April Appointments

Wow!! It's been so long since I posted! Life is crazy busy around here.  But its a good crazy : ).  Cole has been walking, well, running all over the place now for months.  He's growing and thriving and every bit as busy and inquisitive as his siblings.  Inquisitive is the word we use for curious and somewhat naughty toddlers in the Haveman house.  Lol.  He never stops finding things to get into and climbs everything in sight.  He is definitely doing justice to his nickname, Monkey.  He keeps us all on our toes and laughing everyday.  Just this last week or two he's decided he is going to give as good as he gets with big brother Logan, and has started to pull on his hair, push at him, and thump him on the head with the remote control.  Good times.  He loves cuddling his big sisters. He's such a determined and smart little boy.  His latest trick is taking the older kids' snack wrappers and walking them to the kitchen trash to throw away.  So cute.  Love this sweet little guy. 

Isn't he just adorable?!  We've been blessed with so many people praying for and inquiring about Cole's health that I wanted to make sure I updated everyone.  We had our latest appointments with both nephrology and urology the first two weeks of April.  Unfortunately, the ultrasound showed no improvement regarding the dilation of both kidneys and ureters, and his bladder remains quite distended.  As I think I wrote on an earlier post, the original plan, even prior to knowing the extent of his renal damage, was to observe up to 18 months of age in hopes that the UPJ obstructions might open up on their own, at least to a significant degree, before deciding on surgical intervention.  As this hasn't occurred, we are tentatively penciled in for surgery at Devos on June 25th, following another VCUG (bladder xray) on June 5th, which will determine the extent of remaining (if its improved at all) reflux.  This proposed surgery will attempt to correct the reflux, meaning the ureters will be reinserted into the bladder at a different angle.  Cole's ureters are also hydro/megaureter, meaning they are grossly dilated, and rather than functioning as they normally would, are large like colon, and have twists and sort of snake around, pooling large amounts of urine.  So, this surgery will be complex in that it will also involve reconstruction and tapering of the ureters.  It's estimated at 6-10 hours long : (.  I think Mommy and Daddy downers may be in order that day.  From his experience, the surgeon feels that the obstructions are likely due to the twists in the ureters, rather than some intrinsic narrowing or extrinsic compression, and predicts that the UPJ will likely resolve/improve by straightening/reconstructing the ureters during this surgery.  Sort of like smoothing out a partially kinked garden hose.  That said we won't really know for some time following the procedure, and there's a possibility a later surgery may be required to resolve the obstructions.  That would involve disconnecting his ureters from the kidneys and reconnecting.  There are doctors that operate on both ends of the ureter at once, however its viewed by his Dr, and us, YIKES, as reckless, as the ureter receives blood supply from both ends, and runs a risk of becoming necrotic if completely severed from both supplies at once. We had mixed feelings regarding operating at all over the past months, as Cole has been growing, thriving, and infection free since he was 3 months old.  Obviously you want to do all you can to be pro active in maintaining his native kidney function as long as possible, and the reflux and dilation can potentially damage the remaining healthy tissue.  However, it's infection and scarring that have the greatest potential to wipe them out.  This surgery carries a 30% chance of subsequent infection, and it takes approximately 6 months after successful treatment of infection to resolve the inflammatory process that occurs with it, and bring his risk back to base line.  While we'd be hyper vigilant, it's scary to think that we may take our thriving child and subject him to those possibilities.  Especially as the most important thing is that he grow well so that he can easily except an adult kidney if that day arrives sooner rather than later.  We were also feeling, as nephrology has told us a transplant is imminent, and with transplant his existing ureters are removed and hence obsolete,  that it seems to be a matter of how much time are we buying him by correcting the plumbing, so to speak? Six months? Six years?  We know there really is no way to answer that question.  We truly didn't know which way to lean.  At this last appointment, however, it was explained that there are positive implications regarding transplant for this surgery as well.  Because of the degree of reflux, and the fact that his kidneys can hold up to about 40cc's of urine a piece, Cole distends his bladder prior to urinating every time.  He then voids a certain amount, not emptying his bladder, as the urine takes the path of least resistance, and slosh's up into his ureters and kidneys until they are filled to capacity before traveling back down to his bladder, distending it, at which point he urinates again.  Because of this, he will always have this grossly distended bladder, until the reflux is resolved/improved.  If this was not corrected surgically, at the point a transplant was needed, he would have to have his kidneys removed months prior to receiving a new one, bridging the gap with dialysis.  That would allow for a "dry bladder", which causes it to contract and shrink back down to an acceptable size for it to function appropriately and allow for minimal potential of harm to the donor kidney.  I didn't realize this prior to Cole being born, but when you receive a transplant the new kidney is placed low, to the side of the bladder, behind the pelvis, to allow for better blood flow and more protection.  By the nature of its position and the angle of the ureter, all transplanted kidneys have some degree of reflux, which makes the bladder distention a problem.   If the bladder issue is resolved or improved with this surgery, it would likely be possible to remove the old kidneys while transplanting the new, and avoid dialysis all together.  So, knowing that even if his current "plumbing" becomes obsolete, there is still a positive attribute to putting him through this surgery in regards to the transplant, has helped us reconcile the risk vs. benefit.  From the surgeons perspective, he's at an age for a fairly straight forward surgery.  In the best of hands (his Dr's writing books on this stuff), there's a 70% chance of resolving the reflux, but a 95% or greater chance of improving it.  And it can't get any worse.  He did say that we would rather error on the side of too loose and allow for some degree of reflux to remain than make them too tight and create a new obstruction.  So there's that.  It involves a number of stints, I know two are external, running from the kidney, through the ureter wall, into the bladder and out thru his side, draining the urine from the kidney until the inflammation of the ureters subsides, about three weeks, at which time they're pulled in an office procedure.  A third, and I can't recall if there are three in total or four stints (We'll know more later), is internal, and hangs into his bladder.  This one can hit the sides of the bladder causing painful spasms.  Sounds like they try to control this with Ditropan.  It's pretty nerve wracking.  He's just so little.  I hate that we ultimately have to make the decision, vs a Dr. just saying "he HAS to have this".  Lots of praying.  That said, both Mike and I have come to a place where we've accepted we have no control, who does, really, and there's an immense peace that comes from truly giving everything up to God.  Our faith has grown a million times greater than I ever could have imagined. It's been a long year, but were getting there.   I feel like we've made it to a place where we know not only will life go on, but its going to be GOOD!!  Instead of being all consuming, it feels like this is becoming just one piece of our lives.  None of us really knows what tomorrow holds, and now more than ever we really appreciate even the smallest of blessings in our lives.  Right now we're praying for him to remain infection free following the cath needed for the VCUG.  Thank you all for keeping him and our family in your thoughts and prayers! Means the world to us!!



