MCADD Children's Book!

Yes, you read that correctly!  There is now a children's book about MCADD available on Amazon!! - Max the Monkey Has MCADD!

Nearly a year ago, we were very stressed out and struggling to get our two-year old with MCADD to eat much of anything. Every meal was a challenge,.  Nearly all of our plates were hurled off our dining room table and broken - it was just awful! It went beyond simple two-year-old pickiness for us because we knew how important regular meals were to managing our son's MCADD. I tried reading lots of children's books I found at the library about why our bodies need good food, but was really longing for something that started a conversation with him about why his body is special and REALLY needs food. I approached Laurie Bernstein and Joanna Helm (just two of the many amazing staff members in the metabolic clinic at Children's Hospital Colorado) about writing a children's book about MCADD to help my family and others like us. They loved the idea and last spring/summer, we wrote Max the Monkey has MCADD.

We started reading the book (an early draft printed out at home and stapled together) with our son last summer just after he had turned three and it instantly made a difference. He now has words that help him understand MCADD on his level and he seems to have a much better understanding of why he needs to eat often in order to have enough energy, just like Max does in the book. Our five-year old son (unaffected) has also really benefited from the book. We no longer have the argument at bedtime about why his little brother gets a snack and he doesn't. He knows that his brother needs it because he has MCADD. Now he helps us make his brother's snack and encourages him to eat right along with my husband and me!

Fast forward to today and we have completed the publication process! Now any family can buy a book that explains MCADD in kid-friendly terms, written in engaging verse and accompanied by beautiful illustrations and yummy recipes. It can be delivered to your door in as little as one day and hopefully you can be spared the months of mealtime stress we had when we were struggling to explain a wildly complicated condition to our toddler. Words cannot express how exciting this is to me and how proud I am to have been part of this!

Thank you to Laurie, Joanna, Children's Colorado and The Gene Team who supported this project (The Gene Team bikes hundreds of miles through the Rocky Mountains every summer to raise money to support projects that help the families in the Genetics and Inherited Metabolic Diseases Clinic at Children's Hospital Colorado and beyond). The book is beautiful in so many ways and would not have been possible without you!

Please consider ordering one for your family and maybe even order an extra copy to take along to your next metabolic appointment that can be passed on to a family just receiving an MCADD diagnosis with their infant. How cool would that be if every new MCADD baby got to bring home a book!

Meal Stress

I have a confession to make...my MCADD kid has terrible table manners!  I blame myself.  Really, it's all my fault.  When my older (non-affected) son was this age and he would start throwing food off his plate or dropping his plate on the floor, I would just take it away.  If he's playing with his food, he must not be hungry enough to eat it.  Wipe off the child and set him loose to play.  Issue closed.  Food throwing situation solved in relatively short order.

With LB, I haven't been able to do that.  Even if he's throwing his food or throwing his plate, cup, fork, etc., I haven't been simply wiping him up and setting him loose.  I've been attempting to have him continue his meal because after all, he needs to eat, right?  So, meal after meal, we are enduring chunks of food flying past our heads and utensils landing on our plates and oodles and oodles of food wasted on the dog (who may very well need to be put on a diet soon). 

For the longest time, we tried to ignore it thinking no reaction from us would make him think food throwing wasn't much fun.  Or we would take his plate away for a minute, remind him that we don't throw our food and then give it back to him only to have him go right back to throwing.  Needless to say, mealtimes have gotten very stressful.  It's a dangerous spiral -- he throws food because he isn't hungry, we want him to eat any morsel of food possible so we let him continue to throw food and then we get stressed that he's not eating the food, he's only throwing the food and we stack more food onto his plate in an attempt to get him to eat something, anything and he just keeps throwing food anyway.   

Thankfully, if he doesn't eat a good dinner, we always have the bedtime snack to fall back on, but even then it's a matter of chasing him down and popping graham crackers in his mouth anytime he slips out of perpetual motion. He's constantly busy zooming through the house and really can't stop and be bothered to sit down and concentrate on eating.

We know that he's healthy and developing appropriately, so he must be getting plenty of calories, but this mealtime madness thing is really getting hard (and rather embarrassing when we eat in places other than our own home). Does anyone have any good tips for getting through it?  We are all ears!

Fifteen Months

It has been a few months since I've done a monthly update, but there's so much going on in LB's life right now, I couldn't help but give a little update.

Other than a couple typical toddler runny-nose incidents that were snotty and messy, but didn't seem to bother him one bit, he has remained 100% healthy.  Hooray!

We're starting to get a lot more words.  I read a few articles that mentioned children with MCADD can have speech delays and since LB wasn't really talking at all, I was starting to wonder whether I should ask the pediatrician about it.  Then, all of a sudden, we went from a vocabulary of "this," "that," "yes," and "daddy" to a whole lot more words -- ball, catch, dog, juice, night-night, mommy (hooray!!), kiss, and probably a few others I'm not remembering right now.  In any event, I'm a whole lot less concerned about his speech than I was a month ago. 

He is definitely a boy on the move!  He has been walking since January, but is very adept at it now.  He can walk/run easily on all surfaces and doesn't trip and fall all that often anymore (this makes me happy because one of my least favorite things is when my boys face-plant on the concrete - it just makes me cringe to watch it and not be able to stop it).  He is also discovering climbing and can successfully work his way up onto all sorts of high furniture - the kitchen table, the couch, the beds.  He has also figured out that if he can't get up onto something, he can always go grab the step stool from the bathroom (the one his older brother uses to reach the sink) and set it beside the item he'd like to climb -- voila! 

His favorite toys right now are all things sports. He loves all types of balls -soccer, foot, basket, base, bowling, golf, etc. - along with all the long sticks that accompany them.  He can swing a mean golf club with pinpoint accuracy on your shin.  He can play catch with us for 15 minutes at a time and not tire of it one bit.We are convinced that he is going to be our sporty kid because he is literally obsessed with this stuff.  Thankfully, I've been very encouraged by stories of families with older MCADD kids who are successfully able to participate in sports, so the thought of him playing a sport doesn't make me as uneasy as it used to.

He also loves dancing and will bop along to music.  He loves books and brings them to us one after another, happily pointing at the pictures and nodding along to the parts of the story that he apparently agrees with most.  He gives the most wonderful drooly open-mouth kisses while saying "muuuuaaa!" that absolutely melt my heart.

