Home Sweet Home

After a very frustrating and exhausting stay at CHOP we are so grateful to be back home!   We were discharged very late last night after a long conversation with Lucy’s doctors about what we are going to do next. ..

Lucy’s swallow study results confirmed what we feared the most, she silently aspirates with all liquids even thickened liquids.  She had difficulties with almost everything we tried, with the exception of one Cheerio at a time.  When she ate more than one at a time she coughed and that was not a good sign.   So what does all this mean?  Her CHOP doctors are extremely concerned that Lucy is at risk of aspirating anything that goes in her mouth, and because she is a silent aspirator ,which means she does not give any indications of chocking,  this is very dangerous.  They advised me to stop breastfeeding.  Lucy is currently 100% tube fed.  We are devastated by these results!  This is not what we want for our baby girl!  We suddenly feel that we are moving in the opposite direction. 

Swallow studies are a snapshot in time according to our pediatrician and speech therapist(ST).  We are going to do a repeat swallow study in a few weeks when Lucy is back to her “baseline”.  She has been through a big ordeal this past week and is doing better, but is still not back to her norm.   Our hope is that we will get a second opinion that will allow us to breastfed once again.  Breastfeeding is the only normal feeding activity that we have.  Equally as important, it is a means of comfort and bonding for Lucy and I.   We have been advised by our ST and pediatrician that there is a speech pathologist at Du Pont who specializes in aspirations and breastfeeding.  Our pediatrician once again has gone above and beyond and spoke with this speech pathologist earlier today.  We are looking at repeating the study hopefully at the end of next week.  In the mean time I am pumping to keep up my supply.     

These findings have been very difficult to accept!  We feel so blessed to have so many people in our lives who love and support us and who understand how important breastfeeding is to us.  Thank you so much for all your thoughts and prayers.

P.S. Her feeding pump is in the little backpack she is wearing.  We don’t make her carry it on her back, she tips over or falls backwards with it on. 

Monday

Lucy is sleeping and I am just sitting here contemplating the last few hours, days, week, month, year…

Lucy had a pretty good nights sleep, she woke only twice which is considered a good night in our book.  Drew and I have a ratings scale by which we judge what constitutes a good night.  Waking  only once in the night is considered an awesome nights sleep, 2-3 times is a good night, 4-6  times is a bad night, 7 or more is a sh**y night!  After typing this I just realized that it can go from good to bad pretty quickly.

We are waiting to have our swallow study done at 2:30pm.  This morning when the GI team made their rounds they told me that they wanted to wait for the results of the study before talking about letting us go home.  This is so not what I wanted to hear.  From what I understand, they need to asses Lucy’s swallowing issues in order to determine if she can take in liquids by mouth without the risk of aspiration.  This is what I also understand, it is next to impossible to determine if Lucy aspirates breast milk when she is nursing because you can’t fill my breast with barium and have her swallow.  They are going to try their best to simulate the suck and position of breastfeeding  by using  a Haberman  feeder (a special kind of bottle that resembles the flow of breastfeeding).  Here’s what I have an issue with, Lucy has never had an aspiration pneumonia before having the PEG procedure and upper GI, and she has been nursing for 15+ months.  It seems too coincidental that she had this occur immediately after her procedures on Thursday.  According to our ST,  she told me that because of the high antibody content in breast milk it is not likely that a baby can get an aspiration pneumonia from breast milk.   We know that she has aspiration issues when drinking from a cup, I have no problems changing her cup drinking, but to not breastfeed will be an issue.  I have been following the doctors orders, Lucy is currently 100% tube fed, the exact opposite of what we want for her.   If they find she is indeed aspirating all thin liquids than we will need to formulate a plan.  Right now, my plan is to think positive and trust that everything will work out for Lucy’s best interests!

Happy Father’s Day

Today was pretty uneventful as far as medical needs go. We were fever free all day, hooray!   Lucy is on 22 hour continuous feeds until her NPO status gets lifted.  She is tolerating her feeds for the most part.  She does have moments where we know she is too full because she gets  fussy, irritable, spits up, and generally doesn’t move all that much due to her belly being hard and full.  Her doctors agree that she needs a break from the feeds every now and then to allow her body time to digest, hence 22 hour continuous feeds and not 24 hour continuous feeds.   Their was some discussion from the GI team about putting Lucy on Erythromycin to help her with her motility, I’m not convinced that this is the way to go with her.  I think that we need to give this new tube some time.  By the end of the day today Lucy was behaving more like the Lucy we know and love.  Her tone is improving and she is now sitting and pulling up to stand, oddly though she still has a head lag when you go to pick her up.  I think that this is because she is not using her core muscles at all, they were weak to begin with but now she has a hole in her belly so forget about her using them now.   Her IV called it quits on her this morning which caused her a lot of discomfort.  When I asked her what hurts she held up her hand, I was so impressed that she did that!  Thankfully we pulled it out and did not have to place a new one!  We switched her to an oral antibiotic which we can push right into her PEG.  Tomorrow we may have to place another IV in the morning because she needs to fast for at least four hours prior to the swallow study.  Maybe we can figure something out, it seems torturous to have to place another IV, frankly she has no veins left to access.

