Adult CHD Surviver - Hazel

The founder of CHD-UK shares with us her Personal Story of what it is like to grow up and live with a CHD.

---

An Overview to the main story:
I am a miracle to be alive especially when as I was born in early 70's with Transposition of the Great Arteries and without a hole, I needed to be born with a atrial septal defect as well for the blood flow but I wasn't. However, they operated on me by giving me a balloon sepostmy and then I had major open-heart surgery 2 years later (the Mustard procedure - This operation is still performed but ONLY in certain situations where the Switch is not suitable).  I was 2 years and 2 weeks old when I underwent this surgery. There was no information or awareness about CHD then and there still isn't.  Now, there is a growing population of adults with CHD which did not exist 30 years ago. We are called GUCH in the UK but I prefer to be called an adult with CHD.  I am one of the first generation of survivors and I don't know how long I will live for and whether I will get any more side effects - There is no set rules for what can happen with deterioration with Transposition of the Great Arteries for my generation, one person may need a heart transplant, another will need a valve transplant and another may only need a pacemaker.....

Introduction of myself
I am adult with CHD, I am currently in my late 30's and I grew up in Gloucestershire, lived in London and I now currently live in Spain where I teach English to the Spanish. I have one younger sister who does not have CHD and I am still undecided about having children because all through growing up I was told I could not have children but then five years ago I find out that I can from my Specialist. I have been monitored all my life and I will be for the rest of my life.

I belong to the first generation of congenital heart disease survivors.  In the UK we are called GUCH but in America it is called ACCA.

How and why did I survive to become a GUCH/Adult CHD'er?
I will now give you a bit more detail about what I was born with. The congenital heart disease it should be called defect I was born with is what is medically known as Transposition of the Great Arteries. I had several operations before I was 2.5 years old, these were the balloon septostomy in 1973 and The Mustard Operation (open-heart surgery) in 1973 performed by a Dr Besley (the arterial switch is now the preferred operation). What is Transposition of the Great Arteries ? What is a balloon septostomy? What is the Mustard Operation?

Babies born with Transposition of the great arteries are known as blue babies due to the insufficient oxygen circulating in their bodies. Transposition of the great arteries is where the blood from the lungs flows back to the lungs and blood from the body flows back to the body. The two major arteries coming out of the heart, the aorta and the pulmonary artery are connected to the wrong chambers. Such babies look blue because insufficient oxygen is circulating in their bodies.

A balloon septostomy is normally a palliative procedure used to prepare a patient for, or sustain them until a corrective surgery can be performed. At this time the ASD is closed using either sutures or cardiac patch, depending on the size and/or nature of the opening.

The balloon septostomy is the widening of a foramen ovale, patent foramen ovale (PFO), or atrial septal defect (ASD) via cardiac catherisation (heart cath) using a balloon catheter. This procedure allows a greater amount of oxygenated blood to enter the systemic circulation in some cases of cyanotic congenital heart disease (CHD)

After the catheter is inserted, the deflated balloon catheter is passed from the right atrium through the foramen ovale, PFO or ASD into the left atrium, it is then inflated and pulled back through to the right atrium, thereby enlarging the opening and allowing greater amounts of blood to pass through it. The resulting man-made opening is one of many forms of shunting, and is often referred to as an ASD.

The Mustard procedure restores the circulation but reverses the direction of the blood flow in the heart. In a normal heart, de-oxygenated, blue, blood is pumped into the lungs via the right ventricle. Then it is distributed throughout the body via the left ventricle. In the Mustard procedure, blood is pumped to the lungs via the left ventricle and disseminated throughout the body via the right ventricle.

But the right ventricle is not the optimal shape to support the high pressure work performed in a normal heart by the left ventricle. Over a period of 20 or 30 years the right ventricle may begin to fail.

'Was it tough growing up with CHD? Has CHD held me back? How does CHD influence my daily life now?' To the last question, I asked my boyfriend his opinion because I just couldn’t think and his reply was “you think everyone is against you and that you have to fight a battle all time as in "you are very defensive and sensitive”. I suppose what he said is all true and that this all comes from fighting my way to live from being born and trying to survive until I had the Mustard Operation and fighting to live after the operation (that was all a battle). I also think that being defensive and sensitive is a reaction to dealing with the bullying I got for having a CHD at secondary school.... this was mainly due to the fact that I had to wear a 24 holtor monitor, which was the size of the brick, in fact, I remember a kid pulling the monitor off me and throwing it over the netball nets. He got into some trouble for doing that especially as the equipment did not belong to me it belonged to the hospital but that was the extreme bullying and only happened once. I was not allowed to do sports half way through the first year of secondary school this was due to a teacher pushing me and not letting me stop when I knew I had enough and now I am too scared to push myself now when I am exercising. I have also heard since that other teachers doing the same to other children with CHD. There is no awareness, we look normal, like anyone in the street – our condition is not staring people in the face, so they think we are making it up!!!!

I have always lived day to day, having fun. My moto is ‘You have only one life, live life with no regrets and always do what you want to do. Me, I have had a second chance at life so I live it to the full and I am very spontaneous lol.

How did my parents cope?
My parents think that it is a miracle that I am here at all and are proud of me no matter what I have done or do. I am not pushed to do anything great, just to have a life. The reason being is due to the fact that back in the 1970’s there was no internet nor was medical advancement that great. My parents had no one to really turn to; they knew no one in their area who had a child with CHD. There is a charity belonging to Bristol NHS Teaching Hospital where I had the operations and I am still monitored but that was it. My mother still raises money for this charity. My parents had a hard time dealing with having a child with CHD but they eventually coped and to my mind it made them a stronger couple. Before I had the operation my parents had to give me tablets to keep me alive but if I was sick, they could not give me another tablet or it would kill me. This scared them and I cannot imagine what they went through. The lack of help and awareness back when I was born has made me think that there should be more awareness in the world but there isn’t. Can you believe it nearly 40 years later there is still a lack of awareness which is why I decided to set up the CHD-UK page on MySpace and the CHD-UK group on Facebook and now this website.

