Elyse Was born on Friday, June 16, 2006. I was induced at 36 weeks due to my family history of pre-eclampsia. I had been at the hospital since Monday morning.
Elyse weighed 7 lbs. 9.3 oz. If she had been born full term, she would have been 9-10 lbs! Elyse has a healthy brother born Jan. 12, 1996.
We were discharged from the hospital on Saturday, June 17, 2006. The hospital pediatrician checked her around 8 AM. He explained that he heard a "slight murmur" in her heart. He assured us that it was very common and not to worry. That afternoon he took Elyse and did an echocardiogram.
At the time, we had no idea was that was and it didn't occur to us to ask. We were so exhausted and he kept telling us not to worry that we didn't. He returned Elyse and told us to go home and come back in 2 weeks. He was going to be out of town and would later discuss the results. Again, he told us not to worry. We believed him.
Jun. 19 - I was supposed to schedule her first appointment. The hospital pediatrician were she was born at wanted us to see a colleague of his for her first week checkup, but I decided to take her somewhere else. I started to notice that Elyse wasn't nursing as often as she should. She was sleeping more and would have crying spells at night. I didn't think much of it and thought maybe every child is different. I also noticed that her eye lids were a bit swollen, but people around me told me that it was normal.
Jun 20 - We take her to see the pediatrician at Town & County Pediatrics. Everything was going smoothly until I mentioned that the last pediatrician heard a "slight murmur". Well this doctor checked and she said it sounded very loud. She asked if we could stay longer because she wanted to get the medical records from the hospital she was born at. We didn't mind. Meanwhile, she had two nurses come in and try to get her blood pressure and her pulse-ox. At the time, I really didn't understand what was going on. The doctor didn't have any luck in getting her files and, because the nurses weren't able to get a pulse from her legs, the doctor called a cardiologist from Children's Memorial Hospital. He told her to send us immediately. We were shocked. The pediatrician told us that the cardiologist was very good and he would do another echocardiogram. She said she would feel much better knowing we had a second opinion.
Off we go to see the cardiologist. We told each other "well the other doctor said not to worry". Plus my husband had family with heart murmurs. Boy were we naive. They asked us a million questions. One of them asked about her eating habits. I thought she was ok. They just nodded. The cardiologist took us to the basement and did the echocardiogram. That was the first time we actually saw it. Wow. It was just like a ultra sound machine. The cardiologist took about 45 minutes. Elyse started getting very fussy, he asked me to nurse her because he didn't like what he was seeing. My heart stopped. After the doctor left, I leaned over to my husband and asked him if he heard what I heard. I tried to nurse her, but she just wouldn't. It had been over 4 hours since she had eaten.
The cardiologist returned and continued. Afterward he asked us to step out and return to the waiting room. We waited half an hour and when he returned he gave us the news that I will never forget. He grabbed a pad and pencil and started drawing and talking. I didn't understand a word he said. What I clearly understood was "MORTALITY RATES". I reacted. I cried and cried. I only understood that if Elyse didn't receive medication and have surgery, she wouldn't make it and if she did, she would have severe problems. When we told the cardiologist what the pediatrician at the hospital Elyse was born at said. He told me, "Well good thing you didn't listen. You saved her life." You can only imagine how I felt.
That same day Elyse was admitted at four days old to the NICU at Children's Memorial. After many tests she was diagnosed with Coarctation of the Aorta. She also had Ventricular & Arterial Septal defects (VSD, ASD), Patent foramen Ovale (PFO) and Patent Ductus Arteriosus (PDO). She would require two surgeries. I cannot tell you how many times I had nurses and doctors tell me how I had saved my baby's life. That my motherly instinct saved Elyse's life. I could not control my tears. I keep thinking, what if we had listened to that other pediatrician and waited two week to see him about the results. Our baby could have died! Till this day, we are angry. I should also mention that the pediatrician from the hospital were she was born at never called us with any results, which is very upsetting because he told us he was an experienced cardiologist. If he was "experienced" then why didn't he see that her heart was not normal? Our pediatrician called his office and the hospital to make sure this doesn't occur again.
