Stationery card

Hopscotch Time Blue Birth Announcement

Birth announcements, Valentine's cards, & invitations by Shutterfly.

View the entire collection of cards.

2 weeks at home

We have been at home for 2 weeks (tomorrow) and all continues to go well! Last week we had our first metabolic clinic appointment where we met with the geneticist, genetic counselor, and dietician to discuss Evan's progress and course of treatment. Everyone continues to be pleased with his progress and he is continuing to put on wait! He was 7.2 lbs at metabolic clinic last Monday and today at his weight check and blood draw he was 7.92 lbs! Eating and gaining weight like a champ! (Both are VERY good signs).  Right now we will continue to do weight checks and blood draws every Monday and then meet monthly with the genetic team. After each weight check, the dietician looks at the information and determines how much protein Evan should be getting. Right now he gets his protein from breastmilk, but we will soon switch to a regular infant formula as the pumping and feeding is not going as well as I as was hoping. The geneticist gets the results of Evan's bloodwork and determines if we need to continue to supplement the acids isoleucine and valine, then tells us how much of each we are to give him.  This bloodwork also helps to determine how much protein Evan should eat.

 

Evan is growing and changing every day! He LOVES his playmat. He lies there and looks at the lights and listens to the music. When it stops...he starts to cry to get you to start it for him again. He has startled himself a few times by actually hitting the toys that hang above him.  He also likes his swing a lot. He will calm easily when he swings and he also likes to just look up at the mirror and mobile on his swing. 

Aunt Michelle and Uncle Jason with Evan

Aunt Michelle (JR's sister) and Uncle Jason came for a visit last week. We enjoyed having them and I know they enjoyed getting to meet Evan. We got a chance to go out to the botanical gardens and have Aunt  Michelle take some pics of Evan with the flowers and for our birth announcements. They turned out super cute! (Thanks Michelle!)

My 512th Ladies and I on Spouse Day

As for JR and I, we have been adjusting to our new schedule with Evan and trying to sleep when we can. JR is back at work, so night time feeds often fall on me, which is okay since I can try to nap during the day if need be.  On Friday I got to go on a spouse flight FINALLY! (I kept missing them when we were stationed at Moody).  It was so cool to finally get to fly in a helicopter and see what my husband does each day! What an amazing view he has from his "office" in the sky! :) I will definitely fly again when the chance arises!

Easter morning before heading to our friend's house

We celebrated Easter this year with some friends by going to a BBQ.  It was small, but nice to have friends to spend time with for the holiday.

 All continues to go well here and we hope you can say the same!

Home Sweet Home!

We got to bring Evan home from the hospital on Tuesday! YAY!!!!!! We still have to monitor his diet closely (as we will for the rest of his life) and we have to make sure he doesn't start acting lethargic and refusing to eat, but we are home! We will be attending weekly metabolic clinics and weight checks to determine if his diet is sufficient or if we need to make adjustments. Obviously with little ones growing so quickly, we need to make sure he is always getting the right amount of nutrients and just enough protein...not too much and not too little. It will take a lot of monitoring, but right now I am just happy to have my baby home!

The last couple of days have been an adjustment, but they have gone well. JR and I are sharing the night time feedings which aren't too bad since Evan is eating every 3 hours.  He is a fairly quiet baby, so we often have to set an alarm to get up to feed him at night, but as soon as he is up he is ready to eat!  The squadron has been awesome and are providing us with meals for dinner so we can get adjusted before having to take on all the houshold responsibilities again.  We are so blessed to be a part of such a supportive squadron! I have met a lot of other people who don't have the same support.

Evan is still eating like a champ and seems to be getting bigger! He is such a love bug and loves to be held, but he doesn't mind being put down either! Aside from his dietary needs, so far he is an easy baby. We are truly blessed.

Thank you all for your thoughts, prayers, and support while we were in the hospital. We will still need them to make sure he continues to do well, but so far so good!

