Wednesday, December 26, 2012

Merry Christmas!!

Preston's first Christmas...I knew it was going to be pretty wonderful being a parent, but today my heart feels so warm. Although my parents are both recovering from colds and weren't able to come over today and we didn't do our normal Christmas scurry to all of the various family members houses, it felt amazingly nice to stay home with Preston and reflect on our year, and be thankful that we have him at home in our arms.

He is blossoming like crazy the last two weeks and getting interested in standing and pulling up, wanting to do the motions of walking (assisted of course), and tolerating more time on his stomach, reaching for things and attempting to crawl. We've come to terms that the three months inpatient and his medical condition(s) will likely mean he will take longer meeting his milestones, but we've watched him come along way. He is saying Dada, Da do, Baba, dog, book and mouths mom...but won't say it......darnit. He recognizes us in pictures and lights up when he sees us after arriving home from work.....and that feels so amazing.

Sometimes it takes a little reminder from someone that isn't by our sides daily. Tim, Tyler's dad was down from Idaho today and mentioned how impressed he was with the progress that P has made in three months time since his last visit. And he reminded us of what Preston's capabilities were just that short time ago and how much more he's doing now.

Also today we find ourselves reading a blog of another family who's baby was transplanted this past week down at LPCH. This baby is a twin and his sister had a transplant earlier in the year and they are going through the same experience again with their second twin baby. I'm sure it's extremely hard to be inpatient for Christmas.....well, it's tough no matter what day of the year it is, but Im sure its extremely hard for them today. The baby had some complications earlier in the week and briefly stopped producing urine, which is always a worry with a transplanted kidney, and so Tyler and I were both hoping for the best and then were relieved to find out that he was creating urine again. It's amazing how a little pee can make you excited, especially when your rooting for, and praying for a family that you've never met. I know I've said it before but its been a huge help to know we're not the only ones going through this and that it is being treated by world class doctors so close to home.

As I get ready for bed tonight, I find myself wide awake, my mind is racing. I had such a wonderful day here at home, even if we did just stay home, relax and not do much. I won't be sleeping anytime too soon. My racing mind is thinking about what's to come, what lies ahead and the planner in me is trying to think....just how are we going to do "this". I guess it's knowing that Christmas is now done, another year has almost gone, and we have some pretty HUGE things were about to encounter this coming year.....and I feel like I'm going into it with a blindfold on. Since we know that his surgeries are inevitable and his kidneys aren't going to fix themselves, I've been trying to mentally prepare. Preston has come so far this year already and I know that we will have a few bumps in the road but it (hopefully) will not be as trying as the as trying as this past year.

So everyone, hug your babies matter how big/old they are, count your blessings, live every day to the fullest, and just ENJOY the ride.......MERRY CHRISTMAS!

Friday, December 14, 2012


It's getting to be Christmas and I have a lot to reflect back on this past year.  A lot of wonderful....a lot of not so wonderful, but overall a TON to be thankful for!

Tyler last night said, "I guess I can finally settle into the idea that we CAN have Christmas this year." Here it is December 14th and we just put up the tree, although it still is undecorated, the lights went up on the house last night, and we're finally feeling like we can let a little guard down to celebrate the season.  We've been so gun shy to do the normal things in life because we're so scared that at any given moment we could end up back in the hospital for the next round of........

The original plan was that Preston would likely have his nephrectomy in November/December and then move forward with the transplant around February/March.  I think that he has shocked the doctors that he has been able to maintain as long as he has on his albumin/lasix infusions without the risky complications that go with.  We hold our breath daily and know the risks of infection, blood clots, etc.  He seems to be doing so well that it's hard to want to move forward with the kidney removal.  So because we were kind of planning to spend our Holiday's in the hospital, it almost doesn't feel really yet that it's really Christmas.

We've become fans of a few other Parents' blogs that have babies with CNS as well.  The one family with twins now have both children transplanted!  It's been a little silent celebration for us that their second baby is now with his new kidney and past one really tall hurdle.  We've never met, or communicated with this family, and they may never know how HUGE of a help they have been for us in knowing that there are success stories.  I admire their strength in having two babies to care for, both in different stages and having such different medical needs. I am very thankful to only have one baby with this condition.

So everyone asks, "How's Preston Doing?"  I feel like I have the same canned speech in telling everyone how he's doing, and I hope people don't mistake that for an unwillingness to share or that I'm being impersonal.  I basically tell them that he's growing well, eating like a champ, loves to read like crazy, loves his Dad like crazy, nearing his weight goal in needing to be 22lbs to be prepared for the surgery.  I don't go into huge detail, because I also don't want to seem like the obsessed parent who is overly involved in their own child, but I guess that's my own insecurity?

So aside from the generic canned speech......Preston really is doing awesome.  There are some milestones that he's a bit behind in, and there's no doubt that the prematurity and the excessive time in the hospital will set him a bit behind.  He still is not eager to crawl, hates being on his stomach, we think it's because of his central IV line, he has some weakness in his right hand and arm, stiffness in his left leg attributed from his brain bleed.  Once in the sitting position, he'll sit by himself totally content for a LONG time and play with his toys, reach as far as he can sometimes falling over.  We trick him into crawling for things on the couch or bed where it's more squishy and he doesn't get as frustrated.  He's pulling to stand and wants to stand, starting to motor his feet and walking with help.  We drive our PT nuts because he's skipping milestones (like the crawling and sitting up on his own).  I know it's important that he learn to pull him self up off the ground, but right now, he just has no interest.  So more PT in helping to strengthen those extremities so that he'll be able to "toddle" on his own.

