Home Again

Peyton was able to come home from the hospital yesterday {Friday}.  There wasn't really a whole lot more that could be done at that point other than to make sure that she got her g-j tube changed out.  This is something that has to be done every 10 weeks.  She was due to have it changed next week, but we were able to have this done before leaving the hospital.  It was just easier to do it while we were already there.  She was discharged from the unit and then we went to radiology to have her tube changed. From there, we went home.

She's home, armed with a new pain medication {Naproxen} in addition to an increase in her Neurontin doses and her usual Oxycodone dose.  I'm not sure why her pain is so much worse these past few days, but it is intense and she needs to get that under control.  I can't imagine the pain.

Peyton has done well at home so far.  I've managed to get her on bipap tonight.  She really needs to be using it.  It's sometimes difficult to get her to tolerate the mask, but it really is for the best that she wear it.  She's getting oxygen in addition to the bipap.  I think she has finally settled for the night, but it was a little bit of a rocky start.  Hopefully she will sleep through the night.

Her feeding schedule has changed a bit.  The nutritionist in the hospital recommended an increase in the volume of Pediasure she gets each day.  We were on a good 24 hour schedule, but since she came home, the time one feed ends and the next begins is a little later than I'd like.  You hang a bag and it runs for 24 hours.  We had been changing it around 8 or 9am but now it's late in the evening, so I'm debating staying up late versus setting an alarm to get myself up to change the feed.  

I am fairly exhausted right now.  I could barely move off the couch today.  Unfortunately, people needed clothes, so I eventually had to do some laundry.  I don't know that there is a way for you to fully appreciate the level of emotional and physical exhaustion I am feeling right now.  I've you are or have ever been a caregiver for a sick family member, you probably have an idea.  We have our home nursing, so that is good.

Hopefully Peyton's pain gets under control soon so that she can at least be comfortable again.  I don't anticipate her need for oxygen while she's awake to change at this point.  I think that this is a new "normal' for her.  I would like to see that change, but only time will tell.  Today was a decent day, though.  All things being what they are - it was a decent day.  

Back to the Hospital

We thought we were in the clear when it seemed that Peyton did not get that nasty GI bug that Moira, Ron and myself all got.  Yet this morning, we wound up taking Peyton to the ER.  Not with a GI bug, but yet another respiratory ailment.

I've spoken several times lately about how Peyton is requiring oxygen during the day while awake - something that never used to be the case.  This has not changed for the past several weeks.  However, over the past couple of days, the amount of oxygen she is requiring during the day has increased.  She has also been a little on the junky side, with increased heart rate and the odd low grade fever here and there.  I've been in touch with the Pulmonary clinic about her condition and it was suggested that perhaps we might need to start a course of IV antibiotics.  We can manage this at home since she has a port.  By the time last night rolled around, I was thinking I'd definitely make the call to the pulmonary clinic to suggest that we get that started.  Peyton was sounding horrible and it didn't look like we needed to wait on this any longer.

Then this morning came around.  Peyton was hot to the touch so I undressed her and got a temp on her.  She had a temp of 100.8.  That may not seem like a raging fever, but you have to bear in mind that her "normal" is upper 96 degrees to mid 97 degrees.  And, of course, we're ultra cautious anyway, so 100.8 isn't anything to mess around with.  In addition, she sounded even worse and she just looked "off".  I made up my mind that we were going to have to take her to the ER, but I also knew we'd have to call the home nursing agency pretty quickly to call off her nurse for today.  She has transportation issues, so we would have to be sure to catch her before she left, as she has someone bringing her to work.  No sooner did I form that thought did the agency call me to say that she had called in sick so I guess for today it all worked out.  Ron got Moira on the bus to school and I got Peyton and her gear ready to go and we headed off to the children's ER at MUSC.

Ron dropped us off at the door and then went to park the car.  I went in with Peyton and we were taken back to a room pretty quickly.  Once in a room, it was mere minutes before a doctor was in the room.  It seemed almost immediate.  While we did bring her in because she was obviously sick, apparently she was sicker than we realized.  The doctor enacted their "rapid response" protocol immediately based on their assessment.  This meant that her port was accessed within minutes and a bolus of fluids was pushed through very quickly.  They gave her additional fluids shortly afterwards.  Antibiotics were ordered and given fairly quickly as well.  A second antibiotic was given not long after that.

