How are things…

Well, let me tell you…It has not been a carefree summer by any means.  Things with Lucy are stressful at the moment.  She is experiencing what Dr. R describes as an autonomic storm.  We have seen this in her before, with each episode we see similarities but the presentation is different as is the outcome.  Lucy’s heart rate, blood pressure, body temperature, balance, over all coloring-she looks gray at times, oxygen saturation levels, energy level, attention span,  motility, and bladder are all areas of concern.

Several weeks ago Lucy started with elevated temperatures which always has us on edge.  Her heart rates have been swinging from the mid 50’s to 180’s.  I wonder what mine have been?  Lucy has been walking a hypothetical tight rope, balancing between fever and no fever, she has been sitting anywhere between 96.7 and 100.4, anything at or above 100.4 is considered a fever and thus and automatic admission.   Because we have Lucy on continual antibiotics it can be difficult to determine if an infection is brewing.  We did labs and cultures a few weeks ago on Lucy’s stomach and intestinal bile, they were seriously smelling like rotten eggs, to try and figure out what if anything obvious could be the cause of her issues.  We switched up antibiotics to cover some of the bugs growing in Lucy’s GI tract and put Lucy on a two week course of IV antifungals in hopes of seeing some improvements in our girl.  The antifungals helped to clear the yeast from her mouth which has been a problem for the past few months, so much so that Lucy developed some odd licking and sucking behaviors that I am happy to say have ceased since clearing the yeast from her mouth, poor baby.  The antibiotics have helped to kill the rotten eggs some of the new bugs that grew in Lucy’s cultures.  Despite our efforts to control the bacteria and yeast, our girl has not shown us signs of improvement. 

We had an appointment with Lucy’s cardiologist a few weeks ago.  Lucy’s hypertension and tachycardia are being controlled with the use of the clonidine patch, this is a good thing.  However, Lucy’s blood pressures are low, this is a side effect of the clonidine patch.  If we make any more changes in her clonidine dosing we are all fearful that we will see a rebound effect from the medication and we will be right back to the problems with hypertension.  We all agreed that clonidine is a medication that is keeping things under control for our girl, and so we are keeping the dosing where it is at.  In addition to being diagnosed with sinus tachycardia, POTS, and hypertension, Lucy was also diagnosed with an irregular heart rate.  Lucy's cardiac issues are not related to her heart muscle, but rather her autonomic dysfunction.  We have serious concerns for Lucy’s heart, but at the present time our options for treating Lucy’s symptoms come with greater risks than benefits. 

Most noticeably Lucy’s coloring is a few shades whiter than white, with a tinge of gray and purple thrown in there for coloring.  A few weeks ago she herself requested the need for oxygen, this coming from a girl who at her sickest just a few months ago fought wearing a nasal cannula while heavily sedated.  She has pretty much been 100% oxygen dependent for the past few weeks, without it she gets anxious and fidgety and starts looking gray.  We are getting used to the constant hum of the oxygen compressor in our upstairs hallway, Jack even said the other night that he likes the noise of Lucy’s oxygen compressor, “It makes enough noise that we don’t have to be as quiet in the morning.”.       

Lucy’s belly pain is constant and concerning, she continues to drain dark green and golden yellow bile from both her g and j tubes.  I have brought her to DuPont almost weekly to have her gj-tube checked for placement, almost hoping that they are out of position which could be the reason for her belly pain.  Surprisingly, it has been in good position which means that we have managed to keep a gj-tube in Luc for over a month!  Our IR team has been just as surprised as I have to see it still in her small bowel.  We started Lucy back  amitriptyline, a medication that helps to calm an irritable intestinal track, at double the dosing she used to be on before she became sick in April.  We are hoping that this will provide her with much needed pain control as our options for pain control are not options that I want for our little one just yet ever.

