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.

Wednesday, April 16, 2014

Highlights from Alice's Ribavirin treatment

These are some highlights from this last week's stay. It was a long week and I will admit I was a little off some of the time as you will see. I've since gathered myself and am glad we have Alice back home. Tyler

Monday, April 14, 2014

Ribavirin Treatments

Update.

April 9th
The VRP (Viral Respiratory Panel-done by a nasal swab) that was done in clinic today indicated that Alice has three different viruses on board.  They are:
-RSV (Respiratory Syncytial Virus)
- Adenovirus
-Coronavirus

Treatments for RSV require 2 hour fine mist inhalations of Ribavirin for 15 sessions, during a 5 day period...all done in an ICS isolation room.  With each treatment Alice is pre-treated with albuterol to prevent lung spasms.

Counts:
ANC 4400
Hemoglobin 8.8
Platelets 124


April 10th
Hard day for Alice...getting used to the long treatments wasn't fun.  Start to finish, it takes about 3 hours.  She is in isolation during the treatment under a plastic tent.  

Alice received IVIG today, we hope this helps greatly with warding off the viruses.
Alice also received blood today.  It was a busy and hard day for all of us.

Counts:
ANC 2800
Hemoglobin 7.7
Platelets 100


April 13th
Happy Birthday Blair!

Thanks Aunt Amberly for staying with Alice so Tyler could come home and be with Blair on his birthday.  Blair is 7!  He had a fun birthday.  Clam Chowder for dinner and more fun with cousins,  piƱata and cake.


April 14th
Alice receives her last two treatments for RSV today.  In between treatments she will get her monthly dose of Pentamidine-antibiotic, through infusion.  Once all this is finished she will come home and we can't wait to have her back.  I have not seen her since Thursday and I miss her terribly.  I will say I have had a chance to read and do a few things I don't normally get to do when she is around.  Tyler was on hospital duty the majority of this stay and thankfully was given a little break from Amberly.

Counts:
ANC 3300
Hemoglobin 11.2
Platelets 98


Lisy


Just settling in to ICS for another week.

Getting ready for the treatment.

Ribavirin requires this tent over the patient while the treatment is running...it helps keep the small mist particles contained.

This is what the nurses have to wear when in the room during the treatment.

Emily picked out this flower hat for Alice....appropriate for Spring.

It fits Tyler too.



What a sport!

Sleeping during the treatment...the best way to cope.

Princess game with Amberly.

Birthday boy.

Blair's favorite dinner...Clam Chowder.

Another awesome cake... hmmm.

Wednesday, April 9, 2014

Doing good....whoa, wait a minute

 A quick post. Alice did well today at clinic, even after the nasal swab. Her counts were doing well. ANC at 7.5, music to our ears. This week we will take her completely off of her bone marrow medicine and see if she can maintain on her own. I could tell the doctors were excited too. Alice was complaining of an ear ache so we had them check to see if there was any infection. They were a little red so they prescribed an anti-biotic and we were out the door. Awesome clinic day.....

Lisy called 5 minutes ago. The hospital called about the nasal swab results. Alice has RSV and couple other cold viruses detected. She needs to be treated and that means back to the hospital for five days. They are preparing a room right now.

Could all of you excuse me for a moment while I remove my big boy pants and say, "But, I don't want to go BACK to the hospital."  Good grief!  When we check Alice in we'll be able to post more information.

Tyler


Wednesday, April 2, 2014

Climbing Counts

Alice had a good day in clinic, it was a fast visit.  We will continue to give her another week without tube feeds because she has made some bits of progress in the past couple days...less vomiting and more eating.  She really only wants to eat a few items, but if she will eat them, we are happy.

Counts were good and we have decided to cut the Neupogen back to once a week, also cut her nightly fluids in half.  This will give her more urge to drink and possibly eat more.

ANC--4800
Hemoglobin--12.7
Platelets--121

Next week we hope for more good news.

Lisy

Monday, March 31, 2014

Finished Treatments

This past Friday Alice finished the last of her 9 treatments.  Currently her Adenovirus level is not quantified, which is wonderful news and also means that she is below the level requiring further treatments.  Her counts on Friday were also good.

ANC- 3400
Hemoglobin- 11.2
Platelets- 129

We were worried she would need blood because she seemed to have little energy and was complaining of a headache.  Luckily she didn't, and most likely she is tired from her lack of nutrition.  We will discuss her nutrition in clinic on Wednesday.  TPN is not an option, yet her tummy struggles to keep foods down, it will be a tricky thing for her to overcome...the doctors want Alice to have another feeding tube.  We aren't sure how well that will turn out, but maybe it can help bridge the gap.  She has made improvements, just little by little.

