Check-up

Drew and Dr. B

Drew had his five-year check-up yesterday with our pediatrician. I love our doctors! Dr. B answered all my questions, went over all of my concerns and spent nearly an hour with us and never made me feel rushed. Drew is growing well, he is now 40 lbs 9 oz (49th percentile) and 41 inches (14th percentile) and is pretty much sticking to his own growth curve. We updated his electronic medical record as he is no longer taking any medication (!) and his hydronephrosis is considered to be resolved. We are getting another referral to PT to have them check out his orthodics. We are also going to see immunology again in the spring to see if Drew can have his live virus vaccines yet, but I'm not very hopeful about that. He did get two shots yesterday though (most kids also get their chicken pox and MMR boosters) and was very brave. They also did a hearing and vision test and he passed both of those. Yay, we're ready for kindergarten!

Today Drew had a fever, 100.8 when we took his temp and gave him some motrin, then fell asleep. That worried me, so we put a call in to the doc, and they say that kids are more reactive to the vaccines at this age than as babies, and that it is probably from that. Yesterday when we were there his blood pressure was high both times they checked it. I forgot to ask the doctor about it then and mentioned it today. They want to check him out so we're going back tomorrow.

Appointments

Yesterday was a long day!

First Drew had a dentist appointment at 8:30. I knew he would probably not enjoy having to lay down while people wearing scrubs and gloves hovered over him, and I was right. He is so good though! He didn't like it and he cried (sometimes screamed) through his cleaning, but he did open his mouth when they told him to, held still, etc. When it was time for the exam he did much better, opening his mouth for the dentist to "count" his sparkly teeth. He was handsomely rewarded with a blow up dinosaur toy and dinosaur stickers. The best part of the cleaning is that they were able to get the iron stain off his teeth that turned them gray when he was in the hospital. Now they are bright white again!

We got home from that appointment at 10:15 and headed out to UCSF at about 10:30. Our appointment was at 1:00. So we packed lunches and snacks for the road and Drew was a very good traveler, spending most of the trip watching Veggie Tales and cracking me up with his rendition of the opening song. We got to UCSF right on time and saw a the doctor around 2. The immunologist specializes in kids with 22q and she normally doesn't have a lot of encouraging things to say. But this time she told me she was very impressed with how far Drew's come. He had his developmental assessment in April and Dr. Wara said that he scored about average for his age. She told me that this is much better than most of the kids she sees with 22q and that it is something to be really happy about. She said we can expect him to do as well as any other kid his age in school. I did ask about the funky immunology labs we got at Stanford. She said she could not think of an explanation for those "normal" results and she was very interested to see the labs from this visit. I told her I thought maybe they got his blood mixed up with another kid and she thinks that is quite possible. I'm still hoping it was an immunology miracle and his levels will be high enough that he can have his live virus vaccines, but I don't really think that is the case. We will see her again in four months.

We headed over to the lab and the phlebotomist is really good and always gets blood on the first try. I guess she had an off day though, because it took her two tries this time. Drew did really well, he definitely hated it, but he didn't scream as loud as he did at the dentist and he calmed down quickly. Afterwards I took him across the street to the gift shop where he picked a prize of m&ms. Then we began the drive home from SF in work traffic. UGH. Three hours later we were finally home!

Assessment

Drew and I drove out to Palo Alto on Sunday afternoon to stay with Grandma Kathy before his developmental assessment at UCSF on Monday morning. My friend Katie and her cute baby Chloe joined us to keep us company and to help out with accessing the carpool lane on the way home. Thanks guys! We got into PA around 7 and Drew spent some time playing cars and airplanes with grandma. In the morning we left the house by 8:15 and got into the parking lot at UCSF right at 9:30. His appointment lasted more than 3 hours and since he is four now it was different than the previous times he's been assessed.

I had told him we were going to the doctor to play games and puzzles and there wouldn't be any shots or pokes. I also promised I'd stay with him the whole time. The psychiatrist asked him lists of questions (how old are you, do you have any brothers or sisters, i'm thinking of an animal that meows-what is it, what can you tell me about shoes, etc.), had him copy patterns with blocks, did analogies with pictures. Not so exciting for a four-year old to sit and listen for 3 hours! He had one ten minute break in the middle, but that was it! He did really well, but was getting tired and disinterested the last hour. They also did a quick neurology assessment because Drew was part of an MRI study before his first surgery. His reflexes looked good, but his balance isn't great. I'm also concerned about his fine motor skills, so we'll see what the report says.

