Shawn's Memorial Open House
15502 Farmland Ct
Noblesville, IN 46060
Saturday
April 13, 2013
1:00 - 3:00 PM
Wednesday, April 10, 2013
Memorial Open House
Beth and I would like to welcome you all to our home for a Memorial Open House this Saturday, April 13th from 1:00pm - 3:00pm. We would like to gather all those who offered prayers and support to help us celebrate Shawn's fighting spirit and the inspiration that was his life.
Tuesday, April 9, 2013
Your fight is over. Rest now my little warrior.
About 2:30am this morning Shawn started bleeding out of his wound from last night's surgery. The nurses were unable to keep up with the blood and fluid losses and his little heart couldn't take it anymore. At 4:00am they had exhausted all options... the nurses wrapped him up, placed him in our arms, pulled his breathing tube, and he passed away peacefully a few minutes later. He was only with us for a short while but in that time he has shown me the strength of a million lifetimes. He has inspired me and enlightened me to the greatness of the human spirit. He was taken from us far too early but we are grateful for the little time we did have with him.
I'd like to give a big thank you to the medical staff at University and Riley Hospitals for doing everything they could to give us this precious time with our son. And thanks to all of you whose thoughts and prayers lifted him up and proved that miracles do happen.
You fought a good fight but now your fight is over. Rest now, my little warrior. Rest now in peace. We love you.
I'd like to give a big thank you to the medical staff at University and Riley Hospitals for doing everything they could to give us this precious time with our son. And thanks to all of you whose thoughts and prayers lifted him up and proved that miracles do happen.
You fought a good fight but now your fight is over. Rest now, my little warrior. Rest now in peace. We love you.
Monday, April 8, 2013
Out of Surgery
We just spoke with the surgeon and Shawn is out of surgery and back in the ICU. The good news is that he made it but the bad news is that just about his entire colon was gray dead tissue and had to be removed as well as a small section of his small intestine. They also bruised a section of his liver during the surgery which ruptured and bled. They had a lot of trouble with bleeding with all the heart meds he is on hurting his clotting ability so he is getting a heavy dose of blood transfusion. His belly was left open since the surgeon will need to go back in in 24 to 48 hours to ensure the diseased tissue did not spread further and to clean up from the excess bleeding. The next 24 hours will be very scary as they try to stabilize Shawn from this surgery coupled with the heart issues.
The surgeon said that if and when everything else gets sorted out with his heart he will be able to make Shawn digestive system work properly without a colon. This procedure should have a minimal impact on his life.
This will have a major affect on his surgical plan and will likely push everything back regarding his heart until this mess is sorted out and stable.
I hope this makes sense...... I'm an emotional wreck right now.
The surgeon said that if and when everything else gets sorted out with his heart he will be able to make Shawn digestive system work properly without a colon. This procedure should have a minimal impact on his life.
This will have a major affect on his surgical plan and will likely push everything back regarding his heart until this mess is sorted out and stable.
I hope this makes sense...... I'm an emotional wreck right now.
Emergent Abdominal Surgery
Just about right after I posted today, Shawn started a major slide for the worse. His abdomen started swelling, his sats went all over the place, and his blood pressure plummeted. The doctors believed he may have caught an infection. Beth and I immediately feared the worse. They started an aggressive antibiotic treatment but it was unlikely that his body would be able to sustain long enough for the treatment to work. They ordered an x-ray of his belly and it showed a possible hole in his intestine that is leaking fluid into his abdomen wall. This fluid is causing swelling and pressure to build. This pressure is making it impossible for Shawn's ventilator to provide adequate support to his body and heart. This news was devastating. For Shawn to make it this far...
Our little boy, Shawn, is currently in the operating room as I type this with trembling hands. At this point it was our only option for the surgeons to open his tummy and investigate. If they see dead portions of intestines they will remove and patch them. At the very least they will try and reduce some of the pressures he is experiencing and give his ventilator a fighting chance to allow him more time for the antibiotics to take affect.
Your prayers are needing now more than ever as our little heart warrior is fighting another battle against an unknown enemy.
Our little boy, Shawn, is currently in the operating room as I type this with trembling hands. At this point it was our only option for the surgeons to open his tummy and investigate. If they see dead portions of intestines they will remove and patch them. At the very least they will try and reduce some of the pressures he is experiencing and give his ventilator a fighting chance to allow him more time for the antibiotics to take affect.
Your prayers are needing now more than ever as our little heart warrior is fighting another battle against an unknown enemy.
Day 12 and a little of Day 13 too
His arrhythmia persisted all yesterday. Further down on his dopamine and fentanyl as well. Pretty quiet day which all changed early this morning. Around 5:00am this morning he was having some major fits with low heart rates in the 100s and dropping blood pressure. His lactates in his blood were also creeping up which is a sign his heart muscles were under stress. He lost his right hand IV which was giving him the prostagladin medication to keep his ductus open so he was not getting this medication for two minutes which may have caused the issues. After about 2 or 3 hours (felt like an eternity to Beth and I) he came out of it, numbers came up and stabilized, and he is much better now. Sats, heart rate, and blood pressure right where they want it. Lactates in his blood also normalized. They ran another central line this morning, this one in his femoral artery, which should be a more permanent access line to administer some of his medications.
Also this morning he decided to come out of his arrythmia and is now beating normally at a rate of 153 beats per minute!
Also this morning he decided to come out of his arrythmia and is now beating normally at a rate of 153 beats per minute!
Shawn's Snoedel hoodie when they turn the lights on! Thanks Aunt Erica!
Sunday, April 7, 2013
Day 10 & 11 - Our first bad day
Besides getting the news for Shawn's surgical plan, Friday was a relatively dull day in terms of Shawn's recovery and improvements. He was negative on fluids so his swelling continues to lessen (slowly but surely) and further decreasing his medications. Beth and I were able to change Shawn's diaper a couple of times so that was exciting. Never thought I'd see myself be excited about changing diapers!
Yesterday flat out stunk! Shawn evidently is growing tired of being a poster child of recovery and felt like being a little rebellious. He is now having an irregular heart beat or arrhythmia. It's very scary as a parent when first off you don't know what that means and secondly you have no idea the severity of the complications it can cause. To put it simply it is not good in the long term but for the short term Shawn seems to be tolerating it well. The doctors and nurses are alerted by it but not alarmed. Sometimes these heart kids just start this and it can last for a couple hours to a couple days. In Shawn's case the strong belief is that since his swelling is going down his heart is now irritated by the rather large metal stent inside it. His numbers still look good and his blood pressure is handling it well so the action plan moving towards Thursday is, "Keep an eye on it."
Yesterday flat out stunk! Shawn evidently is growing tired of being a poster child of recovery and felt like being a little rebellious. He is now having an irregular heart beat or arrhythmia. It's very scary as a parent when first off you don't know what that means and secondly you have no idea the severity of the complications it can cause. To put it simply it is not good in the long term but for the short term Shawn seems to be tolerating it well. The doctors and nurses are alerted by it but not alarmed. Sometimes these heart kids just start this and it can last for a couple hours to a couple days. In Shawn's case the strong belief is that since his swelling is going down his heart is now irritated by the rather large metal stent inside it. His numbers still look good and his blood pressure is handling it well so the action plan moving towards Thursday is, "Keep an eye on it."
