8:00 pm update…

Lucy’s femoral line repair did not actually begin until 5:40 pm.  They were able to repair the existing line sparing Lucy the need to look for access in her right leg.  They were able to place a longer catheter in the same hole, hopefully this will hold us until at least Monday.  Our biggest hurdle in having this procedure done was finding a peripheral IV access site.  Thank GOD they were able to administer just enough morphine and Propofol to get her to sleep just long enough to place an peripheral IV in her neck, as all other points of access have been exhausted.  They did say that it took quite a bit more Propofol to keep her still during this procedure than the last.

When doing the x-ray to check for placement of her new line they also did a KUB scan to check for placement of Lucy’s J- tube.  At first glance it looks like Lucy’s J-tube has migrated out of the jejunum.  This could explain our terrible night last night.  For now, we are going to stop feeding through the j and just feed her TPN until we can get her tube replaced in IR on Monday.  Ugh…

Lucy has been recovering down in the PICU and she is doing well, tired, but alert.  When she woke from her procedure she asked for her “hansan” which in Lucy talk means  hand sanitizer.  It’s good to know that all of that Propofol has not made her loose her memory.

Not again…

It is 2:50 pm and we are waiting for the PICU to come up and get Lucy so that we can fix or replace her existing femoral line.  Like I wrote yesterday, one of the lumens on Lucy’s central line is not working, they suspect that it is cracked.  We can’t leave it like this because it is a huge source of infection.  Lucy’s temperatures are starting to creep up and that has everyone worried.  Ugh…the longer we are here the more that seems to go wrong!  We can not have a more permanent central line placed today as they do not have the staff to do it, and it is not considered emergent because we have options to fix or replace the existing line.  Lucy will again go under general anesthesia to have this done. 

In addition to the line problem, Lucy’s belly is barely tolerating the 5ml/hr at 1/2 strength formula.  She had a terrible night, one of the worst this week. 

God, please give my baby a break.  She has had enough!

Please, please pray for a smooth procedure and that we can repair the existing line enough to get us until Monday.

Trick or Treat…

Today the hospital celebrated Halloween by having the children trick or treat around the different units.  Child Life did a wonderful job coordinating this event and went out of their way to make it extra special for our little one and her brother and sisters.  Megan and Jack were in school during the festivities;  however, Sophie came with my mom to partake in some of the fun.  Special treat bags were made for all, even for those who were in school.   Lucy’s  friend Abby and her mommy Beth came for a visit and trick or treated with us.   Lucy went as her favorite sesame street character,  Elmo.  Tinkerbelle was our other option, but the costume hurt her “berry”.  Sophie was the most beautiful “Punky Breusterish” purple fairy who kept complaining that her costume was too itchy.  Abby too was a beautiful fairy.

 

We had PT and OT added to Lucy’s care while here at the hospital, more for something to do.  At home we have therapy four days a week and attend music class one day during the week.  Lucy has been very weak and has spent most of her days laying, leaning, or sitting, she has lost her ability to stand.  It will come back, we just need to get her stronger and feeling better.

Today, and really all weekend, the plan is to try and go up in her feeding rate to see how much she can tolerate.  I am sorry to say that it was not much. Every two to three hours we bumped up her feeding rate by one ml per hour, we started at five and we got to seven.  Lucy woke during her nap retching and in pain.  We shut her feeds off for an hour and restarted them back at 5ml/hr. This is the minimum rate we need to run her at to keep the walls of the intestines and bowels coated.  We discussed the situation with the nutritionist and she and I shared a similar frustration about trying to get Lucy’s feeds increased, she said that mito kids are tough in that they can not handle very much in the the way of change.  You really need to try and keep things status quo with their bodies. 

We are having trouble with Lucy’s femoral line.  One of the lumens, or ports of access,  is leaking and is no longer able to be used.  The other lumen is still working but we are holding our breath that this will hold until Monday when we need to schedule a line placement for Lucy.  We have had trouble with her line all day, first with the patch leaking, then the site bleeding, then the lumen leaking, and now we are getting alarm bells every so often that are saying the line is occluded, meaning there is not flow going in the line.  We redressed the site three times, which just opens up the risk of infection the more times you need to do this.  Please pray that we can make it through the weekend with one lumen on this line.  Lucy’s peripheral IV in her leg also stopped flushing today, so we are again worrying about access if we lose her femoral line.  Lucy’s PPN can only be run through a central line, her PPN is her only source of nutrition for now.  Ugh… if it’s not one thing it’s something else.