Today we were able to get a six hour leave pass to get a break from this place.  Drew and the kids came around lunch time.  We had lunch in the cafeteria which the kids think is fantastic, actually it’s not too bad they have a sushi bar.  After lunch we headed to the Please Touch Museum for the  rest of the afternoon.  We all had a blast doing whatever we wanted at the museum. We closed the place down as usual, we were literally the last family to leave.   When we were leaving we bought ice cream for the ice cream truck that just so happened to be strategically parked right outside the museum at closing.  The kids kept saying over and over what a great day they were having.  That was the best father’s day gift Drew could have gotten.

Tomorrow’s plan is to do the swallow study at 2:30pm, I think  she needs to have a repeat chest x-ray done as well, and then go home!   Again, thanks for all your thoughts and prayers!

For better or for worse…

 

Eleven years ago Drew and I made the commitment to spend the rest of our lives together, for better or for worse, in good times and in bad, in sickness and in health.  Today we celebrated our eleven year wedding anniversary here at the hospital, one of  the least romantic places I can imagine spending our anniversary.   Drew bought me a raspberry iced cupcake in the cafeteria that slightly resembled a mini wedding cake the way the icing was swirled on the top.  It’s the thought that counts, and it was also a pretty tasty cupcake if I do say so. 

It  has been a very stressful year for us, as you can imagine, with all of Lucy’s medical issues, but our marriage is strong and we have become even stronger and closer this year than I could have ever imagined.  I love our life!  No, I never imagined eleven years ago today that I would be sitting in the hospital next to our fourth child’s crib side hoping and praying for answers to her health issues.  I did, however,  imagine that we would grow to have the very strong, loving, and connected relationship that we do have that has resulted in the many small children we call ours.  This is our life, and we are committed to making it the best life possible for better or for worse, in good times and in bad, in sickness and in health.  I love you babe!  I know you will probably read this before I see you tomorrow, I’m looking forward to the rest of our lives together…

Now for an update on Lucy, she is doing better, a deep sigh of relief just came from me as I typed those words to you .  I think the saying slow and steady wins the race applies well here.  Her fever is way down, but  ever so slight so they are monitoring her carefully.  Her tube site, or stoma, looks great according to the nurses who have helped me take care of it.  I’ll take their word for it, as a mother it is so strange to see a hole in your baby’s belly that has a tube sticking out of it.  I know I will get over it, but for now it’s still so foreign to me.  Today, I did all of the care and maintenance for Lucy’s tube, thank you very much.  She is still experiencing   a lot of pain at the site,  we are trying to keep her comfortable with IV pain meds.

We started running food through her tube last night, well actually early this morning.  The connection from her pump to her port was put in the wrong port on her tube so it disconnected and they fed the bed instead of Lucy.  She woke at 5 am soaking wet, smelling of that oh too familiar aroma of Elecare.  We have slowly been ramping up her feeds today and weaning her off of her D10 solution.  She has been continuously feed today with the exception of taking an hour off this afternoon when it appeared that she was uncomfortable and stomach bile was leaking into her tube. We currently have her at her maximum feed rate, so far so good.  Lucy is still NPO until we get the swallow study done on Monday.  The  plan is to wait until after her swallow study on Monday afternoon to be discharged.

My mom and the kids came to the hospital this afternoon to play and have dinner with us.  It was so nice to be together as an entire  family tonight.  The kids saw Lucy’s new tube for the first time, they were all relieved to see that she still has her belly button.  I think that they thought the tube was going to come out of her belly button:),  creative thinking.  I would probably think the same thing if I were still a kid.

Drew went home with my mom and the kids tonight.   He needs the rest!   My mom is going home tomorrow, she agreed to stay longer, but we’re pretty sure that we can handle things. We appreciate so much that she comes and takes on the responsibilities of our life for week at a time.  She and the kids have their routine.  In fact Sophie tells me often that Obachan (grandma in Japanese) makes something this way, or does something that way.   According to the kids she makes the best French toast and oatmeal with sprinkles.  She makes me laugh when she tells me how busy and active the kids are and how much they eat, she tells me as if I have no idea.  I know she is exhausted and is looking forward to going home and resting, but she misses them and all their activities and hunger the moment she leaves and goes back to her normal routines.  Thanks mom, for all that you do!

