Nolan 9 Months

Goodness, where to begin? I have told myself I was going to write a new blog update at least twice a week since January. We have had a pretty eventful last few months. As many of you have seen, Nolan got glasses (which are super cute), and tomorrow he will be undergoing a surgery on his eyes to keep them from crossing. This seems to be a pretty common procedure with CDG kids. We are nervous, of course, but we are excited that our sweet boy will be able to see more clearly. Surgery is scheduled for 7:30 in the morning and we hope to be home in the early afternoon.

In all other areas Nolan is doing great. He is still behind developmentally, but is doing well in therapy and showing signs of improvement each week. Right now he gets physical therapy and occupational therapy once a week, speech therapy and skills therapy twice a month. Currently we are working most on sitting up using a side sit method where he uses his arms to help hold him up. He can currently stay up for about 10 seconds consistently. We also spend quite a bit of time working on rolling over. He can easily roll from back to belly, but hasn’t decided he wants to try to roll back. He LOVES tummy time and will actually push against you when you try and help him roll to his back. So I think it may be a while before he has mastered this. His head control is getting so much better; he still struggles some when he is tired, especially if he is working on sitting up at the same time.

We are happy that springtime is here, kind of, what is with this crazy weather?? However, we have been able to get out and about a little more. Nolan has been able to attend a track meet, softball game, and a couple of baseball games. We still have to be cautious with people touching him, and our pediatrician recently warned us that though flu season is over, respiratory virus season is still going strong. He advised that we continue to avoid crowds in enclosed spaces. Open air and hand sanitizer will continue to be our best friends.

That’s really all I have for you. Nolan is maybe the happiest baby ever. He loves kisses, snuggling, being tickled, bath time, laughing, talking (he will say D’s, M’s, and B’s), he also likes to growl and screech. His newest thing is spitting and making tooting noises, typical boy. He loves peek a boo, singing, reading books, chewing on EVERYTHING, and being outside. We love to sit on our back porch and watch the birds.

I am sorry it has taken me so long to write this, I will TRY to do better in the future. As always, we appreciate the continued love and support we receive and would appreciate a few extra prayers for Nolan and his doctors tomorrow. 

 

Germs! Germs! Germs!

Nolans first trip on a plane and he got to meet an NFL player, Jermon Bushrod, one of the positive aspects of your dad being a sports buff, he can recognize a professional athlete anywhere.

We had a GREAT trip to New Orleans, and an even better check up with Dr. Morava. Nolan did great traveling, we were pretty nervous about how he would handle traveling and being away from home for so long, but he was perfect, I think we have a little traveler on our hands. He is also a champ at riding on airplanes. Incidentally, we have perfected the diaper change technique in the impossibly small and stinky airplane bathroom (Nolan, who is no stranger to a dirty diaper, he went to the bathroom upon arrival to the airport, both times, and then again while on the plane, both times, TYPICAL BOY!)

All together, we had a pretty smooth trip, and we LOVE Dr. Morava. With so many uncertainties and unknowns about Nolan’s disorder and what the future holds, it is like we are in a dark closet trying to find our way and here comes Dr. M with a flashlight. Her experience and knowledge of CDG is a great resource to have on our team. She spent 2 plus hours with us in the examination room, first giving us a quick lesson in CDG and then answering every question on our very long list. By the time we were through, we had to exit through the back door because we were the last ones in there.

Overall, Dr M was very impressed with Nolan. Our metabolic geneticist from Cook’s, Dr B (whom we also love) had actually emailed back and forth with Dr M about Nolan and his diagnoses when we were in the hospital back in September. So she already knew all about him, and much to our surprise she was just as excited to meet Nolan as we were to meet her. Based on these initial emails, and him having already been in the hospital twice before he was 6 months old, she really expected him to look much worse. She wasn’t expecting the active, happy, and chunky boy we all love.

We asked Dr M what are the most important things that we can do for Nolan.

#1= Avoid infection (for the next FOUR years)

#2= Physical therapy

Avoiding Infection

I don’t want to get too scientific, but basically the majority of his cells are unable to work properly due to the disorder in the glycosylation pathway. If you remember, the first time we were in the hospital his white blood cells (that fight infection) were astronomically high. The reason for this is his body is able to make WBCs, there is just a problem with the function of them after they are made. They are missing the receptor site that allows them to attach onto the bacteria or infection. This leads his body into thinking it needs to make more WBCs; when in reality no matter how many he makes they will not be able to function properly.

