2011 February - Aria MacDonald

Aria’s health at the moment is pretty frustrating. While she isn’t terribly sick, things still aren’t right.

Her skin is still an issue, although the extra steroid doses she is on have helped. However the flip side of that ‘solution’ is that increased steroids always cause increased outputs. Her outputs are still high and made even higher by the steroids. So she is still off feeds and on TPN, and has a central line and things just seem to be stuck in a not so happy place for us.

To top this off we are still working hard at trying to get her to sleep through the night. Between being itchy, her nunu (ostomy) falling off at inopportune times and whatever else keeps her up at night, she sleeps pretty terribly still. I think she has slept through the night perhaps twice since transplant. Anita and I tag team through the nights, the one who is least tired bouncing up to check on her. It is hard to just try and ignore her because with her health situation, the ‘what ifs’ mean that it pays to check that everything is alright just in case. This leaves Anita and I weary during the day and unsettled at night too.

Given all this at times we are feeling a bit sad about things at time. We long for the days when Aria was on full intestinal feeds and she didn’t look like an extra in a zombie movie. We have been told that it is just a matter of time but we would really like time to be up and things be right, right now.

When things are feeling a bit grim like they are now, it is the little things that make us smile and help us to get through the day.

Yesterday we went to the circus courtesy of Child Life at UNMC. It is put on annually by the Tangiers Shrine each year and it was fun. Wheels the clown took good care of us and got us some great seats. What made this time special however was just a few fleeting moments when the dancing elephant was performing and for a time Aria lit up and danced along and was really enjoying life. It just made me warm and fuzzy inside.

Other times it will be something as simple as Aria and Asher pretending to be pirates or her doing something nice for Asher when he is a bit upset that makes me appreciate that even with all the craziness of her health, she is still a happy, kind little girl underneath it all.

When the big things are out of control and feel too heavy to bear, it can be the little things that make the days worthwhile. Through these times we fall back on our trust that God ultimately has things in his hands, that the timing of these things is under control and that he is doing what is good in these things. We are weary but he is not and we look to him for our strength.

Isaiah 40:28 Do you not know? Have you not heard?
The LORD is the everlasting God, the Creator of the ends of the earth.
He will not grow tired or weary, and his understanding no one can fathom.
29 He gives strength to the weary and increases the power of the weak.
30 Even youths grow tired and weary, and young men stumble and fall;
31 but those who hope in the LORD will renew their strength.
They will soar on wings like eagles; they will run and not grow weary, they will walk and not be faint.

Yesterday we had a positive visit with the dermatologist.  She looked at Aria’s skin biopsy and said that while she can’t rule out graft v host she thinks, along with the transplant team, that Aria is having a drug reaction to one of her meds.  We showed her the long list of Aria’s meds.  These transplant kids are complicated and none of the drugs Aria takes are for fun.  BUT thankfully the one she suspected the  most is the one Aria can most afford to go without.  So we have stopped that med and given her another week burst of steroids just to give her a break.

Starting feeds again today and seeing how that goes.  Aria’s outputs are still up but have settled a bit so it is worth a shot.

Not an hour goes past when we don’t think of Christchurch.  There was a news story about the quake yesterday on the regional news here.  Had to blink back tears seeing NZ on TV here and for such a sad reason.  Lots of prayers needed now and in the future.

This is the second attempt at updating in the past day or so.

Overall it has been a trying week.

Aria is off intestinal feeds and back on full TPN.  Quite disappointing and we are still struggling with it.  She had a biopsy on Monday and it was clear for rejection.  There were abnormal findings and the team are still sure it is a result of her Norwalk virus dragging on.  So we watch and wait and hope to restart feeds once her outputs settle.

Aria’s skin is still dreadful and we have an appointment with a dermatologist this evening.  What a relief.  We are hoping and praying for answers and treatment.  Aria is very itchy and her sleeping is terrible, poor girl.  She scratches herself raw and bleeding.  We put creams on but they are gone within half an hour.  We are managing the best we can with creams and meds but are at our wits end.

Aria is actually doing pretty well herself.  She is still laughing and happy.  She gives me this big beaming smile with her peeling red face (even her eyelids are peeling) and missing tooth and it just makes my heart soar and sag.

Our country is hurting right now and we feel so sad for it and so far away.  Amazing to see the NZ spirit and all the other countries coming to help us.  We have family and friends in Christchurch and thankfully they are well.  Had kind American friends email and ring yesterday to check on our family and friends.  How cool!  Anyway please keep the people of Christchurch in your prayers and thoughts.

