The Natalie & Alice Fish Story

We are the Fishes. In 2011 two of our five children; Natalie and Alice were diagnosed with a rare gene disfunction called Leaky SCID (Severe Combined Immune Deficiency). Their condition is so rare that only two cases are reported in the U.S. each year. Both girls have endured much, and have spent most of their young lives in and out of hospitals due to common illnesess a healthy immune system would overcome. The required treatment for our girls is a bone marrow transplant.
Although the new marrow could mean a normal life for them, it will be a long and arduous road. This blog is to share our experience as parents and the courage of our children.

Saturday, November 29, 2014

Discharged from BMT and a Curve Ball

Somehow an entire month has passed without a post.  That is a new record for me.  I can think of plenty of excuses as to my slack, however, I'll spare myself and just do my best to update with as much accuracy as I can.

I will post most recent things first and then go back.  If you don't have a lot of time then just read the beginning of this post and you will have the current update.  There will be more news to shed this week regarding Alice's situation when pending lab tests return.

Hopefully this post isn't too difficult to follow.


November 26th
Today Alice had her follow up visit with BMT since her red cell transfusion last week.  Her counts held steady over the past week thankfully...(hemoglobin 11.2) but still no results on her chimerism.  Those results should be available next week.  Unfortunately all the viral labs came back negative.  That seems odd to be un-thankful for a negative viral panel, but in this case we were hoping her recent crash was viral related, rather than donor 1 retaliating against donor 2, in which we worry might be happening.  The chimerism will shed more light on the possibilities of this.

The near future plan is to check her blood counts weekly, and if anything drops we will been seen in clinic as needed.

November 25th
Natalie met with neurology today to review her medications and her seizure status.  Over the weekend prior to her appointment she had some small seizures, as she was battling a fever.  Tyler and I decided to administer nasal versed to stop her from the possibilities of having a major seizure, after receiving the versed she slept for a while and once the Advil/Tylenol broke the fever she seemed more stable.  The plan is to help Natalie become seizure free for two years before we can consider altering or lowering any of her seizure medications.  Neurologists are running a new study in which they think one's seizure threshold can be raised significantly the longer one goes without any seizing activity.  The idea is that the brain will adjust accordingly, and in the meantime the seizure medication only artificially raises one's seizure Natalie's situation we are grateful for the protection, while we hope her brain continues to heal.  Every person has a threshold for seizing, in Natalie's case due to illness, etc. (etc. translates into a long story) her threshold has been compromised, leaving it lower than ideal.

Also today Natalie visited Immunology.  We discussed her future care and the possibilities of further allergy testing in reference to some medications that she has reacted to in the past.  We discussed her fever and the possible source.  We expect that our visit with ENT next week will provide more helpful information on this subject.  It is possible that she has a chronic sinus infection...leading to a constant post nasal drip.

November 24th
Alice had an orthopedic appointment today.  The doctor said her arm is looking better but still needs to heal more.  She is supposed to wear the brace for another month.

Alice no longer needs instructions from the X-ray technician, this is second nature to her.

November 20th
Natalie meet with audiology again.  We picked out a hearing aid and it will be available on December 8th.  Natalie is SO EXCITED.  It seems once we decided to get her a hearing aid her hearing got worse.  I think it is because we are noticing it more, on her end too.  I hope that her hearing will not continue to worsen over time.  Another thing I really don't like about chemotherapy...these drugs can affect so many things, and worsen over time.

November 19th
Today Alice received a blood transfusion.  Her hemoglobin dropped even more since Monday the 17th.  Her hemoglobin was 5.8 today.  It took some time to match her blood, so we finished the day in ICS; because the clinic closes around 5:00 and we hadn't even started the blood.  By the time it was all done we found ourselves back home around 11:00 p.m.  It has been several months since we have been in ICS.  Of course it was lovely to see so many of our friends again.  Alice was in heaven and wanted to settle in for a while.  When I told her we weren't going to stay the night she was sad.  Silly girl!  After the transfusion her hemoglobin bounced back up to 11.2.
We found Emily.

Her expression when she heard she needed an IV placed.

Bring on the toys.

And the Barbie house.

Getting blood.

Guess who works in ICS now...our darling cousin Erin. Yeah!