Therefore, until the cold and flu season are over, we will be hibernating in our little house. For the sick months Nolan will no longer be able to attend school functions or church, we are going to miss seeing our church family on Sunday morning and getting to cheer on our Longhorns and Lady Longhorns. But, we have got to keep our sweet boy healthy, happy, and germ free!  

We can still have visitors and get to see our family, Dr M just set some guidelines for us to follow

·      No sick people (obviously) or anyone who has been around someone who is sick or has been sick in the past few weeks. Even if it’s “just allergies”, for the next few months we have to assume you have a cold.

·      If you have been to school, or around anyone who has been to school, please shower and change clothes before coming to visit.

·      We are also going to have to limit Nolan’s exposure to other children, especially those who go to school/daycare.

·      Please don’t touch his hands. You can touch his feet or his legs, but please avoid those sweet little hands. He lives with his hands in his mouth, so be assured that as soon as you let go of his hand it is going straight to his mouth.

I am sure all of this makes us sound like crazy parents, and many of you are probably thinking, my kid grew up in daycare and has never been sick because he/she has been exposed to so much and their immune system is strong because of it. While this is great, Nolan simply lacks the ability to “build up immunity”. Exposure to illness and germs isn’t going to help him be stronger for the next time. We will be treating Nolan like a newborn baby until the sick season is over, and then again every year during the cold/flu season, until Dr M tells us it is safe to do otherwise.

            Just to show how serious this is. After Christmas and being exposed to so many people, Nolan came down with a cold, got an ear infection and had a fever. Just yesterday Nolan got a fever of 102. We aren’t sure what caused him to get a fever yesterday, his pediatrician thinks it is likely just some sort of virus, today he is feeling better and temperature is down to 99.8. I just say this to show how easily our sweet boy can get sick.

Physical Therapy

            Dr M is convinced that Nolan looks so great because of the therapy he is receiving and that we are doing our homework when our therapists aren’t here. Nolan goes to Fort Worth once a week for physical therapy, and then through ECI (Early Childhood Intervention) Nolan receives occupational therapy and skills therapy once a week, and this Friday we have our first appointment with a speech therapist. It is great getting to see how Nolan improves week to week. We want to give a special thanks to Joe’s Aunt Phyllis and Uncle Randy for giving Nolan a physical therapy wedge for Christmas. We have certainly enjoyed it and it has helped tremendously in Nolan’s daily physical therapy routine at home.

We went to Mardi Gras world, pretty cool, it is where they make all of the floats for the Mardi Gras parades, they also make the Chic-fil-A billboard cows. Oh, and we ate lots of bread pudding

Overall

            As I said before Dr M was very impressed with Nolan. A few of the good prognostic factors are:

·      Good muscle tone, in reference to other children with CDG 1A of course. This came as a surprise to us, since we are so used to hearing how low his tone is and how behind he is. She does believe that he has some degree of cerebellar involvement, though she does not suspect it to be severe. This is the cause of his tremors and will cause him to have balance issues. Nolan is having an MRI on Jan 27, this will tell us to what degree his cerebellum is effected.

·      The fact that he doesn’t have a feeding tube and is gaining weight appropriately. He has always been in the lower percentiles for his weight, however, from his 4 to 6 month checkups he went up from the 18^th to the 25^th percentile for his weight (when we were discharged from the hospital at 2 ½ months he was below the 1^st percentile). Dr M loved his chubby cheeks, and she loved even more the fact that he was able to get this chunky eating all on his own. This is good for 2 reasons. #1 having a little “excess” weight will be helpful to have more reserve in the event that he does happen to get sick. #2 his ability to drink from a bottle and eat pureed foods is positive for his speech development since speaking uses the same muscles we use when eating. He is also quite the “talker”. She is unable to say for sure if he will be able to learn to speak, but hey, we will take all the positives we can get.

·      His social awareness. Nolan is, and has always been, interactive with us. We have always been able to get him to smile, and he is interested in everything that is going on around him.

He was happy to be home and maybe a little worn out.