Lucy’s bladder issues have really come to light in the last month.  Since introducing straight cathing, Lucy has amazed us all at her willingness to have it done!  As with all things we have to to do for our girly, once she realizes the benefit she tolerates whatever it is that needs to be done.  She is so amazing!  She began realizing how much better she felt when her bladder was emptied, however we also realized how severe her bladder spasms were.  We started Lucy on the Oxytrol patch in an effort to alleviate the intense bladder spasms that she was experiencing every time her bladder was full and we went to cath her.  This medication has made a huge difference in Lucy’s quality of life.  Lucy is no longer able to empty her bladder without cathing which means we are cathing her every one to three hours or as she requests.  This gets complicated by the fact that Lucy is on Lasix twice a day to help with the third spacing in her body.

With all that Lucy has going on we have decided on Friday to schedule Lucy for a blood transfusion tomorrow.  We are hoping and praying that we will see some improvement in our girl.    

Our Make-A-Wish Memories

Make photo slide shows at www.OneTrueMedia.com

June…

It’s been so long since I last posted that I can title this post the month of June.  I originally set out to write a post about the UMDF’s annual mitochondrial medicine symposium that took place in Chicago two weeks ago, I am still working on that one, in the meantime I feel the need to catch you up on all of the other happenings in our life.

In an effort to just get it all out of my head, I am going to randomly write in list format….

The kids are all finished with school for the summer…still can’t believe that.  Our school year ended on June 16th, almost a week late due to  many snow days.  In the fall, Jack will be entering 5th grade, Megan will be entering 3rd grade which in our district means that she moves on to the big intermediate school, and Sophie will be starting Kindergarten, really. 

We attended the UMDF’s annual mitochondrial medicine symposium in Chicago a few weeks ago, I will post soon about the conference, but let me just say it was an amazing experience again.  Up until a few days before we left we were not sure whether or not we were actually going to be able to go, it was up to little Miss Lucy to make the call.  She was acting “off” here and there just days before leaving and that always keeps us on the edge of our seats.  Just 24 hours before leaving, we took her back to DuPont for yet another GJ tube placement.  Yep, if you recall she only had that tube for five days, it had most likely migrated out after a day or two(which was Friday or Saturday),  but I waited until Monday to schedule her a new tubie, which couldn’t be done until Tuesday.  A new tube was placed, dozens of bags packed, medical supplies galore were arranged to be shipped and some transported with us, our van cleaned…well sort of,  excusal notes for the kids to be dismissed from school at noon on the 15th were written so that we could allow enough time for the 14 hour drive to get to Chicago by the 16th, and off we went but not without stopping on our way out of town to have the oil changed in the van that had not been done since…umm, Thanksgiving.  We have had a lot on our minds recently, obviously none of which includes car maintenance.

While at the conference we learned more about a  new mito drug from Edison Pharmaceuticals, EPI-743.    It was announced just before the conference that Edison Pharmaceuticals had been granted expanded access from the FDA for this drug.  The FDA recently changed the criteria for which a patient can be eligible to participate in the study (click on the highlighted area for eligibility criteria).  Specifically, they removed the need for a confirmation of a genetic mutation which confirms a mitochondrial diagnosis.  Most mitochondrial patients do not have a confirmed genetic mutation, technology has not caught up with science and vice a versa, lifting this restriction has opened up this drug trial to many more critically ill patients.  To define critically ill, “you need to be deemed by the principal investigator (PI) to be within ninety days of end-of-life hospice/terminal care”.   As much as we hate the way this is worded, Lucy meets this criteria due to her being in intestinal failure and her 100% dependency on TPN.  Drew and I had an opportunity to meet with the PI for the study and briefly discussed our girly with him.  He highly encouraged us to send him her medical information.   We have several huge hurdles we will need to jump in order for Lucy to qualify, one her abnormal hematocrit, two her very elevated liver enzymes, and three the fact that the med needs to be administered via the GI tract with fat, aka food.   After discussing all of this over with Dr. R we all agreed that it’s a long shot, but a shot none the less.    

While away in Chicago, Drew and I celebrated 13 years of wedded bliss…ok, maybe not all bliss, but it has been amazing and continues getting better and better.  We are well aware of the statistics for marriages who have a child with special needs and are proud to say that we work hard at being in the minority.

