God's Plan Being Revealed

Today, we went to the endocrinologist. This was on the recommendation of the nephrologist. Well, we got a diagnosis for Princess: pseudohypoaldosteronism type 2 (PHA2).

Here's what the National Institute of Health's Genetics Home Reference says about it:

Pseudohypoaldosteronism type 2 (PHA2) is a condition characterized by problems regulating the amount of sodium and potassium in the body. Sodium and potassium are important in the control of blood pressure, and their regulation occurs primarily in the kidneys.

People with PHA2 have high blood pressure (hypertension) and high levels of potassium in their blood (hyperkalemia) despite having normal kidney function (normal glomerular filtration rate). The age of onset of the condition is variable and difficult to pinpoint; some affected individuals are diagnosed in infancy or childhood, and others are diagnosed in adulthood. Hyperkalemia usually occurs first, and hypertension develops later in life. Affected individuals may also have high levels of chloride (hyperchloremia) or acid (metabolic acidosis) in their blood. People with hyperkalemia, hyperchloremia, and metabolic acidosis can have nonspecific symptoms like nausea, vomiting, extreme tiredness (fatigue), and muscle weakness. (website)

So this explains everything. We knew the hypertension was causing the damage to the heart. The treatment for the PHA2 is one of the medications she is already on. She's on such a minuscule dose though, so I suspect we will be going to a therapeutic dose of it probably on Tuesday when we see Dr. K.

This condition is rare! They're really not sure of the prevalence of it. My mom has been doing research on it and has found things about it that were published LAST YEAR! They've known about it for about 35 years, but are just starting to find out the genetic background for it. There's probably going to be more that's found out about it in the coming years as it is related to hypertension and there is money going to hypertension research.

Anyway, why did I title this "God's Plan Being Revealed"? Well, had Princess not been born premature we may never have known about this at all. She could have died of SIDS that would have really been the cardiomyopathy, the hypertension, a heart attack, a stroke, the hyperkalemia, anything really. But instead, God saw fit to have her born premature so that the doctors were watching things like her blood pressure and her renal function right from the moment she was born. She has already brought Glory to His Name. So no matter what happens, it's okay. No matter what issues she has, we'll be fine. She may not be that tall, but that's okay. She may always have to be on medication, but we'll live. By all means, it could be a whole lot worse than what it is. At least this disorder is treatable. We'll just continue to take it day by day and see what happens.

Yeah, I was totally shell-shocked to have this huge phrase thrown at me. And yeah, I had a moment of "this poor child is going to have to be on medication the rest of her life." But my God is with us and He will see us through. No matter what happens, we're going to be okay.

Trip to Stanford, Day 2

Friday morning, we were up at 6. We went down to breakfast and after getting something to eat, we packed things up and checked out shortly be 8. Our first appointment was with GI at 9. Well, of course we got lost on the way. We wound up finally getting there around 8:30. We parked in the hospital garage because the Heart Clinic is actually in the hospital. We hiked across the street and made our appointment with plenty of time. We were called into the room, they did her vitals but couldn't get a blood pressure. What a surprise! Her weight was 11lbs 4oz! She was almost 22in long! Wow! What happened to my little girl?

The GI nurse practioner was nice. She listened to our history and understood when I told her that the reason we were here was because we had the appointment across the street and the pediatrician didn't like the fact that we didn't meet with the local GI clinic until September. She told me we could start actual food at 4-6 months adjusted. Also said that we should start milk products around a year adjusted. Said we could do dairy products but also bread and crackers before whole milk. She said the bread and crackers actually had dairy in it that was more broken down than something like yogurt or cheese. So that makes me feel better.

So afterwards we headed across the street out the back to get some mocha at Nordstrom. We were there so early that we heard their morning team meeting! So after we had our drinks, we headed over to the hospital. That was a whole totally new experience in it's own right!

