Ava’s 22 Hours

Paul brought me into Ava’s room to give me a brief explanation of why the doctors weren’t able to attempt Ava’s surgery and why she wasn’t going to make it. I called Kristin Spyker and asked her to help me get in contact with Stanford or Boston for a transfer. I had already called her from Kaiser while I was waiting on an update and she already knew something was going wrong. Shortly after an older CHLA cardiology doctor came in and gave me yet another brief explanation of why Ava couldn’t be helped. I immediately demanded to have Ava transferred to Stanford. He looked me in the eye and said that no surgeon on earth could successfully repair Ava’s defect. I simply replied with, “I need you to request a transfer now”; and the doctor walked away rolling his eyes.

My family had decided it was time for them to leave. Some thought I had lost it, others wanted to give me time and space. From the bathroom I could hear someone whisper, “She’s having a mental breakdown.” At times when I tried to contact Kristen I was treated like I was making a mistake. When I went to look at a text from Kristen Paul even said, “Is that your little Kristen” and another family member literally forced me off a phone call with her. My sole intent in these moments was to try any avenue I could to save my daughter. Sadly, this is a common occurrence in situations such as this. As emotions are strained, stress is high and confusion is everywhere it’s common for family to misunderstand and get angry with each other. Some family members were nothing but supportive while others had moments when they weren’t. I’ve spoken with multiple women who’ve had similar reactions from their spouses and family.

Hours passed before I was finally able to ask the charge nurse if the transfer request was sent. Paul asked where she was multiple times and we received no feedback. Ava’s surgeon was also not able to come talk to me on the night of the first. Despite the comment Paul had made about Kristen he was very determined to try to get a transfer if that meant saving Ava. When the charge nurse finally returned she said they spoke directly to Dr. Hanely at Stanford and that Dr. Hanely himself thought Ava was not stable enough for transfer but would not comment on whether or not he was capable of performing the surgery needed. I contacted Kristen again to give her the bad news. She had already been cycling through some connections to see what other options we had for Ava. Next we would try getting her to Boston. This was our last and only hope.

Kristen tried her hardest to get me in contact with Boston and although Paul had faxed Ava’s records we simply didn’t have enough time to wait for a response. Ava’s health was deteriorating quickly because of her pulmonary veins. When I first arrived at the hospital on the night of the first she was able to turn her head and look up at me with her big brown eyes. But by the morning of the second she was unresponsive in every way and frequently gasping for air, although she had a machine breathing for her. Her breathing machine sounded an alarm about every 15 minutes because her lungs continuously filled with fluid. It was very painful to watch but we held onto the hope of hearing from Boston Children’s Hospital.

Then 2:00 PM came and the machines were not enough to keep Ava alive any longer. Ava had already been resuscitated once during her transport from Kaiser to CHLA and we decided we wouldn’t have it done a second time. A Chaplin was provided and we were able to get locks of Ava’s hair, footprints, and hand prints. We called family to let them know they could come say goodbye. Ava’s health began to worsen very quickly. As we waiting for signs that she wasn’t going to stabilize we continued to kiss her forehead, play with her hair, and hold her little hands and feet. Once our family arrived it was apparent that we had only minutes, she wasn’t recovering and the doctors were not going to try anymore than they had. Ava was removed from life support and I held her in my arms as she passed. This took only moments.

This post has taken me such a long time to write because for the longest time I felt like I had failed and I was ashamed to admit it. I had one opportunity to make a good choice as Ava’s mom and that was when I chose the hospital Ava was to be delivered at. I know now that once a Heterotaxy child is born into a hospital the fate of that child lies in the hands of the doctors. Although there are instances in which parents have more time to fight for transfers this is often not a possibility. What else could I have done but chosen the best hospital for her? That was really the only way I could have given her the best care because I wasn’t the one who was capable of providing her needed care. In earlier blogs I mention that CHLA and Kaiser aren’t necessarily bad hospitals but that they weren’t right for Ava. This fact is still true but I’m going to explain how ugly this fact can be for Heterotaxy patients.

I mentioned earlier in this post that I was told Standford was contacted, specifically Dr. Hanely. After Ava’s death it took months and request letters from a lawyer to receive Ava’s medical records from CHLA. Once I received the records I was astonished to find that no transfer request was completed or recorded. Her records made no mention of my request to have Ava transferred to Stanford or the supposed phone conversation had with Dr. Hanely. Her records simply said that a transfer request for another hospital was made but due to Ava’s severe condition transfer was not a possibility. This isn’t entirely untrue, Ava wasn’t in good condition, but I know now that more could have been done for Ava.

