Not your typical Thursday…

We are currently sitting in day medicine watching blood drip ever so slowly into our baby girl.  Dr. R  came over to discuss the details of the day, and reminded me again that transfusion is really our only option for alleviating Lucy’s cardiac symptoms.  On Christmas Eve we received a call from Lucy’s cardiologist.  The results from Lucy’s Holtor monitor revealed what we suspected.  Lucy is tachycardic (high heart rate), and her tachycardia increases even more with changes in position (orthostatic).  Lucy’s heart rate issues are as a result of her autonomic dysfunction, which is affecting her sinus node.  They have diagnosed Lucy with sinus tachycardia, and postural orthostatic tachycardia syndrome, POTS.  We discussed medications, specifically the use of beta blockers to help control Lucy’s cardiac symptoms; unfortunately, the side effects of the medicines would exasperate many of Lucy’s mito symptoms.  Beta blockers are heavy duty meds for “normal” people, but for a kiddo like Lucy they are very risky. 

Last week we learned, after several discrepancies, that Lucy’s blood type is not the same as mine or Drew’s.  Lucy is A negative, one of the rarer blood types in Caucasian people, leave it to Lucy to be different.  Because of this, we were unable to do a directed donor blood donation.  Lucy is receiving packed A negative red blood cells from the Delmarva blood bank, many thanks to those of you who donate blood!

After several attempts, IV team placed a peripheral IV line in Lucy’s left foot. It’s not pretty, but it gives us a point of access to transfuse the blood.  We are able to keep Lucy’s TPN running at the same time as the blood, which helps us avoid the whole hypoglycemia issue.

UPDATE

Everything went as well as could be expected today.  Lucy’s body appears to have tolerated the whole procedure.  It’s too early to say for sure, but it looks like her heart rate has come down slightly, we have not seen too many numbers above 170.  We will monitor her closely for the next few days so that we can get a better idea of where her numbers fall.

Many thanks to all of you for your thoughts and prayers, we feel so supported!  We would especially like to thank the brigade of loving friends who watch over our three other children so that Drew and I are able to sit bedside with Lucy.  Your love and unwavering support means the world to us!       

The Holiday Rush…

Christmas is coming, and with four little ones in our house you can imagine the excitement brewing around here!  Letters to Santa have been written and rewritten, finalized, and sent.  We have a visitor from the North Pole who has taken to hiding in various places around our house.  Jack thinks he is a spy of sorts, Sophie asked if he was a tattle tale, nobody wants Lucy to touch him because it is believed that he will loose his magic with the touch of a child’s hand, and Megan believes in the magic so much that she was upset when our little Elf on a Shelf was found to be in the same spot two days in a row.  She thinks that I ruined the magic because I set him on our mantel the first day he arrived, but what she didn’t realize is that the magic is not lost if a grown up touches the Elf on a Shelf, right!?!  Apparently, the Elf on a Shelf returns to the North Pole every night and then returns back to our house only to be found in a new location, his mission is to report back to the man in the big red suit all of the good, the bad, and the ugly.  So far, so much fun!

Most of our shopping is complete, our house has been decorated since the Saturday after Thanksgiving, and believe it our not I have most of our packages wrapped(a first for me).  I was on a mission, and a bit of an adrenaline high, after coming home from the hospital with Lucy the day after Thanksgiving.   I knew we had to get things done because you never know what life will be like in our house.

We are feeling the holiday rush in the medical world, even though sickness never takes a vacation, the people that treat it do.  We have made some decisions for Lucy this week that are impacted slightly by the holidays.  We are scheduled  next week Thursday to have Lucy transfused.  We are putting off doing it this week  because we want to be home for Christmas.  She has been anemic for a while but is showing more and more symptoms of anemia, and at this point we all feel that there is the potential to alleviate some of her symptoms  with blood transfusion.  Lucy’s body is not able to make enough new blood to make up for the loss from weekly blood draws.   This decision has not been an easy one to come to for us or Lucy’s doctors.  Our biggest concerns are vein access,  her hypoglycemia, and the risk of reactions.  Our hope to is to do a directed donor blood transfusion, which means that Lucy will receive my blood.  This is a process, that’s stating it mildly, but if it means that the potential for fewer reactions is there then we are willing to jump through the million hoops to get it done.  Of course the holidays fall right in the middle of our five business days to get things processed.  I have faith that things will work out…they have to!

