big belly

All continues to be well.
Here are two pictures of Seamus and his gigantic Buddha belly:

I'm tempted to post a picture of me when I was pregnant side by side to see who was bigger.  I think he's carrying a banana baby.  He ate a whole banana and a whole waffle this morning. 

Last week I had to take him to the doctor because he had a fever.  The nurse had him stand on the big kid scale to get weighed.  In the exam room, I started to cry as I explained that this was the first time a child of mine ever stood up to be weighed.  It was bittersweet. 

His favorite words are: Kitty, Doggy, Katie (his daycare provider), and Daddy.  He can say Mama, but it's not in heavy rotation.  He has learned to sign "more" and "horse".  He also likes to crawl on the floor and say, "Woof.  Woof."  He loves to give hugs and will frequently run to you just to hug you.

Eve is doing well.  We are still trying to get her formula sorted out.  We switched to the elemental formula, but it didn't have fiber and it messed up her BM schedule.  So, while we are waiting for the elemental formula with fiber to be approved by insurance she is back on the old formula and her poops are back to normal.  Her pump broke this morning, but within three hours the pharmacy had a replacement delivered.  Crisis averted.

I'm wondering if Eve will lose her last tooth (the other top front) by Christmas. Then I can sing "All I want for Christmas.." to her. 

trip to Boston Children's

Yesterday we made the trip to Boston Children's Hospital for Eve's Urodynamic study.  She was so well behaved and remained calm during the exam.  The nurses even told her she was the best dressed patient of the day.  :) 

The results showed normal functioning. There is no kidney reflux and her bladder fills and empties normally.  There was a little bit of over activity right before voiding, but the urologist was not concerned at all.  So, this is great news and we can cross urology off the list of things to worry about.  Of course, that doesn't explain the pain episodes (but it does tell us what it is not).  Speaking of, Eve had an episode at school on Tuesday morning.  It passed, but then she had another episode at home with the PCA.  Her legs were shaking and we couldn't get her to stop.  Although she wasn't moaning by the time I got home (after the PCA called me), she was very uncomfortable.  I gave her a dose of morphine and she immediately relaxed. 

morphine

I had to give Eve some morphine today.  Jim was at work and she started whining while I was feeding Seamus lunch.  I couldn't get a good answer out of her if she just didn't want to be in the kitchen She's been asking to watch TV when we eat lately.  She gags a lot while we eat.  I'm not sure if the food is making her nauseous.  Anyhow, at first I  couldn't tell if she was in pain.  She was clearly upset.  But then she started ramping up and it looked much more like her night episodes.  She started moaning.    I asked her if she needed medicine and she said yes. So I got the morphine out and she clearly didn't like the way it tasted, but in about 90 seconds she was totally relaxed and comfortable.  It was such a small dose, but it did the trick.  I put her down for her nap after that.  I checked on her to make sure her breathing was steady (it was) and she opened her eyes right away when I went in her room.  The rest of the afternoon went fine.  It was scary to have to give it to her, and it's upsetting to see her in pain.  It's been a while (maybe 7 weeks?) since she's had a pain episode, so we had  nice stretch there. 

As you may have guessed, things have been nice and quiet around here.  Eve is definitely on an upswing.  She's got two loose teeth that are just hanging on (at weird angles).  Incidentally, Seamus is getting two teeth in (his top two teeth - don't even get me started on how late they are!! He's 15 months!!!).  Eve is on an antispasmodal medicine to control the retching.  It's working, sort of.  She still retches here and there, but it's not nearly as bad as before.  She's doing better at school, too.

Seamus is a riot.  He's a total chunk and has no fear.  He climbs on everything and looks so proud of himself as he reaches the summit of Mount Couch.  He's been giving lots of hugs and kisses (and puckers up with little fish lips).  He keeps us laughing.

We're in a pretty good place.  We're enjoying the new van.  I'm relaxing a little bit which is really nice.  

Halloween 2012

Our new van is here!  I cannot explain how much this will change our lives (once we recover from the wallet shock).  I have a feeling I am going to wonder how we ever managed without it.  The van arrived on Friday and we took if for a family spin on Saturday morning.  It's a Dodge Grand Caravan with a side entry manual ramp.  We wanted a manual ramp (rather than an electric ramp and a kneeling van) because that is one less thing to break.  Considering the place we bought the van is 3 hours away, this was a good move.  Although I would have preferred a Honda or Toyota, Dodge is the only car make that has the side entry manual ramp.  We could have gotten a Toyota with a rear entry manual ramp, but we didn't like the configuration of seats with that conversion.  Oh, well. 

Here's what it looks like when you open the door:

Then you just pull down the ramp.

