Entering the next stage in our journey...

Ok so I have been avoiding writing this blog post like the plague. Seriously, I have sat down to write this countless times in the past few months but just couldn't do it. There is no more avoiding it. 2 weeks from now we will be in Philadelphia for Jacob's third and final planned open heart surgery (The Fontan).

Before I get into that let me catch you up on some other pretty big things that have happened since I last blogged (which was months ago!).

Jake and Aidan had their birthdays. Jake is 3 and Aidan is now 6. My boys are getting so big so fast! We partied hard this year lol. Each of the boys had small family parties and then we had a big combo party at pump up the fun.

Jake started pre-school. He is actually on summer break now but he had 2 months in a special needs pre-school class. We love his teacher Ms. Jessie. He did so well. I know it is going to be really good for him to be around other kids his age and have that interaction. He will also get speech twice a week. That is in addition to the weekly outpatient speech he gets.

Jacob's local cardilogist retired. Dr. Heydarian is like family to us. He took care of Jacob like he would his own son. He would call us when we were out of state for surgery to check on his "Buddy". I am very happy for him that he got to retire but we will miss him.

Aidan graduated Kindergarten! I can't believe that I now have a first grader. I am so proud of Aidan. His did so awesome this year. We were so blessed to have an amazing teacher. We love Mrs. Clark and she set the bar really high for any future teachers. Aidan is so smart, he got all M's on his last report card. M stands for mastery and is the best grade you can get!

 
Aidan also lost 2 teeth over the past two months. He is getting so big!

 

THE FONTAN

The Fontan is the third in a series of open heart surgeries that Jacob needs to survive. There are two different methods, Jacob will be having the extracardiac fontan.

The Children's Hospital of Philadelphia has a great video series which describes the surgeries for Hypoplastic Left Heart Syndrome.
Click here to watch the video and learn more about the Fontan.

The phrase Post-Fontan is music to the ears of single ventricle parents. It is what we look forward to, it is a huge milestone for single ventricle kids. Their oxygen levels increase and they have a lot more energy (I find that hard to Believe because Jake is constantly going lol). We know from the get go that our kids need 3 open heart surgeries and we dream of the day that we can say they are Post-Fontan.

Although I can't wait to be post-fontan, I have to go through the darkness to get to the light. I am not looking forward to the next few weeks or the next few months for that matter. It makes me physically ill to think of having to hand Jacob over again for another major surgery. The recovery after his airway surgery was so rough on him. I hate the thought of him being scared and in pain. Despite the fear I know that Jacob needs this. I know he can't survive without this. I know I am strong enough to get him through this. And I know he will rock it. He has to, I can't let myself think any other way. It will be hard, it will be hell for a few days (Maybe weeks) but I am just dreaming of the day we get to come home with our amazing little boy and celebrate being Post-Fontan!

THE PLAN

July 14th
We will be driving 9 hours to Philadelphia (Our home away from home). We are on the list for the Ronald McDonald House but it usually takes days sometimes a week or longer to get in there. Please pray we get in because staying at a hotel is really expensive and we can't afford that.

July 15th
We have a long day planned for pre-op at the hospital. Here is the plan.

• Cardiac Echo- ultrasound of the heart
• EKG
• Bloodwork- If you have followed this blog for a while you know how difficult it is to get Jacob's blood. His veins are all scarred over. They usually have to get him by using an ultrasound guided IV placement. Please pray they get him without hours of screaming and poking. It breaks my heart. 
• Sedated Cardiac MRI- Jacob's echo looked so good the last time they didn't think he needed a heart cath (YAY!). Many hospitals are now doing Cardiac MRIs instead of caths. This is a 3D rendering of his heart. I am actually kind of looking forward to seeing this. 
• Sedated MRI of the Brain- CHOP is currently doing a study which I am actually really interested in. They are doing a study to see if the difference in blood flow to the brain Pre and Post-Fontan has anything to do with developmental delays in single ventricle patients. They are going to do a MRI of his brain before his Fontan and another 6 months after and see if the difference in circulation has any effect on brain development. Another reason I am interested in this scan is when we went to the neuro-cardiac clinic in Cincinnati they recommended we have one done because of his speech and developmental delays. The MRI can show us if Jacob has ever suffered a stroke without us knowing about it. He also had a bad fall a few years ago and it will show if there is any scarring which cause damage to his brain that could be causing delays. It won't change anything in the course of therapy and there is no reversing any damage if it occurred from either the fall or a stroke but it would help give us more pieces to the puzzle. I told them we didn't want the MRI done at the time of the clinic because we knew they would be doing one before his surgery. They agreed that there is no urgent medical reason that we couldn't wait so that we wouldn't have to sedate him twice. This way he can get it done while already under anesthesia.
• Meet with Anesthesia- Sign lots of forms saying I understand all of the risks
• Cardiology Appointment- Meeting with his Cardiologist at CHOP to go over results and surgery.
• Hematology Appointment-  We need to meet with the hematology team to discuss his post-op treatment. Jacob got a blood clot while recovering from his airway surgery. They did bloodwork after that and found out he has a rare factor IV leiden gene mutation. This gene puts him more at risk for clotting if he is immobile for a long time or after major surgeries. Because of this he will need to be on a stronger blood thinner after his surgery. They will be giving him Heparin. He will also need to go back on the lovenox injections. Remember those shots we had to give him for months after his airway surgery. How can I forget, we hated them. Well he will need them again. The good news is it should be a prophylactic dose. That means he may not need to travel to Cincinnati every month to check his levels etc.