In addition to a transfusion, we started Lucy on oxygen therapy.  I know…just what we don’t need more tubes!  Her pulse ox numbers are all over the place and her behavior has been…um…unusual for Lucy.  She is acting more antsy, shaking and bouncing her head on things, waving her arms in the air, and pacing back and fourth on her wobbly legs, we think that she is doing this because for some reason it helps her feel better.  Dr. R and I have had several conversations about this and we feel that her increased activity is moving oxygen through her body faster; therefore,we are starting oxygen therapy to see if it helps her to feel better and also stabilize her pulse ox numbers.  Mito is a disease in which the body does not convert the food you eat or the oxygen you breath into the life sustaining energy your body needs to function. Anybody have any suggestions on how to get a two year old to keep on a nasal cannula?

Yesterday, we had an impromptu visit to DuPont to get Lucy’s belly scanned yet again, and also to have her blood typed and screened, one of the many hoops that needs to be jumped in order to get her transfused.  While we were at day medicine getting blood drawn from her line, Dr. R came over to take a quick look at our girly and her bile output.  On Monday Lucy had close to 600mls of thick, dark green, smelly, bile pour into her drainage bag.  We are again dealing with small bowel bacterial overgrowth(SBBO) and this time it is back with a vengeance, we have only been off of an antibiotic for one week.  We are putting Lucy on a new regimen of  antibiotics, what Dr. R is calling the “big guns”, these meds are generally given as IV meds but we are going to be mixing them and administering them via her j-tube.  Speaking of her J-tube, another KUB scan of her belly revealed what we were afraid of, her J-tube that she has had in place for all of six days had migrated out of her jejunum and into her stomach.  We suspected this because of the  dramatic increase in bile output and also because when we administered Lucy’s meds through her J-port meds came pouring out her G-port into her drainage bag.  Ugh…for the third time in three weeks, we had to have another J-tube replaced in IR.  This placement was by far the worst we have had to go through, as Lucy’s belly did not empty at all for the procedure and they had to suction out the contents in her stomach all the while trying to place a new tube in position.  We are going to discuss with Dr. R what our options are for tube placement and come up with a better plan than what we are doing currently, weekly trips to IR.

In addition to all of the medical drama around here my parents, my brother, sister, brother in-law and the twins came  for a visit.  We have thoroughly enjoyed their visit, in spite of Lucy’s newest medical issues and Megan being home sick with a fever since Monday morning.  Tomorrow morning if Megan’s fever still persists, then I will take her in to have her looked at.

Tomorrow is the last day before Christmas vacation for our kiddos!  We are planning a surprise for our kiddos tomorrow night that I will tell you about later.  For now, I am going to leave you to digest all that I have written, and thank you all yet again for all of your thoughts and prayers.

More appointments…

Today was filled with even more appointments, this time at DuPont.  Last Thursday when we met with Dr. R it was determined that we needed to see Lucy’s cardiologist a.s.a.p.  We received a call yesterday saying that they could see Lucy today at 1:30.  I know, I know, we are gluten for punishment.  Two days in a row where our girly had to forgo her typical four hour nap, it has been rough, and not just on her.  However, seeing cardiology was of the utmost importance so we took it.  Lucy has been dealing with tachycardia since the beginning of November, her average heart rate is in the 160’s, much higher than the average 33 month old’s heart rate.  Her dusky blue spells and mottling have only increased and her pulse ox numbers are just plain crazy.  We had a repeat EKG and echo today because it is apparent that something has clearly changed since her last set of tests in October.  On exam Dr, B, Lucy’s cardiologist, detected a very loud murmur when Lucy was upright, but when she was laying down she was unable to detect it.  She told us that this could be because Lucy is anemic, apparently this type of murmur is a symptom of anemia. 

Lucy’s EKG showed her to be tachycardic, but thankfully her echo was normal .  Dr. B agrees with Dr. R that Lucy’s cardiac issues are related to her autonomic dysfunction, and not her heart muscle per say.  She is concerned that Lucy’s heart muscle is tiring out, as some of Lucy’s lab work is indicated this.  She is describing Lucy as having sinus taychcardia.  She is monitoring Lucy’s heart rates over the next 24 hours with a Holter Monitor.  When the results are back, she and Dr. R will discuss what the next steps will be.

