Day +35 October 27

For EB awareness week, challenge yourselves a little. Think of some ways to help educate others about the disease.  EB families not only battle the obvious pain and heartbreak as a result of the disease, but they do not usually get insurance coverage for wound supplies which can range from 800 - 2000 a month per child for the duration of the child's life. There is so much need and most people have not even heard of the disease -Lets change that! Share Elle's story, website, Facebook page or that of another EB child you know.  There are also useful EB websites with links at the right of this blog too.

Getting Festive for Halloween

Elle is having a better day today.  Many of her central line ports cultured out no growth (of yeast) for Three to FIVE days instead of positive growth in two days.  YES! I would like to give credit to all of the doctors and anti fungals, but I don't know, there have been a lot of prayers for her lately...The increased dose of Caspofungin and Voriconazole, the addition of Simvistatin along with the Amphotericin line locks certainly hasn't hurt.  The yeast war is not over, but we are certainly gaining ground.  The results came in late and we will discuss the next strategy in rounds. The plastic catheters are going to have to come out but when and how??

That is our favorite news of the day.  Other good news is Dad and Stefan actually got out together to watch the World Series at Grumpy's Bar.  However, they did not finish, wanting to get back to Chloe and the baby.  Chloe and I had a girls night and cuddled up, watched The Lion King, and ate popcorn in the BMT room. We checked on Elle a couple of times before the boys returned and Chloe behaved so well.  She colored in Elle's room with a mask on while I worked on a dressing that needed immediate attention with nurse Courtney. Courtney got Chloe to wear the too big mask by drawing a smiley face on it, sharpie style. A good time was had by all.

I also had a nice visit with the Edlings.  Give them your good thoughts too as Baby Daylon is still in a recovering phase with challenges and they are very busy and concerned about him.  One of the twins made pumpkin bread and shared with me -sooo yummy,  good job Sadie!

Now, I had orders to go to bed tonight so even though it is late, it is still an hour to an hour and a half earlier than usual.  Goodnight. 

Stefan took a couple of fabulous photos of Elle today. Here they are:

Oh those eyes...love them so much

Day +34 October 26

This is Epidermolysis Awareness Week- spread the word.
Elle had her biopsy today.  She had 5 not so little holes punched into her left thigh.  The tool used to core the tissue is even called a punch.  Don't worry, she got plenty of local and systemic pain control and did not seem to have ill effects or signs of additional pain.  The information from the biopsy (which we do not have yet, but will in a few days) will help Elle and the doctors in planning her care and in research efforts to help other children. 

Elle slept much of the day today and I am going to see her tonight right after this post.   She has maintained her blood pressures without adjustments to her meds or additional fluids beyond platelets.  Her WBC and ANC counts continue to maintain, and we will not have more culture results until tomorrow.  She has gained quite a bit of weight now; it just took a day.  She is 7.1kg up from 5.9 yesterday. 

Thanks for all of the continued antifungal/yeast ideas.  I didn't know about garlic- I wonder how we can incorportate that.  She isn't tolerating tube feeds yet, but I'll propose it.  I have been putting coconut oil on her here and there, but I didn't know that it caused the membrane to split.  That is interesting.  Yes Ma, I am getting your comments now and they are very sweet, thank you...Oh and she is on Ketaconazole topically. 

The Vikings were visiting the hospital again today at a halloween party in the cafeteria and Chloe got a photo with Freddie Brown who visited Elle before. She ate cupcakes and apple cider and became nuts for a little while but she had fun.



Chloe asked for a rainbow spider...



...and so she got one



Grandpa even wanted manly one

Dad asked, "Does this hospital have a pest problem?"

Chloe jumped at the chance to be goofy in the photo booth

                    

A magic trick

Feeding her dad -looks like he's not as enthusiastic about this

Chloe shy all of the sudden with Viking cutie, Freddie

Day +33 October 25

She was off of dialysis for 12 hours last night and all of her ports have been successfully rotated through with Amphotericin flushed and locked in the lines (instead of saline or heparin). This went OK, except she became fluid up as expected and her potassium level SHOT up, which was unexpected. She sat between 5.8 and 6.4 (normal 3.5-5) for about 7 hours and her heart rhythm was showing a change, but once she was back on the Prisma and her K+ went back down, it went back to normal.  Her weight turned out to be 5.9kg so not too bad.