Last Cidofovir treatment



A few nights ago I was feeling very discouraged and sad regarding Alice.  It was a hard night for me, each time Alice vomited I became more and more emotional.  It was hard for me to watch her struggle so much...and feeling so helpless.  I suppose I realized at that moment that I am not as strong as I thought. Tyler and I have had to be so tough for our girls...we are strong so they can feel secure and lean on us. Being strong isn't always easy, and this particular night I wasn't holding back my real emotions as I have had to do so many times in the past in front of my children.  Not sure why of all the days, this day was more difficult; nothing out of the ordinary occurred, maybe it's been building up. Anyway, Alice looked at me and with a very concerned voice asked what was wrong with me.  I told her that I was sad that her tummy wasn't better.  She said, "Mom, don't be sad for me, I am not sad for myself."

I couldn't believe what I heard, she said exactly what I needed to hear.  I had been in tears earlier asking Tyler what kind of mother couldn't make her children better, just venting in frustration.  Alice understood it all, and helped me understand too.

Alice's last words to me that night before she feel asleep beside my bed were, "Mom, I will never give up!"


Lisy



My most willing kitchen helpers.
Don't let these pictures fool you.  These two girls can be the best of friends, but more often than not they are driving each other crazy.  Evie is the world's biggest tease and just wants Alice's attention...she knows how to get it.
Of all the patience Alice possesses, she saves little for Evie.



Sunday, March 23, 2014

A Stinky Ride Home

A quick week's overview--and for my records.

--Thursday Mar. 13th:  Natalie has fevers and rashes on her hands and wrists...she ate some red licorice.  Not sure the source of the fever.
--Sunday Mar. 16th: Alice is admitted to ICS with fever.  Natalie's fevers continue.
--Monday Mar. 17th: Alice received treatment #4 for Adenovirus, the kids didn't catch a leprechaun and were very disappointed. Natalie's fevers continue and has a small seizure.
--Tuesday Mar. 18th: Alice receives blood (hemoglobin of 7.9) and IVIG.  Natlaie's fevers continue.  Natalie has an appointment with Neurology...more details in the coming text.
--Wednesday Mar. 19th: Alice receives treatment #5 for Adenovirus, Natalie has an appointment with Dr. Burr for her brain's functional psychology.  Alice comes home from the hospital.
--Thursday Mar. 20th:  Lazy day at home, very unproductive.  Blair comes down with a fever.
--Friday Mar. 21st:  Alice receives treatment #6 for Adenovirus, Natalie is finally feeling better.  Blair is still home and acquired a new pet (Bold Jumper Spider), Amber had to catch it.  I am too chicken.

Alice's ANC has been averaging around 1300 for the past week.  A few medications have been dropped. :)  Neupogen is twice a week.

More Detail.

Two weeks into Alice's treatments for Adenovirus, and so far so good.  Of course she had the fever Sunday morning that took her back to ICS.  She was given IV antibiotics, fever never returned, and things are looking okay.  Alice was in ICS for 4 days, seems a bit long for only having the one initial fever, however, she was kept there because she required IVIG and blood, along with her Monday and Wednesday Adenovirus treatment.  I was so glad she received IVIG, it will be helpful in fighting Adenovirus. Alice came home from ICS Wednesday evening.  I wish to post a little something about our ride home in the coming text....first, here's a little information about Adenovirus.

Adenovirus infection mostly causes illness in the respiratory system, however can cause other problems such as gastroenteritis (diarrhea and vomiting).  Could it be that the Adenovirus is the main contributor to Alice's GI issues?  Hopefully we will see some improvements in her gut as this virus clears out.  This virus is something she has tested positive for many times over, although this time it's different. Why?  Because the Campath treatment she received prior to her recent transplant makes viruses more challenging to clear.  Also this virus was somewhat over looked as the team was focused on her Shingles and CMV, all the while Alice's Adenovirus levels were rising.  The testing process to quantify a virus level are not routine or standard, in the past it hasn't been much of a threat.

The adenovirus treatment requires Cidofovir, a strong antiviral.  Cidofovir can be very difficult on the kidneys.  Because of the threat to the kidneys this treatment is followed by plenty of IV fluids, fluids during and plenty afterward.  It's an all day process.  Also Alice is required to take Probenecid three times a day orally.  It's a terrible tasting drug, but also helps protect the kidneys...thankfully this medication is taken only on the days of treatment.  Her kidney functions are being followed closely and seem to be well.  Today is her 6th treatment with three more to go next week.  We hope this will be all, but there is a chance she will need more rounds...depending on her levels.

