Tag Archives: ornithine transcarbamylase deficiency

Heaven is for Real

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I’m sure many of you have heard of the book, “Heaven is for Real.” I had…but I had never read it. Sometimes we avoid those things that we fear the most…and I didn’t want to think about death, or death and children specifically. But I’ve read it now. I’m pretty sure God wanted me to, so I did.

This weekend was our annual Farm Bureau meeting in Minot. George and I packed up Friday morning and headed north. I wanted to testify on one resolution specifically. It would support allowing North Dakota Children’s Special Health Services to open their doors to children with catastrophic illnesses. (Catastrophic illnesses are ones that are life-threatening. Currently, NDCSHS can only assist those children with illnesses specified by state statute…OTC is not one.)

On Saturday morning, I stood in front of the Farm Bureau delegates and tried to give a brief synopsis and explain why we needed to forward this resolution. It was, by far, one of the most difficult things I have ever done. Imagine explaining to a large body of people that your child’s life is at risk, and you would hate for other children and families to have to fight the same fights you’ve been battling. It was pretty emotional for me, and I wasn’t as strong as I would have like to have been. But I made it through, and I thank God that the resolution passed.

After that, we had our district caucus meetings. And I was sitting in the back of a room, along with my friend, Joannie. A lady came up to my table, handed me the book, “Heaven is for Real,” and told me that she thought I should have it. She gave me a hug, watched me wipe away my tears, and quietly slipped out of the room.

Sitting here, I still can’t believe it happened. It was one of those moments that (looking back on it), God was telling me not to worry about the future. No matter what happens, He’ll have George in His arms. I need to quit dwelling on the what-ifs and work on enjoying the now. I get that. And when I falter, and start to worry, I have a book to read to remind me.

I needed that.

And to the wonderful angel who delivered the message to me…thank you.

A Mother’s Love

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I saw this on Facebook tonight…and couldn’t get it out of my head.

A Mother's Love

This was the caption under the photo:

This is a true story of Mother’s Sacrifice during the Japan Earthquake.
After the Earthquake had subsided, when the rescuers reached the ruins of a young woman’s house, they saw her dead body through the cracks. But her pose was somehow strange that she knelt on her knees like a person was worshiping; her body was leaning forward, and her two hands were supporting by an object. The collapsed house had crashed her back and her head.

With so many difficulties, the leader of the rescuer team put his hand through a narrow gap on the wall to reach the woman’s body. He was hoping that this woman could be still alive. However, the cold and stiff body told him that she had passed away for sure.
He and the rest of the team left this house and were going to search the next collapsed building. For some reasons, the team leader was driven by a compelling force to go back to the ruin house of the dead woman. Again, he knelt down and used his had through the narrow cracks to search the little space under the dead body. Suddenly, he screamed with excitement,” A child! There is a child! “
The whole team worked together; carefully they removed the piles of ruined objects around the dead woman. There was a 3 months old little boy wrapped in a flowery blanket under his mother’s dead body. Obviously, the woman had made an ultimate sacrifice for saving her son. When her house was falling, she used her body to make a cover to protect her son. The little boy was still sleeping peacefully when the team leader picked him up.
The medical doctor came quickly to exam the little boy. After he opened the blanket, he saw a cell phone inside the blanket. There was a text message on the screen. It said,” If you can survive, you must remember that I love you.” This cell phone was passing around from one hand to another. Every body that read the message wept. ” If you can survive, you must remember that I love you.” Such is the mother’s love for her child!!

Why did this strike a chord? Aside from just being a mother? (By the way, snopes clarifies that the picture and the story don’t go together…I’m fine with that, but whether or not the two go together, they both hit my heart.)

Well, to put it simply, I’m somewhat in the same position.

After researching and researching and researching some more…I’ve learned quite a bit about George’s OTC. And if he truly has OTC (which has been pretty well proven through testing and improvement with the diet), then we’re dealing with a disease that does a lot of taking.

For example…in OTC, males are hit harder than females. In fact, 50% of males born with OTC do not live 72 hours. And of the 50% surviving, another 50% will die by the age of 5.

Those are the facts.

