A normal childhood

One of my biggest dilemmas is how to make certain you have a “normal” childhood, Miss M.  I want you to be the child who doesn’t let your physical limitations and difference of appearance slow you down.  I want you to be the child who uses your experiences to make a difference, who starts the lemonade stand at age 8 to raise money for vertebral segmentation defect research, who starts the kids support group online, who gets voted class president in high school because everyone recognizes your differences, what you have overcome, and loves you so much.

I was not that child – I was resentful, I had low self esteem, and I was always the last kid picked for the team.  I was unpopular from a young age – even at age six, I already felt different and like I didn’t fit in.  I know my parents probably could have done something to help me with these issues, but I honestly don’t know what.  Therapy?  More supportive of team sports participation?  I always liked to write, so they did encourage me to write.  But I always felt defective, wrong, and not special.  Even as an adult, I blame a lot of that on my cleft lip.

I think conventional wisdom says to treat your child, whatever their limitations, as though they can do anything they want.  Not just treat them that way – truly believe it yourself, as well.  I worry though that this belief, that you can achieve anything, the sky is the limit, ignores your reality.  You will know this isn’t true.  Your neck is very fragile.  Risk of injury from physical activities is very high – it’s unlikely you’ll ever play sports.  You’ll feel different because you can’t play on the teams with all of the other kids at school.  I can’t parent in a way that makes this fact disappear.  Inherently, you will be different.

And I don’t want this difference to be ignored.  I feel that part of the reason that I had so much of a struggle through my childhood is precisely because my parents minimized the effect my cleft had on my ability to fit in.  They ignored it completely.  I don’t recall my dad ever uttering the words “cleft lip”.  I can probably count on two hands the number of conversations my mom and I have ever had about it.  This lack of communication about it made me feel like my problems were all my fault, that the cleft lip really didn’t have anything to do with my difficulty finding friends, that inherently my personality was flawed.

Flash forward 30 years, I’m now highly educated, have a career, have normal relationships with plenty of friends, my husband, and my in laws.  Still working towards a normal relationship with my sister and parents, because of all of the baggage.  But essentially, my cleft isn’t a factor in my life now.  However, my experiences in this regard make me hyper-aware of the barrier you will face to fitting in with your peers as a kid.

So I don’t know how to strike this balance – how to parent in a way that enables you, while not ignoring your struggles and differences.  How do I convince you that you are special, worthy of love, and give you the self confidence you need to weather the storms ahead?  I can’t pretend you aren’t different.  Your differences occupy so much of our time and so much of my thoughts.  To pretend otherwise will place barriers between you and I, and I don’t want that to happen, as it did with me and my own mother.  But will acknowledging those differences mean that I have diminished your abilities unnecessarily, that I’ve imposed my own expectations on what you can and can’t do, and that this will hurt you too?  Is it wrong of me to say to people that I don’t expect you to ever crawl?  Am I already diminishing your possibilities, lowering the bar, and giving you less to strive for?  Or is it important for me to acknowledge your real limitations, but to somehow show you that I think you are extraordinarily special despite those differences?


Following my instincts

The funniest story I heard about “following your instincts” as a parent was when a friend was trying to get her husband to have a more instinctual way of soothing their newborn son.  She handed their crying newborn to her husband, and asked him, “what are you instincts telling you to do”, fully expecting to hear “I should bounce him” or “shhh” or something along those lines.  Instead, he said, in a panicky voice, “hand him back to you?”  Ah.  Dads.

My mom instincts are generally in overdrive with you, Miss M.

This morning your dad took you to the orthopedist for a 6 month follow up.  The orthopedist said you were progressing great, he was really impressed with how well you have done in physical therapy (which he recommended against…).  A second set of x-rays show that you are likely fused C1-C2 and I think it was C6-C7 but you may have some “mobility joints” in between – we’ll continue to get x-rays to monitor whether those spaces open up or not as you grow.  We were worried about your arm range of motion, but he thought it was pretty good, he didn’t see any anatomical issues (that we didn’t already know about) except maybe a slight Sprengel’s deformity on your right side, which might explain some of the decreased range of motion on your right side.  Sometimes they do surgery on Sprengel’s, but he said it is mainly done for cosmetic reasons, and the outcomes were often not great and left a lot of scarring in that area (which is consistent with what I’d read online…). But the main reason your arms have slightly decreased range of motion is because the muscles are attached higher up on your neck because of the KFS.  Everything is just tighter…which is why we will be continuing physical therapy.