The results of the cultures have come back in a confusing blur. There are 7 total ports and 2 types of cultures done on each port every other day. The results never quite come back as ordered. For example, the nurse will correctly label and send as: left PICC brown port, left PICC white port but lab may note as left Hickman red port with growth, left femoral no growth or something like that, so it is hard to tell what is what in the end. Everything has pretty much come back positive so it has been a moot point, but it seems that one of the white ports may have not grown out this last time.

Meanwhile, Elle has developed a pretty advanced pressure ulcer under her head gear despite frequent repositioning. It was an area that was covered for days at a time and so was difficult to assess. We all feel TERRIBLE about it though. It has taken me 3 days to even be able to post about it. I am sick about it really. Her skin in general has started to break down so easily lately. Her legs, elbows, wrists, neck, shoulders. I don't know what happened, but she tears if you look at her too hard. She had been significantly improving in that regard so I hope this is a fluke.  Oh and our my search for a gel type pillow, have come across the Z flow though PT/OT/NICU.  One is on order and hopefully will get that soon.  The unit assistant today brought the warming type in for temporary use.  While we await the arrival of the Z flow, we have fashioned  the warming pad under her body and just do not activate the heat .  It's worth a try.

Dr. Tolar visited with us today (now yesterday) and actually suggested the Posaconazole and may want to get Elle to the point of receiving more meds by G-Tube too. He is batting around ideas and is so not giving up. I love him for that! Elle had her eyes open clear and bright when he arrived and soon after, closed them. As soon as he left she popped them open again -I think she was pretending to be asleep because she knew he was talking about her! He is going to do her 4 week biopsy at 8AM.  I'm not looking forward to that, but am looking forward to the results.

Later, she was trying to go to sleep but kind of fought it.  She kept looking at me and when I touched her as I have described before, she would close her eyes.  Then she got pretty sleepy, but would open her eyes, see me, and close them again.  She did this a few times before she finally stayed asleep.  I think she wanted to make sure I was still there.  She has never been alone, ever in her ten months for more than a few minutes.  Someone has always been so close by that she could touch, see, or hear them.  She likely does not want to feel alone and I don't blame her one bit, bless her heart.  I love seeing her bright eyes again.  She just melts us completely. It makes me and her dad never want to leave her although we are both in the family area right now.

Elle had a nice visit from Stacy, a medical student who bonded with Elle during her BMT/Hemoc rotation. I'm sure Elle recognized her voice.We got to meet her husband too and  their new baby is sooo cute! We have seen some really wonderful pictures of him.

Chloe is doing just fine too.  Grandpa kept her active today and they had fun.  She didn't ask about us much today even though we were gone all day so that makes me feel good knowing that she was not missing us.  I did pick out a video with her, yes a VHS video and we watched part of it before bed (Cinderella).  She really keeps us going.  She is so sweet, imaginative, silly, intuitive, bratty, funny, intelligent, and ALIVE.  She is very special.  We're not biased at all of course.

Dancing during Wii

Making spooky cupcakes at RMH with grandpa

Fruit of her culinary efforts



Day +32 October 24

Baby Elle is hanging in.  They stopped the vecuronium (paralytic) and did a trial reducing the vent rate to see if she could handle it and in the words of the ICU Doc, "It failed miserably".  She became acidodic at her her first blood gas, meaning she needed to be on the high rate of the ventilator to maintain her body's safe PH.  That is OK, she just isn't ready for that.  It was decided to keep her off of the veck though.  We thought she moved while on it -wow, she really moves without it; hands, arms, shoulders, legs, head.  And she opens her eyes wide, looks at us with focus and clarity.  She is still so much IN LIFE, very much with us and fighting to be better.  Even the Docs say she has an amazing will and fight considering what she is undergoing. We love that, but still want her sedated for comfort and protection of her breathing tube and her wounds.  She still gets agitated and calms when I (or her dad) hold her hand and when I put my hand on her head.  Today, I just wanted to be next to her, available to hold her hand for most of the day.  I am at RMH now and wishing I was with her.   I may go keep S company after this.

