Briana Michelle (Bri)
has SMA (Spinal Muscular Atrophy) type 1.

This is a genetic condition
where the signals from the brain do not get to the muscles.
This is because the relays for the signals
deteriorate. Some are gone at birth, some go quickly after birth
and some take years to go as in types other than type 1.
Briana started showing symptoms from about the first month.
She was diagnosed at 6 months of age.
She was 14 months old as of February 7th 1999.

The prognosis for SMA type 1 is less than two years.

Bri was our Sunshine,

our joy in life, our greatest pain.

Please pray for our family.

Our beautiful Briana.

That's me, Briana...so longed for, so wanted,
so loved.
I came into this world healthy and beautiful.
Ganny gave me my first bath
in the nursery.She was so
excited and so were all my relatives.
They crowded the nursery
hallway taking pictures
...and me in my Birthday suit!

My first month was good,
I slept a lot and
everybody thought I was the BEST baby!
I didn't cry a lot,
I just sort of whinned when I was unhappy.
I ate all the time!

Then my Ganny noticed that
I didn't move my arms right
or hold my hands in little fists like I should.
I almost never moved my legs.
So Mommy asked the Doctor
and he said I just was developing slowly.
Everybody thought
that the Doctors knew best.
So they watched me...
and watched me get worse.

Finally at 6 months of age,
they sent me to a neurologist.
He said I had SMA
and they drew my blood to test my genes.
I had a pair of blue jeans
that Mommy used to put on me,
but that's not what they meant!

While waiting for the test results, I got worse.
I stopped swallowing my formula,
it just ran out of my mouth when I sucked.

I lost 5 pounds.
I started to get choked all the time.
I gargled instead of crying...
like I was using mouth wash.

My Mommy had to take me to the ER one day
and they put me in
the hospital.
They put a tube in my nose to my stomach
so I could eat.
I have gained back almost 2 pounds.

Hospice comes out now every day to see me.
The Hospice Chaplain Baptised me.

It was a very nice ceremony at my house.
Mommy fixed lots of pretty food.
My Ganny was there
and Daddy bought me a new pretty dress!
It was too big for me
(you know how Daddys are!)
but my Ganny taped it and I looked so pretty!

I cried when everyone started praying.
Well, everyone had been looking at me,
and making a fuss over me,
sprinkling me with water
and then they all closed their eyes!
No one was looking at me anymore!

Later that day
my feeding tube became clogged.
Mommy took me to the hospital,
I turned blue
because I choked on my secretions.

They bagged me
and then admitted me to the hospital.
I came home the next day
after seeing a pulmonologist.

He hooked me up to a machine for home
and studied how I breathe.
I can't use my breathing muscles anymore.
I use my stomach muscles and I get tired.

Today (August 19th)
I went to see my pulmonologist.
Guess What! I passed my breathing test!
He said that while I was asleep
my O2 sats stayed above 85%
which means that I am doing
OK when I sleep.
It also means that right now
he does not think I need Bi-pap.

He gave my Mommy a prescription
for some medicine that might
decrease my secretions some.
He also said that he will talk to my peditrician
about getting me either a pulse ox machine
or an Apnea monitor to use at home.

He also will talk to my peditrician about
having a meeting of ALL
my Doctors and medical helpers
to go over Mommy and Daddy's
wishes about the resusitation.

That way they all can talk about it
and clear up the misunderstandings.
He also is going to read
Dr. Bard's article on Bi-pap
and look his work
up on the computer to consider
if the treatment is right for me.
Well that's all for now....
see ya later aligator! (giggle)

August 22nd...
Today was not a good day...:(.
My secretions are getting so thick
that they are hard to get out.
My Mommy was flushing my tube
and I turned blue.
Daddy "bagged" me with room air and Mommy
suctioned me. Daddy had to do CPR on me.
They called Hospice and the Ambulance.
By the time they got there, I was OK again.
I think I'll be seeing my friends
Kayla and Rasmus soon.