COMPLETELY unhooked!

Things have continued to progress in the right direction! Evan has been doing very well with the bottle. He took the majority of yesterday's feeds from the bottle and we only had to use the tube a couple of times. He has put on a little bit of weight and is up from 2.995 Kg at admit up to 3.12 Kg last night. We will see at 11:00 tonight what his new weight is!  Evan has continued to do well with his feeds today, so the doctors removed his feeding tube! He is now hooked up to NOTHING and is doing everything for himself.

His leucine, isoleucine, and valine (the amino acids his body can't break down) levels are down around the normal range.  To keep them there, Evan is taking a few bottles of breastmilk per day and the rest of his feeds are the MSUD Analog formula (a special formula for MSUD patients which does not have leucine, isoleucine, and valine, but has other vitamins and minerals needed to grow and function).  We already have some of his formula at home because the dietician here ordered it and had it sent to the house. His formula has to be specially weighed and mixed, so the dietician gave us a scale today that we can use when preparing his formula each day.  Plus we talked to the insurance today and found out that his formula will be covered by the insurance. What a blessing because it is VERY expensive.  As of right now, Evan's isoleucine and valine are being supplemented in a small amount to keep them at the normal range, but we are hoping that soon we won't even have to do that because the breastmilk will hopefully give him what he needs.  We may need to continue these supplements at home depending on the results of his amino acid tests, but we will cross that bridge when we come to it. 

This is Mommy's favorite time of day! After
a morning feeding and getting to cuddle and read!

Since Evan is doing so well with his feeds and his labwork is coming back the way we want it, the doctors are thinking they will release us tomorrow. That is, of course, as long as Evan continues to eat well tonight. We are definitely looking forward to going home, but at the same time it's a little nervewracking. Here we have the doctors and the nurses to make sure all is well. Once we go home, it's all on us. I am sure we will handle it, but it is still a little scary.  Once we do go home, we will have to make sure Evan is weighed weekly because with as quickly as babies grow we need to make sure that we are continuing to give him the right amount of good proteins so he can continue to thrive.  In addition to weekly weights, we will have to attend something called "metabolic clinic".  This will encompass us meeting with the metabolic specialists, learning about Evan's best treatment, support from others, genetic counseling, and dietary management skills.  We will attend weekly clinics for a little while, until we really get into a routine with everything and fully understand how Evan's weight will effect his feeds.  We are very comfortable and confident with the care that we have and will continue to receive from the metabolic team. We are also very pleased with the care we have received at UNM Children's Hospital. The staff here has been incredible!

 Keep saying prayers that Evan does well and hopefully we will head home tomorrow!

Learning to Eat from a Bottle

Most of us would think that a baby eating from a bottle is an easy task and something they just know how to do. Often that is the case, but sometimes babies have a hard time with it. Evan didn't really have an issue with it until he started refusing to eat because of the MSUD and now having had the feeding tube for a while. We reintroduced the bottle to him a couple of days ago. At first he wasn't really sucking on the bottle and would only take about 10 mL.  The geneticist and the dietician have set up a specific feed amount that he is to take each day and divided it into 12 equal feedings. That means right now he is feeding every 2 hours! He gets breastmilk from a bottle 3 times a day. This helps to keep his amino acids in a normal range instead of dropping to nothing. He gets 55 mL of breastmilk at each of his 3 feedings. The rest of his feeds are MSUD formula and he gets 40 mL of that at each feed. Anything Evan doesn't take from the bottle within a reasonable amount of time we place into a syringe and feed him through his tube.  This is to make sure he is getting the calories he needs.

Twice today he has taken his entire MSUD feed from the bottle! YAY! Most of his feeds are averaging about 25 mL from the bottle and the rest from the tube. This is a great start and it seems he is getting better and better with the bottle! This is good because the sooner he is completely on bottle feeds, the sooner we get to go home!

