Ave's Story

Blogged this for CHD week. Wanted to put it here too in case the other blog ever "disappears".

---

Diagnosing Avery:

I was out for hours as result of the anaesthesia (I have low blood pressure so that’s my “normal”) so my husband spent the first few hours cuddling with Avery all alone. While she had excellent initial Apgar scores and was quite tranquil (unlike a lot of the other babies that night!), at about the third hour, my husband then noticed that Avery’s breathing seemed a little laboured/congested. The nurses also determined that she was starting to turn blue so they then did a reassessment where they found her to have low oxygen levels. Initially, they placed her in an oxygen tent which had little effect. Then, and since she was slightly premature, the nurses gave her a nitrogen blast (in hopes of opening up the alveoli to boost her levels).

Given that neither of these two treatments mollified the situation, it pointed to a cardiac versus a respiratory issue. The residents then performed an echocardiogram which proved that Avery had transposition of the great arteries. In this situation, the pulmonary arteries and the aorta are physiologically reversed. As shown in the image below, the oxygen-poor blood that comes back from the veins merely travels in a circle through the heart and never heads to the lungs to get re-oxygenated. Without repair, this defect is always fatal (in most cases, 95% of infants who do not have surgical intervention die before their first birthday).

---

Treatment Begins:

Calgary and Edmonton have some excellent pediatric cardiologists (particularly in the area of catheter procedures). We were at the Rockyview  so both she and I were prepped for transfer to the Foothills (since it is all closely entwined with the Children’s Hospital and University training programs). Since I was still drifting in and out of consciousness, I found it hard to concentrate on what I was being told early on. It was at this time I first saw Avery…strapped to a full stretcher and with any number of machines connected to her, I turned to mush.

After arriving at the Foothills and being prepped, and at a mere eight hours old, Avery had her first surgery…a catheter procedure called a balloon atrial septostomy. Going in through the umbilicus, a catheter guides a balloon to the foramen ovale. That ovale is a hole in the heart that allows for blood mixing while an infant is in utero. Once a baby is born, the hole closes since, in a normal heart, blood mixing is no longer needed. As a way to keep Avery’s oxygen saturation levels high enough until she could have open heart surgery, the pediatric cardiologists inserted a balloon in that hole to keep it open and allow for some mixing of the oxygen-rich and oxygen-poor blood. The surgery lasted about ninety minutes and improved Avery’s oxygen saturation to 85%. Overnight, she recovers well, is stabilized and then prepped for transport.

---

Winging it to Edmonton…

I had been up most of the night (walking the halls…oh the joy of a c-section!) but finally, in the hour before she left, I got to talk to her, hold her hand for the first time and generally confer with all the NICU doctors and nurses on her condition, transport and stabilization once she was brought to Edmonton. And then off she went with a resignation on my part and an amped up desire to get out of the hospital and make the three hour drive to be at her bedside.

Due to our vast geography and relatively small population, health specialities are regionalized (in an effort to centralize expertise and control expenditures). As such all cardiac pediatric cases are dealt with in Edmonton at the Stollery Children’s Hospital. As such, you could see patients in the NICU from as far east as northeast Ontario, as far west as the interior of BC and also from most parts of the Yukon, Nunavut and Northwest Territories. So on the morning immediately after Avery’s birth and first surgery, she was prepped for transport. Now I knew about STARS, but I did not know about the jets they use to do inter-hospital transfers! There were four to six attendants with Avery to monitor all her machines and ensure her respiratory system would handle the changes in pressure as a result of the flight. They just amazed me with their knowledge and reassurance.

---

Getting to Edmonton:

Avery made it to the hospital in Edmonton and was successfully re-assessed and stabilized. Since her oxygen levels were good (~85%), they were able to give her a bath and hold off on immediate surgery. This turns out to be quite beneficial for me since I pop a staple and my c-section scar refuses to stop bleeding. Given the short-staffing of the holidays, and my two older kids being saddled with the flu, it wasn’t until December 27th that we were able to make it to her bedside.

