Emma Ruth

I have decided to attempt blogging again. Since getting pregnant with Emma, I've pretty much abandoned it all together. Between working, 3 boys and a difficult pregnancy there was little energy left to blog. Since Emma is a puzzle we are constantly putting together, I figured it would actually save me time to update her status here rather than text, call and email 50 different people all day long.

I always have interesting pregnancies, and Emma's was no different. At my 20 week ultrasound we found out that we were finally getting our girl, but they also didn't see any fluid in her stomach. That prompted a follow up ultrasound the following week. At 21 weeks they saw fluid in her stomach, but the ultrasound tech thought she saw a hole in her heart. The following day I was sent to Maternal Fetal Medicine to meet with Dr Schemmer who we know and love from many visits while pregnant with the twins.

They did an extensive ultrasound of her heart and couldn't find the hole. While in that ultrasound they noticed she had too much fluid in her kidneys and that I had too much amniotic fluid. I continued to see Dr. Schemmer every 4 weeks throughout my pregnancy. The fluid in Emma's kidneys got worse and worse as did my amniotic fluid. I had 3 times the amount of fluid I should've had. There are different disorders they can link extra fluid to they were muscular dystrophy, spina bifida and down syndrome.

Emma was incredibly active while I was pregnant with her. I think I was kicked more with her than I was with the twins. She was always moving and had a lot of room to do so. It was like water aerobics in there. Because of her activity, which always impressed her doctors, they were able to rule our muscular dystrophy and spina bifida. That left us with a 15% chance that she had down syndrome. We were told once she was born we would know by looking at her if she had it or not. Also, through all my appointments they continued to check her heart to find a hole. They never saw one.

March 15, 2012 Emma arrived! I was 39 weeks and 1 day. I have never been pregnant that long and I've never been so big or miserable in all my life. I had a planned c-section at Orem Community Hospital that morning and shortly after 7:30am she greeted us at 6lbs 15oz, 20 inches long.

Emma did not look like a down syndrome baby. We thought we were out of the woods. The weight was lifted, temporarily.

We know from my previous ultrasounds that we had some issues with her kidneys. They said she had a kink in her tubing that ran from the kidneys to the bladder. There was a chance it would self correct or she may need a procedure to fix it. After an ultrasound 3 days after she was born we discovered that the excess fluid in her kidneys had drastically gone down, she was urinating properly and it appears to have fixed itself. We will most likely do a follow up ultrasound at 2 months, just to make sure that everything is normal.  Another weight lifted.

Saturday March 17, day three of Emma's life, she was tested for jaundice as all babies are. She tested extremely high and was put under lights. She remained under the lights for only one day and her bilirubin levels dropped back down. Jaundice makes babies very sleepy. Emma did not eat almost the whole day

Sunday March 18. She stopped having wet diapers. Her eating improved from the day before, but still no wet diapers. This is also the day her pediatrician heard a slight heart murmur.

Monday March 19, we were supposed to be discharged. Emma still had yet to give us a wet diaper in 24 hours. She was taken to their teeny tiny level 2 NICU to receive some fluids. We decided with her bilirubin levels being higher she became dehydrated. Her pediatrician decided to also run a test on her electrolytes to see what was going on. This came back with an insanely high sodium level, which could've been attributed to dehydration. The plan was to pump her full of fluids and check electrolytes the next day. She was not coming home with us.

In the NICU at Orem Community

Because I was breast feeding Emma and they still wanted me to feed her while she stayed at the hospital. The staff at Orem Community let me stay in my room for free. We just pretended I wasn't there. What an amazing blessing that was for me to stay with her that Monday night to feed her. They also rounded me up some food from the cafeteria for dinner and they have an amazing snack room on the floor that they let me continue to use as well.

Tuesday March 20, Emma is not able to breast feed well. She appeared to be having a hard time extracting milk. The twins had the same problem. I decided to pump milk and feed to her through a bottle, just like I did with Tate and Trey. Her electrolyte levels are unchanged even with all the IV fluid she received. Her doctors then tell us we are dealing with more than dehydration. After analyzing Emma's urine they discover her kidneys are not filtering any toxins. She is urinating water. They then begin to think she in fact has down syndrome. With the murmur in her heart and the problems with her kidneys and the fact they thought there was a chance before she was born they decide they are going to do the chromosome testing. She also needs better care than they can offer at Orem Community and she is transferred to the NICU at Utah Valley. They schedule an echo cardiogram to see what is causing the murmur in her heart.

This was her NICU on wheels

Wednesday March 21 - Friday March 23. Emma is eating well with a bottle. Her electrolyte levels are slowly becoming normal. She is put back under the lights for jaundice. The blood is drawn for chromosome testing and sent to a lab. Her echo is done and comes back with bad news. She has two holes her heart. She has an ASD (Atrial Septal Defect) which we are told is not causing any problems, it will not need repair and she will live with it. She also has a VSD (Ventricular Septal Defect) which will need to be repaired. They then put her on oxygen. Her neonatologist wants to help relieve the pressure in her heart and wants her oxygen levels to be between 92 and 100% at all times.

Emma doing photo therapy for her jaundice

Saturday March 24. Emma's completely removed from her IV and lights. Her levels are normal. We are told that if her labs come back normal on Sunday she will come home. Randy and I spend the night at the hospital to learn how to use her oxygen and heart monitor she came home with.