A better day…

Today we  saw more of the Lucy we know and love.  She is talking and playing more than we have seen in over a week.  We believe that because we are able to feed her through a central line she is starting to feel a little better.  As has always been the case with Lucy, if we can get her the proper amount of nutrition she will show you amazing things.  We are going to increase Lucy’s lipids or fats in her PPN tonight, we are limited to the amount of lipids and dextrose we can feed Lucy due to the type of central line.  We are increasing her feed rate to 10mls, at 1/2 strength Elecare tonight and I can already tell it’s not going to last very long, she is laying down and her tummy is sucking in and out, not a good sign.    

We started Lucy on two more meds tonight because her stomach cultures grew gram negative rods and yeast, most likely due to bacterial overgrowth from her slow motility.  I am so glad that I am not the one having to keep track of all of the meds for Lucy right now.  I think we are up to ten meds dosed two or three times daily.   

I did leave the hospital this afternoon to pick up the girls at music and to get a breath of fresh air.  It was a beautiful day, warm, and the colors on the trees are peak.  I walked outside to drive to music and had no memory of where I parked the van, I had to call Drew and ask him.  You think I have been here too long?

Tomorrow there are a lot of festivities for the kids surrounding Halloween.  My mom, dad, and Sophie are going to come and partake in the festivities with us.  

I am tired and I need to talk to Lucy’s nurse about slowing down her feeding rate.

As always thanks for your thoughts and prayers, they are being heard.

It’s Wednesday, not Tuesday…

I woke up this morning and it was dark and raining.  Dr R came in early, but not as early as I thought.  I thought it was 5:30 am as that was the time I went back to sleep after Lucy’s blood draw/vitals check, but it was already 7:00.  It didn’t feel like an hour and a half  had passed since I went back to sleep, but exhaustion can really mess with your biological clock.  I was also wrong about the day of the week,  Dr R reminded me that this was his day in Phily and that he would be checking in with the resident several times today about Lucy.  My response to him was, “Oh I thought you went to Phily on Wednesdays?”, his response  back to me was, “It is Wednesday and you need to go home and take a break from here today”!  You can really loose track of life time when you are in the hospital.

We started PPN last night and Lucy did well, so far no complications.  Her labs came back with a few elevated numbers, but nothing that can’t be tweaked by some reformulation of her PPN.  We also tried to run 1/4 strength Elecare with 3/4 Pedialyte at a rate of 5 mls/hr.  It was difficult to see her retching and gagging on this, we gave her a dose of Zolfran and that seemed to help.  Dr R wanted to bump up the Elecare to 1/2 strength, but I didn’t think that was a good idea.  Lucy was pretty miserable on the the fluid mix she was on.  It was hard to override Dr R’s orders when he wasn’t here for me to talk with him about it, but I know my girly and I just didn’t feel that was something she could tolerate tonight.  I’m pretty sure we will discuss it further in the morning.

As for our day, we had visitors this morning into the afternoon and evening and that made a dreary day a lot less dreary.  Lucy went in her wheelchair this morning down to the pool area to watch her friend Emma do therapy in the pool.  I was a little afraid that Lucy would want to get in the pool with Emma since she missed her pool day on Monday, but she didn’t.  It was more the other way around, Em wanted Lucy to get in the pool.  When we came back to our room our friend Dianna was waiting for us.  She brought Lucy some scented hand sanitizer, one of her favorite things.  Lucy is big time into smelling things and hand sanitizer is another thing she is into, she loves to rub it all over…well,everything.     Raquel came after lunch and straightened up our room for us and brought Lucy some things including some Strawberry Shortcake figurines that are scented, she loved those too.  Lucy was also visited by her favorite PT and OT (really her only PT and OT, but they are still her favorites) and that really made her day!  Ruth had Lucy laughing, and singing tonight.  Oh that’s a sound we have not heard in a while, Lucy laughing.  Lucy’s spirits really rise when she sees people she knows and loves, it is amazing to see.

Tomorrow is more of the same, we will try and push Lucy to tolerate some feeds all the while running her on PPN.  The GI doctor who specializes in TPN came in and talked with me about our girly’s nutritional status.  Lucy has really not grown much in the last six months,  and they believe that Lucy will require a combination of TPN and j-tube feeds for both nutrition and hydration.  They were not as encouraging about getting Lucy’s gi system up and running as Dr R.  Honestly, I really don’t know what to think.  Drew and I are still processing all of this, and are living hour by hour, or maybe more like two hours by two hours.