So she is off of dialysis for 12 hours right now to free up a line so that each port can be alternated doing the Ampho lock.  Anti fungal will be flushed and locked into the line in hopes of killing any fungal colonies or bio-film from each catheter.

We are going to continue to rack our brains.  These Docs have big, knowledgeable brains, but they think about Elle for part of a shift and she is on mine and Stefan's mind 24 hours a day - yes even in sleep.  I dreamed that I fell through a water bed that became a deep, gel pool and that I had difficulty reaching the surface and my brother's resin covered painting that he had created 20 years ago fell to the bottom of the bed pool and was nearly ruined.  I felt so guilty about ruining the painting and felt very worried that other people, especially kids would fall in to the gel pool too.  I really am trying to come up with a good gel-type pillow for Elle to keep her skin safe and make her comfortable, and I am afraid of failing her. I think that is what that crazy dream means.  It occured to me today, that if this was the president, there would be even more creative interventions until the president was cured. So in my mind, our baby is "Ellebabma". She is our VIP and we are going to keep pooling ideas until we think of something brilliant together with the care team and those of you who are giving us great suggestions as well.

Stefan and I are doing ok though.  Dad really helps, Chloe helps, and everyone's prayers, love, and warm thoughts help. 

Day +31 October 23

Elle is doing OK today (yesterday).  In rounds, we discussed the new ideas and ID, BMT, and ICU docs thought about some things and are dosing the Caspofungin at a very high dose in conjunction with Voriconazole.  The Apmho-terrible as Vicki put it is not as effective in the sensitivity tests for her particular yeast although it usually is.  Also, It takes up 6 hours of line time and they want to do an Ampho B "lock" (still hasn't happened for different reasons but will start tomorrow). 

They said the essential oils and coconut oils are fine, but do not want to use pro biotics at this stage in BMT as it can actually be harmful with septic responses! Who knew yogurt could do that!  But as she improves, I will revisit that. 

Today I reorganized and cleaned Elle's room, changed blankets and Bair Hugger, wiped down all of her supplies and surfaces where the supplies are and her night nurse continued with the rest of the room.  I also cleaned her mouth very well when we did head gear hoping to prevent VAP -the last thing she needs right now is pneumonia on top of everything else.

Other than that, we listened to soft music and lullabies, I talked to her, hummed, held her hand and her dad did likewise.  We having started really watching her positioning schedule so she is turned every 2 hours, and the nurses are doing a great job at keeping her pain controlled.

ID and BMT docs both feel optimistic and believe there is time for treatment to show that it has worked.  She did get positive cultures again, but with the changes that were made, she should have a better result next time.

When Chloe and I prayed tonight before bed, she pulled me close to her and stroked my hair.  She is such an intuitive little sweetheart.  Before I closed the door, I was cheerfully wishing her sweet dreams and she looked convinced and relaxed and gave a happy smile back and asked for a kiss and a hug.  Her dad and I try not to worry her, but she still gets it.  

We kept the car parked and we all walked to Noodles today for lunch (thanks Vicki, used the card) and watched the excitement of the area preparing for Obama's speech.

Thank you again, everyone for the ideas for the infection (Fungemia), I am going to think about how to do the Vinegar thing too.  I am so glad my dad is here.  I can really concentrate on the baby and still spend good, quality time with Chloe in the evening.  It has been good for Stefan as well.

Long, long lines of people waiting to hear Obama speak behind Chloe

Peace chickens with signs that said "END WAR, YES WE CAN"

Day +30 October 22

No significant changes -YET.  We are still betting on a turn around with our baby.  She is tolerating all of her treatments. 