It's really hard to admit (both to myself and to other) that Preston may have some physical disabilities.  He will be getting a splint for his right hand so that we can encourage him to use both hands instead of only his left hand, and it sounds as if he will have splints for at least his right leg, if not both legs, so that while he learns to walk he can learn to use both sides symmetrically.  I know many friends and parents say that they as kids or their kids work some sort of device as children, and then grew out of them later in life.  I sure hope this is the case for P, but as we have learned, only time will tell.  I know that life is so uncertain and you never know what hand you will be dealt, but I want a few more Aces, and a few less Jokers darn it.....

So in reflecting back on this year, I'm very thankful for my time at home, my wonderful family and the support of my friends, family, and co-workers.  I'm thankful for this crazy life lesson that for some reason God has given to me, because I really do believe that I am a better person, mom, wife, sister, daughter, co-worker, boss, etc. because of it. 

So, in looking ahead, my goal for the future is to blog more not only to keep you all in the loop, but to document this journey for Preston so that he can later see how he started as the tiny 4lb 12oz super sick baby and became the awesome man that I know he will someday become.

Relinquish the Control

My mind has been the biggest tornado lately. So much on my mind and so much is still uncertain of what lies ahead for Preston. Work has been crazy busy and thanks to Chelsie, our friend and new hire, we've been able to keep up with most everything and even continued to take on new properties with the management company. I constantly think to myself, am I crazy? Only a crazy person would be taking on additional, new business right now. Preston has not only been a gift, but a true lesson learned for me. And that lesson is: "Relinquish the Control." Tyler's always told me that I have an issue delegating and deep down I've known he was right, but I had no idea how much of a work hoarder I really am/(was?...) Well I have had to get over my habit of work hoarding and I can honestly say now that delegating is getting easier. I have learned to depend on people to help me with things that I never thought I would need help with....even down to the grocery shopping. For the last few years Tyler and I have been on the verge of needing assistance at work with the real estate and property management. Our situation with Preston has forced us...but in a good just let go to the things we cannot control.

We have two appointments coming up in the next few weeks that will likely be an indicator of what's next and we may even be able to tentatively plan for his surgeries, but then again I don't know that for sure and maybe I'm just hoping for some answers. I would love to just get this whole transplant thing done and over so that we can put this chapter to bed. I know that we will never be truly done with doctors and but it will be so nice to see the day when we have an annual, or even a twice annual trip to the nephrologist...

Next week we will be down at LPCH at Stanford for some clinic appointments with the nephrologist, neurologist, endocrinologist. At lease we can try to clump them together to minimize trips. We'll probably end up staying two nights because our appointments are spread out beginning at 8am and ending late in the afternoon. With a 5 hour drive on each side it's impossible to accomplish in one day.

Holy smokes a month has passed since first drafting this blog post and the above is now old info, but still not a lot is unchanged.  Rather than deleting my thoughts I'll post this blog and move forward with the next......

Friday, August 24, 2012

To Pump?.....Or not to Pump???? uuug....

Preston's 7 months old now and on Monday he was 16lbs. 13oz. and doing awesome!  Growing great and status quo at this point and we've been able to keep him healthy and at home for the time being.  He's eating like a champ and loves his butternut squash and cereal, but is not too fond of the sweet stuff.  He must be his mama's kid lovin' the savory foods already!

Now that Preston is 7 months old and working on the solids he's decided that he's done nursing.  Actually, he's been done nearly a month.  Once his teeth cut through he was immediately self weaned.  It was exciting and heartbreaking for me all at the same time.  I had worked SO HARD to be able to have that connection with Preston.

Right when Preston was born, he was not a very strong eater.  For the first 2 months in the hospital, I was not encouraged to breast feed hardly at all.  Everything was all about production in making sure he was taking in the proper amount of mL per kg, which he rarely was able to meet his goals.  Even to this day Tyler and I keep track of everything he eats, the amount of protein he gets, etc.  A little on the obesessive side, yeah probably.  But, we were also told that his trends would be a good indication of his well being and that if we start to see a drastic change that it might be an indication of a potential problem.  While still in the NICU, I was able to nurse him maybe once or twice a day, but it was never comfortable with a crappy rocking chair with wooden arms and no cushion and a curtain pulled around the isolette and the staff poking their heads in every few minutes to make sure everything was okay.  It was hardly private or an nowhere near a neutering feeling.  But for some reason I didn't give up.

Because Preston wasn't consuming enough calories the doctors decided that he would need his bottles fortified.  I had been pumping right from the very beginning, but now this meant that nearly all of his feedings would be by bottle so that he would benefit from the higher calorie content.  It would have been so easy to give up and give him formula from this point forward.  Pumping every three hours is not easy!  It's miserable waking up in the middle of the night and poor Tyler, while I'm pumping he's giving Preston a bottle, so we're both up every few hours.

After the readmission when it was found that Preston had congenital nephrotic syndrome, they then were able to identify that he was not only lacking in calories but also protein because his kidneys were spilling all of the nutrients and immunities.  Knowing this, the Docs then recommended fortifying his milk to 26 calories/oz (normal breast milk and formula is 20cal/oz) and also he was in dire need of an oral protein supplement since he was retaining almost zero protein in his blood.  So now it was an absolute necessity. Weather I liked it or not, my kid was going to have a super fortified bottle and it was going to have a bunch of powdered formula and protein powder as well.  Breast feeding where my milk was absent of the needed protein and calories was almost not an option.  For some reason or another (call me crazy?) I continued to pump and fortify my milk.