As if we have not had enough medical appointments, we had an urgent need to go back to interventional radiology (IR) to attempt to fix Lucy’s J- tube.  Last night we were unable to push in or pull back anything with Lucy’s tube.  I spoke with Dr. R this morning about this and all of our appointments yesterday,  and he put the orders into IR today.  Fixing the tube was no longer an option when, while trying to transfer my baby to the IR table with all of  her numerous tubes and wires hanging off of her body, I accidently snagged her J-tube extension on her wheelchair and pulled the whole thing out.  Anyone want to nominate me for mother of the year?  We had to go ahead and replace the tube.  Drew had never been through a tube placement before, and for good reason.  Let’s just say that he did well for the most part, but had an encounter with the floor that left him needing some medical attention himself.

It’s late and we are tired, too tired to go into detail about our day at CHOP.  I will write later about those appointments.  Thanks as always for your thoughts and prayers.       

Appointments…

Last week Thursday we had through and emotional appointment with Dr. R….I am working on a post about that appointment that I will post when I am finished. 

In the mean time, we are scheduled to see the top motility specialist at CHOP today.  We were originally scheduled to see this specialist on October 26th…well, we know what happened then.  We are hoping that they will have some valuable insight into Lucy’s issues.  This will be the first time that we meet with this doctor, please pray that Drew and I are able to convey all of Lucy’s pertinent medical information to this new doc.  It is never easy for us to meet with new doctors, Lucy’s medical file weighs more than she does!

We also have an appointment with Lucy’s metabolic doctor today.  CHOP aspires to be one of the top mitochondrial research centers in the country.  They recently recruited one the top mitochondrial researchers in the field (Dr. W), and we are hopeful that life saving research so close to home will eventually be in the future.  As for now, we will continue to follow with metabolism in hopes that we can have access to some of this life saving research.

Lastly, we are meeting with the Pediatric Advanced Care Team (PACT) at CHOP.  They provide palliative care, “Pediatric palliative care is a comprehensive approach that focuses on the psychological, social, and spiritual needs of patients with life-threatening illnesses and their families.'”  We are looking to the experts to help guide our whole family through some of the varied emotions and experiences we are facing with Lucy’s life.

It is going to be a long day…we know that! 

Thank you in advance for all or your thoughts and prayers!  For all you prayer warriors out there here are some specifics that you can be praying for.  Please pray that it will be a productive day, that Lucy has the ability to tolerate such a busy day, that Drew and I are able to advocate for our daughter and family effectively so that our needs will be meet.

Some things…

I know, I am behind in updating.   A friend asked me recently if no news was good news?  I think, that recently, no news means that I have been too tired to blog and I don’t know how to put into   words all of the things that have been going on in our world.  I want so badly to write about nothing…about mundane everyday things… about how the stress of the holidays is overwhelming right now.  In reality, the stress of the holidays is a welcome stress, it is something that most everyone is feeling at the moment, and sharing feelings that most people are experiencing makes us feel “normal”.  It is something that we can talk to others about, because we know that what we are living with, feeling, and experiencing is so very different and difficult to talk about.

We often get asked how we are doing, Drew and I, and how the other kids are handling things.  It is impossible give a simple one word answer to this question. There are moments where I want to shout to the world “don’t you know our daughter is very sick and ‘blank’ doesn’t matter” and other times I just want to go on living like none of “this” is even  happening. Some moments are better than others; but, overall we are all leaning on one another, gaining strength from one another.  I love how when one of us is down there always seems to be one of us who is there to pick the other one up, I guess you can say that this  is one of the many blessings of having a large family, someone is always there for you when you need them and vice versa. 

Again we were at DuPont yesterday, this time to get yet another KUB (kidneys, ureters, and bladder)scan  of Lucy’s belly.  Dr.R wanted this scan to examine Lucy’s bowels, tube placement, and to see how constipated she is.   Lucy has been experiencing increased belly pain in the last few days that has been consistent; in short, she is miserable.  Her bile output is yucky, and she is vomiting when we try to push her meds into her j-tube.  When I spoke with him later in the day in regards to Lucy’s scan he said that she has stool that is impacted on her left side but is not totally impacted, however, it needs to be remedied.  By this he was telling me that we need to go back to using enemas and suppositories to get things moving and keep her from getting totally impacted.  Ugh…this is a regimen that neither she nor I like to do.

