CHD awareness month: The Diagnosis

Sometimes I wonder if getting Evan’s diagnosis while I was still carrying him was a blessing or a curse.  I spent the last half my pregnancy in agony…worried about what may be…if he would make it.

But, reading about stories of those who were diagnosed after birth….the shock and heartbreak of thinking you had a “normal” child to now be a child with a lifelong disease…is agony in itself.

I have included 3 stories today….all three late diagnosis.

Finley’s Story

Finley was not diagnosed until 3 days old. He showed no symptoms except trouble keeping his temperature up. Thankfully his pediatrician heard a different toned murmur and requested an echo. Instead of taking our baby boy home we received the most devastating news of our lives! It was very shocking, I had at least 8 sonograms, they saw four chambers at our anatomy sonogram. Since he was diagnosed late, he had been allowed to eat. That is likely why he developed NEC (a serious infection of the intestines that causes the intestines to deteriorate).  Complications from NEC led to a emergency LADDS procedure (stomach and intestine surgery) only days after his first heart surgery and a four month stay in the NICU.

I am thankful everyday for our chosen pediatrician. Without her, Finley would have gone home and likely died from NEC. There is appointment thatgoes by with her that I don’t thank her for saving his life!



 Ellie's Story
 

Ellie was born, at home on January 30, 2012 sometime between 11:59pm and 12am. I got to pick her birthday since it was so close, and since I did all of the work on the 30th, her birthday was the 30th! We were told we had a healthy baby girl developing in my tummy so we went ahead with the home birth. I had a csection with my first daughter and was pretty mentally scarred from it. I wanted a VBAC so badly. And when I felt that I couldn’t get that with my OB, I found a very supportive midwife and birthed at home. It was an AMAZING experience. It was a LONG day. And it was the hardest thing I have done. I was in labor for 20 hours and I pushed for 3.5 hours. Ellie came out perfect. She was breathing, she nursed, I was mentally healed from my last birth and everything was just perfect.

Until we went to the pediatrician on Feb 3…

I was still on my birth high and was happy to show my new baby off. When the pedi came in and asked about her birth, I answered that she was born at home. He looked at me like I had done something wrong and asked if anyone was there with me. I explained that I had a Certified Nurse Midwife with me, so yes. He didn’t like the idea of it… AT. ALL. He asked me why I had a home birth and I told him because I didn’t want another csection. And he continued to try to make me feel like an irresponsible parent. He asked about her coloring while he was listening to her chest. I mentioned her race to him (white, Hawaiian, Portuguese, and Asian). He may have mentioned a murmur (which honestly didn’t really mean anything to me) and he said she looked blue. I didn’t see the blueness that he claimed and I thought he was just saying that because he didn’t trust my midwife and was mad we had a home birth.

He asked the nurse for their pulse ox machine and clipped it on Ellie’s hand. The readings were in the 80s and 90s. He clipped it on her foot and it was in the high 70s. He told us that that wasn’t good, but that his pulse ox was for a child, not a newborn. He wanted us to go to the ER just to get a pulse ox done on her. He said that it needed to be 93 and above. And I was like, “well, her hand was in the 80s, that’s close”. Seriously, I had no idea what it could mean. And he didn’t bother to tell us either. He just told us to go get the test, that it was no big deal.

It would have been nice to have a heads up… considering the fact that he later told us that he knew it was probably her heart. I’m still angry about it.

Since the pedi didn’t make the situation sound urgent, we packed Ellie up and went to get lunch at McDonalds. She nursed and was fine. We had to get my other daughter from my sister’s house as well. On the way to the hospital, I got a call from the pedi and they are YELLING at me, asking me WHY I wasn’t at the hospital yet. So I told them I had to get my other daughter and take her somewhere else (to a friend’s house), so she would be close to the hospital. They weren’t nice, but again… they didn’t mention ANYTHING about her being seriously sick.

I drop my daughter off and we go to the ER. The pedi had mentioned that we would just get this ONE test (the pulse ox) done and nothing else… we didn’t belong in the ER, but that is where they wanted us to go. We get back in a room and SIX people surround Ellie. They start trying to get an IV in her and I start crying. My husband spoke up. Nobody listened. The pedi at the hospital got there and wanted to see her on room air. The ER nurses weren’t listening and were trying to put an oxygen mask on my screaming daughter’s face. My 4 day old daughter. My perfectly healthy VBAC baby. Her sats were in the 70s and 80s. The hospital pedi told us it could be something with her heart or circulation. They did a chest Xray and called Children’s National Medical Center. She came back and told us that Children’s wanted to see her. Ok. And she mentioned that a helicopter was on its way. WHAT?!?!?! Ok, so I can ride with her… we will be ok…. Actually, no. You can’t ride with her.