I think part of me knew that he could tolerate the fortified breast milk and with his track record in health issues, I was scared to death to rock his little world by going to a 100% formula diet, for fear of "what if".  I know ultimately he would have been fine. I know babies get immunities from breast milk and I guess that I felt that if I could provide him with anything extra that would possibly help keep him healthy, then dammit, I better at least try.

After about 4 months of pumping and only breast feeding maybe once or twice a day, the milk production was extremely lagging, to the point of not being enough.  We were forced to start using more formula.  I tried everything to get the production back up. I pumped every 1-2 hours, drank the nasty licorice flavored mother's milk tea, avoided all of the foods you're supposed to avoid when breast feeding, took fenugreek supplements, which only made me smell like a waffle house and I had the aroma of maple syrup oozing out my pores....which sometimes was appetizing, other times just annoying.

Finally at 6 months I'm kicking myself daily, asking myself, do I keep pumping?  Am I really doing that much good in providing breast milk any longer?  I know Preston is tolerating formula now so switching him would be "fine"....will he be missing out on needed immunities?  I pressed forward for another month and kept telling myself that I'll keep going till 7 months and see where things stand.

7 months this week.....All week I've been tapering down the this isn't easy.  I now know that I probably am having the hardest time weaning myself and not Preston. Preston has self weaned and has made is clear, he's done nursing.  I guess my predicament now is all in my head.  Being that congenital nephrotic syndrome is a genetic trait and that it's very likely (over a 25% likelihood) that any other children that Tyler and I might have could possibly also be affected, I know that the likelihood of us having more children of our own is pretty slim.  It's my head telling me that I may never get the opportunity to nurse another baby again.  I already felt robbed that I didn't really get the "full experience" in bonding with Preston like other moms would with their babies.  That's not to say that I haven't bonded.  Being that we've been home with him all the time, I've been so lucky to spend a ton of time and glad that our careers can (somewhat) afford us the time and flexibility to be here as we need to. So lesson learned, don't take the little things for granted.  Someone else may not be as lucky as you and so you count every single blessing.

I'm sure this post shares a side of me that many of you probably have never expected. I know that this blog is mostly to update everyone on Preston's condition, but couldn't help but think this is something about me that I'd like to share as well.  It's really hard when people ask me "how are you doing??" and my answer is always generic "doing pretty darn good....". It's not that I don't want to go into detail, but it's just tough, and I don't want to make people walk away and think to themselves, "geez, I'm sorry I asked!"  I figure with this post I can somewhat share a bit of what I have experienced more on the personal side.  I truly appreciate everyone's concern for how things are going with us and the well being of our family.  I can't wait to be able to share our experiences with Preston in person with everyone and not through a blog, but in the mean time, Thanks again for the love and support.

So.....before you all start to worry about me too much with all of this seriousness.....

Now that I will no longer be breast feeding/pumping, I have one last thing to say.    Good bye "Double D's"  it's time to make way for the "tube sock twins".


Friday, August 3, 2012

6 months old and 15lbs 14oz!

I know everyone would love an update on progress this far, and this post is really overdue. We are adjusting to our lifestyle at home with Preston and are happy to say we have a "routine". After all a routine means we've been home long enough to enjoy or little man and watch him grow like a normal baby. Preston has been successfully growing. Since he loses all of his protein, one of the primary concerns is that he potentially wouldn't thrive and gain weight at a rate that is needed. I'm glad to say that it appears that he will meet his 10 kilo goal (22lbs) in November if he continues to grow at the same rate that he has been and by his percentile growth chart. So he's doing awesome and has chunky butt dimples in his legs and he looks and acts like a happy baby! 

We have several follow up appointments with all of the specialists including Neuro surgery, neurology ophthalmology, nephrology, hematology, endocrinology, and our local pediatrician. This last month we have traveled to Stanford once and UC Davis 3 times. We've transferred services to Stanford for Nephrology and for the upcoming transplant, but we continue to see the Neuro Surgeon at UC Davis for the time being.  We will likely transfer services just before the transplant so we will be seeing all of the specialists at LPCH eventually, but for now we're comfortable with Dr. Lee, Preston's Neuro Surgeon.Being split between two different hospitals however isn't always easy.  Tyler and I find that we're doing the communicating between the doctors, constantly getting copies of labs, scans, etc and sending them back and forth.  Ultimately it will be much easier to be in one place.

Developmentally, Preston is also doing awesome.  He's vocal and cooing like crazy, sitting up but still needing some support. He's reaching for toys and can hold and play with toys.  We do notice he is a bit sluggish on his right side due to the bleed in his brain from when he was born, but he can still move it and grab things he just prefers to use his left hand.  This kid has no lack of stimulation that's for certain.  We're blessed that one of us can always be home with him, since we're kind of forced to with his immunity conditions. We play with him and keep him busy (or rather he keeps us busy) all day long.  On the day's that he has his infusions, my sister or grandma come over to help. Thank goodness for nurses in the family and it's not a one person job.  Their help also affords me some time to work a little bit during the day when they're here.