After the appointment we visited with Tina and Baby Caleb who is recovering from his Glenn at UCSF right now. We had lunch across the street and had a nice visit. I took a quick look around the UCSF Bookstore and found another reason why I feel good about having heart surgery at Stanford. Dr. Hanley wrote the book on Pediatric Cardiac Intensive Care. Seriously.



I was really appreciating Katie and Chloe on the drive home when we got to fly by all the non-carpoolers on the freeway. We still didn't get home til after 5 and I was pretty tired. So was Drew! He fell asleep in the car and then slept until 9 before waking up, eating dinner and watching a movie. He went back to bed around 11.


As for Ava she did great without her pacifier while we were gone. Dan said she went to bed without a peep on Sunday night and did wake up once, but fell back asleep within ten minutes. Then on Monday she took her nap without crying and went to sleep that night without crying. She did wake up once last night and cried for a while so we brought her in bed with us. That will be the next habit we have to break. :) But I am very proud of her, she is doing a pretty good job.

Today I am feeling pretty tired. This morning I worked in the childcare at my mom connection and hung out with 12 two-year olds. That alone would be tiring, but when it was over and I went to pick up Drew he was in the Director's office because he had lost his outside time for not listening to his teachers. I told her about his assessment yesterday and how he was probably pretty tired of sitting still and listening after that and she did say that might explain it. But when I asked if he usually had trouble listening she said it had been getting worse lately. Which makes me sad because my mind automatically goes in two directions. 1. Maybe he knows what's going on surgery-wise, even if it may be subconsciously, and he is acting out because of it. 2. Maybe it's because of his DiGeorge syndrome and his behavior problems are beginning and it will just get worse. Either way, I'm not really sure what to do because he needs to listen to his teachers so I feel like I want to discipline him, but I also feel like he already had his punishment at school by not being able to go outside. I think I will just talk to him about it and work on listening today. I also am feeling a little overwhelmed by sadness. Just thinking about sick kids, kids having heart surgery, kids with cancer, kids with parents that can barely take care of them, it just seems so unjust. And then I think about my boy heading into his third heart surgery and I just don't want to do it. I know it has to be done, but every part of me wants to just run away and pretend like it's not happening. Maybe I need to sit and have a good cry, then pick myself up by bootstraps and get this house into shape. Even though I was only gone for one night, things have really fallen behind.

Warning: This is an entire post of complaining.

This morning Drew had his blood test. My plan was to get ready for the gym and take him right after the blood test because he likes playing there. So I didn't take a shower, I put on my gym clothes and loaded up the kids in the car. When we got to the lab I dropped off our medical ID card and sat down with Drew. I let him open the package my mom sent for him "for being sooo brave." He was all ready to go, in the right mind set, when the woman at the front desk tells me that there is no lab request in the computer. I tell her, it's the same as last week, we just need to check his calcium levels. Nope, nothing in the computer. Fine, I load the kids back up and go upstairs to the endocrinology clinic. I tell the receptionist what we need and since it's also the cardiology clinic I ask about the referral to Stanford. No record of a referral, but she'll leave a message for the cardiologist. Fantastic. They resubmit the lab request (even though she says she can see the original in there) and we go back down to the lab.

Drew did such a good job today! He was the best I've ever seen him. Instead of screaming from the second they touch him, he just said "ouch, ouch, ouch" during the stick. Then he calmed right down. I was so proud of him! The sticker book of diggers and dumpers that grandma sent really helped! Then it was back up to the pediatrician's office to see if they had record of the referral from the cardiologist. Nope. At least we have a month and a half to get this part figured out.

Then we had to stop at the pharmacy to pick up Drew's medication. As usual there was not a single parking space. So I parked wherever I wanted to figuring if I got a ticket it was worth it since there was seriously nowhere to park. I take the kids out and load them into the stroller and make my way into the pharmacy. And guess what? My medicine wasn't ready. AGAIN. The poor lady, I lost it a little. I was like, "the pharmacist said he was going to fill it yesterday so that it would be ready today when I got here." She says, "I'm sorry, it's going to be another 30 minutes." So I said, "This always happens! What can I do so that my medicine is going to be ready when you say it will?" She was very sweet and apologetic and said she would give me her name and personal phone number and I could call her before I come. So I apologized and said I knew it wasn't her fault, but it's so frustrating getting the kids in and out of the car and there's never any parking! So I said I'd come back in 30 minutes. As I'm walking back out to the car I thought I saw the parking people at my car giving me a ticket, and I just thought Great! That is awesome. But it wasn't them. Phew!