For one, we had to check in and get photo ID sticker badges! WHOA! Okay, so it was kind of nice that both kids got my picture stuck to them. Kind of like the whole "if found return to" idea! So then we had to go into admissions because Princess had an echo scheduled. Okay, well then we were there way early because Princess's echo wasn't scheduled until 11:30. Mom had thought about going and meeting up with Owen, but the school was on the 3rd floor and it was just going to be too much hassle to go and track them down. Beside the fact, I didn't want to disturb him in kindergarten. Kindergarteners have a short enough attention span and ones who are going to school in a hospital have got to have bigger things to worry about than just school work.

Fortunately, this is a children's hospital. So they try to make things as kid friendly as possible. So it turned out that there was a train a short distance from the Heart Clinic. Little Man actually liked the train. I have no idea why. There were buttons on side that made train sounds and made the little lighthouse light up.

So around 10:50 we head into the Heart Clinic. I knew there might be paperwork, but I wasn't sure. We had filled out a bunch at GI, so I wasn't sure what we were going to have fill out. There wasn't anything outside of a consent to treat there. They had kiddie masks there at the desk, so I snagged a few. I know at some point this RSV season Little Man will get sick. So in order to hopefully help keep us healthy, he'll have a mask. Try finding child surgical masks. Go ahead. Go down to your local pharmacy and see. You probably won't find any. Well, around 11 it was time for Princess to be fed. So I set about feeding her. I get an ounce and a half in her and they call us for vitals. She weighs 11lbs 3oz now! They get her blood pressure and it's 115/82! Oh the humanity! Okay, so we get ushered back out into the waiting room and I finish feeding Princess.

Then shortly after 11:30 we get called in for her echo. So they wrap Princess all up and she HATES it. Thankfully they have baby candy for her, so I dip her binkie. Now here's the thing, they WANT her to fall asleep! So here Mom and I are on one side of the bed and the tech is on the other side. I'm on my knees most of the time and the other part of the time I'm in a chair. Being we hadn't been here before, we had to do a full echo. I forgot how long the first echo was! At one point, they had to do the carotid artery in the neck and I wound up sitting there with my arm under her neck, propping her head back. Oh it was agonizing! Then, once the echo was done, we had an EKG. Well, it took longer to put all the stickers on her chest than it did to do the EKG! Then we were ushered back into a room.

We must have sat in that room for 45 minutes just waiting! Mom was starting to get annoyed. Remember, I had been called saying that Dr. R wasn't available. Finally, after about 30 minutes or so, the nurse came in and said that the doc had just gotten out with his other patient and was reviewing Princess's chart. Okay, so maybe soon. I told Mom that the doc might have to read everything from Dr. K and she said we could be there until next week! (Yeah, Dr. K is a little OCD.) Anyway, so finally, the door opens and in walks the doc. It's Dr. R! I told him I was surprised to see him because they had called saying he wasn't going to be in. He then said that he would have to check with the front desk people because they probably called the wrong patients! So he examined Princess and she was just a little flirt! He would touch her tummy and she would giggle and then pout and then giggle again! She even put her hand on top of his! It was so cute!

So I gave Dr. R the run down and he essentially said that we were in good hands locally. She would probably always have an athlete's heart. But that he did want to see the blood pressure better controlled. He would let Dr. K know that and if we weren't better controlled by a year adjusted, he would see her again, but otherwise, she was in good hands. Oh what a relief! I'm SO glad and so thankful! Not many kids go into LPCH and are told "You're fine. We don't need to see you again." This was the absolute best news possible! Praise God!

So after we got out, we found Little Man out at the train with Pops. We then went out to the car and didn't have to pay for parking because when we checked in they gave us a pass! Well, by the time we left the hospital and got on the road it was about 2:30. Oh Heavens! That was a nightmare! We wound up in traffic. Okay, so seeing Dr. R was definitely worth the wait in the room. He was the one we were sent there to see and is the LEADING pediatric cardiologist specializing in cardiomyopathy, but did he have to delay us so much to put us into traffic? Oh, it was agony! We all had skipped lunch and Little Man was crazy hungry! No matter how many Cheez-its he ate, it didn't help. Finally after 2 hours, we were at a stopping point, mainly because I HAD to go to the bathroom! Then me being a mom hit. Let's see, went to the bathroom at Panda Express. We decided to eat there, so I got mine and Little Man's food. (Mom and I were going to share.) Then I had to go out to the car to get Princess's medication. Gave her the medication and then had to shake her bottle. Thankfully Mom fed her, but then Little Man had to go potty. By the time I sat down to eat, my food was cold. Oh well.