AUGUST 24TH UPDATE

Today I had to go back to the hospital.
Somehow my feeding tube had slipped and
all the flush my Mommy put in was coming out
of my nose and mouth.
The ER people did not know how to put it in.
They had to read the directions on the tube pack.
They kept on asking Mommy who
had put it in before.
They got it in and took x-rays.
The nurse in x-ray was holdiing
me over her shoulder and
I turned blue again.
I wasn't as blue as before
because my Mommy and Daddy were
standing at the door and saw me.
They got me from the nurse
before she noticed me being blue
and took me outside the door
to where they had my suction stuff.
They suctioned me and put
me on my side. Then I was OK.
They got the tube in wrong and
had to put it down again.
We were there from 6pm
to 1am. Just for my tube.

Stay tuned...everyday there is a new challenge!

AUGUST 30TH UPDATE...
Yesterday I finally got my medicine the Doctor
ordered on August 19th.
There was some questions about
it that my Mommy had
and also the pharmacy
did not send it out
like the Doctor ordered.
This hospice nurse that came today
finally called and got it straightened out.
Then she stayed while I had my first dose.
It was ROBINUL which is supposed
to reduce my secretions.
I did ok so the nurse left.
Five hours later, Mommy noticed that my face
was very red but I did not have a fever.
She called Hospice back and they said I
was having an allergic reaction and
to take me to the hospital as
there is a medicine that they give
to counter the Robinul's actions.
So I had another trip to the ER.
My Ganny hasn't talked to my Mommy
yet this morning as we are all asleep...
or still at the hospital!
Going to the ER really tires us all out.
But Ganny will let you
all know how things came out later.

My Mommy called Ganny
and told her that
by the time I got in to see
the Doctor, the redness
had gone away. The Doctor
said not to give me the ROBINUL
until I see my pediatrician Dr. Smith
on Thursday. It's a shame
because Mommy likes this ROBINUL
as it really dries up my secretions
very well! She hopes we get to use it
and I do too!
See you afterwhile crocadile!

September 5th Update...
I went to see my pediatrician last Thursday.
She is letting Mommy use the Robinul
but at a low dose and then increaseing
the dose after a week.
The meeting to talk about the resusitation
is going to be Tuesday.

I found out that Maggie, a SMA Angel
of 7 months went to heaven SEP 2.
This is very sad for all of us.
Maggie was doing so good,
my Ganny used to see what Maggie was doing
and talk to her Daddy to see what
would help me. Maggie's parents
are very helpful and nice.
Maggie is now waiting in heaven for all of us.

I am doing ok right now.
I get so very fussy because I can't move
anything but my fingers now.
My Mommy tries to keep me happy
but I want to move and play
and I can't. My Ganny says
that at least I feel good enough to be bored.
It's so hard just to lie here.
Daddy and Mommy move me, but
it's not the same.

Please say a prayer for Maggie's Mom and Dad.
They miss her so very much.

Thursday night, September the 10th,
I ran a small fever of 101.8.
Friday I went to see the Doctor.
Right now I am in Brenner's
Children Hospital with
pneumonia. They are treating me with antibiotics
and oxygen. I have started to spit up blood.
My family is very worried about me.
I feel just terrible and am too worn out
to even cry when they stuck me
for the IV. They are giving me
Motrin for the pain. It helps some.
I hope I get over this. I might not.
But if I don't, then I'll get to see Maggie
and Kayla soon.
Please say more prayers for my family.
Ganny will let you know what happens.

UPDATE; September 16th.
Yesterday I came home from the hospital! Yippie!
The Doctors sent me home on antibiotics.
My Mommy and I are so happy to be home
where we can rest.
I actually smiled at my Ganny yesterday one time!
Mommy and I went to sleep after we
got home and we slept for hours.
We were very tired!
Another crisis, another day I have survived.
My family and I thank you all for the many
prayers you have made for us. It helps a lot
to know that people speak to God for us.
Thank you. See you next time!

September 30th update...