There were a couple of mishaps with her right PICC today.  While weighing her (5.55kg), her PICC sutures ripped out and the catheter backed out a bit.  The catheter also got accidentally partially pushed BACK in while the nurse held it  before I had a chance to clean and an X-Ray done -wouldn't normally push back d/t driving more skin contaminants back into the bloodstream.  She did not seem to be in much pain over this though.  Later when Stefan and I finally went to RMH to nap, they re-sutured her.  Having had no prior experience with her skin fragility first hand, the Doc pulled off a good piece of thigh skin when removing her PICC dressing. I am feeling pretty guilty for not being there to help now.  However, now the line is secure and although she has a new wound, her old wounds are healing beautifully.  That is good for infection control if the skin can actually act as the barrier as it is intended!

Today there was a lot of discussion about tweaking her care.  I guess it was found that her yeast was more susceptible to Caspofungin than Ampho B, but one of the research studies I read from Lisa's comment explores the idea that both can be used together without increased risk.  There were also lots of other great anti fungal suggestions and  thoughts that I would like to discuss at rounds tomorrow (today -it is almost 3 AM).  Thank you everyone for the resources and ideas.  The nurse on tonight liked the pro biotic idea and I am going to see if we cant do that through the G-tube and the essential oils thought is worth looking into as well.  Nurse Courtney was also considering other environmental factors that we could control such as changing out the bedding and Bair Hugger more often.  I used to wipe every surface down like lady Macbeth and have kind of let it go more as tiredness, stress, and sadness have started settling in.  Just like I am trying to do with the fungus though, I have to fight that and keep vigilant. 

The new BMT attending this 2 weeks is a man of very few words.  He only said about 5 words today but they were very good ones.  He had the idea to start her on a Statin (cholesterol med -Crestor) which has shown to decrease fungal activity in the test tube.  I like it; he thinks outside of the box!

So she has actually been pretty steady, knock on wood.  BP's a little challenging to keep up, temp OK, counts maintaining.  We have been able to control her pain.  She looks like a sleeping angel -such a sweetie.

Child Family Life, Carin finished Elle's DVD today of lullabies across the generations.  It includes Chloe singing a version of Twinkle Twinkle / ABC, Grandma Etelca singing in Hungarian, my mother singing a song she wrote for Elle, my dad singing a song he remembers his dad singing to him, and me and Stefan singing to Elle.  It was really very sweet.  I played it for her several times today.  It brought both Stefan and me to tears in parts.

Chloe went to the RMH Friday preschool, drew, played, and went to the October birthday party celebration at the RMH. Dad took her and got a few photos.  S is currently with the babe and I am going to retire with the hope of getting to the hospital early.  Goodnight and thank you everyone for caring about Elle.  Obama is coming to Minneapolis and there is a Home game tomorrow.  Going to be a traffic zoo!

Day +29 October 21

"Jammin with Jan" music on Tuesday

Grandpa, Tuba, Chloe, and Sully

We are still waiting and watching.  It is the hardest thing in the world.  We are straining our brains to think of interventions, but mainly it's waiting for the meds to work.  I asked if the Abelcet dose could be increased a fair amount due to unknown affects of Prisma.  It is hard to tell how much of a medicine is pulled of with dialysis in spite of studies and theory.  Sedation and Vecuronium are not supposed to be affected by dialysis in theory, but we can see easily that she needs much higher doses when on the circuit.  Also, we are not sure how her increased metabolic state affects medications.  So the docs agreed and increased her Abelcet and Voriconazole.  The down side is that these meds and increased dosages can harm her kidneys and even liver but in our care team conference, we got "the talk" (about how serious her condition is)so if it is between kidneys and life, we choose life. 

