Make A Child Smile

Makenzie's Story
written by mom Harmony

Makenzie was born on July 6, 2004. We thought we were having a healthy baby, but as soon as she was born, the nurse noticed her being a little blue. They tried suctioning her and her oxygen just wouldn’t come above 91. (A normal person is 98-100).

The doctor said that a cardiologist needed to take a look at her. When the cardiologist came, he was holding a piece of paper with a heart drawn on it. I knew at that moment, something was wrong with my baby. He explained to us how a normal heart worked and then explained to us how Makenzie’s heart had formed. The last thing I remember him saying was: "Your daughter has a condition called Hypoplastic Left Heart Syndrome. She is very critical, and needs open heart surgery".

Hypoplastic Left Heart Syndrome, or HLHS, means that the left side of the heart did not develop normally. The left side is the most important and strongest side of the heart, with its main job being to pump all oxygenated blood throughout the body. Without medical intervention, 100% of all babies diagnosed with HLHS will die within the first week of life.

Once we got to the hospital, they told us that she would have to have a 3 staged surgery. The 3-Stage surgeries reconstruct the heart, allowing it to work, using only 2 of the heart's 4 chambers.

The 1st stage is called the Norwood procedure, because the left ventricle cannot pump blood adequately out to the body. The Norwood procedure allows the right ventricle to pump blood to both the lungs and the body. This surgery is usually performed during the baby's first week of life.

The 2nd stage procedure, called the Glenn, reduces the work of the right ventricle by allowing it to pump blood only to the body, and allowing most of the blood to flow automatically from the body into the lungs.

The 3rd and final stage, the Fontan, allows the rest of the blood coming back from the body to go to the lungs.

I got to hold Makenzie for the first time the night before she went into surgery. It was bittersweet. I was happy I got to hold my baby, but still couldn’t help but think this could also be the last time I hold her.

She had her Norwood procedure just one week from the day she was born. They gave her around a 70% chance of survival. She had a very rough recovery. There were many times we thought she wasn’t going to pull through. Her kidneys stopped working and she had to be put onto dialysis. She had many problems with arrhythmias, with her heart rate jumping into the 300’s a few times. Her oxygen saturations never came above 65 even with oxygen. When they were placing her breathing tube in before surgery, they nicked her left vocal cord and it became paralyzed. Because of this, she could not control her swallowing, she couldn’t take anything by mouth and she had to undergo another surgery to place a feeding tube in. They also did a procedure called a Fundoplication. This is where they sew your stomach to your esophagus so she wouldn’t have acid reflux.

After 2 months of an emotional rollercoaster, we were finally able to go home with Makenzie on oxygen. A few weeks later, the doctors decided it was best that we go ahead and do the next surgery, the Glenn, because her oxygen was still so low. They usually give these kids a 90% chance for recovery, but told us since Makenzie had such a rough go around with it, they were giving her a little less at 80%. I remember walking into seeing her right after surgery and she was as blue as a blueberry. Her body was getting used to having “different plumbing” and her oxygen was only in the 30’s. It was the scariest thing I had ever seen. Her kidneys didn’t function very well after this surgery either. She then developed a nasty cold which made us stay a week longer than we were hoping for but instead of 2 months after this one we only had to stay 2 weeks. She came home on oxygen, but after a week, we got to take her off of it. This was the first time since her 1st surgery, she didn’t have to be on it.

Over the next year, we had lots of moments where we took her in to be looked at because we thought she was bluer than she had been. She also had to have physical therapy, occupational therapy, and speech therapy. Because her vocal cord was paralyzed, she never made any noise. It came back very slowly. She finally started making some noise at around 11 months. It’s still hard for me to hear people complain about there baby crying now, because all I want to tell them is at least you get to hear your baby cry.

By the time she was 2½, she had pretty much caught up to other kids her age. Makenzie spent the next year and a half without any troubles. Right before her 4th birthday, she had a heart cath done to determine if she was a candidate for the Fontan.

Her Fontan surgery was scheduled for August 20th, 2008. The feeling of having to hand her over to surgery that day was the worst feeling I have ever had. It was definitely the hardest out of the 3 surgeries to let her go. We got frequent updates and overall her surgery part went great. It took about 5 hours all together, from the time we said goodbye, until the time we got to see her in the ICU. The hardest part was not letting her eat or drink or getting to hold her. She was so thirsty, but because they didn’t want her to get sick from the anesthesia, they wouldn’t let her have anything to drink. When she finally got to drink, she suck down a whole can of sprite in under a minute.

About 2 days after her surgery, her stomach turned rock hard and swelled up pretty big, and it was like that for about 2 more days. She spent 5 days in ICU. She finally got taken off oxygen 8 days after surgery and now her O2 is around 94-96! When we got moved up to the floor, we had several issues with getting her potassium and sodium levels to where they needed to be. At this point in time, we are still having the same issues. She also kept getting pleural effusions because her chest tubes just weren’t draining so she had to go back to the OR and have 2 new tubes put in just under her ribs. These draining tubes are working extremely well. The problem they are having now, is they are draining way too much.

Makenzie got released from the hospital on September 12th. She did have to come home on 12 different medicines and with both chest tubes. We are getting adjusted to having to be really careful around her tubes and getting up in the middle of nights to give meds and strip her tubes. We are not sure how long she will have to keep her chest tubes in but we are just so happy to have her back home.

Makenzie has now had all of three of the 3 staged surgeries. We are not sure what the future holds for her. There has been mention that someday, she will need a heart transplant but they can't tell us when, it all depends on how her body handles everything. For her sake and ours, I hope its not for several years down the road. There is really no statistics to go off of early, because it has only been about 15 years since they started doing the surgery in 3 steps. Children are thriving longer with it, but most of them are just now hitting there teenage years, and the doctors are not sure how long the repaired hearts last.

(Story and Interests were last updated November, 2008)