With all of his activity, I'm very grateful that he is eating well and enjoying a wide variety of food. One of his favorite meals is our family's tradition of Saturday morning oatmeal pancakes. He also loves things like cottage cheese, apples, PB&J, pineapple, macaroni and cheese, butternut squash, meatballs, just about any recipe for chicken, tortillas, hummus and lots of other fruits, veggies and grains.  

We've figured out a good bedtime snack system that's working pretty well.  Since our dinnertime is usually pretty close to bedtime, it was hard for us to get him back into the highchair for a sit-down snack so soon after dinner at a time when he'd much rather be romping around with his big brother.  So, now we're giving him a sippy cup of whole milk and a snack cup about a half hour before his bath that he can carry along with him during the romping. The snack cup contents vary, but it's usually some kind of whole-grain cereal or crackers (cheerios, graham crackers, peanut butter sandwich crackers, belvita breakfast wafers, etc.).  He'll drink and snack pretty happily, but at the point that he starts spitting the crackers out, we know that he's full to the brim and we don't force him to eat any more. However, we will hold onto the sippy cup of milk and let him drink that as we're reading his bedtime stories (the last step of our bedtime routine before lights out). 

We have officially weaned him off of the bottle during the waking hours, but he will sometimes have a small 4 oz bottle of toddler formula when he wakes up hungry at night.  During most night wakings, he will soothe himself back to sleep within a minute or two, but on the nights where our evening routine was a bit off (which happens to every family from time to time), or nights that we know he didn't eat as much of his dinner and/or bedtime snack as he normally does, we'll happily mix him up a quick bottle when he wakes (we keep the can of formula in our room along with a pre-filled bottle of water). If his fussing lasts longer than a minute or two, it's usually cured quickly with a small bottle. Sometimes he'll drink it all and be satisfied and sometimes, he'll only drink a few ounces before pushing it away and snuggling back into sleep.

Sleeping has really improved in the last few weeks, too.  About a month ago, he had slept through the night a total of 5 times, but now he's sleeping through the night much more often - at least 2-3 nights per week.  He's been working on cutting some molars, so that might actually be factoring into the sleep disturbances more so than the poor sleep habits we've established through his first year MCADD feeding schedule.  I have friends with children of similar age (not affected by MCADD) who are experiencing similar night restlessness due to teething, so it makes LB's sleep seem much more "normal."   

All in all, things are going great and we're excited for all the fun we'll have this summer!

The New Normal...Without an Alarm Clock

As I mentioned in a recent blog post, I had a little bit of trepidation about no longer tracking LB’s feedings.  After our latest round of doctor’s appointments, all signs pointed to me needing to let go and just let him be a normal kid.  He can now have a normal kid day for meals – breakfast, morning snack, lunch, afternoon snack and dinner (kids already eat a lot don't they?) – without me writing anything down.  He can sleep for 10-12 hours at night without being woken up to eat. Granted, we still need to have a general idea that he is eating regularly throughout the day and we’ll still need to keep close tabs when he is ill, but we don’t have to track every morsel quite so closely on a day-to-day basis when he is well. 
 
Our older (not affected by MCADD) son had given up his middle-of-the-night nursing and was regularly sleeping through the night by the time he was about 6 months old.  Although it was only less than 3 years ago, the concept seems completely foreign to me now after parenting a child with MCADD.  “He can really sleep ALL NIGHT LONG?” I asked our metabolic specialists.  “Yes, he can safely go for 10-12 hours now,” they reassured me.  Wow, 10-12 hours is kind of like an eternity when your last year has consisted of gradual steps forward from feeding the baby every 2 hours, then every 3, then every 4…  Whatever will we do with this much sleep?!

This new philosophy on his feedings has been both terrifying and liberating.  Even though our trusty alarm clock doesn’t ring at 3:30 a.m. anymore, my internal alarm clock still does.  I still wake several times during the night with my first inclination being to look at the clock and figure out how long it has been since LB’s bedtime bottle.  Instead of springing out of bed to feed him, I now have to talk myself out of going in and feeding him just in case (which I am successful at doing about 95% of the time) or merely creeping into his room to check on him (which I am successful at doing about 50% of the time). 

It has only been a week since we stopped keeping track of his food intake in our notebook and stopped setting the alarm at night and I’m already sensing some differences.  To all outward appearances, I’m sure he’s just a normal toddler to everyone else, but knowing that I can hand him a sippy cup of milk without measuring how much is in it or calculating how much he drinks is a definite source of relaxation for me.  I'm starting to see LB as a regular kid instead of as a baby to be cared for with extreme precision and diligence.  In addition, I’m starting to see LB relish his overnight sleep.  He is still waking once per night (and I’m still feeding him when he wakes up), but he seems to be sleeping more restfully and waking up more happily with each passing day. 

Our plan forward is to let LB drop the middle-of-the-night feedings on his own.  If he wakes up, we’ll give him 4 oz of formula (which is quicker and easier to deal with in the middle of the night than having to run to the kitchen for milk). Our older son dropped his nighttime feedings on his own without crying it out and I’m confident that LB will do the same when he is ready.  It might take a while considering how artificial his overnight sleep schedule has been to date, but I know it will happen eventually.  It’s only been a week and I’m already seeing him rest more peacefully, so it might be sooner than we think, too.

TIP - Hearty and Healthy Snacks for Bedtime or Anytime

As I mentioned, we got an excellent list of snacks from our metabolic clinic at LB’s last appointment.  These snacks were recommended by a dietician as a healthy way to keep filling and nutritious food in the system of someone with a metabolic disorder like MCADD.  As always, you should check with your doctors before feeding anything new to your little one…and you should only feed age-appropriate items.

A snack can be thought of as a little meal, rather than a treat.  Healthy, balanced snacks should include a source of protein and a source of carbohydrate. 

