When it rains, It POURS!

We knew these couple of weeks wouldn't be easy. We knew Max would be having surgery for a couple of weeks before and we had prepared for that to be a big deal and we knew Addie would have surgery not too long after so we anticipated a climatic end to February. We did not, however, anticipate quite the level of drama that we have encountered. My Facebook updates were getting quite lengthy so I thought I'd do better now just updating properly on my blog...

Max had no issues with his surgery at all and we had been really prepared for him to. We had the whole Max is sensitive and anything can send him into trouble talk with the doctors so we very nervously sent him off to surgery 8 days ago. And we held our breath and prayed very hard and he did really well. And we kept holding our breath and praying fervently as he started Hemo 2 days later and again he did really well so by the weekend we started to relax. Max was off his pain meds and feeling better and had not had any problems with Hemo yet. The relaxing from knowing Max was doing well was dampered by the dread of taking Addie in to surgery on Monday. I felt really sad about it all Sunday because she was SO happy this weekend and it's just no fun to take a child who is feeling great in to inevitably feel worse even when you know it's the best for the long run. I wasn't hugely nervous so much as just dreading the whole post op pain part.

So Monday came and Addie had Hemo in the morning and then surgery. She came out of the OR pretty quickly and we got to go back to recovery to see her. If you get admitted straight to an ICU (NICU or PICU) you don't go to recovery but since Addie was planned to go to a normal pediatric room she went to recovery. We've only been to recovery a couple of times but it is NOT a fun place. Lots of kids coming out of surgeries- tears, nervous parents, no fun. Anyway when we got there Addie was uncomfortable and fussing and her blood pressures were high. Long story short 4 hours later and many many pain meds and blood pressure meds later Addie was smiling and playing and yet still extremely hypertensive. It's just easier to just talk about the systolic number, the first number. Addie's should be between about 95-115 and generally runs about 105-125 so a little high but ok. The doctors wouldn't consider it grounds for needing to come in until it was consistently above 135. A BP above 150 is way too high and above 170 is acutely dangerous because little hearts cannot sustain such high pressures. I remember when I was pregnant with twins, I had to get blood pressures checks daily because my pressures were creeping up into the 140s. So the fact that Addie's BP ran in the 170-180s for 16 hours straight is very scary. They tried every machine and every cuff hoping like crazy it was some sort of error.

We tried to avoid a drip for a long time because she didn't have IV access because it is really hard to get an IV on Addie. We tried every single blood pressure medicine, either orally or through her Hemo catheter and nothing seemed to work. Finally at 2am it was clear she had to get a drip so we then spent 2 hours getting an IV in which was a very traumatic experience for both Addie and I! They ended up having to get a scalp IV in and had to shave part of her hair. And it has taken Addie a really long time to grow her sweet little blonde fuzz so I was very sad about that. But finally after about 7 sticks and over a couple of hours, Addie started on her drip. I was so stressed by this point because nothing had worked yet and I knew we needed to get her BP down soon or her heart was going to run into trouble. I prayed very hard at 4am and asked all of you to pray and thankfully the drip worked! She required very high doses but her pressures finally came down!!

Now her pressures are being managed by the drip so she is not in any danger from such high BPs but we can't seem to get her off the drip. The intention of a drip (which administers medicine constantly around the clock) is to stabilize her and then hopefully wean off and onto oral meds. We hoped maybe having Hemo would help but it has not really yet. So please keep praying for Addie. She cannot leave the ICU until she is stable off her drip for a while. We still don't fully understand why it happened other than it seems to be a rare but possible effect from anaethesia. Why is hasn't worn off yet we're not sure. Addie often seems to run into these confusing medical dilemmas where I really feel like I need House to come and figure it all out...

But the culprit seemingly was anaethesia. Which means you can only imagine our excitement at finding out today that Max would be going back under anaesthesia tomorrow! I mean, really?! Have we not had enough anaethesia fun for one week!!! So Max is not getting surgery, he is going under general to have another MRI done. It's like my twins are competing to see which one can get more medical attention! Ultimately it is just the reality of having twins with chronic health conditions- definitely not for the faint of heart!

So Max's MRI... Max had an MRI about 6 or 7 weeks ago so you may be wondering why we're doing another one. Well, as I've mentioned before Max's MRI showed significant brain 'damage' or abnormality. However, we haven't been able to get a diagnosis as to why. There were a lot of possibilities posed and at our meeting a few weeks ago, the doctors wanted to talk to us about their concern that Max's condition may be 'neurodegenerative' meaning he may actually keep getting worse neurologically, which could actually make it life threatening. It could also not get worse but be stagnant and he will never develop past where he is now. It could not be either of those two options and Max could get better but the doctors were definitely concerned that he was showing signs of a progressive condition. We have sent bloodwork away to a genetics lab but do not have results yet so in the meantime we're trying to look for objective ways of seeing whether Max is getting better, worse or the same.

