Tag: Cowden syndrome

If they would just listen to Meghan.

I mean really listen, and stop dismissing her.

Since before she could talk we have been working to help this kid become more aware of her body.    She can troubleshoot what works for her.  She can tell me what foods and medicines help her or hurt her.  Every day she becomes more keenly in tune with herself.  Its necessary.  That is how we keep her healthy.

My heart always gets heavy for my friends whose children, despite everyone’s best efforts can not articulate their needs, pains, desires, or emotions.  Those are the situations where doctors might miss something- even if they are trying.  Those poor children might suffer needlessly if no one can determine the source of their discomfort.

But not Meghan.  If you will listen.  She will tell you.  If you pay attention.  You will understand.

If she hurts- the whole world might know.  If she doesn’t hurt anymore, even 5 minutes later, its like it never happened.

That’s it.  Plain and simple.

So when she hurt her hand June 4th, I kept waiting for it to get better.

I played tough for a few days, but it still hurt.

Then we tried ice, heat, brace, no brace, different brace – but the pain persisted.

Dr. Jill, our Physical Therapist kept saying, “I think it’s vascular.”

Meghan kept saying, “It pulses like my AVM.”

And on we went – to the vascular surgeon, for the MRI/MRA, to the orthopedist, to the local hand surgeon.  Each one finding a reason to dismiss her, and this pain that persisted.

Pain doesn’t persist.  Not like this.  Not with her.

And, when you have Cowden’s Syndrome, any pain that persists more than 10 days consistently really should be evaluated.  You never know where those tumors may grow.

But the MRI, the three hour MRI with 4,000 images was negative. (Although I still have my doubts.)

And, Meghan calmly told the vascular surgeon she thought it was an AVM.  He said no.

As the pain mounted over the weeks that followed, and we bounced through other routine follow ups that seemed to swallow up our summer, Meghan took solace in Dr. Jill.

You can’t put anything over on Dr. Jill, but she knows kids, and she knows Meghan.

“Look at this,” she said to me.  “It has its own blood supply.  That’s why it stops hurting when she puts pressure on that mass.  She is cutting off the blood supply.”

Meghan would smile, knowing she was understood – at least by someone.

“Vascular pain goes up the arm, orthopedic pain doesn’t,” she would almost  mutter to herself.

And I knew if they were BOTH sure, then I was sure they were right.

I emailed her oncologist, and her genetecist.  I sent them pictures.  “Biopsy those,” I was told in no uncertain terms.

So Dr. Jill wrote a detailed professional letter to a local orthopedist.  I am still not sure he understood all the words.

Then there was the local hand surgeon, who read the letter, and just spent the visit looking perplexed and bothered.  The MRI report was negative, his Xray was negative, and there was this bump below her middle finger.  He sent us away – apologizing for not helping, but convinced there was no need for surgery.

That was the day Meghan coined the phrase “Donkey Butts!”  And I couldn’t blame her.

I went right home from that visit and sent a desperate EMail to the oncologist again.  This time I asked for a referral.

She is a woman of few words.  Her reply was a link, and the words “Go here.”

So, I called and made an appointment.  They took our out of network benefits.  We got in the day before vacation.

He looked at Meghan.  He talked to her.  He sent her for more Xrays.  They were negative.  Then he looked at her hand again.  He told her that he knows all about kids and how well they know their bodies.  He told us he treats a few boys who grow bone instead of soft tissue tumors.  He told her that the boys ALWAYS know where the problems are.  She brightened.

Then he drew a very logical picture of the hand, and traced out the main ligament and tendon.  He showed the connection from the soft tissue tumor to the painful wrist spot.  He let her feel it, and he told her she made sense.  She smiled a huge smile.

The he said to us that even though the Xray is negative, sometimes surgeons have to use their heads.  Clearly the tumor on her hand was causing trouble.  Clearly it had to come out.  Exactly what that would mean once he got in there would have to remain to be seen.  Relaxed and encouraged that someone was using their head, we scheduled the procedure and left for Disney.

Disney had its ups and downs, and one of the struggles was the frequent hand and wrist pain. Meghan is in pain so often, she can differentiate between the chronic pain, like her hips and knees, and the pain that she can’t stand, like her wrist and hand.  Chronic pain is absolutely exhausting.

Today was the surgery.

We went to a LOVELY outpatient facility on 42nd Street.  The place was clean, the reception was smooth and effortless.

The nurses were darling, every one of them.

The anesthesiologist instilled comfort, and the surgeon, Dr. R was warm and calming.

The procedure lasted longer than I expected – almost an hour and a half.

I wasn’t surprised to hear the words that have become normal. “It wasn’t exactly what I imagined, but I am pretty sure I got it all.”

A soft tissue tumor, with roots, AND an AVM (arteriovenous malformation) with its very own blood supply!

So many thoughts went through my mind – including, “SHE KNEW IT ALL ALONG!,”  and “THANK GOD FOR DR. JILL!”

I was grateful for Dr. R, his patience, his trust in Meghan, and his ability to get it done.  I was also instantly tossed into a new level of worry.  See, all along they have been insisting the AVM in the knee was an isolated incident.  I shouldn’t worry about more cropping up.  Well one just cropped up.  Game changer.

I was thinking about the rock wall she climbed June 4th, and even after all this turmoil, I am still glad she did it.  Although we now almost undoubtedly know that the AVM was under the surface all along, and the bruising her hands took caused her body to “hyper heal,” and likely led to more blood being pushed towards the AVM.  The soft tissue tumor erupted some time later.

While the exact sequence of events doesn’t matter, a few things do.

Meghan and Dr. Jill are really intuitive.

AVMs can take place in multiple spots.

Dr. R had to scrape the scar tissue off the ligament, and tendon and nerve in the hand.

Meghan will need quite a bit of therapy (after 9 days of keeping the hand wrapped) to get her mobility back.

I absolutely can not wait to get the pathology report and share it with a few doctors who blew off my girl.

And, most importantly – NEVER DOUBT MEGHAN!