Our trip to the windy city didn’t last as long as we had hoped, for many reasons.  We left town on Monday afternoon ironically after dropping our nurse Helen off at the airport.  We were wondering who would get home first she or us…she beat us by about six hours.  On Tuesday afternoon, I took Lucy to DuPont to have her GJ tube looked at, it was in position but clearly something was wrong with her belly.  On Thursday we brought her back to DuPont to see Dr. R to discuss some issues and talk about the study.  Labs and cultures were done, antibiotics were changed to cover some of the new bugs in her small bowel, as well as another IV antifungal to kill the yeast that is persistent.

Drew took the week off and we spent some much needed time as a family combining fun with daily responsibility.  We took the kids during the middle of the week to Dutch Wonderland, a local amusement park.  Upon leaving Give Kids the World, we were given a farewell gift, a one year family pass to any amusement park in the International Association of Amusement Parks and Attractions.   This gift was given to wish families so that they are able to “relive many of the memories created” during their stay with GKTW.   Many memories were had by all! 

We ended our vacation week with a private showing of the Cars 2 movie in 3D, courtesy of the Mid-Atlantic Starlight Foundation.  The Starlight Foundation is an organization that our family has been privileged to be a part of this past year, we are truly amazed with what they do.  

We are looking forward to lazier days…really I mean that, curling up with some good summer reads, lemonade stands, ice cream treats, sprinklers and slip–n-slides,  movie nights which hopefully lead to sleeping inn, local adventures, picnics in the park, bike rides, evening walks, pool days for most and dipping our toes in the water for some, but most of all making lasting family memories. 

Thanks for all your love and support, thoughts and prayers.  We appreciate them all!    

A day in my life…(Warning, this is not short!)

It’s 11:15 pm, I still have meds to give at midnight and I am waiting up for my husband who should land sometime around 1:00 am which means he should be home closer to 2:00 am, that is if his shuttle service picks him up on time. 

I am tired, there’s no denying it or hiding it…Megan often looks into my eyes and says, “Mommy, you have maps in your eyes.”.  Drew comments about how much I have to do in a day, telling me that I split seconds in an effort to squeeze it all in.  He called me tonight to tell me that his flight was delayed, and then proceeded to ask me what I did today.  I did not have the time or the energy to give him a recap of my day when he called and I felt bad about that,  but I still had to go to CVS to pick up a med for Lucy and then put the kids to bed.  This post is for you babe…

5:29 am - I woke up one minute before my alarm, I hate alarm clocks but had to resort to using one while Drew was away.  Yes, hate is a strong word, but I mean it when I say it, I really do hate them.  In my opinion, it’ such an unpleasant way to wake up.  I am lucky that I am married to a man who knows this about me; therefore, he wakes me in the morning, ideally twenty minutes before I really need to wake up.  I need those twenty minutes to focus my eyes and mentally prepare for the day, but  if truth be told, Drew does wake me most mornings (if Lucy or the other kids haven’t already),  giving me only about twenty seconds before I need to start my day.  Most days we are already behind schedule even before we wake up. 

5:45 am - I started infusing one of many of Lucy’s IV meds, and then quickly checked my e-mail while waiting for her first med to finish infusing.

6:10 am – I began Lucy’s second med infusion, and went downstairs to talk to my mom before she left.

6:15 am - Jack woke up early so that he could say good-bye to my mom who was leavening for the airport at around 6:30 am.

6:20 am - My mom’s shuttle service came and picked her up for the airport, a little earlier than we thought so it was a quick good-bye.

6:25 am – Jack had so much extra time this morning I let him buy the new Rick Riordan book on his Kindle that he has been waiting for. I knew that this would keep him occupied up until it was time for the bus come and take him to school at 7:55 am.  I also knew that our book worm would have a difficult time putting down his new book when it was time to go to school… I was right!

6:30 am - I switched Lucy’s meds again, and then quickly jumped in the shower hoping that Luc would not hear the shower and wake-up.  I knew if I did not get in the shower then I would not have an opportunity later so I took the chance.

