Growing Up

I am so sorry it has been so long since I have updated. I had tonsillitis last week. Normally adults my age do not get it, its mainly younger kids and teens or adults who are smokers. I don't fit into any of those categories. My doctor said I most likely got it from being run down (imagine that). I used to get it when I was younger frequently. I tend to have flare ups during stressful times.

I had gone on antibiotics last Sunday because my throat was hurting but they were not helping so luckily I went to the doctor and found out about the tonsillitis. I was put on a stronger antibiotic and I am so happy to say that I am finally feeling better. Of course I worried about the kids because it is contagious through contact. Luckily I wash my hands like crazy.

EAR, NOSE & THROAT
I had them check the kids out as well but they both were fine. I did find out that Aidan's tonsils are very large, they are not infected or anything but because of their size and his snoring he may need them taken out. I can't bare the thought of watching one of my kids go through another surgery. I just can't. I have to bring him to an Ear, Nose and Throat doctor and get him checked out. I was told by Philly that Jacob should go as well so I guess I will be bringing them both.

While in Philly they told me he passed his hearing but I recieved a letter saying once we were home saying they don't check for everything and he needed to be tested. It is obvious to me that he hears fine. He turns with each voice he hears and is the lightest sleeper ever. But to be safe we will have him checked too.

OXYGEN
Jacob is doing very well. He is off of the oxygen during the day and his SATs stay in the 80s which is wonderful. He continues to need it during the night. Around 4 hours after he goes to sleep he starts dropping into the 60s. I am wondering if it is because he is on the overnight feed. Maybe his body needs to work harder since it is digesting food rather than resting. Either way I am confident we will get off the o2 at night eventually. I can't describe how great it is to have a wireless baby again during the day. It was so hard to move from room to room with his big oxygen concentrator. I still have him hooked to the pulse ox alot to make sure his SATs are ok but I am starting to turn that off periodically since he has been so consistant the last few days.

FEEDING
Jacob is still doing so well with bottles. Sometimes I don't have to use the tube at all. Most of the time he takes about 60mls by bottle and the last 20 go through the tube. I am so proud of him. He is even trying to hold the bottle himself. He also eats much faster because he drinks the bottle in about 10 minutes. I have trouble getting him to eat much at the first feed of the day but his speech therapist and I think it is because its the first feed after his overnight continuous feed.

Jacob's nutritionist (Amy) will be here on Tuesday and I look forward to talking with her about transitioning him away from continuous feeds at night. I want him to experience as much "Normal" as he can. The problem is that most of his food comes at night. He gets 400ml over a period of 10 hours. That is alot to transition. Jake has never done well with high volume.

EATING CEREAL!
Jake has started eating baby cereal. He is doing so well. At first he was not to sure about the spoon but he puts everything else in his mouth so he did not mind. He caught on so fast. He opens his mouth as soon as he sees the spoon and looks like he knows exactly what to do. This kid is a pro! I have started mixing it thicker and he continues to chow down.

First time eating cereal

Old Pro

DEVELOPMENT
Jacob has really started grabbing onto things and getting control with his hands. He also has been passing things from one hand to another. He grabs at toys and is starting to be able to reach for things and grab them. The other day Aidan and I were dancing around and Jake started moving his arms and legs like he was dancing too. It was so cute. I love seeing Aidan and Jake interact.

You would never guess that Jacob just had open heart surgery 3 weeks ago. He just amazes me. I know I say that all of the time but he just looks so good. He is such an inspiration.

APPOINTMENTS
Jake has a cardiologist appointment on Tuesday. He had a chest x-ray done and if everything looks good then he may be able to get off of the Diuril. This would be great because he is now on 8 different medications and 4 are compounded so it gets very expensive. Plus since they are specially made so I can't just go grab a prescription if he needs it, it takes time.

He also will be meeting with his speech therapist and his nutritionist next week.

Start! Heart Walk
I am pleased to say that Team Jacob is doing really well at collecting money for the American Heart Association. We are the 7th highest collecting team and I am the 5th highest collector for the heart walk. I can't thank my team members enough for their support and hard work. Thank you so much for everyone who donated to this great cause. It is so dear to my heart, Thank you!

I am working with a company trying to get a good rate for the shirts. I had to simplify the design for the shirts that the team members will wear. I am going to order my shirt online so I have his picture on it. I also created Jacob a onesie and Aidan a t-shirts.

If you would like to donate to this great cause you can do so by clicking on the banner at the top of this blog.

I look forward to seeing everyone at the walk on September 18th!

Shirt design for team members

Front:
Back:


Jacob's Onesie Design

Aidan's Shirt Design

My Boys

Brothers being silly


Look at those rolls!

