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?

More Complication, more Unknown, more Opportunity to shut up and Listen

We have now been home from Cincinnati for over a month, living in our home now four roughly two weeks after tremendous effort from our church family to make it hospitable to small human life.  The landing back into life here was a bit rough, as is sorting out life with twin infants on top of it all, but we have been, no matter how petty my attitude might become for a moment, tremendously blessed.  I suppose I could write a book over the three dimensional aspect of our experience with TTTS and the other complications which seized our affairs in February and never let go.  The things I heard in it, thought through it, convictions and questions.  I have been unpacking all that material, I continue to unpack it and it continues to convict and mold me as I listen to it then and today as if it were all in one moment.  But our days must march on.  For indeed they are full.  The list of things that need doing here at the house is monumentally large, and, for a spell, disheartening.  I even grew a bit angry by the grip this house and its expansion and renovation has laid on my life at a time when there is so much of more import to concentrate on.  I have eased back into work and really started to catch my stride and my wind of late, and that has been refreshing, though at times caused conflict as I seek to concentrate through the maze of complications life continues to toss our way. 

It has not helped any emotional adaption to current reality that our aunt Martha has been so gripped by illness of late.  Every emotion I could throw at the topic I have tried, just to arrive back to where everything must get to in the end if I am to find some measure of peace about it: acceptance.  Acceptance.  What more can I say.  It is not my place to presently unpack on that issue.  She remains a heroic, radiant light of a love for us all, even in moments that she can hardly speak from her pain she can manage to crack a smile or show some grace.  She has many friends from all over.  She has gathered them well in her places: Dickyville, Tate, Home and so on.  Folks from all walks and bents.  She expressed a wish that she would still find some light to flicker out of her in the hope of her first love in Christ as she walks through this present fire, and I for one have not seen it dim.  This, and our own immediate adventures with our boys has posed some serious questions in how I pray.  But more on that in a moment. 

Concerning our boys.  They are growing like weeds.  Even Bryce is now fed solely from the breast, no more fart making supplements for him (though he can still clear a room).  Both are strong, though River has been seemingly herculean for some time as they can perform bodily feats we are not accustomed to seeing in kids their age.  They fuss, eat, sleep, poop and sometimes, when you are holding them, flaunt about aimlessly with big open eyes.  There’s not a lot of mutual interaction at this point, my least favorite stage: the least interactive stage of life, but all systems seem to be in good order.  However, and that’s what prompted this missive.  There is seemingly always a however in this case. 

We returned to Cincinnati at the end of July for River’s 8 week post-op checkup.  Dr. Dickie, our colo/rectal specialist has a dual concentration in vascular disorders as well.  Odd that.  River was born with a defect we had not forseen: an imperforated anus.  He was born in the only place where the Pena procedure, or Pena pull-through as an immediate surgical correction was pioneered.  Had he been born elsewhere, he would have been given a colostomy bag and sent home.  As it were, he was operated on second day and all his machinery was connected, an anus was formed, and we were sent home when he was discharged, with Nicole having to dilate that opening in increasing succession twice a day for the past two months.  Yet, as “fate” would have it, our Dr. Dickey, a Pena protégé, happens to have an eye and expertise in a what seems to be another complication and it may have been one we might not have known of had we not been connected to her by his no-bottom birth defect.  We thought the mark on his forehead, on Bryce’s as well, was an angel kiss, a birthmark, that like Eden’s, fades in time only to be revealed when they are crying or angry.  The doctors here thought nothing of their marks, but Dr. Dickey showed some concern that his mark may be a Vascular Malformation, or, a port wine stain – something much more impacting than a birth mark.  However, she also initially expressed concern not only because of the intensity of the discoloration, but the size, placement, and reaching for his eyes.  Evidently, vascular malformations around the eyes often times point to possible complications with blood pressure in the eyes: ie, glaucoma.  So, we have been ordered to have their eyes checked and possibly medicated should signs of heightened pressure present.  However, the proximity of the malformations to the eyes are not the extent of the potential complications, but this remains very much in the unknown.  These particular capillary malformations can be associated with lesions of the brain (and eye), called Sturge-Weber Syndrome and can lead to neurological developmental impairment, seizures, because the meninges (covering around the brain) may also be affected.  In severe cases, paralysis can set in on one side of the body and severe retardation may be resultant.  While not all kids who have capillary malformations on the forehead and eyelids have the congenital condition of Sturge-Weber Syndrome, these malformations are associated with this condition and the symptoms of this condition can vary greatly from mild to severe.  What this means is, beyond the eye checkup, we are recommended to seek an MRI of the brain to determine if they may in fact have this syndrome or if the malformation is strictly more superficial.  If, as we obviously hope, it is superficial only, the boys will both need to undergo an uncomfortable a pulsed-dye laser treatment several times to potentially destroy the damaging blood vessels without damaging the skin thus preventing the boys from developing the dark purple staining which can even raise from normal skin level as it hardens in time if left untreated.  So it appears, at the least, we have a bit more to road to how medically speaking, even in the best case scenario as we will need to seek laser treatment from a skilled treatment surgeon in this field, and frequently have their eyes checked for glaucoma.  We do still have the dysplastic tricuspid to deal with in Bryce’s heart, and, our team in Cincinnati may advocate an open heart to attempt repair of the valve as early as 10 months old depending upon how much regurgitation his echo’s continue to read.  His latest did not detect any lessening of the leak post second coarchtation repair, but it could still improve enough in time to push intervention even further back. 

