Two Months

LB had a big day today. It was his two-month check-up! He was 11 lbs 11.5 oz (30th percentile) and 22 inches tall (10th percentile) with a 60th percentile head measurement. I was stunned. After the our older son (who was never less than 85th percentile on any measurement at any point in his life), I didn't think it was possible for us to have a 10th percentile kid. I know I shouldn't compare them because LB is completely entitled to do his own thing, but to put this in context, our older son was 21 1/4 inches tall WHEN HE WAS BORN, so this whole 10th percentile thing is a bit shocking for me. Oh well, at least he's getting use out of those 0-3 month sizes. He's also a healthy and happy little guy who is completely on track developmentally, so that's really all that we can ask for. Very thankful for that!

He is starting to do a lot more smiling and he almost has a laugh, but it's still not time for belly laughing yet (that's one of my favorite milestones). We can tell his cries apart and the cry for "feed me Mommy!" is the most emphatic, followed closely by the cry for "you have no idea how messy my diaper is right now Mommy!" He loves looking at the toys hanging from the shelf over his changing pad and the animal mobile hanging over his crib and he sometimes cries when you take him away from those spots when he isn't ready to leave. Sleeping is one of his favorite things to do, especially when he's in Mommy's arms for a nap. He's also a champion eater and usually packs away somewhere just shy of 30 oz of formula in a day (in 3-4 oz increments about every 2 hours during the day and about every 4 hours at night). We can now set the alarm for 4.5 hours at night based on his weight of a little over 5 kg (we could probably go for 5 hours, but we are trying to remain conservative just to be on the safe side).

Here are some pics of our big (in heart) little guy...

Failure and Acceptance

LB turned six weeks old yesterday. As I write this, I am thawing the final serving of breastmilk that he will receive. It has already been more than two weeks since he last nursed. Despite what I believe were my best efforts, breastfeeding lasted a mere four weeks and my freezer supply only another two. Writing these words pains me. I REALLY wanted breastfeeding to work. I tried VERY hard and I failed. I'm still working on being OK with it.

We seemed to do just fine in the first few days of LB's life, but things deterioriated very quickly. The MCADD diagnosis on Day 3 with instructions to feed, feed, feed him every 2-3 hours sent us into a panic. I erred on the side of caution and had the boy at my breast every 2 hours for the next 4 days not caring the slightest bit about the quality of his latch or the condition of my body so long as he was eating. That lasted until Day 7 when my nipples were so raw and painful that I could barely hold him close to me, let alone provide one more feeding. This is when the first case of mastitis set in, likely because of some combination of 1) the ugly condition of my nipples, 2) the stress of his diagnosis, 3) the sleep deprivation and/or 4) the stress of his delivery, in general.

We resorted to pumping and botttle feeding him with breastmilk exclusively until my nipples healed and that took about a week. At the point that I was gradually reintroducing him to the breast, I was still in quite a bit of pain as he nursed. It felt like I was dispensing shards of glass through my nipples instead of milk. After a consultation with a lactation specialist at the hospital, we discovered that I had a secondary yeast infection called thrush (likely caused by the antibiotics I was on for the mastitis). Subsequently, both LB and I went on medication to clear that up. It had only been two days since we finished the mastitis antibiotic and here we were -- already back at the pharmacy for more drugs...and yes, I was still doing a lot more feedings by pumping and bottling than I was by straight nursing which seemed such an institutional way to feed a newborn baby to someone who was used to nursing (I nursed my older son until he was 7 months old).

The first weekend of April was glorious. Not only did we have beautiful weather outside, I felt like the metaphorical clouds that were hanging over the nursing situation were also lifting.  LB and I were both a week into the thrush recovery and I was able to breastfeed him without biting my lip or curling my toes in pain. Finally! We achieved a healthy nursing relationship. Finally! I was able to conduct a night feeding in less than an hour and a half (how long it takes when you have to add the steps of pumping, sanitizing the pump stuff, bottling the milk, and sanitizing the finished bottle to the usual steps of changing a couple diapers, feeding, burping and rocking the baby back to sleep). I was SO happy. I thought we had made it out of the woods. After another week of finishing the thrush medication, we would be home free.

I was wrong. The mastitis came back the very next day.

As I was pacing through the house, holding LB akwardly away from me to prevent him from touching my very sore breasts, the reality of my situation hit me like a ton of bricks. I was spending so much effort trying to make breastfeeding work that I was missing everything else. My typical day consisted of pumping every 3 hours and feeding LB the pumped milk every 2-3 hours. This meant 20 minutes to prep, pump, store milk and clean my pump parts every three hours around the clock, followed by at least 20 minutes to prep a bottle, feed, burp and give LB his thrush medicine every two to three hours around the clock. In addition to that, there was a daily load of laundry to gather, wash, fold and put away and a twice daily regimen of sanitizing bottles -- both aimed at eliminating the yeast that was causing the thrush from our clothing and his eating vessels. In a single 24-hour period, I was devoting at least 8 hours just to trying (in vain, it seemed) to make this breastfeeding thing work.