July 16th
SURGERY. Jacob will undergo his third open heart surgery. We yet again hand over our baby to the man with the magic hands, Dr. Spray. It never gets easier handing our baby over for surgery but we are really lucky to have such a talented surgeon. Dr. Spray is world re-known. I know Jacob is in the best hands possible.

We were told to expect 2 to 4 weeks for recovery.  Andy, my mom and Aidan are staying for 2 weeks. If we are not home by then, they will head back to WV and I will stay with Jake at the hospital. My mom will watch Aidan while Andy is working and sleeping. I am praying we can all come home together after a week but I always say.. I plan for a few months and pray for a few weeks. I learned a long time ago not to get my hopes up about when we will get to go home. We will be home when Jacob is ready. We are on his body's time table. The bad thing about the Fontan is that the kids tend to have a lot of fluid build up around their lungs. They are put on a lot of diuretics post-op but sometimes they end up needing to be re-admitted for a chest tube. I don't want to rush home and find out he needs to go back. If we do get home and something like that happens then we can always go to Cincinnati for a chest tube but I would rather stay there longer and make sure he is good to go before we go home so we don't have to go back at all.

We wanted to do something fun for the boys before having to spend our summer at the hospital so we recently took a trip up to Cass Railroad. Jake loves trains and talks about them all day long.  I emailed Cass and told them about Jake and wanted to see if he would be able to see the inside of the engine and see the train up close. The superintendent scott wrote me back and gave Jake a trip to remember. He not only gave him a full tour of the train shed but he gave us 5 tickets on the 2 hour train ride and let us stay in one of their company houses the night before. It was such an amazing mini-vacation. The boys had a blast and it was just what we needed. If you read this Scott, thank you so much. I can't tell you how much this meant to Jake and our family.

Jacob with Scott, the man who made this trip possible.

 

So there it is, the countdown has begun.  I will post frequent updates on here while at the hospital. Until then, we will be taking in every precious moment and getting lots of snuggles with our little guy. Please continue to keep Jacob and our family in your prayers as we embark on this next stage in our journey.

Three years ago...

The first time I saw my beautiful warrior... 3 years ago.

Three years ago I was sitting in a hotel room scared beyond belief, unsure if my child would survive. Three years ago I knew my life would never be the same but I had no idea I would find my purpose in life. Three years ago today I was preparing for the birth of my heart baby. I thought I knew what to expect but I really had no clue. I was supposed to be anticipating one of the greatest joys of my life. Instead I was scared to death of the possibility that I may have to say good-bye to him forever.

This journey has been a roller coaster full of emotions. We hit so many different odds and had many curve balls thrown at us throughout the past three years. It has also been the greatest three years of my life. Jacob is such a blessing to our entire family. He has taught us all what life is truly about. We have met so many amazing families throughout our journey who we never would have met had it not been for Jacob. We have been able to help others through his story and find strength in theirs. What I had thought was my biggest fear has turned into our biggest blessing. Jacob and Aidan are my greatest gifts and I am so grateful I was chosen to be their mother.

Tomorrow my beautiful little heart warrior will turn three years old. A day I wasn't sure I would ever see. A day I celebrate with all of my heart and soul. I am blessed beyond words. 

Jacob,
You continue to amaze me by your strength, inspire me to be a better person and teach me that together we can overcome any obstacle that we face. Happy birthday beautiful boy. I love you more than life itself and I am so very proud of you!
 

Spreading Awareness!

It is that time of year again. The time of year when heart moms from all over the world blow up facebook and the web with posts about Congenital Heart Defects. It is Heart Month and this week (February 7 thru 14) is Congenital Heart Defect Awareness week.

I plan on doing a longer post with details about our recent trip to Cincinnati but today I wanted to focus on CHD Awareness. To hold you over until then I will let you know that Jacob's tests went great. His airway looks good, he won't need another scope until after his Fontan (Last planned open heart surgery) this summer. His hearing test went great and showed his hearing is 100% NORMAL!

JUMP ROPE for HEART

This year Aidan is participating in Jump Rope for Heart. It is a fundraiser his school is participating in that raises money for the American Heart Association. Aidan was so excited when he found out about the event, he told his class all about his brother's special heart. He told me he wanted to collect money to help babies like Jacob! I am so proud of Aidan, he is such a good big brother. Jacob gets a lot of attention because of his heart. I try to involve Aidan as much as I can but there is not much he can do. This fundraiser is so great for Aidan because it is a way that he can help a great cause that is so close to our hearts. He gets to make a difference and I want to make this a huge success for him.

I knew immediately that I wanted to help out at the school so I talked to his coach and asked if they needed any information about congenital heart defects. They didn't have any so I offered to create a video about CHDs for them to show the kids. Timing was perfect because I was trying to think of something I could do for CHD awareness week.

I created two versions of the video, one for Jump Rope for Heart and one to share online for CHD Awareness Week. I am really happy with how the video turned out. Every child in the video was born with a congenital heart defect, many were born with the same defect as Jacob. Each of these kids holds a special place in my heart and I am so grateful to their parents for allowing me to use their photos in the video. I am posting the videos below, please feel free to share them and help us spread awareness!

I am also going to go to the school on the day of the jump and help out at the event. I will take lots of pictures and post them on here after the jump. It will be at the end of this month. I plan on making Aidan a shirt with this design:

 

Looking for a way to contribute to CHD Awareness Week? Please help support Aidan by sponsoring him in his mission to help fight heart disease. Make a tax deductible donation in honor of Jacob by clicking on the link below. Online donations can be made until February 21st. Thank you!