Friday, April 5, 2013
Shawn's Surgical Plan
The doctors at Riley have Shawn penciled in for next Thursday, April 11, 2013 for his next procedure. They are going to proceed with what is called the Hybrid procedure and not the traditional Norwood approach.
Stage I, Part 1
The first stage of the procedure will be to remove the stent placed in this atrial septum at birth and perform an atrial septectomy to create a permanent hole for communication between the upper two chambers. This will ensure that he is mixing oxygenated blood and non-oxygenated blood sufficiently. Next they will band his pulmonary arteries so to restrict blood flow and not flood his lungs and starve his body of blood. This will all happen on Thursday. He will need to go on bypass for a short time to allow for the septectomy. This is typically not necessary but they feel differently in Shawn's case since the current stent is quite long and may be interfering with proper drainage from the heart.
Stage I, Part 2
The second part of this procedure will happen the following day or early the next week. They will place a stent in his ductus (PDA) to allow him to get off of the prostaglandin medication that is keeping it open. There is potential for major side effects with prolonged exposure to this medication. After next week his heart should look like the picture above except for the atrial septum stent where he'll just have a hole and not a stent.
Stage II, Part 1 (Norwood) & Part 2 (Glenn)
The idea of the Hybrid approach (and the main reason it's Shawn's best option) is to minimize the trauma to the heart right after birth. It allows some time for the heart to grow stronger and in Shawn's case his lungs to heal. The doctors believe that if they went right into the Norwood after birth then his heart would not be strong enough after the surgery to over power the pressures in his lungs. The typical Stage II of the Hybrid approach would combine the Norwood and the Glenn, as previously described, into one large procedure at 4 - 6 months of age. This will differ for Shawn as well. Instead of one large trauma to the heart at 4 - 6 months the thinking is to break this stage into two smaller traumas to the heart and continue to give his lungs the maximum amount of time to heal. They will do all the steps of the Norwood at around 4 months then he'll come back at 8 - 9 months old for the steps of the Glenn. Both procedures have been previously explained in the "About HLHS" page.
Stage III, Fontan
The third and final stage will be as previously described at 2 - 4 years.
All of this is subject to change depending on the success of the procedures and the state of his recoveries.
Going into this morning I was hoping to feel relief and excitement that we would finally have a plan going forward. Now that I know what I know I am just afraid. The idea that he will have 5 major procedures within his first year of life is just flat out scary.
Stage I, Part 1
Stage I, Part 2
The second part of this procedure will happen the following day or early the next week. They will place a stent in his ductus (PDA) to allow him to get off of the prostaglandin medication that is keeping it open. There is potential for major side effects with prolonged exposure to this medication. After next week his heart should look like the picture above except for the atrial septum stent where he'll just have a hole and not a stent.
Stage II, Part 1 (Norwood) & Part 2 (Glenn)
The idea of the Hybrid approach (and the main reason it's Shawn's best option) is to minimize the trauma to the heart right after birth. It allows some time for the heart to grow stronger and in Shawn's case his lungs to heal. The doctors believe that if they went right into the Norwood after birth then his heart would not be strong enough after the surgery to over power the pressures in his lungs. The typical Stage II of the Hybrid approach would combine the Norwood and the Glenn, as previously described, into one large procedure at 4 - 6 months of age. This will differ for Shawn as well. Instead of one large trauma to the heart at 4 - 6 months the thinking is to break this stage into two smaller traumas to the heart and continue to give his lungs the maximum amount of time to heal. They will do all the steps of the Norwood at around 4 months then he'll come back at 8 - 9 months old for the steps of the Glenn. Both procedures have been previously explained in the "About HLHS" page.
Stage III, Fontan
The third and final stage will be as previously described at 2 - 4 years.
All of this is subject to change depending on the success of the procedures and the state of his recoveries.
Going into this morning I was hoping to feel relief and excitement that we would finally have a plan going forward. Now that I know what I know I am just afraid. The idea that he will have 5 major procedures within his first year of life is just flat out scary.
Day 9
Yesterday was pretty quiet and Shawn's standard m. o. He seemed to be playing a little game with the RN where he'd drop his sats and the nurse would increase his ventilator oxygen then his sats would go too high and the nurse would lower this oxygen. This went on about every 5 to 10 minutes throughout the day. I could almost see a little grin on his face!
I've been feeling a little melancholy the last couple of days as my excitement and adrenaline of the delivery wore off and was replaced with what I imagine to be the usual feelings of a worried parent with a child in the ICU. I have even started thinking that maybe I initially misunderstood the doctors and it wasn't the delivery that was his major risk and my enthusiasm was misplaced. I was also starting to worry that I was getting overly confident and optimistic about his recovery thus far. I told Dr. Cordes (Shawn's cardiologist on duty this week) how I felt and he told me my enthusiasm was not misplaced. The odds stacked against him at the delivery and first surgery were extraordinary. They were just as excited as we were for him to be here at this point to continue fighting. He also echoed my same worries that they are even battling their own confidence and optimism during this recovery as he is doing so well. They still need to remind themselves that he is still a very special case needing specific treatment and a custom surgical plan. I would like to thank Dr. Cordes for taking that time with me yesterday and to validate my feelings of late and somewhat calm a few of my worries.
This morning is Riley's surgical conference and as I mentioned in the last post we'll have a better idea of the plan going forward for Shawn. I have been told that our doctors here have been talking at length with Cincinnati and Boston Children's hospitals about Shawn's case. It is comforting to know that the best of the best in the country are collaborating to help give my son the best care possible. I'll post again this afternoon after we talk with the doctors.
I've been feeling a little melancholy the last couple of days as my excitement and adrenaline of the delivery wore off and was replaced with what I imagine to be the usual feelings of a worried parent with a child in the ICU. I have even started thinking that maybe I initially misunderstood the doctors and it wasn't the delivery that was his major risk and my enthusiasm was misplaced. I was also starting to worry that I was getting overly confident and optimistic about his recovery thus far. I told Dr. Cordes (Shawn's cardiologist on duty this week) how I felt and he told me my enthusiasm was not misplaced. The odds stacked against him at the delivery and first surgery were extraordinary. They were just as excited as we were for him to be here at this point to continue fighting. He also echoed my same worries that they are even battling their own confidence and optimism during this recovery as he is doing so well. They still need to remind themselves that he is still a very special case needing specific treatment and a custom surgical plan. I would like to thank Dr. Cordes for taking that time with me yesterday and to validate my feelings of late and somewhat calm a few of my worries.
This morning is Riley's surgical conference and as I mentioned in the last post we'll have a better idea of the plan going forward for Shawn. I have been told that our doctors here have been talking at length with Cincinnati and Boston Children's hospitals about Shawn's case. It is comforting to know that the best of the best in the country are collaborating to help give my son the best care possible. I'll post again this afternoon after we talk with the doctors.
Thursday, April 4, 2013
Day 8 - Happy 1 Week Birthday, Shawn!!