VCUG at 12 hours old



Bladder with megaureter visualized



And finally, I'm able to look at Cole's imaging with out wanting to break down.  So for all of our Dr. and nurse friends who've been asking, here's a couple shots from the Dec 2012 ultrasound, with the exception of the VCUG, which was done about 12 hours after birth.





Right Kidney





Left Kidney







Cole's Crawling!!

It's August 4th, and you're seven months old today!! Sitting here this morning watching you crawl all over the place and doing acrobatics with that giant smile that never leaves your face, strong and defying every odd Dr's could give.  I've spent months wondering why God gives children such struggles.  This morning, I think I know.  My faith in Him has grown deeper than I ever would have believed possible; I believe in miracles.  Big ones in tiny babies.  You are such a blessing, not just to our family, but to all who's lives you touch. I love you buddy!!!

June 26th Nephrology Appnt.

We saw Dr. Steinke today following a renal ultrasound at Devos.  No change to note in the hydronephrosis. It's still off the charts but hasn't gotten any worse, and we'll take that : )  Cole's growth looks good, in about the 75th for his length and 21st for weight, hanging out at about the 25th percentile over all.  We're so happy and relieved to see that he's growing so well!  They'll continue to watch his progress closely, as his kidneys are largely responsible for his growth.  We talked to the dietitian today about baby foods (he just started cereal).  She had a list of low potassium foods...this is going to be a challenge.  More on that later.  Cole's lab work showed that he was iron deficient (iron stores a little low, hemoglobin ok), and that his bicarb had dropped back down a little, so we've added a multi-vitamin, increased his bicitra to 5ml twice a day, and up'd his amox. to accommodate his growth.  So, fluoride included, we're doing meds 5 times a day now, which is a little overwhelming, mostly because it can be tricky to time that many doses with meals when you're on the move and out and about with other kiddos.  But he's doing great, staying infection free, and HAPPY!!  GOD IS GOOD!!!  We are so blessed!!

Getting So BIG!



Cole with his Jul's



Cole is changing so fast! He's rolling over, and just figured out last night that if he keeps rolling he can get to his sister's toys and shove em in his mouth, much to her dismay, lol.  He loves to babble, and Mike is convinced he heard Daaaad (yeah lots of wishful thinking there).  Mostly its a lot of bababa and one little ma. (It's my blog here people.)  He is pretty in love with his older siblings, especially Julia, who just loves to "mom" him!  He gets the biggest kick out of watching them run around being wild little things; he kicks his little legs and shakes like "let me at em". 

He also LOVES to be outside.  It's a surefire way to calm the crabys.  So much out there to look at, he's in awe.  We went out to the state park the other night and grilled hot dogs and smores, and took the kids for a walk.  He was so happy out there!

