The Road Less Traveled

On May 29, 2013 at 12:37 PM we welcomed Matthew Abram Long into our family. His was a quick, easy birth after an uneventful and normal pregnancy. He looked perfect! His sisters were there to meet him immediately and we were allowed to hold him and bond with him for almost two and a half hours before he was taken to have his first check up. For the first 24 hours we held him constantly. We kissed him and talked to him and thanked God for another precious gift; for a wonderful delivery; for growing our family with this amazing, happy, healthy little boy.

MAL_1

 

At 30 hrs, Matthew began to be extremely fussy and was inconsolable. At 35 hours, he spit up. A lot. And of a consistency that was abnormal (to save you the details). At 36 hrs he was taken for some routine labs and brought back to us with a comment about how fussy he had been.. Our baby who had barely cried in those first 24 hours, no matter how much he had been poked and prodded, who had received comment after comment about how easy going he was, was suddenly a fussy baby. At 40 hrs he was taken by the pediatrician for a discharge exam. Hours passed. Then there was a knock on the door. “I’m not going to be able to let Matthew go home. His breathing is elevated and I want to run some tests to see what’s causing it.” After the first day, Rusty was home with the girls, keeping them busy until it is time to get pick up Mommy and Matthew from the hospital. I’m left standing in a cleaned, packed up room with no baby and some confusing news. I sat down and cried and prayed for hours. “Lord please give these doctors wisdom to care for him. Please protect my baby and send him home with us.” Over the next four hours, I sat alone, debating whether or not to stay at the hospital and wait for Matthew or to go home to my waiting girls. During that time, the pediatrician informed me of the tests she was running and as each came back negative I could see her getting more and more worried. She had long since given up trying to figure out what the problem was and was following advice from the local children’s hospital. At noon on the 30th, I left the hospital without my son. Within an hour we got a phone call: “Mrs. Long? We need your verbal authorization to transfer Matthew to the NICU at CHKD. Now. You can meet us there.” And so we did. At 5pm we saw the first NICU doctor. “We believe your son has a urea cycle disorder. He isn’t responding to treatments. We need your permission to insert a PICC line. Now.” The process, we were told, would take a few hours. We went home and put the girls to bed and were back in the NICU by 9. Once there we were introduced to his nurse and heard about how much trouble he had been. His seizures. His over compensating for the insulin he’d been given. We looked at out tiny little baby, with monitors everywhere, unconscious and we cried and we prayed. And then we met with the doctor. “Matthew has an elevated ammonia level in his blood. It’s very high. We’d like to run dialysis on him. Now” We were told that the dialysis was dangerous and that his ammonia levels were “bad” and that the outcome of those levels was not positive. At 1AM we watched them wheel his tiny little body around the corner of the PICU to prep for dialysis.

MAL_2

 

Over the next few days, we learned about urea cycle disorders and what it would mean for Matthew. His body can’t properly break down protein and instead deposits ammonia into the blood stream. Elevated ammonia levels are damaging to the brain. Normal ammonia levels are below 50. When Matthew was admitted to CHKD, his ammonia level was in the 500’s. By the time he went to the PICU, his ammonia had risen to 1200. Dialysis is a 3 hour process in which his blood is pulled out of his body, filtered to remove the ‘bad stuff’, and then replaced. Matthew’s first round of dialysis brought his ammonia down to about 200, at which point we hoped that medicine would manage the problem. It didn’t. Within a matter of hours his ammonia was rising. And fast. By the time he was brought back down to the PICU his ammonia had spiked to 1800. We met with many specialists who explained what all of this was doing to Matthew and what we could expect after all was said and done. His is a rare type of disorder. Not much is known about it, but we do know that ammonia levels in those ranges are detrimental. It was explained that he would most likely be a vegetable, considering his spikes and the length of time they were sustained. At least 3 times, we were brought in to conference with specialists who told us that no one would blame us if we decided to stop treatment. There was no way of knowing how bad things were going to be, but they knew things wouldn’t be good. Each time they didn’t expect him to survive. And, each time we asked “If you pulled him off of the machine, would he be alive? Is he still fighting?” Each time the answer was yes. How could we give up on him if he was still willing to fight? Who we
re we to decide that his life wasn’t worth living?