CLICK HERE TO DONATE!

CHD AWARENESS

 CHD AWARENESS- JUMP ROPE FOR HEART VERSION




Happy Heart Week! Please feel free to share this video and continue to spread awareness for all of the children fighting this battle and for those who were taken from this earth way too soon!

Hello Blog World!

Hello Blog World! It has been a busy couple of months but we are all doing very well. The boys had an amazing Christmas and they must have been extremely good because Santa was very generous! I have not updated this blog since October so I am going to try and do a quick summary of what has been going on at the Baker Household.

The boys are growing up so fast! Aidan is doing so amazing in school. He is already reading and writing. He has made a best friend (Michael) and they are inseparable at school. He had his first play date here with his friends a few weeks ago. I can't believe my little boy is having friends over. When did he grow up so fast! We have been keeping really busy. Jake has 3 therapy appointments a week and I teach art for Aidan's Kindergarten class once a week. I am also working part time so everyday is a busy one around here but as long as the boys are happy and doing well I would go 24/7 for them. Aidan and Jake are so cute together. Aidan loves helping teach his brother how to talk and Jake loves mimicking Aidan and playing with him. He already looks up to his big brother. It is so amazing to watch their bond grow. I am a very proud mama and so blessed to have such great kids. 

Medical Update:

We went back to Cincinnati on December 5th for another round of appointments for Jake. This trip we met with audiology, ophthalmology, and Speech.

Ophthalmology:
Jake has been squinting a lot and his therapists and I really thought he had a vision issue. I was surprised when I found out his vision was great. He had a little near nearsightedness and astigmatism but they said they counter act each other and it is normal at his age. They will see him again in about 4 months but for now he got a great report!

Audiology:
We scheduled a hearing screen for Jake because of his speech issues. We needed to know one way or another if his hearing is causing speech delays. Jake was not very cooperative but it was obvious that he would turn anytime they had a loud tone play. He was not even flinching during the lower or mid range tones. The test showed that Jake had moderate hearing loss. He was hearing tones at a range of 50 or 60 and they would like it to be under 20. He heard voices at a 35. It is not a accurate reading because of his age, he just wouldn't sit still or let them put headphones on his ears. The only way to tell for sure is to do a sedated hearing screen which we are going for this week. If the sedated screen shows the same results then Jacob will need to get hearing aides in both ears.

I was pretty bummed at first at this news because lets face it every time we go up there we get yet another diagnosis. I just want something to go right for the kid. After I had time to really let it soak in I have made peace with it. Honestly the plus side is it would be an answer. A reason for why Jake is so delayed in speech and why the words he does say are so "Tonal". It is also something that can be fixed. Hearing aides can help him hear and that will allow him to really be able to progress with speech. So if he does need hearing aides we will adjust just like everything else. The plus side is if he gets them now he will be used to them by the time he starts Kindergarten. The downside is I pray he doesn't rip them out of his ears and break them because they are expensive!

I found out from the hearing specialist that a main side effect of IV antibiotics is permanent hearing loss and it is common for kids with congenital heart defects to have hearing loss due to medications. Jacob was on IV antibiotics when his sternal incision became infected after his Norwood surgery. Regardless he needed them to save his life so we wouldn't have been able to avoid it.

Speech: Jake had a follow up swallow study and he passed with flying colors. We were going to schedule his Gtube to be removed but about 5 weeks ago Jacob got RSV. He handled it really well but he didn't eat for a few days and I had to tube feed him. He is eating well now but I have still had to supplement some of his liquids through his gtube so we have decided to leave it in until his next open heart surgery. It is honestly really handy to have if he gets sick. It is pretty much the reason he didn't have to be hospitalized when he had RSV. I was able to tube feed him and monitor his oxygen from home.

We also met with a speech therapy while there who basically told us what we already knew. Jake is making progress and to just do extensive speech therapy. Right now Jake is getting therapy 3 times a week. Speech twice a week and developmental therapy once a week.

Jake is talking a lot these days but the sounds are still very tonal which would make sense if he has hearing loss. It may be the way the sound is going in. He has started staying and signing mommy a lot. He yells it and runs up to me when I get home from work and I just melt. I have waited so long to hear him say "Mommy" and I never get tired of hearing it. He wakes up and smiles at me and says "Hi Mommy" over and over. I LOVE IT! He has such a little twang when he says it to. So cute. Can you tell how happy I am about this lol. He has also been spoiling me with kisses.

HEADING TO CINCY:

We are headed back to Cincinnati on Wednesday. Jake has to be there at 10:45 for pre-op. He is having a bronch and a sedated hearing screen. The bronch takes place in the OR under full anesthesia so it is considered surgery although there is no cutting. While he is under they are going to do a sedated hearing screen which will let them know if he has permanent hearing loss. Hematology is also going to do a blood draw to make sure there is no sign of further clotting in his blood.

I will post updates when we get all of the results. Please pray everything goes well. Although it is a minor procedure it is always a risk when they have to put him under. We decided to make this trip into a mini family vacation and take the boys to Great Wolf Lodge while up there. I like to try and plan fun things along with the medical stuff so Jake doesn't just view these trips as negative. I am also excited to be able to attend a conference while there and see some of my favorite heart moms. On Friday, Andy is going to play all day with the boys and I attend a joint collaborative between parents and cardiac centers from around the country. The collaborative focuses on improving outcomes for HLHS children. I am so glad we were able to schedule Jacob's appointments the same week as the conference so I can attend.

