Long Overdue

Sorry it has taken me so long to update, but everything has been pretty crazy lately.  Work is triple time, and have a couple new large projects at home, and of course the two little ones keep me very busy.  Enough excuses lets get to the important stuff.

We arrived Sunday at our ususal Extended stay and ran into a mountain of issues which pushed us elsewhere but could only stay one night.  There is a race here this week and there is not alot of options.  So on day two we moved again to Creek Rd. (which I highly recommend to our cchmc families  the place was nice comfortable and very reasonable).  When they decided to add an appointment for Friday that hotel was as well full so we moved again........to a dump   we were there all of 5 minutes til I was on the phone moving again, and calling guest services for assistance again.  They were able to help us find a place in Florence (great place and reasonable and CLEAN)  anyone needing info let me know and I will send it toyou.  Unfortunately not one time was we offered the Ronald McDonald house again!  On neither of the four other hotels comparing to the price we had been paying at our normal hotel, I really pushed the issue that it was out of our range but there was no offer  again!!  Oh well on to the big stuff.

                                          
Xray 1 is from March 2011, before surgery.  Xray 2 is from Tuesday.  You can see the improvement as her corrections is now 0.  Dr Jaine and Dr Crawford are extremely please with her recovery and has started weining her from her brace.  We will not see them again for 9 months, but before we come back in October for her yearly checkup they have me on a schedule to have her brace off all the time.  She has become alittle to accustomed to it and seems to like it more that I like but it is her security.  I am still waiting on the decision about school and her assistance needs. 

                                   

Both of these hip xrays are from this visit (you can see the hardware in her back, oh and you gotta love the technicians comments on the second one  lol).  The first one was taken with her brace on so dont be alarmed at the extra loops and rivets you see in the front lower quadrants.  It took three of us to hold her for these and difficult was not the words to discribe her emotions.......Kiristen thought we were killing her.  I requested this visit as she had been expressing increased pain in her hips and knees since her surgery.  I wasnt sure if it was due to a change in her structural balance or something more complex.  Dr Tamai assures me that all is the same but he does intend to proceed very soon, the goal was age 4 for working on her hips and knees.  So just as I thought we were going to go home early due to an appointment cancelling on Thursday I was notified that Dr Jaine had approved a sedated MRI for
Friday at 2:00.  This was needed from both sides of ortho before our return in October.  We will then make a decision about repairing her hips and knees.  We have also been referred to add Dr Cornwell to Kendras team beginning in October as he is the specialists for hurler hands. 

Here is a few additional pics that I hope you will enjoy.  We have tried to do alittle site seeing and relax alittle while we are here.  The last time Kendra was too little to enjoy the zoo, edens park, aquarium, plus a couple more.  I dont know about them but they have wore me out.  But Ive enjoyed every minute watching them learn and love together.....  Enjoy as I have done...........................

                                                

These are from July 4, and the Botanical Gardens in Cincinnati.  Kiristen picked out these as they are her favorites..

 

 

      

Thanks again for checking in and sending love and prayers. 

Good Night :)

Now this one takes the cake

Long Overdue

Sorry I am alittle behind, but Miss Kendra is a 24-7 hands on right now.  We came home on Easter Sunday, she is improving beautifully.  I have been able to reduce her pain meds to three times a day and the valum only at bed time, just so she will relax.  She is VERY mobile and headstrong.  She started getting brave on Tuesday night doing things on her own!  She is cooperateing very well with her sponge baths at night and allowing me to take off and put back on her brace..  Her and Kiristen had a mini-egg hunt and she had a ball!  She looks great and is eating great.  The only thing right now is she is very demanding and whiney which I am told that it is pretty much par for the course.  Here is a few more pics I hope you enjoy.  Thanks and huggs and kisses from us..................

Back to Extended Stay

Sorry that I did not get to update yesterday, but we waited from 1200pm til after 500pm while the coordinator searched for a pediatric wheel-chair.  Needless to say we have not found one in Cincinnati that can be serviced in West Virginia. The Hospital loaned us one to use here until we go home and then we will have to do something different there for trips to the store or prolonged times when she will need to walk.  But we are back at the Hotel and Kendra is progressing very well.  Her trust level has increased and she will slowly walk small disstances by holding only your hands.  She is getting more comfortable and not needing as frequent pain medication, from every two hours alternating the valume and oxy. we have moved to 3 hours.  She is still quite comfortable and not sleeping as much.  We did go out and stroll around the parking lot for alittle more than an hour (she wanted to see her ducks), just for a change of scenery.  She loved it and did not want to come back in.  Hopefully the wind will die down and not get colder and we will try to go to the park later today after she has napped.  Her appeatite is increasing and has ate better today than since Tuesday, Kiristen has been a very big help and is over anxious wanting to walk with Kendra.  They are playing together and trying to get alittle rowdy at times.  Here is a couple more pics I thought you would like.  Kendra looks great and the swelling from laying on her belly during surgery and all of the IV fluids has subsided.  Will add more later.........Thanks again and huggs and kisses

This is her new Monkey....and her new brace.