Well you were brought into this world 1 week from yesterday with quite a splash! Your head at 9:46 am and the rest of your body 4 minutes later. The last week has not been without its ups and down... mostly in oxygen saturations (a little inside joke for me and him), but you have blown away our expectations. You have inspired us all with your strength and resolve. Please continue to persevere and know that you have a massive community of support and the prayers of many behind you, cheering you on. We love you, my son....my little heart warrior!
Yesterday they already discontinued the lung percussion therapy since his x-rays looked good. That's good since I saw them do one yesterday morning before rounds and boy oh boy did he not like that! He is off insulin and continuing to ween off the dopamine. His breast milk feed is up to 10 mL/hr now. They are scheduling an echo for today to get a full and complete pic of his heart. Every Friday they have a surgical meeting to discuss any upcoming surgical planes. Tomorrow Shawn will be the feature topic. So hopefully after tomorrow morning we'll have a better idea about the whens, whats, whys, and hows of his next procedure and possibly the game plan for future procedures.
Shawn, 1 week old!
His big boy socks!
Wednesday, April 3, 2013
Day 6 & 7
Beth and I fell ill yesterday so my apologies for not updating. We both woke up with nausea and diarrhea so we weren't able to see Shawn. It tore me apart not being able to be with him, but it was for the best. So with that I'll update two days in this post.
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Monday started rough but ended well. Shawn is now completely off his paralyzing medication and he woke up within an hour of them stopping the line. He's moving his feet and hands which will help push the excess fluids in his tissue back into his blood stream. He's been opening his eyes too! Beth and I were pretty thrilled about that. However, he did not like being able to move at first and it was a little tense for a couple hours Monday morning. He was grimacing in pain it looked like, so they upped his pain meds. He would fight the ventilator and hold his breath which plummeted his oxygen sats into the 40s so a couple of the doctors had to come in and just watch him for a bit. He settled down a little after noon and he's been pretty good since. Sats holding strong in the upper 70s to lower 80s. Moving his little feet and now taking his own breaths! The ventilator still makes sure he gets his 28 breaths a minute but it doesn't stop him from starting one on his own. If he initiates a breath the vent will help him and make sure it's not like breathing through a straw. The monitor will show a red line on breaths he starts and a green line on the vent breaths.... it's pretty cool and I have another thing to watch! Although we were sick and unable to see Shawn yesterday we were able to get a hold of the nurse in charge and get updates. Shawn was good and stable all day. They stopped the direct nutrition line (TPN) and he's just straight breast milk which makes Beth happy! He's down on his dopamine drip and tolerating that well. His x-ray from yesterday showed that his right upper lung was slightly deflated. This is common for kids on the ventilator. So he is now getting lung percussion therapy every two hours where they take a little suction cup looking device and pat his upper chest and back. I'd like to give a big shout out and a thank you to Jill, Shawn's nurse yesterday, for keeping us informed. She called us yesterday afternoon so we could FaceTime with our little guy! So awesome! Thanks, Jill!
Monday, April 1, 2013
Day 5 - A Word of Caution
Day 5 brings more stability and yet more improvement. His lungs continue to slowly get better as the pressure needed from his ventilator is lessening which is a good indication of the fluid leaving the lungs. They started Shawn on a slow drip of Beth's breast milk (3mL/hour) so she is thrilled to finally see some fruits of her pumping labors! He also had his first poop yesterday! It's nice to celebrate the simple things... makes him seem more like a normal baby boy.
There's not much to update as he was pretty stable yesterday and they haven't wanted to rock the boat too much just yet so I'll take this opportunity to discuss some words of caution. I'm sure many have noticed a change of tone on this blog since Shawn's birth. Justifiably so, I'd say, considering how well the delivery and atrial septostomy went. The prognosis and likely outcomes of last Wednesday's events as the doctors were quoting us leading up to the big day led Beth and I to think and plan for the worst and hold onto only a small thread of hope. As hope turned into reality throughout the course of Wednesday morning, joy washed over me. Our son is alive and we are so very blessed to be at this point today. However, I do not want my elation to misdirect anyone. I do not want to give the impression that he is fine and will be coming home soon. Shawn is a very sick 5 day old in intensive care. We have made very tiny baby steps of improvements towards recovery and all that hard work can be wiped away in a matter of minutes. The issues with the intact atrial septum caused major back pressure in his lungs and pulmonary vasculature causing severe hypertension and developmental issues. These will take weeks if not months to heal and correct themselves. Right now we are playing a waiting game as they tweak little things here and there as he is able to provide more and more support for himself. Nothing major will happen this week. They want to make sure he is as strong as possible for the next procedure. We are in for a long hard road ahead and we are only getting started.
Please continue your prayers and thoughts for little Shawn and all the nurses and doctors at Riley Children's Hospital as they continue to work miracles on our little guy.
There's not much to update as he was pretty stable yesterday and they haven't wanted to rock the boat too much just yet so I'll take this opportunity to discuss some words of caution. I'm sure many have noticed a change of tone on this blog since Shawn's birth. Justifiably so, I'd say, considering how well the delivery and atrial septostomy went. The prognosis and likely outcomes of last Wednesday's events as the doctors were quoting us leading up to the big day led Beth and I to think and plan for the worst and hold onto only a small thread of hope. As hope turned into reality throughout the course of Wednesday morning, joy washed over me. Our son is alive and we are so very blessed to be at this point today. However, I do not want my elation to misdirect anyone. I do not want to give the impression that he is fine and will be coming home soon. Shawn is a very sick 5 day old in intensive care. We have made very tiny baby steps of improvements towards recovery and all that hard work can be wiped away in a matter of minutes. The issues with the intact atrial septum caused major back pressure in his lungs and pulmonary vasculature causing severe hypertension and developmental issues. These will take weeks if not months to heal and correct themselves. Right now we are playing a waiting game as they tweak little things here and there as he is able to provide more and more support for himself. Nothing major will happen this week. They want to make sure he is as strong as possible for the next procedure. We are in for a long hard road ahead and we are only getting started.
Please continue your prayers and thoughts for little Shawn and all the nurses and doctors at Riley Children's Hospital as they continue to work miracles on our little guy.
Saturday, March 30, 2013
Day 4 - Continuing Progress
Continuing to look better each day. He is doing well on the TPN but needed to start an insulin line due to low blood sugars. His weight in the morning was 8 lb 15 oz but that should come down as he continues to lose fluids. He did look rather puffy and swollen this morning but looks much better this evening. He has been negative fluid (more out than in) all day and that is great as he reduces this swelling. They took out his catheter so he's peeing in diapers now! They monitor the fluids out by simply measuring the dirty diapers. They also removed the chest tube for drainage from the surgery so he doesn't have that big tube coming out of his chest. He's far from a normal baby boy but he's looking more and more like one every day. Check out the handsome guy in the picture below!
Today was actually pretty quiet and uneventful as he has maintained very nice oxygen levels in the mid to upper 70s, blood pressures around 60/40, and heart rate in the low 160s. His surgeon, Dr. Turrentine, came by to check on him today and said he is looking and doing well. Once we get the rest of this extra fluid out of his system in the next few days we'll be able to start game planning for the next procedure.
Beth has been discharged and feels pretty good. She had a little elevated temperature last night and feels like she may have pushed herself a little too much yesterday trying to get back and forth from University to Riley in order to see Shawn and still be treated for her surgery. She is doing much better today!
