I was listening to the radio the other day and all I could think of was how many CHD angels there are.  I know quite a few but I know there are thousands more.  So so sad!  I have grown to love these children though I never met them or there parents.  Some parents I do know, have a very special place in my heart. CHD parents are strong, faith-driven, and loving. I wrote this poem for the CHD angels and their families…..Prayers for you and your family and I cannot wait until the day, I get to meet your angels!!                                                    

                                                 MEMORIES OF YOU…

As I am walking through a crowd, I catch a glimpse of your face,

My heart races, I feel my muscles tighten as I fight the tears away.

I close my eyes and memories flood my mind,

Oh how I wish I could go back in time.

I remember the day I saw your sweet face,

I can remember holding you, such a sweet embrace.

I think of the times we got to share together,

In my heart and my thoughts forever.

The nights I sat and cried by your side,

The nights I prayed for you to be alright,

The days we spent trying to cope with the truth,

The hours online learning about what’s wrong with you.

The tears and prayers and cards that sad day,

The day you got your wings and fluttered away.

I think of what you would be today,

Who you would look like, what you would say.

I lay in the bed and cry sometimes at night,

Wishing you were here laying by my side.

I know God is holding you telling you about me,

I want you to know, in my heart forever you will be.

So next time I see you in a somewhere, some place,

I know its you, my guardian angel keeping me safe.

In the last few weeks, I have entertained leaving the CHD community for good.  I never thought I would feel that way and truthfully, that’s why I’m writing this blog–because I hope that somehow this can make the difference we all need.

I read once that Christian’s are the only ones who shoot their wounded but I’ve discovered that statement simply is not true because I’ve watched the ready-aim-fire taking place in the most unimaginible place ever.  HERE.  In the CHD community.  I’ve watched battles for territory, proprietary rights & people who flat out refuse to unite with one another because of any myriad of differences.  I’ve watched as people refused to support each other because their kid is an angel while someone elses’ kid is a warrior.  I’ve watched adult survivors who fight with parents and parents who fight with each other and…I’ve shaken my head in shock at times while at other times been heartbroken.

I don’t understand this.  I really don’t.  I totally understand that we all have a different experience but guess what?  We’re STILL all fighting the same enemy.  We’ve all grieved, we’ve all lost sleep, we’ve all had fear, we’ve all had nightmares, we’ve all lost friends & family and we’ve all faced this bastard that keeps killing & hurting our kids.  Your pain is different than mine but that does not make either one of our pain any less valid.  My CHD child is alive, thank God but the truth is, most of us with CHD warriors live with the blood-chilling awareness that the end could come at any time.  Does this make our pain less than someone who lost their child after only a few days?  Who knows?  Who can tell?  Does it really matter?

What I do know is this.  We’re in this together, no matter what our particular stories.  One of my favorite quotes is ‘be kind for everyone you meet is fighting a hard battle” and that would be no better applied than here.  We may disagree with one another but here’s the challenge I am issuing from one heart parent to the rest:

Let’s be kind to one another.  We don’t always have to agree but we should always respect other’s because we just don’t now what this journey has cost them.  If we don’t agree, we will keep it private and between us and will not bring the entire community into it (i.e. mass messages & status updates slamming other parents).  If we can’t solve the problem, we will just agree to disagree without making a huge fuss about it.  We will understand that many of us are WOUNDED, TRAUMATIZED & may be acting out of our own pain.  We will not judge it, but we will offer as much help as we can and if we can’t offer help, we will at least not offer more pain.  The truth is, many people in this community are fragile & we don’t know what could be the “last straw” or the thing that pushes them over the edge.  God forbid that should come from any of us.

A kingdom divided can’t stand, friends.  Is it any wonder we see very little progress in CHD awareness when the community members are shooting their wounded?  It’s time to lay down our weapons that have been directed at each other and start aiming them where they should be…at Congenital Heart Defects & bringing awareness, impacting lives and helping other families.

With love,

Lisa Basquez

Founder & President

Gabriel’s Gift

www.gabrielsgift.net

Since Natalie was diagnosed 3 years ago with a serious heart problem, we did our best to filter out any negative and scary stories about other families. Once I read from a support “buddy” who had lost her child to CHD on my old Myspace page that said “Natalie may not make it..you have to accept that.” I don’t think that she was trying to be mean and clearly she was going through a really tough time..but that wasn’t what I needed to relate to at that point. I needed something other than talk about how my child will die. Death was something I acknowledged as a possibility and to this day I still have to, but I really needed stories of survival..or at least those going through exactly what I was going through…what Natalie was going through. Because let’s face it, it’s not just all about me. At all.
It’s horrible that you have to filter it..but to stay sane and strong for your child and for yourself, you have to surround yourself with something you can assimilate with.
We needed to somehow positively focus on our child..our child who was like a ticking time bomb every day for 5 whole months until she received her first open heart surgery- the Glenn Shunt.