Sorry for the long time between updates but
my Ganny has been busy.
I have had a virus and my Mommy has
had to cut my feedings back
as they make me have more secretions.
I have had to go to the ER again
2 times since I last talked to you.
I hate that place! One time was
because my tube broke!
The other time it just came out.
Mommy talked to the Doctor about
getting me a tummy tube
but the stomach speacialist wont do it.
He says the risks are too high.
I have my Mommy and Daddy's cold now.
My Ganny hopes I don't have to go back
to the hospital. She worries so much
about me getting pneumonia again.
See you later!

October 9th...UPDATE

Hi there friends! Usually
the things I say here are
not such good news.
But things are going fine right now.
I got over my cold but my Mommy still has hers.
Mommy and Daddy took me to the Fair!
They took me to the petting zoo.
My Daddy put feed in my hand and I got to
to have a goat eat out of my hand!
I really thought that was fun!
I got to taste cotton candy
and my Daddy rode with me on the carousel!
Then Mommy amd Daddy had my picture taken
with a baby Tiger! A real baby Tiger!
I got to lay down on him.
He was so soft! Daddy fed him
milk from a bottle like I used to use
and I laid down on the Tiger's side.
Just thought I say hello and
give some good news for a change.
See ya later!

Me and the Tiger!

Update...Octomer 14th.

We got home around 8 in the morning.
Mommy noticed me breathing
funny this afternoon
before the Doctor appointment.
My O2 sats were 92%.
I had been crying all morning and
my cry was getting weak.
When we got to the Doctor's
they did more xrays. They think one of my lungs
is partially collapsed and I have
pneumonia (aspiration kind) in the other.
They have admitted me to Brenners Children's
Hospital. Home terrible Home.
I stopped breathing again in the xray room.
My Daddy suctioned me
and the nurse bagged me.
Then I started breathing by myself again.
Ganny will let you know how I do.

Note from Ganny:
Thank you all so much for your prayers.
Whatever God's will is for Briana, we accept it.
As for me personally,
it hurts me so to see her suffer.
The Briana in the pictures on this page
is not the Briana we see at home anymore.
She is so miserable most of the time
and it is so rare to see her enjoy life.
Her Mother is so strong and brave,
and her Dad so sure this too shall pass.
Briana has lost so much weight, so much spirit
and so much happiness.
Her little beautiful eyes stay red and weak looking.

Heaven...it has so much to offer her.
I have not given up hope,
just replaced one kind of hope for another.
Is this wrong of me?

Update...October 15th
I am still in the hospital.
Mommy noticed that my face, legs and feet were
swollen tight and my eyes
were swollen shut.
The Doctors say maybe they
gave me too much IV fluid.
So they reduced the amount but I am still swelling.
The Chaplain came today
and prayed with Mommy and Ganny.
They felt better then.
He sang a very pretty song to me
called Jesus Loves Me.
But I couldn't stop crying long enough to hear it.
I've only slept for 20 minutes in the last two days.

UPDATE...October 16th
Today the swelling is the same but
my fever is down and the Doctors say
my white cells are down and my xray is good.
Mommy told the Nurses that
I have not rolled over since May
and can't move myself
so they put a big person bed in the room
and Mommy laid down beside me on it.
I went right to sleep.
This is the first time
I have not been crying in four days.
They say I can go home tomorrow.
I guess the Lord above wants me here
for awhile yet. Thank you all
for praying for me.
Bye-Bye!

Update...October 17th
Today I came home from the hospital.
I am laughing and playing with my Daddy
while Mommy gets some sleep.
My Ganny says I don't seem to
be suffering anymore.
The Happy Briana is back!
Thank you all for your prayers,
as I know they helped me make it
through my bad time.
Ganny has some new pictures
so be sure to go to my other page
to see them all.
Till next time! Teehee!

