Little Zippers

          Jozlynn's Story         

       When I was 5 mo. pregnant we got the devastating news, "there's something wrong with your baby's heart." She had a Single Ventricle defect. Half a heart. We were young and scared. It was a VERY difficult pregnancy. The whole time the doctors were telling us that her prognosis was not good. The defects she had would not support life, therefore the only options we had were to just have her for a few hours after she was born and let her go.  Or they could do an open heart surgery and IF she survived the surgery, it would buy us enough time to get to know her. Then they would give her comfort medicine and we would have to watch her pass away. They said they would be surprised if she made it to two years old. It was supposed to be the happiest time of our lives, but it turned out to be the scariest struggle for life I've ever seen.

       We were devastated. But we were determined to do everything in our power to save our precious little baby. We continued to love her no matter what the prognosis (probably more than if she was completely healthy). We still had a baby shower for her, and all of our friends and family where very supportive. Throughout the whole pregnancy I had an ultrasound every other week. We had to drive 2 hours away to the cardiologist where every time it seemed to be MORE bad news, and they kept reminding us of the horrible prognosis. The doctor was really trying to convince us to not have any surgery and just enjoy the few hours that we would have with her (probably because it would be easier for him). He also didn't think it was possible to do any open heart surgery bloodless. Near the end of our pregnancy, he just outright refused to do anything for her. Although I only had one and a half months left until due date, the search was on for a new doctor. We almost gave up hope when I got a call from a surgeon who heard of our story and without promising anything said that he would at least attempt to save her life (and do it without blood transfusion), but he said that I have to come to the Ronald McDonald House immediately in case I gave birth.

       So off I went a whole state away from my family, friends, and husband (he had to work until the baby was born). It became my home for a very lonely month until she was born. They had to give her medication to keep a hole open that normally closes right after birth so that she didn't die within minutes of birth. 

       On the morning of September 19, 2004 our little Jozlynn was born, 7 pounds 10 oz. She was the most beautiful little baby I had ever seen. We got to spend some time holding her, kissing her, nursing her, then after about 30 minutes while in my arms I thought she fell asleep. Then someone said, she is looking more blue, let me get the nurse. Then suddenly she turned purple and was completely limp. The nurse literally ran in, followed by just about everyone else who worked at the hospital.  She had turned blue because she had stopped breathing. They had to immediately take her from my arms and resuscitate her and put her on life support. I have never wanted anything so badly than to just leap out of that bed and be with her, I felt like part of me was dying, but her daddy followed them and stayed by her side every moment.
       Jozlynn was born with rare congenital heart defects, basically she was missing the entire right side of her heart. Hypoplastic Right Heart Syndrome (HRHS, Single Ventricle) Pulmonary Atresia with Intact Ventricular Septum (PAIVS, Pulmonary Valve completely closed). PAVIS and HRHS occurs in 1 out of 10,000 live births.  This situation cannot support life, since oxygen-poor (blue) blood cannot meet the body's demands, Tricuspid Insufficiency (Tricuspid Valve not working properly), Pulmonary Artery Stenosis (extremely narrowed Pulmonary Arteries Right and Left), PFO (Patent Forman Ovale), ASD (Atrial Septal Defect) ,and Congestive Heart Failure. These defects are among the most complex and rare congenital heart problems known.

     Dr. Starr, her surgeon who was on vacation, flew home early and arrived just in time. Late that night a few of the surgeons doctors and cardiologists took us into a room and laid it all out for us. We had a few different options and we had to make a decision within the next few hours because they had to operate immediately. They told us that no matter what decision we make, there was a good chance that even if she made it through surgery, her body might not be able to handle the new circulation and she may go into shock and die. So, on her second day of life she had her first Open Heart Surgery, bloodless.

     It was supposed to be a 3 hour surgery, and it ended up being 9 1/2 hours. They couldn't get the shunt to work, it kept clotting. Her vitals crashed two times then they thought that they finally fixed her so they closed her chest up and her vitals crashed again. So they immediately had to reopen her little chest and stabilize her heart. The surgeon came to talk to us and told us that he could only attempt it one more time, needless to say for the next couple of hours we were just in one constant prayer. There were a lot of tears, but in that moment was when we really felt the most calm. We had spent most of the day before just telling her all about how much we loved her, and all about our wonderful creator, Jehovah God who had helped us out to this point. We knew that if Jozlynn's little heart just wasn't strong enough, we at least had the opportunity to give her all of our love and tell her that she has already changed our lives for the better. We have a strong faith in knowing that we would be together again someday.

     Meanwhile, Dr.Starr had tried to do the shunt one more time and she crashed again. It seemed like that was the end, but once they got her stable again her surgeon was able to talk the rest of the team into trying to put a larger shunt than they have ever used on a baby and it worked!! "Jozlynn is still with us, she made it through," those words from Lesley, the nurse who gave us updates every hour, still echo through my mind down to this day. She recovered nicely from her surgery, but it just took her a while to recover because every time her chin would get close to her chest, she stopped breathing. Her incision also broke open because of an infection and it took a very long time to heal. We had to keep her secluded at home until she was 3 months old.  She wasn't gaining weight, and she looked so weak. Looking back I felt like I should have realized that the darkness around her eyes was so prominent and she never really even made any noise, she was just too weak.     

     Her second surgery became an emergency when she was 5 months after the pressure in her heart became dangerously high and she was having severe congestive heart failure. All went well, and they were able to get the pressure into the safe range. After that, our little Jozlynn finally started thriving, despite the constant weight gain issues. Then when she was 11 months old she had to have a surgery on her tear ducts. She kept getting sinus infections and had to practically live on antibiotics. It was a simple surgery but she was admitted because of her heart defects. On that morning, there were several other families who were also having the same surgery on their babies. They were all beside themselves with worry. We were thinking, this is a breeze. I almost felt jealous of them, if only that was our biggest worry, a simple outpatient procedure. We still kinda giggle about it now. The other parents were beside themselves with fear, and we were playing cards and smiling. She did just fine.

