Abnormal is Our Normal

It is my job to stay healthy and strong; to monitor everyone else’s lumps, bumps, and discomforts; always on the lookout for anything “abnormal”.  My most extensive surgery was when I was 19 and had my wisdom teeth removed.  I do not carry the Li Fraumeni gene, but I do fit in with my family – like them, if something is going to happen to me – it will be abnormal.
About five years ago our family was out hiking and I fell – apparently I had broken my tailbone.  A week later I was still not walking well and reluctantly went to see the doctor.  While we waited for X-ray results he drew me a picture of a tailbone and explained that it was lucky we have a tailbone to hit rather than breaking our sacrum, (the flat, triangular bone above the coccyx) because so many muscles, etc. are attached here.   Of course – I had fallen while going downhill on top of a large, fallen tree and the angle had caused all damage right there on the sacrum – three months to heal.  My dermatologist still shakes her head in wonder that the mole she removed “on a hunch” turned out to be melanoma-in-situ; and worse is her reaction to that “thing” she removed from my back “the worst one she has seen in her career” – …lovely….(she still shudders when she talks about it)…
But – this is getting gross – enough about me –

We hadn’t seen this MRI view before.  Greg
saw Dr. Hayden recently and we asked if we
could see an image of his sisters’ tumors.
The large white thing attached to her spine
is the chordoma.  Ugh

Today my post centers around Jackie – anything abnormal there??  How about that chordoma?? – the rare cancer that occurs in middle-age men with tumors at either end of the spine – Jackie is a teenage girl and for her it grows right in the center of her body.  Abnormal can also be good – it was operable, she did not lose any vital functioning, and there is (so far) no radiation treatment.
From the day she was born she has been kind, gentle and patient (perhaps excluding some events hovering around age 14 – no one should count those years).  I was always appreciative of this – such a nice addition to our family.  I saw it very clearly when she was about three years old and I took my two little girls to California to care for my mother who had just undergone bypass surgery.  Jackie was so young – yet so attentive.  Her grandma still talks about how Jackie was ever at her side – sitting quietly and gently, just awaiting an opportunity to help.  (Natalie also wanted to be by Grandma, but she climbed all over the place.)

Henoch-Schonlein purpura
This isn’t Jackie – it is from the Mayo
Clinic.  We have similar photos
somewhere – with a more blueish
rash – it changed everyday!  Strange!

I was more impressed when she was in second grade.  Our house is 1/2 mile from the bus stop.  One day she and Greg came home and reported that Jackie’s legs were hurting – he had carried her home!  That seemed odd – then she woke up covered with large, unsightly bruises – or at least what looked like bruises – covering her legs.  Fortunately, we have a good friend who is a retired pediatrician.  He happened to be at our home and without hesitating gave her the diagnosis of Henoch-Schonlein purpura.  Of course – everyone has heard of that!  The way it turned out – this horrible rash would come and go and look completely different every single day.  Some days one or both of her legs would quit working – other days it was her arms.  We went to the doctor at least once a week for six weeks to draw blood in order to monitor possible kidney damage.  I just remember her – so cute and little – sitting in the family room with both arms propped up on a big pillow to relieve the pain while we spoon fed her (she was unable to even lift her arms on her own).  Six weeks of never knowing what joints were going to give out on her and when.  She missed a lot of school and wasn’t able to join in for PE or recess… NEVER a complaint.  Mostly she was proud of her own courage at getting all of that blood drawn!   I watched this little girl accept her condition with such dignity!  I probably had a sense of foreboding that this would not be the last time.
After the Li Fraumeni diagnosis and initial screening last November, there were three main concerns for Jackie.
1. Chordoma – trumps all other concerns.
2. Liver – a spot that we have still not investigated further due to chordoma, phone tag with oncologists (and misplaced MRI), Natalie’s brain tumor/recovery, other distractions and mental fatigue.

1st or 2nd grade? – so brave!

3. Parotid gland tumor – this is at the bottom of the list because it appears to be benign for now.  Mark had the same tumor removed a few years ago and his was benign, but the surgery—yuck.  They told us we could wait a bit, and we are waiting.
Jackie also has three other health concerns that we have been watching and dealing with for 3-5 years.  They are rather personal, and out of respect for her privacy, we rarely discuss them.  I will label them A, B, & C.
A – More of a long-term concern.  I worry mostly because of “B” – I think that the combination signals an underlying problem that we have, as yet, been unable to identify.  My hope is that the horrible chordoma had something to do with it.  I cannot help but feel that even if there is no direct, medial correlation – there was certainly plenty of “stress” on her poor body by having that large, nasty growth right in the middle of everything.  Perhaps within the year she can return to a more healthy state.
B – This is strange and has doctors perplexed.  We have been to several specialists but they don’t have answers and looking back I see that they were distracted by things like Li Fraumeni or the cyst that was on her thyroid.   Ineffective treatments – Unknown etiology – I am hopeful that having that horrid tumor gone will improve the situation.
C – Well, this one bothers her the most, but it should soon be taken care of.  Soon – like in the next several hours.  Surgery can help to correct the problem, at least for now.  It took us awhile before we had a doctor sit up and pay attention – and when they began to do that —Yes!  This is very abnormal and needs to be taken care of – we went to a specialist who sent us right away to a surgeon.  That was about 16 months ago.  The surgeon has been wonderful – he is acquainted with Li Fraumeni and was very supportive of us during the testing period – and all of the “waiting for insurance to approve testing” time.  He wanted to wait and hear from our Li Fraumeni specialists at the Huntsman Cancer Institute – and then followed us through the further testing at OHSU.  Huntsman doctors helped us rule out cancer as a cause and they talked to other specialists who suggested another rare and strange syndrome that no one else has heard of.  MRI ruled out that syndrome – so we are once again left without a “reason” – just an abnormality.
We don’t need a reason (though I am curious) – we are all just relieved to have surgical treatment prior to Jackie leaving for college in the Fall.  She is very disappointed to be missing her girls camp next week – she was on the youth leadership committee and has had a great time planning and working with the other girls… but our other choice was to wait over a month – this would not give her the six weeks that she needs to recover.
I expect that Jackie’s recovery will go very smoothly.  She has a very positive attitude and has relatively good health.  I know that people love Jackie and will want to know how she is doing – I am just asking that all will respect her privacy – no need for details, just her general well-being.  I asked her permission to blog about all of this because I believe so strongly in the power of prayer.  We have been the recipients of the prayers of so many for what seems like an unbroken chain of events since 2014 began.  Well, kind of before that….  We are blessed beyond my comprehension – everything turns out so well, and we have so much happiness.  I would ask that all who read this will remember Jackie (and her tired parents) in their prayers – not just for recovery – but perhaps what we really need to help take care of “A” & “B” is a great combined faith effort; to bring healing or to know what to do to help the process.  Heavenly Father knows what “A” & “B” are – Jackie and I discuss it frequently in prayer.
Surgery will be this afternoon (strange timing – my kids are usually first case, rather nice to have a few hours this morning).  She will need to stay overnight for observation and we should be home tomorrow.

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