6:45 am - I switched Lucy’s meds again in between getting dressed and blow drying my hair, which in hindsight was a total waste of time as it was hotter than h**l today!

7:05 am - I woke Megan up for the day being very careful not to wake the sleeping dragon  Sophie in the bed next to her. 

7:15 am – I finished infusing Lucy’s meds opting to omit her morphine dose to see how she does without it.   She is down to only .1ml for withdrawal symptoms, which she has shown little to none recently.

7:20 am - I pressed Megan’s, Sophie’s, and my skirts as they looked like wrinkled up balls of paper.  Yes, Nancy, if you are reading this I press my kids clothes!

7:25 am - Megan came to me asking if I could “just quickly” French braid her hair for school today because her class was preforming their show and she wanted her hair to be braided for the show.  As you well know but others reading this may not, Megan has enough hair for five people and we need to be downstairs by 7:30 am so that I can actually have time to make breakfast and have time for them to eat it!  Needless to say after several minutes of negotiating, Megan and I came to a compromise, I braided one small braid on the side of her head for her show.  Thank goodness she thought it looked good, you never know exactly how those last minute negotiations are going to turn out.

7:35 am - I turned into a short order cook as I prepared the kids breakfast, eggs.  The kids choose to buy lunch today which made my morning just a tad bit easier, thanks kids!

7:50 am - I threatened to take Jack’s Kindle from him if he didn’t brush his hair for the second time before going to school.  His hair was a mess in the back as he had been laying on the couch reading his book for the last hour and a half. 

7:55 am -  A mad rush for the garage door occurred as the bus pulled up in front of our driveway, kisses were exchanged, a little licking of my finger to Jack’s hair was done, and off they went!

7:56 am – A quick kitchen clean up before Lucy’s nurse arrived, because why, I don’t know it’s not like she hasn’t seen our house a mess.

8:00 am – Lucy’s nurse arrived, I surveyed the mess that is our life  kitchen, and silently laughed at myself for thinking I could even tackle the mess in four minutes.

8:05 am - Sophie woke for the day, obviously not really ready for the day. 

From the time Sophie woke up until 8:45 am I spent that time convincing Sophie to eat, get dressed, do her hair, put on her shoes… all in a speedy fashion so that she would not be late for water camp at her preschool that started at 8:30 am!

8:50 am – I grabbed my keys and started walking out the door to take Sophie to school when I heard Lucy on the monitor…ugh. I swear that child has a sixth sense and knows whenever I am going to walk out the door.  I gave instructions to our nurse to fill the med boxes, and told her that if she wanted or if Lucy started screaming she could get Lucy up for the day.  Confession, I stated the last part of my orders with some hesitation.  I love getting Lucy from her bed in the morning, there is just something about seeing her in the morning that reminds me everyday is a good day.

9:05 am - I dropped a reluctant Sophie off at preschool where the kids were already playing outside with all of the various water activities in the already 90 plus degree weather.  I kissed my girly several times and told her I would see her later in the day after she had lunch with her friend Alena, after she went to ballet and tap,  after she played at Josh’s house,  and after the kids came home from school, but before dinner. I drove away thinking that we are in need of a “day off”!

9:30 am - I arrived back home to find Lucy still in her bed. Secretly, I was happy because, as I just confessed, I love to get Lucy from her crib in the morning, and also I was hungry and wanted to eat a quick bowl of cereal before she was up for the day.  Lucy’s nurse challenged talked me into at least sitting down for a few minutes to eat instead of my usual walk around the kitchen and pick up all the while taking bites of my breakfast routine.

9:45 am - We went upstairs to wake Lucy for the day finding her and her bed covered in diarrhea.  Flagyl reeks havoc on our little one’s bowels, so much so that we sent her stool in for culture today.  We started Lucy back on Flagyl again even though we think at this point she is Flagyl resistant.  This is only because Dr. R is having trouble finding another interim antibiotic that does not require more than 2mls per dose that we can use for our girl to control her SBBO on the weeks we are not using the tobi and coli.  Lucy is unable to tolerate, really, any volume of meds in her GI tract, but we have to give her antibiotics via her J-tube to control the bacteria that is overtaking her GI system.  The meds have to come in contact with the bowel bacteria in order to kill it.