According to the nursing staff, Lucy is the most popular gal on the floor, we have had a steady stream of visitors which has been wonderful!  We are being well taken care of thanks in part to the wonderful staff at DuPont and also our wonderful friends and family who are are support staff!  Thanks for all that you do, we appreciate it all very much!

Tomorrow I am going to follow Dr R’s orders and get out of here for an hour or so, Diana is going to come and sit with Lucy while she naps so that I can take a much needed break.  Please pray for an uneventful medical day tomorrow so that I can have some peace knowing that Lucy will be ok while I take a break from the hospital.  The kids will come here tomorrow night to have dinner and attend Chronically Cool Families, our monthly support group for the entire family.  I think we are all looking forward to the meeting tomorrow night. 

Thank God for Another Day…

Thank you all so very much for thinking of and praying for our family and for Lucy, many prayers were answered.  There was A LOT of drama around here last night, and this morning we woke feeling the after effects of the entire situation.  The adrenaline is wearing off and I am feeling it.  I was instructed by Lucy’s doctor to be ready for the long haul.  He is ordering me to take a break from this place and go home for a few hours, I think I will try and do that sometime over the weekend.  Did you know it is Halloween this weekend? Of course you did, but I totally forgot until my sister called and asked if the kids were set for Halloween.  She said that she would set to work on it for us, thanks so much that helps!

Today Dr R and I agreed to leave Lucy alone!!!  She needs to catch up on some much needed sleep.  He ordered the residents and nurses on the floor to leave her alone and let her sleep!  It was awfully quite around here most of the day medically speaking, and I am in no way complaining about that! 

Lucy’s new femoral central line allows us to give Lucy life sustaining nutrition through her veins, known as PPN(partial parenteral nutrition). Partial because you can not give total parenteral nutrition(TPN) through a femoral line.  Lucy has been living off of D10 fluids for the past week and that is not nearly enough calories for our girl not to mention the lack of protein and fat.  Lucy needs some protein to help pull off the excess fluid that has made her body so puffy and swollen.  It is surreal that we are at the point where we have to rely on IV nutrition to keep our girly going.  Our goal is to NOT to have Lucy be 100% TPN dependent, but rather continue to push feeds through her j-tube and hope that we can get some or all function back in her GI system.

We started Lucy on two new meds in hopes of helping her manage her pain and discomfort.  We are using Zolfran to help control the retching, vomiting and possible nausea, and Amitriptyline a not very effective antidepressant that has a great side effect of calming an irritable intestinal tract.  I have lost count of how many meds our girly is taking daily now, eight or nine dosed two or three times daily.  Thank goodness we have nursing care for Lucy when we get home.

We had many wonderful visitors today.  Friends from church who brought me lunch and prayed over Lucy.  My dear friend Raquel who made it her mission to clean up and organize our room, feed me, and allow me some time to take a shower. Lucy’s speech therapist came to visit and brought Lucy one of her all time favorite things, bubbles.  Lucy’s pediatrician who just happened to call last night  in the midst of our crisis stopped by to see our girl.  I know I have sang her praises before, but she was able to track down the IV team charge nurse who just happened to be the nurse who placed Lucy’s last line that went after only a few hours before the line blew.  This nurse knew Lucy’s name when Dr F called and she knew immediately that Lucy was in trouble, so she in turn paged anesthesia to come and place a line and came up to assist.  Lucy’s nutritionist from CHOP came by to see our gal as well.  I called her this morning to catch her up on all of Lucy’s newest gi issues, and lo and behold she was on her way to DuPont to visit another patient and came to visit Lucy too.  She helped answer some of my many questions about TPN and was also able to meet Dr R in the mean time.

One of the best things that happened today was that my parents brought the kids to the hospital for a visit tonight!  I arranged for our favorite child life specialist to help make our visit with the kids as smooth and as worry free as possible.  The kids were so happy so see me and Lucy that my fears of them being scarred of seeing their sister so sick vanished quickly.  Lucy really perked up when she saw the kids, we even saw the much sillier less irritable side of Lucy tonight.  It was so nice to have all four of my kiddos in the same place at the same time, even if it was in the hospital.  The number one question the kids had was, “when are you and Lucy coming home?”.  I wish I could have given them an answer.

Lucy has so many lines connected to her body she is like picking up a kid and a bowl of spaghetti at the same time.  I do think that the new meds are making her more comfortable, as is allowing her gut some rest.  We are running Pedialite through her J-tube at 5mls/hr in an effort to keep things moving without making her sick, and we started her on PPN through her femoral line tonight.  So far so good.  Please pray for an uneventful night, this whole week and in particular last night was enough drama for a while. Pray that tomorrow brings new hope for an even better day.    

