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.