Courage is not the absence of fear, but rather the judgment that something else is more important than fear.

- Ambrose Redmoon

The way is not in the sky. The way is in the heart.


Wednesday, February 13, 2013

Day 13: MEET AUBREY and ZOE!!!

Today brings us the stories of Aubrey and Zoe....

Aubrey's mother writes.."Sometimes I think becoming a special needs parent, or a heart parent, is similar to a broken piece of glass. No matter how much care you put into piecing it back together, there will always be cracks and lines. There will always be small pieces missing, because you can't ever really truly put it all back together again."  

Zoe's mother writes.."Despite all of her issues, she is the happiest baby, always has a smile on her face."

Such big miracles in such little girls!!!


 Sometimes I think becoming a special needs parent, or a heart parent, is similar to a broken piece of glass. No matter how much care you put into piecing it back together, there will always be cracks and lines. There will always be small pieces missing, because you can't ever really truly put it all back together again. Once Aubrey was born, life became about choices, often even having to choose between my children. The incredible guilt I felt every time I left her in NICU and came home, on top of the pain I felt every minute I was away from my other children. However, since having Aubrey I've found courage and strength that I never knew possible. Handing your child over knowing their heart will be stopped is incredibly difficult. Finding the heart community full of other parents who have been in our position has been one of the things that has held me together.

We learned early in our pregnancy that Aubrey would have health issues. Early ultrasounds showed Aubrey had a cystic hygroma, which is excess fluid in the head and neck area. I went to that ultrasound alone, as this was my fourth child and I didn't think much of it. After my ultrasound I was led into a grief room, a room I ended up in several times throughout my pregnancy. I sat waiting for the doctor and looked around and saw pamphlets and business cards for support groups of parents who have lost a child, photographers who will photograph your child after they pass, as well as literature about terminating pregnancy due to health concerns. It was confusing because I knew Aubrey had a heartbeat. The doctor came in and counseled me on several issues that could be causing the fluid, mainly Turner Syndrome, which in most cases results in stillbirth. She suggested I have another ultrasound as well as an amnio so that I could decide. Over the next few weeks we found out more. Aubrey had bilateral clubfoot, as well as an issue with her spine. Instead of the pretty strand of what look like pearls normally in ultrasound Aubrey's ultrasound technician kept calling more people in as hers looked "really weird." Not something you want to hear from an ultrasound technician.

We had an amnio done after much thought. We decided early that no matter what, we would not terminate. Aubrey was whatever child God meant for us to have, therefore even if we only got 5 minutes with her, we would make sure we loved her in that amount of time as much as possible. We wanted to be prepared either way. After the amnio came back normal, they wanted to do more testing but we declined. We did agree to a fetal MRI, as well as a fetal echo. The MRI came back as "unremarkable" but the echo showed Aubrey had Tetralogy of Fallot. As Aubrey got bigger the doctors felt her movement was also abnormal. Back to the grief room where we discussed arthrogryposis, and we were again offered termination. We were told Aubrey would not be able to move her limbs, and would be born in a fixed position. We were also told that given all her other problems this was likely a terminal variant of arthrogryposis. 

With the stress of everything I developed preeclampsia at 29 weeks pregnant and was admitted. I was told I'd be on hospital bedrest until she delivered. The orignal goal was to deliver at 36 weeks, due to the arthrogryposis it would give her the best chance at having less contractions in her joints. At that point their goal changed to 34 weeks. On August 14th I started having stomach pains, and a raging headache. By the morning of the 15th my labs were bad, they came in my room and told me they had to deliver here now. It all happened so quickly I actually had my cell phone in my hand in the OR. Because of her joint contractures they removed my uterus, and then removed Aubrey to ensure they did not break any of her bones. I remember hearing her cry right away, and looking at Scott as he looked at Aubrey. I had so many fears my entire pregnancy and they were quickly gone as I saw his face. He had the biggest smile on his face, and he looked at me and said “She’s so beautiful. She’s so perfect.”  He also told me soon after she was moving her arms just fine. Soon after a nurse came and told me that with her heart and everything else I should not get my hopes up. She also tried to talk me out of getting a tubal ligation “just in case.”  I gave him my cell phone and he took several photos of her, then showed them to me as I laid there waiting to see her. They brought her to me swaddled and let me kiss her, then took her for an echo. Several hours later she was intubated for work of breathing, then transferred to Riley Hospital for Children. The Lifeline crew brought her to my room so I could say goodbye. Despite being told not to get my hopes up, I kissed Aubrey Hope goodbye and began working on getting out of there.