It is a really hard issue for me as his mom because I think it's really important that I believe in Max and look for his acheivements and celebrating his improvements. So no doubt Al and I and our parents look at Max through a lens of wanting to see improvement. Max's doctors have a different, arguably more objective lens, but they also don't see him very often so the fact that they saw him on a good day in December and then a bad day in January seemed to really concern them that he was declining. And while we could just agree to disagree, Al and I feel it is important that we do try and understand if Max does have a progressive condition. We don't want to live with that fear in the back of our minds if there really is no evidence for it. And if it is the case, I suppose we should 'prepare ourselves' for that, although I'm not sure there's really such a thing. Also, we can't move forward with transplant until we do know whether his condition is progressive or not. So we do want to know. We had Max's PT from the NICU evaluate him today and she did not think he was worse. She also did not think he was better. His OT from home does think he is better, if you look at small specific accomplishments.

So in the end, since the MRI started all this, the doctors think the best thing would be to get another MRI 6 weeks later and look directly at the brain and not at our interpretations of his behaviour. We should be able to basically line up the images and at least see if there has been any significant changes. Please join us in praying that there will be no evidence of deterioration in Max's brain. He has been really really happy and cheerful and social and moving the past few days after his surgery so we are hopeful that some of the 'getting worse' that people were worried about was a reaction to pain and discomfort he had been in from his hernia and reflux. Since having his surgery, we have felt like we got our old Mr Happy Max back and that has thrilled us. We expect we will see abnormalities on his MRI still and we know from his pronounced developmental delays that there are some problems there. But we are praying expectantly that there is no evidence of brain deterioration. And we are continuing to pray for wisdom for doctors and answers from blood work so that we can arrive at some sort of diagnosis and some idea of what the future may have in store for Max. This set of issues we're facing with Max has been much harder on us than the kidney stuff so we'd also love prayer for Al and I and our families as we deal with another MRI results session and more talking with neurologists...

Of course after Addie's drama, we'd also love prayer that Max handles the anaethesia well!

So that's our thunderstorm of drama at the moment. We are all tired and have been worried and very much ready to get these babies back home and get things back to normal soon. We appreciate the prayers and support and visitors while we've been surviving these current trials. We continue to see God answer prayers in His time and according to His will. Healing may not always be as fast or complete but God is there working miracles in his sweet babies Max and Addison and we are so grateful that His hand is upon them.

Hemodialysis

So week one of having two babies on Hemo is nearly done and I realize that most of you probably have no idea what that actually means! So I thought I'd try to explain it, at least briefly so you know what our life involves at the moment!

As you know, your kidneys both remove water and remove wastes from your blood. Without kidneys, toxins build up in your body and water builds up. When I was talking to my students about this, one girl asked if I thought my daughter felt bloated most of the time which was a pretty good question! So yeah without kidneys you just live constantly bloated! The beauty (and at the same time frustration) of peritoneal dialysis (their old home machines) was that they are on it most of the time- about 17 hours a day (pre hernia anyway- it was 21 hours after!). And so it ends up being closer to having a kidney because there isn't as much of a chance for water or waste to build up. Peritoneal dialysis (or PD, as we call it) slowly and gradually pulls using water in the abdomen and osmosis slowly pulling things out so it is a pretty gentle treatment.

Hemodialysis is a totally different approach. In hemodialysis, you build up water and toxins in between treatments- so if you go back to back, you have like 22 hours of build up and then if you have a day off, like Addie did yesterday, you have more like 46 hours off and then if you end up with two days off like we trailed with Addie last weekend, you actually have 60 hours of toxins and fluid build up. Then you go onto a machine. And what is so 'risky' regarding Hemodialysis is that you actually connect directly to your bloodstream and pull out a decent percent of your blood and cleanse it and then you just keep circulating your blood through this machine. So at any time during the approximately three hour treatment, you have a significant amount of your blood in a machine rather than in your body.

By directly filtering the blood, Hemo is fast! In three hours, you leave free of all those wastes and fluid build up and clean enough that you can go many hours before needing it again. So you are looking at like 15 hours total of dialysis a week instead of 120 hours a week on PD. So the sheer Math of it really favors Hemo! The problem is probably self evident from my description- blood is really better off staying IN your body! Particularly on a baby who just doesn't have that much blood. Having blood outside the body introduces risks of blood pressure swings (Addie's favorite Hemo trick- her blood pressure can vary from like 60/30 to 170/110 even with a single treatment!), blood loss (one time the machine backed up and about a 100mL (appr 3oz) of Addie's blood poured onto the floor), blood infection, seizures etcetera. So the risks are pretty enormous! It's also a very intense three hours because while they had a 6ft leash to their PD machines, you have about one foot to the machine for Hemo and that one foot is the catheter full of your blood circulating so not moving is pretty serious business. Because of Max's motor problems, he will lie still so that is less of a risk for him. For Addie, she absolutely will not lie down so she has to sit still and play with her toys without crawling or pulling up on the bed FOR THREE HOURS! And every fifteen minutes, they must get a blood pressure which sends Addie into a tizzy of baby rage. Needless to say, it is a very long three hours!!!