We are beyond blessed that we were able to make this trip to meet Dr M, without the love, support, prayers, and financial assistance none of this would have been possible. Words are unable to express how thankful we are.

This has nothing to do with NOLA, but I let Nolan try a grain of rice the other night and he was quite offended

Plans for the Near Future

            January is proving to be quite the busy month. Nolan had his 6month check up this week with his pediatrician, all things looked well. Today he had his follow up with his endocrinologist; again they were happy with Nolan’s progress with being able to maintain his blood sugar while taking Diazoxide. In the next couple of weeks Nolan has an appointment with his pediatric eye specialist, metabolic geneticist, an MRI of his brain, a fitting for a brace (for him to wear for short periods of time during the day to aid in sitting up), and of course his weekly therapy sessions. 

Here is a quick recap of the trip in Nolan's words.

Merry Christmas and a Happy New Year

Merry Christmas and a Happy New Year

I don’t know about y’all, but Christmas is my absolute favorite time of the year. From Christmas carols to Christmas movies, drinking hot chocolate while looking at Christmas lights, and spending time with family. I cannot get enough of it, it always leaves me sad when it is time to pack everything up and put it away. Until next year, here is a quick recap of our first Christmas as a family of 3. 

Nolan got to play with all his cousins, rode on a horse with PaPa, and got to play the role of baby Jesus twice (at church, and in the play put on by NiNi and Sidney).

Nolan got to meet Santa (a couple of times)

Christmas morning as a family of 3, SANTA even left a little something for Nolan, he must have been a very good boy, we spent lots of time with cousin Bradlee.

This morning we are heading off on our first trip as a family of 3, to New Orleans to see Dr. Morava. We are very excited about our little trip, but nervous on how the boy is going to behave in the airport and on the plane. Luckily, he is the happiest baby ever so I don't think we will have much trouble. Again, thank you for all of your continued love and support you all show us. 

THE Test, Cook's Stay #2

THE Test

12/11/13

3:00 we arrive at the hospital and wait to get registered. We finally go up to the third floor.

Same floor we were on the first time but different room. One thing you can not say about cooks is that they have no Holiday spirit.... Lots and lots of Christmas stuff and spirit. There is a 'jingle mingle' in the lobby with lots of food and music. Sadly there is a sign that says "employees only" before you walk in. The aroma catches me even up on the third floor.

We get to room 3111 and it is HUGE. At least twice the size of our old room. Very nice luxury to have when you have so many visitors like we usually do (which is nice).

3:30 128/66 pulse 131

15.2 lbs, quite the change from last time we were here.

Ben is our nurse, he was very attentive with him last time... Asks Nolan if he remembers him... Surely right ?

4:18 Nolan eats has his first diaper on the hospital... Wow, what have you been eating?  Follows that up with a big spit up... Just like home.

5:39 dinner and relaxing

8:06 we go over the schedule... Fasting starts at 3am, checking vitals at 11 and 3 so we can get some "sleep"... Whatever that means in here. Nolan is in good spirits but I think that is because he is wearing his 'born in the USA' onesie... He loves the boss. Not sure how long this will last but it seems like it is well planned out. They will have to draw blood when his blood sugar level gets below 60 and then again at 50.

They test blood sugar in the room and then draw blood and send it off. Only problem was last time they would check and send off to the lab and it wasn't low enough... Time and again... Let's hope that doesn't happen this time.

10:25 Way past his bed time and he isn't happy. He can tell we aren't at home and not on his routine.

They are supposed to come at 11 to test blood sugar, eat, and get an IV all at the same time. Hopefully that can be accomplished.

11:12 the come and get the IV with very little problems, quite different than our IV's last time.

11:33 we are just laying down to rest and in pops the doctor. Doesn't really tell us much but stays 20 min. Nolan sleeps through it all and doesn't wake with any poking and prodding. The doctor is funny though. Starts talking about blood sugar and saying what they need to watch and why. In doing so he talks about how when you get low blood sugar it can lead to some major problems.

Us - how low is too low
Dr - a very logical question. It is like asking how many concussions are too many? Well you never know about who the quarterback is, it could be Ed McMann or Bart Starr... It is different for the quarterback.

I bet Ed McMann was a hell of a quarterback in his days long before he was on Johnny's couch.

Maybe we will be able to sleep a couple of hours.