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A lot of blood was drawn for labs and a chest x-ray was also done.  The x-ray doesn't look too bad.  We're still waiting on the blood work results.  The doctor was in several times.  We knew pretty early on that Peyton was going to be admitted to the hospital, but there was some concern over whether it would be to the PICU or to the PICU step down unit.  They eventually decided on the step down unit.  With the fluids and oxygen, Peyton's color and mood started to improve a little.  Her heart rate finally came down out of the 150s.

We didn't spend a horribly long time in the ER before Peyton was taken to a room.  She is under the care of the PICU doctors, as opposed to the general peds team.  She wasn't in the room long when her Neurologist came by.  He just happened to notice in their computer system that Peyton was in, so he came by to see what that was all about.  I was glad, since I was actually going to email him to let him know.  We discussed the lumbar puncture and other research type issues that are hanging out there for Boston.  He is thinking that if she is doing ok, he may be able to do the lumbar puncture on Thursday.  

In addition, he is talking about contacting the researchers {Children's Hospital Boston and NIH} to see what we can do about expediting some of the stuff that's hanging out there in the hopes that we can get her some form of treatment.  Of course, it's research and there are FDA guidelines and I'm sure other sorts of hurdles to cross, but he is hoping that somehow he might be able to take some of that from them and perform whatever needs to be performed right here at home rather than have her travel.  He is looking at it from the point of view of being "emergency and compassionate" reasons.  We will see what happens.  I would say that if someone said we were needed up in Boston in the next month, we probably wouldn't be going.  She needs to have all this stuff done, but it's getting to the point where it just isn't going to be feasible because of her medically fragile state.

We aren't sure what is going on.  There is the great possibility that what is {and has been} happening are not so much an illness as they are signs of her overall condition diminishing.  As the PICU doctor said, often children with severe issues like Peyton, over time, will become weakened in the areas involving swallowing, breathing, and so on and what appears to be an illness might actually be more of an inability to perform these functions well.  We just don't know.  I would suspect, though, that because of her increased oxygen requirement during the day that it is the latter.

In addition to all of this, Peyton's level of pain today must be excruciating for her.  She is on Neurontin and Oxycodone for pain, but it seems like that's not doing anything.  I noticed it first thing this morning.  I barely touched her leg and she began screaming out in pain.  I don't know what is different about today but it is unbearable for her to be touched or moved.

We pray.  We pray often and we pray hard for Peyton.  The problem is that we don't know which direction our prayers are supposed to take.  It is heartbreaking to see your child enduring so much.  You wonder how much a body can take.  In addition, this isn't something we've really talked about openly, but we're being asked questions that no parent should have to answer for their child regarding the "what if" scenarios.  As much as you are praying for Peyton, please pray for us that we will have the wisdom to make the best decisions for her.  Please feel free to share this site to people you know would be willing to pray for Peyton. She needs many prayers!!

Thank you!

Happy New Year!

As 2012 draws to a close, I wanted to give you a little update on how Peyton is doing.

The past few weeks have been very difficult for her.  She has been suffering increasingly intense levels of pain with her hip dysplasia.  Her left hip, I can only imagine, is unbearably painful at times.  She is on Neurontin three times a day and Oxycodone for breakthrough pain.  Even with having the dosage of the Neurontin increased within the past week or two, she is still in incredible pain.  Sitting in her wheelchair can be quite painful for her.  When she is laying down, she draws her left leg up towards her body and then across and over to the right.  It's dislocated when it's in that position, yet it seems like that is her position of choice.  There must be some degree of comfort in that position.  However, it makes diaper and clothing changes very difficult.  She has been in her "Rhino brace" {note: if you click on that link, it's the "cruiser" that she is in} far more frequently, including while she is sleeping.  It isn't going to fix her hip dysplasia.  It is meant for hopefully providing some level of comfort for her.  

It is absolutely heartbreaking to see your child in so much pain when you know that you are doing everything you can, but it's not enough.  If only she could tell me what she was going through.  Having a non-verbal child is incredibly challenging as it is, but when the child is sick or in pain, it's worse because they can't tell you exactly what is wrong or what helps or hurts.  I just want to fix this and make her pain go away!

On top of this, for the past few days, Peyton has been having difficulty maintaining her oxygen levels.  She is on a monitor constantly, so we are always aware of what percentage her oxygen level is at.  It should be over 94% but has been in the low 90s and even dipping into the 80s.  To help with that, she has spent the last three or four days on oxygen via nasal canula.  Even at that, she's still having issues.  We have had to bump up the amount of oxygen she gets from about 1.5 liters to 3 liters or so, just to get her where she should be.  We aren't sure why this is happening.  She doesn't appear to be getting sick.