WHAT?!?!

We were angry. My husband told them that we would just take Ellie and drive up to Children’s ourselves (it is a little over an hour from that hospital). It was ridiculous. They STILL didn’t mention how serious this could be. I still have a HUGE problem with that. They convinced us not too and said the helicopter was almost there anyways. And she would be receiving Prostoglandin. I didn’t even know why she was getting it. They probably explained, but I was preoccupied with the fact that they were going to take my baby away from me on a helicopter ride.

The transport team from Children’s got there and started to give her prostoglandin before they took her and FINALLY they explained that something serious could be wrong. We finally felt the urgency of the situation. My whole head was spinning. Everything had just been perfect…

The transport team leaves, with my brand new baby, and my husband and I carry an empty carseat out of the hospital. We went to get Abby (my other daughter) and we saw the helicopter take off. It was horrible.

We dropped my Abby off at home (my parents took care of her) and got a few things and booked it up to the hospital. I pumped in the car. I had no clue what I was doing, I had never pumped before.

We got a call from the transport team while we were driving and they reported that Ellie slept the whole ride and was fine. They were so sweet.

Then, as we were almost in DC, we got a call… I guess it was her cardiologist? She didn’t hesitate. She made sure I was Ellie’s mom and then said, “You daughter has a life threatening heart defect, Transposition of the Great Arteries, she will need surgery to fix it.”…………………………..

I felt like I couldn’t breathe, or hear, or speak, or see.

I had to have her repeat it… there was nothing wrong with my perfect VBAC baby.

She repeated it. I repeated it. And she proceeded to tell me that she had to have a cath procedure. They were prepping her for it now. And that they might start before we get there. We hung up and I lost it. LOST. IT. I could barely get the words out to my husband before I started sobbing. I couldn’t believe my little baby had to have heart surgery!

We got a call a few minutes later from someone else… I think the person who was going to do the cath. He explained the whole procedure to me. Every step. And I said, “ok” at the appropriate times, but I have no clue what he said. I didn’t even know what was wrong with her heart. I had never heard of that before. I didn’t know anything about heart defects.

We got to Children’s in enough time to see her before her procedure. It was terrible to see her little body lying on a tiny hospital bed hooked up to all kinds of wires. I was in shock.

We went to talk to someone during her procedure and he explained, with pictures, what was wrong with her heart. He answered our millions of questions. They were all so kind and patient. I’m sure they are used to parents in shock and crying.

Everything went ok from there. She had her open heart surgery, to perform the arterial switch on February 9th. We got to bring her back home on the 18th. There were only slight setbacks, like her taking a while to come off of oxygen, but we found out that she had fluid around her heart, so it kept us there a could of extra days. Other than that, nothing else has happened.

And now looking back, it all seems like a horrible nightmare. I still remember it, but it doesn’t feel real. My eyes have been opened to the fact that anything can happen to anyone. It is still painful to think about. I’m still emotional when I tell our story. But I realize how lucky we are. The pulse ox saved her life.

Ellie is now 1 and you would never know she was so close to being gone. I looked in the notes from the hospital and when she got to Children’s, her sats were in the 60s. A few more hours or 1 more day, and she would not have made it. We are SO glad she is still here with us. She is the happiest baby. And, I plan to have more home births. 

 

Ava’s story 

I have always been fascinated by birth. I’ve worked as a doula for over five years and attended births in multiple hospitals. I thought I knew just about everything there was to know about prenatal care, safe practices during labor and delivery as well as good postpartum care. But until my daughter was born in July 2011, I had never seen a newborn hooked up to a pulse/ox monitor. I had observed newborns weighed and measured. I had seen newborns receive their first bath, first blood draw and first shot. I’d seen nurses and pediatricians listen to breath and heart tones. But I had never seen a little bandaid-type strip wrapped around a tiny hand or foot to check the oxygen level in the new baby’s blood. In all my reading and research, it was never mentioned (at least to my recollection) that this was a screening that shouldn’t be skipped.