There are two rows where you can anchor her chair with the tie-downs.  There is also a seat belt connector.  You'll notice that the floor of the van is lowered (look at the base of the other slider door).  I want to figure out a better solution for protecting the floor of the van from dirty/slushy/ snowy wheelchair wheels.  It's currently a carpet with the pre-conversion floor mats.  I also have a seat cover on order for the back seat.  The color of the seats is just too light for Hurricane Seamus and Hairball Josie.  For now, a beach towel is doing the trick. 

Look out, world!  Here we come!

Eve and PaPa

Eve petting JoJo

Seamus and Grammie

Eve's new haircut

Seamus has begun hugging back.  He gives the cutest squeezes around the neck.  He's starting to say a few words that are almost totally unintelligible.  He says "doggie" and "din din" and today he said "Boo!"  He's a happy kid.  He's also incredibly curious.  He watches everything intently.  What a little sponge.  He'll be 15 months this week. 

We seem to be doing pretty well.

Last week, Eve continued to have issues with retching.  Last Sunday was particularly bad.  So, the GI sent us to Springfield to have a tube study done (radioactive dye through tubes to make sure they are functioning) and to meet with a pediatric surgeon to talk about a j-tube surgery.  The tube study showed everything working fine.  The surgeon said no to the surgery.  He said it should be a last resort thing and that it will open up a bunch of things that could go wrong.  He felt there were other studies that could be done and medications that could be prescribed before doing that surgery.  So, he sent us back to the GI.

Thursday we went back to Springfield to Shriner's Hospital.  We had made an appointment with the orthopedist to talk about her dislocated hips. Since we made the appointment (August), her hips have become the least of our concerns.  Since the way to fix this would be surgery, there is nothing we can really do.  The doctor and I agree that there is no sense in putting her through that extreme pain, especially when her comfort and quality of life is paramount for us.  That was a hard appointment to be at.  It's tough when a doctor just strokes your child's cheek and looks at her with sadness.

I finally was able to talk to the GI late Friday.  I asked him if we could reduce her feeding rate.  I truly believe that he was pushing her too much.  There's no sense in getting so many calories that she spends her days retching if her body can't handle it.  So we backed off the rate and so far so good.  She isn't retching (other than from too much mucous in the morning) and is gagging much less.  The next thing we are going to do is get her on an elemental formula.  It's a formula that is broken down and easier to digest.  The GI didn't think it would work, but thought there was no harm in trying it (it was my suggestion).  Finally, he wrote a prescription for an antispasmodic to help with the retching.  We will change one thing at a time, but I have the medication on hand if I need it.  If Eve does well with the elemental formula, we will try to ramp up the feeding rate a bit.

In the meantime, Eve had another one of those intense pain episodes last Wednesday night.  She woke up moaning and crying in pain.  It was so sad.  We gave her extra valium and it really didn't seem to help.  Eventually, it passed and she quietly moaned for the next hour or so.  She got more (scheduled) valium and slept for a bit and woke up screaming at 12:30am.  We called the Neurologist and he had us give her even more valium and some Tylenol.  We then talked about how our goal is to keep her comfortable.  So, the next time this happens, we are moving to morphine.  The neuro is having our pediatrician manage the morphine (we need to pick up the script in person and this way she can monitor Eve because she is local).  We met with her on Monday and talked about this.  So, for now, she will get the tiniest amount (short acting) as needed to help with these episodes.  Eventually, if things get really bad, she may be on a scheduled longer-acting dose. Eve has fine since the last episode. This seems to happen every 3-4 weeks. 

In other news, we should be getting our new wheelchair van at the end of the week (fingers crossed)!  This will be totally life changing.  Totally. 

So, I am feeling a bit more comfortable and stable.  Eve has been smiling and laughing.  Today she was in a parade at school dressed as Curious George and had a blast.  Her top right tooth is loose.

I keep meaning to post pictures, but the time slips away from me. 

Eve went to the GI last week for a follow-up.  She has put on 3lbs since her hospital stay!  However, she is still retching daily.  He wanted her to have another series of x-rays (which were normal) and he tried her on a new medicine to coat her stomach in case it is the tube from g-j that is irritating her. 
He said to give it about 3 days and then touch base with him.  In the meantime, Eve vomited at school on Friday and spent much of Sunday retching and gagging.  I actually called the on-call GI because I was so worried.  He called in a new Rx that seemed to help a bit.  This morning she gagged a bit and seemed to have a rough day at school.  Some possible pain issues/discomfort/more gagging and retching/and serious zoning out. 

I was able to talk to the GI today.  He said that obviously the first med from last week is not working, so we discontinue that.  He wants her to have a g-j tube study tomorrow (they insert dye in the tube to make sure it's working correctly).  He said that in his heart of hearts he believes this won't show anything.  But it needs to be done prior to the next step he is recommending - surgery to place a j-tube.  So, the g-tube will go back to being a mic-key button (held in place by a small balloon) so we can give her meds through her stomach.  The j-tube will now be surgically placed right into her intestines (rather than have a tube snake through her stomach).  We are also going for a surgery consult tomorrow. 