Drew and the kids are going to come back here tomorrow afternoon, we are hoping that they will give Lucy a six hour leave pass so that we can all go out and do something fun in Philly as a family tomorrow to celebrate father’s day.   Lucy and I would love the change of scenery.  It’s weird how quiet and empty the hospital gets on the weekend.  If nothing else, maybe we can go outside and take a nice deep breath of Phily air.  

Thanks for all of your thoughts, prayers, and support, we are truly blessed.

Bedtime update

We are all feeling the effects of the past few days tonight, sleep would be greatly appreciated tonight Lucy girl!  I will quickly update you on what we know.  Lucy is still running a fever, but not as high as it was last night.  She has been on antibiotics for almost 24 hours and I think that they are helping her because her fever is much lower tonight.  I feel like this remaining fever could be a result of her autonomic dysfunction and not her pneumonia.    She is still NPO but we have done two 50ml bolus’ of Pedialyte and so far so good.  She does not like us touching her tube, who can blame her.  Drew and I were trained on the ins and outs of her new tube this evening.  I have to admit that it is a bit overwhelming , but then again so was the NG when we first  started with that and overtime that became second nature to us.  I must also admit that there have been moments in the last few days that I have questioned our decision to put in the PEG-tube, but when I look at her naked, tube-free, adhesive free, smooth skinned baby cheeks I feel better about the whole thing.

I have been pumping every three hours to keep up my milk supply.  Lucy is not able to breastfeed because they are not sure if she is aspirating when she is nursing.  I have many opinions about this and tonight I will keep them to myself, besides I am too tired to go into a tirade.  We are scheduled for a swallow study on Monday afternoon if we are still here.  There has been no talk of being discharged, but that doesn’t mean I won’t put the idea into the doctors’  heads the moment I think that Lucy looks well enough to go home.

Overall she is doing better than she was last night, but she is no where near back to baseline.  Her bowels are moving oh ever so slowly, which makes her dad and I so happy, strange I know.  I know all of you parents out there are just as obsessed as we are about our children's bowel movements.  I think that conversations about our kids bowel movements are  about as common place between parents as conversations about the weather. 

Lucy has also been slow to move around.  She did sit up for the first time since the surgery tonight, but than she just laid back down on her back.  Her tone is very low but improving.  She has slept a lot and we know that is the only way her body is going to replenish its energy levels. 

Lucy is sleeping and so must I, after I pump again.  Thanks for all the support, we love hearing from you. 

Just a few of my favorites…

 

Tomorrow’s another day…

I admit that I set myself up for failure when we originally scheduled this admission.  I was really hoping that we would be discharged today so that we could be home in time for the weekend, which coincidentally happens to be our wedding anniversary and father’s day weekend.  Lucy’s body has other ideas, and right now we need to be here.   Lucy has an aspiration pneumonia that was detected by x-rays done last night, we are treating  her with IV antibiotics.  However, she is still running a fever in spite of the antibiotics and Tylenol.   Personally, I think that Lucy’s body has had enough and is responding to all of the stress with a fever on top of the pneumonia.  She is still very out of it and extremely floppy, when she has been awake she is extremely irritable, wouldn’t you be too! 

Lucy is still NPO, which means nothing by mouth, and now with the aspiration pneumonia they are having us hold off  on breastfeeding even longer.  Lucy has a history of aspiration, we discovered this when we introduced her to water and a Sippy cup several months ago.  Lucy’s suck and swallow are weak due in part to her hypotonia.  She is unable to draw water out of a regular child's non-spill Sippy cup, because of this we tried giving Lucy water through a Sippy cup without the non-spill valve in place.  This is when we noticed that she chokes, gags, choughs, gets red water eyes, and can even sneeze whenever she drinks water.  These are all signs of aspiration so we have been thickening her liquids to a nectar like consistency to prevent her from aspirating which can cause aspiration pneumonia.  Lucy’s speech therapist (ST) said that we could do a swallow study to evaluate the severity, or we could just remedy the problem for now and do a study at a later time when we don’t have as much going on, whenever that is.  In order to start breastfeeding again we need to speak with the ST here at CHOP and discuss the situation.  If I had to guess, I think a swallow study is in our near future. 

Lucy’s NPO status means that we still have not tried out her new hardware.  We have, however, gotten a little bit more familiar with it.  Both Drew and I have discovered that the ports on the tube need to be taped shut or else you will be sporting a very gooey stinky wet spot on your clothing whenever you hold her.  We will be getting a training on all the ins and outs of her new tube (no pun intended) sometime later today.  Lucky for us, her nurse for today happens to be the  g-tube instructor.

We did have to place another IV this morning, frankly the others  just wore out.  D-10 solution and antibiotics cause a lot of wear and tear on IVs.  The same IV team nurses from Wednesday placed her new line and were extra sensitive to Lucy’s situation. Often times a little extra compassion can make all the difference in the world when you are stressed to the max!   Let’s just keep our fingers crossed and hope that this is the last line she will need for this visit!

