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!

To Immunize or Not to Immunize - Part 1

Immunizations have been a hot topic in Colorado lately due to pending state legislation (Colorado House Bill 1288) that would tighten the rules for how parents exercise the "personal belief" exemption from immunizing their children. 

When it comes to vaccinations, our family is all in.  We fully immunized our oldest son according to the standard schedule from the American Academy of Pediatrics and he has never had so much as a fever from a vaccine.  He has also been a really healthy kid, in general and does not have MCADD.  We felt that immunizations for our MCADD kid were even more important and he has also been fully immunized according to schedule.  Since entering our parenting years, my husband and I have been regular recipients of annual flu shots and also have gone in for the whooping cough booster shots (tdap).


I was curious whether there was any specific guidance on immunizations for children with fatty acid oxidation disorders and was pleased to find a study in the Journal Pediatrics from 2006.  The basic outcome of the study for MCADD patients was that "because patients with these disorders can become extremely ill with infections in general, but particularly with those that are associated with nausea and vomiting, it is obvious that these children should not be permitted to confront wild-type infections.  Immunizations are an essential part of the health care maintenance of this patient population."

In addition, the study asserted that "contraindications against immunizations were not found in the available infectious disease and metabolic disease databases for inborn errors of metabolism. However, there are some inborn errors with associated impaired immune functions or tendency for rapid decompensation that may require caution and close follow-up after administration of immunizations. The purpose of these follow-up evaluations is to not only monitor for metabolic decompensation but also assess for suboptimal immune responses to the vaccinations that could potentially leave these patients susceptible to major vaccine-preventable diseases."  In other words, definitely immunize, but always observe your child closely after immunizations.

Although there definitely needs to be more research to ensure that our kids are actually getting appropriate immunity from vaccinations, I take comfort in the meantime knowing that my kiddo has some degree of immunity from a vaccine.  That comfort definitely outweighs the unknown of what he might encounter from the actual virus he could contract without that immunity. I know when a vaccine is administered and what side effects to watch for.  We carefully monitor him after immunizations to make sure that he is eating and drinking well and that his system isn't stressed by fever or illness, so it is definitely a far more controlled situation than him catching a random infectious disease, which I think we can all agree that we dread!!

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!

The Thin Line

I often struggle with that thin line between over- and under-reacting to LB's illnesses.  I think most parents of children with metabolic disorders probably do the same thing.  When it's something like vomiting, we know exactly what to do and react decisively, but when it's something more subtle, we tend to waffle, not wanting to overreact and subject our kiddo to unnecessary medical intervention, but also not wanting to under-react and possibly wait too long to bring something to the attention of doctors, thus putting him in danger.

With our older, unaffected child, there would be no question that the runny nose, coughing and fussiness currently being exhibited by LB would be interpreted as your average, run-of-the-mill symptoms of a cold, probably with some teething mixed in.  We would give our unaffected child a cup of juice, a popsicle and some tylenol and chalk it up to those inevitable parts of childhood - colds and teething.  However, when LB starts into any illness, I'm always second guessing myself.  Should we go to the pediatrician just in case?  Is this something that we need to catch early to prevent it from turning into something that would put him in the hospital? 

As he was crying inconsolably for nearly two hours last night, I was ready to call the doctor, head to the ER, anything to relieve his discomfort and make sure that he was OK.  We had given him the appropriate dosage of tylenol and ibuprofen throughout the day yesterday, including right before bedtime.  He had been a picky eater earlier in the day and we thought that the pain relievers would quell the teething discomfort enough to keep him eating.  They did and he ate a great dinner and a respectable bedtime snack before peacefully drifting off to sleep about 7:45 p.m.  It didn't last long, though.  By 8:45, he was in full blown hysteria and we could not seem to help him calm down.  After nearly two hours of rocking and walking the hallways and running a hot shower to get some warm steamy air into his congested nose and more walking and more rocking, it was time for another dose of ibuprofen and my husband also thought to pull out the orajel, too.  It worked like a charm and LB was back to sleep for the rest of the night.  What a relief!

This morning, again though, I was worried.  We followed our normal morning routine right up to the point of almost walking out the door before I put on the brakes.  LB was stuffy and coughing almost to the point of vomiting and I just didn't feel right sending him to the sitter, even though she told us it was fine.  His runny nose was the exact same one that her grandson has (he and LB are only 2 weeks apart in age and have so far seemed to do the teething thing as a team).  My gut told me to take him to the pediatrician just to be sure it was only a cold and teething and not something like an ear infection or strep throat that would require an antibiotic. My husband also tried to convince me that he was fine, but I needed to be sure and a quick trip to the pediatrician this morning would confirm that for me -- HE IS FINE!  He's got a bit of a virus and he's teething.  The pediatrician told us to keep up the regimen of tylenol/ibuprofen as his symptoms dictate, along with some benadryl.  She also said to use nasal saline 10 minutes before meals to loosen the mucus and allow him to eat more easily. He should be feeling better in 5-7 days...hopefully with two new teeth to show for it!