This brings me to the topic of prayer.  How do I pray.  Honestly, I just don’t know.  And I can’t pretend to tell you how to approach the topic concerning intervention for others is concerned either.  I feel less comfortable asking for a specific outcome these days as I feel that is too impositional and self-centered.  Besides, we do not know the why.  We do not know what is good for us, good for all, good for the overall plan, we only know what we want and what we don’t want, but if we have lived long enough, we know what we want may be that which hurts us in the end, and what we don’t want, ie, hurt, may be that which helps us in the end.  So what do I pray?  I certainly do not want to see my boys suffer.  I am certainly deeply pained to see Martha, Freda, Patty, suffer.  So I guess I have to pray that good would be done, and that whatever that is, we would, I would be given the eyes to see it, the good, even though it may look terribly bad at the time.  There was a reason our plans were taken from us this year.  A reason I had to spend months at the RMH in Cincy away from our fam, six weeks with Nicole in the hospital.  It wasn’t for kicks and giggles and it wasn’t so I could taste Ohio ice cream.  I hate that Martha’s illness does cause her to suffer. Yes, it has really angered me.  But that may be in the plan and no amount of pleading may change that.  I cannot see any good that could come of it off-hand, or is coming from it, and then I can when I pray that my heart is only open.  Open to hear.  Still enough to see.  Because often I can’t.  Often I have cotton in my ears, blinders over my eyes, and mucus around my heart.  So right now, my prayer for my own life is one of acceptance.  That I will accept what ever it is I am dealt today.  That I will accept whatever it is I am dealt tomorrow.  But that is about me.  That is still selfish in nature.  Still essentially self-centered.  So How do I pray for River, Bryce, Martha, Freda?  Do I pray for exact outcomes?  Sometimes I do.  I really do, even though I am never sure about it, I am there for the asking.  Or do I pray that God would be glorified, come what may?  Surely I do.  Surely He will.  So why even pray it?  Is that prayer not an act of worship and submission meant to open us to the broader reality that God has to share with us?  None-the-less, I do ask for prayer, whatever way, whatever type, whatever silent worship you feel led to enter into, for our beautiful Martha, and our two little boys, my dad, and all those so closely connected to them, each and every one. 

Another milestone, another gracious day given - surgical guarded success

I am seated by Bryce’s side, now late at night.  Myself, alone.  My beautiful bride is back in our taxing room with River and two other of our kids.  I hope she sleeps.  I doubt it frankly.  

To be honest, she, and myself less, suffered more from this last take-from-home surprise than all the previous jolts in our journey.  We left so full, free, high even from our early release …. From the truly remarkable strength of our little men.  These little fighters who must know they are already so loved, so wanted, so doted over, that they must know each of their community and familial loves first hand.  They will fight through to see us all through it seems.  For every response to a terrible situation has been astounding. Yes, abnormal even.  Take the latest with our Bryce.  We came up following a BP cuff read and confirming echo which read the difference in blood pressure from his arm to his leg at 50 points.  His leg pulse was almost indiscernible.  So he was transferred under medical protection.  When we arrived, I led the EMS through the streets of Cincy and the process of entry in the emergency wing and we were direct admitted to the room beside the one he inhabited after his birth.  That night, the difference read the same.  Things were dicey. How long could his body tolerate such paltry blood supply to the lower portion before his kidneys started shutting down and his legs changing color?  Then the next day, things seemed to level out.  The difference as read by cuff declined to 25, which shoved our case from operate now to operate very soon.  So stable was our boy, so normal his GI functions that we were transferred Saturday to the floor, out of the CICU.  Here in this spacious room on level six of the A building, his brother can actually sleep at the foot of his crib with him and our kids have unlimited access to their brother.  That makes it infinitely easier on us. 

We had our meetings today, though not with Dr. Morales, the chief of cardiovascular surgery who will perform the operation.  In our consult with Dr. Cnota, from team of cardiologists, we learned a good bit more.  He claims that in Dr. Michelfelder’s 22+ years of experience in pediatric and fetal cardiology, he had never seen a coarchtation occur in a TTTS twin.  They have had a few valve anomalies such as Bryce’s dysplastic tricuspid that manifest unresolved after the TTTS laser correction, but never a case connecting to coarchtation.  Furthermore, it is very rare for a coarchation to occur in two different places around the aortic arch.  Nonetheless, it appears that the narrowing in his aortic arch has caused pressure to increase in his lungs because the left ventricle is overworking to service the impeded lower organs thus increasing pressure at or above the heart to hypertensic levels.  This increase in pressure has applied heightened pressure once again against the left ventricle (which pumps blood to the lungs) causing the level or tricuspid regurgitation to increase to levels measured just after delivery when resistance in the lungs was greatest.  This is not alarming, and should most likely resolve after the coarchation is removed, but the tricuspid continues to leak vigorously either way.  So now, fast forward to the day of surgery.