Today was actually pretty quiet and uneventful as he has maintained very nice oxygen levels in the mid to upper 70s, blood pressures around 60/40, and heart rate in the low 160s. His surgeon, Dr. Turrentine, came by to check on him today and said he is looking and doing well. Once we get the rest of this extra fluid out of his system in the next few days we'll be able to start game planning for the next procedure.
Beth has been discharged and feels pretty good. She had a little elevated temperature last night and feels like she may have pushed herself a little too much yesterday trying to get back and forth from University to Riley in order to see Shawn and still be treated for her surgery. She is doing much better today!
Friday, March 29, 2013
Day 3 - Stable and Improving
Day 3 brings much the same as day 2. More recovery and more improvements. They continue to fiddle with his medication cocktail to try and gradually ween him off of a few things. He is showing more swelling today due to the excess fluids in his system but continues to pee like a champ to combat that. They started a nutrition line (TPN) so he'll start to get some calories, amino acids, vitamins, etc. It goes directly into his heart to be immediately distributed and absorbed into his body so his stomach is still sitting idle at the moment.
His oxygen saturations are remaining more stable while needing less intervention to do so. This is a great indication that his lungs are indeed improving. Right now as I write this at 9pm his oxygen is holding nicely at 78% and his blood pressure is at 61/38. Both right where the doctors want it. He's also only getting normal air amounts of oxygen through his ventilator so all that oxygen saturation he is doing all on his own!
Only real alarm of the day was an unplanned echo screening to look at his stent to make sure it didn't move. The stent as shown in this morning's chest x-ray looked quite a bit different then the x-ray I showed on the last post, yesterday's x-ray. They wanted to perform an echo to just make sure the thing wasn't floating around in there. The echo clearly showed the stent, that it passed through the septum, and that blood freely flowed through it. So the stent had not moved. They probably just took the x-ray at a slightly different angle today as opposed to the first two days. A needless scare but if that's all we'll have to deal with then we'll gladly take it.
His oxygen saturations are remaining more stable while needing less intervention to do so. This is a great indication that his lungs are indeed improving. Right now as I write this at 9pm his oxygen is holding nicely at 78% and his blood pressure is at 61/38. Both right where the doctors want it. He's also only getting normal air amounts of oxygen through his ventilator so all that oxygen saturation he is doing all on his own!
Only real alarm of the day was an unplanned echo screening to look at his stent to make sure it didn't move. The stent as shown in this morning's chest x-ray looked quite a bit different then the x-ray I showed on the last post, yesterday's x-ray. They wanted to perform an echo to just make sure the thing wasn't floating around in there. The echo clearly showed the stent, that it passed through the septum, and that blood freely flowed through it. So the stent had not moved. They probably just took the x-ray at a slightly different angle today as opposed to the first two days. A needless scare but if that's all we'll have to deal with then we'll gladly take it.
Thursday, March 28, 2013
Day 2 - Recovery
Shawn is recovering well. Beth and I were present for his first day of rounds and we were told that he is doing much better today then yesterday. That is to be expected as he just had a major open heart procedure yesterday! His oxygen saturations are keeping somewhat stable in the mid to high 70s which is fantastic. He is having a little trouble keeping his blood pressure up but it seems like a major balancing act what with all the chemicals and fluids he is on. They started him on a diuretic which will help get the fluid that's been backed up in his lungs this whole time in utero out of his system. It will also help with his swelling. The doctors are feeling like his lungs are definitely getting better since his saturations are looking so good with also the concentrations they are seeing in his blood draws. Check out this crazy picture I was able to take of this morning's chest x-ray. You can see the IV real easy and if you look real close you can see the stent they placed yesterday. It is HUGE!!! Pretty incredible stuff.
First hair washing!
His foot... just because!
Here's a good pic to see just some of the medications/fluids he is on.
Beth is doing remarkably well. Up and moving like a champ and the doctors are amazed how good her incision looks and how well she is recovering. She still is obviously very tired from everything and with having to pump every 3 hours she has had little opportunity to sleep.
Big sister, Zoey, came for a visit today! Beth and I were so excited to see her. She was as happy as ever and is having a blast hanging out with Grandma and Grandpa! She won't be able to see her little brother for a few months but when she does she'll no doubt love him as we love him. It's comforting to know that Shawn will have a tough brute of a big sister to look out for him.
Wednesday, March 27, 2013
Pics from the BIG day!
Here are a few pictures of the day that we were able to grab and the staff at Riley were kind enough to take for us during the procedure. Shawn and Beth are still doing great. Shawn's blood oxygen levels have settled around 75% saturation which is right where the doctors want to see it. He is already my inspiration. Everything went as the doctors had hoped. We are both so very blessed and Shawn is living proof that miracles do exist!
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About to go into the OR. Always positive and smiling. I know where Shawn gets his strength!
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Dr. Farrell was able to grab some great pics from the operating room for the delivery and Shawn's surgery. Incredible and inspiring but I'll save you the gory details.
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Our little guy hooked up and resting after the procedure. Looking good with a full head of hair. His big sister Zoey will be so jealous!
Beth was able to sit up and get in a wheel chair after barely an hour after her c-section to go see Shawn. The power of a mother's love!
Happy Birthday Shawn!!!!
He made it through with flying colors!!!! Strong, kicking, and stable!! He's my little boy that's for sure! What a fighter!!!! The doctors were able to deliver him and rush him to the next room where his doctors were waiting. They had his chest open within 15 minutes and had a hole punctured through the septum and a stent placed quickly. All without the need to go on bypass! Blood oxygen levels went from the 30s to the 90s immediately!
Beth is also great with no complications! Truly a blessed Poppa right now.
I want to thank everyone for their thoughts and prayers. I assure you they were answered today!
Also a big thank you to all the miracle workers at Riley and University Hospital. You guys rock!!
Welcome to the world, little man! We won the first first round now let's keep going!
Beth is also great with no complications! Truly a blessed Poppa right now.
I want to thank everyone for their thoughts and prayers. I assure you they were answered today!
Also a big thank you to all the miracle workers at Riley and University Hospital. You guys rock!!
Welcome to the world, little man! We won the first first round now let's keep going!
Thursday, March 14, 2013
The BIG day
We had another ultrasound and echocardiogram on Tuesday and Shawn is still getting bigger and bigger. He is now 5 lbs 11 ozs. He passed his biophysical profile exam with flying colors and if not for his heart you'd think he was a strong, thriving baby boy. We got a little more information on what to expect at the delivery and a little bit thereafter but maybe most importantly...
We have a date! Shawn's birthday will be March 27th. Speaking with Dr. Cordes and Dr. Farrell they have scheduled two adjacent operating rooms at Riley for 3/27. That will be only 36 weeks along for Beth and Shawn but the doctors are not confident that with the excess fluids Beth is carrying that she will make it much past that date. Going into unexpected labor and having to pull together the necessary resources in a rush is a much greater risk to Shawn then being delivered two more weeks early. The doctors have stated that he is big enough currently to do these procedures. They will perform the c-section and post op on Beth in one room and work on Shawn in the next. In fact they will be performing what is called an EXIT procedure where they will deliver him halfway and begin working on him while still attached to Beth. They will secure breathing tubes and IVs before cutting the umbilical cord. They will then rush him into the next room where a team will be waiting to open up the atrial septum either via cath or surgery.