In addition to all of the belly pain, Lucy’s autonomic dysfunction has progressed, Dr. R is describing Lucy as having  an “autonomic storm”.  Her pulse ox, which measures the amount of oxygen in her blood, is all over the place but lower than we have ever seen it.  Her heart rate continues to remain high, and her coloring well…she looks pretty pale.  She is having more periods where she looks dusky and her skin is mottled.  Discussions about transfusion and just how exactly we are going to do this for Lucy were had.  Vein access and hypoglycemia are some of the hurdles to getting Lucy transfused. 

We are scheduled to see Dr. R tomorrow,  my list of things that we need to discuss with him is growing every day.

Thank you as always for your thoughts and prayers.        

So much change, so little time…

To write that is, life has been busy in the Marlett house.  I want to apologize to anyone who has called, e-mailed, texted, or tried to communicate with us in, let’s just say,  the last month or so, it has been difficult to find the time to talk , type, or get together.  Please, please, know that we love hearing from you and  will get back to you…soon, I hope.

There are so many thoughts and feelings floating around my head,  sometimes I worry about sharing too much, as a way of protecting everyone around us from some of the intense and varied emotions that accompany what we are living with.  On the other hand, it is the intense emotions that make our life our life. 

We have come to expect challenges everyday… and challenges are what we have been dealing with on so many levels.  For instance, today, Thursday our craziest busiest day of the week, we had an urgent need to get to DuPont to have Lucy’s GJ-tube replaced in IR because her J-tube migrated out of her intestines and into her stomach, causing our girly to have many issues.  We have been dreading a tube change for Lucy because for Lucy it is a very traumatic experience, add to that the fact that we could not use any sort of sedation medication and it made for one stressful afternoon. 

Tomorrow’s another day, and hopefully I will have some time to write more.  I will say that we are adjusting to some of the new challenges in our life and that feels pretty good.  The kids are really doing well and are adjusting to all of these changes,each in their own way.

Were going home…soon

After 48 hours, nothing bacterial is growing in Lucy’s blood cultures, which means she does not have a blood infection, which in turn means that we can go home!  We are treating Lucy for bacterial overgrowth in her intestines…again.  It appears that we are going to have to do 15 days on and 15 days off with the Flagyl in an effort to keep things under control.   We are leaving knowing that we will follow up with Dr. R on Monday in regards to transfusion and blood pressure issues.  Dr. M who was the on call doc this admit believes that Lucy’s issues are related to her autonomic dysfunction too, it is because of the mito. I asked him how long do we let her heart work so hard before we get concerned, he answered we are concerned and we will address her issues empirically.

We are waiting on our infusion company to deliver her supplies and then we are going home.  Thank you for all your thoughts and prayers, we are so very grateful. 

Thanksgiving update…

The nurses on the floor are teasing Lucy saying that she just wants to spend all of the fall holidays with them, first Halloween and now Thanksgiving.  Just when I thought weekends were desolate here, you should see it on a major holiday, it is down right deserted.  We have yet to see any doctors make rounds today.

We are making the best of a less than ideal situation.  Lucy and I have done a lot of cuddling and movie watching, she is loving Tinkerbell and Nemo, who knew!?!  Lucy just melts into you when she is sick, she really just wants to be held by her mama, and I am more than happy to oblige.  Last night was a busy night as far meds go, she is on two different types of antibiotics, one every six hours and one every eight hours.  At 5 am the nurses come in to draw labs so that the doctors can have some of the results back by the time they come to do rounds.  This means that our day starts off bright and early.  Lucy is acting less sick, which has me worried that the antibiotics are indeed working, and this could possibly mean that she is dealing with some sort of infection.  Dr. F called me last night to discuss our exciting entrance into the ER, and then to share with me that Lucy’s stomach bile is a bacterial soup.  We’ll just have to wait and see what the cultures come back telling us.  

(I give up trying to keep her lines from getting all knotted.)

Lucy and I watched a little of the Macy’s Parade this morning until she fell asleep.  She kept pointing to the T.V. saying “I come”, she wanted to go and see the actual parade.  I think she was remembering the parades we saw in Disney last year, at this time.  What a difference a year makes…

Drew, my parents, and the kids are all at home preparing a Thanksgiving day feast to bring here to the hospital later on today.  If I don’t think about what I am missing out on, and look instead at what I have right in front of me at this very moment, I am better able to handle this…We have SO MUCH to be thankful for this season! 

