Izabell after her heart surgery about 7 weeks old

PHACES Fundraiser July 22nd 2008

We are excited for the fund raiser tomorrow and hope that everyone will be able to enjoy some good food!!! Remember it is at Tutta Bella (Stone way location only)

Tuesday, July 22!

Help Support the FACES of PHACES
by dining at Tutta Bella July 22nd portion of your bill will help support the

Faces of PHACES.

Faces of PHACES is dedicated to funding the first ever PHACES conference in November 2008 and to help fund medical research specifically for PHACES Association. They are committed to increase the awareness of PHACES Association and to support afflicted families.

To learn more about PHACES please visit:
www.phacesassociation.com
www.novanews.org/PHACES.htm
www.phaces.org

Tutta Bella Stone Way
4411 Stone Way North
Seattle, WA 98103
206.633.3800
11:00 a.m. –- 10:00 p.m

Izabell was born in March over a week late after an uneventful pregnancy. She was 8 lbs 15oz! She had a small birthmark on her cheek. The doctors said it was bruising and would probably go away. The doctor checking her in the hospital noticed her hips clicked and told us to have our pediatrician check them on her two day check up. She said that she probably has Congenital Hip Dysplasia. Izabell looked great and we spent 24 hrs in the hospital and then we all got to go home. Izabell's 2 day check up came and they did diagnose her with congenital hip dysplasia and referred us to Seattle Children's Hospital. At that visit her doctor also told us that she might have a port wine stain on her face and we would need to see a dermatologist sometime in the next couple of months. Izabell had lost a little weight but that is normal for an infant in the first couple of days. They said she looked good. At two weeks we noticed she was working harder to breath and took Izabell to the doctor again. The doctor on call told us she just had a cold. So we were sent home armed with saline and a bulb syringe. Izabell was nursing every hour for about 10 minutes at a time but she seemed to be gaining weight so no one was worried.

At about three weeks I noticed her feet looked a little puffy. No one else thought they looked bad, so I brushed it off as my being tired and a new mother. Izabell was put in a Pavlick harness for her hip dysplasia at three weeks of age. The harness went over her shoulders and wrapped around her legs holding her hips at a 90 degree angle to help her hip sockets develop. She was to be in this 24 hrs a day. I could no longer see her feet because of the harness nor could I undress or bath her. She started sweating a lot at this point. She also started to bob her head when she breathed. Izabell's hands started to look puffy to me at around 4 weeks old. I called a nurse at her pediatrician's office and told her I was worried. I told her about Izzy's hands turning cold, swollen and a little purple. She told me that newborns had slow circulation and that I was just worried being a first time mother. She said as long as they can warm back up she is probably fine. She said that the hip harness is probably too tight and making her sweat. I was annoyed at this point but her hands could warm up and the nurse did not think Izabell needed to be seen.

Izabell was being followed by the orthopedic surgeon for her hip dysplasia at Children's Hospital and had an appointment the next day so I knew she would be seeing a doctor and decided to ask him his opinion about her hands and the sweating. I knew that the harness was not too tight because I could slip my fingers between her and the harness. At the appointment her orthopedic doctor kept looking at her neck because of the way she was breathing. He also noted her swelling and asked me what the doctor had said. He agreed that the harness was not too tight. He did have me change Izabell's shirt and thought that it was too tight and the culprit for her swollen hands, I told him that it was not too tight a day ago and that something was wrong (later we found out that she had extra fluid because of heart failure). He told me to come back in a week because he wanted to check her again. In that week Izabell’s “cold” was getting worse. She would start to cry and then grunt and then she would breathe normal again. I do think everyone was starting to think I was crazy. I thought about taking her to the ER but I didn't take her because she was going to the doctor in a couple of days. People kept telling me that I was a new mom and just nervous (that was true, but I knew something was wrong). Izabell had been surrounded by doctors at the hospital and I thought that if anything was significantly wrong someone would have noticed. She was also around her grandfather who was a doctor and her uncle who is a pediatrician so if something was wrong I thought they would have noticed.

She is a sneaky little girl :). She did not have a lot of warning signs and the harness hid most of her swelling.

The next week at her orthopedic appointment the doctor said that she was looking very swollen and he thought it would be a good idea to run a blood test. He said that she might have something wrong with her thyroid. He said that he is an orthopedic doctor (bone and joint specialist) and so he would give the results to her pediatrician. He promised that he would call me later that day and tell me what the results where. When they did the heel poke on Izabell she did not cry she just stared at me. They had a very hard time getting any blood but after three pokes and a lot of effort they did get enough for the test. Izabell did not cry once. I thought that was very odd and they said that it was strange and she was just really good. Around three that afternoon the doctor called as promised. He told me that there was definitely something wrong with Izabell’s blood work and that I needed to bring her to the hospital. He said the ER knows you are coming and they are waiting for her. I was so scared at that moment. He said something might be wrong with her liver or kidneys but they would have to see her in the ER to figure out what was really going on. I drove to that ER praying that she was going to be all right. Now I wonder why they didn't tell me to call an ambulance but I guess at that moment they did not know what was truly going on with her.