So, on Sunday we head back to Rochester. And this time, the questions will be a little more pointed and a little more clear. I need to know exactly what the doctor is thinking, and what we need to do. I’ve read on some new research showing hopeful uses of gene therapy…something we may look into.

Whatever it is, we will do…I will stand over my son and let the roof crash on me, so that some day he can stand tall and read, “If you can survive, you must remember that I love you.”

Home Away from Home

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Well, George has had a bad week. Yesterday (Friday) we came in for IV fluids. Unfortunately, George was making his Mama nervous. He kept falling asleep. By noon, he had taken three naps, and by the time we were in Aberdeen getting fluids, he was on nap number seven.

Now, I know that after the week he’s had (tonsils and adenoids out, tubes in ears, staying in the hospital for three days, etc.), he deserved some rest…but for a 2-year-old, well, we were dwelling in a dangerous area.

George’s OTC has “episodes,” which are periods of seizures, brain swelling, high ammonia levels, etc. These are medical emergencies and need to be treated with IV therapy ASAP. In fact, I carry a letter from Mayo and a letter from his pediatrician, instructing any ER we may end up visiting that if George comes in with fever, lethargy, or other unexplainable symptoms, he needs to be treated immediately. No waiting for triage, no waiting for doctors orders, blood draw and IV first, questions second.

So, my question when we came in Friday afternoon was, “Is his lethargy caused from regular post-op blahs? Or are we dealing with something else?” Doc couldn’t answer that, so we drew some blood. And quickly received our answer. George was dehydrated.

Now, I received a thorough grilling from our pediatrician. You see, he was behind in information. He knew that George had surgery on Tuesday, but he didn’t realize that he wasn’t taking anything orally and had only been released the night before with the saline-lock in his hand, with orders to come back each day for fluids until he was drinking 20-30 ounces per day. So we had gone a little over 12 hours without fluid, and George’s stats weren’t good.

The doc explained it to me like this: George’s body thought it was starving. So, it started the process of breaking down it’s own tissue to get enough protein, etc. that it needed. He called it acidosis.

So, we are back in the hospital. We have learned a valuable lesson. George can’t go very long without fluids. He dehydrates in RECORD time…and I mean fast! And although he was producing tears, had a runny nose, moist mouth (all signs that he’s hydrated enough), the blood work showed a way different story.

And now we know.

It is Sunday…and I will give thanks to God for allowing us to have the technology and the means to be able to figure these things out, and correct the deficiencies that are there. I will thank Him for the wisdom of the doctors, the compassion of the nurses and the love and care of all our friends and family. I will thank Him for mother’s intuition.

But most of all, I will thank Him for George.

Thankful Thursday: Nurses

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Today I have a special message of thankfulness to give, and this one is to our nurses.

With our frequent flier miles at the hospital, we get a chance to meet and test out most of the nurses here on the pediatric ward. I know a majority of them…mostly by first name.

But today, this blog post is about them. I am thankful for them everyday…especially this week.

I am thankful for:

  • Not making me feel like a bad mother for wanting to not be in the room when medicine is given. When a little kid’s throat is sore, taking medicine is a form of medieval torture, no matter how necessary it is. I can be the bad guy when I have to be, but appreciate the breaks occasionally.
  • Talking to me like a mother, not a child or a doctor. When the doctor says, “We’ll talk about going home once he starts drinking.” And the nurse letting me know that with 2-year-olds, we could be here a bit.
  • Letting me know what’s normal. The above mentioned issue of not drinking? Guess what? Completely normal.
  • Taking care of one of my most prized possessions. That includes: reading his history, understanding my concerns prior to coming in the room and going above and beyond to do what is right for George.

No matter how you look at it, all of the nurses here are pretty much amazing. And one day, when George is big and strong and a no longer eligible for the pediatric ward, I hope to come back and personally thank each one of you, for your crucial part in our journey.

But for now, a simple “Thank you” will have to do.

What gets George through a hospital stay? His puppy, his blankie and his tractor magazines!

 

Where’s My Relaxing Summer???

