Making changes

It's been recently brought to my attention that this blog has been a resource for many other families with children that are born with congenital nephrotic syndrome.  I've been contacted via phone, by email, and even at my office.  I'm always happy to share our experiences with other families.  I know when our family started this journey I often felt very alone and felt like I had no where to turn.  I found a few blogs with status reports on other children, and a lot of information on google, medscape, webmd, etc that  was VERY scary.

The idea of having a child that was going to have to have a nephrectomy, be on dialysis and need a transplant and likely multiple transplants during his life time, was extremely devistating news to me.  Not to say that it's still not devastating but frankly we're used to this routine now, used to hospital life, and used to the idea that this will be a lifelong journey.  So, with that I would love to lend a hand or an ear to others, to answer a quick question of "have you experienced this?" Or anything I can possibly do to help I'm not proclaiming to be an expert or a doctor, just a mom that's learned the ropes by default.  I've had a wonderful support system (including Tyler my husband, our families, co workers that have covered for us, friends that have fundraised for us, cooked meals for us, visited us, taken us on the lake to get our rears out of the house, etc.) My point is that we couldn't do this alone, and I thank everyone for all that they have done.  I only hope that someday I can be as gracious and generous as our support system has been to us.  Don't take this journey alone. I welcome the conversation so feel free to call, email, or send a message via Facebook.  The Facebook group is here:  https://www.facebook.com/groups/106418559380738/?fref=ts

I don't blog often, and if feel guilty when I don't, but I seem to sit and stew about things to get off my chest and when talking to Preston's nephrologist and telling him that I'm contacted all the time by other parents, he asked if they contact me regarding congenital nephrotic syndrome or about peritoneal dialysis and/or transplant.  So far my conversations have mostly been CNS related and I think that's because that's the stage we have been at with Preston, sot hats mostly what my blog has been about.  So, after giving some thought, I decided to make a change to the title of this blog.  "Living with Congenital Neohrotic Syndrome" was what we we're doing.  But guess what?  Preston's had his nephrectomy and is no longer Neohrotic, so he now has a History of having CNS.  That's right, we have polished off one chapter of this novel.  So the next/current chapter: Peritoneal Dialysis, The next chapter after that: Transplant.  So knowing this blog has not only helped me vent, my family and friends know where we are and what we're doing (especially since we haven't seen so many of y'all in so long), and also helped other families undergoing the same experiences, I've decided to include in the title all three topics 1) CNS 2) PD and 3) Transplant.  

Here's to hoping that you may now be able to Google any of the chapters along the way to kidney transplant and be able to find this blog helpful in your passage. 

-Vici

4 days post op bilateral nephrectomy

Well, we're nearly a week into this current admission, and I can say that this nephrectomy experience so far has not been nearly as scary as I thought.  We arrived late Monday night and Preston went into surgery around 10:30am on Tuesday morning. The hardest part was kissing him goodbye as he went into surgery and then waiting for the long 5 hours until we were notified that he was in recovery.  He did great in surgery. The surgeon was impressed by his tissues and promptly came out to give us the details of the surgery.  He took a picture of his kidneys after they were removed and shared them with us.  One of the surgeons before surgery stated that she expected the kidneys to be smaller than a healthy child's and really light in color; however the reality was that they were actually about twice the size of a healthy kidney for a child his age.  I'm sorry if you're grossed out by this, but I'm more so documenting this for my own purposes because I intend to print this blog later for our own purposes. 

After surgery, his stats were stable, he was extubated immediately and went over to the PICU.  We were greeted by the entire team of doctors, surgeons, transplant coordinator, nurses, etc and they were all very positive and happy.  It was a nice feeling to know we had a great team who all came to see him post op to see the progress and to immediately cheer us on.

Later that evening we started our first round of Peritoneal dialysis (PD).  To explain the dialysis process, as I understand it, water with dextrose is pumped into the peritoneal cavity. Once there it sits for a period of time so that the fluid in his body can gravitate toward the sugar and fluid. Once that extra fluid is drawn to the PD fluid, it is then reversed and allowed to be flushed out of his body.  Without kidneys, Preston's body cannot rid his fluid intake out on his own.  What does that mean exactly? It means he doesn't create urine and cannot pee like a normal person.  It has been very weird this week having dry diapers.  A little sad, but also interesting to learn the science behind PD as well.  One thing I now understand is why other parents who have babies that have been recently transplanted are so dang excited to see a little (or a lot of) pee pee post transplant.  I can hardly wait for the day that Preston is able to make a super wet diaper.  I'm sure there will be a happy pee pee dance (done by me) and pictures involved.