I was discharged a few days later, and went straight to Riley to the NICU to see Aubrey. That began our long three months of sitting in our tall chairs next to her, talking to her, and waiting for her to come home. The first several weeks we were told she just needed to get bigger then she’d come back at 9 kilos for open heart surgery. Finally, at 5 weeks old the decision was made to take her to surgery for the BT Shunt and PDA ligation. Handing her off was hard, seeing her post op was even worse. Her shunt was placed on 9/23. On 9/26 we were sent back to a different NICU where a Nurse Practitioner argued with me telling me she hadn’t had heart surgery, it was stomach surgery, therefore they were not going to start her feeds. Finally she told me “Well, I haven’t read his chart, but there is a pot luck down the hall and I need to go before all the good food is taken so I will read it when I get back.” 

This set the tone for our entire week. Each day Aubrey’s sats got lower, and the amount of oxygen she was on increased. I asked repeatedly if her shunt was working and was always told “Of course it is, Dr. Brown did it.” Neonatology started telling us things like “It’s cardiology so of course it’s never the heart.” On 9/29 we asked for a care conference and no one from cardiology would come. I was patted like a child that day and reassured it wasn’t her shunt, and did I honestly expect her sats to be better just because she had the shunt? Yes, yes I did. The next day one of our NICU nurses saw how upset I was and went and got several doctors to look at how blue Aubrey was. Her sats were actually better that day than they had been all week. I was actually told at one point that week that perhaps the 60’s were just her baseline. Finally they got me to agree to go home and nap, and would do an echo. As we walked in the door we got the call “This is an emergent situation, Aubrey’s shunt is completely occluded.” She went into the OR that night for central lines. They started Heparin that night, and we waited the weekend for the plan. They kept telling us to wait and let the heparin do it’s job. Finally on 10/4 she went into the cath lab where they placed stents in her Right Ventricular outflow tract.

The next few weeks were a blur. Failed extubations, more surgeries, airway issues, failed swallow study, a G Tube. Finally on 11/3 we were able to bring Aubrey home. Initially cardiology stated they didn’t need to see her back until February. I complained as Aubrey had already done unexpected things, so we went for follow up 2 weeks after discharge.

After being home with Aubrey the reality of all that transpired set in. I started calling other hospitals, and while they all had different opinions one thing was constant: None would have waited 7 days to do a post op echo. We asked for copies of Aubrey’s medical records, and upon going through them learned that Aubrey has Factor V Leiden, a clotting disorder no one told us about.  After speaking to other hospitals we learned that it would add to her risks of being put on bypass for open heart surgery. No one had informed us of this. So we began sending her records out to other doctors. As a family I think we made the decision quickly to not go back to Riley for surgery, for fear that if anything happened post op we couldn’t get them to listen to us. We spoke to several doctors, and finally decided to travel to Boston Children’s with Aubrey to see Dr. Emani. On the phone he was patient, kind, and answered all of my questions. I developed an immediate trust for him. Arriving in Boston we quickly knew we made the right choice. Several times when we had to send Aubrey to surgery at Riley we had to give the anesthesiologist report ourselves. When she had her G Tube we were standing outside the OR and the doctors didn’t even know she had a heart defect. In Boston, they knew everything. They had all read up on Aubrey’s case. Aubrey had pre op tests she’d never had done before. The CICU staff were fantastic. 

On February 14th 2012 Aubrey went back for her open heart surgery. It was a long day waiting. Finally Dr. Emani came out and I could see him smiling as he walked towards us, so I knew all went well. They were not able to spare her pulmonary valve so eventually Aubrey will again have to see Dr. Emani for another open heart surgery.
Aubrey’s post op care was fantastic. The CICU nurses are on top of it. She had an echo in the OR, and several more over the next few weeks. After 4 weeks we were able to travel home. Home with a pink baby, who was not on oxygen for the first time in her life. It took me several days to adjust to her new color. I kept asking if she was sick, she looked flushed, etc. Finally the nurses explained to me she looks like a normal baby. 

We got home and life for Aubrey really began. She started having serial casting done to fix her clubfoot, First Steps came in and started working with her. At 7 months old Aubrey was developmentally a newborn. Now at 18 months old Aubrey is catching up. She’s taken her first steps. At a year old she was cleared to eat by mouth. At 18 months old we are working on G Tube weans to get eating by mouth. 