10:00 am - Laundry, laundry, and more laundry…

10:10 am – Continued with Lucy care… and infused more meds, in particular her Lasix which meant diapers, diapers, and more diapers. 

10: 20 am – I attempted to cath Lucy as her bladder was full and she was straining to pee but couldn’t. 

10:40 am - Our attempt was unsuccessful...ugh this is so HARD!

10:45 am – Lucy’s speech therapist arrived.

10:55  am - I realized that I needed to get a hold of Lucy’s nutritionist to get a copy of her labs from Monday. We are scheduled to see Dr. R in clinic at 1:00 pm and it is always helpful to have a copy of the most recent labs in hand when we go to see him. 

11:00 am - I sat down to “play” with Lucy and her therapist, all the while fielding phone call after phone call.  One of which was Lucy’s nutritionist calling me to say that things are not looking good for Miss Lucy’s Liver and now kidneys.   The other calls were calls from the various people in our life that would be helping me out with our other three kiddos today.  It’s always good to double check that everyone is aware of the schedule for the day, that’s experience talking! 

11:45 am - Therapy ended and so began that mad dash of getting Lucy’s TPN and fluid line changed out before we had to leave at 12:15 pm for DuPont.  Thank goodness for our new nurse who learns quickly, she had prepared all of the supplies while we were finishing up with therapy.  This made the task of changing Lucy’s TPN and fluid bag faster.  We did not do any med infusions as it was too early.  Instead, we packed her meds and syringe pump to do them later.

12:15 pm - We packed up and headed out for DuPont.  Only problem, the garage door wouldn't close…ugh!

12:54 pm – We pulled up to valet parking at DuPont with six minutes to spare!  

1:03 pm - We arrived at clinic only three minutes late…hey, that’s pretty good considering the amount of time and effort it takes to get Lucy out of the van and into her wheelchair.

1:10 pm - Dr. R arrived and we were called back with welcome arms for our appointment which lasted for almost an hour.  I felt like we discussed just about every organ system in Lucy’s body and what we are going to do, or rather not do about them.  Dr. R shared with me that Lucy’s TPN cholestasis is not improving and will most likely continue to get worse.  Her liver numbers were very off this week as well as her kidney numbers.  He talked to Lucy about how she needs to let mommy cath her, telling her that it will help her belly to feel better.  Lucy reluctantly agreed, then quickly disagreed..ugh!  He told me that we NEED to get going with this…double ugh!  We also discussed putting Lucy back on an antifungal as her mouth is now covered in yeast, as well as her bowels.   What we both agreed on was that her mood has most definitely improved.  Dr. R. not only gave his approval for us to go to Chicago next week , but asked me to share with him what we learn.   When finishing up he told Lucy to behave, to which she always replies “ok”, and said I’ll see you in a month…hopefully not sooner.

2:05 pm – We walked down to the lab to drop off a '”specimen” and then headed over to IR for a scheduled dye study on her central line and a GJ tube replacement.  On Tuesday afternoon after a day of increased belly pain that was obviously more intense than her average belly pain, a series of e-mails were sent back and forth between Dr. R and I, in which a trip to DuPont was deemed necessary for some belly films to see what could be causing her increased pain. Lowe and behold her GJ tube was found to be out of position.   We have gone nearly ten weeks with the same GJ tube, this is a record for Lucy!  The reason we have been able to go so long with this tube is because ten weeks ago we stopped all efforts at enteral feeding.  We are only using her J for meds, very minimal amounts of meds at that as she can barely tolerate the 5 cc flush needed to push the less than 2 mls of meds given.  A dye study on her line was ordered because her line has been slow and reluctant to to draw blood when we are drawing labs.

2:15 pm – Dye study complete, both lumens flowed freely with no clots or sheaths found.  What we did notice was that Lucy’ s line is very long inside her artery, when we pull back it sucks up against the artery wall.  The fix is to have Lucy lie down when drawing her labs so that the end of the catheter falls away from the artery wall!  I think we can manage this :).