Great news, we were able to get the results back from the GH torture studies that we did on Wednesday, everything came back normal, yippee!  I hope this puts the whole GH issue to rest.  We have yet to speak with Dr. Langdon directly, but the endocrine fellow came in and gave us the results last night.   Now I feel even better about not doing the last study, it would not have been necessary.

We are waiting to see the speech therapist, waiting on orders to start the Pedialyte, waiting to see what Lucy’s body will do with this fever, and waiting to see her metabolic doctor and geneticist sometime this afternoon.  I’ll update you later…

More Updates

Update at 5:15

 

We are back in our room and Lucy is sleeping in her daddy’s arms. When I hold her I have to hold her standing up because when I sit down she wants me to nurse her, she is seeking comfort, I would be too if I had done to me what Lucy has. She is still NPO and will be until at least 9:00pm tonight.  At 9:00 we will try a slow bolus of Pedialyte  and see how things go from there.  Lucy is pretty out of it  right now, and from what we have been told will be for some time.  They put her under with IV sedation instead of general anesthesia, the drug they used to keep her quiet and still for the procedure apparently has long lasting effects.  The best thing for Lucy is to allow her to sleep and wake up when she is ready.  We are fine with that!

Update 9:00pm

Lucy could use some more thoughts and prayers sent her way.  She has spiked a fever and we don’t know why.  She appears to be in more pain than they typically expect for a baby who has just had a PEG procedure.  She is still very out of it!  We just went down to radiology to get a few x-rays of her belly to see it there is something going on inside of her that could be causing her fever and pain.   Right now there are many scenarios that are being thrown out there for possible causes, we trust that we will get to the bottom of this!  Obviously, we are holding off on any feeds until we know more.  She has been connected to an IV of D10 since 5:00AM.  She has not had anything to eat since than and is not acting hungry, big surprise.  I’ll update later.       

Update at Midnight

The films from Lucy’s belly came back at around 10:30 pm.  The good new is that there doesn’t appear to be any sort of perforation.  Now for the not so good news.  Her bowels are full and dilated indicating little to no movement.   This definitely could be causing her some discomfort, but the high fever not likely.  The films of her belly happened to capture the bottom portion of her lungs which looked cloudy on film.  We did a repeat chest x-ray at 11:15 pm which just confirmed the doctor’s suspicion, Lucy appears to have an aspiration pneumonia.  Antibiotics are on their way up. 

Where is Lucy?

Lucy is currently in surgery having her PEG tube placed.  They are a little behind schedule today, we did not actually get downstairs for her procedure until 12:00pm.  Once we arrived they took Lucy back fairly quickly.  They gave her a little bit of medication in her IV so that when I handed her over to the team it would make the separation easier.  They warned us that kids can start to act sleepy pretty quickly and/or they can act kind of freaky.  Once the meds went in she immediately started giggling and then she scared the life out of us with when a horror movie type scream came out of her.  The nurse took her from me and told us you never know how kids are going to respond.

Update 1:40

Lucy’s now back in recovery sleeping like a baby.   We were told she did well, which apparently they were a little nervous about when they first saw Lucy.  According to the nurses here, they have this theory about red heads, apparently they are difficult to put to sleep.  I can attest to that on a non sedation level but I have no experience with the sedation part.   Lucy handled the procedure well, but her new hardware looks a little scary to me.  I’m sure we’ll get used to it, but for now it’s strange seeing a tube coming out of our baby’s belly, it’s just not natural.

The plan for now is to just let her sleep.  That sounds good to us, she needs it and frankly so do we.  I’ll update later…

So far, so hectic!

I will just begin by saying we are not having an easy time of things.  It all began last night when the resident on call and her med student came in our room to get a detailed medical history on Lucy.   I have come to the conclusion that it is very necessary for me to sit down and write out some sort of current detailed summary of Lucy’s medical history.  I think that it is defiantly in Lucy’s best interest for me to do this.   I know Lucy’s medical history like the back of my hand, but it is exhausting to have to retell it over and over, not to mention very long and detailed for even the best historian to have to retell.  Over an hour later they went into detail about the plans for today.  We were prepared to do the GH lab draw one hour after she fell asleep, and we were prepared to do the three endocrine studies today that coincidentally will take about five and a half hours to complete, but we were not prepared to undergo a twelve hour fasting study.  We were told before we came that a mini fasting study might take place four hours before her endocrine studies this morning, but we were not aware of any plans to do a twelve hour fasting study.   I am getting the impression that as long as we are here they want to do as much as possible,  well we think that it’s too much for Lucy! 

Last night the IV team had a difficult time placing Lucy’s blood drawing IV.   She has one, count it, one good vein in her left hand that has been used over and over for IVs.  It’s what we would call her Ol’ Faithful as that is the only vein the team could find on Lucy’s hands, arms, feet, and ankles to use.   She has scars on her hand and wrist from previous IVs placed there.   I just hate that it is difficult for her to have an IV placed!   I hate even more that I know which vein is good and which veins are not. 