CHLA made no mention of ECMO or other methods of stabilizing Ava for transfer. It wasn’t until after Ava died that I learned from other healthcare professionals that more could have been done. Not only could more have been done to stabilize Ava but the records lead me to believe that no actual request for transfer was made. The doctor I initially requested a transfer from rolled his eyes at me while I stood next to my dying daughter. Last but not least I was assigned a social worker who callously made jokes in my CICU room about how, “These things happen”. CHLA had given up on Ava before I even entered the building. The only thing I could have done differently in that situation was deliver at the most experienced hospital.

I share my egregious story in hopes that if you’re on the fence about which hospital to choose, like I was, that you’ll choose only the best available. Although delivering at the best hospital available wont ensure that your baby live a long life or an easy life. In those critical moments, minutes, or hours after a Heterotaxy baby is born family will not understand, you will be confused and given little information, and un-experienced doctors will be just as confused as everyone else. How scary is that? A doctor who knows little about this complex specialized diagnosis, who is learning as they go, using your baby as a test subject, and has fears(as Ava’s surgeon did) that this confusing baby will likely die under their knife. Despite the uncertainty of a Heterotaxy babies length of life or quality of life I would do this all over again to choose a better hospital for Ava. Even if that meant only a little more time with her.

In the morning of the second, the day Ava died, I was able to have a short meeting with her surgeon. His eyes were red and as he gave me the Heterotaxy 101 speech with what seemed to be tears in his eyes I realized he was scared and lost. He told me he wasn’t going to operate because he didn’t want her to die under his knife. It’s hard to have someone to “blame” for Ava’s death. Were the pediatric cardiologists, perinatologists, and surgeons negligent because they didn’t refer me to a more experienced hospital and team of doctors? This idea goes against most hospital policies and practices because hospitals are businesses. Hospitals punish doctors for outside referrals. Dr. Montgomery admitted to me that he was reprimanded for giving me an outside referral for something as simple as a fetal echo.

Does that mean hospitals are to blame for the ugly “business” aspect of how they run? Hospitals see Heterotaxy babies as dollar signs, bottom line. Or is it my fault for not being educated quickly enough to make the best choice for my daughter? My confusion came from lack of understanding. My choice was made because I didn’t know if traveling was safe and I didn’t know for sure if CHLA wasn’t capable. Now that I know I’ll never stop sharing in hopes that parents will be able to make more informed choices. At the end of the day I can replay Ava’s story over and over again in my head and I’ll never know “who’s to blame”. To this day I have unanswered questions and I’m forced to live with this unknown forever.

I would like to leave you with the few positives I experienced at CHLA. Once a child dies it’s important to find a silver lining, a cause to live for, and to let go of blame so that you can be at peace. For my own peace of mind I share what I know but I try not to dwell in these moments. I prefer to remember the nurse who watched over Ava on the night of the first. She was a young woman who cared. Her attentive, gentle work will never be forgotten. This young woman even helped me change Ava’s diaper a few times. The Chaplin was very kind and sympathetic. As Ava was passing the nurses in her room were gentle and caring towards my entire family. One female nurse sang a lullaby and another male nurse sympathetically pronounced her death. The male nurse who had been watching over Ava on the second cleared the room quickly so we could have time alone to say our goodbyes, bathe Ava, and dress her for burial. These are the sad but peaceful “positives” I was able to take away from my experience at CHLA.

Free E Book: Remembering your child

Still Standing is an online magazine for baby loss parents and this morning they shared a free e book that I thought I should pass along. Remembering Your Child by Beth Morey empathizes with the difficulties of losing a child and suggests ways in which parents, friends, and family can remember lost children.