Despite her drama queen antics (which were, btw, particularly out of control on Monday as if she sensed Max was in the OR soaking up all our attention!) and the problems we've had with blood pressure and the one incident of blood loss and the one blood transfusion, Hemo has actually been pretty successful for Addie. With Max, we were more concerned because it's harder to predict whether he will struggle with seizures when his brain is compromised. Additionally, Max has his turning blue trick still and we are now starting to piece together that his neurological condition probably contributes to that as well. The doctors biggest concern is that he will clamp down and shut off his airway during Hemo. He does that with some regularity and is usually able to come back on his own and only sporadically (every couple of weeks) requires 'bagging' (basically CPR with medical equipment) to get him back to breathing. But the fear is that if he were to do it while having a significant blood volume outside of his body and in a machine that is not intended to deal with significant heart rate drops, it could make recovery much more difficult. We were told to be prepared for some serious drama to go down if that were happened and talked through all kinds of interventions from chest compressions to emergency intubation etcetera.

Thankfully two Hemo sessions in and this has not occurred. We are really relieved to see that his brain has coped really well with Hemo- his blood pressures have been nice and consistent and free of the swings Addie shows. He has shown no seizure like activity and seemed alert and stable during the treatments. So we feel better that Hemo doesn't seem too stressful for him at base case. The airway issue is more difficult because he could go 10 treatments without an episode and then clamp down on treatment 11 and drama unfold. So we will have to pray our way through each and every treatment and never become overconfident or stop being vigilant about that. And in 6 weeks when he is healed, we will very happily return to peritoneal dialysis for Max!

Also and it's early to tell but so far- we are loving life with the g tube and fundo. Max's horrible reflux cough has been gone and he seems so much happier. Of course he is getting some mighty nice meds so of course that's not necessarily all related to the reduced reflux but we are optimistic that Max will be happier and more comfortable and back to enjoying life in no time.

I mentioned on Facebook that now Addie will have surgery on Monday. She really needs a g tube as well- the direct feeding tube to her stomach. Partly because she still won't eat normally, although we're hopeful that will change in the months following transplant. But more significantly we want it for immune suppressant drugs. Addie is a spitter and even if we get her back to eating all her food on her own- our realistic goal- we don't want to risk her spitting out those life saving important medications or refusing to eat them. So now we will be able to give her those direct to her stomach. Our transplant surgeon here said 'you want my opinion? Leave it in until she gets married and I'm only half joking'! Older kids and teenagers are notoriously bad about taking their medications and can lose their transplants over it so there is an argument for continuing to give them via her g tube long term. We will just have to wait and see on that but for now anyway we will be using that. She is also having her old PD catheter that she is no longer using removed. Which is actually a pretty scary but exciting step because it means we are officially saying goodbye to PD for Addie. With our transplant planned for May, it seems best to leave her on Hemo until then. So Monday's surgery is really an important and therefore exciting step towards transplant. We are still dreading it because surgery sucks and it's hard with babies who don't understand why they left feeling great and came back feeling awful. But once we get through the other side of next week, we'll have made some great progress for both babies and be feeling very happy about that!

I don't know how long we'll be here. Addie should get released Tuesday or Wednesday. Not sure when they'll release Max. The ICU will surely grow weary of a healthy baby lying in there just in case he turns blue so I imagine if he's still doing well mid next week when Addie goes home, they might start talking about letting him come home to. We can't wait to be back at home all together!

Thanks so much for all the prayers and support. We have felt very loved and cared for this week and really appreciate all of you!

And pictures of each baby getting Hemo...

Addie waving at everyone who walks by during Hemo- the reddish brown lines from her bed to her machine are her blood going into the machine.

This is how Addie passes her time- playing with all her toys!

Addie really enjoys eating her blood pressure cuff- medical supplies make the best chew toys!


See, here is where my blood is!!! (and no she is not allowed to play with her blood lines! I just couldn't resist getting a picture of it telling her no, haha!)

Max's chillaxed approach to Hemo

Hemo with Max- notice the absence of crazy drama we'd feared!


You can see his blood lines coming from his chest in this picture...

Here's a close up of our handome little man- he is still a little loopy from the drugs but feeling pretty good!

Decisions and Direction

A week ago my head was spinning with the number of decisions we needed to make for the babies. Now a week later, we have made most of our big decisions and we now have a plan and direction and I am very grateful for that. Admittedly my head still spins a bit because all of our decisions are just the start of the next big deal so we have lots on our plate! But we know we're headed and that helps me.