Before Natalie had her first repair, we struggled to feed her. I remember sitting for hours getting her to drink an ounce of formula. In the middle of feeding she would just fall asleep and seemingly have no interest in eating. I remembering spending countless hours documenting her feed schedule, crying, and just feeling so horrible that my new baby was so tired, and so tired that she couldn’t eat. I didn’t blog about it. I didn’t talk much about it. I was way too scared to freak everyone else out. We mentioned some slightly unsettling aspects of what was going on and my mom-in-law called worried that Natalie was dying. Neither Rick or I could scare anyone.
I pretty much just focused on the positive with this blog and tried my best to not compare Natalie to James as a newborn. I was quickly learning how different they were and coping with that. Let’s face it, we were extremely lucky to have first experienced a “heart healthy” baby.

And then one day it all came together.
As soon as we were so scared and worried and desperate for answers, every doctor (and many of them have come and gone for sure) helped us get through it. They helped us prove that there were reasons behind our worries. We weren’t going crazy. As soon as everyone noticed a dip in her weight and received her heart diagnosis when Natalie was about 3 weeks old, we increased her caloric intake which was more powdered formula and less water. As with that, our cardiologist noticed that she arched her back when she fed. “She has acid reflux”, our Hopkins pediatric cardiologist stated and quickly sent us home from a heart checkup armed with a prescription of Zantac. So, there were 2 reasons for Natalie’s strange eating habits. And sure enough, not long after beginning her medicine, Natalie was still a sleepy eater, but she was a chunky sleepy eater. She packed those ounces on fast and everyone, especially her daddy and I, were so SO happy. I couldn’t keep up with her growth spurts, but it was a joy to worry about getting her new clothing at the last minute.
Every weekly weight and pulsox check up until her 4th month went well. She was growing and growing and her oxygen saturations stayed in the low to high 90s, which was always remarked as “amazing and interesting” by our Hopkins team. To be honest, I’m still blown away by that too. When we were close to finally getting fitted in for her Glenn Shunt operation, it came almost too late. Her blue and purple spells were so frequent and once again, she was struggling to eat. But out of all of that, Natalie skipped the BT Shunt altogether and it was a surprise to everyone. Because you never know when that bomb will explode and unleash some serious trouble. Natalie’s heart condition carried on without much of a hitch but at any time her ASD could have closed and Natalie wouldn’t be with us. The Atrial Septal Defect that usually comes standard with Tricuspid Atresia needed to stay open for her to survive. Granted, she was so closely monitored that something detrimental to happen was less of a chance, thank god. But there was still a huge chance that she would have suddenly passed away.
I don’t like to talk about that. I hardly ever talk about that. I hate it.

No one wants to even think about the possibility of a major organ inside of their child suddenly ceasing to work. We didn’t mention it much to our family or friends because to us, death wasn’t going to be an option. We were going to fight with every tooth and nail to make sure that Natalie would be okay. Sadly, many parents do that and still lose their child. That’s why this battle with heart defects is so complex and leaves you a bit powerless. The weight of your child’s health rests on your shoulders and you have no idea how it all will pan out.
Every time I look at Natalie I see a miracle. I see a kid who, yes has a life expectancy that’s uncertain, but so does every child– heart healthy or not. It’s easy for me to say that. I know. My child is alive and has handled everything she’s been put through with no complications. Why my child is one of those without any complications I don’t know. I just can’t feel guilty for that anymore. I just can’t.
Most parents who endure the long road of severe CHD with their child (or children) are supportive to us no matter who we are or who our child is. There are some, however, that don’t want to have much to do with your support if your child doesn’t have an almost identical heart problem and that’s what makes me the most sad.
It can be a slightly discriminating world out there. I’m sure the same goes for those looking for support for their child battling cancer.
“Oh, your child has skin cancer? Oh..well mine has brain cancer, therefore you don’t understand.”
And you can’t really get mad at people like that. You just can’t because most people understand that a kid with cancer of any kind is ..well… A KID WITH CANCER..dear god. Plus, how we all cope with such a severe disease can have anyone unintentionally offending others.
Most do not care one tiny bit if your child has HRHS (such as Natalie) as opposed to their child battling HLHS. Most do not hold it against you that their child was in the hospital recovering from open heart surgery for months compared to my child that spent almost a week. Most heart parents don’t mind chatting with you if your child doesn’t need a heart transplant even if their child needs one. As a matter of fact, many parents (with me included) with be right there along with you celebrating each milestone to recovery and staying healthy..well as healthy as any heart kid can be. An RSV or flu free winter for a CHD kid, alone, deserves a celebration.
A kid with any type of CHD is.. well.. A KID WITH A CHD. I completely “get” that some heart defects are technically more severe than others. I totally understand that some kids need more surgeries and medication than others. But to be discriminatory in your support system is beyond my comprehension at this point.