Her IV was finally placed at about 9:30pm and her night feeding began at around 10:15pm because the hospital had a difficult time getting her Elecare ordered, good thing I had some in my diaper bag because the waiting was getting ridiculous.  Drew mixed up her night potion, and they connected her to their feeding pump which alarmed more times last night than we have had with our pump in the last month.   Needless to say, we did not have a very restful nights sleep!

Lucy had her first blood draw at 12:15am which happened to be one hour after she fell asleep.  Well, one hour after that we were finally able to get the blood needed for the first test.  I hope the results are not affected by the variance in time.  At 6:00am Lucy was NPO and that is when our morning began.  Her blood drawing IVs have not been drawing blood,  instead we have had to squeeze the blood from each IV.  It has been a challenging day!

Update at 3:00PM:

We can no longer squeeze any blood from either IV and so we are putting a temporary hold on any more labs until we hear from her endocrinologist Dr. L.

Update at 5:00:

Dr. L just left and we all decided that we are not going to draw any more labs on Lucy tonight! She has had enough for today!  We agreed to come back and have the final study, the CRH stimulation test,  done if in fact we get results back confirming GH deficiency.  We also discussed why and how they would run a fasting study if we agreed to doing it.  I will make a very long story short, we are not going to do it!  Doing a fasting study would mean delaying the PEG procedure a day because GI doesn’t want her on their service immediately after a fast, I think that is very smart of GI.  Our original plan was to have the GH studies done , GI scope, and PEG tube placed, and after much debate we are sticking with our original plan.  I think we all feel better knowing that we are not going to have to stick Lucy any more today!  Tomorrow is a whole other story.

Update at 10:00

The kids came to have dinner with us tonight which really made our day!  Thanks mom for braving the traffic and bringing the kids tonight!

Tomorrow Lucy is scheduled for surgery at 10:00 am.  She will go NPO at 5 am and at that time will get be given and IV of D10, her sugar of choice.   I’ll update more tomorrow, I am tired and am looking forward to getting a little sleep.  Thanks for all your thoughts and prayers today, they helped make a difference!

Good Thoughts Needed

 

Please say prayers and send good thoughts Lucy’s way right now.  We are having a very difficult time drawing the labs necessary for the tests she is currently undergoing. 

She is on her second IV and it is barely working.  We have used all of her access veins and her stamina up.

Here’s the Plan

We have been anticipating this week for quite some time, but no matter how I am prepared I am not looking forward to what the next few days have in store for Lucy.  Today we are getting admitted at CHOP to have her endocrine studies preformed as well as have her GI scope, and finally her PEG tube placed.   This afternoon the plan is to get her IV’s in place.  She needs to have a blood drawing IV as well as an IV for fluids, we have not had much luck with blood drawing IV’s in the past so cross you fingers for this one.  Lucy will need to have an astonishing amount of blood drawn this week for her endocrine studies so a working IV is mandatory!   One hour after she falls asleep tonight they will draw labs to check her growth hormone levels, apparently GH levels are at their peak one hour after you fall asleep.  Lucy will need to do a mini fast before she undergoes a growth hormone stimulation test tomorrow morning.  I will detail more about this test after we learn more.  On Thursday morning she will undergo her GI scope and PEG tube procedure.  After that I am not sure what will happen…

As Lucy is getting older it is getting  more and more difficult  to put her through any procedures!  She is very aware of the hospital environment and has developed some coping mechanisms that amaze us and break  our hearts all at the same time.  We will be bringing our bag of tricks with us this week as well as some of our favorite blankets, binkies, music, baby carriers and my walking shoes.   Lucy feels secure when I “wear” her in a baby carrier and we walk.  She also finds tremendous comfort in hiding under her nursing blanket.  In fact, she will often seek out her blanket and put it over her head when she is unsure of a situation, oh it is amazing what they know and how they cope!

Drew and I will be with Lucy every step of the way.  I will update throughout the week.   Thank you to all of you who have sent us your thoughts, prayers, and well wishes!!!!  Please keep them coming this week , we can feel their power!

We’re Still Here

Just a quick update to let you know that we are still here, most of us are feeling better.  Jack went to school on Monday morning, he was feeling back to Jack by Sunday afternoon.  I however, have not been feeling well in the last 24 hours.  “It’s never good when the mama is sick,” a quote from my darling husband.    Off to bed I go, hopefully I will be good as new in the morning.

We are a family, we share…

I know I say this at least a half dozen times a day. 

“Sophie, can you please let Lucy have one of the twelve Barbies you are playing with.  Remember, we are family and we share!”