2:55 am update…

Lucy’s line was successfully placed and with no complications.  They tried to place it first on her right leg but that was not working so they moved it over to her left leg, near her groin area.  Drew and I have tremendous peace knowing that we no longer need to worry about her not having a point of access. 

Lucy came out of anesthesia better than anyone expected and she is sleeping like a baby, I kid you not!   She even got a salon hair due included with her central line placement since she has not had it washed in about a week, how’s that for service.

Your thoughts and prayers are so very much appreciated!

How we got to where we are…

It has been a rough week , but the past few days have been terrifying as we have watched our little one decline faster than we ever thought possible.   Mito is a baffling disease, there is no guide book on how to treat these kiddos as each and every one of them has different issues.  There are similarities, but no two mito patients are the same.  That being said, Lucy likes being different, heck she is our only red head, blue eyed baby after all.

This morning when Dr R came in he was surprised, I believe that is the appropriate word, to see Lucy the way she was.  We talked about what we wanted for Lucy and discussed putting in a central line as a last resort.  We kept referring to the a central line as “the enemy”.  Central lines and mito kiddos are complicated to say the least, the risk of infection is so great.  As the day progressed Dr. R kept checking in on her and we kept having to make decisions as to how to keep her comfortable and get her gut functioning.  We want to preserve organ function, he explained it to us this way, “if you don’t use it, you’ll loose it”.  We agreed to try and force feeds but Lucy’s body had other ideas.  She was unable to tolerate her feeds and her glucose started taking a dive.  Lucy’s life is dependent on having peripheral IV access.  Lucy’s body had no access points left.

This evening before Dr R left for the night we agreed to place a PICC line in Lucy tomorrow.  We chose a PICC over a central line because with a PICC line you can be put under with sedation instead of general anesthesia.  Well, we learned several hours later that was not really the case for Lucy as they use Versed for sedation.  Since Lucy can’t have Versed she would have to be put under with general to have the PICC placed.  To make matters worse, we could not get a PICC placed tomorrow because there would be no radiologist available to do the procedure.  She could however be placed on the schedule first thing Wednesday morning.  That was no longer an option as Lucy lost her fourth IV in six days at 9:15 tonight.

This brings us to where we are right now.  it’s 1:30am and Drew and I are sitting in the PICU waiting room waiting for Lucy and the doctors to be done placing her line.  This will be a temporary central line, as she will need to have another one placed within the next week for more permanent access.  It buys us time to plan and schedule a line procedure for sometime in the next week.  It was deemed critical to get this line tonight, her life depends on it.  

Update 10:40

Anesthesia was called to Lucy’s beside to place a peripheral line in Lucy, it had to be done in order to do anything and to keep her alive!  They were able to get a line placed in her left lower leg.  We are scheduled at midnight to go down to the PICU to have a central line placed.  Please, please continue praying for our little one. 

Lucy is very very sick, her GI system has shut down and she is having trouble maintaining her glucose levels.  Anesthesia and mito are not a good combination, but we have no other choice.

Please pray for Lucy 10PM 10/25

Lucy lost her Peripheral IV access again about a 20 minutes ago.  She needs to get another line placed ASAP to maintain her glucose level.  She is in trouble!  She needs a line, it is a matter of life or death!

Sunday…

Weekends in the hospital are not fun! However, the best part of our weekend was hearing from and visiting with friends and family!  I can not express in words how grateful we are for the blessing in our life that we are honored to call our friends and family.  Our family has been blessed over and over and we can not thank God enough for all that he has given to our lives.  So many people are praying for our Lucy and our family, please know that prayers are being answered in many ways for us all of the time.  

My parents arrived today willing and eager to help us with our three other major loves in our life, who I miss so much it hurts.  When we were admitted on Tuesday, I told my parents not to come, we would figure things out.  Instead I encouraged them to go on with their plans to help my sister and brother in-law with their new babies.  Matthew and Ryan we born October 15th, and have been a wonderul blessing in all of the chaos of our lives this week.  My sister and I talked last night, and she said that she was sending mom and dad our way, she told me we needed them more than she did, and they needed to be here as well.  That means so much! 

The older three kids had a fun filled weekend of activities, play dates, and sleepovers.  They attended a workshop for siblings who have a sibling with a chronic medical condition. We planned to attend this months ago, not realizing how appropriate this would really be for our kids this weekend.   Whenever I spoke with the kids this weekend I was so proud of them for being so loving, understanding, and having so much care and concern.  I miss them all so much!  We did not have dinner together as a family tonight because I knew that they were exhausted from their weekend excursions, and they needed to eat dinner at home, and go to bed on time, so that they could be ready for school tomorrow.    