As we wrap up the year, we find ourselves dealing with all of Peyton's ongoing issues as well as increased levels of concern where her pain and oxygenation are concerned.  2012 has been a very difficult year for Peyton.  It feels like the bad has outweighed the good this year, even though I try to maintain a positive outlook as much as I can.  This year has brought some new {and serious} concerns that we will take with us into the coming year.  

If you wouldn't mind offering up some prayers for Peyton, we would greatly appreciate it!

As you know, this blog is under construction, so I am adding posts from CaringBridge here and there - sometimes multiple posts in a day.  Please bear with me if you are receiving email notifications of new posts.  Chances are it is old stuff being added, but I'd hate for you to miss something new, like this post!

I hope you had a wonderful Christmas.  Best wishes to all of you for a Happy New Year!

Moving Forward

I feel like we are picking up a little momentum where Peyton is concerned right now.  I mentioned yesterday that I had spoken with Dr. T. in Genetics in Boston regarding the latest news on that front.  I tackled 3/4 of the paperwork that needs to be done for the Manton Center - that's the research group in Boston.  The other 1/4 is Peyton's portion.  Turns out the rest of our paperwork was fairly straightforward.  Peyton's, of course, needs the more detailed medical history and requires more effort than I can muster up right now.  I have been sick for the past few days.  A trip to urgent care yesterday and a Z Pak should do the trick.  Then I will finally be able to get this done and on its way up to Boston.  The other 3 packets of information are already en route.

This evening I received a call from Peyton's Neurologist {Dr. K.} here in Charleston.  He was calling to discuss Peyton's case with me in light of his recent conversations with Dr. T. in Boston.  I think that's his new best friend now.  If ever I could be a fly on the wall in the middle of a conversation - it would be the one between the two of them.  I think it would be fascinating.

Dr. K. is aware of the possibility for Peyton to become involved in this on-going research study at NIH in Bethesda, MD.  He seems to be on board with this plan.  Dr. T. asked us to consider it and contact the other Dr. K. at NIH regarding the study if we were interested.  Dr. T. could go either way. Dr. K. here at MUSC actually knows Dr. K. at NIH.  He seemed quite interested in this study to see where it might lead for Peyton.  I think we all agree that it falls into the category of "no stone left unturned" or "nothing ventured, nothing gained".

The next step right now is the lumbar puncture that I had previously mentioned.  Dr. K. at MUSC is going to do that.  He wants us to think about it, but really, it is the next step we need to do.  It's necessary for Dr. T. in Boston to have results of a lumbar puncture to help establish a base line prior to any form of copper supplementation.  The LP is probably going to happen around the second week of January.

While I had him on the phone, I asked Dr. K. about increasing Peyton's Neurontin.  She is on this for pain for her hip and shoulder.  The generic name for Neurontin is Gabapentin.  For whatever reason, every time I open the fridge to get a dose or Peyton, I keep calling it Yo Gabba Gabba-pentin.  If you have a small child, you'll possibly find that mildly amusing.  Gotta do something to keep things interesting, right?  I digress.  Dr. K. is on board with an increase in her dose {it's already 3x/day, but the amount given with each dose will increase}.  We've noticed Peyton's pain level in the past week or two seems to be on the rise and we're having to give her Oxycodone between doses of Neurontin.

There is the possibility that Peyton will need a hip x-ray to check the status of her hip dysplasia.  He suggested a possible sonogram as well.  He also suggested that perhaps botox might be considered, but I don't know what my wrinkles have to do with her hip pain! I kid.  Like I said, I have to do something to keep things interesting.  We'll see how the increased meds help and go from there.

That's about it for now.  I was excited to have another phone call from another doctor this week.  Things are moving forward.

A Quick Update

Peyton is still at the hospital tonight.  She pretty much slept all day, which is odd.  I don't know if it's just taking that long for that last dose of the neurontin to get out of her system or if something else is going on.  The new neurontin dose was given tonight, so it will be interesting to see how that affects her.

There's really not a whole lot of news today.  Still at the hospital.  We are hoping to be home tomorrow.  By home, I mean the new place.  Ron is going to be pretty busy getting Peyton's room set up tomorrow.  It'll be a busy day in general as we try to finish off this move.  We've lost several days in our process with this hospitalization, so we'll have to get right back into it as soon as Peyton's out of here.

I will update you to let you know if we're home tomorrow or if we're still here!

Another Update

So, we were hoping to be getting out of here today.  However, it looks like Peyton will be spending not one, but two more nights here.  As there is nothing I can personally do about this, I am just trusting in God with His plan and timing.  We're in the midst of a move right now.  And by "we", I now mean "Ron".  The plan had been that I would unload stuff and take over smaller items during the days this week and he'd bring over loads in the evening.  All of that came to a standstill with this hospitalization.