Aidan crawled in this box so he could surprise daddy when he came home from work.
He fell asleep while waiting for him to come home. It was so cute I had to take a picture. Aidan has started using the potty a lot lately. I am so proud of him!

Team Jacob

I need to get a list of everyone on Team Jacob who will be walking at the Start! Heart Walk on September 18th. I have to submit it to the AHA.

Also, if you are walking for Team Jacob and want a t-shirt please email me with your shirt size.
Please email me at thebakertrio@gmail.com.

I need to know an exact number of shirts that I need and the sizes for when I am calling local printing companies. I have been trying to find somewhere who will donate shirts and/or printing for us in exchange for their logo to be on the shirts for the heart walk. So far I have not had any luck.

I was hoping to be able to purchase shirts for everyone but if I am unable to find someone to do it I don't think I will be able to. I just don't have $20 per shirt for everyone. If I am unable to find someone to print them and you still want to purchase a shirt for yourself, I can send you the .pdf file and you can use Cafepress.com; Snapfish.com or a local printing shop to order a shirt.

Please let me know if you are walking and your shirt size by Monday, August 30th.

Thank you all so much for supporting this cause that is so dear to my heart. I look forward to seeing everyone at the walk.

Walk Information:
When: September 18, 2010
Where: WV State Capitol Grounds
Registration: 8:00am
Event Rally: 8:30am
Walk Begins: 9:00am

Shirt Designs

Front of shirt...

Back of shirt...

Amazing Advancements in Medicine

I had Jacob's stem cells harvested when I found out about his CHD. My hope was that one day in the future medical advancements would make it possible to grow a human heart. A heart which one day could save his life. That day has already begun.

This video choked me up because it give me hope. I pray Jacob's heart will last him forever but the reality is that one day he may need a heart transplant.

2 years ago if you would have told me they were trying to grow a human heart I would have told you that it was crazy. That was before my eyes were opened to this world. This world of seeing families praying everyday for a second chance for their child. This world where children fight for their lives with a smile on their face. So many children and adults die each year while waiting for a new heart. This could change that. Plus even better with this heart there would be no chance of rejection. Thank God for these researchers and doctors.



DOING OUR PART

During our last stay at CHOP I met with genetics. I agreed for them to take a DNA sample from Andy, Jacob and myself. They also took a piece of heart tissue that would have been thrown away during Jake's Glenn.

Jake will help with two research studies. One that looks at what causes these heart defects to occur. They are looking at folic acid and how for some women it does not matter how much you take, your body may just not be able to process it like it should. They are also doing a study on the hypoplastic heart condition and why every hypoplast has a different outcome.

I may not hear anything for years but it will be so great to tell Aidan or Jacob what they found. Hopefully we will have a better picture on their risk of having a child with Hypoplastic Left Heart Syndrome or CHD in general.

I want to help in any way I can. The more we know the more we can do to help these babies.

Awareness Video

This beautiful video was created by Lauren. A 23 year old survivor of a Congenital Heart Defect called Tricuspid Atresia aka HRHS. Lauren give us hope.

Jacob is in the video. Timestamp 1:26 and 6:26.

Please help spread awareness for these beautiful babies. With awareness comes research.

Thank you Lauren for allowing Jacob to be a part of this.

Make an on-line slide show at www.OneTrueMedia.com

Our Story

A fellow heart mother started a blog event where we could all link our blogs to her page and share our stories of hope and inspiration as well as the challenges that we have faced on this journey.

Read the other stories here...

Many of you know Jacob's story, for those who do not here it is...

Jacob Dylan Baker was born via a planned c-section on March 23, 2010. At our 20 week ultrasound we had learned that Jacob had a severe congenital heart defect called Hypoplastic Left Heart Syndrome. He also had a mildly restrictive atrial septum. No one thought the restricted septum would be an issue since the echo seemed to show enough blood flow passing through. When Jacob was born things went downhill very quickly.

The first time I saw Jacob... almost 2 hours old

At first Jacob seemed ok. He was pink and cried, I was relieved. Within 2 hours his septum became intact and he was fighting for his life. Even on the vent his SATs dropped down to the 30s. They rushed Jacob into the cath lab to open the atrial septal defect. After what seemed to be forever we were able to see Jacob again. He was so sick. He was bruised all over because they had a hard time getting him out of me. He was on the ventilator and was still out from the paralytic. While waiting for his lungs to get stronger Jake also got 2 bacterial infections. This put his risk for surgery even higher. At 9 days old my husband and I were finally able to hold Jacob for the first time. That was also the day he had his first open heart surgery.

First time we held Jacob ( 9 days old)

Jacob did well in surgery however they had to leave his chest open due to possible swelling. They closed it the next day.