I am going to be a little medical for a bit in hopes that someone has insight on eradication of this fungus. Hopefully this yeast has not gone to far to manage.  It is called Candida Parapsilosis in blood, skin, and airway.  She is being given Abelcet at 10mg/kg/hr (amphoB), Voriconazole 60mg q12hrs, She also had a 2 time order for AmphoB nebulizer.  We thought of decreasing or holding TPN to decrease sugars in her system that may feed the yeast (docs thought this a bad idea in that she may crash without the carbs in her hyper metabolic state).  I also thought of decreasing or holding antibiotics since she was not having signs of current bacterial infections, but that too was not a helpful idea. We stopped Medihoney for the same sugar thought process (although infectious disease thought it wouldn't hurt) and instead are using Ketoconazole on her skin and trying to keep areas open to air as much as possible. Her Hickman, bilat fem PICCs, and new art line all come back positive on recent cultures and cannot be replaced at this point d/t few or no alternative replacement sites, need for access, and possible futility of it at this time.They are managing blood pressures and temps, and checking cultures every other day.  Any other thoughts, ideas, insights??? Ask, research, think for Elle please.  We are hoping in a couple of days she will start to turn around.  She is still fighting for life and we want to help her!

Stefan and I are taking turns at her side.  We did go to a Picnic with the Edlings and Ringgolds though (Daylon and Bella's families) at Hidden Falls in St. Paul for a couple of hours while dad watched the baby.  Jennifer provided most of the meal which was delicious.  We all had a good time, a nice break from the heaviness of the day.  The kids are so fun to watch and all in the same age group.  Later we played Bingo for a little bit then back to the hospital. 

Chloe wanted to see her sister and drew her a picture. Elle still responds to our voices and touch and looks at us.  Getting hard to keep her comfortable right now though since the Dilaudid and Versed drop her blood pressures, but we are still getting her there. 

Stefan does such a good job with head gear; here with RT

A rare look at her uncovered, unbandaged head

We took a couple of hours with the Edlings and the Ringgolds at Hidden Falls Thursday

See the little pumpkins and witch hats -Becca, one of the RTs drew them -I call her an" RTeest" -ha ha

Chloe drew this picture and is showing it to her sister.  In her words: Its Baby Elle with a breathing tube, a breathing machine, and medicines to make her better so she won't be sick any more. can you see all of the crazy lines? That is what all of her tubes look like to Chloe, but she does take it in stride.  She is pretty tough physically and emotionally.  Both of our girls are.

Day +27 October 19 & Day+28 October 20

Day +27: Had a better day -was stable with pressures and temp.   They did her Dialysis circuit change and her temp soared over 103! The dialysis keeps her temps way down all of the time so fevers are not spiking while on the circuit, but every time she comes off, the temps go up.  This was the highest by far though.

She got all of her dressings done and what is good in regards to her skin is that I have been slowly opening up more and more areas to air.  I place her limbs on a burn pad called Exudry (a tip from her primary nurse)with a little Mepilex Transfer directly on the pressure areas and "tent" the bedding so it is not in direct contact.  She now has all four limbs, both hands, and one foot exposed and they are all looking SO much better!! I also have a cup of air (O2 blow-by) blowing on her armpits and ketokonizole (antifungal) on the "cheesey" areas (sorry-gross).  I was never able to do this before because she moved so much and traumatized her wounds further.

Later in the day, we were honored to take part in a balloon release for Anabella, symbolic of letting go and setting her spirit free. It was beautiful and even included a short but poignant ceremony andher dad played Amazing Grace on a woodwind instrument. We watched the balloons float off into the skies, further and further into the distance.

Day +28:  As a continuation to the above, we attended Bella's memorial service this afternoon.  I never imagined that there could be such a beautiful service with not one floral arrangement.  The family wanted donations to go to PUCK for EB in lieu of flowers.  It was held at the conservatory at the Como Zoo with glass all around gardens and a Koi pond and the wind whispering through.  There were several nurses and respiratory therapists, family, the Edlings, and others present. The service was lovely and consisted of scripture, poems, an interactive portion that Stefan and I participated in, and lovely acoustic music.  The Ringgolds delivered flawless, gorgeous Eulogies and were so strong; they were the only ones holding it together.  I was very impressed and moved.  It was pretty tough, but such a meaningful tribute to a special baby.