“The reality is that we don’t forget, move on, and have closure; but rather we honor, we remember, and incorporate our deceased children and siblings into our lives in a new way. In fact, keeping memories of your loved one alive in your mind and heart is an important part of your healing journey.” – Harriet Schiff

The Questions I Wish I Would’ve Asked Ava’s Doctors

If you’re at least halfway through your pregnancy and your doctors have diagnosed your baby with Heterotaxy then I would like to begin by saying, I’m so very very sorry. Heterotaxy is terrifying but you have already started making the right choices by choosing to do research and educating yourself. While I was pregnant with Ava I trusted her doctors and it wasn’t until after she died that I learned how important it is to thoroughly interview your perinatologist, pediatric cardiologist, nursing team, and surgeon. Most doctors are not experienced enough to care for a Heterotaxy child. The days and weeks after you give birth to your Heterotaxy baby are vital. You cannot take risks, transportation is often not an option, and waiting for an unexperienced team of doctors to figure out what needs to be done can cost you the life of your child. Here is a list of questions that can help you understand your child’s Heterotaxy and decide if you’re with the right team of doctors.

For your perinatologist and pediatric cardiologist:

  1. Can I please have a written, detailed list of the defects you are currently able to identify through fetal echocardiogram?
  2. What parts of the heart and body are affected?
  3. Does my child have two atriums and two ventricles?
  4. Are the walls/septums of the atriums and ventricles fully formed?
  5. How many valves are present in my child’s heart?
  6. Can you see blood traveling through these valves?
  7. Are the chambers in my child’s heart proportional to each other?
  8. Are you able to identify the aorta?
  9. Is there an appropriate amount of blood flow through the aorta?
  10. Where is the aorta located?
  11. What are the measurements, in diameter, of the aorta?
  12. Are you able to identify the superior vena cava(svc)?
  13. Is there an appropriate amount of blood flow through the superior vena cava(svc)?
  14. Where is the superior vena cava(svc) located?
  15. What are the measurements, in diameter, of the superior vena cava(svc)?
  16. Are you able to identify the inferior vena cava(ivc)?
  17. Is there an appropriate amount of blood flow through the inferior vena cava(ivc)?
  18. What are the measurements, in diameter, of the inferior vena cava(ivc)?
  19. Are you able to identify the pulmonary veins? *Pulmonary veins are difficult to identify inside the womb. An expert should be able to identify the veins in later weeks of the pregnancy. If your doctor cannot identify the pulmonary veins or wants to wait until after your child is born to address possible pulmonary vein issues, then you should consider finding a doctor who is more experienced.
  20. Is there an appropriate amount of blood flow through the pulmonary veins?
  21. Are the pulmonary veins in their appropriate positions?
  22. Are you able to identify the measurements, in diameter, of the pulmonary veins?
  23. Are you able to identify any obstructions of the great arteries and/or pulmonary veins?
  24. Are the other organs in my child’s body fully formed?
  25. Where is my child’s stomach, liver, lungs, and other organs?
  26. Has my child began to practice breathing?
  27. Do my child’s lungs have consistent inflation?
  28. Are the expansions of my child’s lungs appropriate?
  29. Have your perinatologist and pediatric cardiologist explain the flow of un-oxygenated(blue) and oxygenated(red) blood in your child’s heart. Your doctors should also be able to visually show you the blood flow using a doppler effect. Your doctors can also identify what organ the blood is traveling from using an audible function of the doppler effect.
  30. How often do you diagnose Heterotaxy children prenatally?
  31. After the children were born, how accurate was your prenatal diagnosis?
  32. How many children have you successfully provided continued care for after the first, second, third, fourth, fifth surgeries and/or heart transplants?
  33. What surgeries are you going to recommend for my child after birth? *Your doctors should be overly descriptive.
  34. What are the names of the surgeries? *Some surgeries, like those that involve complex pulmonary vein issues, have not yet been named but your doctor should be able to explain them. If they cannot explain the complex surgeries then you should find a more experienced doctor.
  35. At what ages do you expect my child will receive these surgeries?
  36. What are the chances of my child developing malrotation of the intestines? *More than likely your Heterotaxy child will develop malrotation of the intestines but you should still ask these questions to your doctors to ensure that they are well informed on the struggles Heterotaxy children face.
  37. What surgery will my child need to correct malrotation of the intestines? *Ladd’s procedure.
  38. At what age do you expect my child to need the Ladd’s procedure?
  39. What are the chances of my child having asplenia(absent spleen) or polysplenia(multiple spleens that might not function properly)? *Your Heterotaxy child will likely have one of the two and your doctors should be prepared for this. Lacking a spleen or the vital functions of the spleen greatly increases your Heterotaxy child’s chances of developing a deadly infection.
  40. What are the chances of my child needing a feeding tube?
  41. What risks are associated with feeding tubes versus the risks associated with feeding orally?
  42. What foods should I avoid, if any? What foods would promote healthy weight gain? *Heterotaxy children often have an extremely difficult time eating right and gaining weight. Talking to experienced mothers about what helped their children can help eliminate some of your trial & error process.
  43. What equipment should you have at home for your child? *You will want to have equipment such as a pulse oximeter at home to monitor your child’s blood/oxygen saturation levels. If your child is lacking oxygen in their blood they will have symptoms, such as turning blue-ish, but if you can address a change in your child’s health before the symptoms worsen your doctor will have more time to figure out what caused the decline in your child’s health. You should also consider having CPR(CPR certification required) equipment and a transportation bag(meaning toiletries, clothing, etc.) ready in case of emergencies.
  44. What symptoms should you watch out for? *Have your doctor list possible situations in which your child’s health declines at home; what symptoms will they have? What requires a concerned call to your pediatric cardiologist and what requires an emergency trip to the hospital?
  45. Ask for copies of EVERYTHING as soon as you are finished with an appointment. You should have copies of every detail pertaining to your child’s diagnosis as well as copies of your child’s fetal echocardiograms. Use these to get second and third opinions from separate, well known, experts on Heterotaxy(Boston Children’s Hospital, CHOP). Choosing the right team of doctors can mean the difference between life and death for your child. 