Jacob post-Norwood

My husband had to go back to West Virginia to work and he took our 3 year old with him while my mother and myself stayed with Jacob. Thank God we are blessed with such an amazing family there to support us.

It was discovered that due to severe reflux Jacob's vocal cords were swollen and red. He also had tracheomalacia which caused his breathing to be very rapid and he retracted as he breathed. At 4 weeks old Jacob had a nissen and a g-tube placement. The Nissen is when they wrap the stomach around the esophagus in order to prevent reflux. He had to eat through the g-tube because he breathed so rapidly he was high risk for aspirating.

Jacob spent another month at Children's Hospital of Philadelphia recovering and getting stronger. At 8 weeks old we were finally able to take him home.

Home with my boys (Daddy is taking the picture)

Jacob amazed us daily by gaining weight and thriving at home! His Glenn was on August 5th. Jacob is over 14 pounds and in the 50th percentile on the growth chart which is amazing for a hypoplast. During the Glenn they thought that maybe his atrial septum was not fully cut out so they went into the heart to check it. While attempting to cut more out there was a hole punctured in the back of Jake's heart. The surgeon was able to stop the bleed and stitched up the hole and everything else went well.

Day after his surgery

Jacob continued to amaze us all with his recovery. After 3 days he was only on Tylenol and was smiling again. We were home 6 days later. Jacob used to only take maybe 15mls by bottle and the rest in his g-tube. Since his Glenn he sometimes takes full feeds by bottle, at least half each time. He is also even starting to eat baby cereal. Jacob is still on oxygen although he spends a good part of the day now oxygen free. Occasionally he drops into the 60s and I have to put it back on but for the most part he is in the high 70s to mid 80s.

5 days after open heart surgery!

Jacob is a true miracle and I am so blessed and honored to be his mother!

Progress!

It has been a week of progress at the Baker household.

Tuesday

Jacob had his cardiology visit and an appointment with his pediatrician. Our first task of the day was getting the car loaded and the kids ready to go on time. My husband had to work so my mom said she would go with me. I decided to drop Aidan off with my sister because if Aidan is there I am not able to focus on what the doctor is saying because I am trying to keep Aidan under control. Aidan cried the whole way there. I think he thought I was going to Philadelphia again. This broke my heart and I explained that I was not leaving him but rather going to a local doctor. He was still crying when my mom and I left with Jacob, but stopped soon after I left. He ended up having a GREAT time with his cousins, as I knew he would!

Getting out of the house these days is quite a task. Jake is on oxygen so I have to transfer him to a portable tank. I usually pack his diaper bag the night before. It includes the usual and then all of his meds, syringes, extensions, gauze and anything else I fear we may need. I also have to pack his pulse ox machine in case I need to check his SATs and his formula for while we are out. This is our new normal but still it is exhausting trying to get it all together for a simple outing.

CARDIOLOGIST

It was great seeing Dr. Heydarian and his staff again so soon after the Glenn. Jake was now 14lbs 3oz! Dr. Heydarian was pleased with how well Jake was doing. He was a bit concerned about the oxygen. Most kids do not need to be on oxygen post-Glenn however as we know Jake has always had trouble with his lungs. So I am just going to try and ween him slowly (More on this later in the post). I told Dr. Heydarian about our decision to postpone the circumcision and skin tag removal until later this year. He is going to contact the surgeon at Cincinnati Children's Hospital to arrange this for us when we are ready. He agreed that Jake should be off of the oxygen and a little more stable first. We went over his medications and he mentioned we may be able to start weening him off of the Diurill in a few weeks. This would be great since he is now on 8 different medications!

PEDIATRICIAN VISIT

Jacob was weighed again on their scale and was 14lbs 4oz. Every scale is different but these were only off by an ounce. I was very excited to hear that Jake is in the 50th percentile of the growth chart for his weight and the 25th percentile for his height. This is such an amazing accomplishment. Most hypoplast kids are not even on the growth chart. They generally are very small. Jake is not only on it but he is right where a healthy baby should be. I was very excited about this news!

As if he has not been through enough in the last few weeks I knew that he had to have his 4 month shots. It is not good to get behind and he was already behind by a few weeks. So he got the stitch removed from his chest tube site and then he got his 4 month vaccinations. He did very well. He cried when he first got the shots but after I picked him up he was fine.

I also discussed with the doctor about him being irregular and having lots of gas. They agreed with what CHOP had told me about putting Mirilax in his overnight feed. So far it has worked! Almost to well lol. I don't think I will use it everyday but its better than having to give him suppositories.

Dr. Harris said that Jake looked the best he has ever seen him look. He and Dr. Heydarian both commented that his breathing looked better. After making sure we said goodbye to the staff we headed home.