The initial process is hardly fun. He is on the PD 24hrs a day right now.  The tube that goes into his abdomen is still healing so we can't hold him right now until it heals a little more. They are using the catheter already at a very low volume until it is able to heal more and then they can increase slowly the amount of fluid that can go into his peritoneum. He's right now doing cycles of 50ml (nearly 2oz) in, it sits for 20 minutes and then drains out for 5 minutes. So he is flushed in and out twice an hour, 48 cycles a day.  They are increasing his volume by 5ml daily and this helps to slowly build room/tolerance in his abdomen area for more fluid.  It's kind of like being sick for a long time.  You don't have a huge steak and lobster dinner right after the flu, you slowly start eating maybe mashed potatoes and jello until you feel good enough to have more....same concept.

Once he is "ramped up" to 110ml per cycle, he will be able to use a machine called a cycler, and then eventually not be on the machine for a 24hr nonstop cycle.  He will however need dialysis around 12 or more hours everyday.  It will likely be overnight because he'll need to be tethered to a machine. Suddenly Preston's albumin infusions that were 2oz over 4hrs three times a week looks like a pretty easy routine. 

I apologize if this is too technical or too much information, but I've recently been contacted by many other parents that have children with CNS that have found my blog by google-ing "Congenital Nephrotic Syndrome".  I'm thrilled that I've been able to help others with the blog so they know what they might expect, even if it is just so that they may know that they're not alone in their journey, and that there is and will be a light at the end of this very long tunnel.  I was going to say there would be a rainbow following the pee pee showers post transplant, lol.  Oh, how I keep a sense of humor through this is beyond me....

Here we go!

It's hard to believe it's been nearly 2 years since Preston was born and that we've been inside of this cyclone of emotions.  This last month has been especially challenging with Preston's recent hospitalization for an infections, having the doctors pushing us to make the next step in scheduling the nephrectomy, and still undergoing testing on candidates for living donors.

We are now at ease in knowing that we have a DONOR!!!  After several weeks of testing, labs, scans and many visits with doctors, Preston's Uncle Cody is giving him the ultimate gift.  Words are not enough to express our thankfulness for Cody, the sacrifices that he and his family will have in helping Preston though this bump in the road. We will all be forever thankful.  I've been a blubbering idiot the last few days waiting for the approval from the transplant team.  It's such a hard thing for us to even ask this of anyone, especially when we for so long knew that it would be Tyler or me that would donate our own kidneys to our son.  When we found out that neither of us were a candidate, we felt like we had let Preston down, and that we blew it for our family, although it was purely an anatomy issue that could not be corrected. 

Additionally, I would like to thank all of the generous individuals for stepping forward to be tested to be donor candidates.  The transplant coordinator was very excited that we had a receiving line of individuals that were willing to give this gift for Preston and that this is very rare.  We appreciate each and ever person that has considered this gift, has prayed for our family, that has donated time in raising funds, that has prepared us a meal, sent us a card, paid us a visit.  You're all amazing, and I'm pretty sure that we couldn't have made it this far without everyone's support.

I'm sorry if I've caused you to need a tissue.....Heaven only knows how much tissue I've used.  I should own some stock in P&G for all of the Kleenex this family has used in the last year.  This is all a hugely emotional time.  I feel thankful, scared, excited, frustrated, proud, and every other emotion all wrapped into one big ball of wax.  Another stress-er we've had this last month is with Preston's weight gain, or lack of weight gain.  He's developed a huge aversion to food, but will drink his formula still so we've been pushing to give him enough calories throughout the day, with enough fat and proteins to hopefully help him thrive.  The doctors have warned us that at some point, with his kidneys in place, he will not be able to thrive, and I think we have finally hit that wall.  He can't possibly drink enough calories in a day, and he's not been able to tolerate the high caloric formulas and protein supplements so he's been throwing up his milk.  It's a constant battle to get the calories in him and keep it all down.