The first surgery was done when she was 2 weeks old to correct the Coarctation. During those first two weeks, we waited and waited to speak to CV Surgery. The Morning before her procedure, her pediatrician called. She knew we hadn't spoken to anyone and how frustrated we felt not knowing what their plan was. She explained how the surgeon didn't have a plan yet. This procedure was very complicated. I think she was trying to prepare us for what could happen. My husband and I just laid in bed holding each other quietly. There was nothing to be said. That afternoon we finally met the man that would operate our baby. It was awful. The surgeon gave us little hope. He explained to us that her entire vascular system was abnormal as well as her heart. In the twenty years he had been there, he had never seen anything like it. I wish I had a picture, but we don't. The day of her surgery was very emotional. My husband and I decided to be alone. I still remember when the surgeon came out and told us she had survived and was able to correct the major problem. He wasn't able to repair everything, but he fixed the Coarctation. The second phase of the surgery would be done once she gained some weight. We were happy just happy she was alive.
Three weeks later Elyse was discharged. Elyse was now 6 weeks old. That same night, I started noticing her poop was a little red. The next day, we noticed it much more. I panicked and called the cardiologist on call. He said, that it could be her feeding tube and not to worry. The third day was worse and the smell was strong. I scheduled an appointment with her pediatrician and they tested it for blood. Sure enough it was. Her hemoglobin was at 4. It was extremely low and her skin tone was pale. They immediately called an ambulance and off to the hospital we went. She was admitted to the PICU and they immediately called her cardiologist. The nurses were having a very hard time getting a vein, so they couldn't draw blood or do anything. One of her CV surgeons arrived and tried doing an IV line. He couldn't. She was scheduled to have one the next day. Her cardiologist told me it was a miracle she was doing so well. Her heart was good and her vitals too. After that, she wasn't allowed to eat. They needed to figure out why she was bleeding.
The next few days, they did an ultrasound, MRI and CT of her stomach, then head. That's when we found out that she had many internal and external hemangiomas. Her liver, spleen, GI tract, eyes, eye lids and bottom lip had them. She was started on Prednisone (steroids) and within weeks, the bleeding stopped. They also found that she had many abnormal arteries in her head. She had suffered a minor prenatal stroke and is at high risk of having another stroke. She was also diagnosed with PHACES. At that time, we didn't know how severe things would get.
The second surgery was done when she was three months old in September 2006 to close the VSD and ASD which were causing the many blue episodes. Initially, after her first surgery, they told us they would do it at 3 months old. After she was diagnosed with PHACES, they decide to wait longer until they were sure the bleeding was under control and she had gained enough weight. Elyse had been without feeding for many weeks throughout her little life that she was very skinny. They needed her to be strong.
Elyse had other plans. Toward the end of August and beginning of September, she had many blue episodes, where she seemed to stop breathing and took very long gasps. Her first episode freaked us out. My husband and I were alone with her, at the house we had just moved into in July. We had also been meaning to take CPR classes, but hadn't yet. When she had that first episode, we scrambled, trying to calm her, holding her different ways to see if she would calm down. She just kept getting worse, and gasps were getting shorter and longer in between. We were so afraid that she would die in our arms. Waiting for the ambulance seemed eternal.
Her last blue episode, she wasn't able to regain her breathing rhythm so they had to intubate her. That was the day they decided she needed to have this second surgery ASAP. Again, things got very complicated. This was supposed to be the simple procedure, but because of her hemangiomas she now was in high risk of bleeding to death and having a stroke. But she did well! Again her surgeon said that he was very surprise that she had done so well.