As soon as we got there Apria came to deliver more oxygen tanks. I also had them deliver a back up tank in case we have an emergency such as the other night when the power went out. This is one giant oxygen tank that works like the portable tanks. It does not need power. It will be nice to know that if its storming out, my son is not hooked to an electrical outlet. So relieved.

This is a picture of Jake hooked to all of his machines. The concentrator and the pulse ox machine. He is hooked to these all day and night and then at night he is also hooked to his feeding pump. It is not so easy to move him from room to room. Can't wait until he is wireless again!

FAST FORWARD TO TODAY...

It was a busy day at the Baker's today.

FOOD
Jake has been doing really well taking food by bottle but today he really gave me a run for my money. Jake downed a 80ml bottle in about 15 minutes. He drank it straight down like it was nothing. For those of you who are not familiar with his history... Pre-Glenn Jake was lucky to take 10 or 15mls by mouth. He would get so tired. So 80 is HUGE for him.

The shunt being removed is a huge help. Plus everyone says these kids are so much better after the Glenn. Most feeds Jake will drink about 35 to 40mls. At least once a day he gets ambicous and takes 60mls. Today he really was up for the challenge. That feed I did not have to use his g-tube at all!!!! The next feed he took about 40 by mouth and his last feed before the overnight went up he took 50ml. Overall a great day bottle feeding. Go Jake Go!

OXYGEN
Jake also had a day of progress with his oxygen today. Earlier today I noticed that he was SATing in the high 80s with his oxygen so I decided to do a little experiment. Normall if Jakes Oxygen canula falls out then he immediatly desats to the 60s. I decided to take it off for a bit to see how he did today. I kept him hooked up to the pulse ox the ENTIRE time so I could make sure he did not deSAT.

I figured he would make it maybe 10minutes and I would have to put it back on. He made it an hour! He stayed around 80. Sometimes higher. The lowest he would hit was 74 which is not bad.

I think he would have made it longer but it was time to eat. Eating for a heart kid is hard work. Especially for one who is just now beginning to use bottles. So as soon as he started eating by bottle he plummited down to the 60s. So back on it went. I tried to take it off again right after he was done eating but he still dropped down. About an hour later I took it off and he was great for about another hour! I decided to put it back on while he slept just in case.

Jake hates the nasal canula and tries to yank it off every chance he gets. So I have decided to give him "Oxygen Free" times through out the day as long as he tolerates it. For now i will keep it on while he eats and sleeps. I think this is a great way to get his lungs trained on having regular room air. Maybe it will help the process. Regardless it made me feel better about him being able to get off of it at some point.

HOORAY FOR AIDAN!
I can't end the blog without also giving kudos to my other son Aidan. He finally started showing interest in the potty today (This has been a struggle). Today he pooped in the potty! I know it sound silly to celebrate but to us it is a major accomplishment and I am very proud of him.

I am so proud of both of my boys!

♥♥♥♥♥ SPECIAL PRAYER REQUEST ♥♥♥♥♥

Zoe Lihn

Baby Zoe is scheduled to have her Glenn on September 14th. I have become very good friends with Zoe's mom Stacey. We met prior to having our babies and then in Philadelphia at CHOP. Her daughter Zoe has HLHS like Jake and also like Jake she has not had the easiest time. I am so happy they will be going to Philadelphia soon for her second stage. Please pray for her family over the next few weeks and pray that Zoe's surgery goes smoothly and they are back home very quickly.

Click here to read Zoe's story

I also would like to give a big Thank You to Kelly Smith for this beautiful collage she made about Jacob. She is in a scrap booking class and the project this week was to make a collage using lace and hearts. Very beautiful Kelly, Thank You!

Settling In

So sorry I have not wrote much as of late. Things have been pretty hectic around the Baker household. We are adjusting to being back home. I am still somewhat in shock that we are here so soon after Jacob's surgery. He is doing so well, it is so amazing.

To look at Jake you would never know he just had open heart surgery. He smiles, laughs and plays. he watches his brother Aidan's every move (It is so cute). His incision is almost fully healed over. I am amazed at how great it looks. He still gets Tylenol every 6 hours. It seems he gets fussy around when it is due so I am going to continue it for a little while. He is also teething so it helps with that. I still can't believe that he has been on just Tylenol since day 4 after surgery.

OXYGEN
Jake is still on Oxygen. I am trying to ween him off of that but its a slow process. He tries to rip it off of his face ever chance he gets but his pulse ox lets me know because he usually deSATs to low 70s or even in the 60s soon after. We have a concentrator which is about the size of a nightstand. I was using his portable tanks but those don't last long. Right now he is on .5 of oxygen. I go between that and .25. Last night I had to go up to 1 because he kept deSATing. I never did figure out what it was but he was fine all day today. Maybe he was just a little uncomfortable. He tends to deSAT if he is in pain or even due to his position. He deSATs when he lays flat. May have something to do with the way he breathes.

