Newest Trip to Boston

We spent a VERY long day in Boston today.

The advantages to scheduling all his appointments on one day:
1. Only have to leave the house at 5:30am one day a month
2. Only have to have blood drawn once
3. Only have to endure the horribleness that is Boston drivers once

The disadvantages to scheduling all his appointments on one day:
1. Being at the hospital for 5+ hours
2. Trying to get the kids (whichever ones are with me) to be calm during the very boring appointments
3. Myself having to sit through the appointments (very trying on my nerves)

I imagine there are probably a bunch of other reasons for it, but those are just off the top of my head.  It's torture, plain and simple.  But, i'd rather endure it once then to go back the following week and do it again.

ANYWAY!!!

Today, we had:

7:30 appointment 

8am echo, then EKG

9am appointment with cardiology

10:30 with nutrition

11 appointment with metabolism

Dean weighed 15lbs 3ozs!!!!  We are slowly making progress to the bottom of the growth chart!!!  Proportionally tho, he's at the 15th percentile, which means that he is just tiny. I haven't had any news on the labs (usually takes a day or two before I hear anything) but there was indication that his echo showed slight improvement, which means that his new meds (he is on 8 now, soon to be 9 different meds) are working.

So, good news today! :D

Boston

July's visit to Boston ended up being a very long day.  We got there early, did the labs before the appointment.  I got the lady for height/weight and EKG that I do not like.  She has the bedside manner of something that doesn't have great bedside manner (when i told my story at supper time, i said fruit fly - but I don't know how a fruit fly's bedside manner is, to be honest!).  I just don't like her, I find her rude, but I try to not let it bother me. 

Anyway, it seems as if Dean is not only 14 pounds - he's 14 pounds and 3 ounces! (cheers!!!!)

We saw one of doctors that we don't usually see, which is fine.  He wanted us to try a new medicine.  Earlier this year, they had me fill a prescription and try this medication in clinic.  It can drop blood pressure pretty badly, and that's what happened before.  The doctor had said that he thought that perhaps Dean was not as stable as he is now, that he might tolerate it better now that he's older and weighs more, perhaps they tried too much too soon.  If his blood pressure dropped again, they would leave it at that and let it go, but this medication had shown great things when it came to healing hearts, and hopefully it would make a difference for Dean.

I had with me two of my other three children, and at this point it was 12:30 in the afternoon and they had not eaten lunch.  After they give him the medication, they wouldn't let us leave the office, so I said that i needed to feed the kiddos.  So, we went and got lunch, got back to the clinic, Dean got the medicine, and we waited.

For an hour. In the waiting room.  Luckily, they had Wall-E on the TV in the waiting room for the kiddos to watch, so it wasn't as bad as possible.

They took his blood pressure and it was fine.  I am relieved.  It seems to be something that the doctors really think will work and I hope that it does.  It brings his meds to 8.

At least it doesn't seem to be bothering him.

9 Months!!

We made it to 9 months!!! :D

Today we had an PT appointment as well as the 9 month well-baby appointment.

I think the visits with the therapist are working well.  I definitely feel as she is helping me understand what I can do to help get Dean on track to being where he should be developmentally.  Today we worked on getting him used to putting weight on his legs and lifting his chest off the floor.

The well-baby visit went well as well!! :D  Sometimes I feel like the PCP is the low man on totem pole of doctors (which, she is).  She was happy to know that the PT was coming out to the house (thought we had it sooner then we did) and was a bit concerned that the home nurse's visits were cancelled due to insurance not wanting to pay for them anymore.  We tried to cut back to every other week, but they said that if he was well enough for them to cut back visits, he was well enough for him to not have any visits.

And he's now 14 pounds!!!!!!!

Insurance sucks.

That's it for now!  Off to see the Cardiologists in Boston tomorrow.