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I remember when I was a girl (hey now, don’t laugh, it wasn’t THAT long ago!), summer break from school was a relaxing time. We played, slept in, stayed up late, whatever we wanted to do. We went fishing, took drives to check fields and worked in the garden.

Where is that summer now?

Yesterday started off as a Tuesday, and ended as a Monday. We have 20 days until school starts, and so many things to pack into our “summer” that I almost wish school would start now! (Almost, I said almost.)

In the last few weeks, George has battled yet again another case of strep. We were referred to an Ear, Nose, Throat specialist, who promptly decided on Monday that he needed to have his tonsils, adenoids removed and tubes in his ears…and the sooner, the better. Which means Tuesday. So then we need to have a pre-op history and physical done. Which could only be scheduled yesterday.

With George’s OTC, illnesses are taken very seriously. Two of his strep infections have landed him a stay in the hospital. Both times he had seizures, both times he ended up on high doses of antibiotics, both times I had the bejeebers scared out of me. According to the ENT, removing George’s tonsils and adenoids will improve his snoring/lack of sleeping habit and will greatly decrease his chance of getting strep. Yay! He also failed the tympanogram. Meaning there is fluid in his ears and his ear drum is not moving much. So we will also be getting tubes.

Tubes/tonsillectomy/adenoidectomy…sounds like a fun day, eh? Oh well, as long as it makes things better in the long run, I’m all for it.

But to get back to yesterday.

After George’s pre-op appointment, we needed to get Big Bro’s new glasses. On our way to that appointment, Scooter started crying that his ear hurt so bad that he couldn’t move his head. Thankfully, there’s a FastCare clinic next to the eye doctor. We stopped in and found out the Scooter has swimmer’s ear. Figures. There’s only four days left of the pool being open.

So…after some running around, three doctor’s appointments, we met with Grandma B. at “Donald’s” for supper. We don’t eat out very often, so I wasn’t used to ordering for George off the Golden Arches menu. What do you get a child who can’t eat most of the menu?

I asked the manager (who happened to be the one waiting on me) if I could order a hamburger Happy Meal, minus the meat. I explained to him the situation, and he promptly went to the kitchen and made sure it was made right. Bun, check; ketchup, check; pickles, onions, mustard; check, check, check. The manager also threw in extra apple dippers, just to be nice. As I brought his meal to him, George was the happiest little boy around. He had the same thing as his brothers…and enjoyed every bite! I actually tear up, just thinking about it.

Sometimes, it’s just the little things in life that make your day.

King Arthur Flour meets Grandma Vivian’s Dumplings

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I’ve joined a party…and am creating history all at once!

As part of A Latte with Ott, A’s King Arthur flour Iron Chef Challenge, I decided that I would make Brandenburger family history…I would use my Grandma Vivian’s Dumpling recipe. (Mind you, this recipe has never been written down [to my knowledge] and for certain isn’t on the internet.)

This recipe isn’t something you just “throw” together for a quick lunch, but it’s a wonderfully delicious authentically German dish that is requested time and time again at my house. Especially at family gatherings!!!

First of all, you need bread dough…and this is where the King Arthur flour comes in. For those that have been reading my blog for a while, you know that our youngest son has OTC, which limits his protein intake to 10-12 grams per day. Well, it just so happens that King Arthur flour has a product  that is lower in protein! Woohoo!!! And I used this flour for our bread dough (although it’s labeled for wonderfully, delicious and super light pastries, pies, etc.). It worked beautifully!

Anyway, on with the recipe:

Grandma Vivian’s Dumplings

Ingredients –

3 cups flour (I used King Arthur Perfect Pastry flour – only 3 grams of protein per 1/4 cup!)

1 1/2 TBSP. butter

1 cup water

1/4 cup milk

1 package instant dry yeast

1 tsp. salt

1 1/2 TBSP. sugar

1 qt. chicken broth (liquid)

potatoes

2 cups heavy whipping cream (pic shows half and half, which is what I used…but Grandma always used heavy whipping cream…half and half tasted just as good!)

Everything you need!

First, we need a simple loaf of white bread dough. This is a simple, easy loaf recipe…and trust me, if I can make it, anyone can!
Mix together 2 1/2 cups flour, salt, sugar and yeast. Set aside. Then mix together water, milk and butter, heat until hot to touch, but not boiling. (I microwaved for 40 seconds.)