The ride home from the hospital....an event to never let happen again.  As I was loading Alice into the car, she informed me that her diaper was very wet (note: I had just changed a very wet diaper before we left the room).  I took it off and started to put a new diaper on her; she convinced me that she didn't need to wear a diaper for the ride home.  I thought about it for a second, after all she already had many wet and poopy diapers earlier in the day...surely she could manage an hour drive home without needing to "go".
Although, I said again to Alice..."I really think you should wear one".  Alice replied, "Mom, I'm okay, I promise I can hold it if I need to."  She really didn't want to wear a diaper.  I then said out loud to myself...."I should just make her wear a diaper."  But here goes to letting her have a little freedom from the diaper.  I gave in to her request.
Five blocks into our drive...still on Foothill Drive.  Alice starting coughing, I quickly handed her the pink bucket.  She vomited a fair amount, I wasn't sure if she managed to produce anything from the other end, but pulled over just to see.  Sure enough she had a major blow out.  She was covered.  It was everywhere, Including covering the ends of her central line.  Oh No!!!  I used every wipe and tissue I had available to clean her the best I could.  It was a disaster.  I was worried about her line becoming contaminated...and under my breath I expressed a great deal of frustration towards my own pathetic decision to leave her diaper off.  Alice said to me, "Mom, you should have listened to yourself".  Ha Ha....I then reminded her that next time I won't listen to her.
Upon arriving home we did a very thorough job cleaning her line and now we are crossing our fingers that during the next few days she doesn't develop a fever with a terrible line infection.

Alice's found a new dressing room.



Natalie visiting Alice after her own doctors appointment.

So happy to be with Alice.
Evie loves to visit Alice and all the hospital's toys.

I made Alice some YUMMY homemade salsa...she loves it.
See Recipe below.

Drinking the salsa juice...so healthy, apple cider vinegar and raw olive oil.


She ordered three bags of chips and ate them all....should have packed a lunch!

Natalie wanted to stay after her doctor's appointment and go home with Alice.

Home and doing the usual thing...I hope these days pass quickly.

Natalie's Update

We have been keeping a record of Natalie's fevers and diet.  It seems as though her fever pattern is consistent.  Every three-four weeks she runs a continuous fever for five-seven days.  Not usually are we able to diagnose anything.  The neurologist feels it is something systemic possibly and therefore we will be working with immunology to sort out the source of such fever activity.  Nothing may come of it and something may come.  I often wonder if her celiac diet could have anything to do with it.  Eating wheat or gluten accidentally should not create fever...however, we think of everything.  Also the neurologist and I talked about doing some more extensive testing on her brain to isolate the source from which the seizures come...as there are two locations of damage on Natalie's brain.  If the source is due to scar tissue there is a chance it can be removed and stop future seizures.  This was only a brief discussion and surely not in the near future or possibly at all.  There would be plenty of considerations to take into account with such a process.  The challenge with Natalie's reoccurring fevers are the risk of seizure she has with every fever.

More to come, but for the most part she is doing well.  Tyler and I were discussing how much she has improved both socially and emotionally.  Natalie is determined to succeed...this will be her most valuable asset.



Treatment in Clinic

Helping Mom make Portuguese soup...YUM YUM!



Karen's Fresh Garden Salsa
8 tomatoes chopped small
2 cucumbers chopped small
2 bell peppers chopped small
1/2 large white onion chopped small
mix 1/4 cup apple cider vinegar and 1/4 cup olive oil...add to vegtables
add: salt, pepper, and garlic powder/salt to taste.  It takes plenty of salt.

Perfect with tortilla chips.  FYI--very addicting and refreshing.  Optimal of course with your own grown garden vegetables.  It's so good you could eat it with a spoon and forget the chips.



Monday, March 17, 2014

Back again

Last night was a tough night. Alice could not stop coughing and throwing up. It was around 3am that we took her temperature and found that she was running a fever. By 5am we could see that it wasn't coming down and made the call. We arrived at Primary's close to 7am and checked her in. Her temp read 38.5 C upon arrival. I was exhausted by the time vitals were finished and we could rest a little. Her blood cultures came back negative so far, but the nasal panel was positive for Adenovirus in her nasal cavities. We will be here for at least the next couple days. This is hard to watch her go through, however she is where she enjoys being. I am grateful for that.

This video may be a little graphic; however after watching Alice it made me realize more, that what she does is nothing new to her. This is all she knows with regards to her health. Watch how she becomes sick, muscles through it and doesn't miss a beat when moving on after she is done.

Tyler

Thursday, March 13, 2014

A quick post

This will be a quick post. It has been a few wonderful but cautious days. Wonderful because Lisy and I were able to spend a few days together. Cautious because, we received a call from the doctors telling us they had found a virus in Alice's body they had not thought to search for in the previous months. The virus goes by the name Adenovirus. The treatment will be fairly aggressive as Alice will be required be in clinic three times a week for at least three weeks. She will be in clinic tomorrow, all day. Lisy will be able to gather more information with regards to side effects and in addition write a post more specific to what is ailing Alice.

