Mommy's Online Journal                  

 Thank You for Another Week

Monday, March 30, 2009 4:12 PM, CDT

http://www.caringbridge.org/visit/rachaeli/journal/13

"Mommy, 5 more minutes!"

"Thanks, Ari."

"Chaeli, I'll be back in a second.

I have to check something before I light."

She leaves.

It won't be a second

She mutters from downstairs,

"Oven's off, urn's on, lights are set.

Ari, I'm lighting!"

_________________________________________

D-Day 

Diagnosis Day or Death Day -- You Decide

Monday, July 20, 2009 7:17 PM, CDT

http://www.caringbridge.org/visit/rachaeli/journal/11

Hi.  I finished my 3 week course of Amoxicillin so now my tummy is back to normal and I'm breathing nice and quietly.  It has actually been cool in Atlanta so Mom and I were back out on the bench this morning.  I think Mommy liked it.  My tushy got sore.

Mommy found a journal she kept back when I was born.  She has these journals all around the house and car - just a bunch of spiral notebooks for her to write her thoughts in because thoughts just whiz in and out of her head so fast.  She says it's kind of fun to find one of these journals after a couple of years, but the one she just found is kind of sad because it covers the time when I was diagnosed.  She thought she would share some of it.

Jan. 2003 - ten million entries about cute things my brothers did.

Feb. 2003 - Rachaeli - two months on - big, beautiful smile; loves the bath; never cries; sweet, gorgeous, cuddly, warm - I love her!!!; Yoni, Shui, Ari, and Abba love her, too.

March 4, 2003 - Rachaeli's first "word" - "Uh, Oh"

Tons of stuff about my brothers....

March 8, 2003 (Lola's birthday) - Abba's song for Rachaeli:
(To the tune of "Jenny from the Block" by Jenifer Lopez)
Don't be fooled by the poops that I got,
I'm still, I'm still Chaeli from the block.
Used to drink a breast now I drink a bot,
I won't forget where the milk comes from.

Don't be fooled by the poops that I got,
I'm still, I'm still Chaeli from the block.
Used to poop a little, now I poop a lot,
I won't forget where my  poops come from.
(my abba used to make me dance with him to that song)

Tons of stuff about my brothers...

June 1, 2003 - Yoni - soooo good with Rachaeli.

Ju ne 10, 2003 - Rachaeli 7-8 months old - first words (after UhOh) - "Abba" and "Bubble"; loud breather (in her sleep)  like Shui.

Tons of stuff about my brothers...

August 2003 - 9+months - Well Baby 9month check - "Hypotonia" ; "Developmental Delay"; Neurology/Genetics Referral; Babies Can't Wait referral.

No more stuff about my brothers....

August 2003 - Neurology Assessment - Dr. K. - has delays but good reflexes; good neuro - playful, engageable, good grasp; "doesn't look like a primary neuro diagnosis"; consider a brain MRI; can check a whole bunch of labs tests now  - or - follow up in 3 months to see if she's regressing or progressing.  

I immediately chose the "wait for 3 months" option; stress; denial; Eric wants answers;

Rachaeli - playful, smiles all the time, laughs; can sit for 9-12 minutes by herself but she cannot get herself into the sitting position; babbling a lot; seems to have no motivation or desire to reach, grab or move; floppy arms; increased tone in legs.

Septem ber 2003 - Babies Can't Wait evaluation -
Very engageable with with evaluators; sat for 60 minutes on the floor with minimal support; smiling; cooing; transferring small rings from one hand to another; sucking her thumb; putting large objects into mouth.

Found to have "severe gross motor delay" only.
Speech, fine motor skills, social skills, and self help skills still within normal limits.  Eating great/hearty appetite; thick legs...

Octo ber 2003 - Started physical therapy with Catherine 2x/week.
Rachaeli cried throughout (and she never cries); tried to avoid doing things; painful; like we were torturing her; "no pain, no gain"?; PT Long-T erm goa l - push herself to seated position by 18 months.

Loved playing with cars, especially Ari's hotwheels cars; still in walker and exersaucer (legs and toes extended to balance herself); able to transfer cars and toys from one hand to the other while standing; able to push music pads; able to play with toys; could push cherios around.