The rest of my days were spent attempting to spend at least a little bit of time paying attention to my older son and my husband while also putting breakfast, lunch and dinner on the table for the family, keeping up with other necessary household chores (laundry, grocery shopping, dishes, etc) -- the other stuff like vacuuming and general tidying up were already out the window -- and getting a couple hours of sleep. In short, I was exhausted and that made me grumpy. That led to me feeling even more crummy and also quite guilty for not having the time or energy to spend any "quality" time with my boys or to really step back and enjoy the early days of LB's life. I was either missing it entirely or I was walking through the day as too much of a zombie to appreciate it.

I saw myslef holding LB at that moment (in my forearms, as far away from my breast as I could) and realized just how broken this situation was. The best part of breastfeeding for me last time (with my older son) was the physical closeness we had. We snuggled, we bonded and it was so rewarding. I knew that I wasn't establishing that same bond with LB because the breastfeeding itself was so physically painful that not only did I not want to nurse him, here I was holding him as far away from my chest and the beating heart that he undoubtedly found so comforting, as I could. The psychological guilt that I felt at that moment -- wanting to hold my beautiful, warm, snuggly baby and at the same time not wanting him or anyone else to touch me was THE WORST -- a dagger straight through my heart. I realized that I wasn't hugging my older son or husband tightly to me anymore either. This was all wrong!

Walking through the house that night, I was crying because I wanted breastfeeding to work so badly, but I was also crying because I knew that it wasn't working and that I needed to move on before it consumed me, threw me into depression and/or damaged the establishment/maintenance of a bond between me and my boys. In some respects, it was the hardest decision to make, but at the same time, I knew it was the only decision I could make. So much more was on the line here than just the uncomfortable stares I would start receiving from other moms as I mixed up a bottle of formula instead of donning a nursing wrap. This was my family and I needed to start feeling like a Mom again, not just like a walking advertisement for La Leche League. I needed to let go of breastfeeding and start hugging my kids and my husband again.

I visited the doctor the next morning for yet another course of antibiotics to clear up yet another bout of mastitis and I called the lactation nurses for instructions on how to taper off my milk supply. I was finished with breastfeeding.

It's still hard for me sometimes to admit that I failed at breastfeeding. It seems like such a natural thing, like something that automatically works unless you do something to break it and I must have somehow broken it for it to have been such an epic failure, but I know that I did everything I could. Every single doctor and nurse and lactation consultant that I worked with over the course of those 4 weeks told me that I was doing everything right and that they couldn't explain why I kept getting infections. They also all told me that no one would fault me for quitting if that's what I chose to do. They assured me that millions of babies eat formula and turn out just fine.

I wanted to believe them and I did believe them, but I also knew that I had to give myself permission to quit before I could move past the failure of it all and into acceptance. That's where I am now. I haven't pumped in nearly a week and my last trip to the grocery store included a sizable purchase of powdered infant formula. I have removed the nursing wrap from the diaper bag and packed away the breastpump.

LB takes the bottle happily and is thriving in every way. For the last two nights, he has woken up at around 4 a.m., not because he's hungry, just because he wants to be snuggled in my arms. I hold him close and he squeaks his little infant squeaks before closing his eyes and drifting off to dreamland snuggled against my chest and the comforting sounds of my heartbeat. We are bonding and I know that he loves me. I am happy.

My older son has returned to running headlong into me when I pick him up from the sitter and whenever the mood strikes him to come hug his Mommy. I can snuggle in bed next to him to read him his bedtime story without worrying about whether he is going to bump me in the wrong place during the unpredictable and exuberant wiggling as we get to his favorite parts of the book. We are back in a comfortable and affectionate place and I can return his love for me with great big hugs of my own. I am happy.

Of course, Husband has been uber-supportive of me on this from day one. He supported me in my efforts to make it work and he supported me in my decision to call it quits. He is getting his happy wife back because I am happy. Deep down I know that this is the right decision

Breastfeeding this time around may not have turned out the way I was expecting/anticipating, but it is what it is. As long as LB gets fed and snuggled, everything will be just fine.

One Month

It has been one month since LB joined our family...and what an eventful month it has been! We've had our share of hurdles -- both hurdles typical to those anyone would encounter as a newborn enters your life and a few unique ones on top of that because of his MCADD -- but, all in all, it has been a great first month.

We are so fortunate that LB is such a happy and cooperative baby. He likes to sleep, which is great. He must be doing lots of growing to always be so sleepy. He is starting to show some interest in toys and especially in the mobile over his crib. He gets very excited when the music plays and the animals dance around as he gazes up at them. He also likes to watch his big brother, which is fun for us to watch. He was laying on his quilt today and our older son was playing near him, sort of near his head.  LB practically rolled over since he was looking over the top of his head trying to keep an eye on his big brother.

LB has also been very tolerant of all of the affection bestowed upon him by his brother. He is the lucky recipient of many exuberant kisses and an endless supply of toys to play with. His big brother absolutely loves him and is the proudest big brother in town.

