preemie, that also meant simply being able to breathe on his own.

He was breathing. Amazingly, he had apgar scores of 9 and 8 and gave a hearty yell in objection to being disturbed from his warm cocoon. That was a huge first hurdle. The next big concerns were the size of the cyst, the formation/condition of his brain, and his heartrate. They also needed to monitor his breathing just to make sure he was doing a good job...and he did have tykipnia. (He was breathing quickly and shallowly.) He had tachycardia too. (Rapid heart rate.)

To make a very long story short. In the course of Nate's NICU stay. Our doctors were consistently surprised at how well Nate was doing. At one point, our lead doctor said that Nate was "doing everything that he should be doing, and he's not doing anything that he shouldn't be doing." He also said that "if we didn't know already that there was a dandy walker diagnosis, we'd probably never have found out, because he's not showing any signs of the normal deficiencies and abnormalities."

Now I'm a believer in the power of prayer, and I have to say that at that moment I felt that God had smiled on me.

Even though he was doing well, they did an ultrasound and CT scans of his head and it showed that the cyst was SIGNIFICANT. When I saw the images, it looked to my untrained eyes like half of his brain was missing. The doctor said that it was fully intact, though, and that the corpus callosum was also intact (which meant that he would likely be able to have at least SOME motor skills.) But there was a great deal of fluid filled space that was pushing his brain into a much smaller area of his skull than it really needed. There was concern that the pressure being exerted on his brain would mean that he had brain damage.

This pressure did cause his ventricles to dilate (this is the condition called hydrocephalus) and he ended up having a shunt placed as the treatment. Before the surgery, Nate's head was oversized and they were measuring it consistently to track the cyst growth. After the surgery was done, there was an IMMEDIATE improvement in the shape and size that makes him look completely normal now. (Even though it was difficult to go through, my online research gave me optimism about this because the little bit of information that I had found showed that children who had shunts placed as treatment for dandy walker had positive outcomes. There were even reports of normal development. After the shunt is placed, their bodies even catch up to their oversized heads to make them look even MORE normal.) Our neurosurgeon was amazing and also did a lot to ease my fears. The first time that he saw Nathan, he cradled him against his chest and cooed to him. How amazing is THAT from a neurosurgeon?!?

So to bring you current. Nate is going on 7 months old now and has had bilateral inguinal hernias repaired. He's had neurosurgery for the shunt placement. His rapid breathing and heartrate turned out to be a PDA which is an open valve leading to his heart that is diverting blood to his lungs. It needs to be closed with a catheterization procedure this month. (This valve is a natural part of prenatal blood flow. It's necessary when mom is doing all the work, but this valve is supposed to close at birth. As a result, Nate is currently in congestive heart failure.) And he has another condition called hypospadius which is a urology issue that will require at least 2 surgeries to repair. (This is not going to happen until next year.)

From that description, you'd think that he's a pretty sick little guy. But he's developing beautifully and is actually showing no outward signs of having been through so much or of having so many issues to deal with going forward. The neurosurgery left a horseshoe shaped scar behind his ear and you can see a lump outlining the shunt. He also has a scar on his abdomen where the tube to drain the excess brain fluid was positioned. The hernia repair scars aren't visible at all.

He's currently receiving physical therapy to make sure that he hits developmental milestones, but my little rock star is smiling at me. He's babbling ma-ma-ma-ma when he sees me. He's trying to crawl. He's chewing on everything he can get his hands on. He's laughing with his whole body when I tickle his tummy. He's absolutely beautiful and I'm certain now that he's going to be ok. It'll take a lot of hard work, and it's taken a lot of hard work to get him to THIS point. But like I said, he's beating the odds.

I have so much more to say about the doctors and his care. I'll have to split it into little pieces to tell the whole story.

Nathan is a Rock Star! - Part 1

Nathan Andrew Shirley is my 4 month old son who was born with Dandy Walker Variant and he's beating the odds.

I found out about his diagnosis during an anatomy scan ultrasound at one of my many prenatal visits. My doctors were immediately on the lookout for additional problems since there is a laundry list of associated disorders. I found out quickly that the more physical or genetic problems they found, the worse their outlook was for his outcome after birth.

The ultrasound had revealed that there was a small cyst forming at the base of Nate's brain, so the next step was to visually examine him with a more in-depth ultrasound to see if there were any physical defects developing.

I managed not to worry myself sick during this process and was very relieved to find out that his vital organs were all functioning properly and that he had no additional congenital defects.

We weren't finished there, though. The next step was to look for a possible cause or additional underlying genetic/chromosomal abnormalities like Trisomy 18 or Down Syndrome. I DID manage to worry myself sick during this part, but I said my prayers and managed to find my way to the designated hospital room for an amniocentesis.

Waiting for the results was excruciating, but I was AGAIN extremely relieved to find out that there were no chromosomal abnormalities.

I was fortunate enough to be able to get a more in-depth level of chromosome screening done from the same amnio fluid. This one was called called a micro-array. And for the third time, I got the best possible outcome of no abnormalities observed.

So after exhausting the medical testing that was available (I did this to help my doctors plan for his care after birth. Knowing his condition in detail helped them to know how aggressively they could and should respond to any possible concern without causing an unexpected problem due to an unkonwn condition.) I was prepared for the reality of what was to come while still optimistic about the outcome because of how many things we had ruled out.

By then, I knew that we were dealing with an isolated occurance of the variant which meant that Nathan had a chance at the best possible outcome.

He still had to get here, though, so we were watched very closely. And unfortunately, he wasn't growing as fast as they wanted. (Although we had ruled out everything major, we did also have a two-vessel umbilical cord that was not supplying enough nutrition.)

We made it to 31 weeks before the doctors started getting concerned that he was too small. By this time, the cyst had grown (as expected) and the doctors were also concerned that there may be neurological deficiencies because of the pressure that the cyst was exerting on his brain that might have been keeping his body from performing even the simple functions needed to sustain itself.

I was EXTREMELY stressed. And that was just making things worse.

I got my first steroid shot that week, and had to have it repeated the next day. That was on a Tuesday and they wanted me to return that Friday so that they could take another look at Nate (and they said to plan on getting checked out twice a week from that point on.)

By early Friday morning, something felt very wrong and I started getting sick.

Nathan was born at 32 weeks (8 weeks early) on December 12, 2008.