Daddy gave Evan a bath today while Mommy went to lunch with some friends from the squadron. It was good for Mommy to get out for a little bit. Daddy said Evan enjoyed his bath. After his bath his hair decided to stand up in a mohawk! Too cute! :)

Evan's leucine levels are normal and now it's all on him to take the bottle. It's just a waiting game. When he is ready to go home he will tell us by taking more of his feeds through a bottle. Evan is our "little warrior" and he is doing great! :)

Unhooked

Evan was unhooked today! They took off his EKG monitor and his pulse monitor. They also removed his oxygen.  He is now only attached with his feeding tube and that only runs every two hours!  His feeding tube has now been pulled back from his intestines and is back in his stomach. We are feeding him every two hours by bottle and whatever he doesn't take by bottle is fed through his feeding tube in a 30 minute bolus.  Feeding this way essentially mimics a natural digestive process and gives him a chance to get hungry.  He is doing okay and has taken at least a little bit at each feeding by bottle. Hopefully we make more headway with the bottle feeding tomorrow. The sooner we are on only bottle feeding, the sooner we can go home!  We got word today that his leucine levels are still going down and are quite close to where we need them to be. WOOHOO! His body is doing its job and fighting these amino acids! 

Evan is responding well to sounds and is gazing at his mommy and daddy while being held or fed.  He really likes all kinds of music and stops what he is doing to take it in when he hears music of just about any kind. Daddy figured this out when the bagpipers were playing outside and Evan stopped sucking on his paci and turned his head toward the window from which the sound was coming.  These are all good signs that the amino acids have hopefully not caused any neurological damage. We had a few vistors from our military family today. We couldn't be getting more support from the squadron! Thank you so much to our 512th family for being here for us! And thank you to our 41st family (from Moody) for the beautiful flowers! It's so great to know that you are thinking about and praying for us. Once a part of our rescue family, always a part of our rescue family! :) Love you all!

Today was a good day

Evan's leucine (that's one of the amino acids he can't break down. It's the worst of the 3) levels are dropping as they should be and he is becoming more and more alert. He spent more time awake today than he has in his entire 2 week and 4 day life. It was so wonderful to be able to look into his beautiful blue eyes! Seeing them helps to realize that everything is going to be okay!  He has also been starting to take formula from the bottle. Considering he is still on a constant drip feeding tube at 21 mL per hour, the fact that he still wants to eat is a good thing. Hopefully that means that when we stop feeding through the tube he will realize he is hungry and eat all his meals from the bottle. At this point, the goal is to get his leucine levels down to where they need to be.  If things continue to progress as they have, I am guessing that will be in 2-3 days.  Once his levels are down where they need to be, the next goal is to get him feeding completely from the bottle. We will probably start this by feeding him with a bottle and whatever he doesn't eat in that feeding will be given to him via tube in a bolus form. This will mimic the normal feeding pattern where food enters the body at one time and then is slowly burned off eventually leading to hunger.  This is much more like the body's natural process of feeding than a constant drip feeding tube. Hopefully he does well taking from the bottle and we don't have to spend too much time relying on the feeding tube.

During this time we will be working closely with the dietician and the geneticist to determine how much and how often Evan will need to eat. He will be getting both his special formula and either breastmilk (I have quite a bit pumped and frozen) or regular formula.  The dietician will help us to determine how much of each he will need to keep his levels in the normal range.  Once he is eating fully from the bottle and we have his diet figured out we will get to go home. We are expecting to be in the hospital for at least another week, but at this point there finally seems to be a light at the end of the tunnel.

Thank you all for the messages, thoughts, and prayers.  It is so wonderful to know we have such an amazing support system of family, friends, and of course our military family as well.  Keep the prayers coming; they are definitely working! :)

It's been a whirlwind of a beginning!

As you all know, Evan was born on March 19th. He was born a seemingly healthy baby who just liked to sleep a lot and was a finicky eater. We (and the doctors) chalked it up to my being on magnesium for so long during labor and then having a c-section. He lost quite a bit of weight (11%) in the hospital, but then he started eating better.