Parents who have lost their child are in, what I like to call, across that “acceptable” divided line. They are across that line because they are dealing with something so severe and life-altering that no one should say they understand unless they have lost a child, too. I can’t completely relate, but these parents don’t want me to because it’s so unimaginably difficult and this is a guess..but I think the last thing they’d want to hear or read about is another little child passing away in another family. There’s such a deep level of respect that I have for heart parents who have lost a child..that it’s hard to measure or even put it into words.
I want to hug every one of those parents so bad…
When I hear of another child passing due to CHD complications I just can’t bare it. I can’t stop thinking about that family and I do my best to pray for them, regardless of how “non religiousy” I am.
I can’t ever say that I completely understand what they’re going through. But what still brings us all together is that we see how scary the CHD world is and in reality, we are all in this together. If we don’t do that for ourselves, we have to do it for the kids.

I can’t ever apologize for how well Natalie has always done. I just can’t and I definitely won’t. She is a small child born with a complex heart that she didn’t ask for. Every open heart surgery..every heart catheterization and every test that she’s had deep anxiety over and still endured has brought her to where she is today. Alive, kicking, and as feisty as ever.

It’s amazing as a Mom to a CHD Child what we find out that we already know! We keep hearing Kimmy is Medically Fragile. Yes she is still in away but she is more stable now then when she was first born we should know we sat with her day and night while she was heavily sedated to keep her heart strong. She was so unstable then not now. She was  listed as failure to thrive and still wears that label too but she’s thriving in her own way!!  We are trying to let Kimmy experience everyday things any child would like to do even if it’s outside crawling through a splash station. She will get a scrape a bruise but if she’s not crying and is not affected by it then why interrupt her joy her happiness of chasing her sister?? Yes Kimmy is still small and will always be a CHD Warrior but she needs to be normal and live she needs to feel the sand in-between her toes to be able to splash in a swimming pool. We as her parents agreed to let Kimmy experience all she can and to treasure every moment. Tell me how you can say Kimmy isn’t normal!! She is normal she wants to do things she has feelings she fears she cries she’s gone through so much that she doesn’t understand now but eventually she will be on top of the World. She will take that extra chance just because you can’t live with what if or I should of. Kimmy’s so strong willed and determined to do everything now we can’t limit her she’s trying to be a child and we are going to let her. CHD is only a part of our life it’s only a part of Kimmy. Unfortunetly CHD physically effects her heart but her spiritand personality show no signs of it.  CHD is always going to be there we know that and only time will tell if  DR P will be able to reconstruct her heart naturally or with artificial parts. Kimmy’s very lucky that she has been able to come out with very little delays or set backs. CHD is so misleading to people who read the text books but don’t look at the Children and Adults thriving.  Don’t sentence my Kimmy to a plastic bubble because you think she shouldn’t be doing things. She will never say I can’t do that because I have a CHD she will say Yes Iwill try it. Probably shouldn’t say this but My CHD Child is going to beat up your “normal”" child. I swear I need that on a bumper sticker or T Shirt to wear when some of her social workers come over. We know Kimmy is sick but to us she’s not she’s not in the hospital on a vent No NG tube no changing colors no gasping for breaths she’s Healthier and a lot better.  We still have the   to get through the Fontan but we don’t dwell on that we dwell on us being a family with happy kids and taking lots of pictures and making lots of memories. Isn’t that what you are suppose to do??  My first pictures of Kimmy that we took were when she was in NICU with maybe four wires and she was awake. I was asked why would you take pictures of that? With all those wires??? That’s my baby with all those wires and what if that I never had a picture of her because I waited till all the wires were gone. We know how serious this was we seen Kimmy getting bagged and suctioned every 20 minutes some days we were there holding our breath waiting crying  praying for it to be over and Kimmy to be stable. Just because you might have a degree doesn’t mean that you fully understand a diagnosis especially when your degree is not a doctorate. You will never understand you will never be able to say anything but I didn’t know there was so much   to deal with. What do you think when we live in one town and we have to travel almost two hours depending on traffic to get to Chicago the only place that will treat Kimmy. It’s not easy but we have things planned just incase we go into Kimmy mode we shuffle this and that and manage. I thank God that all we have had to deal with is little colds and a fever and yes we probably should take her to her peditrican more but when she’s breathign funny we think we are saving time taking her to the ER because we don’t want her to be going to two different places then being transported that time is precious if it is life threatning. I know most people don’t think of that but when you learn CPR when you sit with your baby and things are so unsure you learn that it only takes a second for things to change. We kept her out of  Daycare because that’s what the Drs wanted so we deal with the seperation anxiety and people wonder why we can’t even leave her sight. I wish that this wasn’t the way things were but I will deal with Kimmy clinging to my leg at least she’s here. We are grateful to have her and we cringe when she gets labeled. Just so tired of  being reminded Kimmy has CHDs she’s medically Fragile she’s Failure to thrive we don’t see that any more! CHD will always be there lurking in the background but  most importanly she is our daughter Kimberly!