Husband and I are also adjusting pretty well to being the parents of two little boys. We go to bed tired at night, that's for sure, and we also go to bed feeling incredibly blessed. The fun is just beginning!

MCADD - It's Genetic!

So I mentioned yesterday that LB has MCADD because his MCAD enzyme isn't working. What exactly causes that to happen? The answer is genetics. Your body has genes that tell it to make enzymes and his genes that make the MCAD enzyme are what isn't working.

Having errors in your genes isn't all that unusual. Our genetic counselor told us that everyone has errors in their genome, typically around 20. Genetic errors aren't usually problematic because you have two copies of each gene and most of the time, at least one of those copies is fine. The body uses the "good" copy to do what it needs to do. Unfortunately, BOTH of LB's genes that tell his body how to make MCAD either aren't there or are broken, so his body honestly doesn't know how to make the MCAD enzyme. It's kind of like his body's recipe book has that page ripped out or somebody spilled something all over it and it's illegible. As much as he may want to make that enzyme, he just doesn't know how.

How did he end up with two bad copies of the same gene? It's his parents' fault!!  Husband and I just happen to have the same errors in our genetic code and we both passed the "bad" copy of the MCAD gene onto LB. It's likely that Husband and I are both just carriers of the condition. We have one good copy of the MCAD gene and one bad copy, but our bodies can use the good copy to make the enzyme and we are fine. Unfortunately, between the two of us, we didn't give LB a single good copy to work with. With both of us being carriers, the chances of us having a baby with MCADD are 1 in 4. The process looks something like this...

We know through newborn screening that LB has MCADD. That is, he has two bad copies of the gene. We now know that both Husband and I are at least carriers of the condition (Rr) since we produced offspring with one bad copy from each of us (rr). If we are both carriers, then there is a chance that our older son, LM, may also have inherited a copy of our bad gene. He could also be a carrier (Rr) and there is a 50% chance of that. The best case scenario would be that he inherited only our good gene (RR) and there is a 25% chance of that. Since the state was screening for MCADD two years ago when he was born, it is unlikely that the LM has MCADD, but they suggested we re-test him just to be sure -- his sample card may have been mishandled or the wrong test done on the wrong blood spot. Again, it is unlikely that he has it, but we're going to go for "better safe than sorry."

Our genetic counselor told us that in some situations, infant screening has identified the condition in a parent (since testing for it has been only relatively recent). With MCADD, the parent happens to be lucky enough to not have ever been very ill to where their life was in danger due to fasting, but knowing they have the condition going forward can be very helpful for future illnesses, surgeries, etc. Knowing that there is also a possibility that one or both of us could have MCADD (although unlikely), we are both going to be tested to be sure. If one of us ends up testing positive for MCADD, our genetic situation changes slightly...

If one of us were affected with MCADD, that person could only pass a bad copy of the gene onto our child. The unaffected parent could pass on either a good copy or a bad copy. Thus, this would give us a 50-50 chance of having a child with MCADD and all of our children would at least be carriers of the condition.

I doubt either of us has MCADD, but it's also so statistically rare that two people with the same genetic errors have children together, so I guess anything is possible. I'm still really thankful for newborn screening, though. Without it, we would never have known LB had this condition and we certainly wouldn't have know that we were carriers of it either.

MCADD Explained

As promised, here is a little more information about LB's medical condition, MCADD.

MCADD is a fatty acid oxidation disorder found in about 1 in 15,000 babies. Fatty acid oxidation is the process your body uses to turn fats into energy and fatty acid oxidation disorders occur when certain enzymes that break down these fats into energy are either missing or broken. In MCADD, the Medium Chain Acyl-CoA Dehydrogenase enzyme is affected and individuals with MCADD are unable to break down medium chain fats from foods they eat and are also unable to break down medium chain fats stored as body fat. The process looks something like this...

So, if the body can't use the fats, what does it do with them? Basically, it treats them as toxins. Our bodies have substances called carnitines that serve to get rid of waste products.  LB's body is going to end up making a lot of carnitines because not only will they have to get rid of all the "normal" toxins that our bodies need to get rid of, they also have to get rid of the fats that he can't metabolize into energy that his body "thinks" are toxins. The doctors will monitor his carnitine levels periodically and may need to supplement his carnitine levels if he's not making enough.

MCADD can cause bouts of illness called Metabolic Crisis which can include sleepiness, irritability and poor appetite. These can be followed by fever, diarrhea, vomiting and hypoglycemia. Anytime that he is losing nutrients and unable to replenish them, he is in trouble and needs to go to the hospital for a glucose IV, which is, in essence, readily accessible energy for his body to sustain metabolism. If he is not treated quickly for metabolic crisis, he can have breathing problems, seizures and possibly even slip into a coma or die (scary!). Thankfully, metabolic crisis can be avoided simply by not allowing him to fast OR if he is fasting (because he can't keep food down due to illness, etc), we have a can of special formula to feed him. It is called polycose and is basically just pedialyte with HUGE amounts of sugar that his body can immediately use for energy, thus maintaining his metabolism.

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.