I'll try to balance giving enough detail for those who are interested with not boring you with excessive medical info! So first up, sweet Max. Following a very difficult meeting with his neurologist and nephrologist (brain dr and kidney dr), we decided we will be going forward with surgery to repair his hernia and perform a procedure to reduce his acid reflux. (for those interested, he will be getting a g tube and a fundoplication that should eliminate acid reflux and greatly reduce his vomiting). Although surgery is always risky itself, the bigger risk factor involved in this decision is the approximately 6 weeks of hemodialysis Max will require following the surgery. Addie has done really well on hemo which has helped us feel better about this. However, Max is 'neurologically compromised' which basically means that his brain just doesn't work quite like it should and we don't really understand exactly how it does work so it's a lot harder to predict how he will do on Hemo.

But this surgery will greatly improve Max's quality of life and therefore our decision was actually pretty easy. Max deserves to be as comfortable and happy as he can help him to be. So Max will be having surgery Monday February 20 at 8am and he will be in the PICU until his doctors feel comfortable sending him home to come back and forth for Hemo. We expect a 2-3 week hospital stay but know it could also be longer. We would love for everyone to surround Max in prayer starting next Monday and for the days that follow. If you could pray specifically for Max's breathing that he will not have one of his blue spells while on Hemo (this seems to the biggest fear his doctors have) and that he will not experience any seizures while on Hemo. Although we are nervous and aware of the risks, we have a peace that we are making the right decision and believe that Max will be fine on Hemo.

Meanwhile we have also had some great progress for Addie. Our insurance approved Addie's out of state transplant and suggested that if we go to Stanford they will pay for our airfare and contribute to accommodation as well (since it is in network for them). Seeing as Stanford was our surgeon and nephrologist number one choice and arguably the absolutely best place to transplant a baby, we decided that was an easy decision to make! I very nervously left this rambling message for the transplant coordinator. I spoke super fast and gave all these disjointed details and then gave her my phone number and then was like 'oh yeah, my name is Stephanie and I'm from Texas. Ok, bye." I was pretty sure she was going to think I was crazy and maybe never call me. So when she called the next morning and her first line was 'your message is now my favorite voicemail message of all time', I knew it was a match made in heaven! We still have some details to finalize but as of right now, Addie and I will be heading to San Fran at the end of April for an early May transplant. We are very excited that God has led us to this hospital and we are very excited for this big step for Addie. She will also be having a surgery in March in preparation for transplant to put in a g tube for administering meds and to remove her peritoneal dialysis catheter. We plan to keep her on Hemo until surgery.

I mentioned that we had a difficult meeting with Max's doctors to discuss his neurological problems. We still do not have a diagnosis as we are waiting from results from a genetics lab. Please join us in praying that we do get a diagnosis. It will really help us know what to expect for the future and help us better understand Max's current issues. The doctors have prepared us for some pretty troubling 'what ifs' but we are trying not to worry about possible diagnoses at this point as clearly we have enough things to worry about without dipping into the what if pool. We would ask you to please pray fervently that Max does not have a progressive condition. While initially we were told that is unlikely, it seems to now be something the doctors are considering. This has obviously been a very difficult month for us as we've been facing these concerns for Max and we would appreciate continued prayer for hope, peace and strength for Al and I, as well as our families, as we face this new set of medical issues.

Sweet Babies- you'll have to excuse Addie's annoyed face. It is how she generally feels about sharing any attention or spotlight with her brother! He meanwhile is smiling as he always does when he is near Addie!

Big decisions and big blessings

It's been a while since I've posted as life has been busy and filled with its usual level of confusion and chaos! We've been trying to figure out what lies ahead for both babies and don't really have a lot of answers yet so I've been waiting to write until I have a worthy update! But then I figure people are interested and praying so I'd write just to say we're doing well, dealing with some big decisions and just generally enjoying life with the babies.

I realized today that in January Max and Addison both slept at home every night of the month- no hospitalizations! And that the only other month that has been true in their life was September. So I decided that was certainly something to celebrate. We had a crisis free month and feel blessed that on top of staying stable with all their big medical issues, Max and Addie are just staying cold and flu free this winter and feeling good. I said something to our Dr S one day a few weeks ago that it'd been a while since we had gotten good news and she told me that every day that Addie does well on Hemo and there are no complications is good news. And this is certainly true. Addie had a great month on hemo, needed no blood transfusions and has been the happiest she has ever been.

Another huge praise I forgot to write about was that we had Addie's hearing retested and she has been re-diagnosed with only mild hearing loss. It's interesting to think about why things happen and I remember the utter devastation I felt the day of their surgery when they told me they had likely profound hearing loss. I wonder sometimes why I had to go through that when it turns out they do not have profound hearing loss. Of course I don't have all the answers but I do know that it floods my heart with joy every time Addie turns to her name, every time she startles when Lucy barks and every time she imitates her Dad's laugh. I smile probably 20 times a day just at the blessing of their hearing. It has taught me to appreciate something that perhaps otherwise I would have taken for granted. It has also taught me that the future is not certain and that I should hold on to hope even in the face of difficult news. And that is a lesson that is particularly important for me as we stand at the beginning of our journey to understanding Max's neurological problems and look at the road ahead. We value medical opinions and outlooks but we also know they are not set in stone.

