(The post below was written a few weeks ago on my blog, Tell Her This).
it’s all from the waist down
(The post below was written a few weeks ago on my blog, Tell Her This).
Yesterday was our first spinal defects clinic, where we met with her entire ‘team’, including her neurosurgeon, urologist, pediatrician, orthopedist, developmental psychologist (or was he a psychiatrist??), physical therapy, social worker, gastroenterology specialist, and her nurse practitioner who connected us to all of these people. It was here we discussed and learned about her condition, and the complications that have arisen from it, and how to manage them head to toe (or more aptly in her case, waist to toe).
We walked away with so much information it may take me some time to process all of it. Thankfully they are sending us a full written report of all the aspects of her care that we reviewed. I was impressed with this comprehensive approach and am grateful we are able to use this facility rather than the one here in our town (they don’t have a spinal defects clinic anymore, unfortunately). It’s nice that the members of the team are talking to each other, keeping her goals comprehensive while they each focus on their area of expertise.
Here’s what we know.
The neurosurgeon suspects she has a myelocystocele as there is still a pocket of cerebrospinal fluid (a fluid filled cyst) at the base of her spine. From what I’ve read, it won’t be possible to know definitely if this is her actual diagnosis. She also has tethering of her spinal cord, which for the time being we are just “watching”. I am considering a second opinion as apparently the tethering is pretty significant. And though we are certain her brain is not affected, we will do a base-line MRI in the next month (scheduled now for end of March).
The orthopedist wasn’t overly concerned about immediately addressing her contractures, but I disagree. Call it instinct, but I feel the sooner we begin working on correction, the better. She cannot wear any shoes since none of them stay on- and while the social aspects of this are absolutely relevant, I’m more worried about the practicality of foot covering :O) We’ve had our coldest winter in years and it’s been a struggle keeping her feet warm. Not only that, but she’s really active and is out on the jungle gym with her peers, not to mention crawling everywhere- so shoes will better protect her feet from injury. I got the feeling (as much as I find this doctor to be incredibly nice) that he believed my main concern was whether or not Ellis could wear “pretty shoes”. At any rate, Monday at P.T. I will begin talking to her therapist about how I go about beginning the process of serial casting (this was recommended by 3 people we met with yesterday, surgery being a last resort). ** Clarification since you see the photo above, she does not have a clubbed left foot, she is possibly missing tendons on the ankle or they are ineffective. You can see her right foot is contracted pretty severely (points downward), often referred to as “foot drop” **
We learned from the developmental specialist what we already knew: she’s shy. He suspects she may be the type that is so “brainy” that she will lack in the more “normal” range of social skills, though he’s not that concerned. I was as shy as she was. I have no worries at all. Once she opens up to you… well, you’re IN like Flynn (not QQ! Though I swear I would have stolen that name if didn’t belong to such a great friend’s kiddo). We also learned that we are her primary speech therapists, and hearing it from that perspective really opened up how important my role is- not just as her mom, but as her teacher.
With the GI specialist, we talked about potty training and we’ll make that a part of her routine in hopes of achieving “social” continence, since she has a neurogenic bowel (though sometimes she does tell us when she needs to “cho cho“- I’d say 60% of the time or more).
Physical therapy made sure we were connected with the right people here in our town to begin serial casting. They checked how she was doing in general and basically talked about her improvements since we’ve come home.
Her urologist (who I absolutely found to be thorough, concise, & patient) talked about the results of all her testing last week. This is what we were most anxious to hear about. And the news, well, it was disappointing, but not something we can’t manage. First, she doesn’t void on her own at all. A neurogenic bladder, unlike ours, is cone shaped and rippled with muscle along the outside. Due to the interruption of nerve signals from the spina bifida, when her bladder begins to contract to “go”, her urethral sphincter closes- which is common with her condition. Normally, as we void, that sphincter automatically relaxes. So, at most, she leaks urine. We will begin intermittent catheterization within the next week or so (we go back for a separate appt next week). **update: We’ve been cathing now for 2 weeks** It’s necessary she be cathed in order to prevent infection from non-sterile urine being present in her bladder (which is what happens when you have urinary retention or residuals that are very high after voiding). Since she’s had 2 known very severe infections (UTI’s) with a big bug, and then one less severe bug, along with many high fevers, this is crucial. And she’ll learn to cath on her own, probably fairly quickly I suspect since she is a “can do” kinda girl :O)
As for her kidneys, well, we knew she had some reflux into her left kidney, but not how much. We were told it was a Grade 3-4 (which is moderate to high). Not so good. I asked about the amount of damage that can be caused by that- especially in light of the fact that she’s never been cathed and likely has had this issue for the 4 years prior to coming into our lives. He said there was still a lot of “healthy” tissue and that he didn’t suspect she would ever need dialysis. However, what damage has been done is irreversible.
We also learned she has hydronephrosis of the right kidney (and this kidney is smaller than her left kidney). What was baffling to the doctor was that her ureter appeared normal, so there is a chance that there is some other cause of the swelling- a primary obstruction that is not related to her bladder. For the time being, we’ll assume it’s her bladder due to her history, and with daily medication and catheterization, he hopes to see improvement on her next renal ultrasound in 3 months.
She’ll not only be on medication to help relax her bladder (so she can hold more urine, preventing the back flow), but also on a low dose antibiotic indefinitely to prevent and treat colonization with various bacteria. This wouldn’t be necessary if she didn’t have a higher grade of reflux into her left kidney. Thank goodness this amazing daughter of ours is such a trooper at taking any and all medicine, even the worst of it, in fact.
We have our challenges ahead and will one day be facing surgery for her bladder, but probably not until she’s school aged. We were informed that she will probably face detethering of her spinal cord at some point, if not multiple times, in her life. She may have to have tendon releases done surgically if the casting isn’t successful. And depending on how well we can manage her bowel, there also may be a procedure in her future for that.
But every single bit… all of it… will be worth it to help our perfect little girl live a life that is independent, and as fully functioning as possible.
She’ll be guiding us most of the way, I would imagine. She has that way about her.
Yesterday, as we were leaving the clinic, I asked her:
“Did you have a good day today?”
Me: “Is everyday a good day?”
I’ll take that to the bank with her any day of the week and twice on Sunday.