So at six weeks old I rushed Izabell to the Hospital. When I pulled up to the door they took Izabell out of my arms and checked her stats (oxygen saturation). They put her on oxygen and started calling other doctors into the room. Her heart rate and oxygen saturation percent were very low. She had no pulses in her lower body at all but her upper body had weak pulses. At the time I did not understand exactly what that meant; I just knew that she was seriously ill. Her father showed up shortly after we got to the ER. They did a chest X-ray and found that her heart was enlarged. An Echocardiogram, EKG and more doctors followed. They told us that Izabell was in heart failure and that Izabell has a congenital heart defect. I have never been so scared in my life. I have never cried so much. They explained to us that she had “Interrupted aortic arch and a ventricular septal defect”, and told us that without surgery she would not make it.

I was able to carry her and hold her while they were setting up a room in the CICU (Cardiac Intensive Care Unit). I walked into the room and set her on the bed and knew that I was not going to be able to hold again her for a long time. They gave her some medicine to try and open her PDA (Patent Ductus Arteriosis) but it did not work, so a day later Izabell had a catheter angioplasty to open a path for blood to get to her lower body and during that procedure they took images of her heart’s anatomy. They told us that a baby with this heart defect is usually critically ill in the first week of life. They were surprised Izabell did so well for so long. Izabell was now six weeks old!

The catheter angiogram showed that Izabell also had an arterial defect that went from her heart to her lower aorta (past were it was interrupted) and it was giving her body a small amount of oxygenated blood. That is why she did so well for so long. They waited a while to let her heart and body lose some of the excess water and get stronger before the high risk surgery. Izabell had open heart surgery a week later. That was one of the hardest days. Izabell was in the OR for over nine hours. At the end of her surgery one of the two the surgeons operating on her came out to tell us that the repair was done but Izabell's heart was having trouble beating and they might put her on ECMO (Extra Corporeal Membrane Oxygenator - which is like a smaller heart lung machine). I was so scared. Izabell heart was so weak from being in heart failure that it was having trouble starting back up after they had stopped it. While the surgeon was explaining how ECMO worked and how they were going to hook her up to it, the other surgeon called and informed him that Izabell's heart had started to beat stronger and that they did not think ECMO was needed. I was so relieved and still very scared. They said that the next 72 hours would be hard and if she made it through that it would start to get better. That night Izabell slept with the ECMO machine next to her bed just in case. She had an open chest and was very swollen. We were allowed in the room a little while later and I was in shock from how she looked, but so grateful to be able to see her again. That night was very hard and Izabell’s CICU room was full of doctors and nurses working to keep her stable. Izabell had a hard recovery, but she did get stronger every day. She developed chylothorax from the surgery and was put on a special diet. Izabell also had heart block from the repair. After the surgery Izabell's birthmark turned bright red and was spreading across her face. The ICU doctors worried that she had a clot in her neck and I told them it was a birth mark but they ordered ultrasounds to check for clots anyway. No clots, they told us the birthmark is a hemangioma. A week later Izabell went back to the OR to have her chest closed. A week after that Izabell had the breathing tube out and for the first time in 28 days I was able to hold my daughter. She weighed 9 lbs. She had an external pacemaker at the time. It was a little scary holding her and the pacemaker, but I could not wait any longer my arms needed my baby girl. A week later Izabell had an internal pacemaker placed. A couple days later she was moved to the surgery floor and for the first time in a very long time I could hold my daughter without any wires hooked to her. A week later we got to take her home.

Izabell breathing was noisily but we were told this was from vocal cord damage from the breathing tube. When her chest healed Izabell went back into the Pavlick harness to help her hips develop. Her hips responded well to the harness and she came out of it when she was 5 months old. Her hips are getting stronger and she is starting to crawl now!

When Izabell was 14 weeks old when I took her to the dermatologist for the first time. Her dermatologist diagnosed her with PHACES Association. I was so scared to hear that there was more that we would be facing. She told me that Izabell needed to see an ENT because of the way the hemangioma was spread across her neck and mouth. She was concerned that Izabell had some hemangiomas in her throat. Izabell saw an ENT the next week and was put on oral steroids and was scheduled for an angiogram and CT scan. She cannot have a MRI/MRA because she has a pacemaker. The CT showed that Izabell had normal brain development, showed some deep hemangiomas in her airway and also some narrowing at her repair site in her aortic arch. Izabell had laser surgery on her airway to remove some hemangiomas from her vocal cords at 4 months. She had another surgery to remove a Subglottic hemangioma at 9 months. She has been on steroids since she was about 4 months old and is still currently on them.

