We will never stop fighting!

Our son, Palmer Nicklaus Strong was born 4/1/17 in Austin, TX. We received the Heterotaxy Diagnosis 12 weeks into my wife Kristen's pregnancy. We tried to research what Heterotaxy meant for us and our soon to be baby, but the more we read, the more questions we had. The Dr.'s and Cardiologist's, although aware of Heterotaxy, couldn't truly ease our fears or answer all of our questions, because as you know about this condition, there are just so many unknowns, especially in utero. Palmer was diagnosed with Dextrocardia, AVSD, and PA. 

Upon his arrival, we quickly realized it was much much worse. His first echo revealed TAPVR and MAPCAs. Palmer also had bi-lateral SVCs which have proven to be difficult in his current situation on ECMO. Palmer is now 43 days old, 33 of these have been on open chest VA ECMO at Texas Children's Hospital. I could have never imagined a 43-day period this tumultuous and difficult, and my wife and I are praying that we can have more. 

Palmer was given 0% chance of survival in Austin, and since having surgery at TCH, we have continually been told he has very little chance of survival. We continue to fight because Palmer continues to fight. We have created Heart Strong to honor our son as well as all of the babies and parents that are living with a Heterotaxy and CHD diagnosis. We honor Palmer by honoring others. We will never stop fighting! The logo represents the backwards heart that many of our babies have as a result of this syndrome. The HS represents Heterotaxy Syndrome, and the missing pieces in the letters represent the pieces that may be missing or broken within our children. But as a whole, our children are still Strong! Heart Strong!

Little Sammy Braveheart

Tomorrow--May 4th--is Heterotaxy Awareness Day, and similar to Sammy's heart surgery during CHD Awareness Week, he's right on time to promote awareness of his primary diagnosis and the reason for his Ladd procedure tomorrow. Heterotaxy--is literally a "Different Arrangement" of internal organs and a range of accompanying birth defects, including one of the more common ones, intestinal malrotation. The Ladd procedure will untwist his intestines and loosen/divide any abnormal bands (Ladd bands) of tissue contributing to the malrotation. As a bonus, Sammy gets an appendectomy to prevent future misdiagnosis since his appendix is on the left instead of right side. #TheWorksPlease

But my grandson, Sammy, is much, much more than his heterotaxy diagnosis! Just one year ago, before he was even born, a doctor told his mama his condition was "incompatible with nature," but that was old news! Not that long ago few options existed for babies born with more serious forms of heterotaxy. However, not so today! I hope you'll take a minute to meet 7 month old Little Sammy Braveheart (my favorite nickname for him!) Sammy's a bouncer and a snuggler and soft muslin baby blanket lover. He only flashes his big baby blues and broad toothless smile for big brother, Jack...until he sees Mama...then he flashes his big baby blues and broad toothless smile for Mama...until he sees Dada....who always seems to cause his great big cupcake cheeks to erupt in the biggest, widest, brightest, almost one baby tooth grin ever seen. He just plain melts our hearts... and I bet he'll melt yours too. Sammy loves making new friends and you can follow his mama's blog at www.wholeheartedlyblessed.com

#LittleSammyBraveheart

A Letter to Alex's Teacher...

Alex, heterotaxy hero to HC board member and co-founder, Heather Williams, starts kindergarten this week.  So "he" wrote a letter to his teacher introducing himself...

Hi, my name is Alexander Williams, though I like to be called Alex.  I was born in June 2011 with heterotaxy syndrome.  Don't worry if you've never heard of it, most people haven't.

Heterotaxy Syndrome is a congenital disorder, meaning it was present at birth.  If you've ever studied Latin, you might decode heterotaxy as meaning different arrangement, and that's a pretty good start.

Looking at a person from the outside, it’s hard to imagine the complexity that lies on the inside.  People look symmetrical between the left and right side.  A person’s arms are about the same size and length, their feet are about the same size, and the ears are near the same spot.  The left side is much like a mirror image of the right, or vice versa.

On the inside it’s the opposite.  The left side is completely different from the right.  The stomach is on the left side of the body, the liver on the right.  Even paired organs that have one organ on each side of the body are structurally different from each other.  For instance the left lung only has two lobes, while the right has three and the right adrenal gland is triangular shaped and the left horseshoe shaped.

Heterotaxy disturbs this.  During the early days of a pregnancy (between the 3^rd and 6^th week) the internal organs are forming.  They are created from tubes of tissue that are told how to twist and turn to form the intricate chambers of the heart, blood vessels and digestive tract.  In heterotaxy, these twists and turns don’t happen the way they should, and it’s not entirely understood why. 

All those born with heterotaxy are unique, and have individual diagnoses that describe their particular condition.

I was born with multiple congenital heart defects.  I had to have two surgeries (one in Seattle, the second in Boston) to reroute all the plumbing of my heart and make sure I could get good oxygenated blood.   While my surgeons did a great job, my heart will never be quite the same as my peers.  My cardiologist says I'm allowed to play just the same as all my classmates, but need to be allowed to rest as I feel the need.  I also don't tolerate heat and cold quite the same as my peers, and might voice that opinion from time to time.

My intestines were also born mixed up, and I had to have a surgery for those.  My twisted intestines and the surgery put me at a higher risk for bowel obstruction.  Two things that can be warning signs are intense tummy pain and vomiting bright green.  My mom needs to know about those immediately.

Perhaps most importantly for you and your classroom, I was born without a spleen.  Of course one can live without a spleen (I'm five years of proof!), it actually has a very high function in the immune system.  Certain bacteria are specifically targeted in the spleen, and because I don't have one, I'm at a high risk of developing a life-threatening infection from them.  Good hand washing practices (though, I am 100% boy and still resistant to cleaning, much to my mother's dismay), covering our mouths and not sharing drinks will significantly help keep those risks down.  If I ever look feverish, lethargic or otherwise sick, please send me to the nurse immediately.

Finally, all the medical care I have received has left me a bit more sensitive than others.  I startle at loud noises (though I'm getting better) and am sensitive to people touching me.

I am looking forward to being in your classroom and starting my education journey!

Our Journey with Heterotaxy Thus Far…

To start off, I would like to introduce my family. Ian and I (Amanda) have been married for three years in June, and we have one kid, and one pup: Elyse, our 20 month old Heterotaxy Hero, and Trixie, our 3yr old 7lb Maltese/ Yorkie mix. Ian and I met and married in San Diego California, then 6 months later Ian accepted a job in the Central Valley, about 5 hours north of San Diego. Ian works as a Physician’s Assistant at a clinic here and loves what he does! I graduated college in April and am lucky enough to be a stay at home mom to Elyse.

Our journey started the summer of 2014. On July 2^nd (I was 16 weeks pregnant) we found out we were having a girl and were so excited! We named her Elyse right then and there and never had a doubt about her name. On July 28^th we went in for our 20 week ultrasound. Already knowing she was a girl we were not concerned about hearing anything else. We went home and started painting her nursery. An hour later we got a call asking us to come back to the office and meet with my OBGYN. I didn’t think anything of it but Ian, being the medically minded person that he is, knew something was wrong. We walk in and the first thing that my Dr. said to us was “This is not your fault, you did nothing wrong”. Then he explained that they had a hard time finding Elyse’s left ventricle and that the other organs in her body (stomach and liver etc.) were not in the right place. I bawled. He referred us to the local children’s hospital an hour away and said we would be getting a call soon.

A week later we made the drive and met with Perinatology and then they sent us over to Cardiology where they did a 3 hour fetal echo. Finally a cardiologist came in and explained to us what they found and what we needed to do, as well as the fact that Elyse had Heterotaxy. All I could hear was Charlie Brown’s teacher explaining it, but luckily Ian understood it a bit better. Her official diagnosis and all of its components are: Heterotaxy, LAI, Polysplenia, Intestinal Malrotation, right sided stomach, midline liver, HLHS, DORV, Interrupted IVC, and VSD.

That was a lot to take in for anyone, especially for a family experiencing their first child. The next 20 weeks were filled with love, fear, compassion, prayers, and lots and lots of tears. One of the hardest things was keeping it together in public. Our first Sunday back at church after hearing the news was the hardest. We go to church where families are so important and someone always has a newborn. I felt peace there, but my heart was breaking on the inside not knowing if I would ever get to bring my child home. I felt jealousy for these people that had perfect little families and over time that jealousy was replaced with gratitude and love for them. Gratitude that they weren’t having to go through what we were, and love because they deserved it.

We told only our family in the beginning and then as the day grew closer we told more close friends as we needed to have a plan set up for our dog to be cared for while we were in the hospital. We tried our hardest to move on with the preparations for Elyse. We bought her clothes and decorated her nursery even though we knew that she may never get to wear the clothes or sleep in her nursery.

We met with Perinatology, the cardiologists and my new OB (as I had to be seen by a high risk OB an hour away) weekly and more than weekly at the end. We did NSTs, and biophysical profiles up to three times a week at the end (again… an hour away). We even met with Elyse’s cardiac surgeon; that was a hard meeting!

I was induced a week before my due date because the doctors were worried that I would go into labor and not make it to the hospital in time. After 50+ hours of labor and one hour of pushing, Elyse was born! She looked great!! I got to hold her without any wires for just 30 seconds before they took her and cleaned her up. Then they took her for about an hour and made sure she was ready for transport (to Valley Children’s Hospital 7 minutes away) then they brought her back in to see us in an incubator and daddy got to hold her for the first time. She had wires that made it hard to hold her and a bit scary, but she was so alert and interested in what was going on. She was perfect!

The next 3 weeks we stayed at VCH waiting for her Norwood procedure. She was doing great, the doctors weren’t going to do it unless she needed it and so they didn’t. We waited for her PDA to close instead. While waiting, Elyse’s cardiologist mentioned doing the LADDS procedure since we are waiting there anyway and we would be able to go home without as much of a worry of a volvulus happening. She was 11 days old when she had the LADDS procedure done and she handled it very well. 8 days later her PDA was so close to being closed that her doctors didn’t see why she couldn’t go home. Christmas Eve we took Elyse out of the hospital into daylight and she got to feel the wind on her face for the first time. The best Christmas gift we have ever received!

The next months were filled with lots of appointments and talk of her next surgery, but she was doing great so the appointments went further apart. We embraced this miracle and let her be a kid. We took extra precautions when out and she got extra shots, but at home she was a normal, happy baby. She was meeting all of her milestones and became very good at the army crawl!

When Elyse was 7 months old her cardiologist noticed her O2 stats were dropping into the mid 80s. So we started being watched a bit more closely again. They dropped into the low 80s and there was talk of a cardiac catheterization to see if that would help her to go longer without heart surgery. But it didn’t work. Her stats were in the low to mid 70s and we prepped for surgery. 2 days shy of 9 months she went into VCH to receive her Kawashima procedure. That was the longest 6 hours of our lives. But she did great! When we asked what her heart looked like, her surgeon said “scrambled eggs, just like I thought it would”, in a very light and playful tone. As they moved her from the OR to the PICU we got to see her already extubated and wide awake staring at us. The next 8 days were filled with long days and nights in the PICU, but we saw improvements every day. 8 days after her surgery we were blessed to bring her home. We followed up twice in 5 days and the second time they admitted her again. She had a chylous effusion that needed to be treated. Because Ian had already taken so much time off work, I braved this hospital stay on my own. We were informed that she would not be allowed anymore breastmilk (I had been pumping for her up to this point) but instead she was being put on a low fat diet of Enfaport formula and low fat solids, as well as a slew of medicine. So I stopped pumping (as I had a freezer full already and by the time she was allowed breastmilk again she would be almost 1 year) and we adjusted to the new diet. She was again sent home after 5 more days in the PICU getting her chylous effusion treated. We returned 3 times a week at first, then eventually twice a week and then finally just once a week for the remainder of the year. Each visit was an hour drive there and back, plus labs, X-rays, and a visit with her cardiologist (about 5 hours each visit). Come her first birthday in December Elyse was crawling on her hands and knees, able to eat fatty foods again and the happiest one year old ever! In January we were told wonderful news… no cardiology visits for 4 months! That was huge! She started walking at 15 months, which didn’t last long before she was running! At the end of May we were informed that we can go 6 months without seeing cardiology, and then her pediatrician said (at her 18 month checkup) that he didn’t need to see her until her 2^nd birthday… 6 whole months without ANY doctors appointments?!?!? What a shock that is and was to us!

Elyse is now a tall and skinny blue eyed and blonde haired little 20 month old miracle. She loves playing with puzzles; helping mom cook; playing at the park especially the swings; going on walks and hiking; shoes of every shape, size and color; playing with Trixie; daddy coming home from work; getting into anything and everything she can; cleaning (she grabs the swifter duster or a baby wipe and cleans everything!); giggling; smiling and being a kid!

Our little family is so incredibly blessed to have the Heterotaxy community and the heart families to lean on and learn from and to support. We appreciate the love and support felt and to be part of a group of people so full of love! We wouldn’t be where we are without the love and support of friends and family, as well as the love we feel from God.

This journey is not something we would have ever chosen for Elyse or ourselves, but we are grateful to have been able to come this far and are excitedly nervous for what is in Elyse’s future. We have no idea what else she will need, but we are confident that the medical advancements that happen every day will aid in her future and are grateful for every day we have and hope for many more.

Amanda Primavera - Mom to Heterotaxy Hero, Elyse

Advocating for your Heterotaxy Hero

After my son, Noah, was born, we spent the first few months in the hospital just trying to get him to survive. It was when we finally came home, and I started to do my own research, that we saw just how unique heterotaxy is. No heterotaxy child is the same. As I connected with other heterotaxy parents on Facebook, I learned more and more about my son’s diagnosis and what to watch out for. Invaluable information that many times health care providers cannot or do not provide. I think most doctors have good intentions, but ultimately most heterotaxy parents have more knowledge about it than them. Specialists can tell you how they would treat a child within their area of expertise, but oftentimes they don’t know how that will affect the rest of the child’s body.  For example, one complication of heart surgery is vocal cord paralysis because one of the nerves that stimulates the vocal cord runs close by the heart. My son had this happen during his second open heart surgery. When we talked to the ENT doctor, they said not to worry too much because the other vocal cord compensates for the paralyzed one, and it doesn’t cause any problems down the road. I asked about his third open heart surgery and the possibility of paralyzing the other vocal cord nerve. They said it was not close to where they would be operating.  But my concern was that they didn’t know exactly where that other nerve runs because of Noah’s heterotaxy, and it might be different than normal, and they agreed. So it’s things like this that are always in the back of my mind when a health care provider talks to us.

 

I think it’s important to know that most pediatricians have never treated a patient with heterotaxy, and that’s okay. The key is that they take this diagnosis seriously, research it themselves, and are open to your concerns. No one is more committed to your child than you. We, as parents, have the extra time and motivation to do the research to help our children. Its okay to ask questions, and doctors should not dismiss them.

 

Noah is now 4 years old and has come a long way from the scary times we spent in the hospital his first few months of life. His diagnoses include TAPVR, DORV, hiatal hernia, asplenia, and microgastria. He has undergone 3 open heart surgeries and 1 GI surgery. He coded a day after his first open heart surgery and was placed on ECMO for 3 days. Later we would find out that he suffered a stroke while on ECMO and was diagnosed with mild hemiplegic cerebral palsy (fancy name for one sided weakness). You would never know anything was wrong with him if you met him today. We just got back from Colorado where he dealt with the altitude better than the rest of the family. He went fishing, swimming, tubing, ziplining, golfing, and ice skating. He will be going to his second year of preschool this fall. He is absolutely perfect in our eyes. We thank God for him every day. He is our hero, and we could not love him any more. His next milestone is being promoted to big brother this October J.

Alana Madden - Mom to heterotaxy hero, Noah

Just Our Luck...

Years before our Heterotaxy hero, Corbin, was born, I had someone tell me, in reference to me and my husband, "You guys have the worst luck!" It took me by surprise because I had not viewed my life or ours in that way. Sure, we had some bumps along the road, but didn't everybody? 

Fast forward about 9 years and so much more has happened. Most recently, our car broke down on the evening of the 4th of July, on a dark two lane highway, in the middle of nowhere in TN, on our way back to MO from a family wedding in SC. I had some moments when I was extremely nervous. We had no operating electricity in our black SUV. Would a sleepy truck driver side swipe us? Would a party-goer that had one too many ram into us? I remember the changes in the plan for the tow being extremely frustrating. The insurance company recommended one destination and the tow truck driver suggested and convinced us that another was best. We anxiously awaited the arrival of the state trooper to give us a hedge of protection on the road as well as the tow truck to get us out of there. And time ticked on so slowly. 

Later that night, in the motel that the tow truck driver took us to, I thought of what was said to me many years ago about our luck. I thought about the many things that had transpired since that time, especially with Corbin and his health. I thought about the fear and worry while pregnant. Will he be blue when he's born? Will he need to have surgery right away? Will our baby make it? I remember being extremely frustrated with the changes in the doctor's plans. After birth, initially some doctor's felt like his malrotation was a priority and others felt like his complete heart block needed to be addressed first. The plan for his care changed a lot over the course of his first days in the hospital. Then the surgeries. I remember waiting after his pacemaker replacement at 4 days old, after his CAVC repair at 5 months, after his Ladd's at 9 months. Just waiting, for him to get stable and recover enough to go home. 

As we face a pacemaker replacement for Corbin this winter, I think I've figured out why that statement "bad luck" still doesn't resonate well with me. I'm able to see all the blessings that have prevailed throughout our lives: strong support systems through friends and family, resources and information, faith, prayer, and love, kindness and compassion. These things have been provided to us through every step of our journey. It makes all the stuff that we go through not take over and become the focus. And hopefully we can face this next step unburdened and head on.

Angel Swinton ~ Mom to Heterotaxy Hero, Corbin

Advice That Stuck...

Back in 2010 I was happily pregnant and full of excitement.  Expecting another regular appointment I went to my 3rd trimester ultrasound.  It was then that my entire world turned upside down, shaking me into an entirely new perspective on life... what matters and what does not.  In its ugliness, I guess, that is the beauty in Heterotaxy, the way it humbles us and opens our eyes, minds and hearts.  

My first reaction was fear, fear of the unknown.  I had never heard the term heterotaxy in my 24 years of living on this planet and I didn’t know what to expect immediately following the diagnosis.  Going to doctor appointments always brought me to tears.  Luckily, I ended up with a caring cardiologist who trusted me with his personal cell phone number to call whenever any questions or concerns were eating at my brain.  

On August 5th, 2010, my heterotaxy warrior made her big entrance into the world.  Although she was required to stay in the NICU for 3 weeks, she did not need surgery at birth.  She did, though, go home with a NG feeding tube.  It was so overwhelming and scary having her home.  I was afraid of not doing things right or missing something.  I was afraid of heterotaxy.  The first couple of months we were so cautious with her.  We didn’t go to public places, we kept her pulse ox on the majority of the time, and we over thought everything.  

It came time to take a trip out of state when she was a few months old.  We almost didn’t go due to fear of being away from home if anything happened.  Right before the time came to go on the trip she had a cardiology appointment at which we voiced our fears and concerns.  It was that appointment that shaped the rest of our heterotaxy journey.  The cardiologist sat down with us and told us not to worry so much.  He told us to live life as normal as can be, as if there was no heterotaxy.  To give our daughter a normal life and not keep her in a bubble.  To keep an eye out for signs of complications, which we had been told about on many occasions by different medical professionals.  He told us that even in the event of complications arising, not to think that she would die on the spot so long as we followed the instructions we had been given.  His words gave me such reassurance.  I called him on his cell phone right before leaving for our trip to double confirm what he had told us in his office.  He again told me not to worry and said, “Go on your trip and have a great time!” and that is exactly what we did.  

Since then we have traveled out of state and even out of the country on several occasions.  My warrior is now 5 years old and has graduated kindergarten at a public school with minimal sick days.  In her 5 years, she has been to many birthday parties, pool parties, school activities, the river, the lake, the beach, and much more.  She has been through two open heart surgeries and an abdominal surgery, all which have been with minimal or no complications.  To us, quality means more than quantity and for that reason we have chosen to let our special child live her life to the fullest for as long as she has the ability to.  I will not deny that I still feel fear.  I fear that we are being too risky when we allow her to do all the normal things a healthy kid would do.  But, the advice her first cardiologist gave us stuck to us like glue.  In our case, I believe that our warrior has done so well thanks to us following the advice of letting her live a normal life.  Maybe her exposure has been her strength, I don’t know.

Heterotaxy presents itself so uniquely individually, and we all deal with it in our own ways.  Ultimately, we all want the best for our kiddos and do what we think is best for them.  My goal is not to brag about how well my daughter has done and is doing nor to say that my approach is the best.  My goal is to give hope.  To let it be known that sometimes it is possible to do normal things and live a normal life even when nothing about our lives are truly “normal”.

- Irene Belezair (Carlie’s mom)