The latest on Sean

Summer is here, which means two things in regard to my son Sean:

  1. It’s been eleven months — gadzooks, almost a year?!? – since the onset of his Leigh’s disease.
  2. He’s hating the weather.

It’s one of those things you never realize until you have to go through it: for someone who’s mostly immobile, it’s harder to stay cool than it is to stay warm.  We don’t have central heating/air conditioning (Chez Anselmeau was built in the 1920s, and was on the wrong side of the tracks even then), so staying warm or cool isn’t a matter of setting a dial.  It’s a matter of clothing and blankets and space heaters and fans and when you cook meals.  Old-fashioned, but we’ve managed to make it work.

Sean’s illness, however, has raised the degree of difficulty.  If you need to keep someone warm when they can’t move much, you pile blankets on them, you dress them in thick clothes and layer them.  But when the temperature gets above 95°F (35°C), you can only remove so many of those layers.  And fans only help when the air temp is significantly lower than your body temp – otherwise, you’re just pouring more heat on yourself.

So we’ve had to be creative.  An extra syringe full of water before meals.  Longer baths.  Strategic placement of fans.  And most of all, getting him moving around, including … walking.

You read that right – Sean is actually walking!  Not unassisted, mind you – I have to get him onto his feet, help keep him upright, help him turn, guide his hips with myknees a little, and hold his hands so he doesn’t grab at anything and everything and distract himself.  But he’s the one moving his legs, supporting his weight and learning to keep his eyes on his path and balance himself – all the heavy lifting of locomotion.  Once he’s on his feet, all I’m doing is strategy; the real work is his.

And it’s work, but he’s doing it more and more.  I no longer carry him places in the house – just help him walk where he needs to, then lift him up to a chair or changing cabinet or whatever.  When we go to physical therapy, he walks from the van to the therapy building (over a hundred feet) instead of just getting pushed in his wheelchair.  And the more we do it, the better he gets at weight distribution, posture, foot placement and all the other things we take for granted because we’ve been walking for decades instead of weeks.

Furthermore, I’m forcing myself to let him do his own physical work when I can.  When he practices standing during our exercise times, I don’t hold him up or shadow him anymore – it’s his job to hold himself erect, and if he doesn’t he falls.  (Onto a padded surface prepared for the occasion.  I’m not stupid.)  If he wants to play with something, I’ll put it close enough to grab, but he has to do the grabbing.  It’s not always easy – I can sense from him that he’d rather I did more of the grunt work – but it’s needed for his continued recovery.

And at least on some levels, that recovery is going well.  He had appointments this last week with a pediatrician and with the physician overseeing his therapy, and they were thrilled at the amount of progress he’s made over the last six months.  (Remember that Leigh’s patients aren’t supposed to get better; the recovery rate for the disease is microscopic.)  Besides the standing and walking, his grip is getting better (more in the right hand than the left), he follows activity with his eyes, registers pleasure or displeasure with facial expressions and in short can do quite a few things he wasn’t able to at the start of 2010.  And the professionals are impressed.

Still, there’s a long way to go.  His facial mobility is still limited, especially around his mouth.  He can’t yet open his jaw very much – in fact, he’s developed a nasty tooth-grinding habit that no one, including the docs, has yet found a way to stop.  (We’ve tried jaw massages, manipulation, using his bib as a mouth-guard, even wedging little bits of beef jerky between his molars.  Nothing works for long, and don’t even ask about the state of my cuticles from trying to get stuff past his incisors.)  His speech is limited to an occasional moan when he’s in discomfort.

It’s been tough, especially for his mom.  Nina, of course, is a teacher, and most days until mid-June was at work.  Now she’s having to deal with a silent Sean through the day, and it’s about as easy for her as it was (is) for me.  So there’s more family tension too.

But we’re going to get through it.  Somehow.  God, I’m sure, has a plan.

And more things are coming down the pike in the next few months.  His doctors are trying to arrange for speech (or at least communication) therapy for him.  And Nina has contacted the local school district about in-home tutoring; since Leigh’s is technically an auto-immune disorder, it would be unsafe for him to go to what my mother-in-law calls the Great Cold & Flu Exchange (i.e., a public school).  In the meantime, we’ll keep pumping him full of nutrients and water, keep giving him his exercises, keep trying to engage and interact with him however we (and he) can manage.

And keep asking for your prayers, for him and for us.  This is a looooooong road, and we’ll never make it alone.

