Tuesday, January 17, 2012

One Year Ago Today- the day our world turned upside down

One year ago today we found out that Max and Addison had Congenital Nephrotic Syndrome. I had no blog at that point and wasn't even ready to put anything about it on Facebook for over a month. My mom emailed people and asked them for prayer but pretty much I didn't talk about it with anyone other than Hannah and a few texts here or there to other friends. Eventually we started to be able to talk about it and not feel sick. I still sweat if I try to talk to my students about it because well, 14 year olds do have a way of not being the most emotionally sensitive people. But I can generally talk about it now without much emotion or stress. And since I didn't get to share what all we went through a year ago with many people, I thought I'd write about it now a year later. Better late than never...

For the first three weeks of their lives, we had no idea Max and Addison had a kidney condition. We did get a pretty early indicator that Max had more issues than Addison as he struggled to get off of CPAP, his breathing assistance, and he also had really tight muscles and would scream if you tried to hold him or move him. I remember in mid January they warned me that max may not make it home by his due date and that seemed devastating. I cried the whole way home from the hospital that day. I cannot imagine if someone had told me he would be 6 months AFTER his due date- goodness! But in the early days, with crazy post partum hormones, not being able to hold Max and thinking he would be home so much later than Addie was extremely difficult. Addie meanwhile was what they called a 'feeder and a grower' meaning that she just needed to master breast/bottle feeding and she'd go home. We had no indications at all that she had any issues. She seemed on course to be home by the end of January.

If anyone has ever had a baby in the NICU for any length of time, it is tough. You are hormonal and missing out on the parts of new parenthood you had always dreamed of. You are trying to figure out pumping and healing yourself (esp if you had a C section like I did) and so you feel awful. And then things are going on with these tiny babies that you don't understand. It is really scary. And emotional. You would think that those first few weeks before I knew Max and Addie were sick would be happier memories but I probably cried more those weeks than I have the rest of this year. I always felt such compassion for the moms of new preemies who were in Max and Addie's pod last year. Even when I knew it was just a little baby who would beat Max and Addie home and didn't have the issues we did, my heart still hurt for the new mama who would always look like such a wreck coming to see the little baby. The early days are just terrible no matter what lies ahead. But as I started healing and could move around better and I was getting to actually breastfeed Addie and not just pump and my hormones were starting to settle down, things started to get better. There was probably a week or two in January after the initial difficulty and before we knew that I started to get the hang of things and feel more positive and happy.

Because of Max's respiratory and muscle issues they started doing more tests the second week in January to try and figure out what was going on with him. I knew one of the tests they were doing was a kidney ultrasound but I didn't really know why. On Sunday January 17, the head neonatalogist called our house early in the morning and asked if my husband and I would both be available to speak with him that morning at the hospital. He told us both babies were stable but we needed to talk more about the results from Max's testing. We were incredibly nervous and I still remember every detail of the awful scene now. I was breastfeeding Addie at the time and we kept waiting on the dr and I didn't want Addie to wait so I was trying to be all easygoing and it's fine, I'll feed her and if he comes in, no big deal. So of course as soon as Addie starts eating, he totally comes in and the result is that I'm trapped in one of the worst conversations of my life wearing my red robe and attempting to nurse a preemie who is still getting the hang of it! I quickly realized that was not going to work and had to stop feeding Addie. The conversation was upsetting because we knew he was saying something was seriously wrong. It was also upsetting because we found out Addie had the same condition and that blew us away since she seemed so healthy. He told us they had 'congenital nephrotic syndrome' and were losing protein in their urine and how the lab results showed them this and that is was a genetic condition etc.

I remember crying and being upset. But we also really didn't understand that day. We certainly had absolutely no idea just how serious it has been. After all, losing protein in your urine really doesn't sound that bad. I do think he mentioned the word 'dialysis' as like a worst case scenario or something. But for that day it was upsetting just to know they were sick and that Addie probably wasn't going to be going home in the next week now as we thought. It was probably enough to know just that. I remember meeting my parents at Chuy's that day and us all being stunned but trying to be like 'it's probably not a big deal... i mean, how bad can it be to have a bit of protein in your urine'? (for the record, very very very bad it turns out)

Our stage of not really understanding what the diagnosis entailed was short lived. The next day after lunch Addie seemed unusually fussy. The nurse kept saying it could be gas but Addie was very upset and she was not a fussy baby- she still isn't a fussy baby, if she is crying a lot you can pretty much bet she is crazy seriously sick. Anyway, we didn't really know what to do and she seemed to have settled down some so we left for dinner that evening. We got a call as soon as we got home that Addie had gotten really sick and now had a fever and they were starting antibiotics. By the time we got back the hospital 30 minutes later, they had put her on a ventilator because she had gotten so sick she stopped breathing. We showed up and all the doctors were busy and none of the nurses felt they should be the ones to explain what was going on so we just sat there by Addie who was intubated in her diaper and lying on a warming tray and I just sobbed and sobbed and sobbed. I have learned to control my emotions and deal with some pretty awful stuff in the past year and I can get through tough days with a lot more grace now but I was new to this then and I was just hysterical at the sight of Addie looking so sick when she had just seemed so well.

The doctors finally arrived and explained that Addie had a Strep B infection and that because of her kidney condition, her body lost all of its antibodies (which are proteins) so she had a very weakened immune system and therefore got very sick very quickly. They told us they had decided we needed to go that night to the Children's hospital in the city as they felt we really needed a nephrologist (kidney dr) and an immunologist and more specialists helping Max and Addie than the Woodlands had to offer. I think the fact that they wanted them transferred that night was scary for us- we knew it was serious if it couldn't even wait a day. We were pretty overwhelmed and stunned. We suddenly felt how serious the situation was and it was just an awful night. Max and Addie were transported by ambulance around 4am to our city hospital. Al and I drove behind the ambulance in his car and it was a quiet and scary hour trip out to the hospital. We are so grateful for that move. Despite the distance from our house, it has been a great place for them to be and they got the help they needed here.