Example Proteins (can be fat free or low fat):

• ¼ cup cottage cheese
• 1 oz cheese
• 4 oz low sugar/carbohydrate yogurt
• 4 oz milk
• 4 oz infant formula
• ¼ cup beans
• ¼ cup hummus
• 1 slice lunch meat
• 4 oz pudding (made with milk)
• ½ egg or 1 egg white
• 1 oz tofu
• 1 Tablespoon peanut butter
• ¼ cup nuts (non-sweetened, not honey roasted)

Example Carbohydrates:

• 1/3 – ½ cup unsweetened cereal
• ½ - 1 mini bagel
• ½ banana
• 1/3 cup oatmeal
• 1/3 cup porridge
• 3-5 whole wheat crackers
• 2-3 rye crisps
• ½ whole wheat pita bread
• 1 slice whole grain bread
• 2 graham cracker squares (not sugar or cinnamon)

 
Put it all together and you get some great snack ideas, especially for the much-needed bedtime snack:

• Unsweetened cereal (like Cheerios) and milk
• Unsweetened cereal and yogurt
• Graham crackers and milk
• Whole wheat crackers and cheese
• 3 graham cracker squares with 1 tablespoon peanut butter
• 1-2 slices low fat cheese and/or lunchmeat and 4-5 crackers
• ½ sandwich (1 slice whole wheat bread with 2 tablespoons peanut butter or 1 slice deli meat and 1 slice cheese)
• ½ English muffin with light cream cheese or peanut butter
• Mini bagel with light cream cheese or peanut butter
• Pizza bagel or pizza English muffin (1 tablespoon of tomato or pizza sauce and 1 oz low fat mozzarella cheese on half a bagel or English muffin, warmed or toasted)
• Celery sticks and 2 tablespoons peanut butter
• ¼ cup cottage cheese plus ½ cup pineapple or 2 pear halves or 2 peach halves
• ½ small banana with 1 tablespoon peanut butter
• Granola bar that contains fiber and protein

Happy snacking kiddos!

Metabolic Appointment

We just had our one-year appointment with the metabolic clinic and in many ways, it felt like a victory celebration.  I remember at our very first appointment, they told us that the first year would be the most difficult, but that LB would be more and more like a "normal" kid once he reached that milestone.  He would be able to go longer between meals as his body grew and matured.  He would be able to sleep through the night.  He would be able to eat on a more normal schedule (instead of every few hours around the clock). All of this is true now.  When he is ill, we still might need to wake and feed him, but when he is well, we were given the green light to let him sleep for 10-12 hours at night (provided he eats well during the day and has a hearty bedtime snack).

His metabolic specialists are not recommending any special diet for LB.  They did give us a great list of healthy bedtime snacks that I will post later, but other than that, we were told to feed him a normal, age-appropriate diet.  Apparently, they used to recommend a low fat diet for children with MCADD and refer them to a dietician, but they don't see a need to anymore. They said he could transition from formula to milk and didn't need to move into a toddler formula for any period of time beforehand. 

I have read a lot about children with MCADD being given cornstarch in milk as a way to maintain blood sugar, but when I asked about it, they also said it was unnecessary for LB.  They mentioned that cornstarch is often recommended for something called "brittle MCADD" where children with MCADD also have an on-going issue with low blood sugar.  LB doesn't seem to have any issues with blood sugar, so as long as he's eating normally, there wouldn't be a need to dose him up with cornstarch for a blood sugar boost.  We also, of course, still have the polycose in the cupboard which we can use to introduce additional sugar to his diet during times of illness.

They are doing some bloodwork to check his carnitine levels (which they do at every appointment) and they will let us know the results.  If it is low, we may need to supplement him with oral carnitine, which we already have on hand to use during times of illness.  We should know that within the next week.

Speaking of bloodwork, we finally got around to talking about my acylcarnitine profile results, too. Everyone in the family had a acylcarnitine profile performed shortly after LB's birth to confirm that no one else in the family has MCADD. My husband's bloodwork and our older son's bloodwork came back stone-cold normal. Mine came back with two abnormal results - an elevated C4 and C8. They told us that it wasn't MCADD, but I kept forgetting to ask at our other appointments and I was still curious what it might be (it just seems strange that such an unusual bloodwork panel would come back abnormal for someone who is seemingly healthy, but who has a child with MCADD). They surmised that it may be indicative of my carrier status, but that it wasn't anything to be concerned about. It definitely wasn't MCADD.

We spent quite a bit of time talking about genetics at this appointment, too.  The doctors inquired whether we were planning to have more children and at this point, we're just not sure.  We think we may be done, but there is what we keep referring to as "that nagging 1%" chance that we might try for #3.  They told us that there were a few options for pre-natal testing to diagnose MCADD.  We could opt to do in-vitro fertilization and select only the embryos that are not carriers of MCADD for implantation or we could conceive naturally and take our 1 in 4 chance that our next child would be affected by MCADD (and 2 in 4 chance they would be a carrier or 1 in 4 chance that they would be neither affected nor a carrier).  If we conceived naturally, we would be able to test for MCADD using an amniocentesis or we could wait until after birth to conduct a blood screening.  If we waited until after the baby was born, they would recommend that we feed formula and do the blood test immediately to make sure that the baby would be safe until we knew his/her MCADD status for sure.  We would not have to wait for the traditional newborn screening test results to find out, although the screening would still be conducted.

We also got the results of LB's DNA analysis.  They screened for only the common mutations and found that LB has one common mutation and one uncommon mutation that they were unable to locate within the bounds of the DNA mapping that they performed.  We would need to talk to our insurance company to discuss the cost of a full DNA analysis to find LB's other gene mutation and it could be pretty expensive.  Apparently, insurance companies tend to balk at paying for this level of testing since it doesn't change the course of treatment, so it would likely be a hefty out-of-pocket expense for us.  We agreed that we would not pursue the full DNA analysis at this time, but that maybe in 20 years when our kids might start thinking about kids of their own, they may choose to pursue that more extensive testing to determine 1) whether our oldest son is a carrier and if so, of which mutation and 2) what the uncommon mutation is for LB.  At that point, DNA mapping will probably be a whole lot less expensive, too.

In all, it was a great appointment.  It's always great to hear that your child is doing well and developing completely on schedule.  Considering how worried we were the first time we set foot in the metabolic clinic one year ago, this appointment was a breeze.  We'll only need to be seen every six months going forward (another benefit of LB getting bigger and out of the critical and worrisome first year).