 

May 24th Appnt

Cole had his appointment with the peds urologist, Dr.Steinhardt this morning in GrandRapids following a renal ultrasound at Devos.  He said that the hydronephrosis appears worse on the scan, however his bladder was full at the time they took the measurements, and they didn't record the images after he voided.  Because of the severity of Cole's reflux, along with the fact that his kidneys essentially act as another resevoir for urine, much as the bladder does,(the center of his kidneys is hollow and full of fluid for lack of better explanation with out a visual) when his bladder is full it backs into his ureters and bladder which can account for the hydronephrosis appearing slightly worse.  He didn't think it was indicative of causing more damage.  By nephrology's "equation" which is a combo of lab work, weight and height, Cole's number puts him in stage 2 renal failure, at a 61; stage two ranges from 80 to 60, stage three beginning at 59.  There are five stages of renal failure, stage two being unsymptomatic, determined by lab work.  You wouldn't find it if you weren't looking in most cases.  Stage three he would begin to experience high blood pressure, etc.  The last letter we received from nephrology explained that Cole's creatinine level can be falsely indicative of better renal function than is actually present, and they feel this is the case.  Dr. Steinhardt explained this is because it's very difficult to obtain a truly accurate picture of renal function in infants because they aren't large enough to get a body mass index, 24 hour urine, etc.  He was happy with his creatinine however, very happy I'd say.  It's trended down so much more than they ever thought possible as suggested by the imaging.  He told us that he feels crystal balls can be flawed, and not to give up hope for him bypassing a transplant, or at least making it to his teens or early adulthood prior to needing one.  He said while it looks very likely he will need one, he doesn't think anyone can say for certain; that he's had a couple of patients look like Cole does and they astound everyone and become adults with weak, but functioning kidneys.

He told us that the two offices, urology and nephrology had their monthly meeting of the minds the week before, and that the nephrologists felt he needed to correct the reflux and the UPJ obstructions to stop the infections and protect his kidneys from further damage from the obstructions.  The idea is obviously to get his native kidneys to sustain him as long as possible.  Dr. Steinhardt along with his partners disagree with doing the surgery as a rule before one year of age due to a lower success rate, increased complications (such as losing the kidney and needing dialysis) and the fact that at one year of age the angle the ureters enter the bladder changes as the abdomen elongates, dramatically reducing the risk and frequency of infection in boys in both children with anomalies such as Cole's as well as in those with out.  He explained that nephrology feels there is need to go in now and correct the obstruction as they think that it is potentially further damaging the kidneys, and he has such little reserve.  Urology on the other hand does not feel that its possible for it to be true UPJ obstructions, only a partial at this point, as on the VCUG at birth the dye freely flowed into the kidneys, because of the way the dye drained on the nuc med scan at two months, as well as the urine that is back washing now- this in their opinion is not possible with a complete obstruction.  As for the reflux itself, there isn't anyone saying that they feel "certain" that it in and of itself causes damage to the kidneys; agreeing that it does put him at a higher risk for infections that can damage the kidneys, especially if not treated promptly and aggressively.  He shared a study with us that he had compiled, children with one kidney that were followed into adulthood, or until their kidney had reached it's "adult" size.  The study was compiled of two groups of 25, one comprised of children with relux, the other without.  At the end of the study it was found that the one kidney grew to a larger than normal size to compensate for the lack of a second kidney in both groups, however, the patients with reflux measured on a smaller curve than those with out.  The question arrises then, does reflux in and of itself cause a weaker or damaged kidney?  The children in this study were NOT devided however according to those that experienced accompanying infection vs. just the reflux with out infections, so it was inconclusive as far as helping us make a decision for Cole.  Steinhardts opinion, along with most of the urologic community in general, including the top research centers in Scandinavia, feel that the reflux on its own is not a threat, but he wanted to share the new data and make sure we understood there is now a question there.  He is of the opinion that time is on Cole's side, and that he's doing well now.  While nephrology feels that this is a kid whose getting too many infections, he feels that there were two, back to back; the first of which was most likely hospital introduced when he was cathed for a test, the second before he'd had time for the inflamation in his bladder to heal and he wasn't placed on a prophylactic to allow it time to do so.  He feels that if he's infection free (has been for two months now), GROWING, which is the most important thing for him, especially as he's as yet too small to accept an adult kidney in the event they do fail completely, that we let him be, revisiting our options if his condition changes. When he hits one year of age, we can assess at that point the degree of reflux and obstruction present.  He said that the reflux could be corrected surgically (where as nephrology felt you may only reduce it from a grade V to a III with his severity) and that there absolutely was a very good chance still (nephrology felt there wasn't any chance) that it naturally resolve itself dramatically by 18 months still (both the obstruction and the reflux) and that particularly in boys it can resolve itself very rapidly.  If at one year nephrology would like it corrected to make certain we have done everything we possibly can to protect his remaining renal function from anything and everything that may potentially be a threat, he would operate then with minimal complications and a much higher success rate with out being nervous.  He expressed that if Cole were to experience multiple repeat infections, he would break his own rule, but that the surgery would make him "very anxious" at this point.  Since I've had a good decade of experience with Dr. Steinhardt with Kaitlynn and know he's very confident with this surgery, I know if he's saying it makes him "very anxious" its for good reason.  We're inclined to agree with him.  Let him grow with time on his side.  And pray.  A LOT.  : )  And check out the super cute pic...they were checking each other out!

