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.