When I get around to writing about January, I will explain the story of how Max and Addie got their diagnosis but basically we stumbled upon it while they in NICU for being preemies following some bloodwork to try and understand some unrelated respiratory issues. Labwork showed that they had protein in their urine and low 'albumin' (an important protein) in their blood. Lots of heads get together and repeat these tests on Addison and diagnosed them with 'Congenital Nephrotic Syndrome' which is a very rare autosomal recessive genetic condition that Al and I were, unbeknowst to us, both carriers for. Their condition has nothing to do with them being early or them being twins. If they had made it to term, they would still have had their kidney condition. Any baby Al and I have would have a 1 in 4 chance of having this condition. The fact that both of them had it- well, that's just unlikely odds. 1 in 16 chance of it happening and it did... thus proving probability doesn't always predict reality.
So what is congenital nephrotic syndrome, other than a real mouthful? Well, to understand it, you need a basic understanding of what the kidneys do. So your kidneys filter your blood to remove wastes and excess fluid from the blood and turn those into urine. Your kidney is like a colander with very specific holes that remove the bad stuff without taking out the good stuff. Well, Max and Addison's kidneys were like a colander with huge big holes. They filtered out all their good stuff with their wastes. In particular, their kidneys filtered proteins out of their blood where they belong and put in their pee. So when the doctors first told us this, we were like oh well, they lose protein... big deal. Yeah, turns out it is a HUGE deal! Proteins are super important for growing but more importantly are two proteins that were the biggest problems for us. One is a protein called albumin which makes up blood plasma and the role of albumin is basically to be blood glue and hold your water in your blood vessels and keep it from leaking into your tissues. If you do not have enough albumin, water will seep out of your blood and into your tissues causing you to be both dehydrated and very puffy all at once! A healthy albumin level for a baby would be around 3.5-4 and when we tested Max and Addie's blood at 3 weeks old, their levels were around .5 and soon after that, we started to see their little hands and feet and faces puff up with fluid. Untreated, they would accumulate fluid in their lungs and not be able to breathe. The way around this problem is to give them albumin transfusions to replace the plasma protein their kidneys are losing. And of course we can't just give it to them once because what goes in their blood will go through their giant collander kidneys and end up getting peed out. So Max and Addison had to recieve albumin transfusions (each of which took about 2 hours) two or three times a day. Following each albumin dose, we gave them lasix, a diuretic that helped also maintain fluid balance.
Ok, confused yet? Well, their need for albumin was what kept them in the hospital. What scared us to death was the fact that their kidneys also dumped out their antibodies which are the proteins that fight infections in your body. If you were to google 'congentical nephrotic syndrome' (which I do not recommend), it would tell you about high mortality rates. And the primary reason for this is that losing antibodies leaves you very immuno-compromised. Which is something we learned early on when Addison went from bottle feeding and being alert and seemingly healthy to stopping breathing and being put on a ventilator when she was 3 weeks old. And again a month later when Max got a UTI that caused his whole body to shut down and nearly became too much for his little body. Thankfully, our amazing nephrologist Dr Swinford (their kidney doctor) and a wonderful doctor named Dr Pacheco who was an immunologist together created this plan to give Max and Addison infusions of antibodies. I have no doubt that God used this (called IVIG) to save our babies lives. After both having serious life threatening infections in their first two months of life, they both remained infection free for the next four months while recieving this treatment. The power of prayer and amazing medicine.
We reached a breaking point in April when we realized this is how it would be. Max and Addison would require a central line or central port and would need at least two IV transfusions a day. And while all this was working, at any point they could get an infection and not be able to fight it, stop growing or get a blood clot. So we were trapped where we were and at constant risk of things getting worse. And ultimately it just was no way for Max and Addison to live their lives. We needed to get them home. But the only way to stop the protein filtering would be to remove their kidneys. Which is all well and good except well, kidneys are kinda important! No kidneys means wastes build up in your body and more troubling (as we've come to learn the hard way), fluid builds up in your body.
And just quickly because it is everyone's first thought- what about transplants? Well, transplants are definitely our plan for Max and Addison but for right now they are too small still. I will write a whole post just about transplants soon but for now, the babies will need to be around 25 lbs to get a kidney. When we needed a new plan in April, they were only 10lbs. Now they are 20 so we're definitely getting there... but in the meantime, there is dialysis. The thing which I have a total love hate relationship with. I hate it because it's difficult and confusing and so lifestyle restricting. BUT, right now dialysis is saving my babies lives every day and enabling them to grow and stay healthy and get ready to get a kidney so how can I feel anything but gratitude and love for dialysis really?
So that brings me to the title of my post- what is peritoneal dialysis? Well, dialysis is a process of filtering the blood of waste and water. The most common way of doing this is called hemodialysis which actually removes blood and runs it through a machine several times and replaces it all without the excess fluid and waste. Generally this can be done 3 times a week. However, it is just too hard on little babies who have smaller blood volumes. It can require them to recieve a lot of blood transfusions which complicate transplant down the road and involves a lot of fluid swings that are hard on their bodies. So hemodialysis is not the best choice. So instead we do something called peritoneal dialysis. It actually involves putting sugar water in the babies' abdomen (inside the abdominal wall, a membrane called the peritoneum), leaving it there for an hour and relying on osmosis and diffusion to naturally pull waste and fluid out of the blood and into their abdomen. After an hour, the sugar water is now more watery and contains waste so we then drain that fluid out. And we put more in and start the whole process over.
Thankfully a machine can do these steps on its own- we set up the sugar water we want for them and attach them to the machine by their catheder (a tube that was surgically placed in their abdomen in April) and then the machine pushes fluid in, waits, and then pulls it out. This process can be done manually in the hospital but at home their machine does it. And in theory, the machine should be able to do this 'overnight' and then we can take them off the machine during the day, go about our business and then start over. Well, we're not quite there yet as currently 'overnight' is actually 18 hours so we're on it a lot still. But we do have 6 glorious hours off!
I guess what we find most challenging is trying to figure out how much fluid should we pull. See how sugary the water you put in determines how much water you pull off. And while your kidneys are super smart and sensitive and can deal well with you drinking more or less... with dialysis we just pick an amount and hope it's right. And now that they are home, Al and I are the ones deciding all that every day`(with plenty of advice and help from our wonderful dialysis nurse). And we've certainly gotten it wrong many times so far and sometimes it's not a big deal and sometimes it is. So at the moment, we spend a lot of time setting up dialysis and being on dialysis but I don't mind that. What is hard is the responsibility, the decision making, and being the ones in charge. That part is scary. But we're doing it and God is helping us through each day and we might even start to get the hang of it one of these days!
Ok, so that's my very long and at the same time skimming the surface overview of Max and Addie's renal issues. I'll add more as we go and there's more medical stuff and more implications of dialysis for another post. But for now, at least I feel like I've covered the basics of what exactly was wrong with their kidneys and what in the world dialysis is! And just to add a visual of what their machines look like, here's a picture.