Praying I can get him weened in a few weeks because it is not easy to move him around. There is extra tubing I can use with the concentrator but it does not seem to work as good. I have to turn it up higher. Plus a cord running through the room doesn't work well with a 3 year old running around. So basically we set it up in the living room and that is where he stays for the day.

Tonight the power kept going off which did not work well for us. Jake is hooked up to the oxygen concentrator, his pulse ox machine and his feeding pump. Luckily I had the feeding pump and pulse ox charged but the concentrator would not work without power. Therefore I had to bring in his portable tank as a back up. Problem is I only have one tank left and I have doctors appointments to bring him to on Tuesday. I need the tank for that. Seeing him hooked up to all of this equipment and having the power go out made me realize I may need to look into a back up generator.

If anyone knows of anyone who has one that they could donate or sell to us pretty cheap please email me: thebakertrio@gmail.com. We would really appreciate it!

I have already contacted our power company and alerted them of the equiptment we have here. They have it on file but said that they can't bring our power up any sooner than anyone else.

MEDICATION SCHEDULE
Jake is now on 8 different Medications. Here is his new schedule for meds/feeds

8am:
Lasix (0.6ml), Captopril (3 ml), Prevacid (2 ml), 1/2 Baby Aspirin, Bethanichol (0.6ml), Diuril (1.2 ml)
4pm:
Lasix (0.6ml), Captopril (3 ml), Bethanichol (0.6ml)
8pm:
Prevacid (2 ml), Diuril (1.2 ml), Sodium Suppliment (2.5 ml), Aldactone (1.2 ml)
Midnight:
Lasix (0.6ml), Captopril (3 ml), Bethanichol (0.6ml)

FEEDING
The biggest change in Jake since his Glenn is his eating. Pre-Glenn Jake would only take about 15ml by bottle. He would get very tired and I would have to put the rest through his tube. Now Jake takes at least half of his feed by bottle. Sometimes just about the whole feed! He would take the whole feed everytime if I would let him but the Speech therapis at CHOP said to only let him go for 20 minutes. Anything after that and he will be burning more calories than he is getting. So anything left after the 20 minutes I put in his g-tube.

It is so great to see him taking more food by bottle. He even likes to hold onto the bottle with both hands as he is drinking. It has eliminated the pain issues he used to get by doing the whole feed by tube. We still have a ways to go but this is a HUGE step in getting him towards "Normal" feeds.

Dr. Szwast said that the shunt can make it harder for them so he probably feels much better now that it is out. My big challenge now will be finding out how to transition his night feeds to the daytime. It is not normal to feed all night long and Jake gets 400mls at night. He gets 300 during the day. The majority of his daily intake is at night. Jake has never tolerated large volume very well so it will be hard to move it all over. I will be working with speech here and hopefully they can help me with this challenge.

His speech therapist in Philly also said we should be able to try baby food soon. it may be even easier for Jake because he can taste it and not have to worry about the suck, swallow breathe coordination.

OVERALL it has been a great week for Jake here at home. We are so glad to be here and thank you to all of you for the prayers you sent up for my beautiful little boy. We are so blessed in so many ways. His next surgery will not be until he is around 3 years old. Praying we get a nice long break away from the hospital until then.

Love,
The Bakers

Jake and his CHOP Buddy and fellow HLHS survivor Adam.

Happy to be home and in his own bed!

Home Sweet Home

I will write a longer post tomorrow but I wanted to let everyone know WE ARE HOME!

We actually got home last night. Jacob did so well in the car so we were able to make the whole trip in one day. I am still amazed that here we are a week after surgery HOME!

Jacob is doing very well. He is on oxygen still so we are adjusting to that and in the process of trying to ween him off of that.

You would never know he even had surgery a week ago. He is smiling, laughing and glad to be home.

I am really tired so I promise I will write more later. Thank you all for the prayers and well wishes. The support means so much to our family.

Love,
The Bakers

Another Great Day!

Jacob had another great day full of smiles. His x-ray looked world's better today. Not much fluid at all on his lungs anymore. His heart echo also looked really good. No signs of any problems with the pulmonary veins. His heart function was the same as it was prior to surgery so he will still need to be on Captopril due to it being on the lower side of normal. Overall they were very pleased with how good it looked.

Jacob is still on oxygen. We are currently trying to ween him off. He was on 2liters but is now down to 1 liter and will go down a little every 4 hours as tolerated. The doctors all agree that he does not need to be here just for oxygen and if weening does not work he can go home on it. I hope we can get him off of it though because he hates it and constantly pulls it out of his nose.