For your nursing team:

  1. How many Heterotaxy babies have you cared for?
  2. How is your CICU ranked on preventative care? *CICU(cardiothoracis intensive care units are made for children with heart defects. If your hospital does not have a CICU then you should choose a hospital that does have a CICU. Remember, you not only need someone who has years of experience with heart defects but you need experts on Heterotaxy.
  3. How is your CICU ranked on cleanliness?
  4. What is the CICU’s nurse to patient ratio?
  5. Does your attending physician stay attentive in your CICU?
  6. How many beds do you keep in a room?
  7. How often do patients develop infections in your care?
  8. Will I be allowed to observe my child and your nurses 24/7?
  9. What will I be allowed to feed my child? *Can you breastfeed or do you have to pump  your milk? Or will your baby need specialized formula or another feeding method?
  10. When will you be able to start feeding your child after birth? After surgeries/during recovery?

For your surgeon and pediatric cardiologist(Please insist that you meet your surgeon before your child is born):

  1. How many Heterotaxy children have you operated on? *A pediatric heart surgeon is like an all-star athlete. They have statistics and should know EXACTLY how many Heterotaxy patients they have seen. If your doctor doesn’t give you an exact/nearly exact number or says something vague like “many, a few, a couple, several” then you should find a surgeon who is experienced enough to be honest with you.
  2. Please describe what procedures/measures you will use should my child need a complex, emergency surgery on issue such as: Pulmonary Atresia(PA), Total Anomalous Pulmonary Venous Return(TAPVR or TAPVC), transposition of the great arteries(TGA), etc.
  3. What surgeries will you be performing on my child throughout his/her life? When do you expect my child will need each surgery?
  4. How successful have you been in the past performing these difficult surgeries on complex Heterotaxy children? *Your doctor should be specific, they should give you numbers of successful operations. When I say successful I mean that the child survived surgery, recovered, and continues to live.
  5. What year did you perform your first open heart surgery on a Heterotaxy child/how many years have you been handling cases of Heterotaxy?
  6. What team of doctors, nurses, and surgeons will be present in the operating room? *Once you hand your child over for surgery/sedation you will receive minimal updates as the doctors will be focusing on surgery. It’s important to be informed of what will happen in the operating room before your child gets there so you aren’t left in the dark.
  7. What are the possible outcomes of my child’s surgeries?
  8. How risky are these surgeries?
  9. Are there any other options other than the surgeries you have chosen?
  10. What surgeons helped you come to the conclusion that these surgeries would be best for my child? Why?
  11. What are the long term and short term affects of the surgeries you plan to perform?
  12. What methods of sedation do you plan to use?
  13. What other medications will be administered to my child? *Your doctors should be overly specific and should describe all possible medications that your child may receive. Heterotaxy children will need anti-biotics(without a functional spleen they have no immune system), a prostaglandin immediately after birth, pain medications,  etc.
  14. How will my child be affected by these medications both negatively and positively as well as in the long term?
  15. (After Birth) Have you completed a cardiac catheterization?
  16. (After Birth) Are there any changes to the diagnosis or were your initial findings correct?
  17. (After Birth) If there are changes to your child’s diagnosis after birth what exact changes will be made to the surgeries and treatments your child will receive?
  18. How much time do you expect my child will need to recover from surgery and pain medications?
  19. On average, how long does it take you and your team of doctors to extubate children during recovery?
  20. How often will you be available to check on my child’s recovery? *Hesitation or lack of physician involvement can lead to infection or Septicemia