Food

Food has (and I have a feeling that it will continue to be) an issue with Dean since he came home. It's been a challenge to get the required amount of calories in him so that he can grow. Now that Mr. PointZeroFourPercentile is old enough to be eating solid foods, it's been a struggle to find foods that 1. He'll eat and 2. That meet the calories that he needs. So, I've been only feeding him solids at dinner time here, so he still gets the majority o his calories from formula. Today was the exception.

The Boston nutritionist told me that it was okay for him to have (lots of fat) yogurt around 9 months. He is 9 months in 5 days, so I bought some GoGurt. I have now discovered what yogurt I SHOULD have bought, but he REALLY likes the GoGurt!!! I mean, REALLY REALLY likes it!

Success!!! :D

Physical Therapist

Today the therapist came to the house to check Dean out. We talked while she did her stuff, and she noticed that in addition to Dean not using his left hand and foot as well as his right, he rolls over his left arm but not his right. So, we are to keep him blocked from rolling over his left arm and help him roll over his right for a bit each day as well as get him to sit up (with help, of course!).

I think it went well. I think Dean will like her just fine, he seemed to be warming up to her nicely by the time she left. I just need to figure out when would be a good time for her to come by every week.

The Boston people tweaked his formula after the last visit. I'm hoping that they will see the difference that they're hoping to see.

Not much else going on at the moment. I think I'm okay with that.

Weight charts and Boston trip

The docs in Boston plot Dean's weight on the WHO growth charts. Last night, I was looking through the most recent write up I have from some of the docs in Boston and saw percentiles for Dean's weight. On May 9th, he was 12lbs 3.4 ounces which put him in the .04 percentile.

He's now just over 13lbs (13lbs 3ozs). He does appear to be gaining weight on "his own curve" which is good because there was a period of 6 weeks or so when he wasn't gaining any weight.

I just found this a bit crazy.

We were in Boston yesterday for a very long day of visits. I took my 4 year old with me and that didn't go as badly as I thought it might. She was rather well behaved; she only wanted to show off a few times (thanks to my kindle fire, netflix and free hospital wifi!!!).

They are generally pleased with Dean's progress. As stated above, he is growing on his own little curve (which, for the most part, parallels the normal growth curve) but he is growing. They aren't sure that he is small because he has Barth's or just because he is small (older sister was in the 1st percentile for weight when she was younger).

Ive had the home nutritionist come out three times now. Each time I thought that she was useless, each time was just a waste of time for the both of us. I think I might just tell her to not come next time. She told me that I should blend my own baby food because it tastes better (and he'd like it more) than store bought baby food. I cringed at that. I don't know if Dean will have dietary restrictions due to his heart condition. So I told her: "I'm not sure how to go about deciding what to blend" and she went on about how I could do just about anything- fruit in water or fruit juice (no corn syrup) or vegetables- no sodium added. I got the vibe that it should have been a no brainier...but it isn't!! Nothing about this child is like the other three!! So, I brought this conversation up to the nutritionist in Boston. She told me that blending my own baby food was ridiculous. She said I had enough to do and she relieved me of that suggestion and said that store bought baby food was fine. Barth babies just don't really want to eat, and Dean is going through an independent phase (I now have to trick him to eat purées, he eats those Gerber puffs wit such enthusiasm tho! I LOVE those!) and that he was doing well. She said that there usually isn't diet restrictions on Barth babies, but that I will need to add fat whenever I can. We had to do this with Regan as well when she was 2: no margarine, have to make his food with butter. Not just crackers-crackers with butter. And cheese, cheese is good. She suggested I start (high fat) yogurt in the next month. Nothing with chunks. Babies are picky, don't like chunks.

I will write more later, I think. That was the big part of the Boston day. The only other significant event was the lack of abnormal stress when he got his blood drawn. That was nice. :)

Eating and bath time!! :)

It's such a pain to get Dean to eat very much. But, he does enjoy playing with his food, so I'll take that, I guess.

Such a messy boy!!!

He started blowing raspberries.
I knew that  he was done!

Best part of eating?!  Taking a bath
and getting all cleaned up!