His "Unique" breathing has come back. His strider was louder today and he was breathing heavier. Earlier in this blog I stated it was from a floppy esophagus, it is actually a floppy trachea. The name of this diagnosis is Tracheomalacia. It is due to the issues with his lungs that occured at birth and the vent being in so long.

Definition:
Tracheomalacia in a newborn occurs when the cartilage in the windpipe (trachea) has not developed properly. Instead of being rigid, the walls of the trachea are floppy. Because the windpipe is the main airway, breathing difficulties begin soon after birth.

He is doing great taking the bottle. The last feed he took 50ml by mouth. Basically with each day feed I am giving him 20 minutes on the bottle. Whatever he does not take by bottle goes in his tube. He LOVES the bottle and goes to town. He is doing so well. His doctor said it is easier for him now that the shunt is gone. They also said that if my speech therapist at home oks it then we can try baby food in the coming months. His speech therapist here said that we can try it out and see how he does but its easier for them and he would probably do well.

Overall everyone is amazed at how great Jacob looks. His color is good, he is still gaining weight really well. He is my hero. To look at him you would never know he had surgery 5 days ago. He acts just like the Jake we all know and love.

I should have a better idea about when we will be going home after rounds tomorrow. Today they acted like we could be discharged tomorrow or Wednesday. Most likely Wednesday. They usually take a while to discharge you so we would spend the night at the Ronald McDonald House and leave the next morning. We will stay in Morgantown on the way home (Kim, I will call you when I know what day we will be heading through there!) and get home the following day. So if all goes to plan we may get home sometime on Friday.

I will update tomorrow but for now THANK YOU ALL for the prayers and love you have sent our way. Jake is living proof that they are heard.

Love,
The Bakers

Much Better Day!

Jacob had a much better day today!

The day started out kind of rough. Jake was up from about 4 to 6am with gas pain. He had not had a bowel movement since Friday so I said we should hold off on the morphine since it is probably constipating him. We decided to see how he did without it and that proved to be the best thing for him. He is still getting Tylenol every 6 hours. His swelling and redness on his face has gone away and he has been much more alert today. He even had a few smiles for daddy.

He has been having gas pain once in a while. We gave him a suppository and that did not work so we gave him some Mirilax. He had a small amount of diarrhea but we are hoping the Mirilax will get some more out of him and give him some more relief.

He is still deSATing which is a concern. He is on 2 liters of o2 and he drops down to the low 70's or high 60s occasionally. His right lung has some fluid on it so they upped his diuretics. He is now on 8 medications daily! Seriously I thought we were going to go down on meds but we are going up! Oh well at least he is feeling better and he is now peeing like he should be. His SATs are good when he is sitting up but when I lay him down he deSATs. This is due to the fluid on his lungs.

They are going to do an echo tomorrow to get a better idea of what is going on. Praying that it is not because of the pulmonary vein. The hole that was punctured in the back of his heart was right next to the pulmonary vein. If that is leaking it can cause the type of deSAT that Jake is having. This fluid is also common with the surgery even without a leak in the vein. Praying Jake's fluid is just from the surgery and not the pulmonary vein.

It is the weekend and not much happens on the weekend. Tomorrow should be a busy day. I will talk to the team about whether or not he should have a circumcision and the skin tag removed. We will get the echo and discuss the fluid on his lung and what our discharge plans are.

So overall Jake is doing GREAT! We have had a good day and praying for an even better day tomorrow.

Love
The Bakers

This was pretty funny. This bouncer should be upright but Jake is so heavy it is parallel with the bed and the bottom actually touches the bed. Gave us a good laugh.

Moving Down!

Jake is now 14pounds 4 ounces! Even with the surgery our little miracle is gaining weight. We are so proud of you my not so little peanut!

Good-bye CICU hello CCU:
Jake just moved to the step down unit (The Cardiac Care Unit). He has his own room which is nice so he can finally get some rest. It is a really big room because it was once intended for 2 babies but the way it was designed is not good if there were an emergency and you had to get the second baby out. So the other side is my living room lol. When Jake is asleep I can use it to watch TV or talk on the phone and not disturb him but still be right in the same room with him.

Jake kicked off his last IV last night so all he has left is his Oxygen. He is still SATing a bit low without it so we are hoping that is just from pain and it will get better. We will ween him off now that he is over here.

Poor baby has been in a lot of pain today. Those dreaded Glenn Headaches hit with vengeance. He looks like his head is going to explode. He is a little swollen and red. His skin looks modeled (red and splotchy). You can see it in his eyes that he does not feel well. Luckily the morphine is helping and he is resting. He is not crying much but he gets really fussy and kicks and you can just see the pain. Praying these headaches go away quickly.