Each baby is born with a unique set of complexities surrounding their heart defect. It’s important that you have a firm grasp on your child’s specific diagnosis and that you feel confident in your doctor’s ability to care for your child. Feel free to print these questions out for your doctor. Since each and every Heterotaxy child is unique you might have additional questions to ask. If your team of doctors are unwilling or unable to answer these questions then I urge you to take your child to a doctor who can and will be honest as well as informative. The Heterotaxy Network is a wonderful organization of mother’s with a wealth of knowledge and experience that can also help you figure out what doctors to trust and what additional questions to ask. Please leave a comment if there are any questions you feel I have missed.

Ava’s Transfer to CHLA

We were told that Ava had to have emergency surgery and was being transported to CHLA. This was all planned before her birth and they knew there was a possibility that she might need emergency surgery. I spoke with Dr. Kallin, Ava’s surgeon, over the phone and he said he would be at CHLA in 30 minutes. So, I ran out of Kaiser Sunset and left Alex with her family. My mom and I ran to CHLA’s CICU,  about two blocks down on the same street. CHLA already knew what was going on but they had us wait because Dr. Kallin was not there yet. Once Dr. Kallin got there I was told to meet him. The area of the CICU I was lead through was like a labyrinth, I assumed I was where surgeries were performed. I was taken to a secondary waiting room while my mom waited in the main waiting room. Dr Kallin came and gave me a short rundown with two other hospital employees. Ava was still having tests and echos done and I was told the man performing these tests was the best at what he does.

Then I went back to wait with my mom in the main waiting room. We waited until Dr. Kallin called me and my mom into a private room. Dr. Kallin gave me more information about the surgeries to be performed, this is when I signed the release forms for surgery. He made a comment about not wanting to even begin discussing the possible brain damage Ava might have because they had a difficult time getting her across the street. They were still in the process of doing the tests and although they said the veins were small they had not yet told me that they couldn’t do the surgery. Dr. Kallin said that if I were to hear from him soon that it was bad news, that I didn’t want to hear from him until after the surgery. This was at about 7:30 P.M. I asked about survival rates but I didn’t know the right questions to ask so I got the wrong answers. I said that I hoped I didn’t hear from him.

I went downstairs and called Alex’s parents. I told them things weren’t looking good and asked them to not tell Alex yet because I was scared of her reaction. Her parents said they were going to come and speak to Dr. Kallin with me and left Alex with her sisters. I waited in the lobby downstairs while my mom waited upstairs in the CICU waiting room. Alex’s dad called and it sounded like they couldn’t find us. So, I went outside and saw Dr. Kallin speaking to Alex’s parents, grandparents, and my mom. He had found my mom upstairs and came outside with her to find me. That’s where Dr. Kallin explained to all of us that he was not capable of doing the surgery because Ava’s pulmonary veins were too small.

After that he took us all upstairs and we were hassled by security. Ava was still downstairs in an emergency area and we were waiting to see her in the CICU waiting room. Alex’s parents stayed until we all saw Ava. The social worker helped us get Alex an emergency discharge and counseled us on the best way to tell Alex about what was going on. After that Alex’s parents left to get her from the recovery room at Kaiser Sunset. As I waited for Alex I played with Ava’s hair and rubbed her head. When Alex came I met her in the hallway upstairs, she couldn’t walk, and I took her into Ava’s room to tell her everything I was told so far.

I Tri 4 You

This morning I came across this free ebook, When A Friend’s Baby Dies (Helping Your Friend After Babyloss) by  Kristine Brite McCormick who is the mother of Cora.  Cora was only five days old when she died.  (You can learn more about Cora and Kristine on Kristine’s blog, Cora’s Story.)

I agree with Kristine that “Every mother is different.  We all grieve differently.”  (Kristine also talks about fathers in her ebook.)

