Little Lila-Jean arrived on the evening of July 4th! I received a call from my doctor who had some concerns (too complicated for me, not a doctor, to explain), and gave me the option of having my water broken to induce labour. I was a little nervous to do this, but decided it was the best option for the safety of my little one. So, Dan and I headed into the hospital and Isabel and the dogs went for a sleepover at my parents house. My water was broken around 6pm, just after 9 I was holding a beautiful little baby girl in my arms! It was a very quick birth, no time for an epidural, and way, way, way, WAY more painful than I remembered, but thankfully my lady-parts survived unscathed, and I was surprised to be feeling pretty amazing by the next day!
Lila was born quickly and needed a little bit of oxygen after she was born, but otherwise looked perfect as can be. She has been a pretty chill baby since day one. She took easily to breastfeeding, she settles to sleep fairly easily (other than last night! Growth spurt time), and is a total little doll.
Everything seemed to be going swimmingly with the new baby. Isabel was adjusting nicely, and we were all starting to settle in, when we received a call from the Hospital for Sick Kids in Toronto… Every baby born is offered newborn testing for 29 (I believe) treatable illnesses. Lila screened positive for one I had never even heard of; VLCAD.
We had to head to Toronto bright and early the next day to have more testing done. I was feeling hopeful that she would come back not having this disorder. There was no history of it in our families as far as we knew and she seemed totally normal and healthy!
Lila had some blood drawn and then we waited the entire day in the lobby of Sick Kids, which is not a fun way to spend a day with a newborn… Around 5 we finally got the news we were afraid of… Lila does seem to have VLCAD, though her blood work shows that she may have a mild case. We were sent down to the emergency room to be admitted so we could start her on a special formula, and have her monitored over night.
As we were waiting in the ER to be admitted, I was given a bottle with Lila’s new formula in it. Up until this point, she had been exclusively breastfed, but she seemed to be doing ok with the bottle… that is until I noticed it was flowing a little too fast for her. I pulled the bottle away and went to burp her when she went completely limp and unresponsive. When you’re at sick kids, in the ER with an unresponsive infant the medical response is… quick and dramatic. We were rushed into a room where she was placed on a table, hooked to an IV and other monitoring equipment. She quickly regained consciousness, but it was the most terrifying moments of my life. I need to say, the staff at the hospital were all amazing, but that day was a very traumatic experience. Not only was I still hormonal after having only given birth a week or so prior, sleep deprived and dealing with the shock of an unexpected diagnosis, my baby… my brand new baby went floppy and unconscious before my eyes. They determined that the episode was likely unrelated to her VLCAD diagnosis, and most likely she “choked” (my word) on the formula. She’s been totally fine ever since, but it was so incredibly scary.
I’m not even going to try to explain VLCAD in my own words, so I’ll quote from the newborn screening website:
Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare, inherited (genetic) disease.
Babies with VLCAD cannot make certain fats into energy, especially during long periods without food (fasting).
Babies can get very sick if they cannot make fats into energy when needed.
Without treatment, minor illnesses and fasting can cause life-threatening episodes called metabolic crises.
We just recently got the results of Lila’s enzyme test which shows she does have the ability to breakdown some very long chain fats, but not entirely. The metabolic team we are working with consider her case mild, which I am so incredibly grateful for. We are still waiting for more genetic testing to be done to get more specific information.
There is no “cure” for VLCAD, but the treatment in our case is fairly simple on the surface. We just have to feed her every 2-3 hours (and we just go the ok to let her sleep 4 hours overnight between feeds). The tricky part is that we have to combination feed. She gets a small bottle of special formula, then breastfeeds. She tends to feel full-ish after a bottle so waits a bit before breastfeeding, so I often feel like I’m just feeding her around the clock. We have to keep a chart of all of her feeds. If she gets sick and can’t eat or keep food down, we have to take her to the hospital for iv fluids.
This diagnosis and the trips to Sick Kids has really taken a lot out of us. When she first was tested, we had to treat her as though she had a severe form of the illness… just in case! It all seemed so very serious. And it has the potential to be, but it is much more manageable than we had thought. As Lila gets older, she will be able to go longer and longer between meals. She will grow up a totally normal kid. We just will have to be cautious when she is sick, and make sure she has a snack before being active.
The world of metabolic disorders is all so new to me. We are so incredibly fortunate to be able to work with the metabolic team at Sick Kids in Toronto. They are absolutely amazing.
So… As you can see, it’s been a pretty hectic month. But, I feel like we’re starting to settle into a bit of a routine these days. I’m getting accustomed to the feeding schedule and juggling two kids (and two dogs… honestly the dogs are more work). Lila is seriously an amazing baby, she makes it pretty easy. And Isabel is such a helpful big sister, I’m not sure where I’d be without her… and Dan. Dan is in charge of formula and making sure I wake up to feed her at night and staying calm when I am losing it and assuring me everything will be fine.
To add just a little bit of extra drama to our already scary month, last Friday as I was walking into the house with the two girls, I tripped on a loose board on our walkway and fell while carrying Lila in her carseat. Isabel immediately freaked out because I had spilled the contents of the diaper bag. I freaked out because I had dropped my 3 week old baby, and Lila mostly just sat there and looked perplexed. Falling with a newborn, whether she’s buckled into a care seat or not, is pretty much the worst thing ever. She seemed totally ok, but I didn’t want to risk it. I took her into the after hours clinic near our house to get her checked out and of course she was totally fine… This visit made me realize though, that I need to be an expert on Lila’s condition. I mentioned to the doctor (who was amazing!) that she has VLCAD, and I had to explain it to her the best I could. No slight against the doctor of course, there is a reason why doctors specialize. It just was a reminder for me that what we are dealing with is rare and I have to make sure I am well educated so I can advocate for the best care for her.
And before you ask, yes, I am also ok! A little bruised, but Isabel fixed me up with some Finding Nemo bandaids.
I’m hoping that we got all of our drama out of the way in July and can now settle down into a boring, event free August. I have a couple of patterns that I am eager to work on and release soon – my nursing bra expansion pack as well as maternity undies. I also just realized I never released a couple of my harness/vest patterns… I had hoped to get back to taking some lingerie orders sooner rather than later, but with the insanity of the last month I’m going to give myself some time to rest before getting back to work…