     Less than a month later she had another heart surgery when she was 12 months, and again a smooth recovery. And then for the most recent open-heart she had to have injections every day for a few months to build up her red blood count and hopefully make her stronger. Mommy and daddy had to learn to give the shots, and although she dreaded them, she never complained. She even said "thank you daddy" after every one. Then she had her fourth surgery just a few months before she turned 3 years old. Unfortunately, only a couple of weeks before her surgery, we had a housefire that took everything we owned including all of Jozlynn's toys and her beloved blankie. It started from an electrical problem in the shed and VERY quickly spread to the house. I still can't believe how quickly it all went up, thankfully we all escaped with our lives. Although we had no renters insurance, we have the best friends and family in the world that were able to step up and help us any way they could. Also, we were on several different Seattle news channels and people from all over Washington donated enough for us to get back on our feet. We are extremely grateful for everyone's help.

      Surgery day came and it went smoothly. The first time we went in to see her after surgery, she looked a little different from other times. Her hands and feet were warm, but they were purple. They had no idea why. They thought that her little body didn't handle being on the heart and lung machine, sometimes that is a sign that the organs are starting to shut down. She was still not able to breath on her own. All we could do was wait and see if she would pull through. After a long couple of days of not knowing, she was finally stable enough to breathe and her color got better.

     The older she gets, the harder it is to watch her have to go through such a traumatic surgery. There is always a period of darkness afterward, her eyes are sad and empty. It's almost as if a part of her innocence has been lost. We try to explain to her that she didn't get sick because she was bad, but sometimes these things just happen. One time she asked me, "mommy, if I die, will I still be able to dance like a princess?" My heart breaks to see her pain, at the same time we thank God for every day we have with her, and we know that we are fortunate to have her with us today.

     We are glad to say that Jozlynn is now a very happy, active little girl. She can light up a room and capture everyone's attention by her amazing, sweet personality. She loves to go swimming and go to preschool. She can just about keep up with all of the other kids. In fact, most people can't even tell there was ever anything wrong with her unless they see her "little zipper." She also has such a great compassion for other people, especially when they are hurt. I guess she knows what it's like.

     We are now waiting to see if the right side of her heart will grow because she is on a 1 1/2 vent now(so far no signs of growth). Even if it does, eventually her valve will give up and quit working, and also a hole that needs to be closed. So we are unsure how many more surgeries she will have. For now we are thankful for every moment we have with our little miracle. We are also extremely grateful for everyone who has supported our wishes and made it possible to do all of her surgeries bloodless.

UPDATE:  We went to Portland to see Jozlynn's cardiologist yesterday, and first of all I would like to thank Dr. King who is a very caring and compassionate person. He spent a LOT of time examining her and talking to us. He is absolutely the best cardiologist that there is.

As for Jozlynn's progress, there has unfortunately been no growth at all in the right ventricle. Her pulmonary and tricuspid valves have become more leaky. The good news is that the pressure in the right ventricle is staying in the safe range. Although the valves are looking slightly worse, they are still getting enough blood flow through them at this time. As Dr. King put it, "she is somewhat stable." Therefore, they are not going to re-replace any valves right now.

In less than 2 weeks however, on June 15, 2009, she will be having another surgery. There is currently a lot of shunting between the left and right chambers. They are worried that if she gets any blood clots, because of her valves, the clots would travel through her heart and be shunted through the hole going directly into the brain causing a stroke. That would be a very bad thing. For that reason she is going to have that hole closed. The down side to closing the hole is that it will put more strain on her liver, which is already enlarged. It's like a catch 22; I guess the risk of possible brain damage outweighs the risk of possible liver failure.

Overall, the appointment went OK. He was obviously disappointed at the lack of progress, but mommy and daddy think that no progress is definitely better than a giant step backwards. So it looks like our plans are still on for Disney World in October. YAY!!!

Surgery Update: Surgery went well. It took longer than expected because they also found some other things to fix. They ended up discovering that her right pulmonary artery was severely narrowed again and had to put a stint in it to keep it open. They also were able to put an ASD device in there as well to close the hole and it did not raise the pressure in the ventricle too much, which is excellent. Her Sats(oxygen level in her blood) are looking very good. Maybe this will allow her heart to grow now. The doctors are very happy with the way everything worked out, and she should be feeling a lot better very soon. Now we get to just breathe a sigh of relief and enjoy our little girl. She will need more surgeries, but we are just thankful for every day we have. We know that someday God will make her heart whole. Revelation 21:4

 

 

1- Hypoplastic RV(underdeveloped and thick) as well as high pressure in the RA and slightly thickened wall of LV

2- Absent Pulmonary valve repaired though OHS once then twice through valvuloplasty, then replaced with bovine, (current pulmonary valve insufficiency)

3- Tricuspid valve stenosis(narrowing)

4- Removal of obstruction and Gortex Patch

5- Atrial Septial Defect(hole) Repaired with ASD device

6- Patent Ductus Arteriosis(hole)

7- Hypoplastic Pulmonary Arteries(repaired by balloon catheter several times, finally reparied with stents)

8- Blalock-Taussig shunt made with Gortex (for a great movie about BT shunt watch the 2004 HBO television movie Something the Lord Made  I Highly recommend the movie!)

9- Superior vena cava(artery descending from brain) sewn onto the pulmonary arteries