Fevers and so many other things…

Oh where to start?  Lucy has been “off” for a while; honestly, I’m not really sure she has been “back on” for months.  Since being discharged just shy of three weeks ago, things have really been difficult for her, and in turn, us.  She has had on again off again fevers but nothing over 100.4, well…except for the time she got her flu vaccine and shot up to 101.2,  I will not go into detail about the lecture I received from Dr. R about sitting on that fever or any fever for that matter.  The rule with any fever >100.4  in a kiddo with a central line  is to first call Dr. R  and have blood cultures drawn,  then get  admitted to the hospital for a minimum of two days, next start IV antibiotics while we wait for blood cultures to come back to tell us what if any bacteria is has grown in the culture, and lastly formulate a plan from there.  The biggest risk with kiddos who have central lines is infection, any infection can be detrimental; therefore, you need to react to any and all fevers!  Lucy’s fever is above Dr. R’s threshold. 

On Monday of this week when our infusion company nurse came to do Lucy’s weekly lab draw, we all commented on how dusky purple Lucy’s mouth area and tongue looked.  She looked quite pale ,and was having issues with balance and stability.   Our infusion nurse was very concerned, and after taking Lucy’s blood pressure he observed that Lucy was having orthostatic hypertension, her blood pressures where changing more than 10% when she went from sitting to standing.   Like I have mentioned, Lucy has been having episodes of looking “bluish” along with being tachycardic, but she has not appeared in any sort of respiratory distress.  We have discussed this issue with Dr. R and Dr. F on several occasions and Dr. R believes that Lucy is dealing with vascular dysfunction as a result of her body’s autonomic dysfunction.  Lucy is also anemic, her labs have been indicating this for a while, but it is more concerning now as we are seeing symptoms in her that could be a result of her anemia.  After talking with Dr. R about Lucy’s symptoms on Monday, it was decided that we should try an IV bolus of normal saline to see if that would help to alleviate some of her symptoms, mito kids require a lot more fluid for some reason.  We also discussed the need to give Lucy some blood.  We ran fluids yesterday, and are still discussing the need to transfuse her.

My morning started off with a trip to our our ped’s office, she and I have been conversing regularly about how our girly is doing and what her most recent labs and symptoms possibly mean.  She and I decided last night that it would be beneficial to bring Lucy into the office for her to take a look at her.  Knowing that we were heading into a long holiday weekend and feeling uneasy anxious about Lucy’s symptoms, I thought it would be worthwhile.  While in her office, Lucy looked sicker than we have seen her in a few weeks, and her fever started climbing.  Her autonomic dysfunction shined in all it’s glory when her forehead temp read 100.4 degrees and her feet 72 degrees, five degrees colder than the rooms temperature.  Dr. F and I agreed that things were not looking good and called Dr. R give him an update.  Without hesitation he told me she needed to be admitted. 

Lucy and I headed home to grab our necessities for a few days, and make arrangements with my mom and dad who happen to be here for the week (what luck).  Dr. R told me not to take too long as he felt that Lucy’s symptoms could go from bad to worse quickly.  It took us a little over an hour to drive to the hospital today due to all of the holiday traffic.      

When we arrived at the ER a few hours later Lucy was extremely floppy and not very responsive, I checked her TPN pump which had an error message on it.  Immediately, I panicked and used her glucometer to check her glucose.  She was very low, 32 to be exact, and was in trouble.  I have not had to deal with an error on her pump like the one that it was reading, but I knew I needed to get in contact with our infusion nurse to figure out how to fix it so that we could get Lucy’s TPN running again.  Lucy’s glucose drops quickly, within a matter of minutes, she really can not afford to be off her feeds.   In a matter of a minute, what seemed like the entire ER staff was in with Lucy trying to get her glucose back up.  I trouble shot with our infusion nurse over an outside line in the ER, because of course I could not get any cell phone reception in the ER, all the while watching the ER team bolus Lucy 60mls of D10 straight into her central line.  Within ten minutes Lucy’s glucose rebounded nicely and things became a little less critical.  I was able to get her TPN pump working again and we could then address the real reason for coming in today.

Cultures are done, labs have been drawn, we are up on the floor in a private room (prayers big and small get answered for us everyday).  Lucy is running two different antibiotics in conjunction with D10 via her central line. Her TPN is being given when she is not running IV antibiotics.

Drew and I had dinner together at 9:00 after we got things settled for the day.  Plans are being made to spend Thanksgiving with kiddos tomorrow.  I will update more tomorrow, as I am exhausted and Lucy is sleeping.

Your thoughts and prayers are greatly appreciated. 

