Sunday, February 12, 2012

Connected By Heart ~ Day 6

Connected By Heart

Congenital Heart Defects effect 1 in 100...

To me that means 1 in 100 is a superhero miracle (I guess
since I'm a CHDer, I'm included in that)

Superheroes come in all shapes and sizes...

It sure is not easy being a CHDer... but somehow our
superhero powers always shine through.

Christopher's Story:

I am not sure where to start, but I guess letting you know how I learned about CHD. It was when my youngest son was born and I was told , quote, “your son has a lousy heart.” Those words were followed by, “there is nothing we can do, take him home and enjoy him.” Okay, I am thinking, “it’s 2008, what do you MEAN, you can’t fix his heart!?” People in general perceive CHD’s to be something like a minor hole in the heart, something that can be grown into, or “fixed”. The reality is that these children, SOME of them have the option of surgery, but not without lots of risk. Christopher is one of the ones that surgery isn’t an option for. His condition of dialated left ventricular non compacted cardiomyopathy means that that when a fetus is in utero, their heart is sponge-like ?(non-compacted)?. When they are ready to be born the heart will compact and become more solidified like a muscle. CJ’s heart did not do that - resulting in the cardiomyopathy (weakness of the heart). It affects his right and left ventricles where it is very hard for his heart to pump and function correctly. Nothing is missing, out of place, or backwards. It’s the whole heart, all big, stretched out, and stiff.

We took him home at 45 days of age and he was admitted into hospice the next day. The doctors told us that his prognosis was six months or less. We had to learn to accept that each day may be his last. It is a very hard thing looking into your beautiful child’s huge eyes and knowing he is just on loan for a short while.

God is the only reason I made it through that period of time, and I never let go of my faith that God would make my baby whole and it was a win win situation for him no matter what. The footprints prayer, well that was me, God literally carried me through. I remember people saying "they don’t know how I could sleep at night". Well, in the beginning it wasn’t easy and the pulse ox was a huge comfort as I could listen to it beep and know his heart was still beating. But somewhere along the way, I grew more confident, and eventually the pulse ox got dusty and I felt peace in knowing that Christopher was going to be okay.

Some people called it denial, some people called it an amazing a
ct of faith - I tend to agree with the latter.

I trusted God and with that trust came the biggest peace that is very hard to describe. We celebrated Christopher’s birthday weekly and monthly, cake and all. Eventually, his expiration date came and went, he still remained a hospice patient, and we were still taking it one day at a time. His first birthday arrived and I cannot express to you the joy I felt that whole MONTH. The day we were promised NEVER to see was here!!! We
had a huge celebration, and continued to give all honor and praise to the Lord. Christopher continued to get older and when he was 15 months old his hospice nurse decided it was time to take a look at his heart (we weren’t allowed any more ECHO's) with Christopher being a hospice patient and they “didn’t want to keep getting our hopes up”. The doctor told us that his heart had slightly improved but that he was still hospice status. Two days later we received a call from him that our visit left him feeling like it wasn’t finished and he took annother look at his ECHO. He also had all of his colleagues review it and Christopher’s heart had improved so much that in his good conscience, he could not recertify him for hospice! Okay after trying to put into words how I felt on his birthday, this is even more difficult to describe. Our language does not have a strong enough word. I can tell you that God does reward trust and faith and Christopher is a living breathing example.

In February 2010, it was discovered that Barth Syndrome is the cause of Christopher's cardiomyopathy. Barth syndrome is a rare, sex-linked genetic disorder of lipid metabolism that affects males. Typically, boys with Barth syndrome present with hypotonia (low muscle tone) and dilated cardiomyopathy (labored breathing, poor appetite, and/or slow weight gain) at or within the first few months after birth. Other important features of Barth syndrome include bacterial infections because of neutropenia (a reduction in the number of white blood cells called neutrophils), muscle weakness, fatigue, and short stature. Although most children with Barth syndrome manifest all of these characteristics, some have only one or two of these abnormalities and, as a result, often are given incorrect diagnoses. It is very rare, less than 200 known cases worldwide. So much that if people would hold hands from one end of the world, all of the way around, only one of those people would be a boy with Barth syndrome.There is no specific treatment for Barth syndrome, but each of the individual problems can be successfully controlled.