      

I wish I had better news to report today with regards to Lucy, sadly I do not.  She is exhausted and and uncomfortable.  At times she is hypersensitive to things and extremely irritable, and then just when you lest expect it she’ll give you a tiny glimpse of her silliness.   Lucy is not tolerating any feeds.  We attempted again today to start her on feeds and she retched, gagged, and vomited.  Her stomach has completely shut down, as it continues to pour out green bile into her drainage bag.  We are able to run Pedialite through her J-tube for a few hours before she gets uncomfortable and starts retching, but no formula.  Lucy has not had any “real” food since Tuesday of last week.  I am afraid to weigh her, we have worked so hard for every ounce.  We had to place another new line today as the one she had placed on Friday night stopped working, D10 solution is very rough on IV’s because it is so thick.  Thankfully this line placement went much easier than the three hour fiasco we had Friday night trying to place a line.   As for Lucy’s bowels, we feel that the Golytely is out of her system now, her stool has changed from looking like apple juice to now looking more like the color and consistency of  mud. This sudden change has the doctors worried that she could be malabsorbing what little food we have tried to feed her through her J-tube.  Ugh…

Drew and I stayed with her all last night and today, she even went in her wheelchair for a bit this evening, and we walked the empty hallways of the hospital.  Towards the end of our walk she was having a difficult time holding her head up, so we called it a night.  We tried to get her out and about this morning, but she was too uncomfortable.

Tomorrow our main doc will be in bright and early, we have a lot to talk about.  Please pray that Drew and I have the clarity of mind to continue to make the right decisions for our little one.  Pray for Lucy’s comfort and that her little body can get the nutrients it needs to survive.   Please pray for our marriage that it continues to remain strong through these difficult times.  Pray for Jack, Megan, and Sophie that they always have hope and know that no matter what they will always be taken care of and loved!

Intolerance…

We learned the HARD way that Ativan is NOT the drug for Lucy.  It is a sedative, and for most people it makes them calm and sleepy, but not for our little one.  Lucy’s brain chemistry is different, her brain does not react normally to sedative type medications.  For Lucy Ativan had the opposite effect, it made her manic.  We witnessed a similar situation when Lucy was given Versed for her G tube procedures last year at CHOP, she went manic on us then and the radiologist at CHOP had to call me back to try and calm her during her procedure.  I learned today that other mito kids have similar type reactions to Ativan.  I spoke to the doctor on call about it today and he explained it to me this way; kids with ADHD are treated with a stimulate type medication which is the opposite of what you think a hyperactive child should take, this is because their brain chemistry is different.  We will look into other medications that will hopefully be more effective for Lucy.

Without going into too much detail, yesterday was perhaps one of the most difficult days of Lucy’s life and mine as well.  In addition to all of the hysteria that we now know was exasperated by the Ativan.  Lucy started gagging, retching, and vomiting after attempting to start her on J- tube feeds.  Along with her J feeds not going well, her stomach has completely shut down.  She has a gastric drainage bag attached to her G port and it is pouring out green bile.  We can not put anything into her stomach without it coming back out.  We are relying on her IV of D10 to keep her going for now.  However, yesterday her initial IV infiltrated and we had to place another line ASAP as her glucose was falling.  Placing the IV was traumatic, it took over three hours to find a point of access.  The kids came to visit, but it was clearly not a good day for Lucy and we did not want them to see her like that.  I saw them for all of a few minutes when Drew took them to the gift shop, cafeteria, and then to our friend Raquel’s house for the night.  I needed Drew last night more than ever, as I had physically and emotionally reached my limit.

Lucy is no longer manic, thank God, she is the opposite very lethargic.  She is not looking her best, but you can see that she still has that desire to preserver in her eyes.   She is not tolerating feeds and has a temperature that comes and goes.  We are needing to suction her when she vomits.  We are not sure what is going on with her.  

Prayers are very much needed that things start turning around for her soon, so that we can have back the Lucy we know and love so much!

Another quick update…

I am sorry to report that we are continuing day 2 of another bad day.  Lucy is MISERABLE and she could use some thoughts and prayers sent her way.

She is beyond tired, and we have most defiantly upset the delicate balance of energy in her body.  We have increased her Ativan dosing times 4 as she is manic and unable to shut down her brain. 

