Good hands, grateful heart

So we are back in Cincy, honestly, all things considered, happy to be here.  Let me explain.  During our last visit, just an up and down for a River follow-up with Dr. Dickie, the boys were both diagnosed with a capillary malformation on their foreheads.  Not just a birth mark, or an angel kiss, that like Eden’s kiss, fades with age, no, these are port wine stains.  They darken with age, turn deep purple, and can create blisters and welts.  Considering the location of our boys stains, this could be a significant cosmetic complication if nothing else.  But, there is nothing inherently threatening about these capillary malformations typically, and they can often be treated with some measure of success with a laser capillary ablation.  However, the location, over the eyes, down the nose, forehead, back of the head can represent another potential danger.  Sturge Weber Syndrom.  Not a certainty by any means, just a danger. 

Kids born with these port wine stains on the face see occurrence of SWS about 12-15% of the time.  Yet it is not the stain that causes the disease.  Rather, it is a defect present at birth, congenital as they say, where the capillaries are malformed or exploded around the brain, inside the skull.  Furthermore, location around the eye can trigger glaucoma, or a heightening of the pressure in the eye which can lead to blindness or eye-popping if left untreated. So we had the boys eyes checked several weeks ago and all was clear for the time being, but this is something we will have to do every few months for quite a while.  So, we have been on the lookout for signs of SWS, per the instructions of Dr. Dickie.  And, while we have not seen anything conclusive, we did begin to witness movement, or behavior similar to mild infantile seizures.  Nicole caught the tale end of one of these episodes on her cell camera and we emailed it up to Cincy.  Dr. Dickie quickly convened a quorum of experts to review and put together a game plan, if any was needed at all.  It was decided that the behavior was suspicious enough to merit an EEG to check for irregular brain patterns or seizures.  If the EEG finds irregularities, they will most likely order an early MRI of River’s head to look at the blood flow around the brain and how it may be effecting the development of the brain.  If we find nothing, we will leave without anything conclusive, but we can at least rest easier and put off the MRI till he is age one or greater unless concrete signs manifest sooner. 

 This place is amazing though.  On Sunday, before we left I sent an email to Bryce’s last attending cardiologist up here just letting him know we were going to be here if they wanted to see Bryce.  He called me by 9:30 AM on Monday.  Not his nurse, Dr. Matsen himself called.  Because we have had frequent Echos we decided against looking at Bryce now but he did recommend contacting ENT because of his breathing obstructions.  So I sent an email to Dr. Elluru, the ENT specialist that took Nicole’s case once referred from Chattanooga.  He is a pediatric doctor that works out of CCMCH so he was the logical person to contact.  Within an hour I had received an email from Dr. Elluru and a call from his charge RN seeking to slide us in to have Bryce examined while we are here.  Well, turns out Dr. Elluru, like our Colo/rectal Doctor Dickie works in the vascular malformations department as well and he was rounding in that department at the very time we have an appointment with both River and Bryce, after the EEG, in the vascular department.  So, Dr. Dickie, Dr. Hammil, and Dr. Elluru will all be at our appointment tomorrow and Dr. Elluru may have to scope Bryce. 

Why the fuss about Bryce’s airway?  Give this vid a look:  http://www.youtube.com/watch?v=HnMYCCD61oA  This was taken tonight, at the hospital, with him in a normal sleeping position.  He regularly cocks his head back at a 90 degree angle while he is sleeping, seemingly to open his airway.  You can almost always hear him breathing with a whistle or a rasp and at times it becomes short or strained.   We have brought this up two times with our Bryce’s cardiologist in Chattanooga, but nothing has come of it.  In fact, there has been tremendous confusion and punting between our coordinating pediatrician and our local cardiologist about the possibility of Sturge Weber and the need for an eventual MRI.  In Chattanooga, it is difficult to get answers, difficult to get folks talking, difficult to get things done.  Here in Cindy, we can communicate openly with any and all doctors and they are blazingly responsive and they are all experts, as in world leading doctors in their fields.  I honestly feel VERY humbled that we receive the concern and care we have received here in Cincinnati.  Matter of fact, our Chattanooga based pediatrician has no idea we are up here now. 