She also had another catheter angioplasty to stretch her aortic arch suture line because it narrowed and was causing her to work harder. Her heart function was poor after her surgery. Her heart has been getting stronger every day. At five months her cardiologist said her heart function was on the low range of normal (he dad and I did a little dance) and at her last appointment they used the word terrific to describe her heart function! Some valves in her heart and her arch are still being watched very closely but she is doing very well now! She has an appointment with cardiology every 8 weeks now.

Izabell had a swallow study when she was 7 weeks old and was unable to swallow thin liquids without aspirating them. She had a NG tube and I was taught how to feed her that way till she got a little stronger. She could swallow thickened liquids but needed to get stronger to be able to suck them from a bottle. Izabell had a feeding tube for about 6 weeks and then started taking enough formula by mouth to switch over to bottle feeds. She has not yet been able to swallow thin liquids without aspirating them, and we are still not sure why. I nursed Izabell until she went to the ER. I started pumping the night. I pumped for about 6months trying to give Izabell the best start. My body starting having trouble producing milk and I eventually stopped pumping. It was very hard to stop and let the idea breast feeding go. She is on a special formula and is doing well on it. She is starting to eat solids and seems to do well with them. We are hoping as she gains strength she will be able to swallow thins.

She is being followed by all the doctors closely and is still on steroids for the hemangiomas. She is being referred to a pulmonologist because without the steroids she still has some trouble breathing. She is almost ten months old and has had over 6 surgeries. She has occupational /physical therapy and speech therapy once a week.

People have asked me if I am angry with any of the early doctors and nurses who did not catch Izzy's heart defect. My answer is no. Izabell’s heart defect was tricky to catch. She did not have a murmur that was audible. We have the same pediatrician and we think he is great. I have learned that just because a doctor or nurse doesn't think you need to be seen doesn't mean you have to accept that. I have learned that I have the right (with out offending anyone) to tell them that I am not OK with waiting and I want Izabell seen. Izabell’s health has been a learning experience for everyone including her medical team. We have had a lot of scary nights, learning experiences, and also wonderful moments with Izabell. I have learned to trust my judgment and to never let someone tell me I am paranoid about my daughter. Every time I call her doctor’s office he listens and sees her now. She has had plenty of trips to the ER, some that are not too serious but there is no way I am going to ignore my feelings again. Parents, Mothers, know their children better than anyone else. If you think something is wrong, act on it. It may save your child's life. My daughter was lucky that someone finally listened and that we were at a wonderful hospital. She is a very happy easygoing little girl. She smiles at everyone she sees. She is starting to talk and crawl.

Izabell's update

Izabell had another hemangioma removed from her airway in May and June. She has had three laser treatments on her superficial hemangiomas during the summer to prepare for a nasal tip hemangioma removal that was done in October. Those surgeries went well. She has been off steroids since about
July of 07! Her airway is still narrow due to the hemangiomas but she has been breathing well. If her airway starts causing trouble again we are told she may need an airway reconstruction surgery. We hope that she will not have any problems and can avoid this. Her last surgery was in late September! That was four months ago!! This is the longest she has gone without needing surgery and she is doing great! She used to get out a breath very quickly. She would pant when crawling and was low energy. After the surgeries she has enjoyed a much higher energy level and at 18 months old started walking!!!

Her heart has continued to get stronger and she is now seen every six months! She has some areas that need to be monitored and will be on medicines and pacemaker dependent her entire life. She takes all her medicines daily and does a wonderful job taking them! It is natural for her to take them. Her hearing and eyes are followed regularly and are doing great! She was prescribed glasses at 11months and prefers to have them on. She has some hearing tone loss in her left ear due to the hemangiomas on her ear drum but not a concerning level. She is seen by a wonderful pediatric neurologist who has put together a team of doctors to help with preventive care. She has had Doppler imaging done in order to view her cranial vascular abnormalities. We hope that this will help her medical team follow and treat her if any concerns arise. She cannot have an MRI because of her pacemaker so Doppler is currently is the safest way to follow her.

Izabell just celebrated her second birthday! She knows some sign language and is talking much more now! She is playful, social and very smart! She loves music, dancing and has started taking toddler gymnastics!

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wyndi says ... on Sunday, Dec 21 at 2:36 AM

www.althingsizzy.blogspot.com

Jessica says ... on Wednesday, Jul 23 at 10:15 AM

So happy she is doing so good! Reading the story cried for her, for you, and joy that she came through. I saw her pictures and what a beautiful baby. I just had my first in April I can only imagine what you went through what a loved girl good luck!

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