He is now 3 years and 8 months old, his 4th birthday is rapidly approaching and I am basking in every minute with him. I still know better than to take for granted my time with him as we don’t know when god will call us home. It is a true miracle that my Christopher is alive today.

God is still in the miracle business. Also, doctors have to tell you what they have learned in the text books, but that doesn’t mean God or the child in question is reading that same book. I don’t know God’s plan, but I know that the least I can do is to continue to honor and praise Him, while also helping to spread CHD awareness.

You can read more about Barth syndrome at:

And you can follow updates on Christopher here:

Natalie's Story:

Carrying Natalie, before being born, was awesome. Even the nausea, compared to her big brother, was mild. She went easy on me while I was pregnant with her. Everything was uneventful and normal. During the course of my pregnancy, I decided to switch my ob/gyn around 20 weeks along which probably ended up being the beginning of a big mistake. Seriously soon-to-be mamas: don't switch if you don't have to. Lesson #1. The only reason I switched was to deliver at a hospital that was supposed to be "top notch" (it ended up being just as good as the hospital where my son was born). As opposed to my old OB, I had no idea but my new ob/gyn was totally against using ultrasounds hardly at all since my firstborn was born healthy and full term. I had to pretty much beg for an ultrasound since I hadn't had one yet, so at 26 weeks I finally found out that Natalie was a "she". During the scan, the tech noticed that Natalie was very wiggly and the shots of her heart were not clear at all. She told me to tell my doctor, so she can send me for another scan another day. Totally cool. When I followed up at my checkup with the ob/gyn I asked her "so I hear the scans weren't clear enough at the ultrasound" and she states that everything was perfectly fine.

Now I know to never even take a doctor's word as the final word when I feel uncomfortable. But at the time, I just let it slide, even though I felt frustrated, but I just knew that everything was probably..well..fine with Natalie.

The pregnancy went to full term and I had to evict Natalie by scheduled induction on April 21st, 2007- the same day as her daddy's birthday.

The labor was fast and furious and she was born 3 1/2 -4 hours after labor was induced. She was also born with a clean bill of health and nice and pink. Apgar scores both 9, we were so ecstatic. Every thought about our unclear sonogram photos went totally out the window. The nurses and doctors all heard Natalie's murmur pretty soon after the birth, but we were told not to really worry because it's so common to be born with a murmur that closes up quickly. Right before our 24 hour discharge from the hospital, the nurse who checked Natalie out said the murmur was gone and we were given the discharge papers. Home here we come!

We got home and right that evening, my mom in law noticed how purple Natalie's feet were. I was so deliriously tired that I shrugged it off as nothing serious, that "well, she has really fair skin like her daddy". We never noticed much of the "purple spells" again so our overly exhausted brains didn't think much of it. We followed up with our regular pediatrician for the usual few day old checkup and sure enough, our doctor heard the heart murmur loud and clear. Our doctor couldn't believe no one caught it before we left for home a few days ago. The murmur never went away. Getting a bit concerned, our doctor gives us a referral to go and see a heart specialist at Johns Hopkins Children's Center. Within about 2 weeks later, we were in the pediatric cardiologist's office. Natalie's oxygen saturation was 90%, but her weight and color were normal. At the time, I didn't know much of the pulse ox numbers, but now I know why they rushed to get her into the room to have her heart scanned. They knew something wasn't right- 90% is not normal.

While scanning her heart during the echocardiogram, the doctor walks in about 20 minutes into the test and heavily stares at the screen for a moment, then sits down next to the tech and says "hmm..Natalie you're even trickier than I thought". He went from kind of concerned to really concerned. I'm sure my heart rate went up, but I did what I could to keep Natalie comfortable. She laid there like a champ. The echo had to be at least an hour.