So, now that we have a likely donor canidate (still waiting for final approval this week after the transplant team meets), and because we feel it's time and the doctors are pushing us really hard, we've scheduled an OR for 9/24 for Preston's double nephrectomy. At that time he will have a peritoneal dialysis catheter placed in his abdomen and he will undergo about 12-18 hours of daily dialysis.  And we though 4 hours of Albumin 3x a week was hard...  I'm not quite sure how we will manage it all, but we will have extensive training for several weeks inpatient.  At some point, we will be able to come home and perform dialysis at home.

The best part about being the time that P will be on dialysis is that we will hopefully be able to get out and about a little.  During this time he will hopefully have a little recovery in his immune system, so we hope to experience a little more pre-transplant.  I'm not sure how much we'll break out of our little bubble, and I'm sure that we will always live in fear of germs, but I hope that we can maybe have just a little bit of "normal"....whatever that may be.

Infection control

What a whirlwind this last two weeks had been. It all started on Monday 8/19 during Preston's albumin infusion.  Prior to his infusion he was his normal super happy, wild kid wandering throughout the house, playing in the bathtub and having a good time.

During his infusion he spiked a fever while sleeping and was nearly 102 degrees.  He seemed really uncomfortable and shaking and chilling and a complete 180 from his behavior in the morning.  We suspected an allergic reaction to the albumin or infection, so off to the emergency room we went in a flurry.  

On the horn to Preston's nephrologist at Lucille  Packard children's hospital, they were not surprised of the symptoms and said, we'll see you in a few hours.  At the emergency room thing escalated fast.  The fever rose to 104.8 and our amazing nurse got Tylenol in him quick, drew labs and collected a urine sample for culturing and sent them off speedy and skillfully.  Preston did experience febrile seizures but it wasn't after too long that the meds kicked in and the seizures stopped and fever was reduced to normal.  Off we went in an ambulance to the airport in Red Bluff.  I was able to fly with Preston to Palo Alto so I was thankful thathe didn't   have to fly this time by himself with the flight team.  Although it was scary, I will say it was kind of a fun experience, while I hate to admit it.

We did discover that Preston did have an infection of some kind and had symptoms of sepsis as it was in his blood.  They cultured his csf and did a lumbar puncture and thankfully that was negative, although he had already had antibiotics.  All further labs were all negative so it was evident that he was responding well to the antibiotics.  After several days the lab was still attempting to grow the bug to find out what type.  It was apparently a very difficult bug to identify because it ha to be sent to several labs, was slow growing and something that was typically only found in tropical regions and in South Asia. The team of doctors questioned if we had an iguana at home or any exotic plants, if Preston had been gardening or had any plant puncture wounds.  The answer to all of those was no so everyone was and is still scratching out heads.  The bacteria could have been transferred from his gut by means of something he ate that sat in his bowel, so of course I wonder, was it an avocado, a mango or the coconut milk that he are/drank? Or was it just a weird bug that was introduced into his IV line causing the infection? Who knows.

It only took Preston about 3 days to make a full recover, but try held onto us at the hospital for 8 days to monitor and try to identify the susceptibility of the infection and make sure they were treating him with the right antibiotics.

Preston is showing his skills with the stethoscope and his IV pump. We're practically pros!

So, I know I had many cranky Facebook posts while we were in jail, I mean at the hospital, but I am very thankful now in retrospect for the care that we received by all.  This trip also helped to prepare us for the next two steps in this journey.  We're probably going to be scheduling the nephrectomy soon, but we would like to know that we have a for sure donor candidate before we take that step.

Plan A....bust. Plan B....bust. Plan C....bust.

I've had a few folks ask this week what the plan is now. I figure instead of a few short winded vague facebook posts, it's probably time for a little blog update.  My last post, nearly a month ago, we were on track for Preston to potentially have the nephrectomy this month.  In fact it would have likely been sometime this last week.  As we're learning, things change very quickly in the blink of an eye and things have indeed.


