Wordy Wednesday

Articularity - as in "My articularity level is low, given my lack of sleep."

MCAD

The story of Hannah

I started out mommying with Claire, born at 35 weeks but only 3 pounds 2 ounces in size, and diagnosed as having Intrauterine Growth Restriction. A strong little girl, no wilting lily she, but still small and worrying with her diminutive stature. She grew steadily but stayed wee, was brilliant but dainty, and strong willed enough I let down my guard and stopped the panic that comes with suspected incompetence in parenting. After I gave birth to baby numero uno, Claire the Magnificent, I wanted baby number 2. Wanted so badly I moved forward without fear. Hannah was, to my mind, to be my big strong healthy baby, who I would fight to ensure would be born a full term baby, capable of great feats of breastfeeding and eating prowess, who would grow like a weed and wear clothes sized to her actual age. I spent much mental energy telling myself that even though no evidence existed that anything I did carrying Claire might have caused IUGR, I would carefully shepherd this baby out and at least she could be full sized. I fought those high risk OBs, fought them day after day to be allowed to go to term and maybe into labor. Due date came, and a scheduled C-section, and a healthy baby 6 pounds, 1 ounce, with the face of an angel, born on the lucky date of 08/08/08. I was quite ill with complications and maybe not as attentive as I would have liked to have been but Hannah seemed to be an adept feeder, and if not gifted she was at least adequate. A little more than 48 hours after her birth, the nurses who stop by to stick her on the food source and check vitals thought she had a low temp and took her to warm her up in nursery, since I was so busy vomiting they thought it would be better for baby. When she didn't warm as expected they checked blood sugar, and found she had none, so a glucose IV went in and the baby went limp. As in floppy like a rag doll. Glucose gave her a normal blood sugar reading but but other weird signs said keep her in the nursery and watch her. The docs started antibiotics in case there was an infection, and I started pumping milk. Moo. The floppiness remained and my panic started rising over the next two and a half days as the medical team discussed various things that might cause low temp, low blood sugar and lethargy and I consented to any kind of test they might want to do. A young resident would frequently come to my hospital room to tell me I had a Mystery Baby, a safe baby, but a Mystery Baby. Finally the newborn screen results came back and the doc came to tell me Hannah had MCAD - Medium Chain Acyl Co-A Dehodrogenase Deficiency. Kindly, they explained to me that this was considered to be an "inborn error of metabolism" rather than a genetic mutation, but Jason and I must each have a defect on the MCAD gene, making us carriers, and when two carriers reproduce they can produce a child with this disorder. Hello guilt and worry! Howdy, panic! Nice to meet you overwhelming sense of personal inadequacy. Goodbye sleep. Who needs you anyway, you unreliable shit. Sleep is for the sleepy.

MCAD is perhaps a more benign metabolic disorder than some, in that avoidance of crisis has a simple plan of attack - feed the affected person regularly, never allow blood sugar to plummet, and hang on for the ride. Failure to keep blood sugar up means a fast trip to organ damage, coma and death, which is why undiagnosed MCAD kids can represent some of the unexplained SIDS deaths. The disorder was only identified about 30 years ago, testing to confirm it came much later than that, and currently not all states include this disorder on the panel of tests for newborn screening, for example, our state added the test only in January of 2006. If you google MCAD you will find that all the sad stories are those where the diagnosis came after a crisis or at death. Having all infants screened at birth will ensure that parents and care providers can take the steps necessary to avoid a metabolic crisis. Hannah's long term plan, as for all MCAD kids, will be to eat at regular intervals, and guided by her approximate weight we can assess her tolerance for a period of fasting. If at any time it appears she can't or won't take in adequate carbs for quick energy, we rush to the children's hospital, emergency letter in hand, and get sugar pumping in by IV. As long as she never burns all her glycogen, she will be fine, but if she burns all available quick energy, she can quickly produce toxins that affect her major organs, because she lacks the enzyme to burn the medium chain fats. Long term prognosis should be good with adequate nutrition, carnitine supplementation, and good medical care support.