A little bit after getting settled into the exam room, the cardiologist walks in, really stern face, sits down with me and tells me how sick Natalie is. He explains all of these medical terms..but my ears aren't letting much of it in... neither were my eyes because the tears kept me from seeing his diagrams.. finally the tears fell so I could see the diagram he took some time to draw: something to show me exactly how Natalie's heart looked. He explained how she wasn't born with a right ventricle, that it wasn't functioning at all. That she has a VSD and an ASD but those 2 defects typically came with the defect of Tricuspid Atresia and that her ASD (Atrial Septal Defect) was actually helping to keep her alive. It was so much to take in. I was so scared to hear more, but I knew I had to hear him talk, but I wanted him to stop talking. He let me cry and even left the room for a little bit to let me cry it out. Poor James and my mom in law were sitting in the room, too. I was holding Natalie and giving her a bottle and just was in such denial that she was sick. I kept repeating that she didn't look sick- even her fingers and feet looked nice and pink.

From the time that Natalie was diagnosed to the time that she had her 1st of a few surgeries, the doctors at Hopkins and our pediatrician all kept a really (really) close eye on Natalie. We went in for daily weigh-ins a few days a week. We spent almost everyday at a doctor's office. My husband and I studied the anatomy of the heart and I tried my best to figure out exactly how a Tricuspid Atresia defect functioned. As a new "heart mom" I wanted to be the expert at everything- all the way from diagrams down to exactly what to expect when she's an adult. I was terrified of having more horrible surprises. I guess that was part of my grieving process- grieving the loss of a perfectly healthy baby girl.

Even with Natalie's cyanotic spells (now we knew why she turned so purple the day we brought her home and there was even a term for it!), Natalie managed to still gain enough weight to keep everyone happy. She was a little skinny and slept a lot, but she was getting closer and closer to the bi-directional Glenn operation, and getting more past the need for a BT Shunt. The focus was keeping Natalie stable and if she could skip the typical first surgery, we were told her outcome was even better. Her oxygen numbers were always in the high 80s or low 90s and with each point she went down, her age went higher. A few months before her surgery we "beefed" Natalie up with concentrated formula and she went from slim to super chunky. They wanted a chunky baby and they got one! With heart babies, the extra weight is actually great for them, during the surgery and for recovery. She had her very first heart catheterization, to help prepare the Hopkins team, about a week before Natalie's scheduled heart surgery. Right after they finished, the cath doctor tells us "well..we can't wait longer than a week for this surgery, she needs it no later, her heart is showing signs of declining. Please make sure the date does not change." My husband's jaw dropped. I remember how gray Natalie's skin was those few weeks before surgery. It was a good 50% of the time where she looked really sick. Her pulse ox dropped into the lower 80s. It was time.

On Monday, October 1st, 2007 Natalie had her very first open heart surgery: the Glenn Shunt. This surgery helps to prepare her blood flow to skip past the malformed right ventricle. The goal is to eventually have enough blood flow to the lungs without having to use that side, but use the left ventricle 100%.

It was a textbook case to the Hopkins medical team. But it was a miracle to us. I remember the advanced students (they called them "fellows") who visited the PICU would visit each patient 2 times a day to check their progress and document everything. They would always visit Natalie last because they used her as the example of a great outcome. They would always smile at me and there was never any shortage of compassion. Here, my 5 month old daughter lay there with tubes and wires coming from every direction, but she was kicking ass and they reminded me of that. I kept holding onto that. And with each day, more and more wires and tubes came out. Freedom!

By Friday, October 5th, 2007 Natalie left for home! Her surgery went perfect and her body responded perfectly to the surgery. She was sore, but the incision (scaring the crap out of me, I can't lie) was the only most difficult part of the physical care. I was so scared to do something wrong. And hearing Natalie cry from the pain... no mom or dad wants to see their baby in real, uncomfortable pain like that. But within a week of being home, Natalie was showing tons of signs that she was handling it more easily and she was feeling more comfortable.

From the time we brought her home until 2009, we just enjoyed her. She was able to do physical therapy and finally started walking around 22 months. Her energy, everyone could tell, was pretty good! At one point during Natalie's recovery, her cardiologist said "Natalie's body was made for this" (meaning, her heart defect).

In April of 2009 Natalie turned 2, we moved to Colorado from Maryland, but Hopkins gave us the all clear that she should be able to handle higher altitude. We were told that she probably wouldn't need her 2nd open heart surgery- The Fontan completion, until around the age of 4 or 5. We had more time to just enjoy Natalie.