Apologies for the hair, but it was the last of our worries.

This tinkerbell blanket has become a comfort thing, it is very hot but she wont let it out of her sight.  Smiling for the camera, telling me "I'm a princess, Mommys baby, and I love you...:)

Day 2 post-op

Kendra has had a pretty rough night.  I will try to remember everything.  She sat up on the side of the bed once, and walked with 90-95% assistance to the bathroom but needed carried back.  I was hitting her pump in advance but seemed not to offer alot of relief.  She does not like the idea of having a diaper on and wants her panties (one of the first things she asked for when she woke up). But they removed her cath.  As of midnight she had not urinated and they did a straight cath which she hated, but it relieved her.  They are saying her kidneys are working fine it is just from the pain medication and anisthesia that her bladder is not releaseing as it should.  Her bowels are working fine with no issues.  They have put her on medication to help with this but we are still waiting.  About 930am they decided to insert a regular cath again and she peed everywhere!  But til now there has been no more so its just a waiting game as to what they will do there.She has been up walking to the bathroom today with the same assistance as last night but not as much crying from her while doing it or sitting on the potty or toilet.   All of her iv fluids are removed but we still have the ivport for her antibiotic.  It is a large one in her right hand and is only functioning for intake and will not draw so we had to draw labs manually.  We casted her for her brace and I had big discussion with them about how the last one disabled her.  They have assured me that this one will be different, lighter and easier on her.  Kendra had to go down to xray so Kiristen got to pick out the design of purple butterflies for her (they only had pink in camo...).  We are still waiting on PT as the order somehow got misplaced..........  We allowed her to get a good nap with the way her night and morning went and then we are putting her in a chair and takeing her out of the room for a stroll.  I will take pics and post them tomorrow.  Dr Jayne was in and was very pleased with her progress, and feels we will see more mobility and cooperation from her tonight and tomorrow.  She is still trying to be little "jaws", but if it were me in that kinda pain I probably would be too!!  Hopefully the internet will be working in the room tonight and I will add more.  Thanks again and huggs and kisses from Kendra

One More

Post-op pictures

Spine Surgery

Sorry that it has taken me til now to update, but it has been a very rough couple of days.  We arrived at same day surgery yesterday morning at 530am, we were fortunate to meet with the entire team before surgery including Dr Crawford, Dr Jayne, and the entire anisthesia team, nursing team, and pain team.  I went with her into the enduction room and held her (she wanted to hold her own mask with strawberry air), I laid her on the bed as I kissed her cheek they were all very supportive and assuring me they had kids of their own and they would take care of my baby girl.  Her monkey stayed with her as always and I went out.  They called me at the waiting area and told me they were beginning surgery at 922am.  The proceedure lasted 4 hours and all went wonderful.  Dr Crawford and Dr Jayne met with me and was very impressed by how it all went. They were able to correct to a 0%..........She had no surgery issues as her heart, lungs, blood pressure and all went beautiful.  They did intibate but not the traditional type (used micro laser with a scope). Ortho was releasing her to go to the pac unit, but just to be on the safe side I agreed with anisthesia to keep her in nicu over night to be sure of the pain management.  By 230-300pm Kendra was sitting up trying to play with Kiristen.  She tolerated liquids jello and by 900pm was eating her vanilla wafers!!  She had a pretty good night as we increased her pain pump from 3meg to 4 just so that I could reposition her and control her spasms with alittle more ease.  Her and Kiristen are having it rough as they only allowed Kiristen to see her for a few minutes yesterday.  This morning has gone well, they have removed the continuious pain meds (now on orals), and only has one iv left (just in case).  She still does not want to be moved and tried to bite the nurse to let her know!!!  The blue gloves arent allowed they had to get white ones as she gets freaked out really bad when she sees them coming.  We just moved about an hour ago to A3, and they said Kiristen is welcome here to assist with kendra's rehab!!!!!  Dr Crawford has Kendra on a move at will order, so today we will be encourageing her to get in a chair and move what can be tolerated.  She is continueing antibiotics, and is still running a midgrade post surgery temp, but no issues.  She ate a couple bites of bacon this morning but not going to really work hard on solids til later this evening when the meds are alittle more out of her system.  They will be in anytime to cast her for her brace, and she gets to pick the color (I am sure pink will be her color of choice!).  I will update again as more comes up.  Here is a couple of pictures taken yesterday right after surgery when they let Kiristen in.  Thanks  God is good..  And Thank you Daddy as I knew that this day was your birthday and you would be with us all the way. 

