This is a collection of thoughts from the Miranda family and our experiences with our son, Preston's, diagnosis with Congenital Nephrotic Syndrome of the Finnish Type.
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!
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".
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!
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 :)
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.
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.
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 that...it'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!!!