---

Talking to the doctors:

In the world of CHDs, Avery had a rather straightforward and repairable defect (TGA). Her surgeon tells me that he has a 98% success rate (the 2% of children who did not survive had other complications besides the CHD). He is quite confident (almost blase given the number of these procedures he had performed) and it rubbed off on me as I remained relatively nonplussed by what is about to happen. (Note: Here is where I may caution you as to the message boards on the Internet [see links below]. The Internet is an invaluable tool in educating yourself on your child’s diagnosis. However, personalizing the experience on message boards can be tricky as misinformation and traumatizing stories abound. I was almost “lucky” to not find the message boards until after Avery had her surgery as I am sure my faith in the hospital, doctor and procedure would have been shaken and left me quite unnerved.)

He does warn me that given the holidays, as well as the number of babies brought into the NICU over the past week, that Avery would not be priority one in getting her surgery. Nonetheless, he promised me that she would get the repair within the two-week period he outlined as ideal.

---

Bumped and “your baby is very sick”:

As a forewarning to any parent stuck in the NICU, here are two things to remember a) nothing goes according to plan and b) the days drag when you are sitting next to a layette with a sedated baby.

Avery was scheduled for surgery on December 30th. She was bumped due to a baby who required a heart transplant (quite a few parents who had babies with hypoplastic left heart were opting for a heart transplant versus the three complicated surgeries typically performed). Since she was going to be delayed, the doctors elected to extubate her (remove her breathing tubes). Since Avery was no longer sedated, she began to flail wildly and pull out the rest of her tubes (she was a feisty baby).  Learning that she was not about to be undaunted, it wasn’t long before Avery was again sedated and intubated. These procedures are not at all fun to watch so they typically will tell you to leave when they need to do it.

She was then scheduled again for her repair on January 5th. As one of the requirements of the surgery, she needed an IV in the groin. It was late on the evening of the 4th so we went to dinner. We came back and all hell had broken loose with Avery. She was as white as a sheet with splotchy, spiderweb-like markings all over her body. All her other vitals seemed to be OK (save her blood sugar which was super high). Her layette was crowded by three nurses, the NICU doctor on call, a radiologist (taking another echocardiogram), an infection specialist, etc. They didn’t know what was wrong with her but only told me “your baby is very sick”. At the time, I didn’t appreciate what this coded language meant…it was only later that I realized it meant “your baby could die”.

I was never really freaked out before but this scared me. I kept thinking that had she already had the surgery, we wouldn’t be going through this. It has only been a week and a half but I felt like I had lived a lifetime in that period.

As the hours progressed, her condition seemed to better (especially since she was pumped full of antibiotics immediately). It was later determined that she had become septic as a result of the IV insertion and some pre-existing bacteria in her system. Needless to say, she was bumped again from having her surgery.

After recovering from sepsis, Avery was scheduled to have surgery on January 7th. Once again, she was bumped due to a child who had complications with his/her artificial heart. It was now getting close to that two-week date (i.e., the optimal window for her CHD repair) and I was as anxious as I had ever been in seeing that Avery get her surgery (I did not want her to get sick again!). She was rescheduled for the 8th when she would finally receive her life-saving procedure.

---

Open heart surgery:

On January 8th, exactly two weeks after birth, the surgeon at Edmonton’s Stollery Hospital, performed an arterial switch (also know as the Jantene) on Avery. In this procedure they switch the pulmonary artery and aorta back to their intended positions on the heart. The trickier part of the procedure was switching the cardiac arteries from one side to the other (since they each perform different functions for each side of the heart) since they are only the size of fishing line. Avery was cooled and put on bypass for this procedure (which would play a part in a later post-surgical diagnosis) but she nonetheless sailed through the surgery with no complications. Moreover, they were able to close her chest immediately (in smaller babies, they do not stitch up the baby until the next day in order to allow the swelling to subside), remove her pacemaker leads within eight hours and get rid of the chest tube the next day. The nurses told me she had the fastest recovery of any baby up to that date with the same repair (what a relief!).