Post-ENT Surgery

Well another surprise.  We arrived at CCHMC at 9:15am as scheduled and registered.  They took us to the back and started vitals, anesthesia came in and questioned why we would be spending the night inpatient.  I told them that I had been asking the same question.  Well to make it short the scheduling nurse had an error in the plans.  Dr. Rutter wanted us to come home today!!!!  I carried Kendra to the induction room, and held her as well as the mask which pushed her strawberry flavored gas to put her to sleep.  Needless to say she had not been real happy since our arrival this morning and continued to fight me and them.  Once she was asleep I laid her on the bed, kissed her cheek and they pushed her into the next room.  By the time I made it to the waiting room and sat down they called my name (SHE WAS DONE)!!!!  Dr Rutter came out gave me ear drops, and said he would see me between 2 and 8 weeks when ever Kendra had time with her upcoming schedule.  He assured me they wouldnt last even though he used the best ones because of her ear shape.  And when ever I was ready we would discuss her tonsils and abnoids but they were not pressing.  So we agreed to leave well enough alone for now since she was not having any real issues with them.  She was awake and screaming for mommy, home, and NO DOCTOR!  I could barely get her dressed and out of there quick enough.  We are now home, and she is playing as usual.  I will post some photos later tonight.....God Bless

Our Arrival

We have made it to Cincinnati with no issues.  And we do have cell service where the service has been out in Alderson since Friday at 9:00am.  So if you have been trying to call now my phone works.  We have to be at same day surgery at 9:15am for a OR SCHEDULE of 10:45.  Will update then  God Bless

One Week......

Today we have been home for one week and I need to catch up.  Things have changed since we got home.  But first the good stuff.  Kendra's GAG levels came back as "appropriate and stable" as labled by Dr. Leslie in genetics.  Her score was 17!!!  They want her to remain below 30.  Now surgery........ We are having Kendra's pre-op physical done Saturday here at home as the plans for putting her tubes in at the same time of her spine surgery in April have changed.  Dr C and Dr Jayne have ruled that combinding the surgeries would be to dangerous as a risk of spreading an infection from her ears to the spine and hardward.  But the ears have to be done 3 weeks prior to any spine surgery!!!  Yep you guessed it Cincy here we come.  We will leave Sunday for her surgery Monday morning.  Our time is 914am report to surgery and proceedure to begin by 1045.  She will remain inpatient over night and as long as all goes well we will come home Tuesday afternoon.  I promise to fix this page and get the other pages added as soon as I can.  God Bless :)

West Virginia

Kendra and I made it home about 4:30PM yesterday.  Sorry I was very tired and just tried to relax and catch up with Kiristen.  This trip was very hard on her as it had been a long time since she had been away from us for this long. (During Transplant Kiristen was only away from me one week).  Kendra tolerated the traveling alot better than usual,  and I was pleased with that.  I am still waiting on her GAG levels, but Dr. Davies did call me on the way home yesterday and had reminded me that as long as Kendras little boy cells remained in the area they are in we were ok, she would have to drop to 10% AND her GAGS be rising to initiate any ERT or a second transplant.  So we will continue to pray and moving forward with our other issues.  She did caution me as to not let Kendra get sick between now and April as it could delay her surgery and put us behind.  We will now be able to finally get the Chicken Pox vaccine and Michelle will contact Jill here at home for our Pre-Op physical and to get that vaccine taken care of.  Will update soon and check back periodically as I am working on changes to the blog and some photos too.  God Bless

Day 5

Well Kendra never ceases to amaze me, and as usual today was no exception.  We were scheduled for a sedated CT, I just assumed general anesthesia as we always do.  We arrived at radiology about 6:45AM and went straight in.  After all the routine stuff the nurse proceeded to ask me about where we were staying and how they could reach me tonight to check on Kendra........ I told her the number would be the same but we would be back in WV by tonight.  She said we had to stay here over night as they were not using general anesthesia but sedation!! No gas just IV and it takes 4-5 hours for them to wake up and no traveling for 24 hours.  She recommended that we try it with Kendra awake......We got a tinkerbell movie and illustrated the scanner on her monkey (lol) and she did it!!!! I held her arms up and she laid still as I have ever seen!!! thank you god!  We were back in the room by 8:30am, just finished breakfast and almost done packing.  Will be on our way to WV shortly......:)
Will update when we receive the rest of our labs and our pre surgery physical back home with Jill...