So then we got back on the road and it was about another 4 hours before we got my mom's house. We had MORE traffic and it was an hour further north from our house to my mom's house. We stopped at Starbucks and hour from Mom's house and Little Man got an apple juice while the rest of us got decaf mocha's. Oh we so needed them! We got to Mom's house around 8. Pops took their sitter home and I got Little Man into bed and Princess in her jammies. It was a very long day and we all just hung out at the house on Saturday. It was a much needed rest after the craziness that was Stanford.

Thankfully everything was good. I can't believe our good fortune. Every so often I am just blown away at how God has shown Himself in our little family. Princess is truly a miracle. And there are so many things that could have happened to her that haven't. I look at her sometimes and am just blown away. I sometimes wish she looked or acted sick because then it would be easier to wrap my mind around it. Instead, all of her issues are internal. So it's sometimes difficult to look at her and remember that she has all these 'ologists following her. Then I remember than any one of these issues that she's had (the hypertension, the hyperkalemia, the cardiomyopathy), if left unchecked could have taken her from this world and it would have been ruled SIDS. How fortunate we are that our pediatrician decided to send her to Dr. K. Had she not, Princess may not be here. How blessed we are to have everyone in her life looking out for her.

Immunizations

I hate immunizations for the kiddos. It just kills me. Today was Princess's second round of 4 month immunizations. Never mind the fact that she is 5 months tomorrow. Why a second round? Well, I've never been really comfortable with the amount of immunizations they give kids at the same time. Little Man's first pediatrician, Dr. C, only believed in giving no more than 3 at a time. When we moved, he had to get his 6 month shots. We didn't have a pediatrician right away, so we went to county health. Poor kid got so many shots!

Now it's Princess's turn. Well, when she had her 2 month shots, she was still in the NICU. She was attached to monitors and had a nurse hovering over her. (This was also a pretty bad day for her in general, as her GI bleeding started that day AND they wound up stopping her feeds.) But now that we are outside the hospital, it just makes me nervous to give her so many immunizations at once. Call me an over-protective preemie mom, but it just scares me. Thankfully, Princess' pediatrician is okay with us doing 2 a week. I guess I'm not too overly cautious, because some parents do one a week! As we were getting ready to leave for the pediatrican's office, I decided to change Princess because she had had the big ole reflux spit-up during her last bottle. So I lay her down, changed her diaper, and go to put on a new shirt and she spits all over that one! So we try again and again it's the same thing! So three shirts later, we are finally out the door! Of course, when we get there, Little Man trips and scrapes his arm. So Princess got passed around to the front office ladies while I cleaned him up. Then, as we're back to get weighed, I realize that Princess has pooped. Oh the humanity! Then, bad mommy that I am, I forgot her shot records! They were in her binder and I had carried them with me to Stanford and everything! But I had taken the binder out yesterday to run to the grocery store. And on top of that, I forgot to get out the binkie before she got stabbed! Poor kid! I am SO making her baby candy for her next round!

Now that we are home, and she's had a good nap, she's starting to fuss that she's in pain. So I gave her some baby Tylenol (she can't have Motrin). Oh that was a new world of mess! I hate syringes now. I think if she needs it later before bed, I'm putting it in the nipple-doser.

I'm SO not looking forward to the 6 month shots. There will be 5 of them, PLUS the flu shot twice, PLUS the RSV shot (wherever she is getting that from!). Poor kid will get stabbed 8 times over the course of a month and THEN have to go back every month for the remainer of the RSV series. She is SO getting baby candy! (So you know, when I talk about baby candy, I'm simply talking about corn syrup and baby water. It's essentially sweet-eaze like they use at the hospital, but homemade. It's not going to hurt her. It's just a few dips on her binkie. It's a hospital trick to help aid with the pain.)