Dear Heart Families,

I understand you are frustrated. I know how neglected you feel by the lack of red in a sea of yellow and pink and blue wristbands. It is maddening that more of our children die before even one birthday candle can be lit than children of all pediatric cancers combined up to the age of 18. The effects on our families has been emotional, physical, financial, and profound. Our tears from exhaustion, grief, and fear could flood another great lake in the heart of the United States. To quote the nurse on the morning of James’s death, “This sucks.”

That was nine years ago and it still sucks.
66 years ago the first surgery was performed to save a blue baby.
30 years ago parents of children born with half a heart were given a choice in their child’s treatment beyond compassionate care.
50 years ago a pediatric cardiologist identified a means to diagnosis coarctation of aorta without any equipment.

Heart moms, dads, and survivors have been investing bleary-eyed late night hours and every extra borrowed buck to make pamphlets, to write letters, to compete in marathons, to travel to legislators. All of this and more in hopes of getting more attention to this oft-not mentioned birth defect in obstetrical offices and delivery rooms.

Why, dear heart families, aren’t the clinicians that we are praising as life-savers engaged?

A suspicion was raised for James’s eventual diagnosis at the 20 week ultrasound. James had a discrete coarctation. Everything else was there and “perfect”. How is it possible to miss half a heart in an ultrasound?

I have been hearing stories for nine years how parents don’t learn their child has a heart defect until after birth, and often when it is too late and accompanied by an ambulance and EMT delivery of “heart defect”.
Enough is enough.
Sure the obstetrician doesn’t mention it. Sure they need more pamphlets in the office. But why are so many diagnoses for the most common birth defect which is on thee MAJOR organ being missed?
It doesn’t make sense that this keeps happening and the families keep churning out pamphlets and fundraisers.

Why so tolerant of over 70 plus years of research?
Of medical schools curriculum?
Of infections and embolisms and ventilator associated pneumonia when CMS doesn’t even tolerate these preventable errors anymore?
Why patient with faulty medical equipment when The Joint Commission even has standards that must be met?

When are you going to say ENOUGH!

Our kids die within their first year of life; many of those in the neonatal time period. For those lucky ones who survive to hear a birthday melody, their whole beautiful physical self is constantly compensating for the defect. They cannot afford to be given unnecessary challenges within the health care theater. These kids don’t have time for more tolerance of substandard care.

I will be thrilled when laws are passed mandating newborn pulse ox screening. But it scares me at the same time. There are unintended consequences to every action.
I know what it is like to unknowingly have your rights as a parent taken away from you when you hand your baby over for “repair of a [heart defect]“.
Heart parents, please don’t hand over all common sense and parental instinct in trade for wishful thinking.

Life stopped at eleven days for my fully insured, prenatally diagnosed, 8lb 4 oz baby boy with a discrete coarctation “not because of anything he was born with” (according to doctors who were involved in or reviewed his charts). His life was taken because I was not given a choice or a voice in his care.

It is not that “this sucks”. If I could I would tell that nurse, “this is enough.”
Speak up. Help now. All hands on deck. Stop the preventable deaths of heart babies now. They know how.

“I don’t lose my anger, I use my anger.”
~ Ted Stevens

I posted this on his blog before his birthday, I still feel the same though & wanted to repost it here for others, so they can have HoPE for the future also.

♥     ♥     ♥     ♥      ♥     ♥      ♥     ♥     ♥     ♥

As London’s 10th birthday comes around I am very emotional.  When he was diagnosed at just 2 days old we were faced with the very real possibility of losing him, we are still faced with that possibility, it is something that we must think about every day.  I didn’t dare dream of the future until he was safely past his required surgeries.  We wondered if we would see him grow up and we vowed that he would be happy and we would have no regrets.  Now here we are, 10 years down, many many more to go!  London has come this far with flying colors and will surpass everyone’s expectations!  I now, finally, dare to dream…. Dream about his 16th, him driving, his 18th, his graduation, his 21st, college, marriage, and even holding my grandkids!  Things that most people take for granted, these are the things that we, as London’s parents, dream about & hope against all hope that London will be with us & be able to achieve these things.  And something that I know all HLHS & CHD parents will understand, but may sound morbid to everyone else:  MY SON WILL BE BURYING ME!  (Not the other way around)  No parent should have to bury their child, but as a person with HLHS, we do not know what his future holds, the oldest people living with HLHS are only in there late 20′s & early 30′s, but medicine has come a long ways, more & more kids with HLHS are living & my son is one of them!  I love you so much London Max II!  You are my sunshine!

