Sunday, June 29, 2014

6 months and Celebrating!

Last week we visited Luciele Packard Children's Hospital for Prestons 6 month post transplant biopsy.  The idea of having a biopsy was really worrisome for me thinking we're going to disturb a perfectly good functioning kidney.  If it ain't broke, don't touch it.  The team advised us that it is routine to complete a biopsy at 6 months, 12 months and 24 months post transplant.  After that it will be as needed if something (scary) comes up.

I've known that a biopsy is a tissue sample, but what could they be looking for?  The short answer....rejection.  The biopsy is done under general anesthesia, and ultrasound.  A portion of the outer edge of the kidney is taken to identify if the kidney is building any antibodies in fighting the kidney.

Preston's labs have been amazingly stable (knocking on some serious wood right now), so we were wondering why would we even want to disturb that perfect functioning kidney.  The team said that the biopsy could show  advanced signs of rejection, even when it appears that the labs are within normal limits. This week we received news that it doesn't appear that there is any indication of early rejection or growth of antibodies against his new kidney!
We're finally able to start making plans without constant fear that we will have a medical emergency that will prevent us from participating.  We've been able to spend more time with friends, Preston has had a few play dates, he went down his first water slide yesterday, went swimming for the first time, and has gone to a few restaurants, and most importantly he will finally be able to meet so many friends and family members and everyone that has been encouraging us the last few years.  We are having the time of our lives being able to do new things and take Preston to experience life.  I always knew that being a parent was going to be rewarding; however I think the last 2 years have only made my appreciation for the "good times" that much sweeter!

Sunday, February 2, 2014

One month post Transplant

It's so amazing that it has already been over a month since transplant.  We spent 11 days inpatient during the transplant stay.  5 days in the PICU, and 6 days on "the floor" monitoring meds, electrolyte changes and immuno suppression levels.  Preston's primary immuno suppression drug is called prograf.  It is given twice a day (8am/8pm) in conjunction with another med called Cellcept.  At the time of discharge from the transplant, his medication schedule was super intimidating.  We're used to having a spread sheed of medications so it wasn't a huge problem, but everything was so different from what we had been taking pre transplant.  It was just one more new thing to get used to.

 Follow up appointments immediate after discharge were twice a week with lab draws twice a week (or more) depending on his Prograf level and if the dose had to be tweaked.  The first 6 months post transplant are the most critical and the suppression level is kept quite high during this period while Preston adjusts to his new kidney. Apparently it's common to be outpatient here about 90 days post transplant if all goes as planned.  Other than transplant clinic and labs we're going to a serious amount of other clinic appointments including Occupational Therapy, Physical Therapy, Urology, Neurology, Nuro Surgery (follow up for Preston's brain hemorrhage at birth), and Endocrinology for hypothyroidism caused by the congenital nephrotic syndrome.  It's literally a full time job going to and fro all of these appointments.

We're currently staying at the Ronald McDonald House.  We have a small room (like a double bed hotel room) and there his a big mess hall style kitchen and dining area with a common area.  We're keeping Preston pretty sequestered because he is so immune suppressed so he stays in the room unless we go from the hall to the elevator to the garage out to clinic appointments.  We've ventured down to the kitchen and outside a couple times but the Nephrology Team has informed us that there is a raging virus going around and not to expose him or ourselves by co-mingling with the other occupants at the house.  We've considered finding a small house for rent or an extended stay hotel room where we can spread out a little more, but it's hard to do so not knowing how long we will possibly be here.  Our insurance doesn't cover travel or hotel stays, so we're trying to be conservative at the same time as we are looking out for Preston's needs.
(This is Preston keeping track of all of the deliveries at the house.  He loves to look out the window at all of the trucks and cars, and the crazies that are at the Stanford mall right across the street.)