We also found out that Jacob will have his final planned heart surgery (Fontan) this summer. I guess I was not expecting it would be this year so it took me by surprise. It shouldn't have but I had it in my head we could wait until next year. His oxygen levels are not high enough to wait. They would consider it if he was consistently in the high 80s but honestly 80 is a high number for Jacob so he can't afford to wait. We need to do it this summer. I will be getting the paperwork done next month and will let everyone know when we have a date. The thought of handing him over for another major surgery kills me but at least it won't be looming over us. Jake has had a major surgery every year since he was born. It will be good to get this behind us and just be able to focus on speech and giving him an amazing life out of the hospital for a while.

I know I have a bunch more to write about but for now I need to finish packing and get ready for our trip. Here are some pictures to hold you over until the next update!

Hitting the Odds (again): The Factor Five Leiden Gene

Our lives have been so busy that unfortunately this blog gets neglected from time to time. For this I apologize  I went back to work a few months ago, working Wed-Fri. This means I have to fit all of Jake's appointments into 2 days. He has 3 therapy appointments a week and I have also had a bunch of follow up appointments to schedule for him. In addition to all of that I have agreed to teach Aidan's kindergarten class art once a week. Their school doesn't have an art program and I am really excited to teach the kids. It also gives me something special to do just for Aidan since so much of my time is taken by Jacob's appointments.  So as you can see there has not been much time for blogging or anything else lol.

HEMATOLOGY

When we left off I was talking about our trip to Cincinnati. Well we have actually been there again since then but I will get to that in a minute. To finish the blog post I started TWO months ago, the second day Jake had a ultrasound of his clot (Which he screamed through) and then we had to meet with Hematology. The Hematologist said the clot has remained unchanged and has now become part of his body. I am not sure if I have explained this before so if I have please forgive me. When you have  clot for a long period of time the body sees this as a foreign object and starts trying to figure out how to "Fix" the problem. It then creates small veins called collaterals around the clot. So if Jacob's Jugular vein is the super highway then these collaterals would be the back roads. They re-route the blood around the clot.  It is pretty amazing how our body works. Jacobs clot has hardened and become a part of him, the collaterals are a way for his body to continue moving blood through that area. I was concerned if the clot can go anywhere especially since it is in his neck, right between the heart and brain. The Hematologist assured me that it is not going anywhere. It has pretty much fossilized if that makes sense and his body has grown around it.

Before they could take Jacob off of the Lovenox treatments they needed to check for inherited clotting disorders to make sure he is not at risk for clotting. They tried to get blood that day but after 2 hours of holding Jake down as they tried over and over and watching him scream and thrash like I had never seen before, I told them NO MORE. They said they could get an ultrasound machine down to find a vein but I knew my son and he had been through to much and I was not about to let him be poked on anymore that day. I told them I wanted them to check with CHOP and make sure they had not already run the test and also speak to his cardiologist at CHOP to make sure she was even ok with him going off of the shots. The hematologist agreed that it was a good plan and we would come back in a month for blood work if his cardiologist agreed.

Fast forward 3 weeks which also happened to be 3 weeks ago (told you I am behind on updating). Jacob's cardiologist agreed that if his tests came out OK then he could stop taking lovenox. They could not do the blood work here in WV because they needed so much blood that they wanted it done there. So we I decided to drive to Cincinnati and back in one day so Jake could get the blood work done.  This time they had IV team come up with an ultrasound machine. Even with ultrasound guidance it took over an hour and IV team couldn't get him to bleed enough for the test so they had to call a PICC Line nurse up to stick him. She was awesome and they got all of the blood they needed to run their tests.

Fast forward again to today.  The good news is that Jacob is able to stop the Lovenox injections. YAY! Two shots a day was so hard on the poor kiddo and his little arms and legs were constantly bruised from being injected with blood thinners.  Now for the complicated part. Most of the tests came back normal including a test which looked for micro-clots in his blood. One however did not come back as good as we would have liked.

Jacob has 1 Factor Five Leiden gene. You can have either 0, 1 or 2 of the genes. 0 is normal and means your body is producing plenty Protein C, a natural anticoagulant. If you have one or two of the genes then something is not working exactly right so your body is not producing enough protein C to keep your blood thin. Having 2 genes would mean Jake was very high risk for clotting so the good news is that he doesn't have 2.

Having one gene means he won't spontaneously clot however he is high risk if he is immobile for a long period of time or if he has a line (central, PICC etc.)  Only 2% of Caucasians have this gene so Jacob hit the odds again.

Jacob's Crazy Odds

• 1 in 100 babies are born with a Congenital Heart Defect (1%) 

• 4 in 10,000 babies are born with HLHS 

• Only 10% of those babies have an Intact Septum

• I have never found another Hypoplast with Bilateral Vocal Cord Paralysis but I do know that of the kids that had an airway like Jacob had (Pre-airway surgery) only 5% of those kids could breathe without a trache. BRAG: My little man didn't need a trache or Oxygen. 

• 2% of caucasions have the Factor Five Leiden gene. 