After her second heart surgery she started having trouble getting off the ventilator. Another CT was done and was discovered that her hemangiomas were growing. She now had one compressing her right bronchial and it was giving her a hard time with her breathing again. She also had another one near her spine, at risk of leaving her paralyzed neck down. A meeting was scheduled. Present at the meeting were hematology, dermatology, neurology, a hospitalist, and a pediatrician. They all agreed that Interferon was the best treatment. We were informed of the high risks, which I wasn't happy about, but it was something we needed to do. At this point, Elyse's condition was life threatening. I consulted with another doctor in Ohio and she told me that I had a very good team. That made me feel better. Elyse was on interferon for two months and being weaned off the high steroids.
In November 2007 she was having digestive problems. Because of her heart surgeries Elyse's vocal cords were paralyzed and she aspirated any liquids given by mouth. Everything had to be given by NJ tube. Another CT was done and once again her hemangiomas had grown. This time the hemangioma in her GI tract was pushing on her stomach. Her hospitalist and hematologist have consulted with many doctors around the world. Interferon was stopped and Vincristine (Chemo) was started, and they also restarted the high dose steroids.
On Thanksgiving day, I was told that if this treatment did not work, if they could not get these hemangiomas under control by any other treatment, Elyse would not survive.
Since then, Elyse had a central line put in (for the chemo) and had a G J - Tube placed to help relieve some of the discomforts and reflux she was having with the NJ & NG feeding tubes. Her vocal cords were damaged even more after her second surgery and we don't know if she would be able to talk. Only time will tell.
Our little girl has been through so much, yet she continues to be strong. She has been hospitalized most of her life. The longest she's been home was three weeks without an ER visit. Right now, we can only hope and pray that her hemangiomas do not continue to grow.
Elyse has the following:
Hemangiomas -
INTERNAL - GI tract (retroperitoneal, mesenteric, cervical cord); liver; spleen; around the right mainstem bronchus; along the thoracic spine extending into the spinal canal & extraconal within both orbits.
SUPERFICIAL flat - left eyelid; bottom lip & gum; scalp; forehead
Arterial Anomalies -
Isolated left subclavian artery originating from ductus arteriosus; hypoplastic internal carotid artery and tortous posterior cerebellar artery.
Cardiac Defects -
PFO; PDA; VSD; ASD; Double aortic arch with Coarctation 6/28/06; 9/14/06: S/P VSD repair, PDA ligation, PFO closure, and resection of aortic stenosis with graft placement.;
Eye Abnormalities -
Strabismus - intermittent exotropia; some Astigmatism
Other Medical Issues:
GERD; feeding problems due to vocal cord paralysis (total right side; half left side) & Tracheamalacia. - Gagging; desat when she sleeps. Prenatal stroke; possible aneurysm; 100% J tube fed.
Treatments:
Oral steroids: (4 weeks old) 7/16/06, started weaning at 10/18/06
Interferon: (18 weeks old) 10/18/06 - 12/13/06 - Due to the rapid growth and life threatening hemangiomas.
Vincristine: (6 months old) 12/14/07 - 12/27/08
Oral steroids: 12/14/07 - 12/12/08
UPDATE: JANUARY 2008
One year later and Elyse is finally home and doing well. Our baby is now 18 months. In the last 7 months, with the dedication of Early Intervention... She has learned to sit, crawl and now is cruising around our living room. We are so proud of her.
It has been a rough 18 months. One year ago, we were very scare of losing her. She began chemo to control the growth of these hemangiomas that were threating her life. In May, the hemangiomas in her eyes & liver disappeared. The others were still the same. Now she's done with the treatment. Two weeks ago, she had her last chemo. Last week, she had a chest & stomach CT. We are currently waiting for the results.
It's amazing when I think back of everything we all went through. I did some calculations. Out of 516 days, she was in the hospital for 294 days of her life. Wow! She has currently done quite well compared to last year. She is still 100% tube fed. We still have a million follow ups... but things are good. Now we just pray that things continue to go well and really, only time will tell what's in store for us.