 

12/12/13

3am fasting starts.  I don't know why but I expect some sort of trumpet sound or flag waving, sadly the nurse just says "ok it's started."

8:02 the night was better than expected... Nolan slept which was a really great thing. I think the noise maker app in my phone worked... He likes the heavy rain.

Dr Thornton (our endocrinologist) comes by and talks with us. We really like him, very practical. Always asks the questions "why are we doing this" and "how is this going to help him for treatment."

Good doctor, seems like he is really intelligent. All doctors are I'm sure at this place but he seems like a level above.

9:27 Dr Bassinger comes by just for a personal visit. She is great, takes a lot of interest in Nolan it seems.

11:12 lab can't stick him so we are trying from the heel.

11:35 a whirlwind of doctors and nurses coming by... All hoping his blood sugar maintains. Only problem is he gets fussy.

2:12 Nolan cried most of the morning but has been good since NiNi got here.

Labs have came back and he is finally at 47 (11 hours). This is good for us because he didn't go down quickly but on the flip side he can eat soon. They will have to draw more blood first and give him glucagon. Then they have to give him the glucagon challenge that lasts 40 min. He has to be tested at 10,20, and 40 min to see where his blood sugar is at. So although the fast is over... It ends with quite the challenge.

2:32 challenge started!!! It didn't come easy though... Trying to get blood was painful for him and us. He is about wore out.

3:24 we made it through and it wasn't easy for him. He is really tired. He is struggling to eat after we gave him a bottle. He ate at 2:30 this morning  so it has been 13 hours, you wouldn't think he would care what he was eating.

4:26 vitals are good and we are just waiting around. Nurse says there is a possibility we get to go home.

I don't really buy it, bet we are here till morning.


4:37 Nolan's cousins Noah and Harper are here... Which makes him happy.

5:05 our nurse misty is here (she had been great by the way) and she
Is informing us that we will be here till tomorrow morning at the least. (Wah wah Debbie downer) sug was not happy with this news.


6:30 Tony and Lisa arrive and it really raises our spirits... They also bring taco casa which is great.

8:26 Nolan gets hungry so we test his sugar, 56... Which isn't good but we aren't worried yet because of the fasting.  Should be better later. We are going to have to test blood sugar after he eats now... Nolan's poor little heal.

8:54 he ate and it is at 78, which is good.

12/13/13

9:23 Dr Thornton is here and talks really quickly and is from Ireland (I believe) so it is tough to understand him.

Blood sugar low - should be 70-110 his are in the 60s

He went down after 7 hours, should have been 18 ... Something is up.


Needs more sugar... Keytones are low because the body needs more sugar and uses keytones instead

Needs fatty acids levels

He has too much insulin or something isn't working right. These tests are trying to figure that out.

Growth hormone might be low

Once diagnose we need to find a treatment ... That is what we are doing these test for.

He says it will be a relaxing couple of days feeding him and checking his blood. I want to ask him if he knows what relaxing means.

His liver isn't inflamed like last time, which is good.

This is not the news you want to hear but it is at least the knowledge that he is getting treatment and we will find out more about what to do.

This does not change his cdg 1a diagnosis or anything about that, this only tells us what part or parts are being affected by it most. There is no cure or treatment to take it away... But there are treatments that can help, and that is what we are looking for.

Still tough to get news that he is crashing and isn't maintaining at near the level he needs to.

On the positive note he is the happiest baby one could ask for.


12:45 Mae Mae comes by and boosts his spirits.

4:15 Nolan is eating every three hours and we are testing sugar before and after if the before was below 60. This past time was at 64, our first plus 60 before eating all day. His poor heel.

7:09 Dr Dan and Dr Debbie come by and it is a breath of fresh air... Nolan is excited.

9:57 his blood sugar has went up and down this evening. They are still checking it pretty closely.

12/14/13

Grandpa jones birthday
State championship reunion

8:03 Nolan has been sleeping better and keeping sugar up.

9:37 Dr Thornton comes up to visit. He goes a little slower and it helps. He says that Nolan suffers from hyperinsulinism and we just have to figure out how to maintain that. We are going to start taking diazoxide twice a day. This will keep his insulin down so that his blood sugar level will stay up. We are going to have to try this for 3-5 days (at least) and then he will have another fasting studying. In that study if his level will stay above the 60s for 6 hours then we can go home. So it looks like we will be here for a while longer but he and we are encouraged about this and know that it is getting better.