PS. I also want to thank Dr Delius, his surgeon for his 1st 2 surgeries; his surgeon at UCSF; Dr Choy, his cardiologist; Celia, Dr Choy’s wonderful head nurse who has been there for us through everything!; Dr Yumul, his wonderful pediatrician for the first 8 years of his life, and everyone else, dr’s & nurses alike, who helped to keep my son alive and helped us get through it. Thank you to my family for being there for us and to our friends also. Thank you Shannon, for showing me that there were other people out there who felt the same way we did, and for taking the idea of starting our support group & running with it. I know that not only me, but over a thousand people thank you for getting the group started in 2002! ♥

Right after Norwood (1st open-heart surgery)August 2, 2000 ~ 5 days old

Just after Glenn (2nd open-heart surgery) February 1, 2001 ~ 7 months old

After Fontan (3rd open-heart surgery) August 1, 2002 ~ 2 years old

London earned his Yellow Belt in Kajukenbo July 31, 2002~10 yrs old

Abby was diagnosed with Tetralogy of Fallot at three-days-old. I never experienced the infamous 3 options presented to mothers who are diagnosed in-utero. I don’t know what that would feel like or what kind of emotions would run through your mind, both upon hearing the diagnosis and during the remaining months of pregnancy. I had a breathing little baby in my arms that I had fallen head over heels for. Her silky black curls, her beautiful blue eyes, the smell of her sweet breath, her soft skin…I knew that I would fight to keep her alive. I would go to whatever lengths I had to and beyond to keep her here.

This post has nothing to do with my stand on abortion or whether I think it is right or wrong. It has to do with a medical professional giving incomplete information to parents who were diagnosed in utero with Tetralogy of Fallot and trisomy 21. It has to do with that father spreading the word, to who knows what ends of the Earth, that Tetralogy of Fallot and trisomy 21 are conditions that are incompatible with life.

I came across a pro-choice blog in my searches. The blog had a post by a father. They were expecting twins. A healthy son and a son with birth defects. The “sick” son or the child this father refers to as “angel baby” was diagnosed with Tetralogy of Fallot. After receiving the diagnosis, the father stated that the doctor told them “the baby’s condition was inconsistent with life outside the womb.”

Here my heart skipped a beat. I caught my breath. My stomach flipped. I immediately thought of my beautiful ten-year-old daughter who lives everyday to the fullest. She loves Taylor Swift and Lady Gaga’s music. She watches ICarly. She loves to curl up on her bed with a book and she READS and READS like no child I’ve ever met. She is hardly inconsistent with LIFE!

These parents later were told that their son also had trisomy 21. A condition that is nearly identical to Down’s Syndrome and often present with Tetralogy of Fallot. They were told by a neurologist that he had definitely had a stroke in utero. They were also told he had a webbed neck, clubbed feet, tumors and cysts, a small brain, a deformed face, and a deformed nose high on his face.

Granted, this baby sounds like he had severe defects…as far as they could tell by the 26th week of gestation. They were told that if “Angel Baby were to survive birth, and the first 30 seconds of life, he might never attain any degree of consciousness or have any meaningful life or interaction with any one or anything.” The doctor went on to tell them that “to call Angel Baby “retarded” would be a great compliment.”

The parents decided to inject their angel baby with a shot of potassium to the heart, killing him. At 35 weeks both babies were delivered C-section. A healthy son and the deceased fetus, whose “profound malformations of his heart and nervous system” were “inconsistent with life outside the womb.”

I don’t profess that these parents should have attempted to have and raise their angel baby. I don’t profess that the choices they made were wrong. They made choices based on their medical advice and the choices they felt best served their son’s twin and the mother’s life. They did what doctor’s encouraged.

What I do profess is that the article is presented in a way that makes it seem like Tetralogy of Fallot is an untreatable heart ailment. And I profess that the article makes it seem like having trisomy 21 and Tetralogy of Fallot is something so terrible that death is preferable to these abhorrent conditions, when in fact I hope beyond hope that it was other serious defects that made the medical doctors encourage abortion. What I profess is that the article is close to negligent and leads others who find it to believe that children like my daughter and others who have trisomy 21 to deal with as well, should be aborted because their lives are not compatible to living. I profess that the doctor’s who handled this case were also negligent if they led the couple to believe that it was because of the heart condition that made the life of this baby not worth giving a chance.

