What does Jacob eat? (An update)

IMG_0788You may remember from a post a few months ago that we were very excited to start Baby Led Weaning with Jacob. Since then, his food allowance has gone from .5 gram of protein a day to 3 grams of protein! (If you’re new to the blog, children with Citrullinemia are on a severely restricted protein diet. They must receive exactly the amount of protein their body can use – no more, no less. Too much or too little puts Jacob at risk of brain damage and coma, or worse.)

Like almost everything else in my parenthood journey thus far, our solid-introducing experience has not gone, shall we say, by the book.

Initially, Jacob seemed interested in playing with food, but as soon as a lump would hit his tongue he would vomit. Like clockwork. His little face would turn red, his eyes would water, and out would come the offending particle along with anything else that had entered his stomach in the last, oh, few hours. In that way, we lost quite a bit of formula and (horrors) breastmilk. Finally, I had to put a stop to it and introduce purees.  I dutifully went to the farmers market, purchased armfuls of gorgeous organic veggies, and set to making homemade purees, measuring each ingredient and using a spreadsheet to calculate grams of protein per serving – only to have Jacob reject over half of the options I offered him.

Finally, exhausted with the unappreciated effort of producing and measuring my child’s food, I went to store-bought organic purees. Tangential note #1: I recently managed to start a flamewar on the internet in a Facebook group about home organization when I indicated that I feed my child from packets.


Tangential note #2: There is no limit to the uses of this meme. Game of Thrones FTW. I’m geeking out here, people.

At any rate, store bought purees were a life saver. I could buy one flavor at a time, figure out which ones were his favorites, and go from there, introducing new tastes little by little. Since the protein content was indicated on the label, all I had to do was measure how much of the packet he actually ate. Purees, teething biscuits, and occasionally oatmeal have been the staple of Jacob’s diet for the last few months. (Behold below, the puree drawer image that started the internet flamewar.)

Until a few days ago, when Jacob’s little lips closed tight against the introduction of purees. Frustrated, I steamed up some purple cauliflower from the market, chopped it up, and dumped it on the tray. In it went. No vomiting, no fuss, just a happy eating baby. Carrots! Grapes! (Peeled and quartered! Don’t arrest me, mom police!) Cheerios! All happily consumed!


I am finding that Jacob is now eating less, and slower – which means we may need to temporarily adjust down his protein allowance and make up for it by reintroducing natural protein into his milk. But I am so happy to see him begin to practice those fine motor skills and tolerate textures!

I also need to correct an earlier assumption of mine – that Jacob would be vegan. In a conversation with Erika yesterday, I learned that many children with citrullinemia will eat cheese and other dairy products as their protein allowance increases with age. These complete proteins are good sources of nutrition for UCD kids. I have to tell you something she said because the image is so beautiful in my mind. “It will be easier for Jacob to be a vegetarian than a vegan. You know, when he is a teenager and goes to a restaurant with his friends, he can always order a cheese quesadilla and be on his way, rather than having to search out a vegan option.” Can you imagine? Jacob? A teenager? In a restaurant with friends? Ordering a quesadilla? It is so normal and beautiful. Isn’t that what we all want for our children?

Four Month Pumpiversary

I have now been exclusively pumping for a little over four months. That means that I have hooked myself up to my pump about 850 times, and have spent approximately 220 hours or nearly 9 solid days attached to my trusty Medela Symphony. It also means that I have earned this awesome award…


Do I think about quitting? Yes, all the time. I fantasize about throwing my pump off the Sunshine Skyway Bridge bringing my pump back to Walgreens and being done with it. I am still angry that citrullinemia stole my nursing relationship with my baby. Some days I can’t stand the thought of washing and sanitizing yet another bin full of bottles and pump parts. The reason that I keep attaching myself to the pump day after day is complex. Yes, it’s love, but it’s also pride, determination, and I imagine a healthy dose of biological imperative. At the end of the day, I have this sweet face (and those chubby thighs, nom nom) to show for it. And so I keep marching on.*

*Figuratively, of course. The cord on the pump is only a few feet long so I can’t really march very far 😉

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: Baby Bottles

baby bottle Breastflow First Years

Look Mommy, I’m using my hands!

First of all, fun history teacher fact. People were bottle feeding their children four thousand years ago! Of course, at the time there weren’t many bottle options. Today, there are 4000 options. I am easily overwhelmed with that many choices–you should see me in the dish soap aisle at Publix–so you should be very impressed that I was able to decide on five bottles to try. So, voila, my two cents on five brands of baby bottles. For each one, I will mention the price (important) and the number of pieces you have to wash between each feeding (even more important).

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Citrullinemia FAQ: What exactly does Jacob eat? Also, why do you have a gram scale in your kitchen?