Aria and Asher had a visit from our Make A Wish friends today.  The two ladies volunteer for MAW and have been our contacts for organising (and postponing) our Disneyworld trip.  They have been in contact with us and were sad to here of Aria’s health over the winter.  They popped in to visit and brought Aria a very cool Disney Princess Crown and wand and Asher had a Donald Duck soft toy.  It was fun to visit with them.

We are pleased that Aria’s new blood pressure medication has done the trick.  Her legs are slowly starting to deflate/dry out/slim down.  And it appears to be keeping her BP well under control unlike her previous med.  We take her BP at home and every reading we get is in the normal range.

Started the enzymes last night, mixed with apple baby food.  Got off to a hairy start but she ate them all.  Did another dose this morning with the mixture in an oral syringe and she sucked it out.  That seems to work better than trying to spoon it into her mouth.  She told me they were delicious!

But sadly it can’t be all joy and sunshine.  Aria outputs have shot back up again yesterday and today (2000mls/24hours!).  We called the team to let them know and thankfully they didn’t want her back in.  But Aria will have another scope on Monday because it has been a month since her last one.  AND we had to stop the enzymes too, much to Aria’s disappointment!

This past week has been really nice.  Her outputs have been trending down and we have made progress on advancing her feeds and dropping her TPN.  It has given me time to feel embarrassed about how stressed and worried I was that this bowel was in serious trouble and it wasn’t the Norwalk causing this.  This little quote is currently stuck on Aria’s bedroom door at home.  It clear knocked my socks off when I read it.

Stress and Worry: Basically these two behaviours communicate that its ok to sin and not trust God because the stuff in my life is somehow exceptional. Both worry and stress reek of arrogance. They declare our tendency to forget that we’ve been forgiven, that our lives here are brief, that we are headed to a place where we won’t be lonely, afraid or hurt ever again, and that in the context of God’s strength, our problems are small indeed.

Francis Chan from the book Crazy Love (can someone be kind enough to post the page number- I sent my copy home)

Today we had Tinic as Aria calls it.  Or Clinic rather.  Going to clinic is good, it means Aria is an outpatient, which she has been for 10 days now.

We had quite a few issues to iron out with Aria’s health and we were really pleased to get lots of answers.  Firstly she is on two blood pressure meds.  One is making her legs really puffy with fluid.  She was put back on that one last week when we took her off the one what was giving her the rash.  But given the state of her huge legs we had to show the team and ask for something different.  And they ordered her a different drug again which is supposed to be good for kidney transplant patients so we will try that.  So hopefully her legs will look a little better soon.

Firstly Aria’s rash and skin is pretty bad.  She looks kinda terrible.  Her skin is super duper dry and peeling off.  I will leave the description there.  Thankfully we got a referral to a pediatric dermatologist at Childrens and have an appointment on the 23rd of Feb.  The surgeon on this week took photos on her iPhone of Aria’s rash and sent them off along with some of Aria’s history (which is long).  How is that for proactive care?  Awesome!  Hopefully we don’t have to muck around with funding approval too.

Finally we found out today that Aria is malabsorbing fats.  Nothing to do with her gut function rather her pancreatic function.  So she is going to take enzymes four times a day to help like CF kids do.  This might also be a reason for the continued high outputs but isn’t anything to be concerned about.  Hopefully though this is just a side affect from the norwalk and not a permanent thing.  The interesting thing about the enzymes is that we have to mix them with a bit of solid food like applesauce and get her to eat them, four times a day.  That’s going to be fun!  Actually I think we are going to end up mixing them with guacamole because that seems to be her favourite soft food.

Overall it was pretty positive and Aria did really well.

The MacDonald’s celebrated Valentines Day yesterday.  The highlight was seeing three groups of our friends!  We unexpectedly saw our friends Elaine and Issac at the Zoo.  Yes, finally the weather here has been decent enough to be outside and we raced out the door!  We had a really fun time.

Next we delivered a little Valentines Day gift to our friends Brad, Stephanie and Megan.  Later Carol and Cordell came over and Hamish and I enjoyed a Valentines Day date!  What a great day.  The kids had fun writing little Valentines Day cards. I really, really like the American version of this holiday, it is about love, all types of love.  What’s not to celebrate about that?  Our friends are special people to celebrate too.