When making this dough, mix together dry ingredients, then mix and heat wet ingredients before combining.

Then, pour your wet ingredients into the dry and mix until a dough-ball is formed. Take the 1/2 cup flour that’s left-over and add as needed to make a smooth dough-ball that doesn’t stick to the sides of the bowl. (I ended up using just about all of the flour this time, but it will vary, depending on your humidity, etc.)

Dough - finished but not raised.

Cover the dough with a cloth for 10 minutes, to let it rest. Then shape into a ball again and let it raise for 1 hour.

Fluffy, yummy dough ball!

Now, we’re ready to make dumplings! Peel and cut your potatoes, enough to fill the bottom of your kettle (4 or 5 quart kettle or dutch oven works wonderfully!).

Enough potatoes to fill the bottom of a kettle, or maybe even a little more!

Add the chicken broth. (I use chicken broth instead of water to boil my potatoes. It adds great flavor!) You can flavor your potatoes if you’d like, with salt, pepper, onions, etc. Whatever you would normally do for boiling potatoes. You won’t be draining any of the liquid.

I use chicken broth instead of water with my potatoes. Yummy!

Heat the potatoes to a boil.

Potatoes are boiling!

Remove the kettle from heat. Punch down the dough. Tear off about quarter-sized bits of dough and layer across the kettle, on top of the water and potatoes.

Pull apart the dough, enough to fill one layer across the potatoes in the kettle.

Dough, completely covering potatoes and ready to cook!

The next part is very important: Place lid on kettle, put it back on medium heat. DO NOT REMOVE THE LID!!! In fact, if your lid does not seal well, or isn’t a very heavy lid, place something on top of your lid…such as:

If your lid isn't heavy enough to make sure there's a seal, set something heavy on top of the lid! Be creative!

Why is this important? The steam from the potatoes and water is cooking the dumplings. If the liquid in the kettle evaporates too quickly, your dough will fall and will be more solid, than light and fluffy. (Grandma would call them “klutzy” when that would happen.) Once you can hear the potatoes boiling again, turn the temp down a bit and simmer. Cook for 30-45 minutes or until the “dumplings start talking to you.” (Grandma’s terms) In cooking terms, this means to listen to the kettle and the dumplings are done when you can hear them starting to fry a little bit.
And then lift off the lid and pray…just kidding…it should look something like this:

Dumplings are done!

Next, stir up the dumplings, mixing the dough, the potatoes and whatever liquid is left. Then pour the cream on top of the dumplings and stir.

Add cream to the finished dumplings.

And finished product, all mixed up...yum!

And that, my friends, is my Grandma Vivian’s Dumplings…actually wrote down and save for all of posterity. She would have been proud of me! She was my best friend, and has been gone for almost a year now. There isn’t a day that goes by that I don’t think of her, and wish that I would have just one more day with her. She taught me so many things about life, but the thing she cherished most was laughter…and good German food! 😉

These are authentic German dumplings...made from scratch! (And low protein!)

Grandma would normally serve this with chicken, but it goes well with any protein item of your choice: beef, lamb, pork, etc. (Not sure it would go well with fish, but go ahead and try! Life is made for rules to be broken, right???) With this particular meal, my sister and I ate these dumplings all by themselves. Just because we could.
Enjoy!
King Arthur did provide me with flour to use for this recipe contest however the opinions listed here are my own.

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Wordless Wednesday – Mini-veggies

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As I’ve mentioned in the past, I’ve been working hard on getting our garden to make it through the year. You see, it’s not just because I like vegetables, or I think we’re the only ones who can grow a tomato right, or I don’t like getting vegetables from my store or anything like that.

It’s much more simple…I want to provide for my son.

George’s condition? George’s diagnosis? George’s illness? Hmmm…what do I call it…let’s just go with OTC. George’s OTC will not allow us to provide him with the wonderfully delicious beef that the rest of our family gets from the farm. And he never will be able to enjoy it…at least not much. George is limited to 10-12 grams of protein per DAY…and well, that just isn’t much in the scheme of things. And meat is basically out of his realm of menu options. Completely.