I do not believe that is the problem, but I am totally willing to explore all options.  While I do not look forward to subjecting our child to surgery, there is a possibility it could help her.  I'd hate to get to a point where I wondered if we did everything we could to make her comfortable.  I don't know where we will go from here if this doesn't work.  I really don't know. 

In the meantime, we are also headed to Shriner's hospital this week to have her hips looked at.  This appointment was made months ago when her hips and legs seemed like her biggest problem.  I can't explain what it's like to have your worry pulled in so many different directions. 

Eve has been doing pretty well.  We've had some constipation, and some night time wakings, but nothing crazy.  We go for her followup GI appointment tomorrow.

I can definitely notice the difference that nutrition makes.  She's regained the energy to whine and her hair isn't falling out as much.  :)

Grammie and PaPa are here visiting until tomorrow.  After they leave I'll post some pictures.  The kids are having a ball with their grandparents.  Eve chose hanging out with PaPa over helping mommy bake cupcakes.  I can't say I blame her.  She sees me all the time. 

Monday night was a really rough night.  Eve was up at 8:30pm in intense pain.  Her knees were drawn up to her chest, her fists were clenched and her arms with tight at her sides with elbows bent.  She was crying out and having lots of tongue thrusts.  When Jim picked her up, it was like picking up a stone statue - she didn't move.  It was too early for more valium, so we didn't know what to do.  I called the Pediatric Palliative care program and the VNA sent a nurse out.  This pain episode lasted about 10 minutes before she finally relaxed.  The nurse checked her vitals and we talked to the pediatrician on call.  He wanted us to consider going to Baystate, but left the decision up to us.  While the nurse was there, Eve had a second episode, but it wasn't as bad as the first and only lasted 2-3 minutes.  When the nurse left, I packed a bag just in case things didn't get any better and figured I'd give Eve valium when she was due (11pm).  At 10:45, she woke up with another episode.  I gave her the Valium a little early and she relaxed after that and slept the rest of the night. 

I put in a call to the neurologist yesterday to talk about it.  It turns out there is a neuro on call 24/7.  Next time I will remember that.  Anyhow, the doctor told me that people on Valium can develop a tolerance.  He's been very conservative with her dose, and told me to keep the dose as it is and to give her an additional 1ml if needed in a situation like that.  If it keeps happening, we can talk about changing her maintenance dose.  We have a long way to go before we reach her max dosage for her size.  Right now she gets less than a third of the max dose.  Keeping her comfortable is a common objective. 

Eve did well this weekend.  No gagging.  Perhaps moving the prilosec worked.  Yesterday I increased her feeding rate to 52ml/hour.

This morning the school nurse called twice.   Eve was retching at school twice.  The first time with much effort and she got sweaty and brought up some saliva.  The second time wasn't as bad.  I had the nurse pause her feedings for 2 hours to give her a break.  I put in a call to the GI and am waiting to hear back.

I just don't know what to do.  I also am afraid to get comfortable.  It seems as though every time I think, "Ok, we are stable.  Things are quiet and going well.  I can breathe and start to plan for future stuff," she has another episode or setback.  And I feel like such an idiot calling the GI office.  They are probably so sick of me calling.  It's just that vomiting for Eve is not the same as for a regular kid.  It could be indicative of a much more serious problem. 

Update:
GI called back.  He wants to increase the Prilosec to twice a day (morning and night) and see what that does.  I am to call on Thursday.  I am also to keep giving her the feeds and keep it at the 52ml.  I hope this works.

When Eve woke up yesterday she was doing alright.  But when  I gave her Prilosec,  she started gagging and thrusting her tongue out.  Her brow wrinkled and she looked uncomfortable.  So, I gave her Zofran again which seemed to help.  I waited about 20 minutes and gave her the rest of her meds and then sent her to school.  She did well at school. 

The GI called at the end of the afternoon while I was in the grocery store (this always seems to happen!).  He said that the x-ray showed that the tube was in place and that there was a little bit of constipation.  Because she is not retching all of the time, he said he's not comfortable removing the gj tube yet (I didn't know that was on the table).  His recommendation for now is to give her the Prilosec in early afternoon rather than first thing in the morning.  I asked him if she can still have the valium and Prozac in the morning and he said yes.  That doesn't make sense to me, but whatever. 

At 5:30am this morning, Eve woke up.  SHe was doing tongue thrusts and looked uncomfortable.  She emphatically told me she didn't want her leg medicine (Valium).  She said she did want her diaper changed (which was just wet).  She rested after that and was quiet until 7:30. 