UPDATE...October 28th

Hi there friends!
I have been very fussy,
crying a lot and very unhappy.
So my Mommy needed a little break.
She took me to my Ganny's house!
We walked around the yard and looked
at all the Halloween decorations
and at all the flowers!
I smiled and smiled!
Then she propped me up on pillows
so I could watch everybody
while my Mommy took a nap.
My Gannpa is so funny!
I talked and smiled the whole day long!
It was so nice for me and for Mommy!
I cried some too but Mommy
is getting help from all the SMA
friends to help find out
why I cry so much like I am in pain.
Oh! Guess what I am going to be
for halloween? A Kitty CAT!
With a Fuzzy Tail!
Have a Happy Halloween Everyone! Bye for now!

Update...November 3rd

The Doctor is going to put me
on antibiotics to help my bowels move.
The ulcer I have from my feeding tube
and the problem with my muscles in my
bowel not working is why they think
I cry so much.
So many wonderful caring people have
suggested ways to help me not to feel so bad
and Mommy is going to tell the Doctor about them.
Maybe some of them will help.
Ganny will let you know.
Hope you all had a happy Halloween!
Bye for now...

November 21st...

Hello everyone!
None of my Doctors
thought that I would still be
here at Christmas!
Here I am!!!
I have been doing fine. My Mommy
and Doctor let me use Milk of Magnesia
and things are moving along! tee hee
Grandmother Tribble (Mommy's Mom)
took me to have my Christmas
picture done. Ganny will put
it here when she gets it.
I'm with Rudolf the Red Nose Reindeer!
I didn't like having my picture done
so I cried. I liked Rudolf though!
Happy Holidays to you all.
See you later!

Update Dec. 29th 1998

Hello everyone. Sorry I haven't
updated in awhile but things have
been busy here. Ganny, Ganpa and
Uncle Preston all have been sick.
I spent my birthday in the hospital
as my lungs had collapsed in two
places. My pulmonary Doctor put me on
C-pap and they got better.
Now I am on C-Pap at home at night.
My Mommy had a big birthday party
for me when I got home.
Hospice gave me tickets to see the
Festival of Lights show and
my Hospice volunteer gave me a teddy bear.
I got lots of pretty presents!

This is my christmas picture.
I have trouble breathing when I sit up
so I am not very happy here.
Christmas was very good for me.
We went to Ganny's house and my
Mommy gave me some ham to suck on
and a candy cane! I got lots of
presents, clothes and toys! Many
people didn't think I would be
here for Christmas so they
really celebrated because God
answered so many of the prayers
that everyone prayed. He came
to Earth as Jesus, a gift to all men.
He let me be here on earth with my
family as a gift to all of us.
That God, he's number one with us!
We're getting ready to move to a
house that is on the ground
floor as I am getting BIG! I still
only weigh 14 lbs. and the Doctors
have put me on a special calorie
formular so maybe I will gain some
weight.
I have been so fussy lately
as I am cutting 5 teeth at
one time!
I haven't had any come through yet but
they're on their way!!
I promise not to let so long go by between
updates. Have a Happy New Year
and may God Bless you all
as he has blessed us.
See you!

Update January 8th 1999

I do not know God's will or plan for Briana or us.
I Thank Him and Praise Him
for letting Briana stay with us this long.
I also pray that he will have mercy on us
and on Briana.
With God, all things are possible.

Update January 10th

There are other SMA Angels.
Here are the links to those
beautiful babies. Some are still with us on earth
and some are only with us in our hearts and memories.
Please visit them all...

• Maggie Wyn Bonesteel

• Kayla

• Betsy and Katie

• Amanda

• Memorial to Various Angels

• Michelle's Angels

• Cody and Beth

• Memorial to SMA Angels

• Poems to SMA Angels

• David

Please visit these links to learn about SMA...

• Wheel About Neuromuscular Sites

• Families of SMA home page

• Families of SMA Guest Book

• Links to Other SMA related Sites

• SMA.net Main Page

• JTSMA at Home

Thanks go to several people for this page...
for Background graphics- JPG's by Nemo
for Animated graphics- JPG's by Nemo
for other graphics- Liz
for music- Wendy's Midi Haven
The music playing is "Wind Beneath My Wings".
Briana, her Mom Angie and Dad Neal are my heroes.

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