2:19 pm – Lucy’s GJ tube change began and went on for nearly an hour.   It was by far the most difficult tube change we have ever done!  Lucy was a trooper through the whole event…everyone commented on how amazing she was and is!  On several occasions Lucy vomited up bile as her belly was so full of it it made her nauseous.  At one point Lucy announced that, “my belly no hurt!”, hoping that by saying that we would stop what we were doing.  Immediately after announcing that her “belly no hurt”, she proceeded to vomit.  Dr. F had a very difficult time placing and keeping her new tubie in position, it took several attempts to do so.  He was unable to position the J as far as we do normally,  due to the fact that her bowels kept pushing the tube backwards.  We are all hoping that this new tube will stay in position, at least until we return home from Chicago.

3:20 pm - Tube change complete, smelly stickers rewarded, high fives exchanged, and we were on our way downstairs to outpatient therapy for a  quick stop to schedule our appointment with wheelchair clinic sometime in early July.  Experience has taught me to schedule any appointments with outpatient therapy in person as they are notorious for not calling back in a timely manor.

3:25 pm – I realized that in all of the events of the afternoon I forgot to infuse Lucy’s 1:30 pm meds, and so I began the marathon of med infusions on the way home.  This meant that I had to make several stops to change out meds on our drive home.

3:30 pm- We loaded up and headed home, but not before stopping first at the hospital coffee shop for an overpriced under flavored wrap sandwich which was my lunch for the day.

4:24 pm – We arrived at home, latter than usual due to all of the stops for med changes.   Helen was waiting for us, and our other three kiddos were at Beth’s house.  I arranged to meet them at Beth’s for pizza sometime after 5:30 pm, but before 6:10 pm as we had to leave at that time to be at Megan’s school by 6:15 pm.

4:25 pm – I received a call from you saying that your received a text message from the airlines that your flight was going to delayed…ugh!

4:30 pm – Helen and I began our attempt to cath Lucy...

5:30 pm -  After many bribes, diaper changes, attempts at going on the little potty, and a diarrhea accident on the carpet later we were finally able to insert the catheter, but unable to get out any urine out as Lucy was umm… how should I  say this, uncooperative.

5:45 pm – After cleaning up the mess on the carpet, which FYI did not come out entirely, I went over to Beth’s for a quick bite of pizza and to get the kids to take them to Megan’s show at school.

6:10 pm -  We loaded up and headed home to quickly get Lucy and Helen to take them with us.  Thank goodness we only live a few minutes away from school.

6:18 pm – We arrived at school, unloaded the troops, placed Lucy back in her wheelchair, and went in to see Megan’s show when I realized that I forgot to bring my camera and video camera…oh, how could I do this!

6:30 – Showtime!  The kids in Megan’s class put on a talent show with some songs, poems, and pictures in between talent acts.  Megan chose, after much encouragement, to play one of her piano recital pieces, and let me just brag, “ she did great!”.

7:30 pm – Shows over, we again loaded up all four kids, Lucy’s wheelchair, and our keyboard and stand, and headed home.  While pulling into the driveway I bribed utilized my expert parenting skills and told the kids that they could have an ice cream treat if they showered and got ready for bed in twenty minutes.  Guess what, Sophie was not in the mood for ice cream.

7:35 pm – I unloaded the kids, the keyboard from the car, and brought in the flag and tiny umbrella,  as it looked like the skies were going to open up at any moment.  This is when you called to tell me that your flight was going to be delayed even longer, and then proceeded to ask me about my day!

7:40 pm – I went upstairs to referee the showering situation, and to tell Sophie for the umpteenth time to get in the bathtub so that I can help her wash her hair.  Megan was crying at the top of the stairs because apparently Jack “kicked” her out of the shower before she had time to wash her hair and body.  I very sternly told Megan that the point of showering IS to get clean using soap, shampoo, and water and to get back in the shower,  which by the way Jack was not in yet.  I then turned around to tell Jack that he needed to wait for Megan to actually get clean before taking his turn, and to use soap and shampoo, too.