Hannah, with her penchant for making her mark on the world, did experience a full metabolic crisis, but because she was still in hospital, due to our c-section delivery, she wasn't down for long. This should be the basis for us to feel plenty of warm fuzzy feelings and no fear for the future, because we got a diagnosis that was not made at an autopsy. But, fuck, fear is now my middle name! Never, never, never will I get over seeing my normal beautiful daughter go from classic infant appearance to floppy rag doll. Never. And never will I recover from taking her home after a few weeks, certain that we were only ever three hours away from a hospital admission. Now at the ripe old age of six months, based on her current weight, we are six hours away from a hospital admission, because if more than that time might pass without taking in carbs, she might again be in a state of metabolic crisis. Whatever number of hours we have for the longest allowable fast, I will never be totally comfortable. And I know my job is to not raise this child to feel broken, disabled or inadequate, but to accept this is just one thing about who she is but not every thing about who she is.

I want to walk some fine line between taking every necessary precaution, providing all available care, and helping her live the most joyful and interesting life she can without focusing on the hard bad stuff of having health concerns. I struggle enormously with watching every little bit of her development to see signs of lasting effects of the hypotonia she developed in the midst of her metabolic crisis. I see that she doesn't roll over according to developmental milestones, and one hand makes a fist and doesn't grab for toys and I worry about CP or continuing hypotonia or whatever other manifestation of hell might be visited upon us. And then I see that high wattage Hannah smile and take a big deep breath and sing the Hannah Rose song in her ear until she laughs until she snorts. Sometimes when I am driving I gasp and clap my hand over my mouth so I don't scream with worry, then I play a sad song really loud and sing until I get my equilibrium. We are six months into this without any other hospitalizations. Her day care providers are attentive and concerned, and notify me whenever her intake seems off, and so far if I take her home and snuggle her when they can't feed her, the milk goes in, however slowly, and maybe in fits and starts. As she starts to eat food we will learn all over again what her average is, what her minimum intake is, and what she is willing to eat. Don't ask me anything about teenage dramas and eating behavior, two year old tantrums, or where she'll be allowed to go on vacation, because I haven't worked out answers yet.

If you got here by searching for moms of MCAD kids, please feel free to email me directly for any more info or assurances I can give. Imagine this is me holding your hand and hugging you so you feel better. Then hug me back because this scares the crap right out of me. Newborn screening can detect MCAD and a host of other metabolic disorders. In the United States, most states have a panel of metabolic disorders that may now inlude MCAD, but this testing may only have been in effect in any given state for a short period of time.

And now you know why I am nuts.

My vowel is wonky

Pretty much nothing cracks me up as much as when I misunderstand someone speaking to me. The level at which I won't understand you is directly proportional to the amount of sleep I didn't get. Recently, my husband offered to get me a glass eye, and of course, I accepted. Yesterday I explained to my best friend (not my breast friend, no!) that I couldn't meet her for lunch because my valve was wonky, meaning my breast pump couldn't pump, leaving me rather at loose ends and needing to run home to the big pump Ol' Reliable. She believed I said my vowel was wonky, and maybe it was. I once thought that my sister was insisting I should try schnapps because "It's good for you on Fridays" which I found baffling, until I found out she was telling me it was good for my arthritis. Even more baffling since I don't have arthritis. I will likely have arthritis by the time I stop using this damn breast pump though. Who knew that a moderately sized child could give one aching joints? Who knew a breast pump without a hands free attachment would create the dreaded "bottle elbow" pain? I think I once envisioned motherhood through a soft misty glow, seeing myself in a hazy Madonna and child sort of pose. I didn't know then that the filmy gauzy look I envisioned for myself was representative of the film of milk all over me, my pump, my pump parts, my baby, and every chair I sit in to feed my baby. The breast pump is still wonky, and I don't believe its good for you on Fridays. Or on Mondays.

Confessions

This post was inspired by a recent post by one of my favorite bloggers, over at Belgian Waffle, and the comments she generated when she confessed and asked her readers to join in.

Six months post-partum and I am still wearing maternity shirts. I did, however, dump all remaining pregnancy pants, but the blousy tops remain. Why? Not just because they fit, but because I can't bear to shop for replacements and face the mirror. I just can't.

I like food too much for my own good.