MAL_3

If God wanted Matthew, God would take Matthew. So we continued treatment. Over the next few weeks, Matthew went through 3 rounds of dialysis and one round of continuous dialysis (this is the same process, except it is much smaller volumes over the course of a days). He had EEG’s, a continuous EEG, and an MRI. His body was kept cool to help preserve his brain. At one point, we counted 17 lines going to him. Finally, his ammonia levels began to stay in the under 200 range. It was decided that Matthew was not going to be able to survive without a liver and his case was so fragile, that it might be best for him to be transferred to DC, where they have more experience with these cases, and where he would stay until a liver became available. But first, DC wanted to see what he would do off of the ventilator. The expectation? We expected him to be emergency air lifted to DC or to not breath on his own.

But he did.

Matthew came off of the ventilator like a champ. Slightly shocked, we began to see what he would do without this intervention and that intervention and ever so slowly we whittled back the machines and the medicines and we added formula and then formula with breast milk. Each time something was changed we held our breath and waited for those ammonia levels to rise, unsure that his body could survive another spike, and each time he over came the odds. We went to his bedside and talked to him and read stories to him. We told him to fight; to be strong. And then God put 2 Chronicles 20:15 in front of us. ” Thus says the LORD to you: ‘Do not be afraid nor dismayed because of this great multitude, for the battle is not yours, but God’s.” Oh the peace that came from those verses! We didn’t have to fight for him. The doctors didn’t have to. Not even Matthew had to fight. God had already done all the fighting for us all. If this battle was to be won, it had already been won by God. And so, we began to tell him to rest and let God take care of things. After a months time, we were able to hold him again.

MAL_4

MAL_5

 

And then he was moved out of the PICU! He was stable enough to move to a regular room. He no longer needed a nurse to have eyes on him 24/7. He didn’t need a ventilator or a PICC line or an arterial line. He was awake. He cried. He had dirty diapers. He ate (through a feeding tube).  His sisters could now visit him. For weeks, they wondered where their brother had disappeared to, why he wasn’t home, why Mommy and Daddy had to keep going to the hospital and now they could see him, touch him, and sing to him.

MAL_6

 

At almost 6 weeks, Matthew finally came home. God has performed miracle after miracle with Matthew. That the disease was identified so quickly. That Matthew was able to withstand round after round of dialysis and two major ammonia spikes. That he was able to breath on his own. That he is able to move and has strong muscles. That he can see and hear. That he is maintaining his ammonia levels through medicine and diet. That he is alive and home and being cuddled by his family. All of these things fly in the face of his condition, of what was expected.

MAL_7

At any point, Matthew’s ammonia could begin to rise. A cold, a growth spurt, anything could throw off his delicate balance. And so, now, we wait for a new liver. A liver won’t cure him of his disease. The damage that has been done can’t be undone (except through further miracles). Every cell in his body will still have the mutated gene for the urea cycle, but a new liver will function properly and provide him with a protection against further spikes.

The road that we are on certainly is not the road that we anticipated. But we have to trust that the God who put the stars in their place, who knew us before we were formed, knew exactly what He was doing when He gave us our precious little boy. During the pregnancy, I daily put hands on my belly and prayed that God would protect my growing son and give us a perfectly healthy baby. I can’t help but ask “why God?”; to say “But I prayed for a healthy baby! I prayed Every Day!” As each day passes we see more and more that God’s ways are not our ways. His plans are better. That Matthew is a very special boy and is being used by God for a mighty purpose. He may not be what we thought we were getting, but he is exactly what he is supposed to be. God didn’t make a mistake when he formed Matthew. He didn’t make a mistake when He gave him to us. For as long as we are blessed with him, we are going to continue to advocate for his care, to never give up on him, and to trust that “God’s got this!”.

To learn how you can help, click here for our Donations page.

One thought on “The Road Less Traveled

  1. Fellow UCD mama here…it would be an honor to pray for your sweet boy. Each of these amazing children hold so much space in my heart, so rare and so strong! I look forward to reading more as time passes and pray for the perfect liver for Matthew in God’s perfect timing!

    Mindy~

Leave a Reply

Your email address will not be published. Required fields are marked *