So far this morning I have given her the Valium and Prozac and haven't seen any gagging.  Her feeds are going fine.  I'll give her the Prilosec at 1pm. 

Oh, and I called the salesman to get started on the van purchase.  :)

Here are a few pictures to balance the information that I've been posting lately.

Seamus decided to help with the laundry and climbed into the basket by himself.  Thanks, son. 

While Eve was in the hospital, her classmates made her some wonderful drawings.  Eve and Jim hung them up in her room so she can admire them every day. 

Tonight, Seamus was being silly and walking around with a bucket on his head. 

He's also very good at Downward Dog.  A little yogi in the making.  Can you see that belly?  Oh, my. 

I talked to the GI.  He sent us for an xray this afternoon to see if the tube slipped out of place.  I doubt it, because then she would have vomited formula rather than just foamy saliva. 

I gave her Zofran when she got home from school and it seems to have helped with the gagging.  We'll see how tonight goes. 

I'm supposed to touch base with the doctor tomorrow morning. 

You know, for as hard as things are right now, I still feel like things have been working out.  I still can't get over our luck that my mom was in town when we had to go to the hospital in Springfield.  Today, we got to the x-ray department and were told there was a very long wait because they were getting hit from the ER.  I was worried we wouldn't be able to get the x-ray before they closed.  The next thing I knew, the tech came out and said she was going to sneak us in between patients.  She remembered us from last time and remembered how good Eve was.  We were in and out in less than 3 minutes (no joke).  Because we were being fit in, I didn't ask to see the image to see for myself if the tube was out of place.  I was just so grateful. 

I just got off the phone with Eve's mito doctor.  He said to look at this decline from two perspectives.
1. Because she wasn't getting the proper nutrition (maybe because she wasn't able to eat well due to her muscle weakness), we saw a rapid decline in gut function.  Once she gets the proper nutrition, she may bounce back to a degree and regain some functioning/skills. 
2. This decline may be neurologically based and evidence of disease progression.  If that's the case, there is nothing we can do other than to make her comfortable.  He confirmed that this is a serious situation.

I reminded him that never in her life has she regained any skills that she has lost.  I am not trying to be pessimistic, but rather realistic.  He suggested that eve see one of three motility doctors in Boston.  I'm going to call the GI doc today and tell him about this conversation to see if he can facilitate a quick appointment.  A motility doctor can prescribe a medication that can improve motility in upper or lower gut.  This can help with her nutrition in scenario 1.  He also said that we could put her in the hospital for a week on IV nutrition.  If we see an improvement in her functioning (i.e., if she becomes the kid she was before this decline and less disconnected), then we know it's a nutrition based decline.  If there is no change in her, then we know this is disease progression.  He felt this would save us months and months of intervention and would give us a quick answer.  I'm going to talk to the GI and see what he thinks.  I think I'm not ready for another hospital stay.  I asked the mito doc if we could see how the j-tube feeds go a little longer.  He said that's fine.  If we can get her up to her optimal intake (we are 80% right now), then that would be the same thing. 

If we find that this is a neurologically based decline, we would put her on the j-tube nutrition that she can tolerate.

I just got a call from the school nurse.  Eve has been retching all morning.  I had her stop the j-tube feeds and flush the tube.  I have a call in to the GI and am waiting for him to call me back. 

The past two nights have went well.  Eve is tolerating her feedings.  We've also changed her sleeping position. I bought a wedge pillow and placed it under the mattress.  She now sleeps on her back, with blankets and pillows keeping her from falling over to the sides. The incline seems to be comfortable for her. 
She's been getting 50ml an hour of formula for 20 hours and then gets a 4 hour break before we start it over.  Soon, we will work up to 60ml so that she gets the calories she needs.  I bought a backpack made especially for the pump so it can go to school with her (the pump was on a huge IV pole).  It's pretty cool. 

We're both happy to be home. 

Going home!

Eve has been tolerating her j tube feeds very well. She has been on the max rate (50ml per hour) for 13 hours.  We are still hopeful we will go home today. But, that might mean tonight.  She does have her IV unhooked.

We are patient patients.

Jim is on his way to spend the night so I can go home, shower, and sleep in my own bed tonight.  I'm also anxious to see Seamus and my mom.

The plan is to work up to 50 ml an hour of formula through the j tube and see how she tolerates it.  If all goes well we hope to be sent home tomorrow. Eve is doing very well right now.

We're up to 20ml per hour and waiting to hear what the final feeding schedule will be.

It's done and it went well. Eve is resting comfortably and they just started the feeding in the j port at 5ml per hour. Now we wait for further instructions.  She's a little loopy from the drugs, but doing well.