Preston is eating like a champ and the nephrologist says that we can start him on cereals now that he's 6mo and has grown so well.  He still is getting his formula super fortified with extra calories and protein since he pees it all out so we have to supplement him in hopes that he will at lease absorb a bit of it.  So now that he's starting of food, guess what??  He has new teeth to try out!  Yep he's had little teeth nubs since he was 3 months old and one finally broke through yesterday and another one today. Tyler says he'll be eating deer meat before we know it.....we'll see about that.

Aside from the new normal, we're also looking forward to what's next.  It's scarey for me to think of the next steps.  We've just gotten comfortable with what we've got going on now.  In October, we have a transplant evaluation appointment.  At that time, we will do some preliminary testing on all three of us.  Tyler and I will be tested to be potential kidney donors, and Preston will have test too to compare with our labs.  I understand from the transplant team that they will really be putting us through the ringer to make sure that we are physically, mentally, emotionally, spiritually, etc. etc. ready to be a donor.  I guess we will end up seeing about 6-10 different services throughout the evaluation.  I'm sure it will be a long and emotional trial, but of course Tyler and I are willing to do anything to give Preston the opportunity to have a "normal" life.

So then next everyone will an adult kidney fit into a baby?  I don't really know the answer other than the doctor states that they actually will NOT take a transplant kidney from anyone under the age of 18. With them removing both of Preston's kidneys and only getting one in return, they assure us there is enough room.  Apparently a kidney is about the size of a small fist, which still seems so big to me.  I guess this is why the babies must be 10 kilos to be able to be transplanted.

Tyler and I have done a lot of internet research.  It puts our minds at ease of what's to come, and scares the crap out of us all at the same time.  We've stumbled across a blog of a family that currently lives in Texas that has twins.  Both of the babies have congenital nephrotic syndrome, aside from other complications.  I can't even imagine.  They are amazing superstar parents in my eyes!  I know what Tyler and I have going on daily, but multiply that by two?!?  Superstars......

So what's next in our journey?  The next big thing will probably be later this year, but we don't know exactly when.  Nephrectomy.  Uuuugggg....  Both kidneys are coming out and P-man will have to go on dialysis.  He will hopefully only need to be on dialysis for a short time and just to rid his bodies of the nephrosis that could harm his new kidney.  They tell us that he could be in the hospital up to a month but possibly only a few weeks.  Sounds like at least 2 weeks will be in the hospital dialing in the dialysis and teaching us the new routine of how to do a daily dialysis treatment.  We will be able to come home before the next big step (transplant).  There are a lot of unknowns for us at this point and we will defiantly be learning as we go.

Again, I want to say a BIG HUGE THANK YOU to everyone that has helped us along the way.  Every bit is so appreciated. The prayers, the meals, The help with work, the help at home by family members, the cards and to all of our friends that have rallied and raised money for our expenses.  You're all amazing, and the three of us are so glad that we don't have to go this journey alone.

Sunday, June 3, 2012

An airplane ride and 5 days in the hospital for bronchitis??

So, I know it sounds silly and everyone has been asking what's up with Preston and what led to his hospitalization this last week. When I tell them he had bronchitis I see this look and a raised eyebrow nearly in disbelief. It's even hard for me to believe how something that is a "routine illness" that so many people can do easily overcome, could possibly be so devastating to cause nearly a weeks long stay in the hospital. It was a combination of the bronchitis and the congenital nephrotic syndrome that was the problem.

As I mentioned previously, CNS is when your kidneys have porous gaps that allow the spilling of all of the protein in the body. Virtually no protein remains in Preston's body and he literally pees it all out. Included in the protein are antibodies that would help to fight off sicknesses and gain immunities to bacteria. This is one reason that Preston is so at risk for infection and illness. In addition, Preston is also on a medication that suppresses his immune system further. This specific drug is given to transplant patients to help the body avoid rejection if the new organ. While Preston has not yet had a transplant, the drug is being used in combination with some other drugs int he attempt to to reduce his kidney function so that he will hopefully lose less protein. We learned this is called a "medical nephrectomy".

So because he has CNS and has a SUPER compromised immune system, he got some kind of cootie that caused the bronchitis. He was really congested over the memorial day weekend so we kept an eye on him through the weekend.

On top of the congestion, weekends are Preston's "days off" from his IV infusion of albumin (a protein blood derivative) and generally over the weekend he gets a little swollen with excess fluid that his body can't shed on his own and so it was a combination of the bronchitis and retained fluid that then led him to....respiratory distress....dammit.

Tuesday morning we could tell he was overall just generally uncomfortable and struggling to breath. So to the doc we went. Our Ped was still out for the holiday weekend but the Dr on call had some general knowledge of CNS to know that it's not something that could be treated locally in Red Bluff because things could go from bad to worse real fast. He ordered up an x ray to rule out pneumonia but still recommended a transfer to Stanford where Preston's nephrologist is located so he could be monitored.

Of course they wouldn't let me fly with him so Tyler and I drove down to Palo Alto with an empty coarsest in the back seat. It was a LONG ride down. Preston had beat us there and his flight was only about 2 hours and we were walking down the hall and could hear our baby screaming. We followed the cry to his room to find one starving kiddo. Struggling to breath and with all of his congestion, he hadn't been eating well so it was music to my ears to hear the hungry cry.