7:45 pm – I helped Sophie to get clean and ready for bed, and it only took ten minutes!

7:55 pm – I left the kids with Helen while I quickly ran to CVS to get Lucy’s antifungal that Dr. R called in for her earlier in the day.  When I picked up the med I noticed the script read, “give 4 mls by mouth four times daily”…really?, then gasped at the amount of medicine in the large plastic bottle, politely asking the pharmacist if in fact  these were the instructions given by Dr. R for Lucy.  His reply, “Yes, why?”

8:04 pm – Three of the four kiddos were ready for bed.. not too bad, if I do say so myself!

8:15 pm – I began an e-mail to Dr. R asking him to clarify what he “really” wants me to do with the med he prescribed Lucy.  At our earlier appointment we discussed swabbing Lucy’s mouth with some of it and also putting some down Lucy’s j-tube.

Around 8  pm – Helen started the next marathon of Lucy’s meds so that she could would be in bed sometime before you arrived home tonight.  For the record, Lucy had only slept five hours of the past 24…I feared she was beginning a no sleep pattern again!

8:30 pm – Bedtime for 3/4’s of our kids!  

8:41 pm – I actually sent Dr. R my e-mail I started earlier, hoping that he would read it tonight and respond back.

8:51 pm – Dr. R responded back to my e-mail and clarified that he wants 2mls to go down her J-tube and swab her mouth with the remaining 2 mls.  Ok, much easier said than done!

Shortly after 9 pm – You called to tell me that you thought your flight was going to leave sometime after 10 pm.  I sighed, and you said, “ at least my flight isn’t cancelled, many of the flights leaving for the East coast are.”.

9:30 pm – I contemplated attempting to cath Lucy again, but after gagging swabbing Lucy’s mouth with Nystatin I made an executive decision to skip it, plus I was too exhausted and so was Helen.

Helen left sometime between 9:30 and 10 pm

You called to tell me that your flight was leaving at 10:20 pm and told me not to wait up for you.

10:00 pm -  I went upstairs to attempt to put Lucy to bed.  She went to bed crying and complaining that her “berry hurts”, too many meds down her J-tube.

11:00 pm – After numerous backrubs, books, diaper changes, rearranging of blankets, babies, doggies, pillows… Lucy finally fell into a restless sleep.

In between my many attempts to put Lucy to sleep, I began the usual pick up from today and get ready for the next day routine.

11:15 pm – I gave up on picking up and choose to write this insanely long blog post so that I could stay awake to see you when you came home tonight, or should I say this morning. 

12:00 am – I administered Lucy’s midnight meds and then proceeded to put Lucy back to bed for what felt like the 100th time.

1:45 am – You arrived home surprised to see me still awake, laughing when you saw what I was writing. 

2:30 am – We were too tired to talk anymore and fell asleep passed out!

Our Graduate…

Let me preset to you the future graduating class of 2024…whoa!

From Rapunzel to Pixie…

Once upon a time there was a beautiful little girl who had beautiful, albeit long, thin, straggly, red locks of love.  As beautiful as her hair was, it was becoming increasingly difficult to manage.  Stress and  a lot of time on her back made for some umm…pretty bad hair days.

A declaration was made that she will have her first hair cut just as soon as her mommy could handle it.  Months later (some decisions are harder than others) it was decided that said hair cut will take place on a Friday afternoon at the end of May, on our kitchen island, with a wonderful, sweet, kind, hair dresser who volunteered her time to cut our little princess’ hair.  Thanks Sherrie and Helen for arranging this! 

When all was said and done Rapunzel was transformed into an adorable pixie!  Isn’t she beautiful! 

Oh My…

Where has the time gone?  Truth be told, I have been working hard to catch up and move forward with life.  One problem, there just doesn’t seem to be enough hours in the day for me to do all of the things I need to and want to do…like sleeping and blogging.  Due to my lack of posts and scattered mind, I am going to revert to list format as a way of catching up on all of the events medical and non medical.