Back at DuPont…again

We are currently sitting in the ER at DuPont waiting for a bed on 3F.  Lucy is being admitted because she has been running fevers all day…

Will update more when I have time.  Your thoughts and prayers are very much needed.

Just an update…

Really not much has changed since I last posted.  Lucy continues to have all of the same symptoms, her temps have managed to stay below 10o which indicates to us that these temps are related to her autonomic dysfunction, her body’s inability to regulate processes that the body does automatically.  We aren’t surprised to see her responding this way, her body is really trying hard to adjust to all that it has going on.  Her resting heart rate remains high, in the 160’s, which we know is wearing her out.  We are seriously looking into finding some meds to control and manage her pain better.  Lucy’s body does not metabolize meds normally which makes finding a med that works difficult, currently we are using Tylenol with Codeine because…its working.  We met with Dr. R on Monday after e-mailing and talking on the phone a lot over the weekend.  It was an appointment in which we discussed how “sick” our little girl is and how we want to give her “quality of life” at the same time do all that we can.  I think that for anyone who has not walked in our shoes an appointment like the one we had on Monday would have been awful, oddly for Drew and I it gave us a sense of peace.

We are so very grateful for each and every thought and prayer being said for our little one and our family.  We are in awe of the impact that our little one has had one our lives and the lives of so many others.  When I fist laid eyes on Lucy  I stated “ this baby was born for a purpose!”, little did I know!

Not really sure…

I won’t lie, things have been stressful.  We are worried about our little one.  We don’t know what it is that is exactly causing her issues, the NOT KNOWING is so hard!  On the outside, Lucy is beautiful, dare I say “perfect”. 

Her personality shines on most days, and so too does her pain.  We have been on sort of a wild goose chase trying to hunt down the cause for Lucy’s symptoms.  She continues to have all of the same symptoms she had when I last posted, but add to that list bloody, mucusy diarrhea and a ripe yeasty diaper rash.  We tested her for Flagyl resistant c-diff (a bacteria that is contracted in those who have been on antibiotics and in the hospital) and thought for sure this is what she has, her tests are saying otherwise.  We are, however, continuing her on Vancomycin, the antibiotic used to treat Flygal resistant c-diff, for a few more days to see if her symptoms don’t improve.  So far they have not.  Our pediatrician and Dr. R have both been following Lucy’s symptoms and are very concerned.  Lucy’s body is not responding with an elevated white cell count, and this has everyone worried that she is so immune compromised she has very little to fight with.  The biggest concern is that her bowels are slowing down as a result of her GI dysfunction.   I am almost too afraid to ask her doctors what that means.  Dr. R said that it is really up to Lucy’s body to tell us what it will do.  He is most afraid of her bowels translocating bacteria into her bloodstream and thus infecting her body.  We all agree that keeping her out of the hospital is safer than admitting her, at least for now.

Lucy has declined so much in the last month it is difficult to define what her baseline is.  She is on so many new medications, has a central line, and is 100% TPN dependent it is nearly impossible to say what if any of those things might be contributing to her symptoms.  The plan for tonight is to continue to do what we have been doing and report any changes in Lucy’s symptoms to Dr. R tomorrow. 

I know I say this a lot, mito is such a baffeling disease.  Your thoughts and prayers are very much needed.    

Update…

Chest x-ray came back normal, thank goodness it’s not another aspiration pneumonia.

CBC mostly normal, nothing too out of the ordinary.

Electrolytes good for the most part, a few things that are low but nothing that would be causing Lucy’s symptoms.  Albumin was a little low,  we will most likely need to tweak her TPN a little.

Blood cultures are done, and will take approximately two days to get any results from.  We are hoping and praying that it is not an infection brewing. 

We are scheduled to see Dr R on Thursday.  Hopefully it won’t be sooner than that.

It is 1:30 am and we are all home and soon to be sleeping in our own beds.  We are grateful for the staff and our doc at DuPont who know us and trust that we will be in touch with them if our girly does not improve.  

Back in the ER…

Not really sure what is going on, but Lucy has been “off”.  She has had an on again off again fever, she has a higher than normal heart rate (tachycardia) which is giving her pulse ox and heart rate monitor a good workout, and she has had several episodes of looking blue and mottled.  Something is just not right…

We will update when we know more.