Unfortunately, 150,000 infants, children, teenagers and young adults will die each year in the United States (according to The Compassionate Friends2011 Fact Sheet).  And more than 25,000 families will face a stillbirth and more than 900,000 an early pregnancy loss.  So at some point in your life you may need this book to help guide you as you support a friend or to share with your friends.  Perhaps you need this book now.

Thank you, Kristine…

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Labor and Delivery

I went in to begin induction at 39 weeks on August 30th, 2011 at about 8:00 P.M. First Kaiser started me on Pitocin and my contractions started right away. Later I went through very painful induction methods that I would rather not mention. At first my contractions weren’t very painful or strong but by morning the next day I wanted medication. An epidural always sounded too risky, I didn’t like the thought of having an IV in my back. Morphine was given instead and I wouldn’t recommend it to anyone. I wouldn’t recommend morphine because your baby will feel it. It scared me because after they gave it to me Ava stopped moving. At about 4:00 A.M. on September 1st I finally asked for the epidural and it was everything I needed.

Beth, my step mom, and Paul stayed the entire 2 days with me in the labor and delivery room. Kaiser had one chair that extended into a bed but it didn’t look comfortable at all. Beth let Paul sleep because she knew that once Ava was born Paul would need energy. Paul was so sweet to me the entire time. If I was in any pain he was there holding my hand and playing with my hair. He would even jump up from his sleep to help me carry my IV to the bathroom. It was in the labor and delivery room that my family saw just how strong the love between Paul and I had become. Ava made us a family.

Thanks to the epidural I had a very nice birthing experience. I’m not trying to say it didn’t hurt but I had a kidney stone once and that hurt worse. After two days of waiting it took about 45 minutes for Ava to make her appearance outside of the belly. There was a point in time where I wasn’t dilating fast enough and the doctors started talking about a c-section. But in the last 30 minutes I dilated to 10 and was able to have a more natural birth. I wish I could remember the name of the doctor who delivered Ava but I can’t. Unfortunately she was training another doctor on me and after birth they couldn’t find a needle. Don’t worry, they just dropped it on the floor.

Ava was blue-ish, adorable, crying, and had her gorgeous head of hair. That was the glimpse I caught of her as the doctors carried her to the incubator and formed a circle around her. I loved the sound of her crying, it was the only time I ever heard her cry. But everyone could tell she was having difficulty breathing and they rushed her away to the NICU. Paul followed Ava while I waited to regain feeling in my legs. Paul came back into the room about 20 minutes later and said that Dr. Salem kicked him out of the NICU while he did an echo on Ava.

The Week Before Induction

August 22nd, 2011 was the first time I spoke with Kristen Spyker, a Heterotaxy mom. Immediately she began to talk about “top heart centers” and how important it is to get Ava to the right team of doctors. I contacted her because I wanted to know what life would be like with Ava when I brought her home. It never occurred to me that CHLA wouldn’t be experienced enough to care for Ava. CHLA is a reputable hospital but Kristen opened my eyes to more than enough red flags. That week was a panic and I managed to make every wrong decision.

I’ll begin by listing the red flags. First, It was about a week before induction and I still had not had a face to face interview with Ava’s surgeon; I didn’t even know his name. Second, It is beyond unrealistic to tell a patient that a Heterotaxy baby is capable of recovering in two weeks. Third, pulmonary veins are a big deal. Fourth, when I tried to get a second opinion Kaiser wouldn’t give me Ava’s fetal echos. Fifth, Kaiser discouraged me from getting a second opinion. Sixth, Children’s Hospital of Los Angeles is only ranked the 11th best hospital in the U.S.

After my conversation with Kristen I called Dr. Salem and Dr. Sklanksy who told me that Dr. Kris Kallin would be operating on Ava after birth. There was some confusion because I didn’t know Dr. Kallin had access to e mails I sent other doctors but after I threatened to transfer to Stanford Dr. Kallin did call me. Both Dr. Salem and Dr. Kallin explained to me that Dr. Kallin was a Kaiser employee who operated at CHLA and worked side-by-side with their team. Because of Dr. Kallin’s busy schedule he is not notified of surgeries until the doctors deem it “necessary”. Possibly a week before needed surgery or last minute in emergency situations. When I spoke with Dr. Kallin I was nervous and stressed, I didn’t ask enough questions. He told me his background; he had been a pediatric heart surgeon for about 12 years, used to work in Texas, but had only seen a few patients like Ava.