---

Coming back to Calgary:

A mere four days after her surgery, Avery was off of all her heart medications and jetted back to the Alberta Children’s Hospital to a) get off the morphine (she was addicted!) and b) learn to drink from a bottle (I did not know that babies lose their suckling reflex if they do not use it within the first few days of birth). It would take a long two weeks for her to gain enough weight to be released and I am sure the nurses where happy to see us go since Avery was only happy “in arms” (thank goodness for all the volunteers at ACH!).

(Note: Cardiac babies can be real sleepy and not take in enough nourishment so it is a real challenge to get them “on the program”. They could have sent Avery home with a gavage tube but I really didn’t want to have to deal with that so I was grateful when she slowly but surely figured it out.)

---

Note: If you are a CHD parent, I have found the following resources to be quite useful:

• http://community.babycenter.com/groups/a5055/babies_and_children_with_heart_problems
• One in One Hundred
• chd babies
• chdspeaks.org
• The Congenital Heart Surgery Video Project
• Facebook groups:  C.H.D Awareness – Congenital Heart Disease and Transposition of the Great Arteries

---

Coming home:

Avery was brought home on January 25th, exactly one month after her birth. I would have to pump her milk and fortify it with formula but save that there were no special directives required for her care. She grew slowly but surely with regular appointments at the family doctor and clinic (for vaccinations and weight checks), but we would have no other interventions until she was six months old.

---

Our second home:

As part of the post-operative care of Avery, she visited the cardiac clinic for a follow-up appointment at the age of six months. She had a murmur still (since the hole created by the balloon septostomy had not closed) as well as mild pulmonary stenosis. Save annual checkups (where they would check to ensure that her arteries were growing with her), they told me she was doing very well and that no additional therapies/medications would be necessary.

In addition to her typical care, the Alberta Children's Hospital's Perinatal Follow-Up Clinic manages care for children who are premature and/or those who experience invasive therapies. In this clinic, the child is followed by an assigned pediatrician and several nurses. This group will regularly check the child until 18 months of age and then do an additional review at the age of 4 1/2. Should they perceive any development issues with a child, they can refer to other clinics throughout the hospital.

While lucking out in her cardiac post-operative diagnosis, we spent a great deal of time in the perinatal clinic dealing with various minor issues. She worked with an occupational therapist to aid in eating (pinching and grabbing). She had hearing tests due to her relative lack of responsiveness (even to me) at the age of six and twelve months (she passed). As we plugged on, it was becoming apparent that Avery was not hitting her marks when it came to gross motor movement. She did not sit until the age of sixteen months and did not walk until twenty-two months so she was assigned to a physiotherapist. In addition, she was assigned to a speech therapist since she was testing at the age of a nine month old for expression and reception. In the end, this confluence of factors would end up with a diagnosis.

On her eighteen month follow-up, Avery was given a diagnosis of cerebral palsy (mild and only on her right side). Believing it to be due to an oxygen depletion which caused her brain injury, the pediatrician concluded that it was probably due to the time spent on the bypass machine during her open heart surgery. We have yet to make it to the neurology clinic (we have been on the wait list for eight months at this point since Avery's condition is mild) so the causes are still (and may forever be) undetermined. Wait lists seem to be the norm for the more specialized clinics of neurology and opthamology (Avery's right eye tends to wander which requires a visit).

---

Presently:

Avery is now two. Her heart condition steadily improves even if she is still a little smaller than they would like. To keep up with her therapies, she will be going to special needs preschool in the fall (where she will have to go five afternoons a week!). She still doesn't sleep through the night (didn't figure out any self-soothing strategies as a baby thanks to her time in the hospital and an overindulgent mama!) but save that, she is a real trooper with a smile always on her face!

Note: Alberta Learning provides funding for early intervention from ages three to six (if you need more information, click here). The funding is particularly driven by speech delays so if you think you might have a child that would benefit, get them assessed by a speech language pathologist...better now than when they get into elementary school when there is no longer the resources available!