This orthopedist is the individual who first diagnosed you with Klippel-Feil syndrome.  And then told me nothing about how the syndrome will impact your future abilities, and specifically recommended against physical therapy.  My instincts did not accept this limited answer to the many questions I had.  I guess I expected that when you get a diagnosis, you get a handout on that diagnosis listing associated conditions, complications, treatments, support groups, etc.  Maybe not all of these things but some information at least, right?  Not so, in the case of rare diseases.  When I asked where I could get more information, they sent me to a general orthopedic society website.  Not exactly comforting, to know that you doctor doesn’t have any answers to your questions – “see you in six months.”  In six months, she’d have missed multiple major developmental milestones – rolling over, sitting up, and crawling.

Instinctually, I knew we could do more to help you.  So we sought second opinions.  The other pediatric orthopedists said the same thing – just wait and see.  I knew we could do more.  So I kept asking every doctor we saw, what they thought, what should be done, who should we talk to.  Finally, someone suggested we see a neurosurgeon.  I put in a call to the neurosurgeon’s office, and went through a lengthy explanation of why I was calling.  I could tell the nurse practitioner was uncertain about whether to make an appointment for you or not, so she told me she’d call back.  After talking to the surgeon, she made an appointment.  A few weeks later, I again felt like I had to justify why we were taking up this appointment slot. But the neurosurgeon was understanding, provided his opinion that she should have physical therapy, and said, you know, while you are here, it is probably a good idea to refer her for an MRI and just make certain nothing else is going on with her spinal column.  And that was the MRI that diagnosed your tethered cord, so we were able to get you in for surgery when you were 6 months old, rather than discovering incontinence and neurological damage when you were older and it was too late.

Seriously, one of my proudest parenting moments.  I knew that wait and watch was NOT the solution.    And now, 8 months later, you have had your cord detethered and have had 5 months of physical therapy, making you a rolling, scooting, and now even pulling-up, machine of a little 18 pound baby.

woe is me

I’m definitely struggling a bit right now, with the palate surgery scheduled and the count down begun.  The unfairness of what you have to go through, my sweet, just weighs on me some days more than others.

I spend a lot of time every day thinking about you, and your future.  That means I’m not as good at getting other things done in my daily life, which just adds to the life stresses and pressure, when I’m not getting the things done at work and at home that I need to.  And I spend a lot of time mentally beating myself up about that, how much I suck that I can’t seem to focus and accomplish things and that I just use you as an excuse.

I know I need to be kinder to myself.  Because the fact is, I am a different person now than I was three years ago.  It’s hard to define how I’ve changed, exactly.  I think having a child with medical challenges is an extreme parenting event.  When you become a parent, you know there are going to be changes in your priorities, in your preferences, in your behaviors.  These changes happen again, another order of magnitude of change, when you become a parent to a child with medical challenges.

I think, ultimately, I’m still grieving the loss of a “perfect” daughter.  I don’t mean to imply that I would love you more if you didn’t have medical issues – that is entirely not the case.  You love your children for who they are and what they represent – your bond with your spouse.  You have the most amazing, lovable personality, and both your dad and I love you to pieces.  But I am still working through my grief over how this syndrome is going to affect you.  You will have physical and emotional scars from the surgeries, the spinal deformations, and from just being different.  I’m still processing your lifetime of being affected by Klippel-Feil syndrome; apparently ten months has not been enough.  So, we’ll continue to learn, and grow, and stretch our hearts to accept this grief we have been given, until the grief becomes something we rarely notice anymore.


And we have a date

We got the call yesterday from the scheduler at the pediatric craniofacial surgeon’s office.  Palate surgery to be on June 8th.  A little over 7 weeks from now.  Deep breath.

Social Butterflies

We are medical social butterflies this spring, Miss M!  More opportunities to practice your eyelash batting at strangers.

This weekend the NICU where you spent your first 4 days is having their annual reunion.  We’ll be taking you for two reasons.  First, we are embracing our status as a family with a child with special medical needs – it is nice to go to a place where it’s probable there will be other families with children who have overcome amazing odds, and to feel that community support.  That’s just our life now.  The second reason is that there is a bounce house and your brother loooooves bounce houses!

I also just RVSP’d to the cleft family picnic later this spring.  Again, mostly for the same reasons – community support, and they have a train ride for your big bro.  What 3 year old boy doesn’t love train rides?!

I was surprised recently when a very observant friend said how isolating a chronic disease can be, especially for parents.  She said she still sees how much it affects her own mom (and I don’t think she was diagnosed with her chronic illness until she was just out of college, so it’s mainly affected her as an adult, living away from her family.  It has, however, had a major impact on her life, and she was even near death on one occasion).