The move went well, we quickly found a new pediatrician to help us keep an eye on Natalie's health, but during the summertime we noticed that Natalie wanted to rest more and her purple spells were more furious and happening more frequently. She still played and was active, but you could tell there was a drop. She would want to sit and lay around more often than play. We were finally able to get squeezed in to visit our new cardiac doctor at The Children's Hospital of Denver in mid-September for a sedated echocardiogram. That's when they threw a huge curve ball at us- the decided that Natalie was ready for the completion of her heart surgery and they wanted to operate ASAP. We were stunned. We thought we had a few more years!

We had a heart catheterization scheduled for October 8th, 2009. This was a way to prepare the heart team at Children's: to really get a closer look at her heart and past surgery.

We had another curve ball- the heart team found 3 collateral veins that had to be closed off right away. These veins grew at some point over the year or so to overcompensate for the insufficient blood flow. But instead of helping Natalie they decreased her blood flow, which is why she was getting so tired all of the time. After a night stay in the hospital, Natalie recovered well enough to go home and rest. About a week later, Natalie's energy was THROUGH THE ROOF. She was like a totally different kid. She was jumping and running and being a crazy 2 year old. Her collateral veins were closed off with platinum so there's always a running joke now how expensive Natalie's insides are. The only thing missing now are diamonds, which if Natalie could have demanded diamonds she would have.

On the early morning hours of Tuesday, January 12th, 2010, we brought Natalie back to Children's for her Fontan operation. We were so very lucky to enjoy her through Halloween, Thanksgiving, Hanukkah, New Years, but now it was time to get back down to business.

Her body was ready for this operation even if us parents were not. Other than some extra "oozing" as the surgical team calls it, Natalie's transfer from off of the heart/lung bypass machine was a success. The operation took a whole lot longer than her first surgery, but this was it. This surgery was to "finish off" what her heart and lungs needed.

We had some little scares during the days in the CICU but nothing that hindered her ability to recover. At one point her kidneys were a bit "freaked out" with the amount of fluid and blood pumping in her body right after surgery, but her body worked it out. There was also a scare with her blood pressure dipping way low at times, particularly for the 2nd night, but just like her kidneys, her body worked that out, too.

By Monday night, January 18th we were all HOME. She did need continuous oxygen for a while, but we were prepared.

Natalie recovered beautifully. It was, once again, a text book case. We were all so thrilled. Within about a week of coming home, it was so hard to get Natalie to take it easy. Her energy level was pretty great and it became tricky to keep her from hitting her incision. But her incision healed beautifully. There were even moments we were given the green light to let her have a break from the continuous O2. As you can imagine, that was like heaven to Natalie.

We used the oxygen therapy until about April and we didn't look back (except for when she had a nasty case of RSV in 2011 when she needed it again for a like a week).

Since her surgery, Natalie has evolved into such a spunky, energetic, wild and crazy 4 (almost 5) year old. She asks about her scars once in a while and tells us that her tummy and chest scars are "cool". To date, Natalie has about 9 visible scars from her surgeries- her "zipper", chest tubes scars, wrist scars, and neck scars from various lines. But just like her scars, the memories that Natalie has about her surgery seem to slowly fade away. She has attended preschool, a dance class (and another one coming this summer), loves horses, has had a Make a Wish trip to Disney World, and visited a real beach for the first time recently. She loves the big waves of the ocean. She's the kid, out of the 2 we have, that we'll have to keep a close eye on, but not just because of a heart condition, but because Natalie is a true thrill seeker.

Watch out world! Here comes your present day Shirley Temple! : )

Zander's Story:

Zander was born 31st July 2010 14 days over due. Normal pregnancy 4 scans and no signs of any problems. Zander was born naturally, weighing 10lb 3 3/4oz. But when Zander was just 9 hours old we were told Zander had a loud heart murmur and we was asked to stay overnight and a midwife check on zander hourly on his temperature and saturations and breathing I had at that point no idea what saturation moniter was.

That was a long and very stress full night the unknown was scary. The next morning we was seen at 9am, Zander went for a check with a dr who confirmed Zanders murmur was still there and loud and wasn't going to go away on its own.