So aside from Bryce’s breathing issues, and his leaky tricuspid, what is Sturge Weber Syndrom?  “Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenitalneurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face,glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by abnormal blood vessels on the brain surface. Normally, only one side of the brain is affected.”  Taken from Wikipedia.  There is also capillary malformation in and around the pia mater almost always on the same side as the birthmark.  This is why we were told to watch for the boys favoring one side over another in movement early on.  This condition causes calcification of tissue and loss of nerve cells in the cerebral cortex.  Symptoms have a broad range but typically include seizures that start in infancy but worsen with age.  Developmental disorders can arise including retardation and about 50% have glaucoma.  Most kids born with this condition will develop seizures in their first year, and those who show these symptoms and are resistant to treatment are very likely to see intellectual impairment. 

The important thing here is that SWS is not deadly if treated, though it may mean we have one or two special needs boys.  While Bryce has not demonstrated any suspicious muscular behavior, his marks are not very dissimilar to River’s and though it does not cover his eye, it is even more present around his head, highly concentrated in the back of his head as well as his forehead and it dips all the way down to the tip of his nose. 

I will have to say that these two boys are amazing in many ways, and I do not often celebrate it.  They are remarkably adorable, so much so, that all the nurses rave about them when they come in and their beaming blue eyes have started to be accompanied by broad smiles, giggles, and little chit-chats.  Though Bryce has been very fussy of late, sometimes inconsolably, today he had a pretty good day, at least in the first half, and can kill you and melt you with his huge smiles and tiny fingers playing across his face as he oogles.  River has been developmentally ahead of Bryce in every way, so if River is presenting symptoms of SWS, it would be no wonder he would present before Bryce if Bryce has the same condition. 

This has been difficult for us.  Surely more for Nicole.  When we returned home after our last visit in early August, she read obsessively about SWS, while I was more dismissive.  I guess her reckoning was that TTTS kids have more problems, and though the odds have been low for each of our complications, we have gotten them, so why should be expect to be spared this one.  Mine is either to remain stubbornly optimistic, or unrealistically functional by blocking out the fear and concern.  I am not sure which one, but most likely a blend of the two.  So when Nicole called me screaming, afraid that River was having a seizure; I was cool, as if I had been expecting it.  No.  I was not.  Intellectually I spoke like I had, because we knew it was a possibility, but I had not accepted that as our fate.  So I took it cool, kicked into get-er-done mode, and starting all this in motion.  While she worries more, it is always my role to reach out to the doctors and aggressively coordinate care.  Nicole is amazing though.  An amazing mother in many ways in spite of her insecurites.  Really.  Many folks could toss so much dirt on her from her Shi---y past they could bury her, but she is not that person any longer.  She has been remade, and somehow, some of her strong characteristics are amazingly strong now. 

I could not talk about it.  I could not put it into words.  I did not even know what I was feeling, but this did indeed hit me like the proverbial MACK truck.  Having twins is hard.  Having special needs twins with another baby, and 5 other kids is harder.  It has been both incredibly rewarding and taxing at the same time.  Since I got the call from N and set this all in motion, I have been irritable.  I have been short with N.  I have even had to apologize to Elise for being short with her.  Saturday tires went out in the van which set me back 500 that I did not see coming, the day before we had to leave to Cincy.  Thankfully, my amazing mom lent us her minivan.  Work has been intense.  So much professional ambition this year was thwarted by the rain, the cold, our time in Cincy, so many factors.  So I think a whole host of fears started playing out in my emotions but not my mind.  Because this past week I have not been the most pleasant person to be around.  The fear of what lay ahead.  The fear of here we go again.  The fear of complete loss of control again.  The fear of some form of loss (just after we lost George).  The fear of my failure.  The fear of our life being upended again and struggling to gather it back together again.  The fear of forward movement stopping dead or halting.  The fear of a life of hard work with high needs boys.  You name it.  The fear.  I could not put words to it, still can’t in a way, but I think it was playing havoc with me.  I felt depressed a day.  Defeated another.  All-together dull.  And I don’t always react like a man when I feel these ways. 

So coming up here was so comforting and freeing.  I feel such a great confidence and acceptance as we loaded up to leave.  We may not win what we want, but daggum we will do what we can for our boys and our family and this is the best that can be done.  And do I have any say?  No.  So I have to get to acceptance.  I have no power to effect the outcome. And as amazing a mom as Nicole is, neither does she.  Those adorable boys are on loan for use to love and raise from the Giver, our God.  He may trust us with more than we bargained for, but gosh, when Christ decided to adopt me, I am sure there have been days in his human form (ha) that he might wish he hadn’t, when I might have hurt or taxed Him more than human form could anticipate.  But he is not human.  And he knew what a smuck I could be and how high needs I could be when he adopted me.  How am I any different to my King than these boys might be to me if they indeed have SWS?