While we were there he was tested for all kinds of infections and blood work ups and tab swabs. All if which were mostly inconclusive so it was determined to likely be a virus. I never thought I'd be happy to hear it was just a virus. Dreading an infection, we were told that Preston will only have 2 or maybe 3 strikes (illness). If he has multiple strikes against him, he will need a nephrectomy and will have to go on peritoneal dialysis. With one strike already against P when he had sepsis, I was in fear that this would be the final straw, and that we would be back in the hospital another month for the kidney removal.

Luckily we escaped and the doc said that he'll let it slide because it's normal kid stuff and not a more serious infection. After three days in a row of his infusions, Preston was back to baseline (in that he wasn't super swollen with excess fluid) so he was able to breath better and the cough and crud was finally dissipating.

So after 5 days were home again. While down there we certainly got a taste of reality of what it will be like when the time comes for him to have the surgery to go on dialysis and then again for the transplant. We learned just how serious this is, not that we didn't already know. We saw many transplant patients at the hospital and they have to wear masks and respirators to prevent them from breathing in germs that would cause illness and rejection. If I didn't think I was a germ freak already, now I know I'll be an even bigger germaphobe.

I've never seen so many BEAUTIFUL children as I saw this last week. It was obvious many were undergoing cancer treatments and probably other treatments that I cannot even fathom. It was like watching a St. Judes children's hospital commercial and feeling the reality and the seriousness of having a sick child and then baby is one of those sick kids.

So while I'm happy and VERY THANKFUL to be home, I'm also very thankful to have had such an awesome facility and some great Doctors at out service. I felt like if we had to be there, it was a good practice round to get our toe in the water and feel out how life will be at that hospital for when the time comes for the next step. I'm just hoping that next step is when he's 22 lbs and over 1 year old, which is the target that he must reach to receive the transplant.

Saturday, May 26, 2012

We had a fabulous time documenting Preston's homecoming with We Shoot Ya Photography.  Thanks Danny and Dianne for everything! You do amazing work and are two of the most fun, kind and talented people I know! You're a true asset to this little town.  Thank you!

Here is the link for our pictures:

Preston Miranda Proof Set

Preston Miranda Slideshow

Miranda 2

Friday, May 25, 2012

Our new found routine

There wasn't a single day that Preston was hospitalized that we didn't spend 12 or more hours a day at his bedside. Our typical routine started at 7am we would break for lunch and then come back and stay until 10-11ish nightly. So as you can see we pretty much took up residency at the bedside. We would take shifts and one of us would spend a few hours back at the Ronald McDonald house to work and catch up on phone calls. We had a full office setup back at "camp" so that we could try to work and have some sense of normality to our lives. Thank goodness we are both in real estate and have so much flexibility in our careers. We couldn't have done it though without the HUGE support and understanding of Ken and Melina, Stephanie (I still owe you some cocktails and a lot of them!!!), Max, Jen, and Joseph. If I didn't feel it before, I certainly do now feel like you all are a true extension of my family!

Tyler and I have always been a little on the workaholic side. We knew once Preston arrived it would be different, but not to this extent! We thought that we would be able to have him at work a day or two a week while he was tiny and throw him on the Moby wrap and wear him while at the office....I guess you just can make plans too far in advanced!

Boy has life changed! We're now not only working full time in real estate and Property Management, one of us is always at home with Preston due to his immunity so we're stay at home mom/dad trading off work days, and on top of that we feel like we're full time care providers having to do more than a "normal parent" would do. I know there are two of us but feeling like we now have three full time positions is really tricky.  Thank goodness for everyone that has helped us tremendously with the cooking, cleaning, housework and for our business too.

Managing daily medicines in itself is quite a job with 16 different doses throughout the day of oral medications, shots a few days a week, IV infusions 3 days a week. The phone calls ........holy smokes...... 4 different pharmacies for medications and supplies, nurse case managers for insurance companies, the home health nursing agency, and several doctors to report to throughout the week. If I didn't feel like I was going crazy before, I sure do now!

I just thought I would kind of share a little bit of our new found home/work life so that everyone knows what our new routine entails.  I promise this blog will soon be less about us and more about Preston.  I just thought this would help to answer everyone's question of "How are you doing?" aside from my normal response of "things are going okay".

A LONG Overdue Thank You

Being in the hospital for as long as we were, we really didn't get the proper opportunity to thank the staff at UC Davis for their kindness since we were SOOOO anxious to go home. For several days before going home we knew it was likely that we may get to come home, but we didn't want to jinx ourselves by saying our goodbyes too soon....after all they scheduled our discharge date for a Friday the 13th. When we finally were discharged we were so hot to be out of there, looking back I wish that we would have taken the time to appreciate one final time all of those that helped Preston along the way. I hope to now express my thanks publicly so that they all know how much we really care.

First- our primary nurse and long time Red Bluff friend, Tracy. It was YOU that made us feel like we were not in a foreign land. Your compassion for Preston and willingness to be his primary nurse was awesome! We looked forward to the nights that we knew you would be on and knew that we could sleep easy while he was in your care! You went above and beyond in helping us schedule Preston's medications so that we weren't lost when we got home and that was such a huge, huge help since his daily schedule is almost a full time job in itself!

A few other members of the nursing staff that I'd like to thank and I'm sorry if I missed you I wish I would have written down the names if each and every one of you. I will however always remember the faces of everyone!
Marin- Thanks for always having a smile and being so kind. I always looked forward to seeing you and for doing Preston's hearing screening (a half dozen times, lol).  I'll always remember you, after all I wanted Preston's name to be Marin!