Day 3 Pre-op

It has been a cold dreary day here in Cincinnati, and as I just looked out the window of our hotel there is a blanket of snow on the grass and trees.  Our appointments today seemed easy and quick.  Kendra saw Dr Rutter with ENT (we are return patients of his), he is planning on replacing the tubes in Kendras ears during her surgery in April.  He was pleased that her tonsils and abnoids werent bothering her and her snoring is at a minimum at this point but wants to continue watching them.  The home health team called me between appointments as our oximeter was blank!!!!  So we are doing the sleep study again tonight and he turned the alarms out of sleep mode so that I can moniter them.  We were happy to get to see Laurie Bailey and Lisa Berry in Genetics today as it had been a long time and they enjoyed playing with Kendra and felt her interaction with them was marvelous, not the shy clingly child they saw over a year ago.  Dr Leslie was impressed and glowed with Kendras achievements.  She was shocked to Kendras eating habits, and the way she did not feel threatened by her (she wanted to tell Audrey and Terry to keep up the great work) school has done wonders with her interaction with other children and security issues we had dealt with in the past!!! Way to go team......
Over all Kendra's labs were good.......her liver profile was alittle elevated but in the same range as it has been running and no worries there.  Transplant on the other hand has dropped again unfortunately.  September when we did our last level she was 41% and today the reading was 37.5%.  At this point we are waiting to see her urine GAG levels but as long as she is within the scale all will still be good.  As Stella says we will continue to watch with an eagle eye....Hurler's children (although we hope for) does not require 100%, it all depends upon the amount of enzyme that the donor cells produce and how well they function.  We did discuss-as they want me to keep an open mind the options for the future as far as enzyme replacement and a second transplant--but those would all be down the road decisions but things that we keep somewhere in our minds daily.  All of the what-ifs!!
Kendra met a new friend today while we were in the waiting room, a little girl who was just beautiful.  She was four years old and half of Kendras size.  There were alot of resemblances but she had a different syndrome that us.  They walked around holding hands for over an hour.  I so wished I had taken pictures for my own memories because they were beautiful. 
We have to be at radiology at 7:00am for her sedated ct scan, and then return her monitor to home health tomorrow.  I will try to update before we head home depending upon how well she is functioning after we get back to the hotel. 

Day 1 and 2 pre-op

first of all it has been a long couple of days.  sorry that I didnot get this started earlier but we have been very busy.  Yesterday was Day 1 of Kendras tests for surgery and all went really well.  We started with labs drawn at A-5 Hemoc-onc.  We were able to see Anna and Rich from BMT!!  Dr Wallace is on his 10 days off but told them to hug Kendra for us!!  They had to stick Kendra twice because of an accident and the nurse spilled a whole tube.  We then went to Ortho where we had three new x-rays and met with Dr Jayne and Dr Crawford.  They seemed very confident about Kendras upcoming proceedure and look for "nothing less than the one they did last week" (due to hippa they didnt mention names but we know who they are talking about right Elizabeth??  LOL  Kendras current curvature is 55degrees (on the bolus) sorry for my spelling.  Kendra was aggitated and did not cooperate very well as they tried to do some measureing and weights but they were happy with what they had. Dr Jayne added an appointment for this week to have a sedated CT scan to measure for her rods and screws.   We finally proceeded to Cardio with Dr Kimball where we waited 3 hours before they even did her EKG.  When the Dr came in he was quick as he had just seen her in September and said everything still looks great and her micro-valve and PDA was unchanged and he felt we would never have issues with them from the earlier tests.  SOUNDS GREAT!! Finally we were able to make it back to the room about 6:30pm and Kendra and I was done.  She had only  had snacks since breakfast and had no nap, she was not friendly and very clingy!  Today Wednesday we started the day NPO after 6AM in anticipation of her CT AT 2:00, our first appointment was 8:30am with Pulmonology (a new field for us), they were pleased although they scheduled us with home health to do an overnight study on oxygen saturation.  They assured me that this is a precaution to give them a better idea of her breathing under sedation.  We then proceeded to anesthesia where we pre-registered and voiced past experiences Kendra has had with sedation, intubation, and pain management.  We proceeded to radiology where they informed me they were running behind and it would be approximately 4:00 before they could take us in and I made them resecedule for Friday at 7:00am.  After the long trip up on Monday and the very long day at the hospital Tuesday Kendra could not handle another day with all the stress, no rest or meals.  She was very agitated and needed to relax and rest (she was very glad to get back to the room and strip and eat the cabinets bare!!!)  I did get the chance to briefly talk with Elizabeth and Bella.  She looks marvelous and is a inspiration to me.  Tomorrow is a much shorter day and I plan on a visit.  Michelle was off today and I will have Kendras labs tomorrow and will post them with her update.  Please be patient I know the page is bare right now but I am learning to do this and have alot of stuff to add.  If anyone wants to help I would appreciate it.  Missing home, family and friends as I called home and Kiristen cried asking me to come home she missed me so :(   xooxooxoo