Every day, I mix up Jacob’s formula according to the recipe provided by Erika, his metabolic dietician and guardian angel. It is Erika that maintains Jacob’s delicate balance of enough protein for growth and not enough to hurt him. Although Jacob’s medical progress is supervised by Dr. S, Erika is the one who I email or call on nearly a daily basis with questions about Jacob’s various needs. In Jacob’s two months of life, she has probably adjusted his formula recipe at least five times to account for his growth and answered, oh, about 4,733 of my questions. (I exaggerate, but not by much.) I also can tell that she genuinely cares about him. We are so lucky to have her on our team.

Erika’s recipes are carefully calculated mixtures of two different infant formulas, Pro-Phree and Cyclinex-1, both made by Abbott Nutrition, added to my breast milk and water. I have already discussed in detail my experiences as an exclusive pumper, so we won’t revisit that here.

Pro-Phree is described on Abbott’s website as a “protein free energy module” (doesn’t that sound appetizing?). Basically it provides calories and some vitamins but no protein, hence the name. Cyclinex-1 makes up the bulk of his formula. It is specially designed for children with urea cycle disorders. It does not contain the non-essential amino acids, that is, the ones that our bodies are capable of producing. Since Jacob can only have a limited amount of protein, this formula allows him to get a higher concentration of the essential amino acids that he cannot make on his own.

Each day, I measure his formula and arginine medication on a gram scale and my breast milk in 80 mL test-tube-like vials. It’s like Breaking Bad in my kitchen. pro-phree cyclinex-1 gram scale breast milk arginine

He gets more breast milk than is shown in the picture, but you get the idea. His recipe is taped to the wall and all of his ingredients are ready to go. I mix it all together in a big jug and then measure it out for each feeding. It’s very scientific, and not how I once pictured feeding my baby. But we do get plenty of cuddles even with the bottle, so it all works out okay.

I am now known in the St. Petersburg WIC office as Cyclinex and Prophree girl. I think the wonderful ladies who work there are used to having to counsel women on what and how to feed their babies, so they got a kick out of me marching in there and telling them exactly what Jacob needs, down to the gram.

When Jacob gets older and starts on solids, it will be a whole new world for me, and I imagine a much more complicated one. But for now, I’ll be the one in the kitchen measuring out various white powders.

Ammonia by the Numbers

Be warned, readers, I am about to drop some science on you. Feel free to skip this post if chemistry isn’t your thing. On the other hand, I do refer to the classic television show “I Love Lucy,” so there may in fact be something for everyone.

Disclaimer: No information in this post, or in this entire blog, in fact, should be used to diagnose or treat a urea cycle disorder. I am not an expert, and my explanations are based on my best understanding from my discussions with Jacob’s doctors and my own research.

ammonia molecule

the ammonia molecule

That being said, here goes. When we digest protein, we produce nitrogen as a waste product in the form of ammonia. Our liver removes the ammonia from our blood through a chemical process called the urea cycle. A normal urea cycle converts the ammonia into urea, which harmlessly leaves our body when we pee. There are several enzymes involved in making this happen. Jacob’s condition, citrullinemia, is also known as ASS, or argininosuccinate synthase deficiency, because his body does not effectively produce that particular enzyme. Need a visual? Did you ever see the I Love Lucy episode with the chocolates? Think of Lucy as that enzyme. Some chocolates (ammonia) get wrapped (processed into urea), but mostly they stack up and his body doesn’t know what to do with them. Left untreated, his urea cycle would be unable to keep up with the demands placed on it, and his ammonia levels would become dangerous.

While Jacob was in the hospital, he underwent tests every 8-12 hours to determine his ammonia levels. The test requires a venous blood draw that must be immediately placed on ice and processed within an hour of the blood draw at most, but ideally within 20 minutes. This was the hardest part of his stay in the NICU. Jacob’s blood clots quickly, so it would often take several tries for the nurses to get enough blood from his little veins. Occasionally, the lab would not process the blood fast enough, and he would have to go through the whole thing again. It took several weeks after his discharge for the bruises to fade. Once properly processed, the lab results would tell us how well Jacob was tolerating the amount of protein he was taking in via formula and breast milk. Normal ammonia levels vary by age, and different hospitals often have different acceptable results. According to WebMD, normal results for children range from 40-80 micrograms per deciliter (μg/dL). Jacob’s highest level, at 4 days old, was in the 70s. After he was admitted to the NICU and started on his special diet, his levels hovered in the high 30s to low 40s. Though we can’t be sure, these numbers encourage us that he never sustained any brain damage due to high ammonia levels. Ammonia levels over 200 μg/dL for more than 2 hours would put a child at serious risk for brain damage.

Managing Jacob’s protein intake will be our primary way of preventing high ammonia levels. We will have to be especially vigilant when he gets sick (cold, flu, etc). When we are sick, our bodies actually break down existing lean tissue. Our liver then processes that protein the same way it does when we drink a glass of milk. So, when Jacob is sick, there will be additional demands on his liver from his body’s natural response to illness. Jacob’s “sick day formula” has almost no protein in order to compensate, but he is still likely to end up in the hospital if the illness becomes prolonged.