“Megan, it would be nice of you to share one of your pages of your coloring book with Sophie so that she can color with you.  We are a family and we share.”

“Jack, it would be nice if you let one of the girls be P1(Player one on the Wii) so that they can have a turn being in control.  We are a family, we share!”

This is just a sampling of the things we share.  Currently we are sharing some sort of funky wonky high fever, knock you off you feet, splitting headache, very exhausting, fast spreading virus in our house.   It started with Megan who woke up with a 103 degree fever Wednesday morning,  She looked awful, complaining that her ears, throat, and skin hurt.  She was just plain miserable, and just wanted to sleep.  She was most upset, however, that because she was sick she was going to ruin her prefect attendance record.  Her class had been making a big deal out of the fact that she has not missed any days of school this year, apparently she was the only one in her class to hold this position up until Wednesday!  Bless her heart, she has such a goal oriented personality that she was very disappointed in the fact that she no longer held her position.  Jack very sweetly said to her if he makes it until the end of the year with perfect attendance , which by the way is June 17th, than he will share his award with her.  I just love it when they do things like that for one another.  It makes us feel like we are doing our jobs well as parents!

By Thursday afternoon Megan was back to her usual happy healthy self, just in time for Lucy to start acting a little off.   To be honest, I don’t believe that Lucy had the same illness that Megan had.  She was acting fussy, tired, and was more floppy than usual, but did not have the same high fever as Megan.  She did not sleep well on Thursday which surprised me since she looked so exhausted.  On Friday, however, she made up for not sleeping on Thursday by sleeping more than twenty hours.  Lucy did have a low grade fever on Friday night, which is not unusual for her.  What was unusual was the way she was acting, just plain miserable and falling asleep whenever possible.   On Saturday morning she woke up acting more like her tiny little self, which made her dad and I feel better too.  

As for Sophie, well she was doing great up until bedtime on Friday night.  I was reading a Junie B. Jones book in our bed to the kids when she started to say that her tummy hurt.  She had this “I don’t feel so good look” in her eyes which prompted me to take her temperature, sure enough she had a fever.  It came on like that, an hour earlier she was eating a popsicle in the bathtub(the best place for kids to eat a popsicle in my opinion) happy and splashing, the next hour she was out like a light.  She went to sleep in an instant and slept in until past 10:00 am Saturday morning, so unlike Sophie who we  call our rooster.   When she woke she insisted that she was feeling better because she knew that  we had the Gore family picnic on Saturday and she didn’t want to miss it.  She still had that look in her eye which said to us that she was not feeling well, but no fever.  We went to the picnic with all intentions of leaving early.  Sophie not once complained about not feeling well, she wanted to do it all: the rides, the train, the pony rides, eat cotton candy, get ice cream, slide down the big purple slide, and  ride “The Tornado”.  Before we knew it the afternoon had flown by,  Sophie crawled into the stroller, pulled the canopy over her head, stuck her thumb in her mouth and called it a day.  When we came home at 5:30 pm we bathed her and put her to bed for the night.  All day she never complained about not feeling well; however, she did ask to go to bed when we buckled up in her car seat which is so unlike Sophie.

After we put Sophie to bed, I saw Jack lying in his bed reading.  He said that he was tired, which is code for I either don’t want to clean up my room or I am not feeling well.  Since I did not ask him to clean up his room, I knew that he was not feeling well.   He looked tired when we left the picnic and complained that his head hurt, we knew he was getting it.  By 6:30 pm he had a fever and we put him to bed with a garbage can by his side.  Every time he gets sick he thinks that he is going to throw up.  So far  I am happy to report no throw up, but as I type this Jack is laying on the couch, with a fever, and a bucket by his side.

Whatever it is that is making its way around our family we hope that it has mercy on Drew and I.  Neither one of us can afford to be sick!   Hopefully the yuck stops here, and we are spared from whatever germs we are currently sharing!   