Heterotaxy is so rare and so complex that you need to interview your pediatric cardiologists and surgeons thoroughly. It’s also very important to interview more than one team of doctors. Sometimes that may seem unrealistic because of finances, jobs, and long distance commutes but Heterotaxy children have the best results at the top 5 heart centers. I wasn’t able to tell that Ava’s doctors wouldn’t know what to do from what they were telling me. At the time I didn’t know what questions to ask. If I would’ve asked the right questions I would’ve known not to delivered at Kaiser. In another post I’ll list all of the questions I wish I would’ve asked Ava’s doctors.

I should’ve been told about what would be done if the pulmonary veins had issues and names of surgeries to correct pulmonary vein issues. They also should’ve been more specific about what “issues” could possibly be present after birth. In my opinion, meetings with the the surgeon should be mandatory. Heart surgeons are extremely busy and I understand that. If a surgeon gets caught up in surgery with another patient, a phone interview should be arranged. Doctors need to tell their patients everything they know and get them involved.

When I tried requesting Ava’s fetal echos from Kaiser they didn’t want to give them to me. One of the nurses told me to be careful who I speak to about Ava, don’t believe everything I read on the internet, and that getting a second opinion really isn’t necessary. Thankfully UCLA had no problem sending me a copy of the fetal echos done by Dr. Sklanksy. I was trying to send the fetal echos to Dr. Hanely at Stanford, unfortunately he was on vacation and didn’t return until after induction.

Dr. Hanely’s colleague, Dr. Reddy, reviewed Ava’s fetal echo report. His assistant called me back and said that he agreed with the report and the procedures to be done. I asked if she thought Ava’s doctors were capable of handling Ava’s complex case and she said yes. I still wasn’t comfortable with delivering at Kaiser Sunset but after speaking with Stanford I already had my mind made up. I was going to deliver at Kaiser Sunset and Ava’s first surgery would be done at the Children’s hospital of Los Angeles. If anything were to go wrong I would just have her transfered to Stanford.

Kristen Spyker advised against this, she said that transportation is not always an option, she told me that she has seen Heterotaxy babies die this way, and that I should just drive up to Stanford and deliver there. I was almost 39 weeks pregnant and I wasn’t sure if driving eight hours and showing up to a hospital I had never been to before was a good idea. We were all expecting Ava to come before the induction because I was getting so many Braxton Hicks. This wasn’t an easy choice to make and I asked literally everyone I knew what I should do.

I asked my Facebook friends and people told me that CHLA is  a good place for surgery. My family was e mailed every piece of new information I got and they were very supportive of whatever choice I was going to make. They also thought that CHLA was capable but they would’ve supported the drive to Stanford too. Then I contacted Dr. Sklanksy at UCLA to see what he thought about the choice between CHLA and Stanford. Dr. Sklanksy said he used to work at CHLA and he was confident they would take good care of Ava.

Is CHLA a bad hospital? No, it’s actually a good hospital, ok reputation, it has a cardio thoracic intensive care unit, and the doctors have years of open heart surgery experience. Is Kaiser a bad hospital? No, every hospital has flaws but Kaiser is a good hospital. These hospitals were not right for Ava. Heterotaxy babies are so complex that you need experts on Heterotaxy. About 16 babies are born per year with Heterotaxy and many die because there are only a few doctors who really know how to care for them. Heterotaxy is so extremely complex that children can struggle or pass away even with the #1 doctors in the country.

BT Shunt, Glenn, Fontan, & Ladds Procedure

The BT shunt would’ve been part of Ava’s first needed surgery. She also would have needed very complex surgery for her pulmonary veins. Some surgeons at hospitals like Boston Children’s Hospital, CHOP, etc. could have handled it but most hospitals wouldn’t know what to do with a baby like Ava. Here is an overview of the BT Shunt. Ava would have gotten the Shunt within 10 days of birth, the Glenn around six months, and the Fontan around one to two years of age.

I can’t pretend to fully understand these procedures but articles and videos are helpful when trying to understand the Glenn and Fontan procedures.

From what I have been told by mom’s and doctors almost all Heterotaxy babies like Ava have malrotation of the intestines. If the malrotation causes obstruction of the bowels then a procedure called Ladds is needed. The Ladds procedure is performed when needed and usually happens before the Glenn procedure, correct me if I am wrong.