Tracking Food Intake and Transitioning to the Next Stage

We have a notebook that lives on our kitchen counter.  It is filled with dates and times and ounces.  We know and have recorded every ounce of formula that LB has eaten ever since he was born. They pretty much make you keep track of feeding times (and dirty diapers) in the hospital and considering we found out about his MCADD on our first night home from the hospital, we never stopped tracking his feedings.  For awhile, we were writing them on a white board in our kitchen, but after a bunch of traveling this summer (sans whiteboard), we shifted to the notebook method. 

At times, I've felt like this is a bit over the top and that maybe we were being too rigid, but we also felt like it was important for us to track his food intake like this so that we could know for certain if he was falling below that "rule of thirds" (where the metabolic specialists say that eating one third less than he normally does in a day could indicate the onset of a problem).  When illness strikes, one quick look at the trusty notebook and it is very easy to tell his specialists that he typically eats 30 ounces per day and today he's only eaten 18, as opposed to wracking our brain and trying to piece it together during an already stressful time. 

Now, I fear that we've become a little too accustomed to it.  With the end of formula and bottles and easily measured food intake on the horizon, losing the notebook method has me a bit apprehensive.  Granted, we do not track his solid food consumption in the notebook, but we still have a sense of his intake for the day by tracking his formula intake.  Without that, will we know when he's not eating enough?  Anyone have any good advice for navigating this transition?

Ten Months

Here is our happy, 10-month old boy!

 

 

Despite a trying few weeks, he is still having fun and doing great.

Although I must say that it is getting much more interesting to take these monthly photos now that he is so interested in my signs...

Here are some milestones from the last month:

• He is learning to communicate with gestures and body language. He can shake his head from side to side for no (as in "no, Mommy, I don't want you to spoon any more peas into my mouth"). He can also bob up and down for yes (as in, "yes, Mommy, you are quite silly and entertaining trying to get me to eat my dinner"). 

• He is working really hard on forming words. We get a lot of "lalalala" noises now (accompanied by sticking out his tongue) plus he is also trying very hard to say either Daddy, down or dog. We're not quite sure yet, but it is a definite word attempt.
 
• Food has been a bit of a battle recently. The pediatrician recommended we keep him on soy formula in case the gastrointestinal upsets of late have a milk allergy linkage (he still thinks it's viral, but just in case) and we're avoiding all dairy for a bit until allergies are ruled out. He is OK with the soy formula, but turning down pretty much anything that comes on a spoon. He would much rather feed himself with his fingers, thank you very much. So, we've been doing lots of steamed butternut squash, steamed broccoli and cauliflower, steamed carrots, noodles, canned fruit, bananas, some avocado and even some very finely chopped chicken breast and fish. Dinners are a messy, messy affair, but sometimes learning how to feed yourself isn't pretty, I guess. Good thing we have the dog to keep the floor under the high chair clean.
 
• It's also a good thing he has more teeth to chew all of these new foods. He sprouted the two top left ones just before the new year and has the two top right teeth on their way shortly. One of them is just starting to poke through. We're noticing that he's always feeling around his mouth with his tongue, so I'm guessing that he's pretty intrigued by this whole teeth thing, too. 

• He is quite stellar at getting from place to place. He crawls as fast as can be and is also very proficient at cruising. He can navigate an entire room with just barely one hand holding onto the furniture or walls. Put him behind a walking toy or a laundry basket and away he goes on two feet without batting an eye. He will even walk with us holding onto only one hand, although he prefers when we let him hold both hands. He is experimenting with taking steps on his own without holding onto anything, but so far we've only caught him doing this a handful of times -- once he forgot to hang onto the couch and took two steps toward me before realizing he wasn't hanging on (once he realized this it was like a looney toons cartoon where the character realizes he's run off a cliff and immediately starts the plummet - in this case, he dropped to his bum to crawl the rest of the way). The other time, he wanted to get from the couch to the loveseat and made the 2 ft stretch between the furniture after calculating in his mind whether he could do it. He was quite pleased when he did. 

• One of his best movement-related stories is his love for tackling his big brother and his Daddy whenever he catches them laying on the floor. We'll hear him giggle and then it's off to the races before he's crashing onto someone with an enormous smile on his face. He even tackled the dog a couple days ago (she got up and moved to the other side of the room).
 
• He can stand on his own for about 10 seconds at a time, but doesn't seem inclined to stand for much longer. I think it's just that he's too impatient and wants to get onto the next thing instead of standing still.
 
• I'm getting a little worried about his weight gain since he is essentially the same weight now as he was at his 9 month appointment, but with two major stomach bugs and 5 days in the hospital this month, it was probably inevitable that he would lose a little weight. Fingers crossed that we stay well for awhile and can get him moving in a good direction again quickly.
 
• We are finally getting some relief on the sleeping front. I don't know if it was the comfort of having me lay next to him every night while we were in the hospital that taught him that sleeping was actually pretty nice or if it was the cozy new flannel crib sheets we bought for his bed or if he's just been too tired to stay up all night protesting sleep after all that he's been through these last few weeks, but we have actually been getting rather consistent 6 and 8 hour stretches of sleep at night - which is incredible compared to where we were about 6 weeks ago when he couldn't sleep for an hour at a time without needing someone to come comfort him. Fingers crossed that this continues on a good trajectory, too!

Yet Another Trip to the Hospital

Yes, you read that correctly.  We were in the hospital with LB.  Again.  That's twice in two weeks if you're keeping track.  No fun at all.  We are very ready for cold/flu/gastrointestinal nastiness season to be O.V.E.R!

This bug started on Thursday afternoon.  LB had been doing just fine all day.  I picked him up from the sitter.  She told me he ate well, napped well and had just finished a 4 oz bottle at 3:30.  We loaded into the car, drove 5 minutes home, unloaded from the car and were taking off jackets when Husband announces that LB just threw up in the hallway.  He has never vomited before, but he spits up a lot (just like our older son did when he was a baby...hence why all of the baby pictures of our boys have them wearing bibs...usually wet ones), so I was surprised.  Sure enough, there was LB sitting in a pool of vomit.  Gross.