Finally after over 18 hours of not sleeping, she has succumbed to the meds and is sleeping.

Please, please, pray that she finds comfort and stays sleeping for…lets just say a looooong time.  Please pray also for her mommy’s sanity. 

My mommy heart is hurting for her…

A Bad Day

Our day turned sour pretty darn fast!  It is 12:58am and I think that we finally have some control over her pain issues.  I will be brief as I am too tired to write too much.  Lucy had her GJ tube placed under fluoroscopy in IR at around 3:00pm.  At 3:45 we were back up to her room, she was MISERABLE, and she made sure EVERYBODY knew it!  I think that we have “stirred the pot one too many times”, Lucy’s body in particular her belly is done being messed with!  Ativan was order for her at 4:33 pm(um, yes I was watching the clock waiting anxiously for something from pharmacy to come up to calm my baby) after a debate about what to give her for pain control.  Lucy was finally allowed to get some much needed sleep at around 5:00 pm. 

Drew brought the kids over for dinner again, and it was nice to be able to eat dinner with them knowing that Lucy was sound asleep. We let the kids peek in at Lucy for a second, but did NOT want them to wake her.  They were quiet, blew her kisses, and whispered good night.  I waked everyone downstairs and kissed them and said good night and that is when the tears started flowing, both Megan and Jack were sad to go and were upset that they could not play with Lucy.  Last night it was Sophie who had a difficult time parting ways.   Oh my heart broke for them, these are the moments that make “this” so real.

I was called the moment the kids left and was told that Lucy was awake, was pretty miserable, and had also pooped!  I think she woke up because she pooped, and not because she was well rested.  She was pretty darn cranky and unable to be soothed, and after screaming for over an hour we were told that we could move to an isolation room where she might be more comfortable…and frankly so would everybody else.   

We are still awake at 1:27am because I think her bowels are FINALLY starting to move!  They ordered her another dose of Ativan at 10:00 pm and she looks out of it but is wide awake with hiccups and belly pain.  Ugh… not fun, not fun at all!  She is also very swollen from all of the extra fluids, we had to retape her IV tonight because of all of the swelling.

Here’s hoping that tomorrow’s a better day!

A quick update…

First off NO poop yet, it will happen, it has to!  Dr R and I agreed that at this point we are so vested in this that we just need to stay the course for a while longer.  He even went so far as to talk about who was on for the weekend, so I am thinking that we are really in this for the long haul. 

Lucy and I took a trip down to interventional radiology(IR) this morning to do a gastric emptying scan (GES), because…this was as good of time as any to do one.  She already has an IV in place, we could easily make her NPO by turning off her Golytely at 4:oo am for an 8:00 AM GES, and she is exhausted which means she has no interest in using her muscles at all which makes laying strapped to a table under a scanner really not as tortuous as it sounds.

Lucy GES scan showed that she is dealing with pretty severe reflux which despite being on a reflux med is not helping her.  She also suffers with gastric emptying, no big surprise there.  All of these findings have led us to having Lucy’s GJ tube placed this afternoon. 

We are heading down to IR in a few minutes and we are going to attempt to place the GJ tube without sedation.  Please pray for Lucy that this goes as smoothly as possible.  

Still waiting…

How long do you think it will take to “clean out” Lucy’s bowels?  That’s an excellent question.  According to some very knowledgeable mothers I chat with in regards to mito and motility disorders, the consensus seems to be 3 to 5 days!  Lucy has been running a solution of Golytely for over 24 hours via her g-tube, this is what is used to cleanse people  prior to surgery or having a colonoscopy.  In “most” people Golytely works in a matter of hours or overnight… well, as you know Lucy is not “most” people.  She is Lucy!

Our day started early, as Lucy did not sleep well at all last night.  Lucy has some anxiety issues, who can blame her, and when she is nervous she picks at things.  The crib in our room is covered in stickers stuck on from various children over the years,  and well our little one thought that she should do something about that, and started picking at all of the stickers stuck to the crib.  She took her nervous habit one step further and started singing “DDDDDD-RA” all the while picking at a Dora sticker that was literally stuck underneath a dozen or so other stickers.  Seriously, I was contemplating asking for some alcohol prep pads at 2am so that I could try and take the stickers off myself just so that she would lay down and go to sleep.  Sleep finally happened, but not long enough, we were up for the day at 6:30am. 