To get an idea of the level of coordination that will be needed on the 27th, Dr. Farrell sent us the below list of all parties needing involved for this delivery. Shawn will enter this world with quite an entourage! The whole team will consist of 30+ medical professionals.
We have a date! Shawn's birthday will be March 27th. Speaking with Dr. Cordes and Dr. Farrell they have scheduled two adjacent operating rooms at Riley for 3/27. That will be only 36 weeks along for Beth and Shawn but the doctors are not confident that with the excess fluids Beth is carrying that she will make it much past that date. Going into unexpected labor and having to pull together the necessary resources in a rush is a much greater risk to Shawn then being delivered two more weeks early. The doctors have stated that he is big enough currently to do these procedures. They will perform the c-section and post op on Beth in one room and work on Shawn in the next. In fact they will be performing what is called an EXIT procedure where they will deliver him halfway and begin working on him while still attached to Beth. They will secure breathing tubes and IVs before cutting the umbilical cord. They will then rush him into the next room where a team will be waiting to open up the atrial septum either via cath or surgery.
To get an idea of the level of coordination that will be needed on the 27th, Dr. Farrell sent us the below list of all parties needing involved for this delivery. Shawn will enter this world with quite an entourage! The whole team will consist of 30+ medical professionals.
1. High risk OBs
2. Pediatric Cardiology
3. Pediatric Cardiac Interventionalists
4. Pediatric CV surgery
5. Pediatric Cardiac critical Care/Intensivists
6. Pediatric anesthesia
7. Neonatology
8. Pediatric general surgery
9. OB adult anesthesia
10. Perfusion team
11. Cardiac OR/scrub team
12. OB scrub team
If Shawn survives the surgery and the subsequent atrial septum intervention we will play a wait and see game. Both events of the days will be extremely invasive and stressful on the little guy. If they succeed in opening the septum, relieving the pressures, and stabilizing him after the procedure, the rest will be on him and how much he wants to fight. If he responds well and seems to be making improvements then we can proceed with the next stage.
As a parent you want to shelter your children from the perils of the world. You want them to smile and laugh, hug and love. You want to minimize their recollection of pain to a faint and distance memory. It pains me to know that my son will go through more pain and peril in his first 29 minutes than I have in my first 29 years of life. It pains me greater that there is little more I can do for him at this point. I hope he knows that I will be there for him. I hope he knows I will be his biggest supporter, his biggest cheerleader. I hope he knows that I will be right there in the corner as he fights this. Even though he does not know me yet nor I him, I hope he knows that I love him.
Monday, March 11, 2013
So Many What Ifs...
I realize it has been some time since our last update. Things were tough for us the days following our failed trip out to Boston. We tried to spend some time not thinking about it, not thinking about anything. Maybe it was disbelief or denial that all this could be happening to us. Maybe it was the surmounting stress of everything that we were feeling at the time. We tried to simply get away from it for a few days and focus on Zoey and ourselves. We realized quickly, however, that while you can shut your mind off at times along the journey your legs continue to carry you further down the road and it doesn't take long for obstacles to present themselves to snap you back into reality.
The last month has been filled with doctor appointments, more ultrasounds, another echo cardiogram, blood work, and stress tests. Beth has been admitted to the hospital's triage area twice in the last month for extended monitoring. Once for further signs of pre-term labor and again for Shawn being unresponsive during an ultrasound. I've felt him kick like Chuck Norris and Beth says he moves around like an Olympic gymnast in there so they must have caught him in a nap that day. Each time we were discharged after several hours and free to go home but they take a major hit towards our mental and emotional state.
As far as Shawn and how his condition is progressing, it continues to worsen. With each ultrasound he grows bigger and bigger and he is already past 5 lbs, but that is where the silver lining ends. During Shawn's last echo they were able to confirm that his atrial septum is completely intact and there is zero communication and no way to mix oxygenated blood between the upper chambers. His left atrium is also considerably small relative to his condition. That creates accessibility and safety concerns when trying to open the atrial septum after delivery. He is already showing signs of thickening and dilation in his pulmonary veins as well as developing abnormalities in his lungs due to the extreme pressure. The doctors have told us that he will not make it out of the delivery room alive without some unprecedented levels of emergent intervention. They are currently in the midst of evaluating different options, organizing mock trial runs, and developing a delivery and immediate care plan for Beth and Shawn. Due to the immense level of coordination this delivery will require they have said they will likely be pushing our delivery up to 37 - 38 weeks to minimize the risk of Beth going into labor early. We have another echo scheduled tomorrow morning and we will get a chance to sit down with the cardiologists and see what they come up with.
We are in uncharted medical waters here in the severity of Shawn's condition and our level of resolve to fight even for the slightest chance that he can survive and be the beautiful, thriving baby boy that Beth and I see in our dreams. There are so many what ifs at the moment. We are hoping to be able provide a more specific update tomorrow.
The last month has been filled with doctor appointments, more ultrasounds, another echo cardiogram, blood work, and stress tests. Beth has been admitted to the hospital's triage area twice in the last month for extended monitoring. Once for further signs of pre-term labor and again for Shawn being unresponsive during an ultrasound. I've felt him kick like Chuck Norris and Beth says he moves around like an Olympic gymnast in there so they must have caught him in a nap that day. Each time we were discharged after several hours and free to go home but they take a major hit towards our mental and emotional state.
As far as Shawn and how his condition is progressing, it continues to worsen. With each ultrasound he grows bigger and bigger and he is already past 5 lbs, but that is where the silver lining ends. During Shawn's last echo they were able to confirm that his atrial septum is completely intact and there is zero communication and no way to mix oxygenated blood between the upper chambers. His left atrium is also considerably small relative to his condition. That creates accessibility and safety concerns when trying to open the atrial septum after delivery. He is already showing signs of thickening and dilation in his pulmonary veins as well as developing abnormalities in his lungs due to the extreme pressure. The doctors have told us that he will not make it out of the delivery room alive without some unprecedented levels of emergent intervention. They are currently in the midst of evaluating different options, organizing mock trial runs, and developing a delivery and immediate care plan for Beth and Shawn. Due to the immense level of coordination this delivery will require they have said they will likely be pushing our delivery up to 37 - 38 weeks to minimize the risk of Beth going into labor early. We have another echo scheduled tomorrow morning and we will get a chance to sit down with the cardiologists and see what they come up with.
We are in uncharted medical waters here in the severity of Shawn's condition and our level of resolve to fight even for the slightest chance that he can survive and be the beautiful, thriving baby boy that Beth and I see in our dreams. There are so many what ifs at the moment. We are hoping to be able provide a more specific update tomorrow.
Wednesday, February 6, 2013
Boston Bust
So we arrived in Boston on Sunday met by our friends Mitch and Steph. We had a great time hanging out, watching the Super Bowl, and enjoying their company. We checked out Fenway Park, took a tour, and Adam got a beer at the Bleacher Bar underneath the Green Montsa! It was a nice break from everything and almost made us forget the trials of the days ahead.