George loves his fruit! It's a good thing, since that's about the only thing he can eat a lot of!

 

 

So we go with fruits and vegetables. And since North Dakota isn’t real high on the fruit scale of production, we’re going to do our best on the veggie side. Like these:

These sugar snap peas are just about ready!

 

 

The cucumbers are showing up! Yahoo!!!

 

 

These peppers aren't much yet...but they're getting there!

 

 

Tomato...tomahto...can you say salsa???

How NOT to spend a birthday

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As I mentioned yesterday, George spent the night in the hospital. Now, with his OTC, that’s not that unusual, but his method of gaining a bed was a tad unorthodox, to say the least.

You see, Tuesday was my birthday, and as I pointed out earlier, it always rains on my birthday. Well, that tradition is safe for another year, because yet again, it rained on Tuesday. And since rain makes everyone sleepy, I didn’t think much of George wanted to climb in my lap a little before 11 and falling asleep. I had second thoughts when he woke up shortly after lunch, screaming bloody murder.

As I tried to settle him down, I realized he was feeling warm. So I took his temp, and low and behold, it was 103.2. I know we need to be careful, so I did a tylenol suppository (he’s at the age that he refuses to take medicine, as in spitting it back in my face).  A little bit later, his temp was down to 101.5, but he was very lethargic and not acting quite right, so I called the pediatrician. Although his office is an hour drive from here, they said they would see us as soon as we could get there, so I got everyone ready and headed out.

I suspected we were dealing with an ear infection, or something along those lines, so I didn’t think much of it. I figured we would be back home shortly after the boys got home from school, so I made sure Boss Man didn’t plan to leave the yard. I dropped EJ off at Grandma’s house and we were off to the clinic.

We got to the clinic, the nurse weighed George and took his temp (101.4) and we sat down in one of those cozy little rooms. And then it hit.

George had another full-blown seizure. Thanks be to God that it was in the clinic, and not while I was driving down! The nurse called the doctor in immediately, the place was buzzing with action, we had suction, oxygen and plenty of support staff. It was terrifying and a relief at the same time. Terrifying because it was my son, and he was in trouble, relieving because it was at the clinic, and the doctor knew what to do.

It was seven minutes from when things started until things were “OK” again. We were admitted to the hospital for observation, mainly because George is a “special case” when it comes to these things.

You see, febrile seizures (seizures caused by a fever), are common. Very common. As common as they are, they are still scarier than all get-out. But high ammonia levels can also cause seizures. And illness can cause high ammonia levels with OTC. So which is the culprit, the ammonia or the fever? Chicken or the egg?

So we got a cool wheel-chair ride in an underground tunnel from the clinic to the hospital, had a room full of nurses starting IV’s, trying to draw blood and making sure that everything was taken care of. And then we spent the rest of my birthday, and half of his, waiting, and waiting and waiting.

He did not have any other seizures. His temperature only went up once during the night. And by morning he was well on his way to back to normal.

So what was the cause? No clue, really. His ears were clear, his lungs were clear, so the doctor went with the stand-by of “viral illness.” All that matters to me is that we are back home.

And the next time he starts to run a temp, we’ll be on high alert. I won’t necessarily have to take him in, as long as I feel comfortable and nothing to extraordinary happens. But we’ll see. I’m not sure I want to trust my judgment on what may be wrong…especially if a seizure is involved. It’s not that I’m unfamiliar with this territory, we’ve been down it before with Big Bro, but this is a whole new level.

I will say, though, George knows how to kick off a new year. I may be in big trouble if this is just the warm-up to the Terrible Two’s.

Good Friday trumps Earth Day

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I was all prepared to write a post today about all the things we do on the farm that celebrates the Earth, such as using our manure, using no-till whenever possible, using the water from our well to heat our home…then heat our shop…then to water our cows, etc. (That’s right, all the same water, I’ll explain it sometime.)