Blue Elephants..Oh My!

So we were taking a picture this morning to show Grandma how handsome we looked in our little blue elephant outfit she bought for us, and FINALLY caught Cole's super grin!  I mean, we're talking wrinkly nose excited here folks...

                                                          Thanks Grandma!!

April 26th Appnt

Cole saw the nephrologist on Thursday.  We saw Dr. Steinke herself.  She had concerns regarding the repeat infections, and had been in contact with Dr. Steinhardt, who still feels he would like to wait it out barring a substantial number of future infections before he goes ahead with surgery to correct the reflux; he wants to try and get him as close to one year of age as he can.  She felt something may need to be done sooner to protect the kidney function he does have.  We weren't really able to establish a specific parameter as far as how many infections he could have before she felt there was no option but to correct it sooner. We may be going to see another peds urologist out of U of M for a second opinion.  She did explain to us that even with the surgery, the reflux would still be present, improved at best, and that resolving on its own as some kids do is not an option for him due to the severity, though it may come down from grade V to say a grade IV.  She showed us the images from the VCUG again.  I realized then that what I'd originally thought was his ureter the last time we saw the films was in fact the part of the catheter that was curled up on his belly under his papoose; his actual ureter when she pointed it out was severely dilated- I thought it was bowel in the back round before, that's what it looks like.  It was a little shocking.  We had no idea how dilated they were.  So, our discharge sheet now boasts Congenital Hydroureter on its list of diagnosis.  We could have gone the rest of the day not knowing that.  She pointed out that unlike a very straight forward reflux, with the amount of dilation his ureters have, the urine not only travels up to the kidneys, it basically sits in a cesspool and slowly washes around.  No wonder he's soooo prone to infection.  She told us how much more complicated the surgery becomes then, as Steinhardt will have to taper the ureters also, stitching them down the sides as well as the reimplantation.  Nerve wracking decision, especially as it would be four different surgeries between correcting reflux and both obstructions, doing one side at a time.  We also learned that Cole is missing some of his ab muscles, having not been able to develop all of them due to the dilation of his kidneys and ureters.  She predicted he'd still meet his milestones with mobility, that he'd just work the muscles he does have harder.  She estimated his kidneys function at about 50%.  We also saw the last three renal ultrasounds, which don't appear to have gotten worse.  They are definitely echogenic (see all these fancy terms I've learned?), and you can see the healthy tissue all along the outside, thinned, but there, and then the entire middle of the kidney, which should have grey matter throughout, is solid black, just full of urine.  Looks hollow.  There was a resident in the room, following her, and you could see her eyes get wide before she put on her game face.  Then she stood there silent.  Happened to us a few times now.   I think that's when you realize how bad it is.  I talked to her a bit about feeling so guilty, about it being genetic.  I won't delve into that but she is such a compassionate Dr.  I asked her if she had a specific charity that contributes to research they utilize, or to kids they treat, as Mike and I would like to start a small sort of fundraiser in Cole's name to contribute to a larger one, something positive.  She suggested the NKF or Make a Wish.  Then she went on to say that all of her transplant kids will go on a Make a Wish trip, that Cole would.  I freaked a little, saying "I thought that was for terminal things", to which she said, no, "its for life threatening illnesses".  That was so hard.  Makes it very real.  Our son has a "life-threatening illness", or will have.  So, with that thought we headed back over to the lab, and awaited the results which we received by phone yesterday.  His BUN was down, 9; Creatinine .46, which is even lower than the .5 it had plateaued at, Iron stores a little low so switched to a vitamin with Iron, and increased his Bicitra to cover his weight gain.  And FINALLY, a blood type.  I held my breath and prayed- and he's B+...so am I!!  Think I cried a good half hour over that, I'm so grateful and relieved.  God is good!  Doesn't guarantee I'm a match but its such a comfort to know I'm not ruled out.  So that was all wonderful news, and despite the upset of Thursday, the most important thing is he's growing and thriving and his labs look good right now!

Thank you Tina, Rocky, and Elisha for watching the rest of the troops!  I know they had a blast!