Jacob at (almost) three months: a day in the life

4:30 AM

Mommy comes in to give me a bottle. I’m happy to see her and give her a big smile. I might have slept longer but my doctor says it’s important for me not to go too long without eating. I’m totally okay with that and suck down my bottle like it’s my job (which it is). After I eat Mommy puts me back in my crib and I drift off to sleep.

7:00 AM

Mommy’s back! Woo hoo! Time to get my diaper changed and eat again. I love the new warm cloth wipes with coconut oil Mommy is using. My tooshie is the softest one on the block. I’m super excited that it’s morning and so I take forever to eat my bottle because I’m looking around and talking.

7:45 AM

Playtime in the play gym! Mommy is attaching the weird mysterious things to her milk pillows. Not sure what that’s about but I don’t care because PLAY GYM and COLORS and MUSIC! This is the best thing ever!

8:05 AM

TOO MUCH PLAY GYM AND COLORS AND MUSIC! GET ME OUT OF HERE! HOLD ME! NOW!

8:08 AM

Now I am super happy snuggled up on Mommy’s chest in the carrier. What are you doing Mommy? Mixing my formula for the day? Can I watch?

9:30 AM

How long have I been asleep? I would like to look at my awesome bug toy now. Also, read to me.

10:30 AM

I’m tired again. Time for a quick nap.

11:00 AM

Um, excuse me, Mommy, I’m a bit hungry.

11:02 AM

FEED ME NOW!!!!!!

11:06 AM

Nom nom, this is delicious. I am going to suck it down like I didn’t eat just a couple hours ago. After I’m done eating I’m going to laugh for the first time, and you are going to try everything you can think of to get me to laugh again. Nanny nanny poo poo. I don’t laugh on demand, silly.

12:00 PM

Not the carseat again! Where did you go Mommy? I’m not sure I like this. Oh, wait, there’s the carseat cover with the bees again. I love bees.

12:40 PM

How long have I been asleep? Also, how did you get me into the Baby K’Tan without waking me up?

12:45 PM

I love going shopping with you, Mommy. And because I love you, I feel the need to warn you that Daddy is going to make fun of you for buying the 3-pack of 48oz containers of ketchup. He doesn’t understand that buying in bulk and using coupons is pretty much a prerequisite for qualifying as a stay-at-home mom.

2:30 PM

I keep going to sleep somewhere and waking up somewhere else. How did I get in my crib? I’m like a tiny drunk person.

3:00 PM

Mommy is holding the pink square in front of my face again. She keeps talking to people who live inside of it. This is very confusing but I still enjoy blowing bubbles and watching Mommy smile at me.

3:30 PM

Time for second lunch, or first dinner, depending on how you look at it.

4:00 PM

If you keep singing me songs from Mary Poppins my first word is going to be supercalafragalisticexpialadocious.

4:30 PM

Mommy has me sleeping in my crib now instead of the bassinet. That’s fine by me as long as she keeps singing me to sleep. Amazing Grace is my favorite lullabye.

6:30 PM

Daddy’s home! I must tell him all about my day. The best parts were the eating, the playtime, the snuggles, and the naps. Oh, wait, that was my whole day.

6:35 PM

Oh, look at the time! It’s that time of day when I’m obligated by baby law to fuss and generally be grumpy. For the next 30 minutes, I will demand to be alternately entertained and snuggled depending on my whim at any given moment. 

7:05 PM

Mommy, I’m hungry again.

7:30 PM

Mommy, I’m tired again.

10:00 PM

Oh, hi Mommy. Is it time to eat again? Yay!

10:30 PM

I don’t think I actually fully woke up, so I’m just going to go ahead and go back to sleep. Goodnight world!

My Overachiever

Erika called me a few days ago with amazing news. Jacob’s blood test results came in.  One test determines the levels of various branched-chain amino acids in Jacob’s blood. These amino acids, which include isoleucine, leucine, and valine, come from the natural protein he takes in through carefully-measured amounts of breast milk. The test helps us to know that Jacob is getting adequate nutrition and that his body is processing the breast milk well. The other test is a comprehensive metabolic panel, which gives us more information about his liver and kidney function, among other things. Erika says that not only were his levels for both tests normal, but for the most part they were right in the middle of the normal range. She called Jacob an overachiever, and I couldn’t help feeling so proud of this little boy chattering happily in my arms.

baby rattle(One exception: His citrulline, as expected, was high. Normal range is 4-50. His was 1635. Yikes. But, that is to be expected given his diagnosis of citrullinemia.)

In other news, Jacob is starting to learn to grasp and shake a rattle. See, Erika was right, he is an overachiever. So proud of this little guy!

My Two Cents: Sleep Gear in the Fourth Trimester

Like many new mothers, I can’t remember the last time I got a full night’s sleep (which I define as 8+ hours). It was probably sometime in my second trimester. That being said, I certainly don’t have it too bad. Since Jacob was about six weeks old I have gotten about six hours a night, give or take. I think, as my pediatrician puts it, “I have a sleeper.” For me, this is a mixed blessing. As one of the symptoms of hyperammonemia is lethargy, I still get anxious when Jacob is sleeping, and sometimes I feel the need to poke him and make sure he’s responsive. This goes directly against the Craft Household Rule #1, which is “Don’t disturb a happy baby.” But still, sometimes I just need to harass him a little bit to be sure he’s okay.