Update from head doc of PICU: it's going well, but they had a little trouble.  I think they're back on track.  Will update as I know more,

Eve is having the gj tube placed right now. She is under sedation. It shouldn't take too long. With any luck we can go home tomorrow.

We've had a very long day of waiting around. During that time Eve was able to check in on her classroom via FaceTime.  It was so fun to see and hear her teachers and classmates. Technology is amazing.

Also, I want to thank all of you for the kind wishes and offers of help. I feel so fortunate. We were supposed to be in Boston  today for Eve's urodynamic testing, so my mom is visiting to help with Seamus.  What amazing luck that the day she came up to help was the day we were sent to the hospital.

So, it looks like we will be here for the rest of the week. They don't have a low profile gj tube that fits her, and that would take several days to get in (during which time we would need to figure something else out like a n-j tube). Instead of waiting, they are doing a non low profile tube. I'm not exactly sure what that will look like. When she gets the tube in, she will be running on continuous feeds around the clock. If all goes well, we are hoping to have the tube placed tomorrow and to be able to go home Friday. Otherwise, they will place it Friday and we'll go home Saturday.  That of course assumes that the placement  goes well and the tube works for Eve.

The dr has also ordered food for her though the gtube tonight until midnight. They'll start very very slow just to get something in her.  They will stop at midnight to be prepared for the possible placement of the tube tomorrow. But at least she'll get a little bit of something. It's been 42 hours at this point since she last had something.

The neurologist also stopped by today.  It was so great to see him because he was just checking in on her. He examined her and spent time talking with me. He confirmed that this is a very serious time for Eve.  The shutting down of her GI system is scary stuff.

On a happy note, I heard today that we were approved for the loan for the wheelchair van. Once we figure out what's going on with Eve we'll move on to that.

At Baystate

Eve and I are at Baystate hospital.

The nurse called from school today. Eve was gagging and spit up a bit, and it apparently smelled like fecal matter. We called the pediatrician who was more concerned that eve had only had one can of formula in the past 24 hours. She called the GI and after a little back and forth, they decided to have her admitted at Baystate hospital in Springfield (1.5 hours from where we live). Soooo...we packed a bag (or two....you never know) and headed here. We were in the ER for about 4 hours waiting for a room. While we were there Eve got another X-ray of her abdomen and a quick bag of fluids.
It's wierd explaining our situation. Some of the ER docs were talking about looking f or obstruction or infection to look for a cause for eve not tolerating her feeds. I finally looked at one young dr and told her, "I don't expect there to be infection. This is what's happening to her because of the mito. Her systems are shutting down until she dies.". I didn't mean to be harsh, but I had lost patience.  So, now we are in a room and trying to rest. Eve is squirming in bed. I don't expect to get much sleep tonight.

Tomorrow we will see the GI. I'm hopeful that we will get a gj tube during our stay. I'll keep updating the blog. Feel free to call. I have my cell.

Eve had her 7 year well visit yesterday.  She's lost another pound in the last 2 weeks.  She's down to 36.6 from 41 a year ago.

Yesterday at school, the nurse couldn't get Eve's whole feed in.  She started gagging on the last 2oz.  They tried again an hour and a half later, and those 2oz wouldn't go in (via gravity feed).  Oh, and there was still formula in her stomach when they started the whole process from 5.5 hours earlier.

Last night Eve woke up retching at 12:30am.  We stopped the pump and got her calmed down.  She was able to sleep in her bed for the rest of the night.  I didn't turn the pump back on.  That is such a tough decision....stop giving her food that she needs or give it to her so she can vomit it up and be unable to sleep in her bed because of constant retching?  So, she only got about 1 can of formula.  We're just sick with worry.

The pediatrician is going to call the GI to talk to him about the situation.  I told her that no one seems to feel the same sense of urgency that I do.  Eve is just so lethargic and her hair is falling out.  I'll also talk to the pediatric palliative care nurse today. 

On a positive note, Eve is still able to laugh.  A lot.  She actually made Jim mad on Sunday.  He was installing a new front door and ran into a snag.  She laughed her butt off at him.  Gave herself the hiccups.

grrrr

Lesson learned:  Don't worry about being a pain.  Follow up!!!

I hadn't heard anything about the wheelchair van loan application I faxed in 2 weeks ago, so I called this morning.  They never got it!!!! 

I should have called to confirm receipt.  Grrrrrr.....

Oh, well.  I'll try again this afternoon.

Eve is still hanging in there.  She had a good night last night.  I was able to run the pump as planned (I've been having to shut it off early lately because of gagging). 

We're hanging in there.  We've been continuing the 3 hours on, 1 hour off feedings throughout the night.  This morning, Eve was up at 5am this morning gagging and crying out.  We gave her some valium and changed her diaper, but she was still gagging after that.  So I got her up and put her in her chair and here we sit.