They are caused by the pressures backing up in his lungs. Basically they re-routed his blood to go directly to the lungs. The lungs are adjusting to the new added pressure. So the pressure backs up and causes swelling and headaches. They usually only last a few days.

Other than that Jake is doing very well. His bandage is off of his incision and it looks good. His color is good and he is always warm. I am leaving him alone unless he cries and then I hold him. Otherwise I want him to get some much needed rest. His feeds were increased to 75ml during the day and he is tolerating that very well.

We will speak to the team on Monday about circumcision and the skin tag removal. It almost seems cruel to me to put him through that after all he has been through however many of the boys get it done after the Glenn. If we wait until his fontan he will be older and will be more aware of it. It may extend our stay a few days since he will have to be put on anesthesia to get it done. I will leave it up to the docs here to decide if they think he is recovered well enough to have it done. I know its a simple procedure compared to the Glenn but still I don't want to put him through too much.

Thank you all for the continued prayers. They are working please keep it up!

Love,
The Bakers

Amazing what a day can do!

I was so amazed at Jacob's progress today. He smiled two times and I got to hold him all day!

TUBES AND WIRES AND TESTS:
I am so happy to report that Jacob had all of his tubes and wires removed except one IV and a small amount of oxygen. They took out the RA lines, the pacing wires, the art line and the chest tube! He only has one IV left just in case he would need it during the stay. He is still on the oxygen just to give him a little help with his SATs while he is recovering. He pulled it off multiple times today and did ok without it but we noticed his breathing was more labored so put it back on just for the next few days. I am amazed that this all came off so quickly.

He had a chest x-ray this morning and it looked great!

With Tubes and Wires

Look Mommy no lines or tubes!

BREATHING:
Jake still looks much better than he did pre-surgery. His retractions have come back a little and I notice a very faint strider at times but nothing like before. During his first stay they found that he has trachia malasia. It is floppy and causes the breathing issues he has. It may take years for it to fully go away. We still think the shunt contributed to his breathing though because he has improved dramatically. Even though the retractions have come back some, he is only breathing 30 or 40breaths a minute on average. He used to breath 70bpm. Even when he was drinking a bottle he would only go to 50s. That is huge for him. It will not change overnight but hopefully for Jake it will be a bit easier.

MEDS:
Jacob is off all IV meds which is great because everything he is on right now he could go home on. He is off of the Milrinone and the Dopamine. He gets tylenol every 6 hours. We just started all of his meds that he was on at home. They increased his aspirin to once every day. Instead of M,W and F only. He gets Fetinal for pain and he was getting Oxycodone but we are no longer going to give him that because he had an allergic reaction. His eyes became very itchy and his eyelids and nose puffed up and were red. He would not stop trying to scratch them today so I had to hold his hand to prevent him from making it worse. They gave him benedryl but it did not help too much.

Poor Itchy Jakey. Reaction to his pain meds.

Instead we are going to try morphine. I get nervous about morphine because that is what he coded on. The doctors all are aware of that but they said that he was only 4 weeks old then and much smaller. He also had been on anethesia that day and was given two doses within an hour. I agree that most likely it will be fine now and we have to keep his pain managed. They are only going to give him a small amount and only if he needs it.

RESTLESS AND AGGITATED
Jake was restless all day today. I don't think he had a lot of pain or headaches it was more like he was just aggrivated at his surroundings. Everytime he would start to fall asleep the baby next to us would cry or alarms would sound from other kids monitors. Its so hard for him to rest because he is in a pod with 3 other kids. He is not used to so much going on. He was awake all morning and afternoon. I felt so bad but nothing would work. Even meds did not help. He was not screaming but he was wiggling around and fussing. Finally around 3pm he got about an hour of sleep in. I am praying he gets more sleep tonight since it should be quieter and he will be on his overnight feed.

Good news is they are moving him to the STEP DOWN UNIT tomorrow. He should have his own room and be able to get some much needed rest. I think this will make his recovery even better.

FEEDING:
Last night Jake was given 3 bottles. The nurse that night wanted to see what he would take since his breathing was so much better. He gave him 20mls the first time, 40 the second and Jake took 60ml the third time. He must have just been really hungry because today he had a bit harder of a time. He only took 15ml by bottle each time I tried. He is still getting used to taking so much by mouth plus he was on pain/sedation meds and just had surgery yesterday. I think it will be a slow process but we will get there.


I met with our speech therapist at CHOP. Lauren said he sounded really good and she is comfortable with us trying the bottle 3 times a day for about 20min each time. She said that we should still follow his cues on whether we can push him or give him a break. If he gets to tired or shows signs of stress we don't have to do the full 20min. Jake's respiratory rate does go up and his strider is audible during feeding but still less than it used to be so hopefully with more practice he can start taking more by mouth. This would be great since we have so many tummy issues with that tube.