My parents also had to face the fact that they had to move house because where they lived was too cold for me and they always had the heating on. If I was not kept warm I would go blue due to the cold, the heart has to pump harder to keep the circulation flowing and your body automatically protects its vital organs.  Even now I still suffer from the cold, my lips, fingers and toes all go blue.  I need to keep warm or otherwise my heart will be working too hard just to keep my fingers, toes and lips from going blue.

As I mentioned before I am one of the first generation of survivors also known as GUCH/Adult CHD'er. We are the ones who are now having major or minor problems with our health, some with no insurance causing us increased and unnecessary stress (USA), having to pay for prescriptions for medication that is necessary to keep us well. Having to pay expensive travel insurance just to go abroad, having to pay more for insurance just because we have a CHD. To my mind, we are healthier than the average citizen because we are monitored every year whereas the average citizen isn’t. The government will not do anything about this! In fact, we will be a future drain on governments of all countries. They have no idea yet what happens when CHDs with old repairs (people ages 55 and up) mix with acquired heart disease.

For me, having Transposition of the great arteries the major or minor problems that can arise from the Mustard procedure can vary from arrhythmias to having a heart transplant. I currently have intermittent nodal rhythm and intermittent atrial tachycardia/fibrillation and I still do not know what the future is. But with the old repairs yes they do in a way know what will happen but they are not 100% sure because everyone's problems that arise are different and I also think the age should be from 30+ because my arrhythmias which are caused from an old scaring from the operation started when I was in my early 30s and luckily I still do not have the pacemaker yet, but I will need definitely in the future, but after that I do not know what problems will arise nor do the Specialists.

At the moment I wouldn't change anything in my life, I am who I am, defected heart included – it’s made me who I am today. That is a strong, outgoing, stubborn (had to put that in), independent, thoughtful, caring person that I am today.

Baby Ashley Part 2

Continuing from earlier...

-------------------------------------

And then we got the shock of our lives. Dr. Finley, Ashley's cardiologist came in for her pre-op exam. He commented on how much better her heart sounded, and said he had to go look over her echo photos, and then he'd be back. 

An hour later he returned. He started talking as he was walking into the room but for the life of me I can't tell you what he said. I knew what he was saying, but I couldn't register the words. All I know is that he was telling us that surgery was canceled.

Mark and I started crying, as we held Ashley. Dr. Finley went on to explain that her echo showed that the VSD was getting smaller. That combined with the fact that she improved so much with the extra medication, meant they could cancel the surgery, and just wait and see what the VSD did.

We cried the whole way back to the hotel. We went and told the roomful of waiting grandparents and relatives the good news. Then we all went out to dinner to celebrate. It was the first time that the four of us: Mark, myself, Thomas & Ashley had ever been out to dinner together like a "normal" family.

We were amazed, since Ashley's VSD is a type that is rare to close. She has a membranous VSD with an inlet extension, meaning it involves her Tricuspid Valve. Only 5% of VSD's involve the inlet area, and it's very rare for them to close. Also, doctors had been telling us for months, that it was impossible the hole was ever going to get small enough to avoid surgery, and that her heart failure was too significant. Yet here we were, surgery canceled.

Only two days later, after also seeing a GI Specialist to rule out mal-absorption problems, given her slow growth, we were on our way home, a week earlier than planned.

Since she showed such improvement, the cardiologist cut Ashley's medications in half, however, within a week of being home, she was back in heart failure: her liver was swollen, her weight gain dropped and her breathing rate was picking up again. Over the next two weeks her medications were adjusted until finally, she was on more medication than she had been previously.

Once the medications were increased, we started seeing some improvements - Ashley became stronger and more active. She was much more alert than she used to be. We honestly didn't realize how lethargic she was as a baby, but we received numerous comments from friends, family and neighbors on what a huge improvement there was. We couldn't see it, because we were around her all the time, we didn't see the difference.

Shortly after coming home, we were able to get rid of her NG tube. She had increased her intake enough, that while she was just meeting her daily minimum intake, she was doing it. We had to feed her every two hours, but it was worth it to be able to see her beautiful face.

I wish that the story could have ended there, that things would have just continued to get better and easier, but they didn't. Ashley's weight gain continued to slow down. Her liver continued to swell, and she started drinking less again. They increased her Lasix yet again. Doctor's also started talking about surgery again. Her cardiologist said that while it was worth giving her every chance to let the hole close, that it was likely she would still need surgery. Her family doctor also thought that surgery would likely still happen. Shortly after the last Lasix increase, one day I noticed that Ashley wasn't peeing as much as she used to. However, at that time, I didn't really think anything of it. However, after a week, her output volume continued to drop. Babies generally adhere to the rule of "what goes in must come out". If they take in 750 ml in a day, then roughly 750 ml should come out. With a baby on diuretics, it's actually expected that even more should come out, than goes in. I knew Ashley wasn't peeing enough.

We started weighing her diapers as we had been shown to. She was peeing out only 1/3 of the volume that was going into her. Eventually it got so bad that she was only peeing out 1/4 of the volume going in, which was getting near the dangerous levels, in that her kidney's were no longer able to filter out poisons such as creatine.