Sunday March 25 we are discharged. Emma's electrolytes are normal. Her bilirubin levels are still high, not high enough for lights, but high enough that I need to take her in on Tuesday to be retested.

Emma's first trip in her car seat

Tuesday March 27. Emma's bilirubin is retested. It is still high. Babies this old should not still be having problems with jaundice, especially when they have already done photo therapy twice. Her pediatrician, Dr. Anderson, discovers that my breast milk is giving Emma jaundice. We switch her to formula. We haven't had any problems since. Dr Anderson also wonders why we are keeping Emma's oxygen levels so high. In his experience, children with a VSD need to have lower oxygen levels. I had an appointment with her cardiologist for the first time on the 5th. I am told to stay the course and wait and see him.

Emma's chromosomal testing came back this day also. She tested negative for down syndrome. However she has something infinitely more rare. So rare in fact, every doctor she has seen to this point has googled it. She has a disorder called Recombinant 8 Syndrome. You can click the link to read more about it. Apparently geneticists are insanely hard to get into. I currently have an appointment in November. The problems she is having with her heart are caused by this disorder, as were the kidney problems. Thankfully we don't appear to be having any other issues with her kidneys.

I was upset at first about not being able to see a geneticist quickly, but they can't cure her from what she has. We will be taking it day by day regardless of what they tell us. The Dr's at the University of Utah may not have ever seen a patient with her disorder anyway.

Thursday April 5 we meet Dr Hauffman, Emma's amazing Pediatric Cardiologist. He tells us that Emma has a 50/50 chance of the holes closing. If it doesn't close by 6 months he will operate. He also wonders why Emma's oxygen levels are kept high. Dr Hauffman was on vacation until the beginning of April and missed Emma's NICU stay. All tests were done in house and were read by a cardiologist at Primary Children's. All decisions were made by the neonatologist in NICU. He tells me to take her oxygen off. What a happy short lived time that was.

Friday April 6, Dr Anderson sees the report from Dr Hauffman. He doesn't want to take Emma's oxygen off cold turkey. I'm called and told to put it back on and that they are ordering a saturation test. Basically a monitor that tells us what her oxygen levels are. The cardiologist wants her oxygen to stay between 80-90%, but they are worried about her dropping below the 80%, which is lower than most babies are allowed to go, but a lower oxygen level helps the VSD.

Tuesday April 10, I pick up her saturation test. We are also moving on this day. We found a place at the end of March in the exact neighborhood we wanted. Because of the time we found it, we had to give 30 day notice in our place in Orem so we don't have to be out of till the end of April which has been good to give us time to clean and get everything out of there. Emma's breathing started to be very erratic. She had been breathing heavily due to the VSD. Her breathing was the worst when she was in her car seat when we were moving. I hoped that we were just stressing her out with the move..

Wednesday April 11, I kept Emma at our new house all day. That night things became awful. Her breathing was so fast she wasn't able to eat. I started to notice she was sweating while eating. Both were warning signs I was told to watch for.

Thursday April 12, I called her cardiologist first thing in the morning. I was up all night with Emma. She wasn't ever able to get a full feeding so she was up all night either trying to eat or wanting to be held. Her cardiologist told me to take her in to her pediatrician. The pediatrician we saw sent us to be admitted at the hospital. They did an x-ray and found that her heart was working too hard with the hole and was enlarged while trying to compensate. Babies should have between 30-40 respirations a minute. Emma was having 90-110 a minute.

 

 

On Pediatrics

Her hair refuses to do anything but stand up like she stuck a finger in a socket

Emma is breathing so fast because she has fluid in her lungs. The fluid is caused by the hole in her heart. The hole is sending blood from the left side of her heart to the right side. The right side of her heart sends blood to the lungs to be oxygenated.  With that hole some blood is never leaving that cycle and it's creating a fluid build up in both her lungs and heart. They started her on lasix, it should help remove the fluid from her lungs and heart.

Friday April 13 - today Sunday April 15. Emma has taken us on a roller coaster with her breathing. It has definately improved from the day she was admitted. She has moments when she is normal, but she hangs around the 70-80's most of the time with peaks in the 100's still. They won't let her go home until 70 is her highest. They did another x-ray of her heart and it is still enlarged. Which means the fluid is not leaving her lungs or her heart. We are going to give her the lasix through and IV instead of orally. They did that the first 2 days she was here and it seemed to be more effective.

They've also upped the calories in her formula. Normal formula has 20 calories. She is now taking 26 calories. We are trying to give her as many calories as we can with as little effort. It's really hard for her to eat when he is breathing so fast. It also wears her down quickly.

So right now we are just staying the course. Hoping that the medication will work enough that we can take her home. Dr Hauffman doesn't want to operate on her heart until she is 6 months old. That will make the next 5 months incredibly interesting. Now that Emma has started medication the 50/50 chance of her heart closing is pretty much zero. Her Dr said once his patients start medication they usually don't see the hole close on it's own.

Emma is the most adorable baby I have ever seen. I love her to pieces. It breaks my heart to see her poor little body struggle to perform such basic functions.

Emma on Easter

Two days ago, while holding Emma at the hospital, I was telling her how much we missed her and wanted her to come home. This is what she did

I think she was trying to tell me something

And now one last picture. Because I just love to show off my beautiful girl

 She cracks open one eye when she sleeps during the day. She's trying so hard not to miss anything.