Bachelor Weekend

So while we were off at Stanford (Day 1 and Day 2), Hubby had the house to himself. Since we decided to just stay at Mom's house for the remainder of the weekend, he had 3 whole days to himself! Well, most of it was spent working on Friday and Saturday this happened:

Hubby and my FIL fixed the crazy fence. They literally had to disassemble the WHOLE thing and fix it. It was that crazy! Yes, the temporary fence is still up because they have to do the end next to our house. I'm not sure if they're going to do a gate or not. Then Hubby came to church on Sunday. He is the second in command when it comes to the sound booth at church and since the leader of the tech team was taking the weekend off, it was probably a good thing that we weren't there. This way, Hubby just had to get up and get himself ready and leave when he needed to; he didn't have to wait for us.

Now of course, with us not being there, that meant that Hubby could do what he wanted to do, or not do other things, like empty the dishwasher. Oh well. No big deal.

While we were at church on Sunday, I guess my FIL came over and finished the fence. Well, he also brought us a HUGE watermelon, a cantaloupe and this:

Yes, that's a HUGE box of jalapenos! Now here's the rub: Hubby is the ONLY one who likes them! He can literally eat them by biting them. (Think the way Uncle Si did in Duck Dynasty when the brothers were trying to eat hot peppers. Yeah, like that!) I guess my FIL thought we needed some jalapenos. Well, after two days of smelling them, Hubby finally took the WHOLE BOX to work. I guess one of his coworker's has someone in their family who makes jalapeno jelly. He snagged a few out of there and they are near the sink, which is fine, but I'm glad he took them to work. I had NO IDEA what to do with ALL those jalapenos! They were just going to go bad before he ate them all.

At least Hubby's bachelor weekends are mild in comparison to some other married guys. At least the house was still in one piece and not completely destroyed! Hubby usually cleans when he's home alone, but obviously the fence was more important. I'm just glad it's almost done. It's been needed for the past 3 years.

Trip to Stanford, Day 1

Last Thursday, we headed off to Stanford. Princess's big clinic day wasn't until the next day, but because her first appointment was at 9a, we decided to leave the day before. I had written a massive list of things to bring. Most of them weren't any different than when I go to my mom's house, but I went into detail so I wouldn't forget anything. I had things like formula, baby water, medications, a dozen diapers, etc. I usually don't write these things down because I just bring them with me. I also had to bring the stroller, and the pack 'n' play. I don't usually have to bring diapers or bring some place for Princess to sleep, but being we were staying in a hotel, I needed to bring all these things.

We went to Target, so I could get a few of the things we needed and while I was there, I decided I needed some new clothes. Well, nothing fancy. I definitely needed new underwear. Everything I had was either now too small (dang pregnancy weight gain!) or was the post-c-section granny panties I had picked up. I know it sounds weird, but I wanted something new for the big appointment day. So I also picked up a new top and some new yoga pants. Yoga pants are my go-to right now. It's either those or pregnancy pants or size I-really-can't-be-that-size pants. I really hate my post-pregnancy body and oh how I wish I was the size I was when I got married. But at least if I was going to be going to one of the leading children's hospitals in the world and meeting with one of the BEST pediatric cardiologists in the world, I figured I'd better not be too frumpy! Plus, the new clothes took my mind off any freaky nervous feelings I was having.

So then I realized that Little Man would be in the car with us (where else would he be?) for nearly 3 hours! So I had to find something for him to do in the car. So I went a little crazy and had some fun on Pinterest looking for things we could take. The last time I had taken him on a trip was for my step-mother's memorial service back before I got pregnant with Princess. It was then, that my mom suggested a car bag of toys. So I set to work putting one together. I'd been collecting snacks for a bit but still needed to put things together for him. (I'll post about the things in his trip bag later.)

Knowing that my mom was going to try and get off work early, and would be at my house sometime around maybe four-ish, I set about packing everything. Well, after lunch, my phone rang. It was Stanford calling to say that Dr. R would not be available tomorrow and if I would be okay to see Dr. Y. If it wasn't I could reshecdule. I was okay with seeing Dr. Y mainly because we already had an appointment with the GI clinic early the next morning and we had had the appointment with the heart clinic for a month now. I knew Dr. K (our cardiologist) would probably not be happy that we didn't get to see Dr. R, but I was fairly confident that they weren't going to find anything new. The worst thing I knew that would happen would be that they would want to do a cath and we would be there overnight. But I knew that if they wanted to do that, they were going to have to convince me that it was necessary.