Yesterday the GI nurse called me to check on the size of Eve's mickey button (current g tube).  She needed to know as part of the process of setting up the g-j placement.  I asked her about the process and she said it might take a few weeks to set it up.  I reminded her that Eve is eating very little (half of the calories/nutrition that the doc wants her to).  She made a note and will tell the doctor.  If he decides Eve can't sustain functioning on that level, I guess he'll make it happen sooner.  As it is, she is so very lethargic.  I would be too if I was getting 600 calories a day.  Granted, her calorie needs are much fewer than mine due to her size and lack of mobility.

We only got through about a can and a quarter of formula last night.  She woke up gagging at 3am and I immediately shut off the pump.  I also had to give her more Valium because she was cramping up and in pain.  We haven't seen that at night in a while....really since we've been doing the Valium every 4 hours during the day. She rested comfortably after that.

Eve had a better night last night and rested comfortably.  Jim and I took turns getting up to turn on and off the pump. 

The GI called late this afternoon.  The x ray did not show any constipation.  So that means she is not tolerating her food.  He said it's time for a gj tube.  So his staff will work on scheduling that and it will have to be done at Baystate. 

I hate being right sometimes. 

more x rays

I talked with the GI doc this afternoon.  He sent us to the hospital for more x rays.  I didn't realize this, but just because Eve is having diarrhea doesn't mean she isn't still impacted.  She can be still corked up (and not entirely cleaned out...things can sneak by the cork) and that can make her not tolerate her food.  So, he'll get the x ray in the morning.  In the meantime, we need to run her pump for three hours on and one hour off overnight tonight to give her system periods of rest.  So, I'll run it from 6:30-9:30pm and shut it off from 9:30-10:30.  Then turn it on until 1:30, etc. 

As for her not tolerating her food, he wants to see if it's 'a' problem and not 'the' problem.  In otherwords, is there constipation causing her to retch, or is her system shutting down and not tolerating food (which is my fear). 

I'm really struggling with this.  The worry is a constant weight. 

Here's hoping we have a good night tonight. 

So much for doing well.
I wrote too soon.
Last night Eve was up at about 3:30 gagging and retching.  I stopped her feeding pump (she had gotten about 2/3 of the bag).  We changed her diaper and comforted her.  I pulled out the contents of her stomach and she had about 1.5 ounces of formula in there.  I left it out.  We tried to go back to bed, but then she was heaving with her whole body convulsing.  Nothing but a lot of saliva came up (I had emptied her stomach).  She wasn't able to lay down after that because she would retch every time.  So, we put her in her wheelchair and brought her into the living room.  Jim slept on the couch next to her and she dosed a bit.  She did much better sitting up.  She's still a little gaggy, but doing much better.  I"m going to send a can of formula in to school with strict instructions to go very slow.  I'll also put in a call to the GI.  I'm wondering about an anti-nausea medicine and when is it time to look into a gj tube. 
I also need to call the mito doc and update him on Eve's regression. 

Fortunately, the pediatric palliative care nurse is coming over this afternoon. 

Eve has done pretty well on the feeding pump.  We got her system cleared out with laxatives and now she is on a maintenance dose.  I still need to fiddle with it because her poops are not solid yet.  I've been working up to the recommended amount of formula in her tube.  Right now she gets most of it overnight (we run the pump from 6pm to 7am) and this weekend she got another can of formula through the pump from 11am-1pm.  I need to figure out a solution for school.  I"m planning on having Eve be on nearly continuous feeds, but we have to get her pump to school.  Right now it's on a big honking IV pole.  They make little backpacks for pumps, but I have to look into that.  I'm not sure if I can use a regular backpack or if it has to be made to fit the pump (to position things a certain way).  I'll figure something out so Eve can just bring her pump to school with her.  Then we can have a few hours off in the afternoon to let her system rest before starting it all over again. 

She's doing pretty well.  The gagging stopped.  She's on Valium every 4 hours while she's awake and that is really making a difference with her leg cramping up.  She's my little trooper and I love her. 

after a trip to the ER, Eve is better

Eve had a quiet night last night.  When she woke up, I gave her about 20ml of pedialyte and she started gagging.  So, I took her to the doctor in the morning.  Her temp was down and her extremities were quite cold and mottled.  The doctor listened to her stomach and gut and said that her intestines were remarkably quiet.  So, she sent Eve to the hospital for bloodwork and xrays of the chest and abdomen.  Bloodwork came back just fine.  No sign of infection, and all other things looked normal.  The xray didn't show any blockage, but did show that her intestines were full of poop.  Top to bottom.  (How many people get to say their kid is full of shit?) The Dr. felt that this may explain her intolerance to the feedings (by making her feel very full).  So, she called in a script for a glycerin suppository and sent us to the ER for IV fluids. 