Please know obviously Peyton's the first priority, but we do have to get moved!!

So, today Peyton pretty much slept all day long.  With the exception of about 15 minutes, up until about 5pm, she slept.  This is so not normal for her.  The one new thing that had changed was the addition of a new pain killer - neurontin - in place of oxycodone.  It's a medication that you have to start out at a lower dose and work your way up to the therapeutic dose.  She started at the normal starting dose.  The doctors talked to neurology and to pharmacy about this and they think that this starting dose must have been too much for Peyton.  They cut her off and will let her get through the night tonight without it.  She can have oxycodone for pain though.  Tomorrow, with a clean slate, they will try a lower dose of neurontin, but they will do it in the evening, which means another night here.

I did speak with the geneticist in Boston today.  He spoke with her attending here prior to that.  He said that this low copper - or, in her case, an almost total lack of copper can be related to lots of different connective tissue disorders as well as mitochondrial dysfunction.  With some disorders it is possible for the optic, heart, and/or brain vessels to become twisted.  His suggestion is to do an MRI/MRA.  You know what an MRI is, but if you're like me, then you just googled MRA - it's Magnetic Resonance Angiogram.  Basically a cardiac MRI.  This will have to be done under sedation.  This will be something that happens after Peyton's out of the hospital.

Also, he would like her to have an ultrasound of her bladder.  Apparently with these disorders, the bladder can enlarge and form pouches called diverticula.  Urine will collect in these pouches.  He wants to make sure she doesn't have this going on.  She recently had an abdominal ultrasound (last week), so the doctors here are going to see if they have enough information to go on from that rather than put her through another test.

The geneticist in Boston has consulted with another specialist up there regarding her low copper.  At some point she will need to be treated for this.  Unfortunately, it isn't as simple as taking a supplement like you would an iron supplement.  Based on their discussion, what she would need would be a specially prepared copper supplement.  He does want the other investigative studies done first though.  This means getting her through the MRI/MRA, a possible ultrasound, and getting results back on genetics testing that is already in progress.  Not the German study...he has other testing in progress right now.  Once we get through that, Peyton will need to be seen in clinic by this other specialist - at Children's Hospital Boston.  Yes, this will mean another trip up there.  He is thinking early spring 2013.  Perhaps we can coordinate it with Moira's spring break.  

At any rate, they will have all the results from all this testing being done.  I think from that they will be able to figure out how to formulate this supplement.  I don't know how it is administered - through her g-tube or if it's an injection??  I don't know.  I just know that we have another big trip ahead of us and treatment sounds expensive!!!  We will see how this all plays out.  I'm so excited to have so much going on in the genetics arena right now.  We don't have an answer in terms of a diagnosis, but we now know about this copper/ceruloplasmin issue.  If it weren't for our trip to Boston, we might never have known this.  I feel like we've backed out of that dead end street and are at least heading in a new direction now.

I FORGOT:

Peyton has been having more focal seizures since she's been in the hospital.  These are little "staring" seizures.  They are non-convulsive and they last only a few seconds.  Since Monday she's been having these several times a day and they've been lasting longer - closer to a minute.  It's been noticed by several people.  Her seizure meds were increased on Monday.  Again, non-convulsive - she hasn't had one of the convulsive types in a few years.

I'll definitely be keeping you updated on all that is going on.  Hopefully Peyton gets home Friday!

Boston - Day 5

Today was our fifth and final day of appointments for Peyton at Children's Hospital Boston.  She had two appointments - Neurology and Genetics.  Both of these were in the vein of "since we're here we might as well see them".  I am very glad we did!

First let me say that I have no problem at all with Peyton's neurologist in Charleston.  As for genetics, I loved our doctor in Houston and wish he was still her regular doctor.  MUSC just isn't as advanced as the bigger hospitals.  

At the neurology appointment this morning we went through Peyton's history and we pretty much decided to discuss the pain management issue as well as the possibility for some sort of sedative for Peyton at night since she's having an increasingly difficult time getting on and staying on bipap at night.  She has been taking oxycodone for her chronic hip pain for about the past month.  It does seem to help, but long term, it's probably not a good option because it is a respiratory suppressant.  This isn't good for someone like Peyton who has chronic respiratory illness!  The doctor was able to suggest something which, over the long term, will be better for her.  I have already been in contact with Peyton's neurologist back home and gave him the medication suggestion.  We will see what comes of that.