I found these videos from Kristen Spiker’s blog about her experiences with Heterotaxy and her son Logan, a Heterotaxy warrior. It wasn’t until I read Kristen’s blog that I actually knew the names of the surgeries Ava was going to need. I’ll tell you all about the hectic 9 days before induction when Kristen was trying to help me in another post, it was a long week.

Dr. Friedrich & Dr. Salem

June 20th, 2011 was the first day I met with Dr. Friedrich and Dr. Salem from Kaiser Sunset in Los Angeles. Dr. Friedrich was our perinatologist and Dr. Salem was going to be one of Ava’s Pediatric Cardiologists. When we arrived Dr. Friedrich did a two hour long fetal echo while Dr. Salem watched and discussed issues with Dr. Friedrich. They agreed with the current diagnosis. This was a very important meeting because these were the doctors who would actually be caring for Ava after birth.

Dr. Salem said he thought he might have seen Ava’s pulmonary veins and was not that concerned with them. After the fetal echo Dr. Friedrich and Dr. Salem discussed everything with me and Paul. It wasn’t anything we hadn’t heard before and we thought we had as much information as we could have about treatment. The doctors said we wouldn’t be able to get the last 10% of Ava’s diagnosis until after she was born. Her doctors seemed really confident in their ability to care for Ava.

They also discussed the bt shunt, glenn, and fontan with us but not by name. During this meeting we had the various anti-biotics explained to us and medication called Prostoglandin. I don’t remember if it was described by name but they described the function to me. Dr. Salem told us that Ava would need her first surgery, the bt shunt, within about 10 days after birth.

He wasn’t really concerned with her pulmonary veins and I had no idea what kind of care she would need if she had pulmonary vein issues except “emergency surgery”. I asked Dr. Salem how often they saw patients like Ava and he said they recently had a baby born like Ava. He said that the surgery was successful and that the baby went home within about two weeks. I was told that Ava would not be released until she was fully recovered and that could take anywhere from two weeks to a couple months.

Right before my second appointment with Dr. Friedrich on August 3rd RSCR Inland cancelled my health insurance. I was hassled for having medi-cal at the front desk, they almost wouldn’t see me. The meeting with Dr. Friedrich lasted about an hour and everything was the same but she said she was concerned that the pulmonary veins would need surgery. Ava was a big baby, with lots of hair that was visible on ultrasound. At the end of the appointment we discussed Ava’s birth.

I live about 2 hours away from the Children’s Hospital of Los Angeles so I couldn’t go into labor and drive. I was scheduled to begin induction on August 30th, 2011. I wouldn’t see Dr. Friedrich or Dr. Salem again until Ava’s birth. I was pretty confident with the information I had so far but I knew I didn’t know everything. Through babycenter.com I asked if there were any mothers of babies like Ava and what life was like after the babies come home from surgery. This is how I got in contact with Kristen Spyker, I wish I would have gotten in contact with her much sooner.

Dr. Sklanksy

My first meeting with Dr. Sklansky on June 2, 2011 felt like a breath of fresh air. I met Dr. Sklanksy before I met our doctors at Kaiser Sunset in LA. Before I met with Dr. Sklanksy, Dr. Montgomery did not have high expectations for Ava’s survival. Although Dr. Sklanksy agreed fully with Dr. Montgomery’s diagnosis he had a different bedside manner. He said it would be a struggle but that there wasn’t any reason she wouldn’t be able to be helped by surgery. Dr. Sklansky was the first to describe the BT shunt, Glenn, and the Fontan surgeries to me but not by name.

Dr. Sklanksy was impressed by Dr. Montgomery’s diagnosis but said he did not see any vertical confluence. He agreed that Ava would more than likely have asplenia and malrotation of the intestines. If she had malrotating intestines he said there are medications that can be taken and surgery if needed but he said it was nothing fatal. The asplenia was also not something he thought to be fatal but he said she would be dependent on anti-biotics. His biggest concern was that he was not able to see Ava’s pulmonary veins. If she had issues with her pulmonary veins, which she did, she would need emergency surgery.

I saw Dr. Sklanksy a second time on August 3rd, 2011. He was trying to schedule me for an MRI to see if he could get a better picture of Ava’s anatomy. Unfortunately there was a scheduling error and it never happened. He was very apologetic and tried to reschedule but it was too late in the pregnancy. I will post pictures of the fetal echo’s done by Dr. Sklansky but I need to upload them first. If you know how to upload fetal echos to a mac please let me know!