Sick Day

We've been really fortunate to stay so healthy this last year.  Other than a mild cold that worked its way through the family in the fall, we haven't had any illness to speak of since LB's string of hospital stays last year around the holidays.  Unfortunately, our luck ran out this week when LB came down with a mild stomach bug. 

 

He woke up around 4 a.m. on Wednesday morning with a stinky, leaky diaper and after we got him (and his bed) all cleaned up, we immediately launched into his sick day protocol of hourly calorie-rich polycose mixed in Pedialyte and carnitine supplements.  Not sure where this illness would land us and for how long, both my husband and I stayed home with him on Wednesday. We packed a hospital bag expecting to end up there at some point that day, but after being in touch with his specialists throughout the day and carefully monitoring his intake, we were pleasantly surprised to be permitted to weather this illness at home.  They said things would get easier as he got older and although it was pretty stressful to stay on top of his feeding regimen, we were super excited to be able to manage him at home for once.

 

By Thursday, he was doing a lot better, but he still wasn't ready to go back to the sitter. I had some out of town guests visiting the office for a meeting, so my husband took sick day duty with LB.  They had a good day and he continued to keep his intake within the guidelines, so all was well.

 

By Friday, he was still doing fine, but we wanted to keep him home one more day and through the weekend to be doubly sure he was going to stay on track.  I stayed home with him today and we had tons of fun.

 

Pretty much as soon as my husband and our older son closed the garage door to head to work and preschool, LB wanted to wear his big brother's Iron Man costume. 

 

 

Then, he wanted to paint.

 

Then he wanted to play outside.

 

I could get used to this staying home with the kiddos thing.  I had a blast!  I think LB was a little tuckered out, though.  Shortly after this photo, he went down for a much deserved nap.

 

We're very grateful that he got well so quickly and managed to stay out of the hospital.  It DOES get easier!!

Metabolic Clinic Appointment

As promised, here is our metabolic clinic update from Thursday's visit.  LB is doing great!  Developmentally, he is right on target and he's staying on his growth curve, which has consistently been between 15th to 20th percentile for weight and around 60th percentile for height.  He delighted the doctors and genetic counselors as he played with his toys, rolled a ball and talked up a storm to them.  He really is doing great.

A few things that we talked about with the doctor:

• We always seem to feel like LB is smaller than he should be and we've kind of been attributing that to the MCADD.  After you have a kid like our older son who has never been below 95th percentile for anything, you wonder how you can have such a little guy as his brother.  They assure us that LB being 20th percentile for weight is just LB.  It has nothing to do with his metabolic condition.  I guess when I think about it, he is a completely different kid in so many ways from his big brother (personality, temperament, timing of milestones, etc.), so it's not that hard to fathom that this is just another way that he's being his own person.  They also said that it's good that he's staying on his growth curve because they often have trouble with obesity in kids with MCADD.  Parents are understandably terrified to let their kids fast and tend to overfeed them, which can lead to unnecessary weight gain.  So far, we are doing great with LB, though. 
• We brought up our table manners issues (how he throws a lot of food at meals and we just give the plate right back to him to throw more) and we got some very good advice from the metabolic physician.  She told us that just because he has MCADD doesn't mean he has to eat more food than his stomach can hold.  If he's throwing food, he's probably not hungry and it's ok to take the plate away and excuse him from the table.  She stressed that we should discipline him in the same way we did our older son (when our older son threw food at this stage of development, we took his plate away and ended his meal -- the throwing stopped within weeks whereas the throwing with LB has been going on FOREVER it seems).  She also gave us some sage advice to not let food be a trigger for our family on any front.  We are going to have times that we absolutely need LB to eat to keep his metabolism out of trouble and if we use up all of our food capital on this kind of stuff, it's going to be a harder road to convince him to eat when he really really needs to.
• They previewed that in future appointments when LB is able to speak for himself that the physicians will be interacting more directly with him while we are more in the background.  They have a philosophy of empowering the children in their clinic to be able to speak for themselves about their health and how they're feeling and it will give him practice to role play this while in the clinic.  This makes me feel very good about the care that he's receiving here.  I know that there will be a lot of times that my husband or I won't be with him in his future (going to school, going to sports practices, even just playing at a friend's house) and he needs to be able to go talk to a teacher, a coach, an adult and tell them if he's having a problem, if he needs to eat/drink something and what he needs to eat/drink in order to feel better.  The sooner he gets to start practicing that, the better. 

They also checked his carnitine levels with some lab work and we'll get those results in a few days.  We keep carnitine in the cupboard for when he's ill, but his labs have never shown carnitine deficiency, so we don't need to give it to him on a regular basis.  They said they typically don't see low carnitine in kids this young, but that eventually, he may need to be supplemented and they always check, just in case.

We also talked briefly about the impending discontinuance of polycose.  Abbott labs announced that they will no longer be making polycose, so we will have to switch LB to something else for his sick-day sugar-beverage protocol.  The physicians assured us that there are a lot of substitute products on the market and that they will be making a recommendation on one once the polycose supply is gone.  They also said that as he gets older, he can get away with drinking Gatorade or he can add sugar to any number of beverages and he won't necessarily need a prescription sugar solution.

We go back to see them in 6 months and we'll continue to see them twice a year until he gets to be school-aged, at which time going annually will likely be sufficient.

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).

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

 

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.

Polycose Potion

It was a little more difficult to fill out prescription for Polycose than we anticipated.  Our pharmacy called us and said that they couldn't even order it.  We called the metabolic clinic at Children's hosptial and asked them if they could help.  Sure enough, the pharmacy on site at the Children's Hospital is a Walgreen's and our local pharmacy is also a Walgreen's.  Their pharmacy had it in stock, so they put their pharmacy in touch with ours and voila!  This lovely can of powder is now hanging out in our pantry.

Funny.  The first time they said "Polycose," I kept thinking about Harry Potter and his Polyjuice Potion.  

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.