Luke and I went to the hospital around 3 am on July 16th. My water was ruptured around 9 am. There was meconium in the water, so they prepared to treat our little guy for meconium inhalation. I had been told that UVRMC was notorious for keeping babies in the NICU for observation longer than usually necessary. So I figured it would be awhile before we would see him. Maddox Larry Bulkley was born July 16, 2009 in Provo, Utah. After a quick kiss they rushed him off to the NICU to be treated and took me down to Mother/Baby to recover. Several hours later I was able to go up to the NICU and see our little guy. Even though I had seen pictures of him (thank heavens for digital cameras) I was un-prepared for the sight of the tubes and wires that were attached. We were told he would probably spend the night in the NICU for monitoring. Sad as I was about leaving him, I was thankful for a good nights rest.
Early the next morning Luke ran home to shower, visit Bailee and mow the lawn. I went up to the NICU and visited Maddox and was told he wasn't really getting better. One dr. told me that she thought his lungs weren't big enough for his body. Shortly after my visit, Luke had been gone about an hour, I got a call from the NICU. They wanted to speak to us. I called Luke and asked him to hurry back down. About 2 minutes later a team of Dr.'s came into my room and told me that something was wrong with Maddox's heart. They asked for permission to Life Flight him to Primary Children's Hospital and said we could see him before they left. Luke arrived and we went to the NICU for more answers. They couldn't tell us much and wanted a full Echo work up done at PCMC. Luke and one of the medical people gave Maddox a blessing and off he went for his first helicopter ride.
We stopped at home and picked up a few things and gave Bailee a kiss. We are grateful Grammy was here to be with Bailee. On the way up to PCMC I thought of Luke's own heart journey and took comfort in knowing Maddox was in good hands and they'd be able to fix whatever was wrong. (Luke was born with a coarctation and a bicuspid aortic valve. In English that means his aorta was narrow and his valve has 2 flaps instead of 3. He had his aorta repaired when he was about a week old, and had it enlarged when he was about 12. At some point the aortic valve will need to be replaced, but so far everything is good.)
We met with Dr. Chan and Dr. Amula after they had completed an extensive echo. Maddox was diagnosed with Hypoplastic Left Heart Syndrome. Essentially the left side of his heart did not develop fully and is non-functioning. The first thing Dr. Chan and Dr. Amula told us was that they couldn't fix Maddox's heart. They gave us 3 options. 1- we could take Maddox home and enjoy our time with him, knowing he would pass away. 2- we could place him on a transplant list and hope he lived long enough to receive the gift of a new heart. 3- Maddox could have an operation in his first week of life that could help him grow enough to have 2 more surgeries to help his heart function a little better. Luke and I both knew the option for us was the 3rd, and we embarked on the journey of a life time.
5 days later, after his surgery being moved back a day for a more critical case, at 1 week old Maddox had his first open heart surgery. His surgeon, Dr. AK, told us this surgery had 3 purposes. 1-to fix the coarction of the aorta. 2-to remove the wall between the right and left atrium's to allow for blood mixture. 3- to place a sano shunt to help with circulation until Maddox could have the next surgery. The surgery went well and Maddox recovered well. His biggest challenge was being weaned off the ventilator and then high flow oxygen. We brought our baby home for the first time on August 12th. He was a cranky, fussy baby. He cried, he didn't feel well, and it was a challenge. On the night of August 15th Maddox developed a fever and began having a hard time breathing. We took him back to PCMC in the early hours of August 16th, his one month birthday. He was admitted to the hospital in Respiratory Failure.
Later that day it was determined Maddox had a mediastinal infection, an infection in his incision. Dr. AK re-opened Maddox's chest and cleaned out the infection. This included scraping the breast bone and washing out with anti-septic fluids. Maddox was then given some very powerful antibiotics and began his second recovery. After several spontaneous trials to see if he could breath on his own, Maddox pulled out the ventilator on his own. He had decided it was enough. Maddox did very well and we came home again after 10 days in the hospital. He was way sicker during the 2nd hospital stay, and it was a much scarier time for us. We brought him home and gave him antibiotics through a central line 4 times a day for about 4 weeks. This time Maddox was a very happy, calm baby. He loved to be held and cuddled and to watch his sister dance crazily around the house.
For the past 3 months we have followed up with our Cardiologist and Surgeon in preparation for the 2nd surgery. In the past month we have seen a decline in both his oxygen levels and his heart function. We have begun pre-op testing and are hoping to have the surgery the 2nd week of December. That brings us to now!