We were released to go home on Tuesday March 22nd and he was doing much better. Still VERY tired. Rarely opened his eyes. We had a doctor's appointment two days later to check his weight and he had gone up 2 and a half ounces from discharge. We were excited he was gaining weight. The doctor said his reflexes, etc... were all normal.

Fast forward to Friday the 25th. We get a call from the pediatrician and she told us that the results from Evan's newborn screening had come back and Evan may have a condition called Maple Syrup Urine Disease (a 1 in 185,000 genetic metabolic disorder). She told us to watch for him to refuse to eat and to become completely unresponsive. They couldn't do further testing until Monday because the labaratory they needed to send his labwork to was in Oregon and doesn't accept labwork over the weekends (Go figure!)

Over the weekend Evan wasn't eating well, but he was still eating. He was still reacting and crying when he was wet/hungry. He still only opened his eyes a few times a day.

On Monday, we took him to have his labs run and then got a call that our pediatrician wanted to see him again. We took him right in and she did her exam. Reflexes still looked normal. Weight had stayed the same, but wasn't a major concern because he was still in the window of time where some babies lose weight after birth. We left the appointment feeling pretty good.

We got home and Gary ran to the squadron to take care of something. I got a call that the geneticist our pediatrician had spoken with wanted to see Evan right away in the ER. He was calling the ER to tell them that we were on our way. I called Gary and my mom and I met him at the hospital.

Evan was almost immediately taken back to a room. He was no longer allowed to feed orally and was put on an IV with high levels of glucose. They took at least 10 viles of blood and took a urine sample. My poor little man was being poked and prodded like crazy. They ended up putting two IV's into him for precaution.

The geneticist said Evan's reactions were good, but things were still not seeming normal and he wanted to admit him to be safe. So...up to the General Pediatric Unit we went. They continued with his glucose and monitored him closely. The geneticists were very good about explaining everything to us. Right now it was a waiting game to see how his labwork looked. The local labs all were coming back normal. No infection.

Come Tuesday, my mom had to leave and we were still waiting on results to see if he had MSUD or if it was something else. Gary took my mom to the airport and a friend came to sit with me so I wasn't alone. The geneticists came in during this time...we had an answer. The geneticists started to talk and I started to cry. Stef just stood up and held my hand and the doctor's told us the news....Evan does indeed have Maple Syrup Urine Disease. His little body can't break down protein and will never be able to do so. He will be on a special formula for life and has to eat a special diet along with it. His proteins need to be weighed to avoid having an episode that could put him back in the hospital.

At this point, we are still in the hospital trying to get him on the right mixture of formula. He is being fed through a feeding tube and has a central line pumping glucose into his body. It is so hard to watch my baby with all these cords and wires attached to him, but he is slowly starting to react more every day. He has even started to open his eyes a bit more for a bit longer each time.

Right now it is a waiting game. His reactions and pupils are showing us that he is still showing normal neurological signs. There is always a chance that with the high levels of amino acid in an MSUD child during an episode that this could change. Please pray that he doesn't have any neurological damage. Although we would love him just the same with any issue he may have, we have our hands full with him as it is.

We are waiting for the formula for him to work and for him to essentially "wake up". Although he wakes up, he is not fully awake with eyes wide open when he is. Once he "wakes up" we will push the bottle with his new formula and when we can get him completely on oral formula we will be able to go home. It could be a few days from now or it could be a couple weeks from now. We sit and wait. The doctor told me he has NEVER seen a child not wake up. He told me that he guarantees Evan will wake up but it's just a matter of time. So we sit...and we wait....and we sit...and wait.

Please pray for our little man that he wakes up soon and we can get things in order to go home. We know we will be dealing with this for his entire life, but we are ready to have our baby at home.

If you're more curious about what Maple Syrup Urine Disease is, just google it. There is a lot of information out there