We have ordered hearing aids for Addie which should be in in a couple of weeks. Mild hearing loss will delay her speech development (perhaps the reason why she refuses to say anything other than 'Dada' despite our many attempts to teach her mama, nana and papa?!) so they should help her. The audiologist said the prognosis for Addie's speech is very good and that she should develop completely normal speech with a hearing aid and predicts no need for cochlear implants. We hope the same is true for Max. We have not been able to repeat his hearing test yet and his neurological problems make it more difficult to predict how his speech will develop. But the encouraging news regarding Addie's hearing gives us lots of hope for him as well. Addie also got her cute glasses last week. I was worried they would make me sad but I was all smiles seeing them on her. She was precious in them. Certain things feel like reminders of all their medical problems- like feeding tubes. Feeding tubes do make me sad. So I worried her glasses would be like that. But they don't make her look sick, they just make her look adorable. And grown up. She's looking more like a toddler and less like a baby every day.

In terms of dialysis and transplant and all things kidney, we are in a stage where a lot of difficult decisions need to be made and we don't have all the information we'd like to be making all these decisions. We're trying to move forward with an out of state transplant for Addie and hopeful for a summer transplant in Atlanta for her but it is currently out of our hands and in the paperwork and insurance stage so we're just praying every day that it will work out. In the meantime, we need to be deciding if we want to try to put Addie back on her home machine or keep her on Hemo. Hemo is awesome for her quality of life. She has gone from 17 hours of dialysis 7 days a week to 3 hours of dialysis 5 days a week- she's loving the freedom and her development is progressing as a result. But hemo means BIG risks and knowing that a blood infection or serious blood loss or a seizure or any number of unpleasant things could suddenly and swiftly happen and put her at great danger. Her machine may not even work and she could end up with fluid in chest again. But if it did, she'd be safer on it. So it's a difficult decision and one we are praying very carefully about. Because we so want a transplant for her and are so frustrated that hasn't been able to happen at our hospital, we are struggling to be happy with any other plan. For right now, she will stay on Hemo for a while longer but we keep praying for wisdom and guidance in making that decision.

The situation with Max is equally complex. We have been very happy with him on his home machine but he has now developed a hernia which is basically a tear that causes fluid to push into places it is not meant to (in Max's case, his scrotum). It is painful, especially on higher fluid volumes. To ease his discomfort and risk for damage, we've had to lower his volumes and increase his time on the machine so he is currently on his machine 21 hours a day which is just so sad. He also has been struggling a lot with his reflux and vomiting and he have a had a couple of scary choking on vomit issues the past couple of months. We have been looking at surgery to repair the hernia and to have a procedure called a fundo that would eliminate vomiting and reflux for him. But, it would involve putting Max on Hemo for about 6 weeks and because of Max's airway and neurological issues, Hemo would be even riskier for him than it is for Addie. We'll be meeting with Max's doctors soon and doing lots of thinking and praying about the best plan for him. We realize that as complicated as things have been with trying to arrange a transplant and debating hemo and PD for Addie, they will only be exponentially more complicated for Max because of his other health concerns. So for now, we're just looking one step ahead and trying to decide what to do about his hernia and reflux.

I find it difficult that there are so few clear answers at the stage we are at right now. And I'm grateful that our doctors value our opinions and inputs. But it also feels like a lot of pressure. I am very worried about making the wrong choice or making a choice and feeling very guilty if something ends up going wrong. I suppose that really that is one of the fundamental difficulties of parenting- how do you know what is best for your child and how do you live with the decisions you make. The stakes are high for us when we are faced with life or death consequences but the fundamental issue is common to all parents. And so we do what anyone else does... we pray, we talk, we make little pro/con lists, we google things, we ask for advice... and finally we make a decision and trust God with the outcome.

So anyway, that is our life at the moment. It's stressful but it's good. The babies are doing well and we're enjoying them very much. I took Addie to church yesterday for the first time and she was so delightful! Perhaps a little too delightful as she talked, laughed and blew raspberries through the very serious prayer time! We thank God for their smiles and their happiness and for their precious lives.

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!

Big decisions, confusing results and a wonderful party- our week in review

We had a wonderful day yesterday at Max and Addison's first birthday party! It was at my parents' house and we had 52 adults and 17 children come and it was a whirlwind of socializing and catching up with people for a couple of hours. We had a lot of people from our church who had or had not met Max and Addie but pray fervently come and get to see them. I had friends I work with now and also friends from when I lived here before Australia come. And we had nine special friends from our hospital come- 8 who took care of Max and Addie in the NICU and their wonderful kidney Dr S came as well. Max got to relive his NICU glory and spent the whole time being passed between his favorite nurses cuddling and smiling at them! Addie's highlight was when I finally let her play on the floor and people gathered around to watch her and she just would laugh out loud! She loved it! My only regret is that I didn't take pictures! I just had my hands full and totally forgot but that's ok- sometimes it's more important to live in the moment than to try and capture the moment so you can enjoy it later!