I still don't know why we can't hit these kind of odds with the lottery lol. Oh well we have our million dollar baby and the most amazing 5 year old EVER so we are rich where it counts :) 

This gene is passed down from a parent so either Andy or I also have this. Most "healthy" people with this gene go through their entire life without getting a clot so it is never a problem.  The only time it becomes a problem is if you are sick and need to be in bed for a very long period of time or have lines which can increase your chance of clotting. When Jacob had the clot he had been stuck in bed for 10 days on the ventilator. He also had a central line. Throw in the infiltration which added more pressure to the vein and it is not surprising that a clot formed. It is good that we know about this gene though because now we know that Jake has to be put on a heavier anticoagulant anytime he has a surgery or may be stuck in a hospital bed for a long period of time. They know about it now so it can prevent future clots. His cardiologist at CHOP and the hematologist in Cincinnati both agree that there is no reason for him to be on daily injections. 1 gene won't be enough of a risk to cause spontaneous clotting so he is not at risk on a daily basis.

As for the rest of us, they did not see a need for us to be tested. Andy or I have the gene however we are healthy and like I said before many people go their whole lifetime without a clot. I was concerned about Aidan and if I should have him tested but again they said there is no need. They said that the only time they feel it would be necessary to test him or us would be if we had a major procedure which required us to be immobile for a long period of time. Even then there is a chance we would not clot. Again I feel better knowing because if anything happens to any of us I would know to mention it so they could test and be proactive.

This was not the news I was expecting but it could have been worse. We are excited to be able to stop his shots. This will make him down to only two medications, his enalapril for blood pressure and a half a baby aspirin a day (Which we will start back up now that he will be off of the Lovenox shots).

SPEECH

Jacob continues to learn more words. He can now count to ten and can recognize numbers and some letters when he sees them. He also has learned his colors. He is making progress and he is showing us more and more that he knows these things and it is just a matter of getting the brain to be able to communicate it verbally. We have increased his speech therapy to help aggressively treat his apraxia. He is still getting speech once a week and developmental therapy once a week from Birth to three at our home.We are now also going to Cabell Huntington Hospital once a week to do outpatient speech therapy. So far it is going very well and Jake enjoys it.

We have a whole bunch of appointments coming up for Jake in December at Cincinnati Children's. So far here is what we are looking at.

December 5th

• Hearing Screen
• Vision Screen

December 6th

• Swallow Study (Speech)
• Speech Evaluation (Discuss thin liquids and getting gtube out)
• Speech (Apraxia specialist)get
• Hematology (Follow-up)

I am also trying to get his 6 month follow up broncoscopy scheduled to check his airway and also see if we can get his gtube taken out while up there.

It will be a busy week!!!

Happy Halloween
from my little super heroes!

Iron Man and Clark Kent: AKA Aidan and Jacob!

Stay tuned for my next blog post: 

THE TERRIBLE TWOS HAVE REARED THEIR HEAD!

( I promise I will post this soon, I need to vent lol)

Cincinnati Appointments: Part 1

Yesterday Jake and I traveled to Cincinnati Children's Hospital Medical Center. We had a ton of appointments and it was a very long exhausting two days. I have decided to break this into two blog posts because there is so much to cover.

We dropped Aidan off at school Tuesday morning around 7:30am then hit the road. 3 and a half hours later we were at the Children's Hospital but we had no idea just how long of a day we were in for. We went right up to Cardiology for Jacob's heart echo. He was not a happy camper and the ultrasound tech, child life specialist and myself worked tirelessly to get him to stay still so they could get what they needed. About an hour later they got all of the pictures (As good as they were going to get without sedation). We went straight to another room for the Neurodevelopmental clinic. We were going to participate in the clinic they offer at CHOP but found out that a cardiologist from CHOP recently went to Cincinnati Children's and started a clinic there. Since it is much closer to home I decided to bring Jacob there.

The Neurodevelopmental clinic is one of only a few programs in the country which provides individualized care to offer children with complex heart problems early intervention. Research shows that children with complex heart conditions are at greater risk for developmental delays. Jake is significantly delayed in speech so I want to be able to give him as many resources that I can to help him. Jacob saw a team of specialists that included a Cardiologist, a Cardiovascular Geneticist, a Developmental-Behavioral Pediatrician, A Pediatric Neurologist, an Occupational Therapist, a Nutrition, Social workers, and a Child Life Specialist. Each specialist came in one at a time, back to back and spent quite a bit of time going over his history, how he is developing and what we can do to help him. Then they all met as a team and went over their findings and came up with a plan to help him reach his full potential.

NeuroDevelopmental Cardiologist:Dr. Marino was the cardiologist who brought the program to Cincinnati and I really enjoyed meeting with him. He explained that kids with HLHS are considered very high risk for developmental delays compared to children without a congenital heart defect. That being said as far as HLHS kids go Jacob is actually at the low risk range because he has never had a stroke, seizure, been on ECMO or had other major issues which may put him in the high risk category. He told me that Jacob's echo looked great! This was music to my ears because I have been concerned lately that he has been more "Blue" and had lower sats. He said his heart function was great and everything looked beautiful. YAY!

Cardiovascular Geneticist:The geneticist went over our family history. We talked a lot about how two of my first cousins have children with heart defects and my uncle has a heart issue. The specific defects that my second cousins have do not seem to be related to Jake having HLHS. She does want Andy and Aidan to have cardiac echo's because they have found that there is a strong genetic link to Bicuspid aortic valve issues. This issues wouldn't show up until we are around 50 years old however it can cause serious complications then so we need to make sure we are not at risk. I have already had a heart echo and there was no issues with the Bicuspid Valve. I have been wanting to have Aidan have a heart echo anyway just to be safe and she was very persuasive that we need to have this done because of the strong link. So I am going to talk to Jacobs local cardiologist and see when we can get Aidan checked out.