Her pediatrician finally ruled that the Lasix was the problem. She was on too high of a dose, and it actually dehydrated her. The purpose of Lasix is to reduce fluid volume in the body, which reduces blood volume, thereby reducing the workload of the heart. Unfortunately, her last dosage increase had reduced her blood volume too much, and it reduced the amount of blood circulating through the kidneys, which in turn, reduced her urine output.

Her Lasix was cut in half immediately, and she had to undergo blood work to check her kidney function, to ensure that her poison levels were okay. Thankfully, we had caught it early, and all was well. Left untreated, it could have led to kidney failure. It took a couple weeks, but her output did eventually pick up. Not as much as it had been a month or so ago, but more than it had been after the Lasix.

We had no sooner gotten over this scare, when it was decided that her NG feeding tube had to be reinserted. Ashley had gone from drinking 25 ounces per day, to only taking 18-20 ounces. Her family doctor felt one of the reasons that her urine output wasn't back up to normal amounts was because she just wasn't taking in enough fluid volume. Also, her weight gain had dropped significantly. She had gone from gaining an average of 13 ounces per month to only having gained 12 ounces in the last 2 months.

I was devastated. Part of me felt guilt, as it was our decision, with the doctor's okay, to take the NG tube out in the first place, and see how she did. Granted her gain had slowed even before it came out, but still... Also, I'll admit, I was enjoying truly seeing my daughter without a yellow tube coming out of her nose, and her cheeks covered in tape. But the main reason my heart broke was for Ashley - she hated the tube, and was a much happier baby without it.

The NG tube re-insertion did not go well, and it only lasted a week and a half, three or four of which days the tube was actually out. She screamed, she cried, she didn't sleep well, and oddly enough this time around, as soon as it went in her nose would get instantly congested, to the point that she actually couldn't even breathe while eating. She would want to nurse, but couldn't breathe, and would cry and scream.

I finally had enough, ripped it out one morning, and the next time she saw the doctor, I told her they will never put another one of those things in my daughter again. She said the difficulties this time around were likely due to the fact that she was so much older, and much more aware. Again, I felt the guilt. Perhaps, had we just left it in, she would have remained conditioned to it, and it wouldn't have been a problem.

Finally, on December 21st, 2010, Ashley had her follow up with cardiology. Two months had passed since the canceled surgery date. We were both anxious for this appointment, as we knew we were going to be informed about the likelihood of surgery being needed. We honestly didn't know what to think. On one hand, Ashley was the strongest and most active we had ever seen her. She was not only meeting physical milestones, she was meeting them early! At 6 & 1/2 months of age, she was sitting completely on her own, she was crawling, and able to pull herself up to stand.

On the other hand, she was still on three different heart medications, her weight gain was slow, and she wasn't eating very much, two of the major criteria for if surgery was indicated. We went to the regional hospital located three hours from our home, where the cardiology clinic was being held. Ashley underwent her 5th echo, her EKG having been done a couple weeks earlier at home. We went through all the normal preliminaries, weight and height check, and blood pressure check. We were then left to wait for the doctor.

I will never forget December 21st, it will always be a special day in my heart, for it is the day that Christmas came early for us.

Dr. Chen, the cardiologist who we were seeing that day came in, and did his thorough exam of Ashley. He talked in depth to us going over details of what had been happening the last couple months, where we were at now. And then he got down to what we were waiting to hear, the echo results.

Once again, we sat stunned, and I in tears, while listening to what the man in front of me had to say. Ashley's ASD was gone, and her VSD had closed significantly! Her VSD was now so small, they hadn't even bothered to measure it, and the pressure gradient had increased from 64 to 108! There was no sign of either her tricuspid or aortic valves leaking. Her echo and EKG were perfectly normal, her heart function was perfectly normal, and it was back to a normal size.

"Your daughter is never going to require heart surgery," he said. Tears ran down my face as I thanked him for the news. Tears ran down my face the whole way out of the hospital.

We were also advised that she was completely out of heart failure, and were told to remove all medications, that she didn't need them. He said he expected that her VSD would close completely - a true miracle, since we had been told by numerous doctor's that that was a complete impossibility. He cleared us for a year, at which time they would bring us back just to check and make sure the VSD had closed okay, and without complications - of which he didn't anticipate any.

When we got to the car, I started sobbing, absolutely sobbing. I can't even begin to tell you all the emotions that were flooding through me at that moment. It was the culmination of six months of agony, fear, frustration, anger, worry, love, stress, exhaustion. Of hospitalizations, endless doctor's appointments, tests, blood work and feeding tubes. It was tears of thankfulness and praise to God, whom I credit fully with Ashley's healing - though I do also think that a large portion of her miraculous healing is her own spirit and determination, but that too, was given to her by God. It was the relief and utter joy of knowing that she would never require 

 open heart surgery. It was the release of a lifetime lived in only six short months.

 
♥

Ashley did phenomenal coming off of the medications, and within days not only did her appetite pick up, but her urine output is now back to normal. She continues to remain on concentrated calorie formula, as she still doesn't eat enough in a day to take in the required amount of calories, and her growth is still slow. 

At her December appointment, the cardiologist told us that Ashley's weight issues no longer have anything to do with her heart, and probably hadn't for a month or two. She is going to be evaluated by a team of speech, occupational and feeding therapists in January, to see if there is a physical reason for her poor eating and slow growth. Depending on the results of that, she may go through testing to see if there is a problem with how her body absorbs and/or breaks down proteins and nutrients. I, however, remain optimistic that there really is nothing else wrong, that she's just playing catch up from her very rough start.