But as I was sitting down to type last night, I decided to check out George’s lab work that was done at Mayo. (They have a really cool set-up, where you can register to log-in and receive the lab results yourself. No more waiting for that stinkin’ doctor’s call!) Anyway, I logged-in and for the first time ever, and I truly mean EVER, all of George’s lab work came back within normal ranges! (Well, minus the Vit. D and iron levels, but those are diet/sunshine related, not illness/disorder, so they don’t really count.)

I’ll admit it, I cried a bit. For the last just-about 2 years, I’ve dealt with continuously feeling like something wasn’t right, that we weren’t on the right track, bloodwork continuously showed something off here or there, nothing fit together, etc. It was a whirlpool of nightmares. Since George was born, he has seen: three pediatricians, two pediatric geneticists, two pediatric gastroenterologists, pediatric cardiologist, two pediatric endocrinologists, two pediatric neurologists, two dieticians, pediatric oncologist, pediatric neurosurgeon and several other various ER docs, nurses and staff. He has had: two colonoscopies, an upper GI series, multiple x-rays and ultrasounds, a liver biopsy, three MRI’s, an echocardiogram and two CT scans. He has given more blood for bloodwork that some people donate to the blood bank. He’ll be 2, and he’s been through so much, but is still such a wonderful little boy.

One of the things that I hear the most, when people hear about George, is that he doesn’t LOOK like anything is wrong with him. That’s always the problem. I wonder how many of those doctors blew us off because they felt he didn’t “look” sick enough. I know for sure one did, I overheard him tell the students that were following him those exact words. It went something like this, “And in this room is a 6-month-old male patient, case seems somewhat unremarkable. Mother has sought care at Mayo. Came in with fever, slight dehydration, etc. There’s no clear diagnosis, and I’m not real sure why they’re here. Their local hospital probably overreacted. We’ll keep him through tomorrow to satisfy the Mother.” (Needless to say, I requested a discharge immediately and we never returned to that set of physicians.)

We have so much to be thankful for.

This morning, Big Bro told me that they didn’t have school today because it was Earth Day. That sealed the deal for me. I’m not writing about our farm today. I’m not writing about how to recycle, or how to reduce your carbon footprint, or how to reuse your milk carton to make a mailbox. We should all know these things, and we should all be doing them. Every day. Period.

But today is Good Friday first. It’s a day that we celebrate all that has been given for us. The Blessing that was bestowed upon us so many years ago. And for me, today is a day of thankfulness.

Yes, I won’t be brushing my teeth with the water on, I’ll turn off lights where I don’t need them, our bulbs are already energy efficient. Those are things we do everyday. But today, I’ll spend extra time thanking God for those gifts that He has given. Including the ultimate sacrifice of His only Son.

Today is definitely a Good Friday.

Day 2 – More good news

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I had to make a quick, mid-appointment update to bring some really great news…George no longer needs to have growth-hormone shots!

Now, this may only be temporary, we will have to come back in 4 months and double-check on things, but I’m certain that things will continue to go on the right track. Sadly, our visits with Dr. Lteif (endocrinologist), may become fewer and farther between…but that’s only sad because she’s such an excellent physician, and thinks pretty highly of our little man.

Up next, we see the neurologist, who will become the lead doctor in George’s care. Dr. Renaud is a pretty great doctor, too.

So, as long as everything goes as planned, we’ll be on the road heading north shortly after lunch. And for once, I’m leaving Mayo feeling as if we’ve finally turned a corner, not just headed further into the abyss. What a great feeling that is! (And for those new to my blog, George is suspected to have Ornithine Transcarbamylase Deficiency, a disorder that doesn’t allow him to break down protein…which means he won’t be enjoying full servings of meat, dairy, etc., for most of his life.)

Yesterday, when they drew his blood, I saw on the paperwork that his tests were heading to Baylor for the genetic testing. George hasn’t travelled much in his almost-2 years, but his blood certainly has!

Ag Book of the Day 13:

“What Do I Make? (Let’s Find Out!)” by Dale Simpson. A great board book, with turning wheel, where you can match up the animal with their gift to us! Great for the little guys! Include milk from cows, honey from bees, clothes from sheep, etc. And with the cute cow-shape of the book, and great illustrations, it keeps the little ones interested in the book!

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