Yesterday was a great blessing to us particularly because it followed a very difficult week. We enjoyed spending a day reflecting on what all we've come through after many days of looking ahead of what all we have to get through. Starting on Monday, we realized it was going to be a big week as we are trying to work out what the best thing for Addie is with her still being on Hemo and transplant at our hospital not looking like it's going to happen very soon. All off my re-testing came back great and I have my official approval from the medical board to be Addie's donor. But, once the idea of transplant went from an idea to a reality, a lot of concerns arose. Addie is significantly smaller than the smallest baby they have ever done and it was the opinion of her doctors that the risks associated with that are probably greater than the risks with staying on dialysis and waiting (and we are looking at waiting a significant amount of time, perhaps a year). We were of course very disappointed and spent a lot of time this week both recovering from that disappointment and frustration and trying to wrap our heads around 'what now?'

I don't have the answer yet to what now. We are just trying to ask all the right questions. We had a really good meeting on Friday with Dr S and she is so supportive or our family and really trying to help us work out what is best for Addie. We are actually looking into an out of state transplant and going to potentially Boston or California to go through Harvard or Stanford medical schools where the surgeons have operated on a lot of little babies. This would allow Addie to transplant now which we think would be advantageous for her but without the risk of a team who is nervous and relatively inexperienced (at our hospital, the surgeon himself is experienced and confident but the teams working with him less so). Another alternative is that our doctor Dr S goes to a mayo clinic in Minnesota and follow a baby through a transplant and be further trained through that and come back and arrange Addie's transplant then. Of course, there's no real timeline for when that would happen so whether by the time it did, Addie would be a lot bigger anyway, we don't know. Possibly Dr S would come with us to Boston or California and actually be there through Addie's transplant which we would love. So right now the next step is looking at insurance and feasibility of the out of state option. We really don't even know if we CAN do it or if we think it's the best thing for our family overall. Addie and I (and of course then my mom as well) would be out of state for at least 2 months and so it's a lot to think about...

So I'll keep you informed as we navigate our way through this. In the meantime, for now Addie is staying on hemo. We may end up trying her back on her machine if it does look like we really won't transplant for a year. Please pray for wisdom for Dr S and for us. Unfortuntely there simply is no 'right' answer and no way to unequivocally know what would be the best. All the options have pros and cons and risks and benefits. So there's not an easy answer for the doctors or for us as parents. Only God knows what is truly going to be the right thing for Addie so we just pray as we sort through our options, that he will make it more and more clear to all of us what the best plan for the next year is for Addie.

Interestingly, Max is actually significantly bigger than Addie at the moment and closer to being the size they'd actually want a baby for transplant. However, Addie will still go first because Max's airway issues pose additional risks for surgery meaning that we will most likely be waiting to transplant him until his airway has hardened, which will be presumably another 6-12 months. We've tossed around the idea of trying to do both together if we do go out of state but right now every time my mind goes there, it kinda panics and feels overwhelmed. Al is getting some more of his donor testing done this week so we're continuing to move forward and leave that option open. But Max is doing well on dialysis and the idea of introducing a big risky surgery to a baby who is so stable just doesn't seem like the right coarse right now...

So that was part of our week. One other small thing which I'll write more on (and include a cute picture of) soon is that Addie is going to be getting glasses to correct her cross eyes which are actually a symptom of far sightedness. I won't lie and say it didn't break my heart some for my sweet little girl to now need hearing aids and glasses and I certainly didn't feel very happy about it Monday when we'd already been dealing with all the transplant issues. But, she will of course be adorable in her baby glasses. And it will fix her cross eyes and it won't be permanent (although she'll be wearing them for years and not merely months). So in light of everything else going on this week, it really did end up in perspective. So stay tuned for Addie in some adorable pink glasses...

Then there is our final and arguably biggest issue that we are facing and that is relating to Max and his MRI. We found out on Monday that there were abnormalities in his brain. (when it rains, it pours around here and we found all these things out on Monday!) We didn't really know much about what was there as they said we needed to talk to the neurologist. So on Friday we went straight from our long meeting with Dr S re out of state transplants to the neurologist. And even though we spent 90 minutes with the man and looked at like 50 MRI pictures, I still struggle to explain what we know. Partly because it's all very raw and emotional and partly just because it's all very confusing.

What we don't know is 1) a diagnosis... no idea why there are abnormalities on Max's brain or 2) a prognosis... we don't know what these will be mean for Max when he is older. So without a diagnosis or prognosis, we arguably don't know much still. We are getting blood work done on Wedneday to look at several possible diagnoses so we may eventually get a diagnosis (or we very well may not) and a diagnosis would help with a prognosis (although with most neurological disorders including the ones we are testing for, there is such a range in their functional outcome and such a range in what the lives of those that have them look like... that even then, I don't think a doctor will ever be able to tell us really what the future looks like for Max).