Ashley also continues to battle GERD (gastro-esophageal reflux disease) and is on daily medication for this, as well as special pre-digested, hypoallergenic formula. We are hoping that she will eventually outgrow this.

While many times this journey has broken my heart, and certainly brought me to tears, it has also taught us so much. We have learned the value of family and friends. Without the emotional support and physical help of our family and friends, be it in the form of a meal dropped off, housecleaning or childcare, we could never have gotten through this.

I have learned more about faith, prayer and God in these months, than in my entire life. God is real. He is my source of strength, peace and comfort. I truly believe that he has a plan for all us of, in all things, good or bad, and I know that he has something special in store for Ashley.

And Ashley. She is the strongest person I know. She has been through more in her short life, than some will go through in a lifetime.

She has taught us the meaning of unconditional love. There were days when I was restraining her to insert her NG tube or perhaps her father was holding her while lab techs drew yet another blood sample. She cried and screamed through these painful procedures, and yet, when it was all over, she would look up at us and smile through her tears.

Even though we have caused her repeated pain, she loves us. Who else, besides God, is capable of that kind of love?

God knew we had lessons that we needed to learn in life: patience, faith, trust, perseverance, humility, strength, love, dependence and many more. So, he wrapped them up and sent them in the form of Ashley. She is my angel here on earth.

Tennessee Pulse Oximetry Bill

I would like to share that Karin Coulter with Saving Little Hearts has led a magnificent effort in the state of Tennessee to push for newborn screening with the pulse oximetry.

Both the TN House and Senate are moving forward on the bill to screen newborns in the state of TN. http://wapp.capitol.tn.gov/apps/BillInfo/Default.aspx?BillNumber=HB0373&ga=107.  If you are not in TN and want a law in your state please feel free to copy our bill, send it to your state legislators and ask them to file a bill as well.

TN residents that want to help with the pulse ox bill: Please contact your legislators and ask them to co-sign/support HB 0373/*SB 0065. Go to http://www.capitol.tn.gov/legislators/ to find your legislators. The more people that sign on the better the bill will do. Thanks to Ryan Haynes for introducing it in the House and Doug Overbey for introducing it in the Senate.

Baby Ashley Part 1

This post is a long one, I pray you will still read it as it is worth it. Every CHD is different and every child with a CHD is different. There is always hope. This story is shared in two parts. Ashley's mom gave me permission to edit but I couldn't.
---------------------------------------------------------

June 7, 2010. I was booked for a repeat csection, after failing to progress while delivering my son, Thomas, almost three years earlier. We left for the hospital at 5:30 am. We were excited and nervous as all expetant parents are, first time or not.

I remember laying on the operating table waiting to hear that first cry. Suddenly, I heard it! My baby girl was here! They whisked her away to be cleaned off, and have her newborn tests administered. She scored a perfect ten on her APGAR - an achievement I'm told is quite rare. 

Since our son was over 8 pounds at birth, I was shocked when they brought her over to me - she was so tiny! She only weighed 5 pounds, 15 ounces. She was a little string bean though, at almost 21 inches long!

We named her Ashley Louise. Ashley being a name that my husband Mark and I had always liked, and Louise being a family name on both sides. We thought it suited this dainty, beautiful little girl we had been blessed with. 

The next morning a nurse came in with a doctor, saying they wanted to listen to Ashley's heart. The nurse said she thought she had heard a clicking noise the day before, after her birth. They both listened, but heard nothing abnormal, so I shrugged it off, too tired and sore to get upset.

The day progressed in a typical day-after-birth haze. Ashley was having a harder time breastfeeding than my son had. She would latch well, but fall asleep within minutes of starting. I chalked it up to newborn fatigue.

The next morning, 48 hours after Ashley's birth, the same nurse came back to listen to Ashley's heart again. This time, I knew something was wrong by the look on her face. She listened a second time, then turned to talk to me.

"Your daughter has a loud murmur in her heart." She went on to explain that this was a common occurance, that many babies had murmurs, but it didn't mean that anything was wrong. 

"All the same," she continued, "I'm going to send the doctor up to see her." She left the room, and I lay there, stunned. I looked over at my beautiful daughter, sleeping in her bassinet, not ready to believe that anything could be wrong with her.

However, I did pick up the phone and call my husband, repeating what the nurse had just told me. I knew he was worried. He got our son ready for daycare, and then took the day off work to come be with me at the hospital, while we waited for the doctor. It was 8 am.

Thus started one of the longest days of my life. Visitors came and went, but we didn't mention our concerns. I know I barely even registered their visit. 

Finally, at 7 pm that evening, the doctor came to see Ashley. We had waited almost twelve hours to find out if there was something wrong with our daughter's heart.

Dr. Mutrie, the doctor on call that day, looked her all over, listening carefully to her heart, and told us that she definitely had a loud murmur. He mentioned that sometimes they are caused by "holes in the heart", but reiterated what the nurse had said, that often, it's nothing. He was going to send the pediatrician up the next morning to see what he thought. He was sure it wasn't anything serious, since she was doing so well.

And she was. Aside from not nursing very well, she was perfectly healthy. She had great color, was very alert, and all her vitals were stable.

The next morning, when Ashley was 3 days old, the pediatrician, Dr. Mark Messenger, arrived to see Ashley. He did a thorough exam, and then questioned us about our family's "heart history".

He sat us down, and told us that he was very sure Ashley had a hole in her heart. He said it likely wasn't large, and that they are very common, often closing on their own. He went on to say that he was going to send us to a larger, regional hospital on hour away, where Ashley would undergo an echocardiogram, to diagnose her problems. The echo would be set up via live Telelink, with the IWK Children's Hospital. He would call us later that morning with the time. With that, he left. It was 10:00 am.