I move my car and see a parking space open up! I wasn't going to take any chances so I pulled right in and it luckily still had 30 minutes on the meter. Which was a good thing because all I had in my car was two pennies. I didn't even have a dollar to put in the change machine. So now I have to figure out what to do with my two frustrated, tired, and whiny children for half an hour. So we walked around the grass for a while and then went back in because I was going to ask the pharmacy lady for a quarter for the meter. If my medicine was ready when it was supposed to be I wouldn't need any more change! But by then the medicine was ready. Thank goodness, because the kids were tearing up the place running back and forth in front of the automatic sliding doors and some lady even said "excuse me!" to one of them to walk by and get to her window. Whatever lady. They were well behaved two hours ago when we started this wild journey.

By the time we get into the car it is 11:15 and I know if we go straight to the gym they will be starving before my workout is over so I give up on that idea. We did have a good yell session in the car which made us all feel better and we all ended up laughing. We drove by a park close to our house and wanting to be a fun, spontaneous mom I thought "let's stop at the park." So we did and I asked Drew when we were next to the bathrooms if he had to go. No. Then, not two minutes after we get to the playground I hear "I have to go potty!" I tried to take him behind a tree, but it was already too late and he had gone a little bit. ARGH! This is why I asked you three minutes ago if you had to go! Luckily I still carry a change of clothes so he got dry underwear and pants on and we finished our playtime at the park.

I hope the rest of the day goes a little more smoothly. And that I can get to the gym at some point today.

Updates

Pharmacy Update:
I summoned up the energy to call the pharmacy again on Monday to figure out what happened to our medicine. I spoke with the supervisor who called me back after about 45 minutes. He said it looked like they dispensed the proper amount from their records, but that he looked at our refill history and saw that we are typically very regular about when we request our refill so he is going to have a month's worth of medicine ready for pickup on Friday and he won't charge us our typical co-pay (which is $50 so that's almost even). He was very nice and even spoke with the insurance company who will only give us 22 days at a time and won't authorize a refill until the 22nd day. How does that make sense?! Apparently I need to call the cardiologist and ask him to get a new authorization if we want to have that changed. So I'll add that to my list of things to do.

Surgery Update:
Yesterday while I was at mom connection the coordinator from Stanford called me twice about authorizations and referrals. Last time we talked I left her a message with the phone number of the pediatrician so she could give them whatever information they need to make a referral through the insurance company. When we talked yesterday she was like, "I don't usually do this for families, but I'll do it this time." Uh, thanks for doing your job this time. So she called the pediatrician only to call me back and tell me that I need to call cardiology and have them send the info to the pediatrician before they can call Stanford who will THEN call the insurance company. I'm pretty sure she has the number of our cardiologist, but whatever. I called two different numbers yesterday, neither of which was the proper place to call, and eventually left a message for our cardiologist on his voice mail, hoping he knows what to do next.

Other News:
Not much, just doing our normal routine. We do have one more blood test on Friday to make sure Drew has outgrown his need for calcium (fingers crossed!) and then the developmental assessment has been scheduled for Monday with UCSF. I hope he does well! Oh, and I finally got my mother's ring for Ava. Two years late is better than never and it is beautiful! I'm wearing it stacked with Drew's ring on my right ring finger. Thank you Honey!

Endocrinology

We had good news from endocrinology today. Drew has been taking calcium every day, at least twice a day, since he was born. But today, we finally got the news that he may have outgrown his need for extra calcium. Yay! He is currently taking it twice a day so we were told to do it once a day for a week and then we will check his blood calcium levels next Friday. If that looks ok, we will discontinue it all together and check his blood the following Friday. And if that looks good then we can discontinue the calcium completely and we will be down to only two medications! AND the doctor said he'd like to see us in six months and that may be the last time we ever have to see endocrinology!