This is really hard…

We have been living day by day, sometimes hour by hour recently, which is NOT a typical way of living for me.  I am a planner, a quintessential type A personality, but with perspective.  I like to think that having perspective helps to tone down some of the annoying characteristics of a typical type A person.  We were in-patient for seventeen long, very stressful, extremely emotional days, and when we finally saw a light at the end of the tunnel, we became anxious and may have rushed the process just a bit.  There is A LOT of preparation that needs to be done to take care of Lucy and I don’t think that we realized until we got home last night just how unprepared we were. 

We have an infusion company that is in charge of getting us all of Lucy’s new medical supplies.  They also have a wonderful infusion nurse, who we just happened to meet way back when we did the NG tube movie for DuPont.  He is assigned to our case and met us at the hospital and at home last night.  His role is to help us with all of the care and treatment of Lucy’s central line and TPN.  Medical supplies galore have been delivered to our house and we need to get organized quickly so that I can keep my sanity, and also so that the things that need to stay sterile don’t accidentally get opened by curious hands.  We had to get a separate small  refrigerator just for Lucy’s TPN because you can’t store TPN on a shelf with any other items.  Lord knows every single inch of refrigerator space in our house is always occupied.  Our changing table is now our official TPN table.  I wiped the entire thing down with rubbing alcohol, and the new mini refrigerator, and the bins in which we store the TPN…My hands are raw from wiping and sterilizing, washing and sanitizing.

In addition to organizing our home for Lucy’s medical needs,  we needed to organize a med schedule for our little one.  She is currently taking ten different medications dosed two or three times daily.  In the hospital they were dosing her on a 24 hour schedule which works well when you have nursing care around the clock, we don’t have that at home so we had to create a new med schedule to meet our needs.  Thank God for our nurse Helen, she has taken on this responsibility with a little help from Lucy.

Of course in addition to all of the organizing, sanitizing, and stressing, I still had to do all of my usual mothering activities which I have missed but have to admit was a bit overwhelmed with today.  It is going to take some time to adjust to all of this… 

Home Sweet Home…

After a loooooooong day, we arrived home around dinner time.  Drew and I chose to surprise the kids with our home coming, I just love a good surprise!

The enormity of our situation hit me when we were loading all of our stuff and our little girl with all her tubes and accessories into our van in the pouring rain.  Life is different now, we will adjust, but it is going to take some time.

We want to thank all of you for your support, meals, babysitting, carpooling, phone calling, visits to the hospital, lending us a shoulder to cry on, thoughts, and prayers.  We could not have made it through this without you.

It’s in…

Lucy’s central line is in and after several hours of searching for the final radiologists report, we are now able to use it!  They left Lucy’s femoral line in so that they could use it during her procedure.  Thank God they did because we would not have been able to feed Lucy her TPN this afternoon since we did not get approval to use her new line until 5:30 pm.  Although, something tells me that they would have found the radiologists report sooner if that was the case.  We chose to place a single lumen (one port)  Broviac because the risk of infection and vein damage was less than if we placed a double (two ports) lumen Broviac.  Dr R thought that if/when Lucy gets and infection and her need for two ports becomes necessary, then we will most likely replace the line for a two lumen Broviac.  For now we are going to do our best to minimize her risk of getting an infection.

    

Lucy’s procedure went well, and she was in and out of the OR  in just about two hours.  They let me dress in scrubs and take her back to the OR  to help ease her transition,  I think we all appreciated that.  The anesthesiology team that worked on Lucy told us that it took A LOT of Propofol to keep her still during her procedure.  They wanted to get her out of the OR as soon as possible since they kept having to administer more medication.  According to every anesthesiologist we have meet, apparently red heads are are difficult to put to sleep.  I can attest to that as a non anesthesiologist. 

 

Lucy was wide awake when we were reunited with her after her procedure, and she stayed wide awake until 5:20 pm, even with all of the medication she had today.  Lucy is one strong willed little girl! 

Just after we finally got Lucy to sleep, the residents came to us telling us that they  had to pull out her femoral line.  My response, “are you kidding, let’s wait until she wakes up.”  I stood my ground and told them that they would have to wait for her to wake up.  Their response, “she might just sleep right through it.”  Thank God the resident who has been assigned to us during this admission knew enough about our girl to say, “not Lucy, she is a very light sleeper.”  It was eventually agreed that Dr R would pull the line in the morning.

Thank you all for your thoughts and prayers, care and concern, it means the world to us!

Dear God…

It’s me Lucy…Please watch over me as I get my central line placed today.

Silly Goose…

We love you girly!