We were stunned. We were worried, but still trying not to blow things out of proportion until we knew what was going on. We received the call at 11:30, that we were to be at the hospital by 2:00 pm. My husband flew home to get things settled for my son, pack up my room and get everything into the car.While he was rushing around taking care of things, Ashley underwent her first cardiac test - an EKG. They had a hard time getting all the leads on her, since she was so small. The next challenge was getting her to stay still long enough!

I'm glad I had the foresight to ask the doctor for some pain medication for the trip. I was only 3 days post-surgery, and I was crammed into the backseat of our small Ford Focus, unable to move or change position. I was in misery, but no way was I not going with my daughter. At the Dr. Everett Chalmers Regional Hospital, Ashley underwent her first echo. When they were done, we were able to talk to the cardiologist at the IWK Children's Hospital, as well as the pediatrician, via teleconference.

They confirmed the shocking news that Ashley did have a Congenital Heart Defect, known as a VSD - ventricular septal defect. She had a "hole" in her heart, in the wall that divides her two lower chambers. Honestly, we really had no idea what that meant, or the significance of it. 

We were given a few signs to watch for, told to call our doctor if there were any concerns, and sent home. We had several follow up appointments scheduled, including with cardiology, later that fall. The drive home was silent, with each of us lost in our own thoughts and worries. We called our family when we got home, but everyone was optimistic, saying things like, "Oh holes are so common, they're not a big deal!" It seemed everyone we talked to knew somebody indirectly that had a hole in their heart, and was just fine, they never had any problems with it. 

It was falsely reassuring, and while I think they meant to be helpful, after later on, when things got worse with Ashley's health, I got tired of hearing it, because it felt like they were trivializing what we were going through.

We settled into our life at home. Ashley was still struggling with breastfeeding. She would latch on, start to suck, but then fall asleep quickly. She would only eat for 5 minutes, on one side only, and there was no waking her up to eat, once she fell asleep. 

Aside from the difficulty breastfeeding, and jaundice setting in, the first week passed easily, and she appeared perfectly healthy. Our worry started to ease. 

However, starting into the second week, we started noticing some different symptoms: her breathing rate seemed quite high, and her heart often felt like it was pounding when you held her. Her breastfeeding wasn't improving, and actually seemed to be getting worse. She often seemed like she was struggling to breathe while nursing. Ashley would latch on, start to suck and then pop off suddenly, almost gasping for air. She'd repeat this over and over, sometimes screaming after her gasps. It was so frustrating and worrisome for us.

The night before Ashley's two week check up, I was holding her, and could feel her heart pounding. This wasn't unusual, but what was alarming was how fast it was going. I counted the beats, but didn't believe my numbers. I counted again and again and again. I kept getting the same number: 200 beats per minute.

I called the hospital and they told me to get her to ER immediately. However, by the time we arrived at the hospital, her arrhythmia had stopped, and her rate was normal. While there, they decided to do a thorough check up anyways. First stop was the scales, where I was shocked to learn that Ashley had gained a full pound in only 6 days! 

They hooked her up to the vitals monitors. Her heart rate had dropped into the upper limits of normal, though her breathing rate was a bit high, but no one seemed concerned. 

The doctor came in and I told him about her VSD and what had happened with her heart rate. He commented that her belly looked quite swollen, and I told him about the rapid weight gain. They watched her vitals for approximately thirty minutes, and decided to let us go, since nothing appeared amiss. He sent us home after telling us that her heart rate had gone high likely because she was trying to have a bowel movement, and her belly was likely swollen from gas. 

Ashley's check up was the next day. She was seventeen days old. We sat out in the waiting room and finally our name was called. I went into the room. There on the desk was a copy of Ashley's echo report. I read it while waiting. 

I was shocked to learn that not only did Ashley have a moderate to large VSD, but she also had a second heart defect: a small ASD (atrial septal defect). I kept reading the numbers and figures, which made no sense to me, until I saw the following sentence in bold, and underlined: 

Watch acutely for signs of congestive heart failure.

My stomach dropped, and suddenly I knew what was coming.

Only minutes later Ashley's family doctor came in. She scanned the report, listened to my account of what had happened the night before, some of the symptoms we'd been seeing at home, and then listened to her heart and breathing. She asked if we'd noticed any rapid weight gain. I told her about the 15 ounces in 6 days.

She left immediately saying to was going to make a call to Ashley's pediatrician, and would be right back. I barely breathed while she was gone, and was fighting back tears. It was a weird feeling - I knew what was coming, and yet was denying it at the same time. 

She came back in only moments later and said, "Pack your bags and go, they're waiting for you." She told us she was sure that Ashley was in congestive heart failure, and she was sending us back to the regional hospital, immediately. 

I remember leaving her office and falling into a chair in the waiting area, crying, while I tried to get Ashley back into her car seat. I was stunned. I called my husband and told him to leave work immediately and meet me at the doctor's office asap, so we could get our daughter to the hospital.

I remember looking at her, terrified. I didn't know anything about congestive heart failure. I knew that older people died from it. I kept looking at her, terrified she was going to die. Thankfully, I've since learned that that's not how congestive heart failure works.

We flew down the highway to the hospital, definitely breaking a few speed laws. My mind was working overtime. I kept thinking, "What did I do wrong?" I felt so guilty. I am the mother. I am the one that carried her, that formed her in my womb. I felt sure it was something I had done wrong.

