Life goes on….Zachary’s story

 

 

1 in 4,  A  25% chance, with each pregnancy that you might pass on that genetic defect.

Why can’t my odds of winning the lottery be that good?

So we start again. Ammniocenticis at 16 weeks.

Waiting.

Phone call from genetic counselor. She still wouldn’t discuss the results over the phone, so I had to make an appointment for the following week. I spent that week trying to come up with reasons she needed to see me in person if the news was good.

Yeah, I still couldn’t come up with any. 

Waiting.

Finally the day of the appointment, I drove the hour and a half to the hospital, crying the entire way there.

Again, simple, direct, right to the point – the test confirms a diagnosis of citrullinemia…

*crashing….darkness……NO!.. I can do this….I am strong and so are my boys…..*

I thanked her, told her I would contact the genetic doctor at Cincinnati Children’s myself.

 They know us very well in the genetics department.

Life goes on.

Zachary William Dale was born via scheduled c-section on September 26, 2007.  

 They whisked him away and I didn’t get to see him for 3 days. No ice storm this time, I just really needed that extra day of rest. Zachary did well. His initial ammonia level was 56.  He responded well to his IV meds. He tolerated his low protein formula and oral meds, although he did require an NG tube for the first month of his life. Zachary spent 10 days in the RCNIC.  Again, we were sent home with a very strict feeding schedule. 3 oz every 3 hours. His sodium phenylacetate and arginine were mixed into his required formula on a daily basis.  Zachary had issues with sucking and with forming a good seal around the nipple. He saw a speech therapist for the first month, at the end of which he was no longer having  problems with his feeding.

When he reached 5 kilos, we scheduled his transplant evaluation. He was 2 1/2 months old. This time around our team was thoroughly convinced that he should be transplanted as soon as possible They were no longer on the fence about whether or not to wait. He was approved and placed on the waiting list.

That day was December 19, 2007.

We received the call on Sunday, December 23, 2007.  A donor had been found. Drop what you’re doing and get down to the hospital. What?!?!?!?  I know, right? Did you catch that? 4 days! We waited exactly 4 days.

Zachary had his surgery on Christmas eve, 2007. He was 2 days shy of his 3 month birthday.

Zachary’s course after transplant was very different than Jonathan’s. Zachary developed a hepatic artery thrombosis 3 days post transplant. In English?  He had a clot smack dab in the middle of  his liver.  The hepatic artery brings blood to the bile ducts at the edges of the liver. It is a one way street – blood flows in through the hepatic artery, up and out to the bile ducts. If the bile ducts do not receive blood, they wither and die. They do not grow back. Without bile ducts to carry bile to your belly – you can’t digest food.  Bad things happen if you can’t digest your food. So he was put on a constant drip of heparin, to help thin his blood in the hopes that the clot would dissolve. The liver is an amazing organ, it can regenerate itself. Did you know that? It can. And while the clot never dissolved, the vessels grew around the clot, forming new pathways to deliver blood to the bile ducts.

Of course this took time. Time in which the bile ducts were deprived of blood and started to wither. So we waited.

We spent 3 weeks at CCHMC.  Jacob and Joanthan spent 3 weeks going back and forth between our house and Grandma’s house – Jacob was in preschool 3 days a week – I still  think they had more fun.

Life goes on.

About 3 weeks after discharge, Zachary developed a fever. He was lethargic, he vomited, his vitals were unstable. I called 911. They came and took him to our local hospital, within an hour he was being transported via helicopter to CCHMC.

I drove the hour and a half to the hospital,  crying the entire way there.

He was delivered to the ICU.  An ultrasound showed a bile duct that was dialated. He needed surgery to try and open it.  They scheduled it for the next day.  Interventional Radiology. The ‘plumbers’ of the body. Percutaneous transhepatic cholangiogram, PTC for short.  Their job was to place a drain in the bile duct. They insert a small catheter into his bile duct, drain any yucky gunk that was clogging it up and hope the bile duct heals ‘open’. He was put on IV antibiotics (he had a central line so I could administer IV antibiotics at home) and sent home. He had this first drain in for 1 month.

Wait.

About 2 weeks after they pulled the first drain, he had white poop. Really is looked like he had pooped out liquid chalk. Fortunately we were at clinic with the GI doc when he had the acalic stool. They admitted him. Another PTC was done. This time they found 2 dialated bile ducts. They inserted 2 drains. He was put on IV antibiotics and sent home.

They know us very well in Interventional Radiology. 

Within a week, we were back for yet another PTC. They found 2 more dialated bile ducts. Bringing his total drain placement to 4.  Our IR doctor peformed a miracle. Zachary was only 6 months old, to have 4 drains placed in his tiny liver was nothing short of amazing. But it had to be. If the drains did not work – he would have to be placed on the waiting list for a 2nd liver transplant.  

*crashing…..darkness……NO!.. I can still do this….I am strong and so is my baby boy…….*

Wait.

Life goes on.

 4 drains protruding from his belly.  He still has his ‘extra belly buttons’ as Jonathan calls them. Deep scars on his belly where the tubes exited his body. He had drains in place for a total of 8 months. Following the placment and subsequent removal of the drains, Zachary experienced 4 episodes of rejection. One right after the other. This earned him a year of increased immunosuppression, 2 meds instead of one and steriods. Almost a full year of steroids.

Steroids prohibit linear growth. In other words, Zachary is short. Or he was until this past summer. He finally made it onto the growth chart. You know, the percentile chart that the doctors use to plot a child’s growth. He finally made it to the 10th percentile.  So maybe he won’t be the tallest boy in his class. But he will be able to function to the very best of his abilities. 

 Zachary just turned 3. He hasn’t seen the inside of the hospital for a solid year. He never had any episodes of hyperammonemia.  And while he did work with the speech therapist for about 18 months, he doesn’t any more. 

Life goes on.

And that brings us to David.

I’ll met you over at his page.

Advertisements
Leave a comment

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s

Create a free website or blog at WordPress.com.

%d bloggers like this: