After the Darkest Clouds Come the Brightest Rainbows. ~ A Dad's Perspective.

Hi, this is my story and personal perspective of entering a world previously unknown to me.

I’m Dad to Ava - 7 in May, with heterotaxy, LAI with IIVC with Azygous drainage, polysplenia, right sided stomach, midline liver, two bi-lobed lungs, and LADDs performed at 7 months old with appendectomy.

We fell pregnant on honeymoon, and I couldn’t wait, having a niece and nephew I thought I knew what I could expect. Things got exciting for me as we came up to the 20 week scan, it was time to see our little miracle of life getting bigger.  We get to find out if it’s a boy or girl, and get to see it wave it’s little hand at us…I could then start to plan whether it’s football and karate for my little dude, or whether Daddy will be looking after my little princess, and painting stuff pink.

I completely missed the obvious clues of it being called an anomaly scan, why would anything go wrong? As we sat there looking at this beautiful image in front of us, it suddenly took a turn the wrong way.  The sonographer wasn’t sure what she could see, so went to get someone else to have a look. Panic sets in, this shouldn’t happen, someone tell me what’s wrong!  As more people come in and try to understand what they are looking at, you feel the fear rise inside, if they don’t know what’s wrong, this can’t be good. We are told to come back tomorrow to see the senior sonographer. We break down, but know at least we have a little girl now.

I believe I’m a strong guy, I’ve done martial arts for over 24 years and learnt how to keep control, but I am an emotional person. This was literally tearing through me.  That next day seemed like it took forever to come.

As we went back, walking into the unknown, it felt like night and day vs 24 hrs ago, nervous, scared, wondering what it means….and the scan didn’t help.  He too had never seen it in 20 years of scanning, they noticed her stomach on the right. More people came in to help, and we felt like bystanders in some bizarre medical programme.

Why is this happening to us, why can’t I stop this, hold my wife and make everything ok?  It’s my job to protect her and I’m failing her.  We then went through rounds of scans both at Reading and Oxford at the children’s specialist hospital, and privately in London to see the best there was to understand the issue.

As soon as we found a diagnosis of some kind, I hit the internet. I spent all my time on it, at work I did the minimal possible to survive and spent time looking for answers instead.  This was my mission now, to understand what our doctors didn’t, and try to help.  I felt completely helpless and my position of protecting my wife and child had gone out the window, how could I protect them from this? In a way I wished I hadn’t turned to the net. What I found after countless hours/days/weeks was what most people have probably encountered now, massive odds of having this condition, with very bad prognosis (80% mortality rate by 1 etc.).  There wasn’t the forum there is today, and examples were few and far between, and most seemed bleak.

I couldn’t share half of what I found with Mel, as she had so much going on already, as she was carrying Ava and dealing with everything inside her both mentally and physically.  I felt my duty was to help provide hope and hide the problems the condition could provide, I had to do something to protect her, as otherwise I felt hopeless.  Looking back, I’m not sure the approach helped either of us.

This happy time was turning into a survival game, and not one I was enjoying.  We were the first of our mates to go through having a child, I felt massively alone in this struggle to keep it together amongst them.  Most forums I found were mainly the mums, which helped me understand the condition and the struggles they faced, but sometimes you need a guy to speak to, to appreciate the turmoil and pain you’re suffering, yet still trying to be strong outside for your family. I’ve got great mates, but until you’re in the middle of something like this you don’t appreciate just how hard it is, and what it does to you, so they couldn’t really relate.

This changed me from who I was. I hit rock bottom thinking we would lose our daughter and that I would have let her down as her father by not protecting her.  In my mind, as men, we protect, but how can we protect against this, I was fighting an impossible fight…

Then the outlook started to improve for Ava, she avoided heart surgery, we made the tough decision to do the Ladds, and we started to work towards what life was like living with a complex condition.

I became a Trustee and Treasurer for the Heartline Families charity in the UK to help save them from collapse, and help others out as a way to focus my thoughts.  In hospital with Ava we’d seen so many babies and children in worse scenarios, I felt I had to help keep a network going for them.  In hindsight, it didn’t help us as was more pressure on me, but has helped provide hope for others.

Over the following years we’ve had ups and downs with lots of emergency hospital check-ups, extra tests, waiting for results, always worrying about Ava and what could happen.  My phone is always on in meetings waiting for the call to say she was in big trouble, it wears you down.  It came before Christmas and was the worst call I’ve ever had to take, as she collapsed at school and taken off in an ambulance….lucky she turned out ok.

We were always told she had asplenia, and now after that call and further scans they’ve found 4 J (which she named J). She’s now signed off from heart care, her LADDs is still working well, her immunologist now doesn’t want to see her for another 2 years, it feels like we’re coming out the other side, of a very long nightmare.

I find my resilience to anything is lower now, my tolerance of trivial issues has pretty much gone, and I have little patience.  Once I’m back into training (I put it on hold as priorities changed) things will get better and hopefully we can start to move on a bit.

I still fear for my daughter now, what does the future mean? I still research, but much less than before, and organisations such as Heterotaxy Connection help massively by bringing us and the latest info together, and help provide a more balanced outlook on what it may all mean. It’s sad but also great to see so many people on the forums now.

We’ve been very lucky with Ava, my wife has been amazing through it all, and although I don’t show it all the time I truly believe she’s amazing for being her, and thank her from the bottom of my heart for her support. I hope I’m a good dad to Ava, I want to protect her more than ever, but know there is still so much unknown with her.

We now have Alfie as well, just turned 3, and that journey through pregnancy was tough, but luckily he’s a healthy heart with no sign of heterotaxy. His no fear attitude helps balance out the house.

I take my hat off to all the mums out there, us partners go through a lot, but you carry our children through these tough months, and help keep them strong.  And to the other dads - stay strong, find us on FB (Heart Dads Support group) if you need to talk. 

After the darkest clouds come the brightest rainbows. However, through places like Heterotaxy Connection, Heartline Families and Heart Dads Support, hopefully those clouds aren’t as dark for people now.

Thank you for taking the time to read my open and honest thoughts, and how this journey has been for me.

Darren Pickard

Heterotaxy...sometimes, it just works.

My son, Cal, is nearly 2 and a half.  He has heterotaxy, but he has had no major medical problems related to his heterotaxy diagnosis.  We know we are very lucky.

Cal’s specific diagnosis currently includes: left-atrial isomerism with an interrupted inferior vena cava with hemiazygois continuation, stomach on right side, large midline liver, polysplenia (at least 5), intestinal malrotation (specifically, non-rotation).  He has also had malformations noted with his gallbladder and kidneys.

We discovered Cal was different at his 20 week ultrasound scan, you know, the ‘gender-reveal’ scan.   I was thrilled to be having a little boy, and the tech was bubbly and talkative until she wasn’t.  At the end of the scan, she brought in a doctor and another tech to look at the images and they told me my son’s stomach was on the wrong side.   The doctor said she had never seen it before and didn’t know what it meant. 

We eventually got referred to a pediatric cardiologist where I had a fetal echo and we got the diagnosis of heterotaxy.  The cardiologist assured us that Cal’s case was very positive compared to many heterotaxy cases.  She said that there were no guarantees, but a likely worst-case scenario for him heart-wise would be that he would need surgery to close any potential atrial septal defects, or he might require a pacemaker.   She also warned us, however, that other systems were affected with heterotaxy and we would need other consultations.

Getting the diagnosis of heterotaxy changed things for me.  I am a geophysics professor, and though I am not medically trained, I am scientifically trained and I also have access to virtually all medical journals.  This is, perhaps, both a blessing and a curse for me.  I began voraciously reading about heterotaxy, both online blogs and medical journal articles.  I read the harrowing stories of parents that had fought for their children to the end of their short lives and I also read the medical journal statistics showing the multitude of potential problems and high mortality rates for heterotaxy.   I made lists of possible issues Cal could have, and filled a manila folder with journal articles on heterotaxy.  My MFM doctor and I made a plan for his birth that included having a Level 3 NICU team in the delivery room to evaluate him right after birth.

Cal was born on a Friday evening after only two hours of labor.  I was thrilled when the NICU team said that they saw no reason to take him and that Cal could stay with me or in the well-baby nursery (well baby!), and that he would just have a few extra tests.   He looked like the perfect little baby.

Cal being the perfect heterotaxy case, however, was broken the day after his birth when we were told that he had no visible spleen on an ultrasound; I contested this finding since his particular heterotaxy characteristics fit so well with left-atrial isomerism, also frequently called polysplenia because the features usually occur together.  A few months later an ultrasound tech identified 3 round spleens and later an abdominal scope to look at his intestines found 2 more spleens. 

Cal (3 months), big sister Eva and Mommy celebrating ‘Spleens Day’ the day that 3 small spleens were found on Cal’s ultrasound scan.  Some spleens must be better than no spleen right?!

We currently treat Cal as functionally asplenic with a very low dose of daily antibiotics, since polysplenics may or may not have fully functioning spleens.    I was very nervous about this particular diagnosis when he was a baby because functional asplenia makes Cal very vulnerable to sepsis, which can occur incredibly rapidly in babies and small children.  Alex (my husband) and I were constantly checking his forehead for fever, or any other signs that he might be sick.  You know how some women wake up at night to check if their new baby is breathing?  I was checking to see if Cal was feverish.

Two years later, I am much, much less worried about Cal’s heterotaxy.    Cal does all of things two year olds do.  He loves watching Elmo and splashing in mud puddles. He goes to the gym kid care and throws magnificent bedtime tantrums. Aside from the daily antibiotics (which we struggle to remember) very little reminds me that Cal has heterotaxy.  Instead we worry about his speech progression (he is a bit delayed, but he is catching up rapidly), his frequent falls while running (his is pretty klutzy, like his mama) and trying to get him to go to sleep at night.

Cal, 2.5 years, at the Columbus science center COSI. 

I am not a really open person on social media. But I am so grateful to all of the women and men who have shared and are sharing their stories about heterotaxy.  They helped me to understand what I might be dealing with after Cal was born, when I was preparing for this unique, complicated baby.  I honestly don’t remember reading a story about a baby or young child that was as positive as Cal’s story.  So I am adding this out into the internet as another heterotaxy experience.  Yes, many times heterotaxy is difficult and dangerous, but sometimes, it just works.

Ann Yockey, mom to Cal

Heterotaxy Awareness Day - May 4th!

Heterotaxy Awareness Day (HAD) is less than 2 months away and it is a time to not only raise awareness, but also to connect with other heterotaxy families. Here are just a few ideas. Please share additional ideas in the comments!

1. Get moving. We are thrilled to announce our inaugural Heterotaxy Connection Anyway 5k, a fundraising opportunity that also raises awareness. The best part is that you can do it however you want. You can walk, run, or roll! You and other local families could organize a traditional 5k in your area or you could pledge to cover that distance on your own over a week. It’s up to you! More details coming soon!

2. Get together. One of the most rewarding parts of HAD is joining together with other families who have been on a similar journey. In the past, families have met at zoos, aquariums, parks, and houses to celebrate our heroes.

3. Proclaim. A great way to raise awareness is by getting your state or local government to proclaim May 4^th Heterotaxy Awareness Day.

4. Wear Awareness. Proudly wear teal and gold on May 4^th. T-shirts and other items for the whole family will be available soon.

5. Spread the word. Share your story with your local media.

6. Goodies. Create items to hand out on awareness day.

7. Balloon release. Release balloons in memory of the Heterotaxy Heroes we have lost.

We can’t wait to see how you decide to celebrate Heterotaxy Awareness Day!

Please e-mail stacy@heterotaxyconnection.org with any questions and/or suggestions.

Life...beyond perfect to us now!

After the birth of my daughter in 2006 we decided our family was complete. I signed paperwork to have my tubes tied only to call and cancel a week before my procedure. There was a sadness that took over me at the thought of not being able to have children again.

After nearly eight years we decided we would try for another baby. There was just something missing from our family.

We first talked with our other two children to see how they felt about it. My son, who was 11 at the time was all for it. My daughter, who had been "the baby" for 8 years was devastated. She was so afraid that the new baby would "take her place." I reassured her that she would always be my baby & I loved all my children equally. My son wanted a new baby so badly that he even tried convincing his sister. Once she decided she was ok with it she wanted the baby now!

We found out we were pregnant in November of 2013. Three months after we started trying.

Everyone was over the moon excited!!! We included the kids in everything that had to do with the baby. We scheduled a 3D/4D ultrasound at around 18 weeks to find out the gender of the baby since they kids wouldn't be able to attend the anatomy scan. The looks on their faces & the excitement in their voices when the technician placed the wand on my belly and their baby brother or sister showed up on the big screen television nearly brought me to tears. I could tell right then that the love that they had for this baby was immeasurable.

That day we found out we were having another boy. I think my daughter was secretly happy about that because it meant she was still "Daddy's girl." The whole way home the kids passed the 4D ultrasound pictures back & forth in the backseat, chatting about who he looked like. We all agreed that he favored his big brother. They were both so proud!

I got my repeat cesarean delivery date at the beginning of July 2014. I delivered our baby boy on July 16th, 2014 at 7:55 in the morning.

Once back in my hospital room we all waited until he was released from the nursery. They were having a bit of a time bringing his temperature up. My daughter was so anxious! She just couldn't wait to get her hands on him. Both of my children walked back and forth from my room to the nursery window across the hall. The anticipation was almost too much for them to bear. For months they rubbed and talked to my belly and finally the baby they'd waited nine long months for was within arms reach!

After what seemed like an eternity, my husband and two children, all with smiles from ear to ear followed the nurse as she rolled the baby into my room. Life at that moment was perfect. We all basked in the beauty of this new born life that we had been blessed with.

Hours after Tate's birth things went downhill fast. What started out as a celebration of the birth of our third child, their baby brother, quickly turned into a day of tears. A day of uncertainty. A day of sadness.

We didn't know what was wrong with Tate, we only knew at the time that he was sick. Very sick.

Witnessing my children's faces go from complete happiness to fear & wanting somebody, anybody, to tell them what was going on was almost more than I could take. This wasn't how it was supposed to happen. They weren't supposed to ever have to go through something like this.

The day of Tate's birth we received the news of his sick heart & that he would have to be flown to the Children's hospital in Birmingham Alabama the next day. That night my daughter stayed with my husband and I at the hospital. She was terrified. She crawled into bed with me and just cried and cried. All I knew to do was love her and tell her that everything was going to be okay.

The next day I was discharged so that we could travel to Birmingham and be with Tate. There was mainly silence from the kids. Every once in a while there would be the sudden question. The ones where you just know their little minds had been pondering. It was heartbreaking. They were scared of the answers they may get.

We made it to the hospital that Tate had been transferred to and after many tests we received his diagnosis. Heterotaxy Syndrome with asplenia and complex CHD. We got his surgery date. July 24th, 2014. Eight days old. Having to explain to my children that they would have to cut open their baby brothers chest to fix his heart was one of the hardest things I've ever had to do. They were 11 and 8!!! This wasn't something I thought they would ever have to endure. Never in a million years. But, much to my surprise, they were okay and they were understanding. They were still scared and fearful, but they knew this had to happen. My babies, my sweet, sweet, understanding babies.

Surgery day came & everything went as planned. My daughter spent a lot of her time standing at Tate's crib side rubbing his baby hair to the side & saying over and over "you're too sweet for this." She was so precious to him.

During Tate's recovery the kids had to travel back home with family to start the school year. This was hard. We had spent the summer break as a tight knit family, preparing for, delivering, and ultimately standing by our baby as he fought for his life. But, once again, they understood. They knew that with Tate is where we needed to be at the time and that this was only temporary. Their willingness to work with us as a team and set their own feelings aside for the sake of their brother was overwhelming to me.

The days and weeks came and went. There were many tearful phone calls with our children, mainly because we missed them & they missed us. They never lost sight of the ultimate goal and that was to bring the baby brother that they'd waited so long for home!

The glorious day finally came! We were discharged from the hospital and sent home three weeks after Tate's birth. Having our three children at home was something we were unsure would ever happen. But it did! We survived. We worked as a family and we made it work. I'm not sure where we would be mentally if it hadn't been for our oldest two being so unselfish and understanding. Life may not be how we had envisioned it while I was pregnant with Tate but it is beyond perfect to us now!

Amanda Orso (mom to heterotaxy hero, Tate)