And CLEAN!!!

Meds

Dean was prescribed yet another medication. This one is an amino acid supplement. Supposedly it should help his heart function by raising the amino acids that he is deficient in.

This brings his meds list to 6. He takes all 6 in the morning, 3 in the afternoon, and 4 at night. And I know what most of them do. It's hard to keep track, and a couple have the same function.

I'm exhausted. Jim has been gone this week and it's been busy. Tomorrow we go back to Boston for echo and cardiologist appointment.

Edit:  My phone likes to auto correct Dean's name to Sean.  I have tried to figure out how to adjust the settings so that it won't do that, but I fail.  I promise that I didn't rename him! :D

Foods

So Dean has had apples, green beans and prunes (prunes were Regan's favorite). So far, it's been quite a struggle. He screamed with the first few feedings. I mean.. SCREAMED.. It was awful.

Tonight I tried the prunes. He didnt scream. He would let the food pool in his mouth, then spit it out. I would put the food back in and it would repeat. I tried placing the spoon on his tongue to get him to swallow and he would choke. I have a feeling that eating will be quite a struggle for us.

He got messier with the prunes...he is such a sweet boy. :)

First Foods

Was finally given the green light to start Dean on puréed foods. Last night I tried to give him some rice cereal with formula. He wasn't happy. I was a bit bummed, but I read that babies with Barth have been know to have issues with food and feeding, and texture can play a huge role so I'm trying not to stress about it. A link to the article I was reading

So, since I was told that purées are a go (this is all very limited, he needs to eat this in addition to all his formula), we got him three different baby foods to try: carrots, peas, and apples. Last I read they still recommend giving vegetables first.

I tried him with the peas at lunch time. He did better with them then he did the cereal. He didn't do great, but he didn't act like I was trying to poison him.

Yesterday, i got a phone call from Leah, the metabolism nurse practitioner. We had questions for Dean's main cardiologist.

Question 1 was about the solid foods. We got the green light on the purées.

Question 2 was about Dean being banned from live vaccines. The issue when he was born was that there was a blip on his newborn screening that indicated an immune issue and that was what initially gave them reason to ban live vaccines. They plan on waiting until one year and then they will revisit it. The only live vaccine that he has missed was the rotavirus one. The MMR and Varicella are both live and both at a year.

Question 3 was about the supplements that metabolism wanted to give Dean. The cardiologist said that it was okay, so they are just waiting for labs to come back to make sure that he's okay to start them.

We saw both metabolism and cardiology this week. We got all labs done, which was nice because they have been having problems drawing his blood. No Echo this week, will have another one in 2 weeks.

We also saw the intake lady for the OT/PT and perhaps a speech therapist. It seems likely that Dean will get those services, which is nice. They'll come up with a plan and perhaps give us ways to get him up to developmental age. Depending on how he takes to food, he might need help with eating and swallowing, so they (along with the nutritionist that comes) will help us with that if needed.

I think that makes me up to date (with the exception of the facebook posts...was having issues downloading my FB profile).

Weight Gain

It's been rough this last month with Deans weight. The last two weeks, the home nurse has come and he hasn't gained any weight. Today, he's up 4ozs since last Tuesday!! I am so happy! I hope that this continues and that Dean might not need a feeding tube. :)

Metabolism, Nutrition, Genetics...oh my!

Off to Boston we went today!

We saw Metabolism first. The nurse said that he looks great, he has great muscle tone, his color is good, he's very social (especially to the young semi-attractive nurse that came along) and he looks like he's a good size )with the cheeks and chunky thighs! The doctor came in and we talked about supplements that they may start Dean on that could help-but definitely wouldn't hurt-but they need to talk with Cardiology first. So, nothing really new on that front.

Talked with their (metabolism's) nutritionist next. She gave us a scale so that we didn't have to measure out formula the way that we have been (1 cup, 1 Tbsp -or 17 Tbsp of formula). I've tried it since then and it's much more simple and straightforward. She said that we will talk about the transition to solid foods (or the start of it at least) at the next visit (at 6 months), but that we need to focus on getting more calories in him, which means we have to try to get him to eat more often.