I am so happy I found out about my daughter’s beautiful heart…after. The emotions were hard to deal with. The grief was overwhelming for days. The fear and anxiety compounded the normal emotional task of birthing and caring for a newborn. But, no doctor ever told me my baby’s life would not be worth keeping. No one ever encouraged me to abort the life of this baby…who also had other congenital issues that could have led them to believe she was severely handicapped as well. No one ever made me think that my daughter’s life wasn’t worth every last ounce of fight we had to keep her here. I hope that all those of you who find this know that Tetralogy of Fallot is most definitely compatible with life. I hope you know that trisomy 21 children are amazingly beautiful children. I hope you know that those of us with children with perfect broken hearts wouldn’t trade them for the world!!! They are worth every second or minute or day or week or month or year that we get to spend with them in our arms…loving them and feeling of their amazing and tremendous spirits.

They are most certainly compatible with LIFE!

I really love it when people ask me, “Is Abby done with heart surgeries?”

To be honest, the answer to that question is we hope so…at least OPEN heart surgery. Dr. Kaza at Primary Children’s was able to put a valve that technically should last Abby through her full grown body. Of course, replacement valves wear out but, he is confident that by the time Abby needs another valve replacement, it will be done through a vein in her leg in the cath lab! So, while we feel so relieved that our little warrior has fought this battle with CHD so valiantly, Abby has an additional congenital defect we have to deal with–Congenital Scoliosis.

I have learned that in children with Congenital Heart Defects, especially Tetralogy of Fallot, 23% will have congenital scoliosis as well. In the general population 1 in 10,000 children will be affected by congenital scoliosis. This makes information less common to come by, additionally, most people confuse CONGENITAL scoliosis with FUNCTIONAL scoliosis. They are different and will require radically different treatment.

FUNCTIONAL SCOLIOSIS is a much more common scoliosis. This is the type of scoliosis they test for in Jr. High and High School by having children bend down and touch their toes while their spine is examined. Functional scoliosis is caused by a factor outside the spine, meaning that a muscle on one side of the spine develops more, pulling the spine slightly to the right or left. Or, one leg may grow slower than the other leg, causing an imbalance in the hips which pushes the spine to one side or the other. In this scoliosis they attempt to treat the cause of the curve. Most often it is treated with a back brace, shoe inserts, or chiropractic care. It is easily treated and rarely causes problems–keep in mind that the spine essentially FORMED straight in the womb.

CONGENITAL SCOLIOSIS is a different type of scoliosis. It is much less common. The word congenital means “at birth”–this type of scoliosis was present at birth and was caused by a birth defect in the spine. There are two different kinds of birth defects in the spine: A Hemivertebrae (or butterfly vertebrae), and a unilateral bar. The hemivertebrae is where one side of the vertebrae forms and the other does not. The unilateral bar is when a number of the vertebrae are fused together on one side so the spine grows lopsided, so to speak. Congenital scoliosis requires surgery to correct since bracing can in no way fix the deformed bones. In a small percentage of children, congenital scoliosis may be outgrown or the curve may remain stable. In 70% of cases, the curve progresses as the child grows and will require surgery to ensure that the child can walk uprightly and has no problems with other organs in the chest cavity.

Abby has both Hemivertebrae and unilateral bar defects in her spine. The picture above looks very similar to Abby’s X-ray of her spine. She has a curve of 53% (which is considered severe) in her mid-back where she has unilateral bar and a smaller curve in the upper back area where she has Hemivertebrae. We are now looking at surgery to correct this defect around 12-years-old or so. One-and-a-half years time.

This surgery always felt far away…and for some time it sounded like it might be possible for her orthopedic surgeon to remove some growth plates on one side to correct the curve; however, at our last visit it became clear that her curve is progressing faster than he likes and is already a severe curve. Her surgery will require fusing of the vertebrae affected with bone grafts and metal rods and screws. The ICU stay is 2-4 days and the total hospital stay is 5-7 days. Children are then required to wear a brace for 6-9 months to protect the spine while it heals. Additionally, some patients require rehab therapy for 1-2 weeks after the surgery to help with walking, moving, and loosening the spine. This rehab is usually done with the child sleeping at the rehab center, although sometimes children are allowed to go home and attend therapy close to home.

Having said all that….so clinical and matter-of-factly….I want to cry when I think about this sweet girl going through ANOTHER surgery. It is true this surgery is not as “serious” as open heart surgery, although children with CHD do run a higher risk of contracting staph infection which can cause serious problems to the already repaired heart. Whether it is serious or not…it inflicts pain on her. It involves a hospital stay, which is scary to her. It requires ICU stays, arterial lines, catheters, intubation, and invasive procedures on her body. It scares her. It BREAKS MY HEART.

