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.