This is just leading to how we are going to treat Nolan more than anything. We are happy to have a possible solution to the problem that doesn’t involve putting in a “G button” (a feeding tube that goes straight into his belly) so that he can be put on continuous feeds overnight.

9:28 pm a day full of eating and visiting with friends.

Jim
Noah
Staley
Nini
Lovey and papa
Kristen, Ellie Kate, and Margie

Great day... Especially because the diazoxide is working and keeping blood sugar high and insulin down. His blood sugars are better than they have ever been.

Also nini filled us with Holliday spirit by bringing a tree and Christmas decorations. Sug loves Christmas, and so does Nolan.

12/15/13

8:47 Dr Thornton comes by, no changes, everything is looking good. He is responding to the treatment well.

12:10 NoKo and JoKo watching some football.


4:21 Lovey comes and stays with Nolan, always a fun time for both. The hospital is a zoo though. It is Santa day in the lobby and you wouldn't believe the amount of people coming to see Santa. The line wraps around the entire lobby and the lobby is huge. I run into a friend from Alvarado and he says they waited for two hours last year and didn't even get to see Santa... No thanks, we will stay in room 3111. It is supposed to be for employees and kids in the hospital... Maybe seeing an elf would do.

They finish with a huge fireworks show. It is pretty awesome, and I am not the biggest of fireworks guys.

12/16/13

2:30 am the night nurse asks if she can feed Nolan... It is the most glorious question we have ever been asked.

7:15 it is probably the best night sleep we have gotten since being here. Sug is still asleep and I don't blame her. She is great about getting up all the time.

Nolan has been keeping his blood sugar up and the medicine seems to be working. They said it tastes awful and be ready for the kid to have some stomach issues but so far (knock on wood) Nolan hasn't been any different.

3:53 pm today has been crazy. We look out our windows, and guess who was at the hospital - the Dallas cowboys... Not all of them but a good amount, around 20, of them were and there was a big star in dez Bryant. Beasley, Fredrick, smith, hatcher, scandrick were some of the others. Interesting stuff. Ben Bass and Tyrone Crawford came to our room and got our picture with Nolan. Really cool guys and really good deal they are doing, really cool.

We also participated in 'SPAtacular' which was a free massage and haircut for parents in here. It was very relaxing and thoughtful.

6:12 Dr Starr comes by to visit... Great words of wisdom.

9:43 Nolan has a big couple of days, he will be testing to see if he is producing enough growth hormones tomorrow. It is common in these kids and is something his tests have leaned towards the last few days. Not sure how that will be treated but Dr Thornton will know the trick. We like him more everyday.

12/17/13

4:30 am lab comes to get some blood!!! They don't find any after 15 min... Frustrating because they are putting an iv in at 10 this morning and can get the blood.

Rain (the best person from lab) is about the only person who has been successful with Nolan. Many MANY others have not been.

9:25 Nolan gets done with his workout and we are ready for the 10am slate of testing. It is testing his growth hormone and I am not sure how that will be done... We will figure it out together. My largest concern is getting the IV. Hopefully, it goes smooth. Usually it is not easy.

12:45 getting iv and blood drawn for the last 4 hours was the worst thing ever...

Nolan sleeps for the rest of the day


12/18/13

10:02 after yesterday was such a tough day everyone is a little wore out today. We are fasting again, which started at 6 am. Sara is our nurse and she was the same as our first admit to this hospital some months ago. All we want today is for Nolan’s blood sugar to stay above 6o for at least 6 hours.

2:00 Nolan’s blood sugar after 9 hours is still in the 80s, fast is over, no lab work needs to be drawn, Nolan gets to eat

Dr. Thornton comes in, says Nolan did great with test, and we get to go home! We are all so excited. No one is more excited than Nolan to get the IV out of his hand,  he is amazed that under all that tape his hand is still there (his favorite “toy” these days).

4:30 After a long and tiring week in the hospital, we are happy to be heading home. Most importantly, we are happy to have some answers and a solution to keep Nolan’s blood sugar up. PLUS we are so excited that we no longer have to wake him up every 3 hours at night to eat. 

So happy to have his hand back and be heading home