Aubrey was born with congenital scoliosis, which is now at a  67 degree curve. It is effecting her lungs so we are working on our plan for that. She has a hemivertebrae. We have several options and are looking into what is best and safest for her. We do know her spine surgery will take place at some point in 2013. Heart wise at this time she’s stable. Her sats are 98-100, she’s pink, and energetic. We will travel later this spring to CHOP to have her spine evaluated then we will start making choices regarding how to go forward surgically in whatever we feel is Aubrey’s best interest.

This has been quite a journey for our family, has taken it’s toll on our other children, our marriage, our friendships and relationships with others, but in the end Aubrey’s smile makes everything all better. She is truly my hero, the greatest blessing, and I can’t even remember what life was like without her. I never thought I’d be a heart Mom. After having 3 children I took their good health for granted. I knew a few heart mom’s but never really understood, only marveled at their strength and wondered if I myself could ever have that in me. It turns out I do. We tell our children all the time Aubrey’s smile is all the proof any of us need what a great team our family makes. 

One thing I hope other mother’s can take from our journey is to never give up hope. Sometimes I wish I could go back in time and tell myself to be stronger, but I think even at some of my worst moments  in the weeks after Aubrey’s failed shunt surgery that helped me gain the strength and courage I have now to keep asking questions, keep seeking opinions, and doing what we feel is best for Aubrey.  Never be afraid to step on anyone's toes. I will stomp on feet if I have to if that means getting Aubrey the best care. I wish I’d known that from the beginning, but it’s something I no longer fear now.


Zoe Fiona-Grace is our 2nd child. She was born on Superbowl Sunday, February 5, 2012. She was diagnosed with Heterotaxy Syndrome. People born with Heterotaxy Syndrome usually have an abnormal arrangement of the organs and in most cases, complex congenital heart defects are present. Heterotaxy effects 4 out of a million live births. Heterotaxy is completely rare and each case is different from the next.

We found out about Zoe's condition in utero at my 20 week ultrasound. It was the saddest and scariest day of my life thus far. We received "gloom and doom" reports and diagnoses every week thereafter. It was basically insinuated to terminate our baby from weeks 20-24 by numerous doctors. Mike and I would never terminate, no matter the condition, diagnosis, etc. The unknown was extremely scary. It was suspected that she could possibly have Turners Syndrome, Noonan's Syndrome, DiGeorge's Syndrome or Down Syndrome. They weren't sure if she had Heterotaxy and since its very rare (see stats above) the other possibilities were more likely. I did not do an amino until week 29. I had to for my own sanity. I had some very dark days during my pregnancy because I couldn't imagine carrying a child and not knowing if she would survive or what could possibly be wrong with her. The amino came back negative for everything. So it was safe to assume Heterotaxy (cannot be tested by amino). Nothing was certain until her birth.

Zoe was born with Interrupted IVC, Double SVC, ASD, VSD, Duodenal Atresia (intestinal blockage) and polysplenic (multiple spleens). Her heart is midline (in the middle of her chest). She is on penicillin twice a day until she's the age of 5 to help fight off viruses since her spleens do not work.  She spent 32 days of life in the NICU. She underwent duodenectomy surgery at 5 days old to fix her intestinal blockage. She did not eat for 3 weeks. Eating was a struggle at first, but she grasped on pretty quickly.

Due to being in the NICU for an extended period of time, torticollis and plagiocephaly happened. She only favored the right side and would not turn to the left. So we started Physical Therapy at 8 weeks old to help stretch out her neck muscles and work on tummy time to help her misshaped head. Unfortunately, physical therapy couldn't fix this, and she had to wear a cranial band for 6 months. 

As for Zoe's heart, she had open heart surgery on September 25, 2012 to repair her small ASD and large VSD (8mm).  She has a residual tricuspid valve leak, and will be monitored.  As for her Interrupted IVC and Double SVC (basically weird anomalies to her heart) they do not intend on fixing and rerouting the veins (Interrupted IVC) to the proper chamber or taking the extra vessel out (Double SVC).

Despite all of her issues, she is the happiest baby, always has a smile on her face.  We just have an uphill battle to go regarding her immunity system.  We’ve overcome so much so far, and we will continue to do so.  

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