Then I felt angry. "Why me?" I didn't drink, smoke or do drugs. I got regular prenatal care, took my vitamins plus an extra Folic Acid. Why not some crack-head who didn't care? And just as soon as those thoughts came, they vanished. Looking back, I know that is the first time I really felt God was with me. Instead, my thoughts took a new turn: "Why not me?". I had a loving, supportive husband. We had a wonderful network of family and friends. We had stable jobs and a solid roof over our head. While we sure didn't have a lot of money, we had enough to get by, pay our bills and have some left over for extras. I doubted any "crack-head" would be able to provide such a stable environment for a needing baby. 

It was a real definitive moment in my life. I made the concious decision to leave my daughter's life in God's hands. To trust that He has a plan for her, for all of this. Good or bad, no matter what the outcome, He was and always is, in control. 

We arrived at the hospital where the doctors confirmed that Ashley was in early stages of congestive heart failure. At that point they put her at a level of 3, on a scale of 1-10 for severity. They said they wanted to avoid her hitting a level of 7 or 8. She was started on a heart medication, a fluid pill called Lasix, and we were sent home. 

I remember all of that so clearly. It's everything after that day, the rest of the summer and fall, that passes into a blur for me, because so much has happened. It has been a whirlwind of appointments, tests, hospitals, phone calls, emotions and fears. 

Ashley continued to decline steadily until she was approximately 10 weeks old. Shortly after two weeks of age we had to start bottle feeding her, as breastfeeding was just too much work for her. We had to buy special bottles and nipples, ones that created the least amount of resistance, so she didn't have to work as hard to eat her food. Even with those measures she was only eating 12-15 ounces per day. Sometimes it would take us an hour just to get 2 ounces of formula into her.

By six weeks of age she was hardly gaining any weight, so the pediatrician started her on two more prescriptions, another fluid pill called Aldactone, and another medication to help strengthen her heart function called Digoxin. 

He also started her on what's called "concentrated calorie" formula. Normal baby formula & breast milk is mixed so that for every ounce the baby drinks, they receive 20 calories. Ashley 's formula was now being concentrated to 27 calories per ounce, in hopes that she would start gaining some weight.

However, by nine weeks of age, she was officially diagnosed with "Failure to Thrive" - her weight had dropped completely off the growth charts. She was admitted to the Dr. Everett Chalmer's Hospital, which is an hour and a half away from our home. There she had an NG feeding tube inserted. We were taught how to administer her bolus feeds.

It was really difficult at first, as Ashley c0uld only tolerate tiny amounts of food at a time. On average that first week we had to feed her only 1 & 1/2 ounces, every 90 minutes, around the clock. It was draining. Thankfully, by the end of the first week, we had built her up to a full 2 ounces, every 90 minutes during the day, and every 3 hours at night. Mark and I would take turns getting up at 1:00 am & 4:00 am to feed her.

However, after only three days at home, she was re-hospitalized. A combination of the NG tube and her heart failure (which slows digestion) had caused her to develop reflux. Over twenty four hours she threw up every feed she took in, and ended up very dehydrated. 

Back in the hospital, after she was treated for her dehydration, she was started on reflux medication, and they also changed her formula to one that was easier on her tummy.

Up until she was diagnosed with Failure to Thrive, open heart surgery had been talked about, but nothing definite said. They were more saying, "some day, maybe around a year of age". However, once she was diagnosed with FTT and the NG tube went in, surgery became a definite. 

How do I describe what it's like as a parent to be told that your tiny baby, is going to require open heart surgery. It's the riskiest, most invasive surgery there is. Knowing that they would be literally stopping my daughter's heart to put her on bypass was terrifying. I tried my hardest to avoid even thinking about it, but every now and then I couldn't stop myself. I could almost picture her lying there, covered in tubes and wires with an incision down her chest. I could hear the alarm bells going off. I was terrified that something would go wrong. Those times, during those "day-mares", I cried and prayed and prayed for strength - and I always received it.

Ashley had a busy schedule. She had to have weekly blood work to check her electrolye levels. Her medications had the potential to throw them out of whack, which could cause her to have a heart attack or potentially fatal arrythmias. Blood draws were agnozing for her and me. Her veins were so tiny, it was common for them to be blown out during the draws, meaning they would have to start again and try another arm. Sometimes they couldn't get her veins at all, and would have to do heel pricks, which in a way was even worse because it took so long.

Then we had to worry about the sample hemolyzing (blood cells bursting, ruining the sample). Many times we were called back to the hospital because the sample had gone bad, and we'd have to put her through it again.

She had doctor's appointments every other week, and weight checks every week. Ashley has a large medical team following her which consists of: her family doctor, a pediatrician, her cardiologist and his nurse, a neo-natologist, two different dietitians, and Early Intervention. 

There's just nothing I can say to make you realize how overwhelming, physically exhausting and emotionally draining this time was. Unless you've had a seriously ill child, you just can't imagine. 

I admit, at times I would see a mother with her healthy baby, and feel jealous. Sometimes sad and frustrated. I guess you could say I felt sorry for myself. But not even so much for myself, as for Ashley, at the injustice of her having to go through all of this. Thankfully I never allowed myself to indulge in those feelings very long. I would just pray instead.

Around ten weeks of age, Ashley's heart failure started to stabilize. Her symptoms were being better controlled by her medications, and I think we began to breathe a little easier, as we counted down to her surgery, which we knew was going to be sometime mid-October.

However, around this time, she also developed a problem with high blood pressure. To this day, they're unsure what caused it. We had to take her on a rush trip down to the IWK Children's Hospital, which is six hours away from our home, where she underwent another echo, to make sure there wasn't another heart defect that they had missed. That came back okay.