Dr. Morales paid us a visit around 8 am to discuss his approach.  Evidently, the team meeting yesterday had many attending and was a rather lively debate with many attending cardiologists as well as the surgeons.  The meeting adjourned and the plan was to open his chest, put Bryce on by-pass, and perform the arch advancement with possible elective tackling of the dysplastic tricuspid repair.  However, Dr. Morales spent the evening weighing all the input he had received and simply came to tell us that he was going in, putting Bryce on by-pass and was going to fix what needed to be fixed the best way to fix it.  He had not set agenda on how to pull that off.  Furthermore, he had already determined NOT to address the tricuspid issue, primarily for two reasons.  One, if he attempts a repair, he circumvents the bodies opportunity to possibly resolve the issue over time, on its own.  A natural repair is always a preferred repair.  And thought the leak is not likely to ever stop short of intervention, it is possible it could slow to the point that medication could sufficiently treat it.  The other has to do with the physiology of the valve material at this early age.  Evidently, reshaping a valve this early is far less likely to produce the desired outcome because of how supple the material is.  However, over the next 6 months or so, the flesh in the valve alters and evolves and success in a repair effort becomes much more probable.  So, he thought the risks of going back in were much less than the potential loss of either preventing natural repair or insuring a greater chance of success in the repair at a later date, as in, between his 8^th and 12^th month of life.  He was going to look at the valve with an internal echo, but felt somewhat convicted to heed the counsel of the cardiologists and resist the temptation to intervene so early. 

Fast-forward once again to post-op consult at 5 pm.   Bryce was taken from my arms outside the OR at 9 AM, so this was an all day affair.  Morales said the procedure was very complicated.  It was, in his words, difficult to perform for several reasons.  One, the coarchtation was limited to one place, however, there had been substantial thickening in the wall of the aortic arch over a large segment, essentially the entire arch.  Because of this, it made not sense to perform the arch advancement where the descending portion is attached frontally just above the heart and the descending line is cut off and sewn into a basket.  This because there would still be the problem of the thickened wall and narrowed passage to the ascending arteries, but also because his descending line was not plastic enough to relocate to that extent.  So, he had to filet a long section of the arch and graft a patch over this portion composed of Bryce’s own pericardium that roughly doubled the size of the aorta in this area.  The flesh of the artery will grow over the patch in time.  The other complicating factor was the placement of the laryngeal nerve, which had essentially braided itself, abnormally, around the aorta.  This nerve had to be moved to complete the reconstruction of the arch.  In doing so, it had to be stretched.  That stretch may have damaged the nerve with possible side effects of poor utilization of the vocal cords for noise making and difficulty swallowing liquid without choking.  He seemed genuinely concerned about these possible side effects from the procedure but was not certain it would occur.  Furthermore, it is something that can resolve in 2 to 3 months so long as the nerve was not inalterably damaged. 

Finally, the team here is somewhat confounded by this case.  They have no idea what would have caused the second coarchtation, again in an atypical location and disassociated from the ductus.  They also have no idea what would have caused the aortic line to thicken as it had.  Because of the nature of the repair, we are very unlikely to have issues again with any of the troubled area which was repaired, however, it remains a concern to Nicole and I that if this anomalous narrowing and thickening could have occurred as it had, in various places as it did, that it might happen again with another portion of the aorta or another artery elsewhere in the body.  Because it was apparent that there was problematic tissue in this specific area, they believe there is greater likelihood it is an isolated incident.  But we will not know till time has proven so. 

Our petition for our youngest son is as follows: 

1)   That there would be no damage to the nerve and consequent complications associated with it.

2)   That this would in fact proof to be an isolated incident attributable in part to defective tissue.

3)   That extensive inflation would not occur where sutures were made or new flesh was attached because Bryce does not seem to tolerate foreign intervention in his vessels well.

4)   That the tricuspid leak would wane to the point where it is medically manageable and a reconstructive surgery will not be required, at least until well into adulthood. 

5)   Finally, that his recovery would clip along at an acceptable rate, that he will take to eating once again, breathing normally on his own, and that we might be discharged within 7-10 days. 

Please forgive me at this point for drawing to a close at the end of a rather dry presentation of medical data.  I haven’t the mental or emotional reserves to offer any personal reflection.  I cannot say we are dramatically relieved.  We are not high fiving.  We do not feel in the clear.  We have never really been in the clear and every time we think the end of the tunnel might be approaching, the light turns out to be a train.  So we walk in faith, one step, one day at a time.  Doing what we can to make the most of our days, the most of our relationships, the most of our struggle even, and doggedly clinging to a complete trust in a plan we cannot know.  Too much celebration would be ill-advised.  Too much foreboding would amount to self-pity.  So we pleasantly trust our way through the unknown.  And THANK YOU each and every one of you that has said a prayer, spared a compassionate word or thought, given support, and cared about our family.  We are deeply grateful.