Lori- Thanks for being Preston's daytime primary nurse, and thank you so much for teaching us the ins and outs of giving the infusion.  Your teaching helped us feel more comfortable when we got home

Carrie- Thanks for your knowledge and expertise!  Many times you were the go to for the tough questions and we appreciate your support.

Jim- You were with us during some of the TOUGHEST days and you saw a side of me and my emotions that not even many of  my closest friend have ever seen.   You're an awesome nurse and glad that you were with Preston one-on-one.  It's clear you love your job and your great at it.  P.S.....I have a sister that's a nurse and she's single.....I'm not sure she'll be thrilled with me posting that, but what the hell I just thought I'd throw it out there.

Angie-  Thanks for being an excellent IV starter.  I always dreaded IV starts because I knew they would be tough, but you  nailed them every time. I also love your bedside tidyness!

Jill- so easy to remember, you share my sisters name. Always compassionate

Ebony- Thanks for landing the PICC line.  After four previous attempts by others you were finally able to nail it so that he would have less IV pokes!

Katerina- Thanks for being patient with all of our 500 questions and always finding the answers to them.

Sharon- Thanks for always bringing a smile in the room and thanks for the pictures too.  ps...I love your son's name :o)

Christian- Thanks for helping to lighten the mood.  Even if you were just breaking someone for a few minutes we knew you would always give us a chuckle about something.

Krista- Thanks for always making me feel better.  I don't think I'd be able to burp or swaddle a baby without your guidance. I loved hearing about all of your culinary delights too.

Val- You're an awesome lady!  I'm thinking an honorary Grandma :o)  I hope I see you again!!!

I know there are soooo many others on the nursing staff, and if I forgot you it's not because your work went unnoticed, it's simply due to my lack of sleep and energy during those 3 months.  I tried to write down names, but failed to catch many!  So if I didn't name you specifically, THANK YOU!!!

Ward Clerks- I'm not sure what that department would do without you!  Mike, thanks for being so kind in showing us the ropes of the NICU at 2am on the first night that we arrived.  Ramey, you run circles around that place and it's apparent that everyone knows they can count on you for EVERYTHING! Anita, you are one sweet lady! 

The Attending Doctors, Residents and Fellows, there are so many of you but thank you for being so patient with us through all of our questions and again the second time we asked those same questions. We know you aren't used to having needy parents at the bedside for 12 hours a day, and we appreciate that you have not only cared for our son in treating his symptoms, helping to determine a diagnosis as well as educating us on what Preston's needs are and will be in the near and distant future. THANK YOU!

Friday, May 18, 2012

Just pictures

I have tons of requests for pictures. Enough of the medical garbage for now, here are some of my faves. Our one and only outing so far (aside from doctors offices) was to Weshoot ya's studio to see Danny and Dianne. I hope to post a link to their photos as soon as its up :)

So, now that we know he has CNS, what the heck is it and how do we treat this?

The Internet is such an awesome wealth of information. Like TV you can't believe everything you see/read and quite frankly some of it can really scare the crap out of you. We searched high and low for info about CNS but mostly found info about pediatric nephrotic syndrome and they briefly touched on CNS but only to state that the outcome was not good....well when it was first rumored that P man had CNS and we were feverishly reading online our first thought was, what the heck does "not good" mean? Apparently treatment was not always available and without treatment the condition would be fatal. Luckily, there is a team of Doctors at Stanford that are very reputable in treating cns, and patients have traveled from all over the world to receive treatment here in California. I guess if we have to be the unlucky ones, we're at least lucky enough to live close to such a world renowned Hospital and Doctors with the expertise to help. (ps...Michelle Bunt- thanks for the referral, we feel like we finally have an experienced Doc that's on our side of the ring and not one that's referring to the "literature" and anxious to gain experience with an unusual nephrotic syndrome case).

Tyler and I have probably looked at nearly every online article we could find about congenital nephrotic syndrome. The link at the very bottom of this post is the report I found most useful and pretty much has summed up Preston's condition and symptoms for the most part. In case anyone's interested in understanding more about congenital nephrotic syndrome, check it out.

We learned that treatment in the short term would mean Preston would have IV infusions several days a week which meant that he would need to have a semi permanent IV port called a broviac implanted in his chest. A broviac is a long term IV. Since an peripheral IV (in a hand or extremity) is in a small vein they only last a few days that was no longer an option since Preston will need an infusion at least 3 days a week along with multiple lab draws. With the broviac meds can be given and blood taken through the same port.

Three days a week at home Preston gets an IV infusion of a blood product called albumin which is a type of protein that helps the fluids within he tissues of the body shift back to the kidneys so that he can excrete the fluid. His infusion lasts about 5 hours and during that time he's tethered to the IV pole an pump just like if he were in the hospital. Immediately after he gets the albumin he gets a diarrhetic to help Preston to shed the fluid. And that's when we crack open another box of pampers!

We hope his body will be able to accept this treatment until he's big enough for a kidney transplant 22 lbs and 1 year old). If for some reason this treatment shall fail then the next option would be for Preston to have a nephrectomy (kidney removal) and to go on dialysis. Which eventually he will need to do briefly as a step prior to the transplant. If all goes as we hope with the albumin infusions Preston will only have to be I dialysis for 6-12 weeks. If he fails the current regimen he may go on dialysis sooner.