They then put her through full kidney function blood work, as well as an ultrasound of her kidneys, to make sure there wasn't a different defect there. We were having to go to the hospital every day to have her blood pressure monitored, which at times was running very high. At one point, we were to see a doctor or the hospital every day (yes, even on weekends) for 14 days straight, sometimes twice a day for two or three different appointments. I was running on fumes and prayer at that point.

Thankfully, it seemed to resolve itself on it's own, and while we never did find out that cause, Ashley hasn't had problems with her blood pressure since.

Ashley started receiving Synagis vaccine shots which are a monthly immune-booster, to help her body ward off RSV. Almost all children have the virus by the age of 3. For most healthy children, it poses no problems, however, in children who are immune-compromised or have heart and/or lung disease, it can extremely serious. A simple cold for Ashley had the potential to turn into pneumonia.

I don't remember the day, but sometime in September, we received the call with the date for Ashley's surgery. I remember crying pretty well the rest of the day. We knew it was coming, but getting that call made it "real".

Aside from the NG tube in Ashley's nose, you would never suspect that she was seriously ill. She looked like any other healthy and happy baby, albeit tiny. It was easy to delude ourselves into thinking that it would be okay, that she would outgrow this, that it wasn't as serious as it is. That call changed that. There was no more pretending. It was real.

Those next few weeks my emotions were on a rollercoaster. I felt tense, angry, scared, exhausted, basically just a wreck, though so many people have commented on how strong I was, and how well I was holding it together. That was due solely to my faith in God, whom I cried out to daily.

Only three weeks before her operation, we were blessed to have a benefit breakfast thrown in Ashley's honor, to help with the growing financial costs. My husband's family put it on, and I will never

be able to thank them, or the community enough. The amount of people that donated food, money and their time for the breakfast was phenomenal. The amount of people that physically came to the breakfast and donated money was unbelievable. They had food prepared for 400 people, and they ran out, went out, bought more, and went through most of that.

Mark and I took turns going to the breakfast. When I walked inside our church hall and saw the place completely crowded with people there to support us, I started crying. I don't know that I had ever felt more overwhelmed, but in a good way.

Knowing that all those people, not just our friends and family, but complete strangers, were there for my daughter touched me in a way that I will never begin to be able to explain. It was humbling. It was to me, yet another proof of God's love, and that to anyone who doubts it, humanity at heart, is still good. I learned the true value and meaning of community and neighbour that day.

At Ashley's pre-op check up, only a bit less than three weeks before surgery, she was back in significant heart failure. Her weight gain had dropped again, she was starting to have difficulty breathing again, and her heart rate was fast and erratic. They doubled her medications in hopes of stabilizing her before surgery.

The week of surgery arrived, and we packed our bags for the six hour journey that would take us from our home for the next week to ten days. Thankfully we had several family members who were making the trip with us, not only to help us with Thomas, who was coming with us, but just to be an emotional support for us.

Ashley's surgery was set for October 21, 2010. Her pr-op day was set for October 20th, a day I will never forget. I remember waking up, with a mini anxiety attack. I couldn't believe that we were here, that we were less than 24 hours from her surgery. I was feeling panicked, and was doubting my ability to get through it. I was sick three times before we left for the hospital. I remember thinking, "If I'm this bad today, how am I going to handle tomorrow?"

We arrived at the hospital at 7:30 for a day scheduled full of pre-op testing for Ashley, and numerous meetings for us. She underwent three rounds of blood work, a chest xray and then a sedated study which consisted of putting Ashley under sedation, and then a detailed echo and EKG.

While she was in recovery, we were taken to another room where we had a meeting with various members of her surgical team. They went over step by step, the actual procedure, and all the risks - they left nothing out. We learned that she had between a 2-5% chance of dying, a bit higher than normal since she was less than 6 months old, and that she also had a higher than normal risk of requiring a pacemaker after surgery, due to where her VSD was located.

Then we were taken back to Ashley's room, where she was starting to wake up, and we had a meeting with her clinic nurse, who went over things like what to expect when we first saw her, explained about the ventilator she would be on, post-op care, what to expect when we got home etc...

The amount of information we received in those hours is mind-boggling. It was completely overwhelming, and at times it felt like we must be watching someone else's life. Surely, this wasn't our life? It wasn't our daughter they were talking about, was it?

And then we got the shock of our lives.

VW Superbowl Commercial, Darth Vader kid

We absolutely love it when we are inspired by those survivors of congenital heart defects. Below is the Today Show interview where they interviewed Max Page with his mom and also talked about how he was born with Tetralogy of Fallot(TOF) and had surgery at three months.

VW's little Darth Vader Kid Unmasked!!

http://www.youtube.com/watch?v=Y9lC-DQIWPM&sns=fb

CBS's the Talk also interviewed him and his mom. As a CHD survivor it is great to see the media willing to let them talk about the congenital heart defect. So glad there was a little bit of awareness made with the interviews.

Cora's Law - Pulse Oximetry

I feel this is a perfect story to feature today. The life of little Cora is one that has made an impact.

When Cora was born, she was beautiful and looked perfect. Everything about her was beautiful and her parents were madly in love with her. When she was five days old their lives were shattered though because an undetected congenital heart defect took her life.

The road has been difficult for her parents, but Kristine and Ben have decided to bring awareness and to do what they can so that no other family has to find our about congenital heart defects from the coroner. I highly encourage you to visit Cora's Story and read her entire story.