I would love to ask WHY? But that really doesn’t matter and I won’t get the answer answered. So, we take a deep breath. I have shed a few tears over the thought of more. I have realized that it could be worse and she could have had much more to go through like so many other heart children do. And, I remember that she is a huge ball of fire in a little body and she has a soul full of fight. She can do it and so can I. So here’s looking ahead to yet one more mountain Abby must climb…and that I will follow her to the top and over the other side.

I can’t believe that a year ago today, our daughter Sophie was in the hospital after her 3rd open heart surgery.  This time to replace her mitral valve with a mechanical one.  Sophie’s biggest concern that morning was giving up her sleeper pajamas to change into the hospital pajamas. I had no idea what things would be like after this surgery since an artifical valve meant going on anticoagulants.  I was doubly concerned since just three months prior, this surgery was attempted and had to be aborted due to abnormal bleeding which took everyone by surprise and the cause still unknown. We went thru countless blood draws and tests in the three months leading up to the surgery with nothing coming up abnormal.  But it’s like Sophie’s anesthesiologist said, Sophie likes to throw us curve balls.  Let me tell you more of her story.

I was born with Holt Oram Syndrome which is a hand/heart related defect that presents itself in various forms.  I was born with curved arms, no radius bone and 4 fingers on each hand (no thumbs). I also had a VSD that closed on it’s own yet was monitored by a cardiologist. As I grew up I never had a name for it, it was just how I was born.  I met my husband, Colin, and 3 years after we were married I became pregnant with my first child.  There was some concern that I might pass on something genetic, so we scheduled a level 2 ultrasound and a meeting with a perinatologist. The ultra sound came out fine and my son Connor Daniel was born with a 9/10 apgar score. When I got pregnant with my second child, we had no worries of passing on any of my birth defects.  Things progressed as normal until I got a call that my quad screen marker blood test came back with a high risk of down syndrome, we were then sent back to the perinatologist and geneticist.  I remember laying on the ultra sound table forever and knowing something was not right when they kept scanning over the heart and leaving the room and coming back.  They finally told me there was something wrong with the baby’s heart & referred me to a cardiologist.  They also wanted me to have amniocentesis done and discussed possible syndromes that are usually associated with heart defects.  I declined the amniocentesis and was committed to continue with the pregnancy no matter what.  After meeting with the cardiologist we were told Sophie had a very large VSD that had a good chance of needing surgery.

Sophie was born February 21st, 2006, 7lbs, 4oz via scheduled c-section.  They flashed her in front of me & then rushed her to the NICU for oxygen.  She had swallowed too much amniotic fluid.  I was able to meet up with her about 4 hrs later in a wheelchair and then she was brought to our room than night.  The next day we met with countless doctors and had xrays, echos, etc run.  She was noticed to have smaller shoulders and her thumb on her right hand was underdeveloped and webbed to the hand.   We later found out after genetic testing that she too, had Holt Oram.  The first few days with Sophie were rough.  She did not eat a lot at a feeding & started to breath faster, get paler, etc.  We got her to the doctor as soon a we could and we were told she may not make it long. They started her on lasix, but said it might not help much.  We sure were in for a shock when we had full diapers every hr.  We woke up every 4 hrs to feed her and saw the cardiologist on a monthly basis.  They finally said that they wanted to schedule her surgery for the beginning of August and would go in and patch her VSD.  However, when checking with my insurance, was informed that the surgery would only be covered at 50% due to being out of network, but a partner facility.  I was forced to switch doctors and hospitals, but am now so thankful I did.  The team at Legacy Emanuel was wonderful and got her set for surgery at the end of July.  They informed me that not only did she have a VSD, there was a small ASD as well and her heart was very enlarged. If she had been in their care from the start, she would have had surgery much sooner.

So on July 27th, 2006, Sophie had her 1st surgery.  We were told that if they were to successfully patch the VSD and stitch closed the ASD that she may be done with surgery.  That wasn’t how it was to be.  We get word from the surgeon that it was a very tricky surgery and they had trouble getting her heart started again.  They also said that after they closed her up, her mitral valve was leaking.  They were confident, however that she would be able to go home on some medications and they would attempt to repair it around age 2 or 3.  We filled prescriptions for lasix, digoxin, and captopril & after her recovery, were sent home.  We struggled with giving her medication 3 times a day that she kept chocking & gaging on & throwing up her food which we spent so long getting her to eat.  Everyday I had an uneasy feeling every morning I left for work.  I came home the night of Aug 8th to find her breathing very fast and retracting her chest. I said we are taking her to the ER.  We arrived and they got us right in and determined she was in severe heart failure.  They rushed us up in an ambulance to Portland, a good 2 hr drive away.  Her next open heart surgery was Aug 11th, just two weeks after the 1st.