We were at the ER for  5 hours.  Ug.  But Eve was a total angel.  She was so quiet and good.  She just chilled out in her wheelchair and even dozed a little.  The put in the IV and took a urine sample to test for a UTI (which she doesn't have).  That process was difficult.  It took 4 attempts to get the catheter in.  Eve was a trooper.  Finally, they gave her 20ml of water through her g-tube to see if she would throw it up. She didn't, so we were allowed to go home. 

I  gave her about an ounce of formula after dinner and it seemed to go down just fine.  But I stopped there.  Tomorrow morning, I'll give her formula very very slowly.  I'll send her to school with just one can of formula (rather than two) and tell them to give it to her very very slowly in the middle of the day.  If she starts to retch or gag, then the feeding is over.  We'll see how it goes.  I'm also calling the GI first thing in the morning to ask for a pump.  If she has a feeding pump, we can give her all of her fluids slowly during the 12 hours she's in bed.  I think it will be much better to have a slow continuous feed rather than dumping large amounts into her stomach 5 times a day. 

So, it was a rough day, but I think things are looking up.  Oh, and the suppository was successful when we got home and she got a bit of poop out.  However, I know there's a ton more in there!  I'm concerned that her intestines aren't working as well as they should, but I think we can manage that with meds if it becomes a problem. 
We're hanging in there.

Eve is sick

On Friday, Eve was sent home from school because she threw up most of her feed and had a low grade fever (more on that later).  For the rest of the day, I slowly gave her a few more ounces of formula and she did fine.  Saturday, I got in four feeds and did them slowly, but she was fine.  Today, I got in her morning feed and that was it.  When I tried to give her the next feed, she immediately started gagging.  I tried to push through just a very little bit at a time, but I stopped after about an ounce.  I called the on call nurse through the pediatric palliative care program and she said to try to give 2 oz of formula very slowly and see if she keeps it down.  We put Eve down for a nap first.  I woke her at 3pm and we tried to give her the little bit of formula slowly, but she started retching and we only got an ounce in.  I called the nurse again and she came over.  She checked Eve's vitals and listened to her stomach and bowels.  Eve continued retching all afternoon even though she wasn't being given anything.  She never actually vomited, but that's probably because there was nothing left in her stomach (the nurse checked by pulling out the stomach contents through the tube). 

So, when Seamus went to bed, we gave Eve about 10ml of pedialyte with her Valium.  After about 25 minute, we put her to bed.  She so far has kept it down (it's only 2 tsp) and has been pretty quiet in her bed.  The nurse called the on-call pediatrician who said that if Eve started vomiting bile or just vomiting a few times, or if she has stomach pains that we need to take her to the ER.  If we weren't able to get in a decent amount of pedialyte (we weren't) we need to call the office first thing in the morning and have her seen by a doctor.  So, that's what we are going to do.  I'm cautiously optimistic that tonight will go somewhat smoothly, but I have some things together in case I need to make an ER trip. 
I'm worried and scared.  I don't know if this is a virus or further decline in functioning.  I need to focus on the problem at hand and stop letting my mind jump to future problems. 

Seamus is walking!!

first steps

Seamus has begun taking his first steps.  His record is about 4 steps at my parents' house.  He is definitely not walking yet, but is on the cusp!

endoscopy

Today, Eve had her endoscopy at Baystate.  It went very smoothly.  I was amazed at how well Eve did.  She was great in the car and great at the hospital.  Have I mentioned how wonderful Prozac is?  It has changed our lives.  Eve was soooo much more relaxed and not scared.  The anesthesiologist said she did better than the 13 year old before her.    Anyhow, the results showed that there is no anatomical reason for her to stop eating (as expected).  She does have mild chronic GERD (no surprise) and her g-tube was placed in a perfect location (Also no surprise.  Go, Dr. Saripkin and Atlanta Children's Hospital!).  So, she can go back on Prilosec and we are going to look into overnight feeds using a pump.  Speaking of feeds, we are still waiting for insurance to approve the formula.  I've been paying out of pocket and it's getting old. 

still knocked out

waking up

The staff at Baystate was great.  I had them laughing in the procedure room.  They let me go in with Eve as she was put to sleep with the gas mask.  This was the only time she was upset.  She looked so pitiful and was crying underneath the mask.  But her little cries were muffled by the mask and started to get weak as she drifted off and her eyes rolled back.  I started laughing at her and apparently that is not the usual reaction of a parent.  Oh well.  It was funny (and I may have been nervous).  Later, the anesthesiologist said that it seemed as though I was seeing the big picture and she wished more parents had that attitude. 