Dr R came in at 7:00am and discussed holding off on placing the j-tube until after we see the motility specialist next week Tuesday.  He wants to make sure that placing a j-tube is really going to help make a difference and is interested in hearing what Dr B has to say about Lucy’s issues.  We also discussed using an injectable med on Lucy to help her empty her bowels.  For the life of me I can not remember the name of the med, but he did say they use it mainly in patients who are heavy narcotics users and have no ability to move their bowels.  Our main goal is to get Lucy cleaned out and feeling better, so far we have yet to accomplish that.  She looks puffy and bloated, thanks in part to the IV D10, and also to her dysmotility. 

We spent our day walking around the hospital, knocking several tests and procedures off our medical to do list, and setting off alarms bells when we tried to go to the coffee shop located quite inconveniently near the outpatient exit.  Lucy’s ID band is equipped with a security band, and lets just say her band is pretty sensitive.  We were originally scheduled to see Lucy’s cardiologist today, instead all of her cardiology testing was ordered as in-patient testing.  We heard back this evening from her cardiologist that everything looked great with Lucy’s heart, we can follow up again with them in a year.  We also walked downstairs to outpatient therapy where we saw some dear friends of ours who are living in it like we are, Lucy’s aquatic therapist, and her augmentative communication specialist all at the same time. Now how many of you can say you have these types of people in your lives, much less see them all in one room coincidentally.  Tomorrow morning we have set up a consult with her augmentative specialist in hopes of starting the process of getting Lucy her own Vantage Lite.

We had some wonderful visitors today which just made our day!  Drew went home this afternoon and picked up the kids and brought them here to have dinner with us.  They really love to see where Lucy stays when she is in the hospital, it allows them to “see” what things are like for Lucy and I when we are here.  Jill, our friend and kiddos music teacher came by this afternoon and spent some time keeping us company and played with Lucy which helped to keep her mind off of her discomfort for a while.  Helen, one of Lucy’s nurses surprised us this evening by visiting.  All of the kids loved seeing her including Drew and I!

Please pray for poop…what can I say it’s our reality, my entire life revolves around it.  Tonight when I was talking with my sister we were discussing the twins poopy diapers over the phone and I just had to laugh at the irony of our conversation.  All mama’s are obsessed with their baby’s BM’s, but when they go as far as Lucy’s have gone it becomes a very scary reality.

Thanks for all of your thoughts, prayers, and well wishes we appreciate them very much!

In Patient

No sooner did I go and blog about Lucy’s latest issues did she go and take it to the next level.  Lucy is severely constipated, so much so she is experiencing episodes of pain.  She can tell us now when she has pain, she keeps telling us “berry hurt”.  This helps us to decipher even more where her pain is coming from, although she generally always tells us that her “berry hurt”.  She is experiencing episodes of intense pain which if  you saw her belly scan you would understand why.  Lucy’s is full of it, by it I mean poop!

On Saturday she was acting “off”, she woke in the morning saying that her “berry hurt”.  Her belly resembled the shape of a peanut, her g-tube site was slightly sucked in and her lower belly was distended. I knew that things were not right when I went to lay her down for her nap and she began crying out in pain.  We called her main doc Dr R, who just happened to be on call over the weekend, and discussed the situation.  After administering an enema and a suppository at home with NO results, he had us bring her to the ER to take a scan of her belly and to administer a more “industrial strength” enema, which by the way did nothing.  

After taking a look at her scan, we knew we had to clean her out.  We formulated a plan with Dr R to attempt to clean her out at home, because after all nobody wants to be in the hospital much less in the hospital over the weekend.   Fast forward three days and many, many capfuls of Miralax and enemas later and our girly still has yet to have a substantial bowel movement.  I knew this morning when she was crying out in pain that it was time to call Dr R and discuss the next step, being admitted for an in-patient clean out. 

Lucy was admitted this evening for an in-patient clean out, some GI studies, and GJ tube placement.  We are not really sure how long we will be here, it’s up to Lucy’s body, which as you all know is not the most reliable.  They want her pooping water before we begin her feeds again.  She had an IV placed with just one poke, a first for her, and D10 is running.  She is exhausted, but not sleeping because …well… we are here and not at home. 

Our friends the Puffs took the kids for dinner tonight while Drew and I got Lucy situated here at the hospital.  Drew is now at home with our other three kiddos.  He will get then up, dressed, and off to school in the morning.  I was working on the laundry up until the moment we left to come the hospital, the clothes are clean, but folded…not so much.  Sorry babe.

I will update tomorrow.  Your thoughts and prayers are greatly appreciated.