As many of you know my sister was the first to be tested as a donor and she was unfortunately ruled out.  It was the first low blow straight to the you know where.....  Tyler wanted to be the next person to be considered so completed the last of his tests (labs, EKG, sonogram, erays, MRI) and met with several doctors and all determined he would be a good candidate for Preston's donor with the exception of the Kidney Surgeon.  After reviewing Tyler's MRI she had found that Tyler's kidneys were both more suitable for an older larger person because of the number of veins that were coming into the kidneys. Apparently smaller children have fewer vesstles and the worry is that there wouldn't be adequate blood flow to the kidney and it could cause scarring or failure.  One of Tyler's kidneys was way to close to his spinal column with a very short vein and would be too dangerous for Tyler to donate.

So Plan C.....me.  Totally up for the challenge of being a kidney donor, I immediately scheduled all of my tests and scans and went down last week to Stanford.  All was good with everything and so we were just waiting for the MRI to be reviewed by the surgeon before getting the go ahead.  I received a call from the surgeon this morning saying that my anatomy was very similar to Tyler's with the exception of me having an even shorter vein on my right side, so I would be an even lesser donor candidate than Tyler.  I now know how my sister and Tyler felt, and it sucks.  I honestly feel like I have let Preston down, and that I've let our family down. I was certain that I was going to be the "cure all"....dammit.

So at this point, we're not out of options, we have other family members that are willing to be considered and Preston can also be a recipient of a deceased donor.  In order to do so though, he will need to have already have his kidneys removed and be on dialysis and be "ready to receive". So we may need to make a hard decision here pretty soon on if we proceed with the removal without knowing if we have a viable donor, just in case one MIGHT come available.  I'm not hugely in favor of doing this unless absolutely medically necessary or emergent.  The doctor is pushing us toward this direction, simply because Preston will lose less protein while on dialysis and might possibly grow a little better.  There are of course tons of risks associated with either plan of treatment.

We're feeling a little defeated right now and appreciate all of the prayers and support.  We're beyond ready for a little good news and fewer u-turns.

Surgery date penciled in.....

If there's one thing we have learned about the medical world, it's that there is never a date that is "chiseled in stone". It seems like we have had so many plans to move forward with Preston's surgeries that have all been based on speculation on his weight gain.  Tyler and I roughly keep track of the calories per kilo that Preston eats/drink (yes we are over the top OCD crazy) so that we can guestimate how long it will take him to get to 22 lbs. (ie. transplantable weight). In looking at his growth curves last year it was looking like February/March 2013 would be about the time he would make it....but that came and went, and he's been steadily hovering at 21 lbs. for the past 3 months.  Not moving a muscle, in fact this last appointment he had lost 4oz. Feeling frustrated that Preston may just be "maxed out" in calorie intake, and the fact that his kidneys that are leaking protein like a sieve, it may not be possible for Preston to gain much more weight until he ditches these kidneys and goes on dialysis.

Preston's Doctor has recommended that we have his kidneys removed and that he will likely have an easier time growing while he is on dialysis.  There is however the fear that he will lose weight as well while he's on dialysis.  If he does lose weight, it will likely be fluid weight that he's been retaining due to his current junk kidneys spreading his fluid out to his tissue.  So, we'll have to wait and see what happens once this beans are removed.  During the time when he's on dialysis, they will monitor his labs and they will be able to monitor the point when he his no longer "nephrotic".  They tell us at this point his immune system will be relatively normal and that we can escape the safety of our clean home environment, go places, see things, meet people!!! We will have a short period of time to do so because once he's transplanted, he'll go back on the immune system suppressing medicines and will have to be closely monitored and probably again isolated.

This "penciled in date" is July 15, 2013. This hospital stint will be about 2-4 weeks, we're told and we'll be down at LPCH for that time.  At this point our contingencies for the nephrectomy is really only one thing.  We are waiting for the green light for Tyler to be approved as Preston's kidney donor.  This last week he completed a boat load of labs and scans including (and I'm sure he'll be pleased that I'm sharing this, lol) a 24hr urine collection.  Yep, that's right, he got to pee in a jug for an entire 24hrs. He also had fasting blood labs, a second set of labs and they probably took a half pint or so, ECG, MRI, he met with a nephrologist, and a nutritionist, and he still will need to meet with a surgeon, who apparently is an advocate for laproscopic removal in the donor, a kidney advocate who will determine if Tyler is safe and sane enough to be a kidney donor, and one other, but shoot I can remember what/who it is now.