Right now, I want everybody that reads this to forward to everybody they know in the state of Indiana. Because of Kristine's advocacy about screening for congenital heart defects and contacting state senators there is a bill currently in committee that will mandate pulse oximetry screening on every newborn in Indiana. (Indiana Senate Bill 552, authored by Senator Brent Waltz)

Kristine has a post about writing the senators that are reviewing this bill here... Writing Senators in Support of Bill 552  And you can read the bill here ... Indiana Senate Bill 552

For those of you that are not in Indiana, that does not stop you from writing your own state senators and letting them know how important the pulse oximetry screening is. While it does not detect EVERY heart defect, it detects more than doing nothing.

Take advocacy into your own hands and bring awareness to congenital heart defects.

International Children's Heart Foundation

guest post from Charles F. Miller, International Children's Heart Foundation, www.BabyHeart.org

 

1,000,000. That’s a BIG number. 

1,000,000 …  what?  Dollars?  Miles?  Minutes?  Pounds?  Think about it. 1,000,000. BIG.

We are talking about Children. 1,000,000 children are born each year with a congenital heart defect (March of Dimes Report*).  This makes it the Number One birth defect. Did you know? That’s 2,740 children born around the world each day with a heart defect.

The University of Michigan Football Stadium is the largest in the USA and holds 107,000 people. Fill that huge stadium up over 9 times….each year…with newborns…all suffering from a congenital heart defect.  As a point of reference, 207,000 people were diagnosed with breast cancer in 2010 (National Cancer Institute*).

There is an organization really making a dent in the problem around the globe. The International Children’s Heart Foundation (ICHF) has a mission of: bringing the skills, technology and knowledge to cure and care for children with congenital heart disease to developing countries. ICHF does this regardless of country of origin, race, religion or gender. Additionally, ICHF actively trains the local medical personnel so they can build their own self-sustaining pediatric cardiac center and ICHF can go away and help other countries. In 2011 ICHF is operating in the following countries: Honduras, Nicaragua, Dominican Republic, Brazil, Ecuador, Egypt, Ethiopia, Iraq, Ukraine, Belarus, Russia, and India.

ICHF will operate on about 900 children this year across the globe. Is that significant or too small a number? It is significant for those kids and their families. Also, by building self-sustaining programs in these under-developed countries more and more children will be saved year after year. That’s what it takes to make a difference --- teaching others to do it themselves.

The children in these poor countries do not have a chance because there aren’t typically any physicians who have been trained in pediatric cardiac surgery. The children may also live very far away from any medical facilities. Often the parents are told to take their child home and love them as long as they live.

In the United States we are blessed to have so many facilities available that can help our children who have congenital heart defects. We feel that it is our obligation to help those less fortunate.

*http://www.marchofdimes.com/downloads/Birth_Defects_Report-PF.pdf

* http://seer.cancer.gov/statfacts/html/breast.html

______________________________________________________________________________

Taking CHD submissions until the 15^th of February so that we can include as many “faces” as possible. (To submit a post email faith (at) heaveninourhearts (dot) com.)

Benji's Heart Story... #CHD

When Benji was born there was no idea in our head that there would be anything wrong. His apgar scores were good and he was a good size, although he was easily the smallest of my four. Mommy intuition kicked in though, I knew something was wrong. My other three babies had been tanned and Benji had a bluish appearance to me. (The nurses said he was normal color, except for his feet) He didn't want to nurse though and that kept my concern for him high.

It was the next morning that we knew something was definitely wrong. Benji had not nursed at all and didn't take a bottle either. When the resident pediatrician checked him out he heard an "odd" murmur. In light of the not nursing and the murmur, a pediatric cardiologist was called in to check on him. That's when our world was turned upside down.

After hours of waiting and trying to take in what could possibly be wrong, we got the news. Benji had CoArctation of the Aorta and a Bicuspid Aortic Valve with a thickening of the wall (stenosis). The doctor immediately put him on medication to keep the ductus arteriosus open, or patent. Before we could even really take in what was going on, Benji was on his way to the Children's Hospital and into the NICU.

There were many terrifying hours over the next seven days. When Benji was a week old he had what they call closed heart surgery. They were able to repair the coarctation, or the narrowing, of the aorta by using the subclavian artery. At that point, it was decided to leave the valve alone. His recovery from the surgery was excellent, he went home five days after.

We were told that Benji would most likely have to have another procedure by the time he was two.  This has not been the case. While Benji does still have the bicuspid valve and we do have to keep close tabs on him, he has thrived and exceeded expectations. Benji will be six on March 2, and is on no medication for his heart.

In relating Benji's story, we know that we are one of the lucky ones. We have shared the sadness of others that were not as fortunate. This is why we feel that we have no choice but to bring attention to Congenital Heart Defects.

We praise God for Benji's health and we pray that with more attention, there will be more survivors.

Congenital Heart Defect Awareness Month

February is National Heart Month. It is when the American Heart Association chooses to make an active campaign about heart related illnesses. The Congenital Heart Defect Community has also claimed February for CHD Awareness in order to bring attention to the many children born with heart defects. 

Congenital Heart Defect Awareness is a passionate advocacy for us since Benji was born with CoArctation of the Aorta and Bicuspid Aortic Valve with Stenosis. We are blessed that Benji is with us and thriving, there are many babies throughout the world born with heart defects that do not survive.

Our hope for the month is that we can bring heart stories to the forefront to give congenital heart defects a face.  We also hope to point out some resources for support and advocacy within the CHD community.

After much thought, we have also decided to take CHD submissions until the 15^th of February so that we can include as many “faces” as possible. (To submit a post email faith (at) heaveninourhearts (dot) com.)