It’s been awhile…

…I know!!  I have been trying to carve out the time to put down in writing all that has been going on around here, I just don’t seem to have the time or the energy to put into words all that we are feeling or experiencing.  We have a lot going on in our everyday life in addition to Lucy’s medical life.  I am so far behind in updating you on the everyday stuff, that I am not sure where to even start this post…I think I will attempt to put  in writing some of the medical stuff occupying my mind.

I did it again, I jinxed myself  by putting down in writing that we were hoping to take the month of August off from any medical appointments, you think I would learn.  I wrote back in July that Lucy was finally, for the first time in her life, stooling daily sometimes twice daily. We thought that we had at last found the proper med dosing and things were finally working the way nature Miralax and enemas should work.  Little did we know…

Lucy started acting “off” for lack of a better description early to mid August.  She was stooling more frequently, but very small amounts, not what you would expect to see in a kiddo who was using so many meds just to have a bowel movement.  Lucy can only have a bowel movement after she has slept for a period of time, because she simply does not have the muscle strength or the energy to stool any other time.  Due to the increase in her Miralax dosing she was waking in the middle of the night and during the middle of nap time with dirty diapers.  This has caused a big disturbance in her sleep and in turn mine as well.  If we try to cut back on the Miralax even slightly then we revert back to no stool for days.  Ugh…what’s a mama to do?  After several weeks of this, my mothering gut telling me something was not quite right, and our nurses encouraging me to take her to see her GI doc (our second least favorite specialist, metabolism being our least) to check things out and at the very least get a scan of her belly to see if there was any noticeable blockage that could be causing her issues.  We feared that perhaps she could still have had some barium left in here system from the barium enema that she had done in early July.  After scanning her belly we learned that she did not have any barium in her belly, but that she had many pockets of stool sitting in her intestines despite increasing her med dosing.  This is directly related to her poor motility, Lucy’s GI system, in particular her lower intestinal motility appears to be declining.  She is dealing with chronic intestinal pseudo obstructions (CIPO), this is something that she has most likely been suffering with all of her life but is a bigger issue now for some reason or another. 

After discussing the issue ad nauseum, we came to the discussion about “quality of life”.  We can’t “fix” Lucy, Lucy has a progressive degenerative disease that has no cure.  We know this, but that is not a reason to not try and treat her symptoms as best as we possibly can.  I’m not saying that GI is suggesting we do nothing, they have made several suggestions.  Unfortunately, with every suggestion, we have new issues that could potentially arise, we just don’t know what will work best for Lucy.  What we did determine is that Lucy needs to be seen by the top motility specialist at CHOP, and soon. 

So begins the barrage of appointments this month.  We are scheduled to see GI nutrition, neurology, cardiology, Dr R, metabolism, and the GI motility specialist at CHOP…not all in the same day mind you.  We are once again putting all of our preverbal eggs in one basket and hoping and praying that Dr B  will have some valuable input and advice regarding Lucy’s motility.

I have so much more I want to share but for now sleep is calling me…

Your thoughts and prayers are once again needed and greatly appreciated.       

2010 Energy for Life Walkathon…

We all woke bright and early Saturday morning to show our support for Lucy and all who are affected by mitochondrial disease!

We are so overwhelmed…in a good way…by all of the love, support, care, concern, thoughts, and prayers that are given to our family!  It means the world to us to know that so many care!

It is for the Love of Lucy, and the many others affected by mitochondrial disease, that we came together to raise awareness and funds for mitochondrial disease!

Our team shirts were simply…adorable. 

Many, many thanks to our dear friend Kimberly for designing our team logo “For the Love of Lucy”.

Friends young and old gathered to walk.

Honestly, I think these girls could have walked a marathon…

…they were so happy to walk together!

We pushed Lucy in her wheelchair…

While others pushed strollers…

Some took turns pulling wagons…

While a very special brother took turns pushing Lucy’s wheelchair so that Mommy could snap a few pictures for posterity sake…

 

Megan offered piggy back rides to Sophie, as long as the ride wasn’t too long!

Others found the view from atop of their daddy or mommy’s shoulders to be the best!

Some pulled more than their fair share of the weight for the walk…

  While other’s went along for the ride.

A few scootered…

While some were content to just sit and take in the view…

…of the Philadelphia skyline.

Perhaps one more could have used a ride…

In the end all of the hard work and planning that went into organizing an event of this magnitude paid off…

…the First Annual DelVal Energy for Life Walkathon had over 300 walkers and raised over $30,000 for mitochondrial disease research!! 

We look forward to doing it again next year for the love of Lucy and the many others who are affected my mitochondrial disease!

Please continue to support the United Mitochondrial Disease Foundation in the efforts to help find a cure!