Sometimes I am not nice, and I am not sorry.

I say the same inane things to my kids that my parents said to me - most notably "Whatever you're doing, stop it!"


I have some untreated OCD, but it doesn't manifest in useful ways, like keeping my house or clothes or babies clean. Oh no, I have patterns of how my breast pump bottles must line up on the counter between cleaning / using.

I correct other adults' grammar, despite it being rude.


I love the English Beat song "I Confess" as much as Elvis Costello's "I Stand Accused." Am I guilty? Oh, you bet.


I named my blog Gas Food Lodging because I feel like a a busy exit on the interstate of life - I have been chosen by two babies to give (and get) gas, food, and lodging. And they don't pay. As rest stops go, my bathroom is just as dirty, the chance to get out and stretch is just as welcome, and at chez moi, like many rest stops, maybe a squirrel will drop nuts on your head as you rearrange your crap.

Knee, Knee, I Fell On My Knee

Today as I struggled with staying vertical on the slab of ice by where I park my car, I realized that all of my life would be funnier if I made up songs about it to the tune of "Skip to My Lou" and so I spent the morning singing "Knee, knee, I fell on my knee . . ." and various similar snippets to narrate the progress of my day.

Falling on my knee was unpleasant, but much better than my rising internal panic of the last few days as the family struggled to feed The Little One. And why should such a "Little One" be hard to feed. Does anything about this child look hard? Doesn't she look the picture of baby health, nay, perhaps she is the epitome of baby well-being? Looks can be deceiving, as can babies. This baby has . . . bad DNA. My DNA. DNA that gives a baby MCAD, mighty crappy aggravating DNA. Are you confused? So am I . . . Come with me, gentle reader, for a fairly short journey into Mommy madness. When Hannah was born, I spent two days believing I got a big (bigger than her preemie older sister) healthy (healthier than her preemie older sister) baby girl, and then baby's blood sugar dropped below a detectable level, and she went limp. Floppy babies don't give doctors piece of mind, let me assure you. Now, at six months of age, we know what to do, and what we do is we feed, feed, feed that baby who has shown a distinct lack of interest in food. With MCAD, without food it is a short trip down from gurgling, cooing, and waving one's tiny fist to brain damage, organ failure, coma, and death. This is why I cry a little bit whenever I get to hold Hannah alone. This is why I am not just a Mommy but a mad, zany Mommy. This is why I will become a Mommy who blogs.

Gas - we have it! Food - we have it! Lodging - it's here!

WARNING: this post contains references to bodily excretions. Sensitive readers should just look at the pictures of cute kiddos. Food! Gas! Lodging! Here at Chez Dward we are reduced to our most basic life functions of eating, engaging in various activities that follow eating, and then resting from our bodily functions and labors, in tiny little chunks of time. Excretions: we got 'em! Child #1 eats like a big girl, and poos like a big girl. She needs to potty train. Need I say more? Child #2 can poo like a big girl, especially if she coughs when not wearing her diaper. Mommy can poo like a big girl too, but prefers not to talk about it in polite company. Not wanting to be outdone by her big sister in grossing out the parents, little infantina is practicing the ancient art of mucous making, and uses her version of the back-to-school cold to glue her own eyes shut while sleeping. Farting: the smaller the human who lives here, the more gas comes out. Weight: Baby gains it, Mommy loses it, but not quite as fast as Baby, disproving the old maxim that within any given group of people the number of pounds are constant although the distribution between people may change. Daddy and #1 remain relatively static in their poundage. Hair: Mommy cut hers off, choosing a sassy new mommy-do, while #2's hair falls out from the front back, leaving a cute little Mohawk. Again, Daddy and #1 have little to report in the hair loss department. Books: #1 likes them, likes to hold them up and read them to you, and likes to show them to her sister. Mommy is ready to step up from bad gossip magazines and get back to the world of books. Send recommendations, please. Dates: Mommy and Daddy have a standing date with an alarm clock, a breast pump, a bottle and a slightly damp baby. #1 had a date with her toddler boyfriend, and the two won matching blow up hammers in a somewhat rigged game at the State Fair. Guess who had more fun?