The Plague

A brief health update on Jacob.

Last Saturday, Jacob started to sniffle. Next thing we knew, he had developed a full-blown upper respiratory infection, complete with rivers of thick green snot and a fever. We pulled back on his protein intake – He wasn’t hungry anyway. – and kept tabs on him. Daddy got evacuated from the master bedroom so I could have a comfortable sick room for us to basically lay in bed all day.

This was terrible timing, because Jacob’s Uncle Kevin and his family were visiting.

We were hoping for a quick recovery, but on Monday afternoon Jacob started acting strange, crying pitifully and shaking. Jacob’s Mamaw and I rushed him to the Lakewood Ranch hospital. I didn’t feel we even had time to go to Tampa General.

When they told me his fever had reached 105, I was floored. How did I not notice that he was getting worse? Was he regular sick, or, to borrow a phrase from my fellow citrullinemia mom blogger, citrusick?

Jacob barely moved when they drew blood and set up the IV, just reached for me crying, “Mama, mama” so, so softly. I steeled myself for a long hospital stay.

His ammonia was 16.


That’s the lowest it has ever been.

We gave him Motrin and his temperature dropped to 102. We were home by bedtime.

You know, I have no idea what an ammonia crisis looks like for Jacob. I don’t ever want to find out. Regular sick is hard enough.


Avocado Toddler Muffins

I am not naturally good at baking. At all. But I would like to learn. As I’m more motivated to bake for Jacob than for myself, I figured I’d start with mini toddler muffins.

I had a ripe avocado that needed a purpose, so I thought I’d find a toddler muffin recipe that called for avocado. These Avocado & Blueberry Mini Muffins seemed perfect. I didn’t have any blueberries, but I figured we could do without.

I was also really excited to use my new mini muffin pan, which I convinced my husband we definitely needed because Jacob, being a mini person, certainly needs appropriately sized muffins.

Lucentee® Large Mini Muffin Pans – Top Non Stick Bakeware for Muffins, Cakes and Cupcakes – 24 Cups Texas Jumbo Silicone Mold / Baking Tray – Heat Resistant Tins up to 450°F- Easy to Clean – Blue


I decided to start by making the actual recipe without any low protein substitutions as a baseline. The resulting muffins are a little higher in protein than I would like them to be, but Jacob can still have one or two and stay well within his daily protein allowance. Here’s how I calculate the protein:

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I didn’t include items like sugar or vanilla extract, which have no protein or negligible amounts.

Next time I’m going to try the recipe with a protein-free egg substitute and a lower-protein coconut milk based yogurt. Hopefully it will turn out just as delicious as these were! Stay tuned, because I’ll update this post with the results.

On House of Cards and Liver Transplants

Spoiler Alert – If you haven’t watched Season 4 yet, you might want to skip this post.

Whether you hate to love or love to hate Frank Underwood, there is no question that this season – with the attempted assassination, wild hallucinations, and the emergency liver transplant – was intense and even sometimes difficult to watch. Perhaps especially for me. I don’t even know if I would have paid much attention to the doctors description of high ammonia levels before Jacob, but now that I’ve received an unwelcome education, I watched rapt, terrified of what can happen when a liver fails to do its job.

I haven’t discussed liver transplants much (at all?) on this blog because that course of treatment is not currently part of the conversation with Jacob’s doctor. A liver transplant would “cure” Jacob’s citrullinemia. We could serve him a prime rib the next day. (Actually, there would probably be some restrictions on what you could eat the day after major surgery, but you get the picture.)

There is some disagreement in the UCD community over whether it is wiser to transplant early and often, if you will, or manage the disorder with diet and medication, as we have done for the last 13 months. In short, the arguments, as best I understand them, go like this:

Pro-Transplant: This disorder is a ticking time-bomb. A single crisis of hyperammonemia, and the child could suffer permanent brain damage. Besides, we don’t know what effect the permanently high citrulline levels (in children with citrullinemia, specifically) have on the brain. Some people contend that high citrulline can cause headaches and autism-like symptoms in otherwise well-managed children. Also, we don’t really know the long-term impact of ammonia scavenger medications like Buphenyl. In short, it is safer to transplant, because issues related to transplants are more predictable. There is simply more research. In fact, a 2013 study determined that “[liver transplant] was associated with the eradication of hyperammonemia, removal of dietary restrictions, and potentially improved neurocognitive development.”