The lady from Genetics came next, she didn't have much more to tell us. However, it seems as if Dean's mutation is not documented for Barth Syndrome, and that he was diagnosed mostly on symptoms. Tho, I admit I may have that mixed up. I guess that I'll need Jim to explain it to me again.

Hematology

We went and saw the hematologists today. We were there for awhile, not a long time with the doctors, it just took awhile.

No shots at the moment. He may be neutropenic, but he isn't having any issues of diseases, so there isn't a lot of concern at this moment.

Next appointment with them on May 11th.

Posting

I haven't gotten a chance to fill in the posts from Facebook. I'm trying to make a good effort in writing in this as I think it will be infinitely more helpful than Facebook in keeping track of all the nuanced stuff. Facebook is just not the forum for all of it. It isn't that I think my family would care if I post a bunch of detailed stuff, I just don't think that they'd care. It's silly. I have two days off and only Tuesday class this week so I'm going to make it my goal to get that done. :)

Off to work!

30 calorie formula

Dean is now on 30 calorie formula. Just got off the phone with the nutritionist. Have to mix 1cup, 2Tbps, 2tsp of formula (enfamil) with 2 1/2 tsp of vegetable oil (yea, that's right...crazy!) then fill to make 24ozs.....

February 15, 2012

Today we finally met with the metabolism doctors. Dean had two appointments today, one with metabolism and with the heart failure doctors. We had originally had an appointment with metabolism at the beginning of the month, but then Dean was seen inpatient and they didn't see the need to have us come back the day after Dean was discharged to see them, so they moved the appointment back.

The genetics tests for me came back and I am a carrier. I'm unbelievably torn about this. I know that it isn't my fault, but I still feel like it is. So, I just am trying to not think about it. Will have to get the girls tested.

Metabolism has set up an appointment with the genetic counselors so that this can be explained better and we can have all our questions answered.

Dean's weight today didn't follow the chart curve. If it continues along thus new path he will go further and further away from the normal curve. I think the NP said that he was in the .95 percentile for weight. They expect him to be small, or it won't be a surprise if he is, it seems as if many kids with Barth's are small. I told them that my big concern right now is when he transitions from formula to food. The NP seemed to understand my worries and said that they have a metabolism nutritionist but that she is a metabolism one not a cardiology one and wanted me to talk to the cardiologist about the issue first. We also talked about fortifying his formula (he's on a special 26 calorie recipe of enfamil right now) to bring up the calories because he has been sleeping through the night the last few nights so he's 1) not eating enough at a feeding and 2) missing a feeding now that he's sleeping through the night.

But, on the positive side, he is doing well developmentally, so I'll take that!

I went to the cardiologist and we talked about the nutrition and he didn't seem to think that there was a gigantic issue with the transition from formula to food, that most of his calories will be coming from formula until a year so that wasn't a huge deal. Also, he said that he would talk to the nutritionist that they talk to and let me know if they think that fortifying the formula would be for the best.

Then we talked about the genetics testing and he gave me what he things. The metabolism doctor said that he would wait until the girls turn 18, and he didn't think that they needed to be told anything, but cardiology said that he thought the sooner the better because if the girls aren't carriers then there wont need to be a discussion. I plan on talking to the geneticist then getting them tested for it.

We did our usual blood work and we went home.

After I got home, the NP called me and said that he blood work had come back and that Dean's thyroid is fine (there was a blip on the newborn screening about it) and that his neutrophil counts were low.

As I understand it, neutrophil are most common white blood cell (which fight off infections). Normal would be around 1,000. Concern would be if his was 500. His is actually at 50. That's right, FIFTY. So, he is at an extreme disadvantage if he gets sick. Because of this, we now have an appointment next week with hematology! :( they aren't TOO worried because he was so well in clinic, but they are worried.

It was a long day.