The genetics appointment was LONG.  We were at CHB for about 3 hours!  At least 90% of that time was spent WITH the doctor.  He was impressed with the doctors we have already seen in genetics (Houston).  He basically said it doesn't get much better than that.  He took Peyton's ENTIRE history.  He examined her.  He also took family history - our parents ages/any medical issues, death/age at death/cause of death, our siblings/their children/any medical issues, any random medical issues in the family, history of illness, etc.  He measured our (Ron and I) heads.  Turns out Ron and I both have large heads.  Yes he did actually comment that I have a large head...for a woman.  Nice.  :)  He gave a suggestion for a genetics clinic that is in South Carolina - we actually have an appointment in September with one of their branch offices.  However, he said he would NOT go to one of the branches, but go right to the "mother ship", which is about 3 hours from Charleston.  I think I will be looking into relocating our appointment.

The most recent genetics testing that Peyton has had was done about a year or so ago.  He suggested that at some point perhaps we should redo it as the testing process is becoming more enhanced.  We discussed a test which is part of a research study at CHB.  We had Peyton's blood drawn so they can basically bank her DNA.  This way if they decide to proceed with any testing through CHB, they will already have her blood.  The doctor is going to present her case next month to a committee that will determine if Peyton is a candidate to be a part of this research study.  If she is, they will need blood from both Ron and I.  We can do that in Charleston and have it sent up apparently.  If she becomes a part of the study, though, it is going to mean that one of us (not Peyton) will have to come back up here because there is a very lengthy consent process to be a part of the study.  Then when the results are in (again, if she is approved for this study), we will have to come back up.

There obviously aren't any answers on the genetics front, but it is great that we have another set of eyes looking at Peyton.  The doctor took a variety of pictures of Peyton and he will use those when he sits down with his colleagues to discuss various cases.

This week has been very tiring.  So many appointments packed into just 5 days.  I feel like we've been here two weeks or more, not just one.

Now that all the appointments are over, we will head back to Charleston and bring with us all the knowledge we gained so that we can discuss everything with all of Peyton's doctors back home.  I've already been in touch with a few and, in fact, have heard back from a couple already.

I have to say that if you are ever in a position where you need a second opinion (and, my God, I really hope you do NOT find yourselves in our position!), our experience with Children's Hospital Boston has been outstanding.  We have been extremely impressed with the facilities and, of course, the physicians.  We actually had the experience yesterday of traveling a short distance to Waltham, MA to see one of the doctors in one of the CHB satellite offices (this was where he was going to be IF we wanted to see him while we were here).  All the facilities we visited were outstanding.  We encountered volunteers in the Orthopedic clinic who really went out of their way to make the kids comfortable.  Most clinic waiting areas had a variety of activities for kids to do while waiting.  The Pulmonary clinic was all decked out in displays filled with tons of Jiminy Cricket memorabilia.  The lobby area was incredible!  There's a huge aquarium, an "pond", and a musical staircase.  It's just a regular staircase that you have to go up to get to the main elevators for each wing of the hospital building (there's a glass elevator for special needs/wheelchairs/handicapped/strollers) though.  But this staircase...on the one wall there are sensors and lights.  As you go up or down the stairs on that side of the staircase, the sensors play music as you pass them.  And the lights light up as you go up/down as well.  Pretty awesome stuff.  Then they have this huge plexiglas box in an area of the lobby.  Inside, they have this amazingly intricate wire track that goes all over the place - like a roller coaster - up and down, loops, etc.  Balls travel the course of the tracks and there are various metal parts to the track which, if they tilt one way or another, will determine which path the ball will take.  They ball might bounce down a little staircase to the next path, or zip around a few loops ending in the ball being tossed through the air into a wire basket before it moves on to the next piece of the track, or maybe it sits in a wire cage until 5 balls in total are in the cage causing the cage to flip and eventually dump the balls into a bowl where they will circle the circumference of the bowl before the finally descend into a hole in the bottom of the bowl.  At various points on the tracks, the balls cause a hammer to strike a bell or chime or a xylophone key or some other "musical" object.  It is the coolest thing.  I could have stood there watching it for hours.  Seriously.

Check out these youtube links:

http://www.youtube.com/watch?v=efF0JcO6UEo&feature=related

http://www.youtube.com/watch?v=h_wy9mz3Hpk

Did we get concrete answers to anything?  No.

Did we get recommendations/suggestions?  Yes.

Was this trip worth our while?  Most definitely.

Now it's time to get some sleep because we're hitting the road in the morning!  We will be getting home on Sunday.