We increased his daytime feeds to 75ml instead of 70 and he tolerated it very well. He is still on 400 at night. We were able to find out that the formula he is getting at CHOP is the same formula we use at home. So far he has not had any retching or even any gas issues. He is still having regular bowel movements and urine output which is great.

Please continue to pray he keeps doing well and feels better each day.

We Love You Jake
Mommy, Daddy and Aidan

Jake update

Jake Post-Glenn

Jacob is doing really well so far. He has woken up a few times in pain. His cry was so sad but they gave him more pain meds and I held his hand (Which he squeezed very tightly around my finger) until he calmed down. He seems to be resting much better right now. He is doing well off of the vent. All of his numbers look very good. His blood pressure and heart rate have come down and his blood gasses are perfect.

He is no longer on dopamine but still on the Milrenone and pain meds. The chest tube still is not draining much so they think it will come out in the morning along with the RA line and probably the pacing wires. He still has great urine output which is also a good sign.

They are going to start feeding him tonight so I pray that goes well. They don't have the exact formula we use and it caused some gas and wretching when we gave it to him after the cath so we are starting him on pedialyte and then we will see how he does on the formula. I may have to talk to nutrition tomorrow about alternate options.

Dr. Szwast said we have to watch the pulmonary vein for a few months to make sure that the hole did not go through to that. She said they can see it on echo's or chest xrays. If it is going to cause a problem it may not show up right away. This will not prolong our stay here however once home we may need to have a echo or xray once in a while to check on it. I so wish that we did not have that to worry about however in the grand scheme of things this is a minor issue. Most likely it is fine, Dr. Spray stopped the bleeding and stitched it up. Hopefully that will be the end of it.

Aidan came in to see his brother and sang him a song "You are my sunshine". He told Jake that he would be just fine and to feel better. It was so sweet. We covered most of Jake's lines so all Aidan could see was the dressing over his incision but he did not notice it. He is such a good big brother!

Tomorrow will be a very tough day for Jake. During this surgery they re-route the blood so it no longer goes through the heart. It goes directly to the lungs. The lungs in turn have to deal with added pressure. While adjusting the pressure goes to the head and causes some swelling and very bad headaches. The kids tend to be unconsolable and cry a lot. Please pray we can manage this for Jake. Luckily it should only last a day or two.

NOW FOR THE GREAT NEWS...

I may be jumping the gun here but so far Jake's breathing looks so much better. He is breathing 20 or 30 breaths a minute. Jake used to breath 70bpm and always had that loud strider. Even when he was awake today and upset he had no strider. Now he is still under sedation so not sure if it is better or not but he is no longer tugging under his rib cage. His breathing actually looks like a wave pattern but he is using more of his lower belly and chest. The thought is that the shunt may have been causing some of his breathing issues. Only time will tell for sure but I hope he keeps this up. The only reason we restricted his bottle feeding was his fast breathing. If he continues to breath normally then we can start pushing that and maybe not have to use the tube as much or at all.

He actually takes even pauses between breaths... so great to see!

Ok well it has been a long day so please continue to pray that Jake has a quiet night and has a smooth recovery. Also please pray that this hole does not cause any other issues. Thank you all for the continued support and prayers.

Love,
The Bakers

Waiting before Jake's bi-directional Glenn

Post-Glenn

Hope nudges me forward...
When things don't seem clear...
It reaches for my hand...
And says, "Have no fear"...
It points to the mountain...
And says..."Are you ready"?
It's time to begin...
Just keep yourself steady...
And when my steps falter...
I cry..."I can't do this"...
Hope pulls me up...
And says,"We will get through this"...
Together we walk...
Toward the ominous hill...
Then I ask hope again...
Are you sure it's His will?
It's so very dark...
Toward the long road ahead...
I wonder...if I should just stay here instead...
Hope squeezes my hand...
I squeeze back real tight...
Hope says we must go...
And... I know he is right.
As we trudge up the mountain...
Into the unknown...
Hope soon reminds me...
I'm never alone...
Then we reach the summit...
We have come so far...
At the top of this mountain...
I see every star...
Did you think we would make it?
Hope asks, with a smile...
Did you think you'd keep going...
In the midst of your trial?
God surely led you...
Yes...every...day...
He marked out your path...
And then...He paved the way.
And now we'll part ways...
For just a short while...
As you have surpassed...
The worst of your trial...
The path from this place...
Is only downhill...
It's much easier...
And it takes little skill.
I thanked my companion...
For leading the way...
And helping me get...
Where I'm standing today.
God paved the way...
When I thought..."I can't cope"...
He taught me to climb...
And then, He sent ..."Hope".

~Stephanie Husted