We got to Boston Children's at 8am to start out our full day of tests and screenings. We met with Dr. Wayne Tworetzky for another fetal echo and a consultation. He confirmed the diagnosis along with the intact atrial septum and we discussed the following days procedure. We were feeling good and ready to tackle this procedure. We continued over to Brigham and Women's Hospital for an ultrasound and to meet with the high risk OB there who would be working with Dr. Tworetzky. That's where everything took a turn for the worse.
During the ultrasound they noted Beth's cervix had shortened as if she was already showing signs of labor. They rushed us over to the labor and delivery floor and hooked Beth up to some monitoring devices. The monitors did pick up some consistent yet mild contractions. After four attempts to start an IV and 10 hours in a cramped 8x8 room we learned that with the pre-term labor we were no longer candidates for the fetal intervention procedure. We were now at a much greater risk for the procedure to result in Beth going into full blown labor in which case our little guy would certainly not survive. There was also a much greater risk to Beth rupturing membranes or the placenta breaking away from the uterus wall.
We are now back to square one with the added worry that Beth could now go into labor at any time. Sometimes it feels that the universe is aligning against us and little Shawn. It's getting harder to hold on to hope.
We got to Boston Children's at 8am to start out our full day of tests and screenings. We met with Dr. Wayne Tworetzky for another fetal echo and a consultation. He confirmed the diagnosis along with the intact atrial septum and we discussed the following days procedure. We were feeling good and ready to tackle this procedure. We continued over to Brigham and Women's Hospital for an ultrasound and to meet with the high risk OB there who would be working with Dr. Tworetzky. That's where everything took a turn for the worse.
During the ultrasound they noted Beth's cervix had shortened as if she was already showing signs of labor. They rushed us over to the labor and delivery floor and hooked Beth up to some monitoring devices. The monitors did pick up some consistent yet mild contractions. After four attempts to start an IV and 10 hours in a cramped 8x8 room we learned that with the pre-term labor we were no longer candidates for the fetal intervention procedure. We were now at a much greater risk for the procedure to result in Beth going into full blown labor in which case our little guy would certainly not survive. There was also a much greater risk to Beth rupturing membranes or the placenta breaking away from the uterus wall.
We are now back to square one with the added worry that Beth could now go into labor at any time. Sometimes it feels that the universe is aligning against us and little Shawn. It's getting harder to hold on to hope.
Saturday, February 2, 2013
Boston or Bust
Well we finally got the word from the coordinators at Boston Children's and we are set for the fetal cardiac intervention on Wednesday the 6th! We will be heading to Boston on Sunday. Monday will be a relax day and hopefully be able to catch a few of the sights in Boston. Adam said something about maybe taking a tour of Fenway Park. We are looking forward to seeing our friends Mitch and Steph. Steph lives around Boston and has been gracious enough to let us stay with her. We're so fortunate to have such awesome friends! Tuesday will be a full marathon day of ultrasounds, echocardiograms, and meetings with the doctors who will be doing the procedure to make sure that Shawn is still a candidate for the fetal cardiac intervention. After that we will meet with the high-risk OB doctors to make sure that I am able to go through with the procedure. When everything turns out okay we will just have to sign consent forms and then we will be all set for the procedure on Wednesday. We are not sure on a time for the intervention but we will know more once we get out to Boston. We will have to stay in the hospital for at least one night maybe two, and then will stay around Boston another day to make sure everything is fine. Just wanted to thank everyone for the support you have been giving us and please contiune to pray for Shawn as we go through the fetal cardiac intervention.
Friday, January 25, 2013
Fetal Intervention and Boston Children's Hospital
We posted twice today so make sure you read the post about the Jan 16th echocardiogram first before reading this one! So this post will bring us all up to present day so you all will have the current status of where we are on this journey. Sorry so long to get people caught up but there was a bucket load of information to relay since starting this blog. Back to it...
Beth and I waited 6 days to hear back from Boston and finally received word on Wednesday of this week that we are indeed candidates and that a fetal intervention procedure would benefit our son. We spoke with Dr. Kevin Friedman from Boston and he explained the procedure, some of the logistics, and potential risks and outcomes. Here's quick breakdown of what he said. Now this is an oversimplification but basically they take a long needle and will go in through Beth's abdomen, into the womb, into Shawn's heart, and poke through the atrial septum. They will blow up a tiny balloon inside the right atrium and pull it through the atrial septum hopefully leaving a sufficient hole. They will go back in and implant a stent to keep the hole open during the remainder of his development. The hopes of this procedure if successful is that it will allow immediate flow and blood communication between the two upper champers. This will effectively relieve any current pressure in his cardiovascular system and prevent any future such development. We will hopefully catch it early enough to allow any damage already done time to correct itself in the safe environment of the womb. This procedure also has the potential to eliminate the need for Shawn to undergo emergent intervention right after birth. He'll be able to recover from the strains of birth and get nice and strong for the Stage I surgery.
Now I want to make something clear. This is not a fix all. This does not place us back in the 80% survival rate of the typical HLHSer. As with anytime you invade the environment of the womb you have a chance to lose the baby. In this case we have a 10% chance of this to occur. Also only 75-80% of these procedures are deemed successful and an adequate hole was created. A lesser number yet that a stent is able to be properly implanted. Overall survivability for this condition in where a fetal intervention was performed is slightly above 50% which is a significant bump from 10-20% if the atrial septum was left alone and dealt with after birth.
As with many parents dealt these cards, when the time comes, we want to be able to throw the best possible hand down on the table. Beth and I feel and Dr. Farrell agrees that this procedure will give our little guy the best chance at survival and it looks as though we'll be heading to Boston! We don't have anything final yet but the Boston doctors want this to happen within the next 2 to 3 weeks. We'll keep everyone posted when we find out more.
Beth and I waited 6 days to hear back from Boston and finally received word on Wednesday of this week that we are indeed candidates and that a fetal intervention procedure would benefit our son. We spoke with Dr. Kevin Friedman from Boston and he explained the procedure, some of the logistics, and potential risks and outcomes. Here's quick breakdown of what he said. Now this is an oversimplification but basically they take a long needle and will go in through Beth's abdomen, into the womb, into Shawn's heart, and poke through the atrial septum. They will blow up a tiny balloon inside the right atrium and pull it through the atrial septum hopefully leaving a sufficient hole. They will go back in and implant a stent to keep the hole open during the remainder of his development. The hopes of this procedure if successful is that it will allow immediate flow and blood communication between the two upper champers. This will effectively relieve any current pressure in his cardiovascular system and prevent any future such development. We will hopefully catch it early enough to allow any damage already done time to correct itself in the safe environment of the womb. This procedure also has the potential to eliminate the need for Shawn to undergo emergent intervention right after birth. He'll be able to recover from the strains of birth and get nice and strong for the Stage I surgery.
Now I want to make something clear. This is not a fix all. This does not place us back in the 80% survival rate of the typical HLHSer. As with anytime you invade the environment of the womb you have a chance to lose the baby. In this case we have a 10% chance of this to occur. Also only 75-80% of these procedures are deemed successful and an adequate hole was created. A lesser number yet that a stent is able to be properly implanted. Overall survivability for this condition in where a fetal intervention was performed is slightly above 50% which is a significant bump from 10-20% if the atrial septum was left alone and dealt with after birth.