What could cause failure of the current treatment?
Infection- CNS causes a suppressed immune system. Additionally on of his medications is an immunosuppressive. This is why we're stuck at home keeping him as isolated as possible. Blood clots- CNS patients are at very high risk of getting blood clots.
Failure to thrive- inadequate growth would also be considered a failure. So long as he has his current kidneys, he will lose nearly all protein in his body, which will make it even more difficult to grow at a normal rate. Preston does get the IV infusions that are a type of protein as well as a oral protein supplement that we mix into his bottles to try to supplement him with extra protein and his milk is fortified with formula to add extra calories.

So as you can see this is the reason that were home and keeping within our clean comfort zone at home and trying to keep Preston healthy and growing. Everyone keeps saying that Preston will be 22 lbs and a year before we know it. I have such mixed feelings, on one hand I can't wait till he's big enough to get the transplant so that we can get it done and over with, but on the other hand it scares the crap out of me that we're going to have another long bout of hospitals when that time comes. I guess we've just filed a temporary separation from the hospitals for now.

Tuesday, May 15, 2012

Catching up...

So I've pondered, how can I possibly share what we have been through the past 4 months? And it's going to have to be an abridged version, so while this seems like a long version I could probably write a multi chapter novel. So here we go...

Preston was born January 21,2012 on the same day that we completed our childbirth class. In fact my water broke probably a half hour after while going to have lunch with our friends that were also in the class with us, Leah and Casey. The day was the fastest day of my life. 4 hours later Preston had arrived. He was having trouble breathing so was flown to Mercy Redding he was there for nearly a week and boy, we thought that was tough! In retrospect those first 6 days were a cakewalk. On day six we got a call from the Doc and he asked us to come to the NICU ASAP. We knew something was up when he took us into his office. We've learned that when the docs take you to a private place you are in the "bad news room". He proceeded to tell us that they were concerned about preston for several reasons so they had completed several ultrasounds and a CT scan and had discovered that he had a bleed in his brain. Devastating news of course and Preston was then flown to UCDavis within hours. We drove down I-5 to Sac while our son was in the air and beat him to the hospital. I think we arrived around 1am. We were asked dozens of questions about our family history, the course of events thorough the pregnancy, birth and the events that took place during the first week of his life. He was closely monitored for weeks for the bleed in his head and several tests were done to find out why it had occurred . Apparently it's common for premature babies to have bleeding but not babies born at 35 weeks. A hematologist closely followed and studied Preston for bleeding disorders. It seemed like the took a pint of blood from him weekly for all of their tests. All tests were semi inconclusive and left the Docs scratching their heads as to why this occurred. We did learn from the neurologist and Neuro surgeons that the bleeding could possibly have some hindrance on Preston's motor skills on the right side of his body but they have reason to believe that he will be fine cognitively because of the location of the bleed. Preston did have a surgery to implant a catheter called an inter ventricular reservoir so that the fluid could be released to attempt to remove the blood fragments from his brain so that hopefully the spinal fluid could flow properly without a permanent shunt (drain). The reservoir is a little bump on the top of his head that can be poked with a syringe to remove the spinal fluid to release pressure. Most baby's have a flat soft spoof with a narrow opening. Because this bleeding occurred Preston's soft spot was wider than normal and at times slightly bulged. To this day he still doesn't have a shunt but that's not to say that at some point it will not be necessary. Finally, after about 6 weeks it seemed Preston's condition had stabilized enough for us to take him home. We were home and back a few times for planned re-admissions for monitoring of his little noggin and during each re-admission, if they found something in the ultrasounds that showed that his head was not stable they would take home into surgery that day to put in the vp shunt. It was so hard knowing that the ultrasound would determine if we would either go home the same day or else go to surgery immediately. He escaped the surgery through two of the re-admissions so we were able to take him home for two weekends and then the third time he was safe again we thought we were home for good. The plan was that we would then come back to see the neurosurgeon every two weeks for close monitoring.

Finally, home, so we thought.- we were home for 4 days when we noticed daily that Preston's abdomen was slowly growing in size. It was even a little distended before we were discharged from the hospital but it was attributed to the fact that they had fortified his diet with a higher calorie breast milk and formula diet to help him catch up in size. However in addition to his stomach puffing out he became really pale, lethargic and his eating dropped dramatically and then overnight he stopped peeing and pooping. That was the final straw. This kid always has a dirty diaper and we can expect to see a "prize" in nearly every diaper. So when there was nothing all evening and through the night we rushed him to the emergency room.

My sister just happened to be working in the ER that day and she shared with the docs that he was a hard stick for an IV and that they would never be able to get a line started. Previous attempts from when he was hospitalized he had several nurses attempt to get a line and sometimes it would take several nurses multiple hours. I always tried to give the nurses space and go in the waiting room but I also saw several nurses cry because they felt do bad for our little man after not being able to successfully start an IV after multiple pokes. We knew ultimately he would not be able to be treated locally and that he was headed back south to UCDavis. The doctor determined that Preston was stable and that it would be faster for us to drive him rather than fly him down so there we left. When we arrived they stuck us in the bunny room, which is the room that you are in just before going home. They thought they would be treating him for dehydration and maybe constipation but things escalated from there and at a scary rapid pace. Tests were done to determine that he was septic (blood infection) and that he was getting more and more puffy throughout his body. Additionally he was extremely anemic and required a blood transfusion immediately. Over the next week he was treated for the infection and his condition seemed to get worse for several days. At his worse he was so swollen all over his body that he looked like the Stay Puff Marshmallow man on Ghostbusters. The team of doctors was avidly trying to put together the pieces of the puzzle. One doctors said that it appeared that he had the symptoms of Nephrotic Syndrome. Preston went from being in the least critical section of the NICU to the most critical overnight. When we arrived and they said we were in the Lion room, my heart hurt so bad. For so many weeks I looked through the glass in the lion room as we passed by and knew that's where the sick babies were on that side of the NICU, and now there we were.