1. After discussing the results of Lucy’s urodynamics study over with both her urologist and Dr. R it was decided that we are going to have to “help” Lucy’s bladder empty.  In other words, we are going to start straight cathing Lucy every four hours.  Last week Tuesday we met with the urology nurse practitioner for an informative appointment on the in’s and out’s of straight cathing…pun totally intended.  Technically, straight cathing isn’t very difficult compared to many of the other things that we have to do to Lucy, emotionally it is!  As with all things, we will get used to it, it’s just going to take some time.  As for now we are working on getting Lucy comfortable with the prep process, so far she is doing much better than any of us expected she would.  On Wednesday, when our delivery of urology supplies comes from yet another medical supply company, we will officially begin the cathing process.   At the present time, surgical options for inserting a drainage tube or pulling the bladder to the skins surface to create a “port" of sorts is too risky for Lucy and has a higher rate of infection.  Straight cathing has the lowest risk of infection and is the least invasive of all of our options for emptying Lucy’s bladder.  Since infection is what we are trying to avoid we are going with the option that has the least risk of it.

2.  Last week was full of the usual things and then some.  We officially started our new nurse on Tuesday, and so began the process of having her learn all that there is to know about Lucy and the rest of the gang.  As you can imagine there is a lot to catch her up to speed on, but I have a good feeling that she will learn quickly.  Nonetheless, it is a process of getting to know one another and how things work around here and as a result I am a little more tired than usual. 

3. Over the weekend Drew and I, ok… really more Drew than I, accomplished a huge garage shelving project, way to go babe!  We have been wanting to do this for quite sometime (five plus years, but who’s counting) and finally set forth and completed it!  Several things were motivating us to do this, but the driving force is the need to make our house more handicap accessible.   Lucy’s needs are changing, and as a result so is her need for a new wheelchair.  We need to build a ramp from our garage into our house in order for her chair to be used indoors, hence the need to get all of the stuff off of our our garage floor and onto shelves.   I love her current chair, it looks more like a  souped up stroller than a wheelchair, but her medical needs have literally outgrown her chair.  We hang all of her pumps and bags in one backpack off the back of her wheelchair.  When they are full, the weight of all of those items can tip her chair over backwards if you are not careful.  I have been putting off scheduling an appointment with wheelchair clinic mainly because I know that once we order her a new chair so comes our need for a handicap accessible van.

4.  It’s the end of the school year and with that comes all of the end of  school year festivities.  It is bitter sweet, I am looking forward to having a looser schedule with the kids at the same time I am worried that things with Miss Lucy’s schedule are going to get more challenging with the kids being home.  My busiest time of day is from 12 noon to 2:30 when we infuse meds, change Lucy’s TPN, and put Lucy to bed for the afternoon.  This school year seems like it has gone faster than any of the years before, I suspect that I feel this way partly because of the circumstances of our life, but also our kids just seem to be growing up so fast. 

5.   Summer plans are being made, well…as much as we can plan anything in our lives.  As we all know Miss Lucy can really mess with your plans in a wink of an eye, yet I foolishly optimistically attempt to plan a few things.  We are hoping to be able to attend the UMDF’s annual mitochondrial medicine symposium in Chicago next week. Yikes, is that next week already!  Last year Drew and I attended this conference for the first time.  In just a few days we were able to meet with and listen to some of the top doctors and researchers in the field of mitochondrial medicine describe what they know and are researching about the disease.  In the mito world, this conference is well worth your time and money if you are seeking to learn as much as you can about the disease.

6.  Drew is traveling this week and my mom is here to visit and help me with the kids.  I never really get to spend anytime with my mom when she comes as I am always at the hospital with Lucy and she at our house with the kids.  It is nice to be able to spend some time with her, even if the time we have to talk is late in the night. 

Times up, I need to get the kids up and ready for school.  I want to apologize for my lack of posting, and thank all of you faithful readers for all of your love and support, thoughts and prayers.

Tonight is preschool graduation night…I will post some pics soon.