As with many parents dealt these cards, when the time comes, we want to be able to throw the best possible hand down on the table. Beth and I feel and Dr. Farrell agrees that this procedure will give our little guy the best chance at survival and it looks as though we'll be heading to Boston! We don't have anything final yet but the Boston doctors want this to happen within the next 2 to 3 weeks. We'll keep everyone posted when we find out more.
Jan 16th: Second Echocardiogram
So Wednesday last week was our second echocardiogram. The last few weeks prior have been a whirlwind of activity from switching hospital systems, OB appointments, more ultrasounds, and hospital tours with this next echo always in the forefront of our mind.
We went in with a few questions prepared but our main concern was that atrial septum. Would she be able to see that hole and if not could she tell if his system was already under pressure? As Dr. Farrell started the echo it became evident to us that the atrial septum was her main goal as well. She focused heavily on that wall looking each and every direction trying to see some relief in the left upper chamber. Here are some of her observations as she scanned:
The drive home was not pleasant as everything started sinking in. January 16th was a bad day and hope was waning. Later that day Beth received a phoned from Dr. Farrell explaining that she was conferring with her colleagues about our echo. Riley does not have much experience with this sort of HLHS complication since it is so rare. It came up that a children's hospital in Boston is performing procedures in utero to combat this complication in attempt to decompress the system and allow the baby a better shot at life. We went back the following day to have another echo and send the images to Dr. Wayne Tworetzky (profile here) at Boston Children's Hospital who has been specializing in this condition. He would determine from the extra set of scans if our little Shawn would be a candidate and see some benefit from this kind of procedure. After this echo it was just another waiting game for Boston to take a look and email us back.
We went in with a few questions prepared but our main concern was that atrial septum. Would she be able to see that hole and if not could she tell if his system was already under pressure? As Dr. Farrell started the echo it became evident to us that the atrial septum was her main goal as well. She focused heavily on that wall looking each and every direction trying to see some relief in the left upper chamber. Here are some of her observations as she scanned:
- No hole in the septum or flow between the two upper chambers were obviously detected so Dr Farrell began looking for other signs.
- The atrial septum was bowed outward toward the right atrium chamber kind of like a balloon blowing up. This creates a lot of suspicion that the septum is indeed intact and the restricted blood flow from the lungs into the left atrium is causing pressure and this bowing.
- The septum wall was thick looking where it is typically very thin. Another suspicion that the wall is counterattacking back pressure by thickening.
- There were two possible "squirts", as Dr. Farrell described, of blood flow in between the two chambers that may be some relief of pressure. This was inconclusive.
The drive home was not pleasant as everything started sinking in. January 16th was a bad day and hope was waning. Later that day Beth received a phoned from Dr. Farrell explaining that she was conferring with her colleagues about our echo. Riley does not have much experience with this sort of HLHS complication since it is so rare. It came up that a children's hospital in Boston is performing procedures in utero to combat this complication in attempt to decompress the system and allow the baby a better shot at life. We went back the following day to have another echo and send the images to Dr. Wayne Tworetzky (profile here) at Boston Children's Hospital who has been specializing in this condition. He would determine from the extra set of scans if our little Shawn would be a candidate and see some benefit from this kind of procedure. After this echo it was just another waiting game for Boston to take a look and email us back.
Tuesday, January 22, 2013
Our Visit to Riley
So we had our tour of Riley Hospital for Children and the hospital Shawn will be delivered at. I will labor and deliver in one room and then after I have recovered Adam and I will move to another room. After delivering Shawn he will be taken to a room called the resuscitation island where they have every kind of machine you could imagine plus more! Hopefully he won't need anything but if he needs it it's there. Adam will be able to go with him in there and supervise the situation. He will likely have an intern standing with him to explain everything they are doing. Once stable and there's no need for emergent intervention he will come back to our room for a few minutes so I can see him before he is wheeled over to Riley via a tunnel. He will be set up in the NICU where he will stay until his first surgery. Adam will be able to follow him over to Riley and I will be able to go over once I am stable and can be wheeled over to see him! The only bad thing about Shawn going straight to the NICU is that they don't allow anyone under the age of 18 in there so Zoey will have to wait a little while to meet her baby brother.
After the labor and delivery, Riley will become our second home. It's hard to estimate with any certainty but Shawn will be in the hospital for at least a month and possibly longer depending on any complications that may arise. They have many things for us to do while at Riley; they have a library where you can check out books, games, CDs and even go and play games on the game systems! Whenever Zoey will get to visit she'll have a blast going up and down the open glass elevators, riding in the red wagons, and tearing up the lobby like the little tornado she is. They also have a small Ronald McDonald house at the hospital where you can go and take a nap or a shower and they also have a pantry that we can use to make a meal! A lot of generous groups also come and supply cooked meals at the Ronald McDonald house for Riley families and we have been urged to take advantage of that. It's nice to know we won't have to live on fast food and hospital cafeterias while we're there.
Riley is one of those places you never hope to have to go but with all the wonderful things they are doing and accomplishing, it's comforting to know it is there if your little one needs it.
After the labor and delivery, Riley will become our second home. It's hard to estimate with any certainty but Shawn will be in the hospital for at least a month and possibly longer depending on any complications that may arise. They have many things for us to do while at Riley; they have a library where you can check out books, games, CDs and even go and play games on the game systems! Whenever Zoey will get to visit she'll have a blast going up and down the open glass elevators, riding in the red wagons, and tearing up the lobby like the little tornado she is. They also have a small Ronald McDonald house at the hospital where you can go and take a nap or a shower and they also have a pantry that we can use to make a meal! A lot of generous groups also come and supply cooked meals at the Ronald McDonald house for Riley families and we have been urged to take advantage of that. It's nice to know we won't have to live on fast food and hospital cafeterias while we're there.
Riley is one of those places you never hope to have to go but with all the wonderful things they are doing and accomplishing, it's comforting to know it is there if your little one needs it.
Monday, January 21, 2013
Intact Atrial Septum
At the first echo when Shawn was diagnosed Dr. Farrell mentioned a possible complication of what is called an intact atrial septum. I'm going to try and explain what that is and what that may mean for Shawn.
The atrial septum is the wall between the two upper chambers (left and right atrium) of the heart. All fetuses have a hole between these chambers that will close up after birth. In HLHS'ers this hole is key to their anatomy because without a left ventricle there is no way of getting oxygenated blood to the body. This hole allows the blood coming from the lungs to enter the left atrium and mix with the blood in the right atrium and then pump to the body. Dr. Farrell was unable to positively identify that hole in the first echo and was concerned about the pressure and tension this is possibly causing our little guy. She explained to us the outlook of HLHS along with an intact or highly restrictive atrial septum is not good and greatly reduces his chance of survival.
The atrial septum is opened up as part of the standard Norwood procedure so fixing this condition is not an issue after birth. The major complications that arise occur as a developing fetus. With that wall intact there is no where for the blood from the lungs to go causing a lot of pressure and tension in the left atrium and pulmonary veins from the lungs. The veins react to this pressure by thickening and muscularizing to toughen up. That then becomes our biggest threat to our son if it were to happen and there is nothing we can do about it while he is growing in the womb. He will likely need emergent medical intervention after birth to open the atrial septum and relieve that pressure. Even after he becomes stable from this procedure and they go in for the Norwood the damage from 4-5 months of high pressures in utero cannot be undone. The thickened pulmonary veins will still restrict flow from the lungs and he will not be able to deliver sufficient oxygen to his body.