Sounds pretty good, except…

Anti-Transplant: An organ transplant is a major surgery, and irreversible. Why would you take the risk of transplanting an otherwise healthy child, when the treatment options and management protocols for UCDs are getting better every day? Either way, the child will be on some kind of medication permanently, whether it’s an ammonia scavenger drug or anti-rejection immunosuppressants. Besides, considerable research is being done on liver therapies that could eliminate the need for transplant well within our lifetimes. If the UCD is well managed, it’s better to wait and see what kinds of options you will have in the upcoming years.

So, yes, we are somewhere between the devil and the deep blue sea here.

Last year, in coordination with the National Urea Cycle Disorders Foundationthe Patient-Centered Outcomes Research Institute (PCORI) approved a $2 million study to determine which treatment approach, if any, has the best outcomes. The project is scheduled to take 37 months. Is it possible that we will be able to put this debate to rest by the time that Jacob turns 4?

What Jacob’s Eating (And What He’s Not)

When Jacob turned one, our new daily protein limit (goal?) became 9 grams.

Now, this might not seem like a lot… a cup of Greek yogurt contains 24 grams of protein. But when you have a toddler who is, shall we say, selective – nay, mercurial – about his eating habits, 9 grams is a challenge.

Whereas I plan my weekly dinner menu a week ahead for Justin and me, I’m usually at a loss for Jacob. Without fail, mealtime arrives and I stick Jacob in his high chair with a handful of Cheerios while I scavenge for something he might like. As a result, he eats a whole lot of avocado, yogurt, strawberries, and bananas, along with assorted prepackaged veggie purees.

In an effort to branch out, I promised myself that I would try a couple made-from-scratch foods for him along with some frozen goodies that I picked up at Fresh Market.

baby eating spinach patties

So far, the storebought dino-shaped Dr. Praeger’s spinach littles (.5 g protein each) have been a huge hit. On the other hand, the butternut squash and paprika low-pro mac and cheese that I made from scratch – immediately rejected. What kid doesn’t like mac and cheese? (Okay, to be fair, the “cheese” was mostly pureed butternut squash with a smattering of dairy-free Daiya cheddar. Not exactly your typical comfort food. But still.)

Trying to think outside of the box, we turned on Regulator by Warren G to encourage him to eat. It didn’t help, but did make for some entertaining video. Fast forward to 0:40 for the best part.

This morning’s breakfast of toaster waffles with butter and jam was also rejected. I may have eaten those. I don’t regret this. Waste not, want not, right?




It has been a long time since I have published a post. Months. The longest since I began this blog.DSC_0361

Here are my excuses.

  1. Jacob has been doing great. This is the happiest reason to not publish. We haven’t been to the hospital since I can remember. If you are reading this, please shout hallelujah and knock on wood at the same time, just to cover all our bases.
  2. I have been doing lots of writing outside of this blog. I have the great fortune to be working as a freelance marketing coordinator for Big Sea. I can’t even tell you all how lucky I am to have stumbled into this job. I work for and with amazing people who live their values. If you need marketing services, you would be foolish to hire anyone else.
  3. I have a 13 month old at home. He’s walking. He has figured out how to open doors. I no longer sit down.

But, enough with the excuses. It’s time to ramp up this blog again, because it brings me great joy to talk about my little ray of sunshine, and because my mission – to become a resource for other citrullinemia parents – has not wavered.

Also, I know a whole heck of a lot more about blogging now. And I might as well put that to good use.


And here we are, in 2016. The year after the year of Jacob’s birth, looking back at so many firsts. First smile, first laugh, first tooth, first Christmas. We are a month and a day away from Jacob’s first birthday. Then we will begin to count in years instead of months, and the firsts, while still plentiful, will slowly dwindle, gradually supplanted by lasts. It is fitting, maybe, that firsts are marked, celebrated. They are an occasion. Whereas lasts happen unnoticed. When was the last time Jacob wore a newborn-sized outfit? I couldn’t tell you the day. The last time he rode in his infant car seat before we graduated to the big-boy seat? No idea. One day he will ask for his milk in a cup, and, just like that, I will know that he has had his last bottle. There will be a last morning nap, a last meal in the high chair, a last poopy diaper, a last game of peek-a-boo, on unremarkable days in an all-too-soon future.

So, hello, 2016. Nice to see you. Please stay awhile. I have a feeling that when you leave, you will take my chubby-thighed baby with you and I will find a walking, talking little boy in his place.