We did end up calling the metabolic clinic tonight, too.  Although he has been sucking down liquids like crazy, LB's food intake was pretty poor all day.  They assured us that as long as he's acting normal and he's drinking, he's getting calories and is probably just fine.  They suggested that we might want to wake him tonight for a feeding to give him a little boost, but that there wasn't any need for polycose or other intervention unless he stopped drinking.

So there it is, another lesson in parenting a child with MCADD - he's probably just having "normal" kid symptoms, but it never hurts to have it checked out...especially, if the same inconsolable crying thing happens again tonight.  This time, I'll have the peace of mind to realize that it IS just a cold and it IS just teething.  As long as he's eating well, this too shall pass.
 

Processing the Last Few Weeks

Wow, what a month!  Due to an unprecedented number of viruses "going around" and the fact that so many people were out and about during the holiday season, our family got crushed with illness this last month.  I was sick with a cold/allergies, Husband had a cold and both boys had two bouts of stomach illness.  There were a lot of families that were going through the same thing that we were.  Practically everyone that I've spoken with in recent weeks has had a horror story of sick kids, sick kids at relatives houses for the holidays, sick kids on airplanes, sick kids missing breakfast with Santa...you get the idea. 

Our sick kids story was a little bit different than many other stories, though.  This is because we have a child with MCADD.  Food intake is so critical with MCADD kids, especially when they are already vulnerable because they are so young (LB was 9 months old during this spell of illness).  When they stop eating because of a tummy ache or when they lose nutrients in large quantities through diarrhea or vomiting, it is a medical emergency.  We have some methods that we can take at home to help him along, namely feeding him a glucose solution called polycose and a medicine called carnitine, but when that isn't enough, we head for the hospital so that he can have IV support to help him safely weather the illness and prevent metabolic crisis.

If LB was unaffected by MCADD, like our older son, he would have had a spot on the couch next to his big brother these last few weeks.  They would both have been drinking pedialyte, eating toast, vomiting in a bucket and waiting for the illness to run its course.  That method is just a little too risky for LB, though.  His body doesn't work the same way that his older brother's body does.  Pedialyte isn't enough to keep him hydrated and nourished during illness.  It doesn't have the sugar he needs.  It doesn't have the carnitine he needs.  His body would become hypoglycemic, his blood would become acidic and he would become very, very ill if he just sat on the couch with his big brother drinking pedialyte.  We know this thanks to the newborn screening that identified his MCADD.  His doctors know this thanks to the newborn screening that identified his MCADD.  No one takes any risks with his health. 

Every doctor we work with has said that it is safer for him to go to the hospital for IV support when he is sick than to risk him suffering a metabolic crisis which can have serious mental and physical side-effects, some of which could be irreversible.  So, when LB is sick, we call the metabolic clinic, we heed the advice of our specialists and we head for the hospital.  We stay in the hospital for a day or two until LB is eating well and no longer suffering from the vomiting and/or diarrhea that robs his body of essential nutrients.  Then, we come home.  LB goes on with his busy life of playing with toys, following his big brother around and making everyone smile.  We are so grateful that we know how to keep him safe in this manner.

Sometimes people tell me that they can't imagine how difficult it is to have to go to the hospital everytime your kid is sick.  While it is true that we have to juggle a lot of things (childcare, work, pets, household chores, meals, etc.) when we are in the hospital with LB, it is difficult to juggle all of those same things when any child is sick.  Sick kids are tough no matter if they are sitting on your couch vomiting in a bucket or sitting in a hospital room with an IV.  Sick kids are tough no matter if they have MCADD or are unaffected.  I'm slowly learning that the divide and conquer strategy of me in the hospital with LB and Husband at home with our older son is just going to be the new normal for us whenever we get this kind of illness in our family. 

In closing, we are thankful for:

1. LB, our sweet, happy baby
2. the newborn screening that identified LB's MCADD
3. the doctors and specialists who have taught us how to keep him safe
4. the hospital staff who have cared for LB during illness
5. the strong network of friends and neighbors who have supported us during his recent illnesses