HLHS or Hypoplasti Left Heart Syndrome is a birth defect that affects normal blood flow through the heart. As the baby develops during pregnancy, the left side of the heart does not form correctly. Hypoplastic left heart syndrome is one type of congenital heart defect. Congenital means present at birth. (As per google definition)
Diagram of Healthy Heart and One with Hypoplastic Left Heart Syndrome. Photo placed into public domain by Mariana Ruiz, http://en.wikipedia.org/wiki/File:Hypoplastic_left_heart_syndrome.svg
Basically, the left heart was underdeveloped becouse that heart cannot pump enough blood to the body and reqires several surgical procedures to fix the heart. Note: the procedures are not cure. They are done to make the heart function as it supposed to be.
Is HLHS genetic?
Not really. Genes are not actually the biggest factor on having HLHS. But if you have a family history of having heart defects, then there will be a chance that it can be hereditary.
What causes HLHS?
There is no specific cause of HLHS. There is no concrete study that it can be 100% genetics, or from diet, or other environmental factors. But it can be associated to genetic diseases such as Turner Syndrome, Jacobsen Syndrome, etc. Probably a parent of the child already having these syndromes.
Can HLHS be prevented?
As per study, no. Since there is no chance of knowing whether your child can have it or not, there is no way on preventing it. The only way to know whether the baby will have it or not is getting an anomaly scan. In that way, if ever your child has it, you can arrange with your health care provider/medical team the proper steps to do when it’s time to deliver your child. You can arrange your birth in a hospital where they can provide right away the treatment your child needs.
What are the procedures done for HLHS?
There are 3 major operations that are performed to the child. The 1st one is done shortly after birth, which is Norwood. The 2nd is Glenn. Which is done at about ages 3 to 6 months. On our child’s condition, it was done at 9 months, because as per her doctors, she was doing great and no need to rush the operation. I believe it is in a case to case basis. The 3rd one is Fontan. Which is done at 18 months to 4 years. Our child didn’t have this operation yet. So we will be updating you as the time comes for us to get the Fotan.
What to expect after getting the procedures?
A newborn underwent a major procedure is not easy. Being opened and observed after the operation is such a huge thing for a small baby. The constant monitoring and the loud beeping of apparatus around is not a pleasant experience.
As parents whose child had been to two of the operations, it wasn’t easy. Maybe on the 1st operation all our minds were just focused that the operation will be done as quickly as possible and she’ll recover. We don’t exactly knew what’s happening. All we know was our child is getting the treatment she needs and she’ll be okay.
The 2nd operation was a different story. When we thought that we knew already what’s happening or what will happen, and we are ready for it, that’s the time you will realize that you are not. It was another experience that you will feel familiar but something new. You maybe aware already the things happening but you can still get surprised with other things.
The thing you need to expect after each operation is the healing process and other complications can arise. Your medical team/health care providers will always tell you the things to expect. It is physically exhausting for a child and well as to the parents. It is emotionally and mentally draining as well. Seeing your child undergo such huge operation at a very young and fragile state is not easy.
Child and Parents recovery
Yes, the child underwent a big challenge in their life at a very young age. It takes time for them to recover. Numerous doctors check ups. Which you should not miss. Because it is very important to have a constant monitoring with their heart, especially their oxygen saturation. It is also important for them to get their medications on time and on proper dosages. Their motorics and cognitive aspect should be consider as well. Since we don’t know how long can they be on a hospital bed. There are children also develope problems with feeding and their nutrition. These things we can share and discuss on the coming posts.
For parents, healing. Healing in a sense that this is a traumatic experience whether we admit it or not. Besides being exhausted physically, our mental and emotional state had been challenged. Us parents need time to recover from this events. Make sure to have time for yourselves. After recovering from all of the hardships, make sure to make the most of your time with you child and your family.
Note: Every family has their own journey with HLHS battle. We encourage the parents to take care of themselves as well. It is physically, mentally and emotionally exhausting. Make sure to get the support you need. Never hestitate to talk to professionals if you feel the load is too heavy to deal with your own. You and your child’s health are both important too, to win this battle.
GWEN. The name means blessed, white and holy. This girl was born December 2, 2022 at 8pm. The girl that changed our lives.