He said because Zander was feeding well and sats were good we were allowed home but if Z had any blue/grey spells to contact hospital asap he explain what a blue spell was to us and said we would receive an appointment for a detailed heart scan within the next two weeks to see if the murmur had gone.

We put things to back of our mind a little as Z was eating well and doing well.

We got an appointment for August 19th 2010 I took Zander and his heart healthy sister, Avril to rotherham general hospital where he was born for his scan I was dreading it. Zander was so good laid well. I remember well Z let off the loudest trump and the ladies commented on it.

The scan seemed to take forever then we had to go to out patients and wait to be seen by a consultant. This took forever. Then I was called into a consultants’ room, I could tell by his face it wasn’t good news.

He drew me a picture of a heart & went on to say several things about Zanders and his

problems. Then said he think he has something called TOF, tetralogy of fallots. But we had to be referred to leeds for a more in depth check with the specialists.

While he tried to contact Leeds, I rang Dan (at the time
my partner) & explained what I could to him from what
has sunk in from the consultant. Dan was trying to
arrange a lift to the hospital to be with us. The consultant
managed to contact Leeds and said we would be called
with an appointment. They rang the next day, at 12pm
and I had to get us a taxi to Leeds for 2pm as all the
family was working.

The scan seemed even longer than the previous day.
Zander's consultant Dr. John Gibbs an amazing Dr.,
confirmed Zander had ToF with a large VSD and
Pulmonary Branch Stenosis. What the heck was this I
thought?!!? He went on tosay that if Zander behaved and
stay well he would need open heart surgery before his 1st
birthday. What? Why would my tiny baby need open
heart surgery?! Please tell me why?! Some bits went over
my head, feeling like my world was crumbling.

We was aloud home the same day, he gave us a booklet on TOF that I have read over and over since that day.

For the first 5 months Zander did really well he thrived and apart from regular heart checks, appointments a sedated echo he was doing well. He was our page boy in Oct 2010 he looked fabulous. But he started to get blue tints to his hands lips and feet. By ec time he was completely off his bottles and food. and from Dec-April that was a downward spiral with no one listening to me. During this time Zander started having TET Spells. the first was in Feb 2011. it was awful one minute he was eating the next he was screaming then white and floppy, while I was on the phone to the ambulance he came round. His sats were low and we were admitted to RGH over night the next day Zander was put on beta blockers propananol (spelt wrong sorry), 3 times a day to try and keep spells at bay.

Feb 14th Zander had a heart cath at leeds done by dr john gibbs as they needed more details for Zanders surgeon. He had more spells another bad one in April 2011. Another admission to hospital his sats were low 70s late 60s.

At this time we were finally referred to a dietician.

We came home the same night. Zander from December he had become more blue and more and more breathless. In April we met Zanders surgeon Mr Kevin Watterson (God).

He explained Zanders heart was a very tricky condition and would be a long and very risky surgery, he said it would be sometime in the next six month his surgery. Oh my 6 months that seemed a lifetime away.

But at Zanders regular cardio app in May Dr brown Zanders cardiologist decided it was time to push Zanders surgery forward hi sats were dropping he was so tired. She said she talk to his surgeon.

Dr brown rang a week later saying Zander had pre op appointment for the following week. Oh wow so soon. We went May 31st nd Zander had lots of tests and we met his anithnatists, bloods done ecg etc.

We was told surgery would be within two weeks dr brown rang me that Thursday to tell me Zander should have had surgery that day but had to be cancelled. We was rung weds 8th June dinner time by Zanders surgeons secretary Zander was due for Surgery Friday 10th we had to go for admission by 2pm the next day. Was awful mixed feelings at home, what ifs etc. Also Avril not knowing what was happening she was only just 3.

Was hard leaving her with family. We got there for 2pm, was given a room, and we waited for Z's endless tests for checking he was well enough for the next day.

We went in playroom, tried to keep as normal as possible Mr. Watterson came with consent forms and went over surgery plans again as said it could be 8 hours even longer.

