Tuesday, February 28, 2012

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.

Friday, February 24, 2012


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!

Sunday, February 12, 2012

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!

Monday, February 6, 2012

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.