This time they had to leave her chest open a few days due to swelling.  They said that they got a pretty good repair at first, but as the days went on, the valve started leaking more, but she was stable.  We adjusted her medication and eventually switched to pills which we crushed.  It was nice not having as much liquid for her to swallow. We were sent home and followed up with her cardiologist every 6 months.  Sophie started to grow more and sat up at 13 months and learned to walk at 21 months of age.  All of this was with help from EC Cares/Early Intervention program.

The next blow came to us when her pediatrician mentioned that her head size seemed abnormally large.  We were sent for an MRI and told there was fluid in her head.  The thought of her needing head surgery as well seemed more than we could take.  We took the 2 hr drive up to Portland to meet with the neurosurgeon.  Our minds were full of questions.  She said that it was extra axial fluid and due to recent research found that surgery was not necessary.

Life was falling into a comfortable routine, the time between doctor visits was getting longer.  Then in January 2009 we get a call out of the blue from her cardiologist.  He had consulted with the surgeon and due to the fact that Sophie’s heart was still enlarged they wanted to replace/repair her mitral valve within the next few months.  We set her surgery for March 28th since it would be during Spring Break since my husband was finishing school.  The week prior to surgery, she got very sick with a respiratory virus, tested negative for RSV, but was not 100% well by the 28th, so surgery got moved to April 10th.

This time we knew the routine.  We knew where they wheeled her back from surgery and everything.  We had difficulty getting her blood drawn during the pre-op, so we were OK when they said they would just take what they needed after they put her under anesthesia since all her labs for her prior surgeries turned out fine. We were at the Ronald McDonald House taking a short break when I get a call on my phone thinking it was a little earlier for an update.  We were told to proceed directly to the hospital ASAP.  Surgery had been aborted and the surgeon and doctor needed to speak with us.  A lump rose in my throat as we rushed across the street.  They said they were about to make the 1st incision, but noticed abnormal bleeding from her arterial line in her leg.  They ran tests and her blood would not clot. They swore up and down that they did not give an overdose of heparin.  We rushed into see her.  She still had to be extubated & there were about 5 nurses around her all applying pressure.  A huge bruise was forming in her groin area and down her leg.  We meet with the hematologist and after several hours and a substance called factor 7, we got the bleeding to stop.  My husband and I were nervous wrecks.  We were now no closer to having the surgery behind us and had to figure out what happened before we could proceed.  We worked with the hematologist for about 3 months and it was deemed safe to proceed with the surgery, yet with an unsolved mystery of what happened that day.  I told my daughter that she was not allowed to meet any more specialists at the hospital after visiting the cardiologist, geneticist, neurologist, and hematologist.

Now I think we are full circle from the start of this post about Sophie’s surgery a year ago today.  We know the journey is not over and we will face more surgeries along the way.  I however have meet some wonderful people along the way and I am thankful of their support.  Our last visit to her cardiologist did show that her heart is still enlarged and there was no improvement in her heart function.  She is thriving and doing great, yet follow up this Wednesday to see if any changes have occurred.  Our cardiologist said if things don’t improve or decline further, we may have to discuss transplant.  Not the words I wanted to hear, but in the meantime I enjoy, marvel and love every second I have with Sophie.

I feel like you think it is funny to have tortured me for the time that Khloe has been here, I don’t think that you sending me into depression and not being able to function is not ok. I didn’t realize how angry you have made me at myself for so long and how horrible I now feel because I have just been on pilot mode for so long. I feel like i am coming out of a coma. I am coming to find that you are a horrible thing to my mind and I feel defeated in life because of you. I wish that everything was just better and I could get over it but I can’t, I am dealing with it but I feel like I have let down my babies and my husband because of you and because I feel like there is never sunshine on the horizon, I don’t like being Mrs. Doom and Gloom and I can not let it affect me anymore everyone see’s it. I am done. I have two beautiful kids one that you have affected for her life and my beautiful heart healthy boy which will deal with you on a different level. I know he will always worry about his big sister being ok and it is all because of you. My wonderful husband will just shove you down and try not to think of you as he does with a lot of things but you make me feel like I am right there again, I will have to get over that one day at a time. You will always be there but I will not let you interfere with my performance as a mother, wife,lover,friend and person anymore. I can’t it isn’t ok and we have to live together for the rest of our lives. So please try to ease up because if you don’t I think I will need a vacation at the looney bin for a little while and I really don’t want to do that.