And it is isolating.  I think that could be the title of this blog.  Isolation.  But that is too depressing.  So, these events are so important for us as a family.  We get to feel normal, and your older brother gets some special attention.  Before having you, Miss M, I didn’t get that.  I would have thought that hanging out with a bunch of kids who’d spent a lot of time in the hospital would be sad and depressing.  Now I realize it’s the day to day challenges that can get depressing, so these family events are so important to provide a place to feel normal, to feel like part of a bigger medical family.

The isolation ebbs and wanes.  As you get older, we spend less time worrying about your medical milestones, and more time enjoying you as a baby.  Which makes us more like all of the “other” families, and I feel more comfortable talking about all of the wonderful things my baby is doing with friends who have babies who don’t happen to have the same medical challenges.  I know there will be plenty of isolating moments again in the future – each surgery, each diagnosis, each milestone missed, each therapy session.  So for now, we’ll continue to embrace the opportunity to meet others who have/are struggling with similar challenges.

Ask Me, Please

I’m not ashamed of my little girl.  I know she looks different.  I see it very clearly.  But I don’t know if I see it so clearly because I know, or because it is so clear?  Do strangers notice, or not?  Do they notice that she doesn’t look like everyone else?  I guess the answer is that sometimes they do, sometimes they don’t.  But when they notice, I wish they’d ask, so I’d know that they noticed, and so that I could tell them just how amazing you are, Miss M.  Just ask me – why does she have two scars on her upper lip, what are those from?  Why does she hold her head at a funny angle?  I won’t be offended, I see those scars, that tilt, every day.  If you ask me, then I can tell you how incredibly special my little girl is.  I’m proud of her toughness, her resiliency, her shear willpower and determination.  But you won’t know about that, and I won’t have the opportunity to tell you that, unless you ask.  So, please, ask me.  I want you to know.

We’re all related

I’m beginning to feel like a total genetic FAIL has occurred in my lineage.  Maybe someday we’ll figure out why.  If we do, I don’t think it will be any thanks to our current geneticist, who told me in all seriousness at our last appointment that folic acid supplements would not fix Miss M.  Reeaaallly.  No, incredibly intelligent woman who has totally lost touch with reality, I’m well aware that a dietary supplement will not change Miss M’s structural congenital defects.  I’m not an idiot.  I was trying to ask whether there was some chemical processing deficiency on my part (and my mother’s part) which led to my congenital cleft lip (2 surgeries), my only sister’s congenital club feet and femoral anteversion (4 surgeries), and now Miss M’s KFS (2 surgeries and counting).  For the record, I took ~4 mg/day of folic acid at least 3 months prior to, and during the first 2 trimesters of, both my pregnancies (these types of congenital defects occur during the first 6-12 weeks of pregnancy).  My son doesn’t appear to be affected.  So it’s just us lucky ladies in the family!

My sister, who will be 39 years old this summer,  found out yesterday that she has yet another congenital issue – a specialist has diagnosed her with distal muscular dystrophy.  Ironically, you, Miss M, started her on this search to find answers to explain the muscle weakness in her legs, which has affected her since childhood.  Now she has a motivation to join a gym again!

Families have a way of creating stories to explain “how things are”.  My parents generated many stories.  One of them was that my sister’s leg weakness was because of her surgeries on her legs.  I don’t know if a doctor ever told them this, or not.   But that was their story, and they were sticking to it.  I’d love to be a fly on the wall this weekend when my sister finally reveals this information to my parents in person – she’s been keeping them out of the loop until she had some actual information on her condition.

Also ironically, our geneticist with the terrible bedside manner was the one who woke me up to the fact that our family story was not true.  At our last appointment, she asked if my sister had ever had an MRI.  Total lightbulb moment.  She didn’t even know that my sister has leg weakness (a possible symptom of a tethered cord), I had just reported her club feet and leg structural issues in our initial medical history, believing the family story that the rest was just a result of that.  In an instant, I immediately realized – that’s not right.  Sister does have something else going on.  Maybe she could have an undiagnosed tethered cord?  I called her that evening, and she had the same lightbulb moment and realized she needed to figure out what this was.  About 6 weeks later, she finally has an answer – not necessary the answer we expected, but an answer nevertheless.

And…I’m left with even more questions.  The genetics journey* is still at its start.  How in the world could 3 members of my family have seemingly unrelated congenital defects?  I hope there is someone out there who can help us find out.

* another funny story about our geneticist – when we tried to ask questions about Miss M’s KFS at our first appointments, she took a deep breath, kind of sighed, and said, “think of this as a journey”.  I’m not a violent person, but it took a heck of a lot of willpower to not slap her!

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