Anyway, I have been blessed with a great sleeper, but even great sleepers need to be set up for success. So, behold, my list of Sleep Stuff That May Save Your Sanity:

1. The Arms Reach Mini Co-Sleeper.  I bought this on recommendation from a friend. I like it because it slides right up next to my bed, essentially extending it to create a safe sleeping space for baby within (wait for it) arms reach. I also bought a soft organic mattress pad from Baby Turtledove on Etsy. In the first weeks Jacob wanted to be closer to me, so I did snuggle him in bed with me until he fell asleep, at which point I would move him to the co-sleeper. (I would occasionally fall asleep snuggling him between me and the co-sleeper, which scared me despite the fact that I have never once rolled onto my stomach while sleeping and am thus confident I wouldn’t smother him. But still.) Anyway, now he goes down by himself without any fuss. And I sleep better because I can simply reach out and soothe him during the night as necessary.

2. The Miracle Blanket and the Zipadee Zip. My mom sent me the miracle blanket when Jacob was born, and he used it for the first six weeks or so. If you use the miracle blanket, you have to get past the feeling that you’re putting your baby in a straightjacket. They don’t mind, really. Actually, I would say that he really liked it and found it comforting. However, I never swaddled him for naps, and I noticed around six weeks he started sleeping with his arms out – cactus arms, we call it in yoga class. So I thought he might be ready for a little more freedom and ordered a Zipadee Zip. The picture below of Jacob in his “zippy” is from his first nap. It looks a bit too big for him, but he has since grown into it. Regardless, he napped so well in it that I decided to try it at night. And just like that, I seem to have hit upon the magic formula (at least for now): footie pajamas, zipadee zip, muslin swaddle blanket on his lower half, standard-issue hospital beanie (also pictured but for some reason not on his head), and “soothie” pacifier (the only one he’ll sort of keep in his mouth for 5 minutes until he falls asleep).

3. The Sleeping Environment. Since I have yet to put Jacob down to sleep in his crib, his sleep environment is our bedroom. We sleep with a tower fan on for white noise and to circulate air. We also use this diffuser with lavender essential oil to create a calm environment for bedtime. Now I turn it on for Jacob before every nap as well. It seems to help him drift off more quickly.

Other places that Jacob enjoys sleeping include: in my arms after a particularly satisfying bottle, in the Baby K’Tan, in the car seat*, and on his Papaw’s chest. Peculiarly, he almost never falls asleep in his swing.

*Jacob only likes the car seat when the car is moving. When he is asleep in the car seat, I will go to great lengths to avoid red lights. I have been known to make an unnecessary right turn at a red light and drive around the block a few times just to avoid waking him. Jacob sleeps well in his car seat but holy cow does he get upset if he wakes up and can’t see me.

So this showed up at my front door today

buphenyl

Thanks to the wonderful people at Hyperion Therapeutics and to the helpful advice of Mindy Mooney, Jacob is now equipped with enough medication for approximately 555 days, at no cost to us.

I am now thinking that part of the trouble we had was that our prescription originally called for an oral suspension rather than a powder, which had to be specially prepared at the compounding pharmacy. However, the powdered version can easily be mixed in water and pushed through his g-tube the same way. So bring on the gram scale, because we are in business!

So do not worry, saying, ‘What shall we eat?’ or ‘What shall we drink?’ or ‘What shall we wear?’ (or ‘How shall we afford Jacob’s medication?’) 
For … your heavenly Father knows that you need them.
-Matthews 6:31-32, with my addition in parentheses

Clinic: Tuesday April 14, 2015

Jacob had his first clinic visit today. Here are the highlights.

We got to see our favorite dietician for the first time in nearly two months. Erika adjusted his formula recipe again to help him keep up his impressive growth. Jacob weighs over 13 pounds now!

Justin and I also met with a genetic counselor to talk about Jacob’s particular mutations and their implications for our future family planning. With a natural conception, we would have a 25% chance of having a child with citrullinemia, a 50% chance of having a child who is a carrier, and a 25% chance of having a child who is completely unaffected. When we are ready to talk about another baby, it will be important for us to be armed with our genetic information so that we can conduct prenatal screening. There is an option to use IVF to eliminate the possibility of having an affected child by implanting only unaffected embryos. This is a great option for some families, but I personally couldn’t do it. Discarding the embryos with citrullinemia would feel like throwing away potential Jacobs.  However, I would want to have prenatal testing done so that, if necessary, we could begin to treat right away rather than wait for the newborn screening results.

Finally, we saw Dr. S, who was pleased with Jacob’s progress so far. However, we will have to wait a few days for the results from the ammonia test and amino acid panel, which are the numbers that will tell us more definitively where Jacob stands metabolically. It is still too early to tell whether Jacob has a mild or moderate case. Dr. S feels fairly confident that his case is not severe, because he would already have experienced a metabolic decompensation. We do need to remember, however, that the first year is sometimes easier for a child with a urea cycle disorder. Dr. S called it the “honeymoon period.” They grow so rapidly that their bodies are using the proteins before they have a chance to become toxic. When Jacob’s growth rate slows, his tolerance for natural protein may diminish. In other words, this is not a predictable disorder. We are thankful that Jacob is healthy today, and we will leave tomorrow in God’s hands.