A normal but challenging pregnancy
Year 2022, I got pregnant. The whole pregnancy was challenging for me as a first timer. I was 36 years old when I got pregnant. The sudden change in my body from pregnancy was a challenge. During my pregnancy the only thing that made it hard was I have a higher blood sugar. So during my first and 2nd trimester my diet was extremely monitor. Besides this struggle, I only had the usual pregnancy symptoms and changes. Vomiting, gaining weight, swollen feet, and other minor things. The entire pregnancy was normal as it can be.
I gave birth exactly forty weeks gestational age. A day before the due date I had the feeling of contractions but they go away. Until about 2am the next day the contactions were getting closer so we decided to head to the hospital. It was a long 16 hours labor. I felt I was Rachel Green from Friends sitcom for a while because how many pregnant women come and go that day and I was still there not pushing until the last few hours. Kidding aside, to be honest I already forgot most of the details how I was doing on the delivery room. I can only remember how the made me do a lot of positions, how many times they look how much my cervix opening was, and of course I remember how exhausted and hungry I was after I delivered her out.
To be honest the nurses and doctors really took care of me and supported me during the delivery. I couldn’t thank them enough for being there for me during my pushing, turning and screaming.
The suspicion
Fast forward, the next day while I was at the maternity ward, I already noticed that my child looked darker than I expected her to be. I wasn’t sure ifbit was just the lighting in the room or it’s just that she was still adjusting that’s why her skin is darker for my sight. But besides her skin, I noticed that her nails were purple in color. I didn’t mentiom right away the lips cause I wasn’t also sure if she’s adjusting to the outside world. I approached the nurse and asked her if the color my childs finger nails were normal. They looked at her and said yes. So I didn’t even bother to ask anymore.
The start of an agonising wait
The day when we were supposed to be discharged, they took her to be cleaned and dressed and do the final new born check. I already felt that something wasn’t right when the other baby that was taken together with mine already came back and mine wasn’t. Few more minutes had pass and a nurse came and told me that my baby will not be coming back anytime soon because the doctor are checking up on her because they noticed my child’s lips were purple. I asked her what was happening but she told me I needed to wait for the doctors to come and explain to me what’s going on.
It was an agony of waiting that I didn’t know what really was happening. It made harder as well because I couldn’t speak the language so well. We live in Croatia, the language is not english, and I am from the Philippines. I understand and speak basic Croatian, I speak english and my native language. Back to the story, two doctors came and told me they checked on my daughter and they found out that she has a problem with her heart. I asked for the exact diagnosis but they said it wasn’t sure yet. So, they will send her to a bigger hospital to be totally checked and give the proper treatment.
The world collapsed
I felt my world collapsed on me. I did’t really know what to say. My body shook and like I’am floating. I just said please bring my husband to me. I was all alone when things happened and when they told me about my child. When my husband came, he saw our child already put in an incubator and ready to be transferred to a bigger hospital. It was a scenario that I have never ever thought that will happen to us. He saw her for the first time in a situation that was unpleasant. It wasn’t a happy first meeting. That wasn’t what I pictured at all.
I wasn’t sure what will happened next but the doctors told me that my child will going to get the best treatment she will need.
The battle has begun
The next scenarios were all happened at Rebro Hospital in Zagreb, Croatia. That’s where they took my child to be examined and give the treatment she needed. After being placed on NICU, they told us and explained to us that our child has HLHS tricuspid atrezia, and she needs three heart operations. Thet discussed us the first operation. It was totally confusing for me to be honest. I didn’t understand anything. I know it is a serious condition and it needed to be operated immediately. When the surgeon asked us do I want to ask anything or do I not agree with the process that they will do, I just said, “Please do the necessary thing for our daughter.” And that is all a parent could ever say. At seven days old, our child got her 1st heart operation. She stayed for one and a half month in the hospital before we got discharged.
Slowly but surely winning
Moving forward, within year Gwen already finished her two major heart operations. Which is Norwood Btt shunt and Glenn. She has still one more surgery to undergo, which is Fontan. These we will briefly tackle on our future posts and other more topics related to HLHS.
To this day, Gwen is already a year and two months upon writing this blog entry. She is a very happy kid. Active, smart, pretty, naughty at times but looking normal as like any other kids at her age. Slowly we will introduce her to all of you and we will share her story and ours as parents.
This is the story of our Gwen, our little miracle.
Jeni ❤️