Eve came out of anesthesia a little rough.  She had trouble clearing her airway a bit and was slow to wake up.  She also was trembling quite a bit.  She finally seemed to get it together when I put her in her wheelchair and put her on the phone with Jim.  She was an angel for the rest of the day. 

So, one procedure down, and another to go.  The next one is in Boston next month.  Eve is having full urodynamic testing done.  That one will be a big deal and Jim and I will go together. 

 Two weekends ago, we went on a newt hunt at The Clark.  Eve had made a bug hut at summer school and the newts were plentiful.  We lost count after we got to 20.   

We've been researching wheelchair vans because it's definitely time to get one.  During our research we found out we could rent a wheelchair van.  It had been 1.5 years since we've been to NJ, so we took a trip last weekend.   We couldn't have done it without the van.  It was great.  We saw my parents' new house and even escaped for a dinner date.  Jim's brother also made it up to see us. 

Oma and Seamus

Eve and Scott

The trip was a whirlwind - about 48 hours in total, but it was great.  We're looking forward to future, longer trips, during which we can visit with more people.

As for the van, I'm making progress.  We've decided what kind of van we want (and don't want).  We don't want the fanciest conversion.  The most expensive conversion (roughly 22K) involves and automatic ramp that either folds out or slides out from underneath the floor.  The whole van then kneels to reduce the angle of the ramp.  Fancy, but totally unnecessary for us.  Eve will never have to push her self up the ramp, so there is no need for those features.  More importantly, those features are something that will break and then we'll be up the proverbial creek since the dealer that we will purchase from is 2.5 hours away.  I say will because even our rental had problems with the ramp and kneeling.  No thank you.  So the van that we are planning to get is called a Braun Companion Van.  This is a side entry van with a manual ramp.   It suits us perfectly.  I'm now working on funding.  I think it's going to work pretty well.  Without getting into too many details, we will apply for a low interest loan through Easter Seals (specifically for wheelchair van purchases).  Then we will pursue funding from the Knights of Columbus and the Catastrophic Illness in Children Fund.  Between that and the sale of my CRV, we should have the van nearly paid for (if not totally paid for).  This is exciting.  Ever since last weekend, I've been dreaming of the new van.  I keep thinking how much easier life will be.  It was like tasting the forbidden fruit!  I didn't know it existed, and now I can't imagine living without it. 

Seamus has been a real sweetheart lately.  He will still body slam you, but he also likes to give kisses and hugs.  His hugs are literal face plants on your chest, but the sentiment is there.  Kisses are adorable, but disgusting.  He puckers his lips and presses them against yours.  The problem is, you get smeared with drool.  It's worth the price. 

Giving JoJo love

Eve has found a nice comfortable resting place on the couch.  Doesn't she look comfortable?? 

We've been pretty worried about her lately.  She barely eats anymore and we're waiting for insurance to approve the formula for her gtube.  In the meantime, we've been giving her Carnation Instant Breakfast through her tube when we don't think she's eaten even close to enough (which has been 4 of the last 5 meals).  I'm going to call the GI tomorrow to ask what we should do.  I bet there is ensure or some other formula in the grocery store.  Thank God for the gtube, or this kid would be starving to death.  Literally.

Also, do you notice the small dot on the side of her right knee?  You might need to click on the picture to blow it up. That's a pressure sore.  She can't move around during the night and consequently is at risk for these sores (from legs pressing on the bed or each other).  This one showed up and has lasted a few days (it's still there, but is much better).  Since then we've been placing a folded up blanked in between her legs to prevent more.  She's been waking nearly every night (5 of the last 6) needing at least one dose of muscle relaxer because one leg or the other is jacked up and stuck.  I think Jim and I are pretty stunned by her decline.

She had a hip xray on Friday and we compared it to the xrays taken last year at Shriner's.  Now the right hip is dislocated as well as the left. It doesn't seem to be bad enough to require surgery, though.  So, we seem to have left our plateau with her health.  I just hope we land on another one.

1 year well visit

Seamus had his 1 year well visit today.  He's doing great.  22 1/2 pounds (65th percentile) and 30 3/4 inches long (80th percentile). 

We took away his bottles last week and he now drinks from sippy cups exclusively.  He's also drinking whole milk. Sometimes he fools around and takes big mouthfuls of milk and then lets it run out of his mouth onto his bib.  That's my cue to remove the cup.  When he's thirsty, he'll drink.  When he's not, he'll play. 

He loves finger food and feeding himself.  He continues to become more steady on his feet.  Right now both kids are playing together.  Eve is in her wheelchair touching his head and shoulders and he stands at her side, holding onto her tray.  It's sweet. 

Seamus is 1!

Seamus had his first birthday this week.  I just can't believe it.  This year has gone by so very quickly.  Here are some birthday and bath shots.