Daily living…

Today I had one of those moments where I looked around my house and laughed silently to myself as I examined all of the things scattered around me.  Drew and I often  joke that if a criminal investigator came  into our house and tried to put together a timeline of events it would not be difficult to determine what we ate for breakfast, lunch, or dinner.   The dishes are often partially loaded in the dishwasher, sitting next to the sink, and sometimes left out on the table.  They would also not have a difficult time piecing together what we played with that day or week,  or what we wore recently.  All too often the dirty clothes make it somewhere in the vicinity of the laundry room, but rarely in the laundry basket where they belong.   If they looked in my van, well ,they would probably be able to put together where we have been in the last month, it has been a while since it has been cleaned out.  As much as hate to write this one, on some days they might even be able to count the number of dirty diapers I have changed as I am out of Diaper Genie refills and I have been piling the dirty diapers next to the diaper pail to take downstairs the next time I go or whenever I remember, gross I know.  Today I just smiled as I looked around because our home looks just like it should, lived in!  I love having various sizes of socks and shirts laying around my laundry room floor.  I especially love to see Drew’s socks next to Lucy’s.  I love having piles of laundry for each member of our family stacked up  in a row.  Notice I said piles, with Lucy crawling around there is no way to keep a stack of laundry folded unless you fold it and put it away when she is sleeping.  Unfortunately that doesn't happen very often, when Lucy is napping laundry is not a priority, our laundry room is upstairs near her bedroom.  I love seeing the doll house family set up all over the girls room.  I love even more that one of the members of the doll house family has taken up residence in Lucy’s room.  She just loves to suck on their heads.  I love looking in the bathroom and seeing an entire library of reference books on the floor.  What can I say, our boy is an avid reader, of non-fiction books none the less!  I love that Sophie’s favorite kitty is laying in my bed right where she put it when she came to lay next to me this morning.  I love that Megan’s blankets are piled in the middle of her bed because that is where she sleeps.  She sleeps with her head in the middle of her bed, never at the top of her bed, and never on a pillow.  She sleeps in her bed the same way she used to sleep in a crib.  All of these little messes constitute many of the events of our daily life.  Sure, at the end of the day Drew and I will do the usual pick up and get ready for the next day routine.  We might complain about doing the dishes,  and folding the laundry,  we might talk about having the kids become more responsible and have them clean up after themselves, and I am sure he will say something to me about remembering to buy Diaper Genie refills the next time I am at the store, but know that we are so happy that we have these things in our life to do and take care of. 

A little Mothering Guilt

Last weekend when I posted pictures from our Memorial day weekend I did not post any pictures of Sophie, mainly because we did not capture any good ones of Soph.  Well, I am feeling a little guilty about that so I am making up for it in this post.   By the way, recently we have been to a lot of kid functions where face painting has been popular.  The pictures with the messy face that don’t look like a professional face painter did the work, are from our outing to the strawberry patch.   For every one berry Sophie put in her basket two berries went in her tummy!

Enjoy!

We just love this girl!

An attempt to fill in the blanks…

Recently I realized, when talking with a few friends, that I may not have updated all of the most recent medical information.  Information seems to be coming and going at an astronomical rate these days so I am going to attempt to fill in the blanks.  This is as much for me as it is for those of you medical junkies out there.  Writing all of these events down helps me to organize my thoughts as well as provide me with a way of sharing the latest with all of you. 

Since March we have made a lot of realizations about Lucy’s condition.  Humor me if you have heard these before, I am going to attempt to list what we now know.   We know that Lucy is unable to grow without the aid of supplemental nutrition, we knew this before March, but have only recently come to grips with this reality.  Lucy’s GI discomfort has improved some since we have stopped our attempts with solid foods and switched her to an amino acid based/no milk protein formula, Elecare.  We suspect that Lucy has a milk protein allergy which is why she had so many “blow out” diapers on the other formulas.  Lucy is continuing to grow on tube feeds and without these feeds we believe that she would be at a stand still on the growth chart, much like she was from her second month of life to her eighth.  She loves to eat Cheerios, but we feel that she eats them not because she is hungry but because she enjoys the activity of  picking up the O’s, putting them in her mouth, and chewing or swallowing them.   We know that too many Cheerios in her belly is not a good thing, and we believe this is related to her body’s inability to digest foods the way most of us digest foods.   She gets fussy and irritable when she eats too many, her stomach can get distended and she burps a lot, Cheerio burps mind you.   Lucy’s G.I. doctor believes that she suffers from delayed gastric emptying (DGE), this means that food stays in her stomach much longer than average, thus causing her discomfort when too much food goes in at once.  This is perhaps one of the reasons why Lucy only drinks about two to three ounces of breast milk in a feeding, three ounces is considered a great feed.  We also feel that this is the reason why Lucy doesn’t tolerate a very high feeding rate on her feeding pump. She is happiest when the rate is  at or below 25ml per hour.  Because we have not had success with increasing her feeding rate, we have increased her calories per ounce in her breast milk and formula.  An average number of calories per ounce of baby formula is 20, breast milk is equally about the same.  We have increased Lucy’s calories from twenty to thirty calories per ounce, this is a huge jump in calories.   Despite this increase in calories, Lucy’s growth has slowed down. She was gaining at a rate of  a little less than a pound per month,  and is now gaining at a rate of about a half a pound per month.  We attribute this to the fact that she is more active now than she has ever been, thus requiring more calories on a daily basis.  You would expect that because she requires more calories for growth that she would be taking in more calories per feeding or feeding more frequently.  This has not happened!   Lucy eats approximately every four hours, with a hand full of Cheerios twice sometimes three times a day in between feedings. If I attempt to feed her sooner,  say every three hours,  she nurses for only a minute or two before she pushes away.  Now that she has teeth, she can do more than just push away from me!  Lucy continues to take in anywhere from fifteen to twenty ounces of breast milk per day,  this does not include night feeds.   Since starting enteral feeding we have thought of night feeds as bonus calories since her night nursings are so variable.   So, this volume  is approximately the same volume that she was taking in on an average day when we started enteral feeding in November.  It is amazing, not only to us but to many of Lucy’s doctors, that she has little to no appetite!  This has been a symptom of Lucy’s that she has had her entire life.  In fact, this was the reason that my “mothering instinct” brought me to seek the advice of or our pediatrician in the first place.  Lucy would often refuse to nurse, and ate very sporadically for very short periods of time.  Knowing what we know now, we understand why all attempts at adding additional calories via bottles or solid foods were not successful for Lucy.  She could only eat so much and that was it!