Aria is doing ok.  We are currently stuck at 20mls an hours on her feeds.  Her outputs go up and down each day.  The lowest being 800 in 24 and the highest being 1200.  Today they are trending down again so we hope to get up to 30mls and 14 hours of TPN.  But overall we are encourged because what is coming out looks digested whereas previously it looked like water and curdled milk.  Sorry TMI but my transplant mum friends will get it.

Aria skin is still pretty terrible.  Last night I coated her in thick white cream, you couldn’t see her skin.  She woke up this morning looking a bit better but still very flaky and dry.

With managing her outputs, trying to get cream on her and her nunu (ostomy) falling off 2+ times a day it makes for tense times in our house.  Thankfully her current nunu has lasted over 24 hours!  Wow.  The cream is the biggest issue and she hates it, hence why I do it while she is asleep!  And the fact Hamish and I are up 6+ times a night!  And today is bath day for Aria.  Sigh.

Clinic tomorrow so looking forward to that and hopefully getting some progress.

Well since transplantaversary we have gotten out of hospital again and back home.

The steroids have done there work and Aria’s rash which at its worst made her look like one of the walking dead has now progressed to being a whole lot of dry and peeling skin. Not great but much better than it was, unfortunately this means we need to keep putting moisturiser on her which she HATES. Its funny the things she will happily tolerate and those that drive her into a screaming fury.

As for Aria’s outputs which have been tormenting us for sometime, they are currently behaving themselves better even to the point that last night we were able to restart her tube feeds again. This was a really nice feeling, even though that means Aria at times has three cords between her and her faithful little pushchair which carries her TPN pump, replacement fluids pump and now her Enteral feeding pump and their associated bags. Aria loves any time of the day that she gets free of those and runs around like a maniac to celebrate her freedom.

We are hoping these reduced outputs will continue to trend down and we can start aiming to get rid of the TPN again. Thanks for your prayers about these issues, they have been doing our heads in at times but we are hopeful that God has seen fit to return her outputs to normal now.

Today marks the day of Aria’s 1st anniversary of her 1st transplant.  Not to many emotions really, the only thing I am really thinking about is her donor family.  Who they are and what they are doing.  Grieving still obviously.  Heartbroken forever.  Please spare a prayer for this family, God knows who they are and what their hearts are feeling today.  Hopefully a tiny sense of peace that their child was able to save another.

Aria was readmitted yesterday.  Her dumping is still really high and her kidney numbers (hydration) were totally off.  So hoping to get her back on track and discharged again.  Currently working theory is that her steroids make her immune system less active AND her FK/prograf/tac/immune surpression drug levels are CRAZY high.  Meaning her immune system is fairly wiped out and the virus she is battling in her gut has made a comeback.  It some ways it is a tiny relief because it means this is still the virus and not anything more like rejection.

Aria is pretty happy today although does have a case of the jitters because of the FK level.  She isn’t keen to stand cause her legs are all shaky.

Sorry for those who get the subscription emails.  I wrote this awesome post and then manage to delete a chunk of it when mucking around with the photos.  Defeated I changed it from published to draft but the subs program must of sent it anyway.

Aria’s outputs are crazy high today!  1400mls in 12 hours- Huge!  We know it goes without saying but we deeply covet your prayers for this.  It is quite difficult and stressful and it is a dark cloud over our little family esp tonight.

Aria herself is a box of birds and doing great.  The steroids bring out the best in her, lots of talking walking and hugs and kisses.  Her rash is also much better but her skin is peeling and yucky.  Looks like we cooked her in the sun too long.

Aria is home from a 24 hour hospital admission.

Yesterday she spiked a little fever to 38.3.  Which isn’t a major but she was pretty sleepy and her rash looked so much worse.  Deciding we really needed this rash eyeballed Aria and I went in.  Braved the cold -15 degrees here yesterday!

The skin biopsy didn’t show Graft v Host (PHEW!) but they think she has psoriasis and certain drugs can make it worse, one of them she started 4 weeks ago when this rash first started.  So hopefully with some steroids and stopping that drug and replacing it with another she should be better soon.  In the meantime it looks really nasty and we are busy trying to put different creams and potions on (which she hates).

Also tackled the team about her continued dumping.  Unfortunately Aria is still positive for Norwalk virus and they reckon it might take ‘months’ to resolve. ARGH!  They also changed her formula to something much more basic and easily digested so that should help too.

Kinda feel like the 24 hours was quite worthwhile although Aria was less than impressed.  I feel a lot more at peace about this dumping although it is still quite bothersome.  We will just have to wait it out.  I feel like God has been gracious to allow answers and some progress, so going to take it and chill a bit.