Vomiting is something that's very dangerous with MCADD because the food doesn't get digested and he is essentially fasting (which is bad).  I called the sitter to double check when his last meal was before 3:30 because now we couldn't count the 3:30 feeding since it had just puddled in our hallway.  She said he ate his last bottle at 11:15, just before he ate some solid food for lunch.  It had now been a little over 5 hours since his last meal.  We were good for another 3 hours, but that meant he had to keep down his next feeding.

Thinking it may have been a fluke, we made a bottle for him and he prompty threw it up.  We waited another 30 minutes, tried again and he threw that up so we called the metabolic clinic.  They told us to start his polycose solution.  He needed to eat 0.5 ounces of polycose every 15 minutes for the next hour in order to stay in the clear and we needed to call them back in an hour to let them know how he was doing.

In the next hour, he drank a whopping total of 0.25 ounces of polycose and threw up 3 more times.  We were already packing our hospital bag when we called the metabolic clinic back.  It was about 7 pm when we got everyone into the car.  We left a key with the neighbors to let the dog out in the morning and we changed our older son into his jammies and dropped him off for a sleepover night with his sitter (at his choice -- we asked him if he wanted her to come over to sit with him for awhile or if he wanted to go sleep at her house and he wanted to go there).  This ended up working out nicely because this meant Husband could stay the night with us and since our plan was to go directly to Children's Hospital to avoid another IV incident like we had at the other, closer hospital, it was going to be about twice as far for Husband to run back and forth from the hospital to home.


When we got to the hospital, I was a little worried because there was a waiting room FULL of sick kids waiting to be seen.  Thankfully, as we were getting checked in with the desk, they already had LB's file ready and the metabolic clinic had already called ahead to let them know we were on our way.  After getting checked in and giving them our emergency letter, we sat in the waiting room for less than 5 minutes before we were called back.  This was good because it was already after 8 pm and it was rapidly approaching 8 hours since LB's last feeding (and he can only go 8 hours without eating when he's well anyway). 

We were seen by the doctor quickly and the IV situation was much improved at this hospital.  The nurses were able to draw bloodwork with the first poke (his inner elbow), but weren't able to advance the catheter to start the IV at that site.  They poked him a second time (left hand) and the IV was flowing.  Not bad.  Now, LB just had to keep up his end of the deal -- stop vomiting and start eating. 

Unfortunately, the second part proved a bit more difficult.  He was still vomiting in the ER and he wasn't very excited about drinking anything (do you blame him?).  So, within a couple hours of walking through the door, we were admitted.  It's a good thing I packed extra clothes this time because all of the vomit was landing right on me.  The vomit was all polycose (which is essentially sugar water), so as it dried, it was like I had patches of heavily starched clothing that was stiff as a board.

Overnight was a bit better.  The anti-nausea drugs they were giving him through the IV quelled the vomiting and he was eating a little bit every couple of hours.  I sat holding LB in a recliner.  Husband stretched out on a very uncomfortable couch.  The nurses woke us every couple hours, but that was a good thing.  We were both so tired, I'm sure I would have slept through any alarm I would have set.

In the morning, we were seen by a gaggle of pediatric residents (they literally have 8 people on a team since it is a teaching hospital and all 8 of them crammed into our room to chat with us) and a doctor from LB's metabolic clinic.  They all agreed that as long as he was eating 2/3 of what he normally does and didn't have anymore vomiting or start having any diarrhea, we would be going home that day.  He had likely caught another gastrointestinal illness and it would work its way though his system just like the others had done.  As long as he was eating, he would be fine.

By mid-day, LB was going about 100 mph.  He wanted to play.  He wanted to crawl (which he couldn't do on the hospital floor -- ick!).  He wanted to pull on that thing that was attached to his left hand.  Keeping him from tugging on the IV was very difficult this time because it was so accessible by being in his hand.  He was chewing on it, tugging on it, trying to use that hand even though it was splinted...  Needless to say, at about 1 p.m. I noticed his splint was sopping wet. It was too wet for it to be just from him sucking on it when we weren't looking (trust me, we were looking about 99.9% of the time), so I called in the nurses to take a look at it.  Sure enough, he had dislodged something and as they were attempting to salvage it, he was squirming around so much that they lost it.  No more IV. 

However, when the docs came back to check on us they were unconcerned about the IV.  They wanted to see him eating solely on his own at this point anyway and we were on target to get out of there by dinnertime.  We stopped at the pharmacy to fill his prescription for more anti-nausea drugs (just in case he started vomiting again) and were on our way home.

We picked up our older son first thing and he was very happy to see us and he told us about how much fun he had sleeping at his sitters house.  We had forgotten to pack his toothpaste, so he told us about how her toothpaste tasted different.  That was the extent of his "trauma" from this experience.  He was fine...until he wasnt.

Last night, he started vomiting.  LB was on the mend and now his big brother was just getting started.  Oh well, back to toast and gatorade for us again.  At least we're home.

Husband and I have been wracking our brains trying to figure out how the kids keep getting sick like this. Sure there has been a lot of talk on the news lately about flu and nora-virus outbreaks in the area, but we literally haven't left the house since Christmas.  We have been to ZERO public places...except for church.  We went to church the Sunday before each illness started.  As much as it pains me to say it, in the interest of keeping ourselves AWAY from the hospital for the rest of this unprecendented season of viruses, we are going to start avoiding all crowds for the foreseeable future, including church.  Two hospitalizations in two weeks and three in the last three months is plenty.  I have no plans of adding to that total anytime soon.

Second Hospitalization - A little scary

LB caught another stomach bug.  He had a couple messy diapers for both us and the sitter on Tuesday, but he was happy and eating just fine (26 ounces of formula, plus three meals of solid food and a nice bedtime snack).  As I was rocking him back to sleep very early on Wednesday morning, he had a diaper blowout that required both of us to change our clothes, so we kept him home on Wednesday to keep an eye on him. Once again, he was his usual happy self and eating just fine (28.5 ounces of formula, plus three meals of solid food and a nice bedtime snack). 

Thursday morning, he had three messy diapers before breakfast so he once again stayed home with me.  Although he was still happy and eating just fine, he was getting a little bit upset about the nasty diaper rash he was developing. I was getting a bit concerned about the volume of diarrhea he was having because it was to the point that I could barely throw the diaper outside in the trash and get my hands washed before we were changing another one.  By the afternoon, I called his specialists at the metabolic clinic to see what we should do.  The last time this happened, he had messy diapers, but he was also not eating. He was eating just fine this time, so I wasn't quite sure what to do.

