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!

The other day, I once again, felt that kick in the gut.

In the military, there is a program called the exceptional family member program, or the EFMP. The purpose of the program is to make sure that the base you are stationed at has sufficient medical facilities, or educational programs, for every member of your family.

So, for example, Zachy and Natalie are enrolled in the program. When we go to move, a red flag will go up in Matt’s file saying we have special needs. The base we go to will have to be able to accommodate their needs, or we can’t go to that base.

Since we are getting ready to move (PCS, permanently change stations), we have to update all their EFMP paperwork.

The paperwork requires letters from their doctor, stating what exactly, they need. Since we are PCSing, the doctor also gave us a copy of their records.

Since I always feel the need to read every report I’m ever given, I decided to read this one too.

I read through Zachy’s. His diagnoses were, TAPVR, Sick Sinus Syndrome, and pacemaker present. Yep, all pretty straight forward.

I then went to Natalie’s. Her diagnoses read TAPVR, <i> and </i>Pulmonary Vein Stenosis.
It also goes on to state that there is a chance, although slight, that she will need another surgery to correct the stenosis.

So here’s where I felt the kick in the gut….we have always said, in passing, that one of her veins might be slightly narrow, but I never thought it was something that would be a diagnosis. I never really thought she would need another surgery. And, she probably won’t. But the fact that it was written in her records, it just makes it that much more real.

I’m sure her nurse from the NICU, Jeremy, would be telling me she is doing everything that is expected. He was always good at telling me that. Still, even if it is to be expected, I just never expected to see it in print.

Stenosis is not something we ever dealt with with Zachy. And we know it is the main complication of TAPVR after repair. It’s just not supposed to happen to us. She is supposed to have one diagnosis..not two diagnoses.

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 have a new interview up at my blog, this time with Daria – a 17 year old living with Corrected L-Transposition with Pulmonary Stenosis.

I first met Dar when she was a fourteen year old camper at Camp del Corazon, a non-profit I volunteer with. Dar and her group of friends quickly became “my” kids, a group I look forward to seeing each year. Daria graduated camp last summer and I am eagerly anticipating her return in a few years – only this time as a counselor!

Click the image to read the interview

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.

As many of you know, I volunteer for Camp del Corazon – a non-profit organization that sends kids (7-17) living with cardiac issues to a five day camp. We have space left in sessions 2 and 3 and I’d like to give you a Top Ten List of why you should send your special heart child to our wonderful heart camp!

1. There is absolutely NO COST to your family! That’s right, your child is granted a lovely five day camp journey with boat ride (to AND back!), room & board, activities – all at no cost to you!

2. Your child is amongst their peers with dozens of fellow heart buddies to spark new friendships and forge bonds that can last a life time!

3. Camp del Corazon is an ACA (American Camp Association) accredited camp!

4. Camp del Corazon was founded by a pediatric cardiologist and nurse! Trust me; round the clock care is there for your child with nurses, doctors and a fully equipped infirmary. ALL activities have a nurse stationed at them!

5. PIRATES! How can you go wrong with an awesome theme like pirates? Costumes, props and decorations are thoroughly encouraged for campers AND counselors alike to get into the spirit of our theme!

6. Your child’s itinerary may include just a sampling of these awesome activities: Swimming, kayaking, archery, arts & crafts, boom ball, court games, climbing wall, high ropes course, nature PLUS a free choice activity of their choosing!

7. Your child will stay off the California coast at Howland’s Landing, a private cove on the leeward side of beautiful Catalina Island! Gorgeous ocean view, fresh air and balmy, beachy days.

8. All of our activities center on building a camper’s self-esteem & teamwork skills through positive reinforcement and encouragement.

9. Thoroughly enthusiastic counselors (such as myself!) are there for the kids ~ to mentor and lead by positive example, to provide safety as well as fun. Did you know we also have quite the number of former campers turned counselors?

10. This is a completely unique experience no heart kid should have to miss, as it was designed specifically with them in mind. <3

Camp del Corazon (website)
Session 2: August 30th – September 3rd
Session 3: September 3rd – 7th

Camper Registration Forms here.

Feel free to email me with any questions, or call the camp office (provided at the camp website)

I cross-posted this to my blog.

It is fairly common for children to have a security blanket of sorts. Now, this doesn’t need to be a literal blanket ala Linus, but can take on many forms such as a stuffed animal or other keepsake.
I started off with a blanket, but due to a Grandmother who thought “out of sight, out of mind” cold turkey was the best method to break a six year old out of a thumb sucker habit, I had to switch to stuffed animal. (Totally didn’t work, Grandma. Old school child psychology FAIL!)

Specifically, my yellow Puffalump bunny who my mom officially dubbed Boo Boo.

Boo Boo with her hospital bracelet

I tried to give her a proper name for when the nurses and doctors asked, but Boo Boo always stuck.

This noble, stuffed bunny was dragged everywhere I went – sleepovers, doctor appointments and surgeries being her most needed moments of comfort. I never went a single night of my childhood without her either closely hugged to my chest or as a makeshift pillow under my head, her ears as a substitute for my long, lost blanket.

She was there with me during cold echocardiograms, annoying EKG’s, painful IVs, obnoxious student doctor visits and those frightening moments between being wheeled away from my mother into the operating room and officially under the anesthesia.

When I woke up from every procedure and operation, Boo Boo was right there next to me. I wouldn’t know until I was twenty-two that she was never kept in the OR with me (note to parents: Please don’t tell your kids. It’s easier to believe than it is to know).

Over the years she has needed a few surgeries herself – stitch-ups and patches, and years of love has given her a permanent cannot be washed worn look. Since my last major surgery in 2004, Boo Boo has enjoyed a peaceful retirement, sitting quietly on my pink bedside drawers next to where we sleep at night.

I put on a brave, loud and fairly obnoxious front as a child, but part of the ability to do so was having my bunny clutched tight next to me, providing comfort, love and security.

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.

image source

I am beginning a series of interviews with adults and teens living with congenital heart defects. You are invited to learn more about the individual behind the obnoxiously long Latin medical term. This is cross-posted at my blog Glass of Win.

Meet my first interviewee, Carissa!

The interview is quite lengthily, so please proceed after the jump.

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