But what we do know is- there are several abnormal things on Max's brain that are contributing to his lack of motor skills and his general 'not seeming to be at the same place as his sister'. I wrote down all these random observations on the MRI and have tried googling them and just end up in all these medical journals with lots of big words I don't understand. The best overall summary I can give is just that Max's brain is underdeveloped and smaller than it should be. There's not really a specific place of brain damage as you would expect to see if he'd had a stroke or an episode of oxygen deprivation. The doctor said it appeared more 'chronic' than that. In particular, it looks like his frontal lobes are significantly smaller than they should be and there is a place where the lobes of his brain should meet and be closed and it's open. There's also some issues related to white matter and myelination. From looking back at previous MRIs, this seems to have been the case since he was born and probably was the case in utero. It is more pronounced and easy to recognize on his most recent MRI which was more thorough and done under anaethesia. The doctor was very clinical and just pointed everything out to us very bluntly. I have this fear about ever discussing doctors on my blog with it being public and I may form a great working relationship with this neurologist and he might follow my blog for years to come! So I'll just say that the information was given to us in a very detached manner and I'm not sure if that made it easier or harder.

He did say that Max doesn't seem to have a progressive problem. So Max's brain should not get 'worse'. Instead, he should continue to grow and develop. And physical therapy will help him. The neurologist described it as the 'tail wagging the dog' meaning that if the part of his brain commanding movement is not working properly at the moment, we can just train his body to do the movements through repetition and in the process, may actually strengthen that part of his brain as well. This doesn't work as well with adults but babies brains still have a lot of 'plasticity' or moldability. So I guess no matter what diagnosis or prognosis we may or may not ever get, our aswer is really the same and that is that there are reasons for Max's delays but we will keep believing in the very best outcome for him and fighting to do all we can to help him develop every skill he can.

If we had two kids a couple years apart, we would expect them to have different needs and be at different places in their development. And with twins, you expect them to have different personalities and different interests and to develop different strengths and in their own way. So, really it's no different than that- just on a bigger scale I suppose. Max and Addie are the same age but they are very different. And they have very different needs from us. And that's ok. Of course medically at least they'll always be compared to each other. I mean, every question at the neurologist regarding Max was followed by 'and what about his sister?' For all the Science geeks out there, Max has an inbuilt 'control group' in his twin sister who is 'neurotypical' (just a fancy way of saying neurologically normal). So we won't be able to avoid comparing them at times. But I think the most important thing Al and I can do is recognize them as two amazing individuals and embrace their own unique accomplishments. The other day, we saw that Max was batting at his inflatable penguin to make is bounce and we were so excited and praising him and Addie seemed to sense she was missing out on this moment so she crawled over, lifted the penguin over her heard, shook it violently and then crawled off with the penguin in tow. Did it make Max's batting at the penguin any less exciting? No, because purposeful and controlled arm movement is a great thing for Max and very hopeful progress. So we were stoked. Did it make Addie's ability to shake and crawl off with penguin any less adorable? No, (except for attention hogging and stealing of her brother's toys which we do need to work on!) because that's what she's doing right now and we're thrilled for her. They are both amazing and complicated and they both bring us great stress and great joy! Our journeys with them are sometimes similar and sometimes different but that is just parenthood. Children are never going to behave exactly as you expected and you learn that parenting real babies so much more difficult (and rewarding) than when you used to play house with dolls. Our issues are huge and the things we face are difficult and scary but ultimately we are just like any other parent loving and trying to figure out the kiddos we have!

So that's where we are now. And we didn't really share most of this with people this week because it was really important to me that Saturday at their birthday party we celebrate the miraculous year they have had and rejoice in all their accomplishments and how far they've come! It wasn't the time to focus on what all still lies ahead of us. And we really did celebrate and it was a wonderful day. And now this week and in the weeks ahead, we remember that joy and use our encouragement of how much they've come through to get us through the long days of facing our newest trails and our current dilemmas. I think the second year of their life may prove to be as eventful as their first but I know if we made it through last year as well as we did and were there looking healthy and happy with real genuine smiles yesterday, I can only assume we'll be celebrating just as hard and happily on the 2nd birthday. And maybe one day things will settle down and there won't be any more new diagnoses or huge medical decisions and while we'll welcome that day, for now... we'll just keep riding this rollercoaster and praying our way through the lows and praising our way through the highs!

Addie update and Max's big tests

If you've been reading for a while, you have probably worked out that there are a lot of highs and lows in our journey and sometimes really good news that we celebrate ends up getting watered down in the weeks that follow. It makes me hesitant to share it when we do get good news but I usually do anyway because it is so exciting and such a blessing at the time! Anyway, our journey to transplant has been one of those times. On December 21, the day before the babies birthday, they started rushing all of my testing with this bold plan that transplant January 3rd. The surgeons had it on their schedule and they were like telling me what time to arrive on the 2nd! I didn't share that date with anyone aside from my best friends and family becasue I didn't really believe it. Dr S was out of town and I was pretty sure she thought it was too soon. And yet, it did stir up hope and excitement in me as I rushed through my testing and found I was all ready!