After hearing our saga, the doctor immediately said that LB was too young to be losing that much fluid.  She wanted him to have lab work done immediately to check his levels and the only place we could go at that time of day (5 p.m.) was to the emergency room.  She asked where we were going so she could call ahead and warn them that we were coming.  I packed the diaper bag with lots of formula, wipes, bottles and our special can of polycose (sugar solution) and our prescription of carnitine that has been waiting in our cupboard for use when little brother is sick and headed for the ER.

When we arrived, they were waiting for us and we were ushered back into a room quickly.  The pediatrician who saw us had already talked to the metabolic specialist and had ordered his needed labs and IV fluids.  She said she was surprised that LB looked so good.  She had expected him to be lethargic and dehydrated, but here he was laughing at her, drooling and trying his darndest to wiggle out of my arms so that he could explore our new surroundings.  Things seemed to be going well, although she warned us that we likely wouldn't be going home right away since the metabolic doctor had advised her that we should be admitted for observation and fluids.

Things started to get worse when they came to start his IV.  It was a team of two nurses.  One nurse tried the inside of his left elbow with no luck.  The other one tried the inside of the right elbow, again with no luck.  Then, the first nurse said she would go get another nurse.  The third nurse I recognized as the one who was able to get his IV in on the first try the last time we were in the ER, so I felt confident that this would be accomplished quickly.  I was wrong.  She tried his left elbow and his left hand with no luck either.  We were up to five pokes and there was a bag of IV fluid that he needed sitting right there on the counter.  I was beyond frustrated that they couldn't find a single vein.

A little time passed and Husband arrived to be my emotional support since our older son was now asleep with the neighbor watching him.  They called down two nurses from the floor, one from the PICU and one from the NICU.  They tried to get veins in his ankles on each foot without success either.  We were told that babies this age were hard in the first place because of the layer of baby fat they have.  LB was extra hard because he was dehydrated and that made it even more difficult to find a vein.  We were now at seven pokes.  Still no IV.

Fortunately, he was still drinking just fine, so the pediatrician in the ER called the metabolic physician and asked if we could attempt to have him maintain on his own orally.  She agreed and gave us instructions for mixing his polycose and carnitine with pedialyte.  He needed to drink a minimum of 2 ounces every hour and that he did.  Once the 8 tablespoons of polycose was added to four ounces of pedialyte, we had 5 ounces of liquid that he happily downed every time a bottle of it was presented to him.  I'm sure it was like drinking a pixie stick.  I was worried that he would never drink formula again by comparison.  We were admitted to the pediatric unit shortly after midnight and I spent the night sleeping on the hospital couch with LB and waking every 2 hours to feed him his sugar solution.

By the morning, his diarrhea still had not subsided and his labs looked bad.  Really bad.  The physician on the floor said that even if she didn't know he had MCADD, his labs indicated that he needed IV fluids.  He was losing more fluid than he was taking in and at the rate of loss (3 diaper explosions per hour at a minimum), there was no way he could make up that ground just by drinking.  Even an adult couldn't do it.  They needed to get an IV in.  So, they called in some more nurses from the PICU, each of whom poked him once and neither of them got the IV in either.  We were up to nine pokes now and I was practically hysterical.  I couldn't stand to see anyone else dig around under his skin for  vein while he wailed in pain.  I knew he needed the IV.  I knew they were just trying to help him, but it was still so hard to watch.  I hated seeing him hurting. 

The doctor said it was time to start thinking about plan b -- possibly a central line or possibly a pic line -- because he needed those fluids badly and the longer we waited, the worse it was going to get.  Now, I was really scared.  It was hard enough to watch the IV attempts.  Now, they were talking about sedating him and taking him to the OR?  And the scariest part was that no one was telling me reassuring things like "he'll be ok" or "he's still doing fine."  The conversations with the nurses and the doctors all said one thing -- "he needs IV fluids fast" -- yet no one seemed to be able to help him.

At this point, LB was exhausted and had fallen asleep in my arms.  The doctor said to let him rest for a bit.  They would try one more time for a peripheral vein with their very best person and that they would do everything they could to stack the deck in his favor (warming his hands and feet with warming pads, taking him to the procedure room on the floor where the light was best, looking at all the options for veins before taking the very best option).  If it didn't work this time, we were going to get serious and head to the OR.

The entire time LB was napping in my arms, I was praying.  I was sobbing and I was praying.  I called/texted/facebooked everyone could think of and asked them to pray for LB.  I needed some divine intervention in the form of someone who could get an IV in him.  I didn't want to see him go through a procedure like a central line.  I needed to see that bag of IV fluids that had traveled with us from the ER to the floor doing him some good.  I needed to know that he was going to be ok.

Shortly after noon, his nurse came in and said it was time.  I took a deep breath and carried him down the hall to the procedure room where we met Heidi, the nurse from the NICU who was our last hope.  She looked closely at all the options she had to place the IV before deciding on his left foot.  I laid him down on the table and held him securely while singing a song softly into his ear.  I couldn't watch again.  He cried and I sang to him.  After a while, I heard Heidi ask if she should draw the labs, too.  I looked up and asked if the IV was in.  Heidi smiled and said she had gotten it on the first try.  I was so happy that I started weeping.  He was finally going to be ok.  He was finally going to get the fluids that he needed.  Our prayers had been answered. 

We returned to our room and set about letting the IV fluids work their magic.  They wanted at least 12 hours of fluids, so we would need to spend another night in the hospital.  They also wanted to give his intestines a break, so they switched him to a special lactose-free formula.  They said I could feed him as often as he was hungry, but only 2 ounces at a time and to try and space the feedings at least 30 minutes apart so we weren't overwhelming his system with large quantities of liquid that would inevitably just go right through him. 