Update:December 16, 2009
Maddox had the Glenn surgery on Deceber 8, 2009. In the Bi-directional Glenn procedure, the Superior Vevna Cava is hooked directly to the Pulmonary Arteries. This relieves some of the stress on the Common Atrium and Right Ventrical, and increases the amount of oxygenated blood that flows to his body. The shunt that was placed durring the first surgery was removed, so there is no longer foriegn material in his body. Dr. AK also removed scar tissue from the previous 2 surgeries. The most visible side effect is that Maddox is PINK, instead of blue (and more recently, gray), and his feet are warm! He is back to his happy, giggling self and we are LOVING each day with him. Maddox spent just 6 days in the hospital and is recovering so well. It seems to hurt most when he coughs or sneezes, but otherwise he appears to be pain free. We hope to wean him off oxygen in the next month or so. His saturation levels are visabley better, which shows in his coloring.
This is the last 'planned' surgery for a couple of years. The Complete Fontan will be preformed sometime after age two, depending on how his heart is functioning. Until then we will continue enjoying the time we have, and making the best of what's been given to us.
UPDATE: July 10, 2010
No news is good news, right? I just was reading through this post and I can't believe our family and our little man went through all of that! As his birthday approaches, I am so extremely grateful for the time we've had with Maddox. He has taught me so much about faith, trust, endurance, and life. Since the December surgery Maddox has been ok, but not great. In February he got a cold and ear infection, and fought those clear until June. We took him into the Dr. about every 2 weeks, and he was on antibiotics almost continuously. It was rough! In April he had 2 hospital stays. The first was at PCMC where he was treated for Pneumonia and the 2nd was at UVRMC where they treated him for Rhino Virus, which is the common cold. Yuck! June 1st I took him in to check out his ears to be sure they were all clear, and he was starting another infection. That put the total up to 11 DOUBLE ear infections in just 4 months. At this point we had already seen an ENT and decided to have tubes put in.
June 23rd we took him back to PCMC where he had tubes placed in both ears, circumcision, and an ECHO of his heart. Each of the procedures when as planned, there were no surprises. I had prepared myself to stay overnight, but Maddox was able to come home a few hours later. He healed quickly and has done well. I am happy to be able to say the June 1st ear infection was the last time he was sick! (knock on wood).
On June 28 we met with our Cardiologist. She had reviewed the echo and found nothing new. This is both good and bad news. The good news is that Maddox's heart function has not declined. The bad news is that Maddox's heart function has not improved. We increased his dose of Enalapril (helps the heart squeeze) and will continue to follow this. We had more good news too: Maddox can be off of O2 all of the time, as long as his saturation stays at HIS normal. (between 75 and 85%) and we don't go back to Cardiology until October or November. Wow.
Other than his Hypo-plastic heart, Maddox is doing well. He is growing better than we could have hoped for! At this point he is 21 pounds! What a blessing! His "other than heart" challenges are so minor compared to what they could be, and I am so grateful! Maddox is delayed with both his Motor Skills and his Oral skills. Meaning, he doesn't crawl or pull himself into sitting, and he eats like a 9 month old. We are working with a physical therapist and an occupational therapist to increase these skills. Being 'behind' is expected, but after doing some research I felt it was better to start addressing these issues now rather than 'wait and see'. We haven't seen any delay in his social or emotional skills. With the 'lack of' oxygen, I am surprised. But this kid is so smart! I love watching him play. I can see his mind working and figuring things out. Incredible.
So that's a little update! We are so excited to celebrate our Miracle Man's first birthday next week. What a year it's been!
April 21, 2011
I just wanted to update about Maddox again. He is doing very well, and keeping us busy. Last month his SATS were low and I put him back on O2. I watched him for a few days and didn't see any improvement, so I called Cardiology. They wanted to see him the next morning (March 16th). We did a chest xray, blood work, and an echo. He was so good through it all. Everything looked good though, so we decided to increase his Enalapril (they were going to wean him off of it, so it hadn't been increased at the last checkup). He was on 02 continuously for about 2 weeks, then just at night for about another 2 weeks. We just went back to Cardiology this week and he's doing great again. Not sure what the hiccup was, but may be attributed to the Enalapril. He started walking on his own on March 25th, and is walking and almost running everywhere. It's really so cute. Dr. Pinto is pleased with everything and said that best case scenario would be Fontan next spring, after the sickness clears out of the hospital. This is what I had been hoping for all along, but the little episode last month made me worry it would be sooner. The other good news is that he is big enough for the surgery, so if he took a turn for the worse before next spring, he'd be ok to have surgery. He is 25 pounds right now! That has been such a blessing, and a tender mercy, that he hasn't had major issues gaining weight or growing. The boy loves food, and he will eat anything. Well, except mashed potatoes.
February 10, 2012
Maddox had an appointment with Cardiology yesterday. He is doing so well. We have a plan for surgery this summer. He will have a pre-surgery cath and echo in early to mid May, and then have surgery 2 weeks after that. We are shooting for early June. It's really a bitter sweet time. We know that he needs this surgery, but it's hard to imagine going back to that life. The last surgery was over 2 years ago! Maddox weighs 30 pounds now, so that is great. He is walking, running, playing, learning, talking like crazy. We just couldn't ask for more.