Thankfully, Ava was taken to the newborn nursery shortly after she was born to keep her warm while my OB finished up my c-section. Why am I thankful that my newborn was taken away from me? Why am I thankful that I missed out on critical bonding time? Because while she was in the nursery, her nurse decided to hook Ava up to a pulse/ox sensor. There wasn’t a “need” to. Ava’s APGARs were 8 and 9. She wasn’t showing signs of respiratory distress. But when that nurse, forever one of Ava’s guardian angels in my mind, hooked her up, Ava’s oxygen level was 54%. Definitely NOT enough oxygen, even in a newborn.

Why was Ava’s O2 level so low? Because she was born with a congenital heart defect.

It wasn’t caught during the level 2 ultrasound done at 23 weeks. In fact, it wasn’t caught during any of the 7, yes I said 7, ultrasounds done on Ava during my pregnancy. The screening that saved Ava’s life was having that little bandaid-type strip wrapped around her pink little foot.

There is a push from the CHD world, the Heartland as Czarina has so aptly named it, to have every newborn screened with pulse/oximetry. Too many babies are still being born with a congenital heart defect that wasn’t caught during pregnancy. Tragically, babies are still dying from undiagnosed, and undetected, CHDs. However the need for all babies to be screened with pulse/ox is not met with unanimous approval.

There are some birth proponents, especially in the homebirth world, that view most post-birth “routines” with a jaundiced eye. They feel as though those things can be harmful to the essential mother/baby bonding that occurs in those critical first hours. I get it. When I got pregnant with Ava, I explored the option of homebirth. I’ve always been an advocate for homebirth, especially if you have a low-risk pregnancy. I’m of the mindset that the majority of births can be safely attended either at home or a birthing center. I advocate for delayed first bath, newborn exams performed while baby is laying on mama and not getting weight and length right away.

So the first time I was met with resistance at my assertion that ALL babies receive the pulse/ox screen, I was taken aback. How could anyone think this was a bad idea? There’s nothing invasive about it. It doesn’t hurt the baby. The baby doesn’t need to be held in a certain position. Baby can stay snuggled up on mama’s chest while the sticky probe is put on a foot or toe or hand.

So why would anyone be against it?

Not every baby is going to have perfect O2 levels a few minutes after birth. Some are a little lazy and it takes time for them to get the hang of this breathing thing. Not every nurse or doctor is adept at getting the probe placed correctly on a wriggly foot. So the baby may present with a “false” positive. And here’s where the concern comes in. That this baby, who is perfectly fine, then gets taken away from it’s mama and sent to the NICU for further tests. For xrays, EKGs, ECHOs, etc. And the bonding window is slammed shut.

So how do we fix this problem?

How do we make sure that babies get to bond with their mamas but also get this crucial screen? Where’s the middle ground?

Frankly, I feel that if a homebirth midwife or a birthing center is willing to have a portable oxygen tank and vials of pitocin on hand at a delivery, just in case, then there should be little resistance to adding a pulse/oximetry machine to their equipment. Heart and breath tones are listened to with a stethoscope, so what is the harm in putting on a sensor to check O2 levels?

Will some babies be referred to their pediatrician needlessly? Maybe.

But how many babies will be saved?

I think that the biggest key to all of this, to satisfy both sides of this debate, is education.

Help birth attendants understand which pulse/oximetry machines are preferable for newborns. Explain proper placement of the probe.

One of the first things heart mamas learn is how to read a pulse/ox machine. Is the sensor picking up “junk”? Are the numbers good? Because for us, with babies that have lower O2 levels all the time, knowing if we can “trust” the number on the display is the difference between calling the on-call cardiologist and calling 911. So we can teach birth attendants the same thing.

Have a newborn giving funky O2 readings during the pulse/ox screen?

Ask the following questions:

-Is the probe placed well? Should it go on a toe or hand instead of a foot?

-Is the extremity warm? Should a sock go over the foot?

-Does the probe need to be changed?

-If the machine runs off battery power, is the battery charged?

Many times, if you are seeing “scary” numbers on the pulse/ox machine, one of the troubleshooting steps above fixes the problem and gets you a good reading. Determining a baseline for what’s considered a “bad” reading would also be helpful. Is an O2 level of less than 90% when measured 20-30 minutes after birth a problem? Or can it be as low as 85% during that time and still be ok? Should O2 levels be measured at predetermined times? For instance, 15 minutes, 1 hour and 24 hours after birth?

Pulse/oximetry screening for newborns doesn’t have to interrupt crucial bonding between mother and baby. If you are pregnant, or considering having a baby, I strongly advise that you speak to your care providers about pulse/oximetry screening. It is a simple, life-saving test.