It was hard to keep him happy with only 2 ounces at a time, especially because I was also preventing him from practically all physical activity to protect that IV site from any damage.  I had a stir-crazy baby who hadn't been allowed to crawl, cruise or walk since Thursday afternoon or inspect the contraption wrapped around his left foot, despite the fact that he was being fed pure sugar for 24 hours before being switched to intravenous sugar that afternoon.  Let me just say, it was an interesting stretch of time.  Thankfully, Husband brought us a nice assortment of books and toys and the floor had a highchair that they had brought to our room.  It ended up being a rotation of me singing songs to him while holding/bouncing him around the room, followed by a stretch of sitting in my lap reading books, followed by sitting in the highchair playing with toys, followed by a bottle and sometimes a nap.  We did whatever kept him happy and prevented him from pulling at his IV and it worked.

By Saturday morning, his diarrhea had subsided and his labs were looking much better.  His sugar numbers were normal.  His acid numbers were normal.  His salt numbers were high though so they disconnected the IV since he was probably getting too much salt through the combination of IV and liquid intake by mouth.  We were told that they would repeat the labs that evening and probably send us home if he continued to feed well, continued to have normal stools and had good lab results at the end of the day.  We counted the minutes until the labs were drawn and were ecstatic when the results came back normal.  We were going home!  Three days before Christmas, we were headed home!

That night, as Husband and I tucked our boys, safe and warm, into their very own beds, under our roof, we were profoundly grateful.  While LB and I were in the hospital, our older son was home with Husband suffering from a similar stomach bug.  In all, the last few days in our lives were not pretty -- hospitals, a crying baby getting poked for IVs and blood work, dirty diapers, vomit, you name it -- but even if we only ate toast on Christmas and even if no one in the house felt well enough to change out of their pajamas, it was going to be just fine because we were going to all be home together.  I didn't need anything else for Christmas, just the ability to be home with my boys.  My Christmas wish had already come true.

Eight Months

Eight months is a very mischevious stage...

We like to take books and toys off the shelf (and toilet paper off the roll).

We do lots of exploring in the kitchen.  My tupperware drawer is strewn all over the floor in the background and he is sitting in the oven drawer.

He "helps" with all household chores.  His favorites include vacuuming, loading/unloading the dishwasher and being carried around in the laundry basket while I do the laundry.

He is a champion at pulling himself up to stand and cruising around the house holding onto the walls and furniture.

We've reached the stage in his development where we put him down and within 3 seconds, he is getting into something -- the dog food, the toilet paper roll, pulling things out of the drawers or the pantry, trying to "help" his big brother use the potty, etc.  He is a speedy little one, that's for sure!

He loves following his big brother around and the dog, too.  She is a great sport as he uses her collar to pull himself to standing.  We really couldn't ask for a better dog.

His favorite toys are anything that rolls.  We have some great pull-back racecars that he thinks are funny, and a cute dumptruck with three balls that rattle when you shake them.  He likes to roll the balls down the incline of our treadmill and he has no fear whatsoever as he crawls up and down the treadmill (while it's turned off, of course) chasing them.

He's still a great eater and is usually eating around 30 ounces a day, plus 3 meals of solid foods (baby food at lunch and dinner and cheerios at breakfast).  We are also doing a bedtime snack of Cheerios and it's working out nicely because we are using the Cheerios Fun Book as a bedtime story.  We read the story and he munches the cheerios that serve as wheels on the car, buttons on the pajamas, etc.

We still don't have any relief on the sleep front yet, but I'm hoping it is soon. Maybe we just need to give those antacids more time to work.

Six Months

I can't believe it!  Our little boy is already half a year old! Where does the time go?

We went for his 6 month appointment yesterday and he is still doing great.  Developmentally, he's right where he should be, a little ahead, in fact.  For height and weight, he's still lingering in the middle of the pack.  He was 16 lbs, 10.5 oz (45th percentile), 26 3/4 inches tall (50th percentile) and his head circumference was 17 inches (50th percentile).  He jabbered away to the doctor all through the appointment along with trying to eat his stethoscope.  The only minor concern we had for the appointment was that he's had red eyes and a runny nose for a few days.  We were wondering if he had a cold, but the doc suspects it is seasonal allergies.  He told us we could give him 2 mL of Benadryl if the stuffiness is affecting his eating or sleeping.  Considering he has been waking every 40 minutes for the last week, we jumped on the opportunity to give him some Benadryl last night (everyone needed a more restful night of sleep) and he had his first full night of sleep since vacation, save for the 2 a.m. feeding we have to set the alarm clock for.

We were a little nervous about all the vaccinations (he was so mad after his 4 month appointment that he stopped eating to within an ounce of the point that we need to take him to the hospital), but he seems to have done fine this time around.  He even got a flu shot on top of all the other shots and he was still just fine.  Thank goodness!

He is officially crawling.  It started about 3 weeks ago with some partial army crawling, partial frog hopping, partial belly off the ground crawling.  It wasn't pretty, but it got him where he wanted to go with a great deal of effort.  As soon as we returned from vacation (4 days after he turned 6 months old), he had put things together into a respectable crawl -- look out world!

He can sit on his own for long stretches of time.  He's still trying to master getting from sitting to crawling, but he's getting better at it.  While we were on vacation, he tipped forward from sitting to reach for a toy and he forgot to catch himself.  His poor little face hit the hardwood floor and he cried and cried.  By the time he lifted his head from the safe comfort of my shoulder as I comforted him, there was a little trail of blood running from his nostril.  His first bloody nose.  =(  He went right back to playing after a little snuggling with Mommy, but he does seem to be being more careful when he tips forward -- thank goodness!

He's doing very well with eating.  He still takes about 32 ounces of formula per day, but it's starting to be in more 6 oz servings instead of all in 4 oz servings.  He always takes 6 ounces at bedtime, in the middle of the night and first thing in the morning.  He's taking in quite a bit of solid food at dinner, too.  He eats sweet potatoes, squash, apples, bananas and peas (he LOVES peas).  Sometimes, he'll finish an entire container in one meal, but most of the time, he only eats about half.  He also gets a few banana puffs at breakfast (mainly just to keep him busy while we eat), but although he can pick them up, he doesn't seem to want to put them in his mouth.  His older brother tried helping him the other day ("Open wide!"), but the puffs that made it into his mouth that night must not have made much of an impression on his palate because he's still not interested in putting them in his mouth.  He picks them up and throws them off his tray, much to the dog's delight.