Developmental-Behavioral Pediatrician:
We spent a lot of time with the developmental therapist discussing Jacob's speech issues. I talked to her about my concerns with him speaking in "Tones" and asked her if she thought it could be speech Apraxia. Apraxia of speech is a motor speech disorder. Children with this speech disorder have problems saying sounds, syllables and words. It is not because of muscle weakness or paralysis. The brain has problems planning to move the body parts (e.g., lips, jaw, tongue) needed for speech. The child knows what they want to say but their brain has difficulty coordinating the muscle movements necessary to say those words. Jake meets many of the criteria such as not babbling as an infant, not speaking until late, words made up of mostly vowel sounds, problems combining the sounds and having long pauses between them along with simplifying the words.

Jake talks in tones. Instead of the word "Thank you" he says "ahhh ooooou". "Outside" is "aaaa iiiide". He kind of has his own language which I have gotten used to and can understand. He has made so much progress since his airway surgery. Prior to his surgery his signing was more like babbling with signs. He didn't attempt any words. Because of his speech delays he is more like a 18 month old right now instead of a 2 and a half year old. This is based on how many words he can say. That being said there are so many factors involved so it is hard to know if the delay is Apraxia ( I went over this more with Neurology), or if it is because for the first 2 years of life he had to focus on breathing and couldn't focus on talking. Plus he couldn't hear due to the breathing so the "Sounds" that we were making sounded different to him. He also was not able to eat by mouth. The fact is he has really had about 5 months of hearing and being able to try and talk. In that time he has gained 7 months as far as "words". So he is making progress and should be able to talk but he needs aggressive speech therapy.

I discussed therapy treatment with her and she is going to help me find local resources as well. She wants Jacob to have a repeat hearing screen to make sure that he can hear words correctly. His last hearing screen was prior to his airway surgery so we need to know what sounds are going in to make sure that the "Tonal" speaking is not just because that is the sound he hears. We are also going to meet with a speech pathologist who specializes in apraxia. They can evaluate him and help us find resources. I have been talking to his local speech therapist about initiating a picture exchange system. PECs is a system that is used to help non-verbal children communicate.  I will create boards with pictures of objects that Jacob eats, uses or wants on them. We will teach Jacob to use these boards by picking what he wants and bringing it to me when he wants it. I will then give him that item. This will give him a more universal way of communicating. Once he masters this then we can teach him to use the iPad apps to be able to communicate with us and tell us what he wants or needs. We will continue to teach him sign language however most people do not know sign language. People are moving toward this way of teaching especially for when he goes to school. Not all teachers know sign language, most do not. So if a child would use sign language they would need an interpreter. Ipads have opened up a whole world of possibilities for children with communication delays. There are now programs where the child can type a sentence using pictures, hit a button and it will speak the sentence. Now kids can communicate with others despite the communication barrier and not need an interpreter. So we are going to continue signing and add the Picture exchange system along with finding more resources for aggressive speech therapy to teach him to talk.

We also talked about getting a vision screen because Jake has been squinting a lot and going really close to the ipad and TV (I mean nose against screen close). So we need to make sure that is ok as well.

Pediatric Neurologist:
The Neurologist and I discussed Jake's speech issues a lot as well. He really wanted to have Jacob have the hearing test as well just to rule that out as a cause. He said it is really hard to pinpoint at this time because of everything Jake has been through. It is hard to say if he was born with the heart defect and the Apraxia or if the heart defect caused the apraxia or if it is because he has hearing issues etc. Basically like the developmental therapist he wants to start at the most basic level and get the hearing test. He also said it is good that CHOP is doing a MRI prior to his fontan surgery (3rd open heart). This will show us if he ever suffered a stroke that we were not aware of during his surgeries or recoveries. He said it is possible for this to happen and us not know about it unless a scan like the MRI is preformed. They don't want to do one to check because he will have one pre-Fontan and honestly if he did then the damage is done and the treatment plan is the same... aggressive speech therapy. He did say that he was not convinced Jake has Apraxia because his motor skills are so good. Usually if there is Apraxia of Speech the kids show developmental delays in other area's as well and Jake is age appropriate in those areas. He did not have any other concerns as far as Jacob's neuro development. He did say Jake had a little bit of a big head for his height lol but so does Aidan. My boys have short legs but big brains!

Occupational Therapist:
I really enjoyed this session. Jake got to play! I was really impressed because he sat down and did her puzzle with no problems. At home he tends to play with the pieces but he grabbed her shapes and put them right where they belonged. She would draw a line on a piece of paper and say zoom and Jake did the same line and made the same sounds. The only things he didn't do was string round circles onto a string and use scissors but we have never worked on those things at home so we think it is from lack of exposure. I didn't realize kids that young should be using scissors lol but it is good for their hand strength. So now we know what to work on but it was great to hear that his motor skills are great and age appropriate!

Nutritionist:
Jake has always loved to eat so the Nutritionist was very happy with his weight gain and height. She said to keep doing what we are doing! We did talk about his continued problem with thin liquids. We agreed he needs to have a repeat swallow study so we can come up with a long term plan. His airway is healed so at this point if he were going to be able to do liquids he should by now. So we need to come up with a long term plan. I am going to try and get him to use the thickener and drink thickened liquids but if he refuses then we need to figure something out. Right now he drinks his milk through a 60ml syringe because he can only get small amounts out and he doesn't choke on them. This is not ideal though especially when he goes to school.