We're back inpatient now for a urinary tract infection.  It's interesting to me that a UTI for a "healthy" adult is a simple clinic appointment where they throw a random anti-biotic at you and then you go on your way.  For Preston he experienced a fever over 101.4 at the time we brought him to the ER.  It rose up to 102.8 and was up and down all day.  We were in the ER from 11am to 9pm until we finally were admitted to a room upstairs. I've never quite seen an ER like there is here at Stanford/LPCH.  It was CRAZY.  Metal Detector and airport style screening at the front door, triage right there in the waiting room, and luckily since Preston is a transplant patient with such a low immune system, we were in the door and in a room in about 10 minutes time.

Why are we still inpatient instead of getting antibiotics and being sent out the door?  They have to determine what kind of bug Preston has. (Pseudonymous) and now have to determine the "sensitivity" of what antibiotics will effectively treat and kill the bug.  He is currently on 2 different broad types of antibiotics and once they find out the sensitivity, they will be able to switch him to an oral version to treat him with a 10 day dose.  They want to keep him here for 48 hours after changing to the oral dose to make sure he doesn't spike a fever again and to be certain that the antibiotic is doing it's job.  So here we sit, knowing we have at least 2 more days here if the sensitivities come in today.  However being that it's (Superbowl) Sunday....the likelihood of anything happening today is slim.  Weekends at the hospital are like molasses.  You pretty much can count on waiting for anything to happen until Monday.

Aside from this little hiccup, Preston is doing AMAZING and we're proud of how many milestones he has accomplished in the short time post Nephrectomy.  In that time he's learned to crawl, walk, run, army crawl, bear crawl, belly flop (even though it scares me to death!), slide down of the bed and so much more. He's gained about 6 lbs and several inches as well. It feels good to finally feel like he is "healthy" and catching up.

So Long Dialysis!

The last two months have absolutely FLOWN by extremely quick.  In the midst of it and our nightly 12 hour dialysis cycles, Tyler and I constantly reminded ourselves that people do this dialysis thing for years, and that we should feel very blessed to only have/get to experience this for such a short period of time.
Good Bye Baxter Machine!!! This was the last time we had to see "End of Therapy"!

I think because it's been only 2 months we almost feel like we've just learned the routine and have settled in and now we are again preparing for another new adventure.  This next adventure (although not our last adventure, I fear, in this scary kidney journey) I feel is going to be the most exciting venture that will help Preston thrive, grow, and develop into the person that he will be.

There are many things we will not miss about dialysis. Like sleeping on a twin bed in P's room on the floor, trading off nights monitoring Preston's machine and tubing, getting only a few hours of sleep every other night (we trade off), not sleeping together in our own room since September, Preston's nightly vomiting an middle of the night linen changes, and I could continue.  The important thing is that we are almost done with this chapter  for the time being, and we appreciate the sweet things in life even that much.  Ill never underestimate the luxury of sleeping in the same bed more than one night in a row or sleeping a full 8 hours a night.

On the other end of our complaints, we have much to be thankful for this year, and for the past 2 years thus far.  We're especially thankful for our family and specifically Preston's donor, Uncle Cody. Not only has Cody willingly volunteered to be Preston's living donor, without any hesitation, his family has made extreme sacrifices on our behalf and on Preston's behalf.  We are and will be forever thankful for Cody, Megan, Louden, and Cael for allowing us to interrupt your Christmas celebration this year, and by sharing more than a kidney.  You guys are all amazing and we love you.

I have no doubt in my mind that this will bring our families closer not only this year but from this point forward during this time of the year, not only will we have Christmas to celebrate, but we will have a day of thankfulness and celebration every year on December 20th, and then Tyler's birthday on the 21st  (might as well make a week long event) and I hope we can all celebrate all of the festivities as a family.

May you all have a Merry Christmas and I ask that you say a little prayer for the entire Miranda family. Friday is the big day and we've been preparing for this for about 19 months.  It seems like its taken forever to get here but it's crazy that the day is almost here already.

Sunday, October 27, 2013

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:

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. 


Saturday, September 28, 2013

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....

Saturday, September 14, 2013

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.