The rest of the night me & Daniel played hangman tried to keep upbeat. I was awake by 6.30 just watching Zander sleep. Was so hard knowing what was happening that day. It was dark in the room, I was so scared so damn scared. I just prayed my boy would be brought back to me. They brought a gown for Zander about 8am, we took the long (well not so long but very scary walk to the theatre and they gave Zander his mask to send him to sleep was awful to watch saying goodbye was hardest hated leaving that room, was harder watching him go to sleep this time than when he had his cath done :'(..)

We went back to ward 10 we had been offered parents accommodation in the old nurses building so we moved our stuff over there. We walked into leeds centre in a daze. We went to the pub longest pint ever had. We sat with our phones close by at all times. We decided to go to the cinema knowing that it would be several more hours until Z was out of surgery and we had our phones on our knee the whole time. We went to see Hangover 2 but I have to say I spent more time looking at my phone than the film, we went back to parents accom and waited and waited, when it was around 8 hours we went back to ward 10 and waited in the parents room, it then got to 9 hours than 10 hours than 11 hours we was stressing thinking the worst.

It was almost 12 hours I went to the nurses’ station they said Zander was just being taken to CICU by the time we got took to ICU it was gone 10pm and Mr Watterson wanted to see us first he said surgery had been more complicated than expected and Zander was a very tough case. He had also found Z had several more layers of narrowing in his branched arteries and it was very severe. He patched up what he could get too and closed the hole. But he still had narrowing between heart & lungs and hoped they grew in time. He went on to say it was up to Zander next and the next 12 hours were critical and he still might not make the night.

We finally got to see Zander almost 15 hours later he looked so puffy but So PINK! He looked amazing in color. But he struggled lots and those first 4 days were very up/down touch and go. He had bad temperature problems, he was on dialysis, his heart rate was very low, he went into complete heart block, he had shadows on his lungs, enlarged liver. By day 7 he had still not got his own heart rate/rhythm back. Mr Watterson had decided Zander was going to have a permanent pacermaker implanted. A Pacemaker?!! He was 10 months old!!!

Mr. Watterson explained his surgery would last around 2 hours and they was slight risk of infection with the pm being a foreign body :(.

I broke down near Zanders bed Adrien the nurse was amazing i had to know would his heart be stopped again and so much more.

I rang Daniel that morning and explained everything he was already coming that day so was relieved.

Daniel arrived early pm, we saw Zander for an hour. We had a call about 5pm Zander was going for surgery. We decided to go to pub and a wander, we was called at 7.30pm to let us know Zander was on the ward and surgery went well. They slowly started weaning him of the vent and other medicines. Though he started showing signs of infection and was blue tinted for first time since surgery :(. He was put on a course of antibiotics. Saturday 18th we saw him with his eyes open properly. It was beautiful, he moved beds and then by Monday he moved into a side room.

Tuesday he was moved up into HDU ward 10, he ate his 1st food in 11 days by mouth too, and Avril was with me that day and me Avril & Zander had our first cuddles since surgery was amazing!!! Love that amazing lil dude and his big brave sister!!

Zander moved into his own room the next day he was getting stronger and stronger. He was allowed home Sunday 26th June 16 days post surgery. He came home with 2 lots of diuretics and some other medications.

He went from strength to strength crawling shortly after his 1st Birthday, his 1st birthday was very emotional. And lots of people came. Thank you all.

August he had a cardio app and was taken off his diuretics.

Oct 2011 we had his 2nd pacemaker app with Dr. Blackburn. Dr. Blackburn found that since August Zanders inlet Valve had broken causing enlargement on the right side of his heart :'( meaning possible surgery sooner for valve replacement or repair. Oh Geeez what else did my poor lil mr has to go through?! He was put back on his Diuretics.

Dec we had his 3rd Pm appointment no change :'( scary to hear. Zander does get slightly breathless still and very very sweaty we are back in leeds Feb 14th I pray for improvement.

Also have had endless other appointments and checkups scans/etc. Too many to mention all.

I just want to say above all this Zander is a very happy maybe tiny but amazing 17 month old who amazes me daily. I Hate to think of the future but enjoy life daily.

Thank you for reading A Shortened version of Zanders life, so far his amazing journey.

Cassie Zanders Mummy! x x x

**Remember: 1 in 100 are born with a CHD


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