Tyler will make another trip down to LPCH probably within the next week or two to meet with the last few Doctors and once we have a green light that Tyler will for sure be the kidney donor, that chalked in date for the nephrectomy, will maybe be written in erasable ink but certainly not indelible marker.

Planning for the big event.

I have failed at my plan to blog more frequently for the sake of keeping everyone informed on Preston's progress.  Things have been nice and quiet and we're doing everything that we can to keep P healthy and growing.

Last month at Preston's visit with the nephrologist, we discussed the coming events.  There wasn't anything too surprising to us, but it's just getting to be a closer reality.  Preston weighed in at 20lbs 15oz, which was actually down 1oz from his appointment the previous month.  The minimum weight he can be for transplant is 22lbs (10 kilos). So, we've been trying everything we can to plump the little guy up.  They did also warn us that he is likely retaining a good amount of fluid that he may lose after his nephrectomy that he will likely have to catch up on.

Preston still has a little aversion to solid foods.  He's okay with purees, but is VERY particular.  He of course like's ice cream and milk shakes, but we've noticed that he does have some intolerance, and we assume to the lactose.  Go figure, it bothers Tyler too.  But, he still loves his baba, so that's what we give him.  Regular formula is 20 cal/oz and Preston's is 40.  So it's literally like sludge.  We don't make it with water either....vanilla coconut milk...he loves it.  We've noticed since we've changed to the coconut milk that he's taking more, so getting a few more calories in a day.  He's growing appropriately within the 40-45 percentile on his growth charts, which is amazing considering he's leaking all protein that he consumes.  Bottom line, he's growing....just not fast enough for us.

So what's next.  Aside from the weight gain, we're still undergoing testing on the living donor to make sure the donor's kidney is a viable match.  Once we for sure know we have an approved donor, we'll likely schedule the next step....the nephrectomy.

So once he has the nephrectomy, Preston will have a dialysis catheter placed in his stomach, where he will get fluids to filter out his little body.  He will undergo the PD (peritoneal dialysis) for probably 2ish months before the transplant takes place.  Everyone asks, "will you be in the hospital that entire time?"  From what we understand, if all goes well, they will train us on how to administer the PD and we will be able to come home after 2-4 weeks providing that all goes as planned......we know that nothing goes as planned....so we don't hold our breaths.

After this step, the transplant.  That will be another 2-4 week or so hospital stint, and then they tell us they like to have their patients close to the medical facility for frequent checkups, labs and "just in case"....So we'll likely be staying at the Ronald McDonald house, or will need to find housing somewhere that is "germ free" if possible because he will need to live a little sheltered because he will be acclimating to the anti rejection drugs, which are immuno suppressants.  This won't be too strange of a scenario for us.  We're already the biggest germo-phobes on the planet right now and have completely turned our lifestyles upside down for the past 17 months.

So this is the "plan" as we know it.

Aside from that, on a fun update, Preston is doing awesome.  He's wanting to walk....heck, who needs to crawl.  He can walk while holding one finger, so it won't be long.  He does still have upper body limitations on his right side that has prevented him from crawling.  He's not hugely mobile, but he's learning.  He's getting his knees under himself and is irritated that he can't crawl.  The hardest thing for us is to let him fuss, and figure it out.  We constantly have to tell ourselves not to come to his rescue.  We're afraid of falls, since he doesn't have the quick reflexes on his right side, and I fear that he's going to have broken bones along the way.

What else....CARS!!!  Preston absolutely loves Disney Pixar's Cars, and anything to do with it.  He recognizes the characters and loves the Tractor tipping, where he blows raspberries (okay....let's get real here.....fart noises) every time he sees them. So, I'm pretty sure he's a normal boy in that department.

We're having a lot of fun and he teaching us new things every day and it's amazing to see him grow and meet milestones that we were never sure if he would ever accomplish.  Aside from the occasional emotional roller coaster, and missing being able to just run a quick errand, or go hang out with the all of our friends at the drop of a hat, this parenting stuff has been pretty darn rewarding.  I think all of those lifestyle changes are an adjustment for any parent and not just The Miranda Family.


Vici