Last Wednesday, they officially cancelled the 3rd which again, came as no surprise to me but some disappointment. However, it was cancelled because Addie's doctors thought it was too soon following her previous surgeries and hospitalization and I totally respected that. Unfortunately, it seems to have now just gotten indefinitely delayed. They have decided to do some more testing on me to look at a couple of the tests that were ambiguous in their results which will take a while to get done and they want these results back and more clear before I donate. And there's now a conflict with anaesthesia and what 'protocol' the surgeon wants to use versus what they want to use on Addie with her being little. The long and short of it is that somehow we went from transplant Jan 3 to transplant not being on anyone's calendar.

We are discouraged as we are ready to move forward. We are also trusting both Dr S and God that Addie must just not be ready. We do not want to risk Addie losing my kidney early or any dangers to her so I know in our heads it is better to wait and do it right than rush to do it sooner. I say in my head because my heart still needs some working on. I have been very attached to the idea of transplant for Addie and I'm having to really work on letting that go for a while and being ok to be where we are. After all, she will transplant. There is no doubt about that. And although I know when I want it to happen, I certainly don't know when it is best to happen. So please keep praying for wisdom for the doctors and that it will happen at God's chosen perfect time for Addie. She is in the meantime doing ok on hemo. It has had some ups and downs as we were warned and there are days it's hard on her and days it's a lot better than being attached to a machine 17 hours like her brother. She's happy though and home and doing really well crawling now all over the floor and we are just going to have to focus on how happy and good she is now and stop looking with such anticipation to the next step.

So that's what is happening for Miss Addie. Meanwhile, Max is having a big week. Max has been delightfully easy for his Daddy and I during Addie's drama and been full of smiles and cuddles and is growing leaps and bounds. He's doing really well on his home machine. Yesterday he saw his pulmonogist who looks at his airway and she's going to be making some changes to his meds to reduce reflux and vomiting but generally seemed ok with how he's doing with his breathing. The big deal for Max comes on Friday. He needs to have two brain tests- an EEG which measures brain wave activity and an MRI which looks more at brain structure. Unfortunately it is crucial for the MRI that he not move so they are going to put him under general anaethesia for it. Although we've done the anaethesia thing enough now that it's lost a lot of its scariness, it's still a big deal every time and Max hasn't been under since June so it's a big deal to have to go through that again. So please keep sweet Max in your prayers on Friday for a smooth anaethesia experience.

And then please be praying for the results. He has had two EEGs and MRIs in February and in early March when he was about 2 months old due to some muscle stiffness and breathing abnormalities. The later set were said to be normal and neurology stopped following him. He certainly didn't have any brain bleeds or significant problems on them. Dr S suggested we take him back to neurology a few months ago as his motor concerns have become more obvious.

Because the babies lived in the hospital and have serious medical issues we expect them to have some developmental delays. And while Max is improving and getting stronger, he is not able to hold his head up or grab onto objects and at one year old, this is troubling and makes it more likely that there is some sort of other medical concern there and that is is not just a 'hospital delay'. His primary issue seems to be with his neck and his arms. He moves his legs enthusiastically and his core is actually strong enough that if he could hold his head straight, then he'd be able to sit up. But his head is still floppy and he his ability to use his arms to grab for things is limited.

We love Max so much and think he's the best baby and he has the most perfect disposition but his lack of motor skills are sad for Max as it means he isn't able to play with toys the way his sister is. Watching the difference between Addie and Max is difficult for me as I rejoice as she develops new skills and is growing into this little toddler and at same time am heavy hearted for Max and these milestones that he is not reaching. The widening gap between them also seems to suggest there is more to Max's delays than hospitilization or dialysis. And so we need to start looking again at his brain and seeing if we can't find an explanation for what is going on. When he met with the neurologist last month, the neurologist reviewed his previous MRI and felt that while there were no big obvious problems, there were many subtle abnormalities on the MRI. And so he wants another one now that he's bigger and he wants a completely sedated one so movement can't invalidate any results.

I'm praying that the MRI clearly shows us what is going on in Max's brain. If there are no neurological problems then I will rejoice in a completely normal MRI and we will keep working with physical therapy and get his muscles stronger with use. However, if there is a neurological basis to Max's motor problems, I do pray we get some answers. We do know that when it comes to the brain, answers are never concrete. A doctor will not be able to tell us when Max will be able to hold his head up or when he'll be able to walk or if we'll see any of these problems manifest themselves into adulthood. He may be able to give us some predictions and some ideas but with a lot of neurological problems, the progneses are widely varying and the ability to know what lies ahead is impossible. But a diagnosis and some direction is nonetheless, an important step forward. It will also be important for us that we accept what we find out and find peace, hope and understanding in what we learn. We really appreciate your prayers.