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2 Responses to The latest on Sean

  1. Lori Knutson says:

    My infant daughter was recently diagnosed with Leigh’s, and I’ve been heavily researching it on the Internet. Your son is the only person I’ve found who is improving.

    Do you mind if I ask how old is he, and how long has he had Leigh’s? Is he on a mito cocktail? My daughter is on Vitamin B, L-carnitine, and CoQ10. Any information would be greatly appreciated by my husband and me. Thank you.

  2. Ray Anselmo says:

    Sean turned 6 in January, and has had Leigh’s for almost a year. His mom has Charcot-Marie-Tooth disease (CMT, type 2A), which also has a mitochondrial component, so they’re probably connected. We think it was triggered by a cold or flu bug he caught in late July 2009, and the rest is … well, not history exactly, but you get the idea.

    He is on a “mitochondrial cocktail” of Levocarnitine, Co-enzyme Q-10, vitamins B, C and E, and biotin, plus Ranitidine (Zantac) to protect his esophagus (since among what he lost was his gag reflex, or whatever you call it that allows you to suppress the urge to throw up). He gets all his meds and nutrition (Nutren-B with Fiber) through a gastric tube, as his swallowing ability is both minimal and frustratingly inconsistent.

    In addition to the “cocktail,” we put him through a pretty strenuous set of almost-daily exercises, some of which were suggested when he was in the hospital in Aug/Sept ’09, some of which have been recommended by physical therapists since then, and some of which we whipped up on our own as we thought of it. (I say “almost daily” because he gets Saturday off, as well as days when Daddy is too tired to put him through his paces.) We started out mostly with flexibility work, and have since added more muscle-building activity to the menu. He doesn’t like it much, but the improvement is substantial, and he seems to grasp that it’s something that needs to be done. He also has weekly P/T through the local school district, since he was just about to start kindergarten when he took ill and was already registered.

    Despite what has happened, the consensus of the doctors, scientists and our own eyeballs is that inside, he’s still the same sharp-witted kid we knew before the sickness — his motor and speech centers were devastated, but he didn’t lose his cognitive abilities, only the ability to clearly express and apply them. We have to remind ourselves (and occasionally others) that he’s not a baby — he is a bonafide six-year-old who simply lost the physical ability to demonstrate it. (Even his therapists note how fast he picks up the movements they’re trying to teach him; the memory of how to do them is there, he just can’t always make his body cooperate.)

    But even the experts who have seen him are (pleasantly) baffled by his slow yet continued recovery. You mentioned that Sean is the only person you’ve heard of who is getting better; that’s also the case with the professionals, as far as I can tell. (And that includes the #1 expert on mito. disorders in Northern California, who examined Sean months ago and had precisely zero suggestions. At least he was good enough to admit it — in our experience, there’s almost nothing scarier than an MD who’s unwilling to admit their ignorance.)

    The only thing I can attribute his improvement to … and I hope you’ll forgive me if my saying this bothers you … but from the start, even before he went into the hospital, we asked people to pray for Sean. My wife contacted her congregation; I’m not in a congregation presently, but I contacted everyone I knew who I thought would speak to God on his behalf (and several others besides, ’cause you just never know). Many of them not only prayed, but passed it on to others. Even when all we could pray was that God’s will be done in Sean’s life, we kept praying, and kept begging other people to as well.

    At this point I don’t know how many people are praying for him, but the network stretches from California to Chicago, Atlanta, Scotland and New Zealand and includes who-knows-how-many congregations. The post you saw on my blog is a modified version of an e-mail that went out to about 60 families with whom we’re in direct contact. And apparently it’s not His will that Sean go to Heaven just yet, as He had ample opportunity to take him there. What the future holds, we don’t know — but we’re doing our best to trust that Someone sharper than us is running the show.

    I hope some of this helps. I know there are days when I just want to run away and become a beachcomber, and only responsibility (and/or guilt) keeps me in place. I have times when I look into his eyes and see the shoulda-been grade-schooler looking back, frustrated by his inability to act on what his brain tells him he can do, and I want to cry. I don’t have an answer for that, or for the loss of sleep, or the marital stress, or for the day-by-day physical and emotional exhaustion, except to keep plugging away and trust that God has a plan that will be to everyone’s benefit — including Sean’s. It’s not much to go on, but it’s what we have.

    Take care, and we’ll be praying for you and your daughter.

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