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

Our special baby is fighting.  Her yeast dug it's fungal teeth into her and does not want to let go!  She is still growing out the yeast quickly and frankly, it's scaring the bajeezus out of us.  Her blood pressures have been soft and she is back on 3 blood pressure meds again. Her temps get low again and these are indicators that she is sick from the infection.  Also, yesterday her temperature probe that goes into her esophagus slipped out and the edge was cutting up her mouth and maybe her esophagus.  But her mouth had no blood today. The same principles still apply; prayers help the white counts to help her immune system to help the antifungals to help her. 

Another phenomenon that has happened today is that she has been so awake.  We could not knock her out.  She was wiggling and moving and looked uncomfortable and maybe scared.  She got more agitated when she heard my voice but calmed down almost instantly to touch, so I shut up, held her hand, and gently stroked her forehead.  Poor baby.  The nurse finally got her quiet and asleep with sedation. 



Keira, Ali, and Chloe in "cooking class"



Day +26 October 18

Elle's Day nurse cracked us up talking to Chloe and making up the Blue Bird of Happiness, soon after, Chloe completed this drawing.

Sweet Elle had a little bit of a rough morning.  After I went to sleep, her pressures slowly drifted down as did her temperature.  She had to get quite a bit of fluid as well as the addition of NorEpi (her 3rd blood pressure med).  When I entered the room, there was a lot of quick moving to get her pressures up (from low 70s or 60s over30s and maps of high 30s to 40s -should be >50).  She did stabilize again, but the docs are worried as are we.  It was said that the yeast in her blood is being cultured out of all of her lines, her ET tube, and is likely in her organs.  It is a nasty bug that likes to stick and stick around.  She has all of the best medicines (anti fungals) so we have great care team, the best medicines, her counts are coming in (WBC 5.2 and ANC 4,400 today!), she is fighting, and we have God too.  Should be the right combination.

 Our other sweetie, skipped her nap and was so excited to pick up grandpa from the airport,she got a little overly awake.  she danced at RMH to the Nintendo Wii, then her dad gave her Cinnamon Toast Crunch right before bed (Thanks hon).  She started laughing and flopping around on our bed like a freshly caught fish as I was trying to get her to bed!  I finally got there and she went out like a light!

After everything, she crashed for the night

Day +25 October 17

At RMH

I wish I could say there was a big change with little Elle.  She is doing OK, nothing worse happening.  She vascilates between stable and poor blood pressures, labs,and ventilator peak pressures but she is still fighting fungi.

Her dad did her head gear today.  I guess that is turning into quite a task as is her body dressings.  I really do try to think of the positives too, but right now I guess I am mentally tired and worried and tired of being worried.  I can't leave her tonight and I need to get myself to bed.  We are okay though, just wishing we could do more to help her and to hasten healing.  Thank you everyone for your thoughts, prayers, and insight.  She is quiet and comfortable right now, so I think sleep is on the horizon for me.

I want to share something that happened at church back home today.  We have a pretty large congregation, yet during the healing children's message, more than one child stated that they wanted to pray for Baby Elle and one said, "...she has a really, really bad cold and we pray every day for her to get better".
 

After church today, Chloe and I shopped for winter wear only to buy bath gel and baby dresses.  We were passing through the baby dept to look at winter accessories and Chloe kept getting side tracked by cute baby outfits.  She would pull one off the rack and then another, each time exclaiming, "I want to get this for Baby Elle" or "My sister will really like this".  So I diverted the mission and searched for the longest time to find clothes that miss Elle can actually wear.  EB kids are hard to dress in the very best circumstances.  Try adding a line on every limb, a breathing tube, and lying down constantly. She usually just wears a fancy diaper lately and nothing else. Chloe and I finally did come up with a couple of winners and one in particular, Chloe wanted to be from her.  How sweet is that?  So the rambling story ends with no winter wear and Chloe presenting her sister with a new polka dot dress!  You can see it another day.

This was Elle's resident, Maggie.  She had to move on to her next rotation :( We sure like her! She cares a lot about Elle.

My brother recently emailed this to me from 10 months ago -cute, huh?