Social Workers and Child Life:
The social workers and child life specialist just helped us entertain Jake and discuss traveling and resources available to help us.

Once we met with everyone the specialists met and discussed their evaluations and came up with a Global Plan for Jake.

Global Impression: The doctors findings
1. Speech delay, and we need to rule out apraxia
2. Jake is age appropriate in fine motor, visual motor and gross motor skills
3. Jake eats a wide variety of foods and is growing well. He still struggles with fluid intake and takes everything by syringe.
4. No concerning dysmorphic features (Genetics)

Global Plan: The plan they came up with for Jake1. Hearing Test
2. Speech/Language evaluation by speech pathologist specialized in apraxia
3. Initiation of PECS (Picture Exchange Communication System), with the goal of using an augmentative communication device.
4. No formal occupational therapy recommended at this time however recommend follow through with home program recommended by occupational therapist in clinic.
6. Repeat swallow study and follow up with speech/ot to work on getting off the syringe and onto a sippy cup. Work on increasing fluid intake. Recommend additional milk to daily regimen.
7. Obtain genetic testing records from CHOP, Father and Brother to get echocardiogram to rule out bicuspid aortic valve.
8. General follow-up in the Clinic in 1 year

We didn't get out of the hospital until 6pm Tuesday night, it was a very long but productive day. We will be going back to Cincinnati Children's for a swallow study, hearing test and to meet with the speech pathologists to discuss resources and treatment.

To learn more about the Neurodevelopment Clinic visit www.cincinnatichildrens.org/hi-ndc


I will write all about Wednesday's visits in Part Two which I hope to post tomorrow night. Stay tuned...

Leaps and Bounds...

This blog post is dedicated the greatest man who ever walked this earth, my Papa.

George Cunningham lived 90 amazing years and I am so blessed to have had him in my life. He served our country, worked hard to provide for his family and was the best grandfather I could ever ask for. He never complained and always had the most contagious smile. The earth lost a great man but heaven gained the perfect angel. We will see each other again one day papa. Jacob now has the best guardian angel anyone could ask for. You will forever be in my heart.

I realize it has been over a month since my last blog post. My dad reminds me DAILY that I need to update the blog. I am sorry that I take so much time between posts but as you know "Life happens" and I don't get to jump on here as often as I would like. I do love that Jacob has so many fans and am so grateful that you care so much about our little guy. So lets get to it shall we!

Jacob's scab finally fell off of the back of his head. It looked pretty nasty at first (I will spare you the pictures) but thankfully it healed up very nicely and looks great now. He is still bald along the back of his head but I think it will all start to come back now that the area is healed.

We have been traveling so much the past few months. Philadelphia twice and 2 weeks ago we went to Massachusetts for my Grandfather's funeral. The boys did so well on the trip which is amazing because it was 16 hours in the car on the way there and 14 on the way back. It was so nice to see all of my aunts, uncles, cousins and most of all my nana. I missed everyone so much. It was very hard not having my papa there with us but I am so glad I was able to be there to see him one last time and say my final good-bye. The trip was really good for Jacob's speech therapy. The time in the car allowed us to have a captive audience with him to work on his signs. My sister was awesome and worked with him so much. Se used his baby signing time flashcards to teach him new words. The funny part is he memorized the order of the signs. He started telling us the opposite word whenever we asked him what a card meant. So now if we ask if he is hot, he responds with Cold. If we say Jake are you Dirty, he signs he is Clean. I was feeding him earlier today and told him it was hot. He looked at me and signed "Cold" lol. So it is kind of hard to teach him the meaning but it is still progress and something for us to work with. It was also great for him to be in a house full of people talking. It really encouraged him to jabber and try to talk. Overall he made great progress during the few days we were there.

Jacob is showing progress daily with his speech. It seems slow at times but when I look back to where he was prior to his airway surgery it is amazing that this is the same kid! He has been trying to say vowel sounds for a while now but a few weeks ago he started saying Choo-Choo. At first it was more like Wooo Wooo, then shoo-shoo but now he is making the "CH" sound which is not an easy sound to make. When he does it he puts his arm in the air and pulls down like he is ringing a bell. He does it every time he sees a train or if we say "Chugga-chugga". He is in the Thomas the Train stage big time. He would watch Thomas the train and Yo Gabba Gabba all day if I let him. He LOVES them. He grabs my hand brings me into his room then points at the TV and signs train. He won't fall asleep unless Thomas is on in the background. I have to sneak in once he falls asleep and turn it off. If I try to put him to bed without it he will scream and cry and yell Choo Choo until I put it on. He is so stubborn and strong willled, I have no idea where he gets that lol.

Jake has also started saying Hi (Hi-yee). I am always over pronouncing things to try and get him to say the words. It may have backfired a bit because instead of Hi, he says Hi-yee lol. It is really sweet. He waves to you as he says it. He still waves backwards like he is waving at himself. He gives you this big old grin. Too cute. He has said Bye a few times. Today he came into my room and signed "Yes" while saying "Yeah, Yeah, Yeah" and then he did the sign for "No" while saying "O, O, O". He was so proud of himself and I was jumping up and down I was so excited. He also FINALLY started saying "Mama" today. I have been trying to get him to say that again since his airway surgery.