So we ambulanced there at 4am on Tuesday and then that day met the nephrologist on service (only 1 kidney attendings out of 4 will be covering the hospital on a given week. The others cover outpatient clinics or research). Dr W was on service and he had been communicating with the Woodlands hospital and had really established the diagnosis. He wanted to meet with us that day. So after having been awake for way too many hours and after a very traumatic series of events, we sat in a little meeting room with a whole bunch of people- some of whom I don't remember but I know included Kitty their nurse practioner (who we are still in touch with and came to Max and Addie's birthday party!) and Dr Chapman a neonatalogist who followed them the next few months. The meeting was horrible. How on earth I made it through without a huge breakdown I'll never know. Dr W is a wonderful doctor who sadly is no longer at our hospital but he is wise, trustworthy, honest and genuinely caring. He also has a way of telling you everything and telling you all the potential problems and kinda painting a fairly bleak view of things. He got the tough job of being the one to have to explain things to us initially and there was probably no good way of doing that. But we left the meeting feeling very stressed about nephrotic sydrome and clinging to a futile hope that maybe they were wrong and they didn't have that (we got genetic testing done in the next week that confirmed it).

On that first meeting in January, Dr W told us Max and Addison would have to have their kidneys removed and go on dialysis if they wanted to survive the condition. He told us that dialysis is hard on babies and the smaller they are when they go on it, the worst the odds of it being successful are. And he told us that although transplants will be a part of the plan for them, they are not a cure as they will only last 10 years before they need another transplant. He told us all about the risks of central lines and about the possibility that they would just stay sick with one infection after the other from not having any antibodies. In the end, most of what he told us, as much as we didn't want to hear it, was kinda spot on. We ended up coming up with a solution about the antibodies that made a huge difference and kept them infection free and the other doctors have indicated we may have more like 15-20 years from a parent donor kidney but... on the whole, he was painting a pretty true picture. It just wasn't one we were ready to see yet.

You always wonder what it must feel like to just have the floor fall out from under you. to be faced with that kind of news. I think you just automatically go into some degree of denial. We certainly did. We dealt with what we could and assumed he was crazy and all that bad stuff wouldn't happen to us. We didn't google it. (it took me months to be ready for that)- we didn't ask a lot of questions. We just survived. I mean, that's still how we handle things. Right now we're hoping for the best for Max and his neurological condition and trying not to be consumed by it. You can't sit and wallow or you simply can't make it through things like this. So you do a lot of choosing not to dwell on things and choosing to focus on what is good. And in January, Addie got better pretty quickly and was off the vent and back to eating and doing pretty well by the end of the week. So we were happy about that. We were adjusting to a new hospital, adjusting to a lot of new information and just trying to figure out what the next step was and not think too much about how much our lives had just changed.

It wasn't until the very end of January that we saw the first lingering 'symptom' of their nephrotic syndrome (Addie's rapid infection was clearly related of course). The very last day in January, Max and Addie started swelling. One of the proteins they lost in their pee, albumin, helps hold fluid in your blood and keeps it from seeping into your tissues. Because they were losing this protein, we saw Addie's little feet and legs get puffy and sadly for little Max, his whole body swelled up. January 31st was Al's birthday and his parents were arriving the next day and

I'll never forget Al crying and saying 'he looks like the michelin man' over and over. We started needing to given them twice daily IV albumin treatments which meant they needed central lines and from that point on, we were just chasing their body trying to replace the proteins we could as fast as they could pee out the replacement.

So that was last Janaury. I think it was probably the worst month because it wasn't mixed with as much good and joy as other months. As the babies have gotten healthier and bigger, the hard days are sprinkled among wonderful happy days soaking in their cuteness. We were also too overwhelmed to share what was going on as much so I think it was a lonelier time. We still often take a few days with new news to process together and share only with family or very closest of friends. But I'm learning that letting people know what is going on through Facebook and now this blog has been not only therapeutic for me but incredibly encouraging. I really appreciate the people who have taken the time to let me know they are reading. We feel very surrounded by love and support and it is such a blessing.

And for some photos from January...

Nana holding both babies on our last full day at the Woodlands hospital- one day after we got their diagnosis and only hours before Addie got really sick...

Max at one month old- he is actually on CPAP and we managed to sneak a photo of him while his mask was being cleaned!

Addie at one month old! Her skin looks so sad from IVs being put in :(

First ever family photo (at the Woodlands Hospital still)

Already a mama's girl.

Their first photo together and still one of my favorites.

Our family on their one month birthday

He was so tiny! It's hard to believe because he's such a big boy now!


Lindsay said...

Oh Steph! I just love reading your blog and seeing all the sweet pictures. I cried as I read through the journey you have been on. I am so amazed at your strength and faith. You have an absolutely precious family and as devastated and sorry as I am that you have to go through all of this, I am also so happy for the sweet and special memories you are making along the journey. You are loved and prayed for often!

Vera Erdosi said...

Dear Stephanie,
I hope and your lovely kids are very well!
We've just learned that our nephew has CNS too.
I would ask a lot of things, so would be nice to email you, if you don't mind.
If so then please send me your mail address. Mine is: vera.erdosi@me.com
Thanks in advance!
Warm regards,