November 17th
Alice has been noticeably more pale lately.  We called the doctor and had a CBC order sent to Riverton lab to check her hemoglobin.  We suspected it would be low, and it was.  It was 7.0.  This is considered a critical value and the doctor was notified immediately.  I called bone marrow to inform them, as we have been discharged from their services...apparently not anymore.  We discussed many possibilities as to why this sudden drop in her blood.  They suggested it might be a virus but are leaning more toward the idea of possible complications between the first donor and the second donor. We sure hope this isn't the case.  The doctors don't want to discuss too much with us until they have more information.  In the meantime we will find her some blood and run more tests.

Look how Alice's hair has grown.
This is how she wanted it styled.

November 9th
Happy 11th Birthday Natalie!!!  We can't believe you are 11.  Wow!  Time is flying.  This time three years ago you were getting baptized and preparing for a bone marrow transplant.  Oh so much can happen in three years.  We are proud of you and how far you have come in your journey to better health.  Thanks for being a great example to all of us in so many ways.  You are a beautiful girl and we love you!!

Grandma Schellenberg found this awesome birthday hat.

Cousin fun!

October 22nd/27th -- 1 YEAR POST BMT REVIEW
Alice had her one year post bmt clinic visit, including a phycology evaluation, a hearing test, and dexa scan (bone density scan).
Counts are as follows: Hemoglobin 10.7, Platelets 221, ANC 3300
The dexa scan indicates Alice has poor bone density, which comes to no surprise.  The physcology evaluation was positive.  Her hearing test was great.

To our surprise they discharged us form the regular services, we are graduated to long term bone marrow care.  This means our next follow up visit with BMT is next summer.  What?!?!  Alice is doing so well, she is stable and we are excited to move ahead.  There are some loose ends to tie off with some minor things.  Hopefully minor.  Her ferritin levels are extremely high.  This is likely a result from receiving so many red cell transfusions.  The body can't rid of iron from donated red cells...therefore the excess collets in the liver.  As long as her ferritin level continues to take a downward trend we will just watch it.  If it gets questionable then we will need to treat it...treatment for this is tricky, and isn't typically done in children.

Alice's whole blood chimerism has been 100% donor #2, but with some new showings of her T cell chimerism being 80% donor #2 and 20% donor #1.  The doctors are not worried and seem to be okay with this.

We have plans to see endocrinology soon to discuss her growth status and other things (things that have taken a back burner).

Alice is currently off all medications.  She takes a calcium supplement, a multi-vitamin, and plenty of vitamin D.  We can't believe this is actually real!

We have allowed Alice to experiment with gluten again, so far no complaints of tummy aches.

To think that we are done with bone marrow for a whole year is strange and hardly real to us.  Alice's healing has taken SO SO SO long it seems, and to now be at this point is bizarre.  Being healed is quite relative in SCID kids.  A SCID kid is never healed/cured, however, having a better quality of life is more appropriate to say.

Dexa Scan at the U of U

Hearing Test

We found Bo Bo the Bear.
Having Lunch
Missing the Broviac Line on days like this
Saying good bye to Sandy and Pam for a whole year.
She thought this was a great place for a picture.
See you in a while hospital!

October 31st--Halloween
I have to admit that I was a party pooper this year.  I told the kids if they want to dress up for Halloween--they are on their own.  Natalie created her costume piecing things together, Matthew wore his costume from last year, Blair wasn't sure what to I did help him, Alice and Evie decided to do what they do best each day and call it Halloween...Princesses!  And little Isaiah of course was something too.

A Pirate, A Chemist, and A Purple Morph

Isn't this a natural look for him...we went to a chemistry magic show at BYU and Blair was in his element.
He Loved it!

Tricker treating with cousin Brandon.
Every day is Royal at our house.

 Little skeleton boy!

October 20th

Swimming for real for the first time ever.  She went under water!!!  This was an exciting event for Alice.  She did great.

Still the best swim teacher ever...thanks Nicole.

Evie and Addie playing during Alice's lesson.

And More Pictures...

I love posting pictures of this little man.

Dinner Helpers

The busy Momma trick...who hasn't done this?

Getting stronger.
Wishing that were me.

Discovered bubble blowing.
I love him.

These goof balls tried to convince me into letting them drive these through Wal-Mart.

Her favorite boots...I was giving them away, they are too small for Evie now, she couldn't part with them so I made her a deal to take a seemed to help. 

Alice with Aunt Jamie. 

Evie, cousin Ava, and Alice
I love this picture because first of all you can really see how short us Fishes are...Ava is two and a half.  Evie is three, Alice is Five.  And I love it because Alice is pouting about not getting to stand in the middle for the picture.  I caught a moment of life in it's real great.