He said the letters A, O, I and C. He also says yo, awe and tries to make up words all day long. He jabbers all day which is normally what infants do before they start talking. Jake never did that before because he never tried to talk when he had the labored breathing. He jabbered with signs then started signing appropriately. So now that he has had the airway surgery he is starting over verbally and is learning to speak. It is a very slow process but he is making progress and I am so proud of him. He is also learning more signs everyday and also learning to put multiple signs together. I think the more he learns the more excited he gets to try new words and signs.

Speaking for Jacob is much more difficult than it is for other children. Even though he had a complete airway reconstruction his vocal cords still are and always will be paralyzed. He can make sound but it is more breathy. It is hard for him to prounounce words. He can make shorter sounds easier than holding the sound out. It is much easier for him to mimic the tone of words rather than pronounce the word. He has started signing "Outside" and when he does he almost humms the word. It is really hard to put down in writing so I will have to get video of it. He basically will hear me say a word and then he will hum the syllables and sign. It is still huge progress for him, he is trying so hard to talk.

I have also noticed that it is easier for Jacob to say words if he makes a deep voice almost like a growl. Jacob was bit by a mosquito last weekend and his eye swelled up really bad. Aidan made a joke that he looked like a pirate and said Arg. After that Jacob would laugh and say Arg or Yo in the "Pirate voice". He started saying other words in that voice and I noticed it was easier for him to pronounce some letters when he would use the deep voice.That is actually how finally got him to say "Mama". I said it in the pirate voice and he repeated it. I am not sure if he can just get more sound or what it is but I am going to talk to his speech therapist about it. Not that I want Jake going around talking like a pirate or growling at people but it is a start lol.

Jacob's swollen eye from his mosquito bite.

Jake still gets very frustrated because he can't communicate as much as he would like. He gets angry if you don't understand what he is trying to tell you. It is getting easier because he is getting better at showing me what he wants but it is still hard, for both of us. I want so badly for life to be easier on him. Selfishly I won't deny that it is hard not hearing "I love you" from him. I see other moms talk about their child and how much they are talking and how they run up to them and say "I love you mommy".  Don't get me wrong I am so happy for them but sometimes I wish things were not so hard for Jacob. But then there will be moments when Jacob will run up and give me a hug and a huge grin and I know he is telling me in his own way.

We reached another big milestone. I finally felt comfortable enough to have Apria come pick up all of the Oxygen tanks, the big stand alone tank and the concentrator. It was a great feeling to see those taken out of the house. They have been here for two years. We are going to keep one small travel tank and the pulse ox machine. I don't see me getting rid of those anytime soon. I can pretty much guarantee that I will keep the pulse ox machine until at least 6 months post-fontan.

We will be heading to Cincinnati in about a month or so to meet with hematology and get another ultrasound of the clot. We will also be discussing the plan for his lovenox shots. Hematology generally will take kids off after 3 months of therapy but Jacob's cardiologist here and his Cardiologist at CHOP want him to be on it longer, most likely until his Fontan. Jacob is very high risk so we need to make sure he doesn't get any more clots prior to his next open heart surgery.

While in Cincy Jake will also get a cardiac echo. I have been a little worried lately because his lips have looked more "Blue". His oxygen levels have also been dipping into the mid 70s some which is not his norm. They always level out around 80 though which is normal for him. I spoke to his cardiologist and Dr. Heydarian said as long as he is leveling out around 80 then we should not worry. If he starts dipping and staying in the lower 70s or below then we may need to push up his Fontan. They want him to be at least 15 kilos for the fontan and right now he is about 12kilos (28lbs.). Right now the plan is to have his Fontan next year or possibly 2014 if he can wait that long. I am praying he can because studies are now showing that HLHS patients are showing signs of liver issues and the Glenn circulation is much better for their liver. CHOP is currently researching this but the hope is that if they wait as long as they can for the Fontan that the patients may not have as many liver issues like they are seeing now. I am kind of nervous for this heart echo because he has been more "Blue" lately so please pray that his heart function looks good. Jake has already had one major surgery this year so I do not even want to think about the Fontan yet.

We are also going to try and participate in the Neuro Cardiac Clinic while we are there. This is where Jacob would spend a day seeing about 6 different specialists who would evaluate him and give us advice on therapy that he should be getting. Hypoplasts are at risk for developmental delays because of the low oxygen levels. We know Jake is significantly delayed in speech so I think it is even more important that he participate. They will see him once a year prior to him going to school to help prepare him and make sure he is getting the most early intervention possible. I love that there are so many great programs for our kids.

I can't believe how fast these boys are growing up. Aidan will be starting Kindergarten in a few weeks! I hope to find a local "Mommy and Me" group for Jake and I to go to while Aidan is in school. I think having him around other kids who are verbal will really help him with speaking. It will also help him socially. Jake is a bit of a bully when it comes to other kids and sharing. Aidan being in school will give me more one on one time to work with Jacob.

Here are some pictures from our trip to Massachusetts. 

Jake and my Uncle John in Jail
(the bathroom aka the dogs room)

Brady fans eating cookies

Jake isn't really sure about this guy lol

Aidan, Jacob and their second cousin Tyler

So blessed

Plymouth Mass.

Mayflower II

The boys and I exploring the Mayflower

The boys and I in Plymouth Mass.

Plymouth Pebble Rock

Nana and Jake

Nana and my boys

Aidan with Uncle John's baby (His Shelby Mustang)

Aidan driving the Shelby

My Family

My Uncle Paul, Aunt Diane, Mom, Uncle John and Nana