He still loves watching his big brother and is fascinated by his red crocs shoes.  Whenever he sees them, he crawls with all his might to touch them.  He also likes his brother's trains and matchbox cars.  He also still spends a lot of time thoughtfully contemplating his opposable thumbs.  He can literally sit and stare at his fist opening and closing, and his thumb appearing and disappearing for 5 minutes at a stretch.

He's starting to be very roly poly on the changing table.  It's sometimes a miracle we can even get a diaper on him as he's tossing and turning and flipping.  He's just too busy sometimes to pause for diaper changes.

He's starting to add more vowel sounds to his babbling.  We get lots of "ahhs" and "eeeees" along with lots of happy squealing and belly laughs, but I'm also staring to hear some D sounds, which probably means this will be our second baby who says Daddy first =)

He still takes the title of the world's best baby.  He is a great sleeper, a great eater, he plays quietly and happily and he is the sweetest little snuggler.  We really are the luckiest parents to have such a good baby.  I love this little munchkin SO much!

4 Month Immunizations & 1 Mad Baby

We had a bit of a scare with LB this weekend. He went for his 4 month check-up on Friday and all was well, but AFTER he came home from the doctor, he wasn't quite himself. He was mad and crying (he's usually super happy and he hardly ever cries unless he's hungry or needs a new diaper). We suspected he was a bit fussy from the shots, so we gave him some Tylenol, but that didn't help either. He also started coughing and his nose started running. He was so mad that he would barely eat anything.

We weren't sure if the coughing and stuffiness was from all the crying or if he might have picked up a virus while he was at the peditrician, but the fact that he was barely eating was really concerning. Our instructions from the metabolic clinic are that if he eats 1/3 less than he normally does (they call this "the rule of thirds"), it is cause for concern and we should give them a call. All weekend long, we were practically force feeding the poor kid and he was within an ounce or two of the rule of thirds. I got nervous and called the clinic on Saturday night. They reassured me that he would be ok as long as he didn't drop below 20 ounces for the day (he normally eats 30-32 ounces). They also suspected the shots combined with a possible cold were to blame. We kept him dosed with Tylenol and reverted to our routine of waking him every 3-4 hours during the night instead of every 6, just to make sure he had enough nutrition in his system.

We had a couple of tough nights, but by today, he was right back on schedule. It's amazing how quickly kids bounce back from illnesses.

Metabolic Appointment

We met with the Metabolic Specialists at Children's Hospital this week. It was our first visit to a Children's Hospital and we were immediately in awe of how kid-focused everything was -- from the colorful waiting areas complete with toys to the little red wagons they use for transporting kids around.  We were anxious to hear more about the condition LB has and get complete information on how we would be able to keep him healthy.  I admit that I was a little bit scared of what we were going to hear.

The first thing they did was to weigh and measure LB and they were keen to note his weight in kilograms (for reasons I'll discuss later).  We then met with a genetic counselor who talked to us for more than an hour.  She took a complete genetic history of both my husband and me, in addition to educating us on how LB inherited MCADD and how the newborn screening system alerted them to contact us.  She then told us about MCADD and what we would need to be aware of to ensure that LB remains healthy.  More details on these in future posts...

Basically, we need to keep careful tabs on how much and how often he eats, especially during his first year.  His body cannot rely on stored fats for energy like that of a person with normal metabolism, so his body has to rely on glucose.  Right now, he is getting glucose from breast milk, but when he expends that energy, he has only a small store of glucose in his liver than he can rely on for back-up power.  As he grows, that store will grow larger and he will be able to go longer between feedings.  For now, the rule of thumb is that he can go one hour between feedings for every kilogram of body weight he has.  He is 3.375 kg now, so he can go about 3 hours between feedings. 

In times of illness, we need to be extra careful to monitor how much he is eating and how much he is keeping down.  If he vomits, for example, we would need to go back to his prior feeding and count the number of hours it has been since his last meal in order to accurately assess how often it has been since he last ate (and retained the food).  We were advised that they would likely have a very low threshold for simply admitting him to the hospital for IV fluids in times of illness so that they could be sure that he is getting the fluids that he needs to keep his body from going into metabolic crisis, the consequences of which can be devastating.  We were provided with an emergency letter to present to the Emergency Room staff which describes his condition and his need for immediate medical intervention to prevent life-threatening complications (it sounds very scary for a reason -- it's meant to scare the ER docs into treating him swiftly and aggressively, even if his illness seems minor on the surface because it's what's going on with his metabolism that is of concern and we can't observe that just by looking at him).

We were also given a prescription for Polycose, a glucose-rich powder that we will need to feed to him in times of illness to make sure that he's getting enough glucose to maintain his metabolism.  In a typical child, you would feed them pedialyte or gatorade to maintain their electrolyte balance in times of illness, but we were told never to feed LB straight pedialyte, only pedialyte that is doctored with his prescription polycose since pedialyte doesn't have any sugar, which he will desperately need when he is sick.

We asked if he would need a special diet and they told us he wouldn't.  He just needs to eat regularly and probably have a good, hearty bedtime snack once he's old enough to sleep through the night.  He doesn't have to avoid any foods or do anything special.

We asked if he would be able to do all the things that normal kids do -- play sports, etc. and they assured us that he would, as long as he eats well and avoids fasting.

We asked if the clinic treats a lot of kids with MCADD and they said that the state screening program has picked up approximately 20 kids since the screening program for MCADD began 6 years ago.  All of them are healthy and doing just fine, although they cautioned us that some parents of MCADD kids tend to over-feed their kids, which is unnecessary and can cause weight issues.

Unfortunately, they did relay a story to us that was quite sad.  Ironically, it was also one that I remember reading about while I was pregnant with LB.  A baby died when he was four-days-old and his newborn screening came back positive for MCADD the very next day.  The baby fell asleep in his mom's arms and never woke up.  His parents didn't know that he had MCADD and needed to eat frequently.  The hospital where he was born didn't courier their screening cards to the lab, there was a weekend involved and the end result was very tragic.  How very thankful we are that our newborn screening result came back so quickly.  I don't dare think about what would have happened to little LB if we hadn't gotten the call until Monday morning, instead of when we did -- late on a Friday night.

The clinic will see us every few months for his first year, since so much changes in kiddos during their first year.  After that, they will likely only see him about twice per year.