Eventually the docs narrowed down the symptoms and it was starting to for sure look like nephrotic syndrome. Now, is it congenital? Congenital nephrotic syndrome occurs from a genetic mutation and shows its ugly symptoms within the first 3 months of life. The body basically loses almost all of the protein in the urine and so the body becomes very deficient. The kidneys are responsible for so much in our bodies. So many of Preston's symptoms were now making sense. His blood pressure was always slightly high which could possibly be the cause of the bleeding in his brain, but that still has not been determined. The preterm birth is explained along with so many other items that occurred during the delivery and the first several weeks of his life.

Apparently nephrotic syndrome can be caused by many things such as an infection, which Preston coincidently had but with the symptoms not correcting it was appearing that his situation was not an incidental condition and that it was something probably more permanent. The doctors debated on doing a biopsy but between all of the doctors they couldn't come to an agreement on the appropriate method of biopsy google with a needle or an open incision). Finally, someone asked the surgeon after about a week of debate and he stated that a biopsy wouldn't be conducted until he was much larger and hopefully healthier so at this point being that he was still only about 6 lbs was to do a send away genetic blood test. We learned that the results take 6-8 weeks to come back and that there are only a number of labs in the entire country that have the ability to conduct the test. Regardless of the results his treatment was pretty much the same. After 10 weeks of waiting, the results did come back and there was in fact an issue with the gene that then points to congenital nephrotic syndrome.

After 12 weeks in the hospital and stabilizing his fluid shift, we were finally able to bring Preston home. It was exciting and terrifying all at the same time. It took at least a weeks worth of coordinating with pharmacies for the supplies for his continuing care, scheduling deliveries and getting our house ready. Finally on Friday the 13th, I ignored the fact that I'm somewhat superstitious, and I held my breath as we drive up I-5 with our kiddo in tow. Tyler joked the first time that we came home that he was going to drive with both hands on the wheel at 10 and 2 going 55mph with the emergency flashers on....after an additional 6 weeks in the hospital he said the same but suddenly it was 35 mph.

So glad to be home!

Sunday, May 13, 2012

Where to begin?

It's amazing how quickly you can fall in love. I know it sounds so cliche, but I never imagined how much one single day and event could be so life changing. Preston is 16 weeks old today, so I have a bit of catching up to do on this blog in sharing our journey so far.

I guess first off I'd like to answer, why am I doing this? Tyler and I were both born and raised in Red Bluff. While many people in town see living in a small town as somewhat of a detriment, I have always embraced it. Why would I not want to live in a area where I everyone knows everyone? It just feels like "home". Even when Tyler and I moved to Sacramento for school we knew right away knew that once we were done with college we were coming right home.

So again why?
1- To share Preston. Since Preston has been born, I feel like I have had a huge blanket of support from so many people that I know and even people that I've never met, and this goes beyond my friends and family in Red Bluff. I know we've been a part of prayer requests everywhere, received cards and calls from so many including some people across the country that are friends of family. Little Preston is not just Tyler and my son, but he has had a place in so many people's hearts that I feel that I owe this to everyone to share our experiences and progress. Now that we're finally home, we're still home bound. Preston has a compromised immune system and unfortunately has to stay safe from cooties. He is at high risk of infection and blood clots. We've experienced one infection scare already and Preston got sepsis dirigible our first short discharge from the hospital. After being home only 4 days he was re-admitted and was hospitalized for over a month. So probably for the first year of his life, he will not be able to meet many people. Although I know a blog is not personal contact, I still want people to know him so that when he is well enough to come out and play he will not be a stranger.
2- For my sanity. It feels good to vent and I probably need to do more instead of keeping things bottled in so tight.  One of my memories through this process is hugging my father in law as he was headed back to Idaho and he said to me, "Vici, you don't have to be strong every minute of every day".  I realized that, yes I had been keeping things inside and that it was okay to just let it all go.
3- To inform. Remember, we do live in Red Bluff, and while I love this small town, the rumor mill and "telephone game" is evident. Hopefully by sharing what we've been through to this point and what we will encounter in his future, it will help everyone to know where we are at and how little P is infact doing.
4- Finally, to document our story. Many people keep baby books. So far I have a baby book (that I haven't gotten to far on). I'm sad to say I have more medical charting, logs of our daily events in the NICU, lab results, calendars of appointments, etc. Well, enough of's time to be a mom. Time to keep track of the "first" as Preston grows. While I'm sure I'll always have the binder with all of his medical particulars, I also want to be working on something creative as a mom. While I've always considered myself a crafty person, the reality is that I'm a much better technical person. This is why I'm choosing blog over scrapbook, aside from being able to share this more far and wide.

I also would like to take some time to THANK EVERONE for everything star they have done for our family so far. The thoughts, prayers, cards, gifts and financial contributions are all so appreciated. I've tried to send out thank you cards through all of this but I know I've failed to thank everyone. Our experiences so far have made a deep impact on our immediate and extended family. I only hope that we can help others in times of hardship and despair the way that so many have done for us. From the bottom of our hearts, THANK YOU!!!