The kids looking out the window at our appointment today

After our appointment I took the kids to the zoo to meet some friends. It was a beautiful day, but a little windy. Ava loved the penguins, the flamingos, the parrots and the tigers. She was so cute and was saying bye to all the animals. Drew loves the lions and tigers and the crocodile. He mostly just had fun being with his buddies. We had a picnic lunch and ended the afternoon by taking a ride on the train. Both kids were exhausted and fell asleep in the car on the way home!

Drew and Ava at the zoo

Looking for the lemurs

Zoo Friends

On the train

Surgery Date and other stuff

I spoke with Stanford this morning and we have a date. May 23. I think that sounds like a great date for surgery. Drew will be out of school by then, we'll be out of RSV season, it's after Ava's birthday and Mother's Day and it's still early enough in the summer that we can enjoy the fun summer activities after Drew's fully recovered. So May 23 it is.

This day has been exhausting. First of all, it started at 5 am. That is exhausting enough. I feel like I have been on the phone all day long! I called Stanford to schedule surgery. I called UCSF to schedule the developmental assessment (more on that later). I called UCSF again to get Drew's immunology lab results faxed to me so I can bring them to his appointment tomorrow. I called the alarm company to make an appointment for them to install a sensor on the exterior garage door. I called the community police officer twice to get them to come out and look for fingerprints (they couldn't find any). I called a locksmith to come and install a deadbolt on our connecting door to the garage (he already came and did it!) Dan called the insurance company then they called back and I gave them the recorded interview. And in between that I took Ava to her first gymboree class and have swept, vacuumed, and cleaned counters in preparation for lifegroup tonight. I still need to go to the store and buy my items to bring for dinner tonight.

When I called to schedule the developmental assessment it was the same psychologist who evaluated Drew because of his participation in a cardiac MRI study. Well, they wanted to evaluate him again when he was four so she is going to try and coordinate with them so this assessment will work for both reasons. So we don't have a date on that yet, but it will be in April.

I got Drew's lab results from immunology and I noticed something that bothered me. I haven't had a chance to discuss it with the doctor yet, but I will be making a phone call. Drew's CD3s, which are the T-cells that we care about, were lower than they were last time. They were 36%. I don't think they've ever decreased before, so I'm not so happy about that. But he has been healthier this year than he's ever been before, so I'm trying not to worry about it too much.

Consult with UCD

I'm sorry I didn't post sooner, but I wasn't exactly sure what to say. We did meet with the surgeon at UCD yesterday and we learned a lot. When we were told that Drew could have a two-ventricle repair by our cardiologist we thought it meant that they would undo what they've done and reconnect his heart to be just like a normal heart and he wouldn't need anymore surgeries.

Well, that is not what they meant. Or at least, not what Dr. Raff would recommend. It gets a bit complicated unless you know a lot about the anatomy of Drew's heart, but basically they would leave everything they've done, place a patch so that Drew would have two separate ventricles and place an external conduit from his right ventricle to his pulmonary artery. So his blood flow would follow a normal pattern, but his physiology would definitely not be normal. And conduits don't last forever and need to be replaced. Dr. Raff was guessing that a Hancock conduit would last about 15 years before it needed to be replaced. Risk of this surgery was estimated at 2-5% (of any complication from infection to death). Possible complications with this anatomy include stenosis and arrythmias, with Dr. Raff predicting we'd eventually have problems with arrythmias. When I asked him how they treat that, he said sometimes with medication, sometimes with a pacemaker, and sometimes with a transplant.

He presented us with the details of the other surgical option, which would be a Fontan. Risk of the surgery is lower, at about 1-2%. These surgeries typically last about 20 years and then a pacemaker or a transplant is needed. I told him I was scared of the Fontan because I knew that kids eventually need transplants and I just learned that transplants last about 10 years and they rarely re-transplant. He said that his bigger concerns were the possibility of developing PLE or plastic bronchitis. PLE I have heard of due to the unfortunate situation of a heart buddy developing the condition. I haven't heard of plastic bronchitis and I don't think I'm going to look it up. Also, he said with a Fontan Drew's exercise and endurance would be limited.

So we were a bit discouraged to learn that this was not the miracle fix we thought it would be, and that there will definitely be more surgeries in our future. It was also just reinforced that we don't know what the future holds as far Drew's heart is concerned. Transplant seems to be a possibility no matter which path we choose, and I don't like that. But it was still a good meeting, we learned a lot and got one extra opinion under our belts. I am very anxious to talk to Dr. Hanley from Stanford and hear what he has to say. We should hear from him tomorrow evening or Thursday morning. After that I want to meet with Dr. Azakie from UCSF because I haven't had a chance to talk to him myself yet. And then we'll make a decision about where to go from here.