So after I got of the phone with Stanford, I was actually feeling okay. So I wasn't going to see Dr. R, but things were going to be okay. So around 2p, I had to give Princess her blood pressure mediation. This is not the easiest task to do with an infant. If you've ever tried to give your baby Tylenol, you kind of get where I'm coming from. The only difference is that usually even the infant Tylenol or Motrin is flavored. One of Princess's medications doesn't have a flavor. I don't think they can add flavoring either. The reason I think this is because on one of the packets of instructions I got it said to mix it with applesauce, water, or pudding to help with the taste. The packet specifically said NOT to mix it with formula or breast milk. So here I am giving my infant who isn't developmentally ready to eat solids or drink water nasty tasting medication. Well, usually she does okay, but sometimes she spits it. This ALWAYS worries me. I KNOW I'm not supposed to give her more if she spits it, but is the reason her blood pressure isn't coming down because she's not getting enough of the medication? If she doesn't spit the medication, sometimes she chokes on it. This is what happened. She spit some and then choked on the rest! I knew this wasn't going to be the easiest day at that point.

Thankfully, Little Man laid down by himself and took a nap, so I was able to finish packing the rest of my stuff and Princess's stuff without him being underfoot. So by the time I got everything together, I had the stroller, car seat, pack 'n' play, two bags for Little Man (one was his car bag and one his suitcase), two bags for Princess (one was her usual diaper bag and the other had all the extras), and one bag for me (I had put my clothes and Princess's clothes in there). I knew my mom was only going to be bringing one bag and probably her work computer. The rest of the trunk was going to be our stuff!

My mom showed up right when she said she would. I had Little Man up and while Pops packed the car, everyone did a potty stop. Then we got on the road. It only took us three hours and I managed to map the way that avoided all the tolls going there! I honestly don't think that was that difficult. I think the tolls were for those crazy people who want to get there faster. We had Taco Bell for dinner, which was a little difficult to find because in the Bay Area they don't allow signage on the freeway. I guess it would get too difficult to see anything if everyone had their massively tall signs all the way up in the air all over the place. Of course, when we went in there, Little Man said he had to go potty but came out screaming a second later. Taco Bell apparently had the hand dryers on the wall that would just turn on automatically if you put your hand underneath it. So I had to go take Little Man into the potty to make sure the "Loud Thing" didn't come on.

As we were on the way to the hotel, we saw lots of tech companies: Dell, Microsoft, etc. We were definitely in the Silicon Valley! We wound up staying in Mountain View because we couldn't find a hotel that was decent in Palo Alto. Apparently, it was the weekend before school started! I didn't get pictures of the hotel, but when we got there, we had to get one of those carts because we had SO MUCH stuff! Once we got into the room we rearranged things slightly to fit the pack 'n' play in there. I then set about making formula and rearranging the counter by the little sink and fridge. Mom remembered the paper towels that I had forgotten and brought her plastic measuring cup as I didn't have one. We showed Little Man the bathroom and he about had another fit when he saw the blow dryer on the wall. He thought it was another "Loud Thing." We unplugged it and let him press the buttons on it to make sure it wouldn't come on. I know this seems like a lot for something so simple as a hand dryer, but he's had some bad experiences with some of the Dyson jet hand dryers when he was younger. I think it was RIGHT at the point where the fears would kick in. So we have to explain a lot to him, but he's okay eventually. He even took a bath in the hotel bathtub! Though he was quite disappointed he didn't have any toys.

When MeMe and Pops stepped out for a smoke, I sanitized all the doorknobs and faucet handles. Yeah, I knew the room was clean, but a lot of times, the doorknobs and faucet handles aren't done and they are GERM MAGNETS! We then settled in to go to bed. Well, Little Man was WAY too awake and wound up staying up until 10! I was up at 3:30 feeding Princess and then finally fell back to sleep around 4. I knew the alarm would go off at 6 to start our long day at the hospital.

Day 2 of our Stanford trip still to come!