This condition affects only 6% of HLHS babies so if this is Shawn's case then the odds are truly not in our favor! Further echocardiograms will hopefully provide the doctors with better images and they can pin down the severity of what's going on in there. We will keep you posted.
Friday, January 18, 2013
Information on HLHS
A video from the Children's Hospital of Philadelphia:
There is no known cause of HLHS. It affects 1 in 10,000 babies and is more common in males than females. It is sometimes (about 10%) accompanied with other developmental birth defects but that is not evident or is it a concern in our case. Something in our favor! HLHS is treated with a series of three staged and highly intensive open heart surgeries as listed below.
Stage 1 - Norwood procedure @ 1 week old
Stage 2 - Glenn procedure @ 4-6 months old
Stage 3 - Fontan procedure @ 2-4 years old
Now these procedures do not cure HLHS. Shawn will have HLHS for the rest of his life. These procedures only work to reconstruct the heart and circulatory system to manage with only one pumping chamber.
If successful these kids live a somewhat normal life. They go to school, play sports, hang out with friends, and even attend college someday. That's not to say that after the three surgeries Shawn's story will end in roses and happy ending. He will never be able to run a marathon, be a power lifter, or any other physically extensive things. The road will be perilous and potential for complications, infections, and other developmental problems will always be around every corner. He may need subsequent surgeries to clean up scar tissue or a pacemaker or even a full heart transplant are possible down the road. These procedures were developed in the mid-80s so there is limited knowledge on the outlook of survivors as they mature into adulthood but we are anxious to find out as we continue this journey together.
Some useful links:
Boston Children's Hospital: HLHS
Children's Hospital of Philidelphia: HLHS
Our Journey Begins
We truly appreciate all the outpouring of love, concern, and offered
prayers for Shawn and our family that we have received from friends and family over the past few
weeks. It really has helped us weather this storm. The main reason for this blog and email list is to give all of you updates about little Shawn as we forge this path down the road of HLHS. We will do our best to try and post as updates come to us.
Let us start at the beginning of this journey and I apologize for the novel here, but there's a lot to update. It was just another 20 week ultrasound. We were so excited to finally find whether the baby would be a boy or a girl. We had the technician put the sex of the baby in the envelope as it was our plan to open and reveal at dinner that night. From our untrained eye and the technician's remarks everything was looking and measuring on track. When we spoke with our OB doctor he confirmed that everything was good but they didn't get a good look at the heart and he wanted us to go for a high resolution ultrasound at the hospital. We were told this is customary. Babies tend to move around in there (a lot in our case) and it's tough sometimes to get a good look at everything. We didn't think anything of it after that and were anxious to get to dinner and open that envelope! We had a nice relaxing night out and when we opened the envelope we were ecstatic to find out we were having a boy! Neither Beth or myself have any siblings of the opposite sex so we were excited to watch that dynamic and relationship development between Zoey and her new little brother.
A week went past and we showed up to the high res ultrasound ready to knock it out and move on. This ultrasound was like all others except when the Maternal-Fetal doctor couldn't find all four chambers of the heart. She left and came in with a textbook and started reading to us about the possibility of Hypoplastic Left Heart Syndrome. The words hit us like a ton of bricks. We were both devastated and terrified by what the doctor was telling us. From what the ultrasound was showing our little guy had a severely underdeveloped left side of his heart. Basically he would be born with half of a heart. This is an extremely rare and complex congenital heart defect and the doctor didn't know much else about it. We were asked to come back in two days and see a cardiologist from Riley Children's Hospital for a fetal echocardiogram. The next 48 hours were grueling. Filled with endless research on the internet but with all the maybe's and potentials we just ultimately switched to denial. Our hopes shifted to the doctor being wrong and we'd go to the echo and everything would be fine. The echo proved the opposite and all our fears were realized when the pediatric cardiologist Dr. Anne Farrell (see her profile here) confirmed the diagnosis that our precious baby did indeed have Hypoplastic Left Heart Syndrome.
What happened over the next couple minutes as Dr. Farrell spoke surprised us. We were no longer in despair, no longer in the grips of the unknown. We knew our enemy and Dr. Farrell was giving us the weapons to fight. She spoke of the three stages of surgeries that we will outline in another post. She spoke about Riley Hospital and the fantastic things they are doing there for sick children. She gave us hope. We came out of the appointment emotionally exhausted but also feeling somehow renewed. There was no doubt this journey will be difficult but now we know to steel ourselves for the tribulations to come.
Let us start at the beginning of this journey and I apologize for the novel here, but there's a lot to update. It was just another 20 week ultrasound. We were so excited to finally find whether the baby would be a boy or a girl. We had the technician put the sex of the baby in the envelope as it was our plan to open and reveal at dinner that night. From our untrained eye and the technician's remarks everything was looking and measuring on track. When we spoke with our OB doctor he confirmed that everything was good but they didn't get a good look at the heart and he wanted us to go for a high resolution ultrasound at the hospital. We were told this is customary. Babies tend to move around in there (a lot in our case) and it's tough sometimes to get a good look at everything. We didn't think anything of it after that and were anxious to get to dinner and open that envelope! We had a nice relaxing night out and when we opened the envelope we were ecstatic to find out we were having a boy! Neither Beth or myself have any siblings of the opposite sex so we were excited to watch that dynamic and relationship development between Zoey and her new little brother.
A week went past and we showed up to the high res ultrasound ready to knock it out and move on. This ultrasound was like all others except when the Maternal-Fetal doctor couldn't find all four chambers of the heart. She left and came in with a textbook and started reading to us about the possibility of Hypoplastic Left Heart Syndrome. The words hit us like a ton of bricks. We were both devastated and terrified by what the doctor was telling us. From what the ultrasound was showing our little guy had a severely underdeveloped left side of his heart. Basically he would be born with half of a heart. This is an extremely rare and complex congenital heart defect and the doctor didn't know much else about it. We were asked to come back in two days and see a cardiologist from Riley Children's Hospital for a fetal echocardiogram. The next 48 hours were grueling. Filled with endless research on the internet but with all the maybe's and potentials we just ultimately switched to denial. Our hopes shifted to the doctor being wrong and we'd go to the echo and everything would be fine. The echo proved the opposite and all our fears were realized when the pediatric cardiologist Dr. Anne Farrell (see her profile here) confirmed the diagnosis that our precious baby did indeed have Hypoplastic Left Heart Syndrome.
What happened over the next couple minutes as Dr. Farrell spoke surprised us. We were no longer in despair, no longer in the grips of the unknown. We knew our enemy and Dr. Farrell was giving us the weapons to fight. She spoke of the three stages of surgeries that we will outline in another post. She spoke about Riley Hospital and the fantastic things they are doing there for sick children. She gave us hope. We came out of the appointment emotionally exhausted but also feeling somehow renewed. There was no doubt this journey will be difficult but now we know to steel ourselves for the tribulations to come.
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