What are we waiting for…

We are waiting for an add on time slot for the OR for today. Last night Dr R called from his car on the way home( I think because he was too afraid to tell me in person) to say that he could not get us an OR slot until Wednesday at 10:30 am.  He came in this morning and said that he knows the CRNP in the OR that does the scheduling and that he was going to go and see what if anything she could do for us today.  We know this game, it’s called “hurry up and wait".  We turned off Lucy’s J-tube feeds just so that we can be ready if in fact we get an OR time slot today…never lose hope!

Yesterday was a quiet day medically speaking.  We had visitors in the morning which made our day fly by.  People have been delivering meals to our home and to the hospital.  I can’t tell you enough how much that has helped us.  We have not gone down to the cafeteria to eat in a while, for two reasons.  One we are a little tired of cafeteria food, and two we are avoiding the cafeteria with Lucy because she can no longer eat her beloved Cheerios.  I am not ready to address that issue, so we are avoiding eating in front of Lucy at the moment.  I know when we get home, we will have to address the issue, and address it we will.

I had a unique opportunity yesterday afternoon to meet with a wonderful very energetic nurse who is a certified healing touch specialist.  She and I had a session where she “refocused my energy centers”, or at least that’s what she set out to accomplish.  What I do know, it was relaxing and calming, I even fell asleep during part of it.  It was a welcome feeling to all of the chaos we have going on in our lives.  I loved it and would do it again.

Today we are working on getting some of the training for Lucy’s new hardware.  Social work is arranging for the infusion company that works with our existing HHC to come to the hospital and show us how to use Lucy’s equipment  if not today, then tomorrow. It’s all forward progress, but I am thinking that I won’t tell my kids when we are coming home until the actual day of discharge.

Thank you all for your thoughts, prayers, care, and concern, it means the world to us!

We have a plan…

Dr R came in this morning and made a comment about our very eventful weekend.  He then said that it was time, time to schedule her for a central line placement.  We are going to be placing a Broviac double lumen central line because it is what Lucy needs.  We are waiting to hear from the OR to see if we can get an add on OR time slot today.  I am not holding my breath that this will happen today, I am feeling more like it will happen tomorrow.  None the less we have stopped all J-tube feeds just in case we do get a slot today.  Our social worker has already begun the process of getting the ball rolling to get us all of the necessary equipment to care for and bring Lucy home. 

I agree with Dr R, it is time…

Happy Halloween…

I feel like I have watched the seasons change looking out of our hospital room window.  The leaves are past peak now and some of the trees are bare. It has most defiantly gotten cooler as we see people wearing warmer clothing.  However, in Lucy’s room it feels like the tropics, everyone that comes in comments on the change in climate the moment they enter our room.  They have turned up the heat in our room in an effort to keep Lucy’s body temperatures up.  Our girly does not like to wear any clothes, she is hypersensitive to any weight on her body which includes clothing.  Thankfully she will wear a hat and a pair of baby legs leg warmers but no socks.  What can I say, our girl is all about the accessories!

Medically speaking we had a quiet day.  The on call doctor came in to check with us this morning and talked to me about Lucy’s J-tube placement.  At first glance it looked as if  her J-tube had migrated up and into the stomach, but after much debate they do not feel that to be the case.  Lucy’s anatomy is a bit unique, big surprise, and because of this it makes it look as thought the tube is feeding up and into the stomach.  If this was the case then we would be seeing all of what we have been putting into Lucy’s J-tube come out her G-tube drainage bag, we are not seeing this.   She  also added Senna to Lucy’s prescription regimen because until Lucy’s gi tract starts working again we can not use Miralax.  It was decided that we would start Lucy back on 1/4 strength Elecare at 5 mls/hr and work our way back to 1/2 strength at 5 mls/hr in morning.  I’m Looking forward to talking with Dr R in the morning.

The kids came to the hospital with all their Halloween gear and pumpkins today.  Since we could not carve pumpkins together at home we did it here.  I was actually surprised to see how quickly things got done.  Lucy seemed to enjoy all of the activity and the kids had fun carving and getting ready.  Sophie was a wee bit whiney (I may be underestimating this a bit) again, I know she is struggling with all of this change. 

It was really difficult to wave good-bye to all of the kids tonight as they were leaving to go home and Trick or Treat.  I wish that we could have been all together as a family this evening.  These are the moments that make this so real.  I hope and pray that we will be able to take all of our kids Trick or Treating next year.

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.