Tonight however, Alice was feeling pretty good and was in the mood to talk a little about the medicines she likes and doesn't like. So, while at dinner our tradition is for each of us to take a moment and talk about the most interesting parts of our day Alice chose to speak on her oral medicine.





A Much Needed Get-A-Way
....many thanks to Amberly and Tyler's parents for taking care of things at home.  We were so worried about leaving, but it turns out that the kids needed a break from us as much as we did them.  The children were much better when we came home than when we left.  Our favorite part about our get-a-way was just driving in the car and having no real agenda.  Full length conversations that were uninterrupted...Wonderful!

Historic Cove Fort

Newport Beach

Just a quick stroll and visit...a little cold.

He has always wanted to go to a banana stand.
p.s.  The doctors called us with the news regarding Alice's adenovirus about two hours into our trip.  We sat on the side of the highway for a very long time trying to decided what to do...turn around or continue.  We prayed about it and discussed everything.  We felt good to continue, so off we went.  It was a much needed time away.  If anything the few nights away we slept better than we have in months and truly needed the rest.

Thursday, March 6, 2014

A Wish Bone

Every time I think I am going to get ahead and post promptly...I find myself a week later realizing I have yet to post.  I am so glad Tyler has been good to keep the record updated.

Update:

Friday evening the 28th of February Alice came home from the hospital.  She was there only three days, which was a nice surprise.  We weren't sure what to expect with her and the Croup.  Thankfully she recovered well and quickly with little intervention.
A regular position for Alice.

Ready to go home...thanks Emily for sending us on our way.

Alice's Tummy:
It's been a long rough recovery...her tummy is trying so hard, but struggling.  She sometimes poops a dozen times a day, and vomits plenty.  At one point we decided to just simplify things and remove her diaper, put her on the little potty and move her around the house as needed.  She could "go" whenever and give her little bum a break from diapers and increased rashes.  The picture below might be a little too much but someday it will be appreciated for what it's worth.  The hard part was trying to eat a decent meal while her business was going on next to us.  It didn't take long before it made us all giggle at the reality of the situation.  I will admit it did require a little focus on my part to pretend the smell wasn't affecting my appetite.  The kids cherish dinner time, as they each take a chance to tell about their day.  Don't be mistaken, eating around the dinner table doesn't happen every night, as often as possible though.


March 4th, Natalie had a 4 hour long psychology review in Salt Lake through the neurology departments psychologist Dr. Burr.  We hope to learn and understand more as to how her brain processes information. Long day for Natalie.  I was thankful that Tyler took her, I wasn't feeling up to it.

Clinic today (March 6th) went well, no infusions required...just the typical labs and medication review. Tomorrow we will learn if her CMV levels are finally low enough to take her off the IV ganciclovir, which contributes to her suppressed counts.  Crossing our fingers!  If not, she will go another week on ganciclovir before we check her CMV level again.
Counts today:
ANC 300
Hemoglobin 12.9
Platelets 147
Good news...liver function tests have continued to improve.  We know it's because of the TPN (IVnutrition), why they had been so high; glad we are past that point and pray that we can continue to avoid TPN.
Clinic days...this is my version of a time laps :)  I know it's boring!

Alice keeps asking us, "What's wrong with my tummy?" and "When am I going to get better?"  I can't imagine being nauseous all day for months on end.  I was explaining to her that her food and drink needs to learn how to leave the stomach and go to the intestine, it has forgotten and needs a little help.  She said, "I know what's wrong....it can't see where it's going, maybe if my stomach had a light the food could see where to go."  Later I found her with a flashlight, shinning it down her mouth.  Oh Alice, I want to make this all go away.

We have been working hard to make homemade bone broths and all kinds of foods that help the gut heal.  It seems to be helping!  We saved out one of the wish bones for the kids to enjoy breaking.  Matthew and Blair claimed the wish bone.  I told them to think of their wish but not to tell.  Matthew ended up with the bigger half and of course they told me their wishes.  Matthew wished for Alice to be all better and sweet Blair had secretly wished that Matthew could have any wish he ever wanted.  So for Alice, not only are WE earnestly wishing you well, but many many others who know you or know of you are too.  It will happen....it will happen!

Lisy




Friday, February 28, 2014

The Croup

Yesterday I was outside raking up leaves. It was an unseasonably warm day and it made sense for us to be outside and enjoy the weather. The kids were jumping on the trampoline,  and while I was raking the leaves Alice spiked a fever. So, I took her up to Primary's. Turns out she has a case of the croup. It took only one cough for the doctors to diagnose her. It resembled a barking seal. Keeping her healthy is such a struggle; it weighs heavily on Lisy and I. Nevertheless, these are the cards we have been dealt, and we will play the best hand we can. There will be more blood draws and tests in the next couple days. We will be sure to keep all of you updated.