Lucy sees eleven specialists and therapists.  The consensus from all of these specialists is that Lucy does not fit the profile of a patient for any of the specialists that we see.  Lucy’s multiple systems involvement has confused even the best experts in their field.  All agree that she is dealing with something, that has never been up for debate, what it is and who she needs to see for diagnosis is what is up for debate.  All of her specialists, aside from her metabolic doctors, think that what Lucy is dealing with is metabolic in nature.  Her CHOP metabolic doctor believes that almost any disease can be traced back to metabolism, but that does not mean she can do anything about it.  The lack of answers is what lead us to seek the opinion of Dr Shoffner, a mitochondrial disease expert.  I think that it was fait that lead us to Dr. Shoffner.

While in Atlanta, we were able to hear Dr. Shoffner’s opinion of Lucy’s medical history.  He told us that he believes that Lucy suffers with autonomic nervous system dysfunction, known as dysautonomia.   Below is a statement taken from answers.com:  

“Dysfunction of the autonomic nervous system (ANS) is known as dysautonomia. The autonomic nervous system regulates unconscious body functions, including heart rate, blood pressure, temperature regulation, gastrointestinal secretion, and metabolic and endocrine responses to stress such as the "fight or flight" syndrome. As regulating these functions involves various and multiple organ systems, dysfunctions of the autonomic nervous systems encompass various and multiple disorders.”

Dr. Shoffner believes that dysautonomia is the reason for Lucy’s lack of appetite, G.I. issues, DGE,  fluctuations in body temperature, and generalized low tone.  We had no idea that so many of Lucy’s symptoms could be explained by this.  Dysautonomia in itself is an illness, but for Lucy it is related to a symptom of a much bigger problem.  The best that we can do with this knowledge is treat Lucy’s symptoms individually, much like we have been. Dr. Shoffner is also  looking at various neurotransmitter diseases as a possible explanation for Lucy’s symptoms.  We were told before we saw Dr. S that he will look at everything, and at this point we feel that everything needs to be looked at .  It will be several long months before we hear anything back from our testing in Atlanta.  Personally, we are trying our best to forget about it.  We are looking forward to the summer and are going to try and focus on all that life has to offer us! 

Lucy had a series of endocrine labs drawn in December and May.  The tests that were ordered in December, unfortunately, were undiagnostic , so more labs were ordered in May.  These labs were looking specifically at Lucy’s growth hormones and thyroid function.  Lucy’s thyroid has been looked at several times, as this was an obvious thing to investigate early on.   However, her growth hormone was not so obvious, as Lucy has many symptoms that do not fit the profile of a person with growth hormone deficiency.  Much to everyone’s surprise we found out several hours before leaving for Atlanta that several of Lucy’s growth hormone(GH) labs came back showing very abnormal findings.  Her endocrinologist and Dr. Shoffner both believe that Lucy’s GH issues are secondary to whatever else she has going on with her body.  Dr. Shoffner told us that mito patients can have GH issues.  Lucy’s GH issues can explain her hypoglycemia, which is why we now feel that a fasting study is not necessary.  Instead she will need to undergo several growth hormone tests when she gets admitted in June for her GI procedures and PEG tube placement.  We are not sure what to think of these labs.  Apparently, her thyroid numbers were on the low side, which is also new for Lucy.  I will write more about the specifics of the GH testing in a later post.

As of Last week Wednesday we have the dates set for Lucy’s upcoming admit at CHOP. We will be admitted on Tuesday, June 16th and if all goes well we would love to be home in time for Father’s day!   I spoke with Lucy’s GI doc about her GI procedures and he told us that he is going to combine her scope and PEG procedure in one!  I didn’t ask why he is going to do this, I just thanked him for doing this as this now means on less anesthesia for Lucy!  He just needs to work out who is going to place the tube, surgery or radiology.  Miracles happen everyday and we are very grateful for this one!

I have written a lot and if you have followed me to the end of this post, congratulations.  I feel better having written all of this!  Thanks for all your thoughts and prayers, we know they are helping!