The best laid plans...

What a long day we have had! Everyone in the Himmelberger household is exhausted!

So, today was Drew's big appointment with the craniofacial team at UCD. And I wasn't looking forward to it. I knew it would be long, I didn't think Drew would cooperate and I thought they would have unpleasant news for us. But I made a little plan and figured out a way for me to be ok with it. The plan was that Dan would come with me both for moral support and so I didn't have to bear the total responsibility for answering the questions of a dozen doctors and reciting a medical history that can be mind-boggling. A friend of mine was going to watch Ava so that we could both go to the appointment. I got myself all ready last night. I loaded the diaper bag, set out everyone's clothes, packed toys and snacks to occupy Drew, even got the coffee maker all ready to go. We set the alarm and everything was going according to plan, when Dan interrupted my shower with this news: "Ava just threw up." What? That was NOT part of the plan. And then she threw up again. And pretty much the rest of the day. So obviously all my plans went out the window. I have to say I was pretty discouraged this morning, almost to the point of tears. I really didn't want to go at all and I especially didn't want to go by myself.

But, for a day that started out pretty crappy, it turned out pretty good. My son is amazing. We sat in the same room for 4 and a half hours and saw 12 doctors. He was so good! I almost cried watching him. I told him I was so proud of him for being so patient, for listening to the doctors, for being able to calm himself down, and just being so darn cute and sweet for so long in such difficult circumstances. Seriously, what three-year old can sit in a room for that long and not have a total melt-down?

I was expecting less than good news from the doctors, but they all seemed to be impressed with how well he was doing. At one point Drew had to have his ear canals cleaned with this metal instrument, while sitting in a chair kind of like a dental chair, and had to hold really still. He let them do the first ear, but wasn't ready to let them do the second ear. One of the doctors said if he couldn't hold still she'd have to wrap him (like swaddling), and I knew he wouldn't like that. So I told him, you have to hold still and if you can't they are going to wrap you like a baby. Can you hold still? And he said "yes mommy." Then my sweet, sweet boy started taking deep, cleansing breaths and he held perfectly still the rest of the time! I was so proud of him, being able to soothe himself and do what he had to do. And the doctors were so impressed! I even heard them talking in the hallway about how he did better than the five-year olds they see and how cute he was with his deep breaths.

Three hours into the appointment he had a hearing test, where he had to sit in this tiny room with headphones on and listen for more than half an hour. Every time he heard a beep he was suppose to pick up a toy and put it in a bucket. And I sat there watching him, thinking what kid does this? He is sitting here, following the directions of a stranger, not playing with the toys they way he would want to, not throwing the toys off the table because he is done with these people, but quietly listening for beeps. Every once in a while the audiologist would remind him to wait until he heard the sound to put the toy in and he would look at me and say "I have to listen."

So the basic news is his hearing is fine, his speech is fine, his palate is fine, his teeth are fine, his head shape is fine. The geneticist was impressed that Drew can recognize all his letters (she didn't even know he knows the sounds they all make too) and she did recommend we get an x-ray of his cervical spine. We'll see the panel again in another year or two, and we may see the speech pathologist for another evaluation in about six months.

On the way home I told Drew that he did such a good job that we could do whatever he wanted the rest of the day. We could go to the park with his friends, watch movies, bake cookies, go to the toy store...He picked toy store. We were about half way there when I looked back and he was asleep in his carseat! He must have been exhausted! So I took him home and let him sleep for a few hours. Then I woke him up and said let's go to the toy store! He picked a Mickey Mouse Christmas video to bring home and spent a lot of time looking at dinosaurs, cars & books.

Not only do I have the best son, I have the best husband. He took care of Ava all day while she either slept on him or puked on him and didn't complain about it at all! He is such a good daddy. Poor Ava could barely keep any water down and by the time she went to bed tonight the only thing she had eaten all day was a banana. Needless to say I have been trying to keep her and Drew apart since we don't want him getting sick before his cath next week. I bought some clorox anywhere spray and have cleaned all the toys Ava likes, all the toys Drew likes, the tables, the chairs, the remotes, etc. I also had Dan mop the floor with bleach to kill any germs that might be lurking. We may still all end up puking, but it won't be for lack of trying.