Love d eating cherios - that became her reward for PT;
Short term goals - getting into crawling position; tummy time  and push up torso; She Hated It!

She seems to have "decreased motivation" to reach for things far away; still moving around in bed but not grabbing for the bars.

Very engageable, playful, sweet, smiley.

Late r October 2003 - When PT started, she seemed to stop babbling, and her fine motor skills worsened. Is she replacing gross motor work for fine motor and speech?

Set up Occupationa l Therapy and Speech Therapy Evaluations.

October 25, 2003 - One Year Birthday!!

N ovember 2003 - Occupational Therapy Evaluation (really long written report).  Even though she could put some of the really small pegs in a peg board and pull a string which pulled a bunny, she was found to have "Severe OT Delays" - like a 5 month old and was in the 0-1%ile (not good).

Novem ber 2003 - Speech Therapy Evaluation - found to be at the 6 month level - not awful.

November 2003 - signed up and started Gymboree. 
Rachaeli LOVED it!!!  She sat with minimal support while staring at the other babies.  She loved it when the other babies crawled up to her and on her.  She talked to them!!!  Lots of sounds!!!  She didn't do this at home .  Maybe we're just not stimulating her enough?
She liked to go in the inner tube an push up her legs.  She liked the seesaw with the furry rug. She HATED any rolling or slides (startle).  Everyone loved her outfits and her smiles!


Nove mber 17 - November 20, 2003 - Asthma!!! Rapid crump.
Monday - dry cough, then shortness of breath and retractions, then wheezing; took out Shui's nebulizer and gave an albuterol treatment, then took her to the ER.  At the ER, her O2 saturations were 93%.  That plus her "low tone" pushed her to the front of the line.  Immediate PICU admission.  It looked like she should be intubated - she was so tired out. 
Diagnosis :   Status Asthmaticus, Hypotonia, Developmental Delay.
Treatment:  Xopenex, Racemic Epinephrine, Oxygen.  Needed a few rounds to stabilize her and Choral Hydrate to relax her.  Slow improvement.
Discharge after 3 days with referral to see Dr. Harsh (Pulmonary).

Then came home.  OK. We've done asthma before. No prob.  Eric said he spoke to Dr. H. in the hospital and he said there is probably a connection between her development al delays and her breathing problems.  I said that was probably not the case since we have asthma in the family (Shui was diagnosed at 9 months) and she's going to get better at Gymboree, and if I have to, I'm going to make our house into a Gymboree. (What's the name of that river in Egypt??)

Nov ember 2003 - Time to get ready for Thanksgiving.  Opa and Lola, and MomMom and PopPop are coming.

Very stressful.  Bad results from OT and Speech Evaluations.   Bad feeling that something is wrong.  Maybe it's not just "Benign Congenital Hypotonia"?  I received the Hypotonia books I ordered before the hospitalization.  I also ordered lots of therapy equipment - mats, exercise balls, innertube that floats from the ceiling thingy, rolling/crawling thingy, piano, educational equipment...and I'm looking for more. 

Thanksgivin g 2003 -  Major Breakdown the night before Thanksgiving.  On Thanksgiving day, Rachaeli choked on mashed potatoes. 
By Friday - lots of coughing.
Friday night/Saturday - reactive airway symptoms and started Prelone.  Needed Xopenex treatments every 2 hours, then every one hour.  She was unable to rest. 
Saturday night - went to the ER. Sats at 96%. Not as bad as the last time. 
November 29-30, 2003 - Admitted to the General Pediatric floor. Treated with Xopenex every 4 hours with Racemic Epinephrine every 4 hours - then changed to every 2 hours.  Lots of suctioning and Chest PT.  Tired - took a while to start feeling better.  Spoke with attending, Dr. Teague.  The plan was to wourk up the hypotonia and to rule out aspiration.

December 1, 2003 (Mon).   Abba working.  Mom in hospital.  Boys at school.

Dece mber 2, 2003 (Tue). Consult with Dr. X of Genetics.  Eric was there, I was at home.  Neurologist came, too.  Dr. X knew by looking at her that it was likely a Lysosomal Storage Disease - Severe startle response every time the door opened, slightly enlarged liver, low muscle tone, regression of developmental milestones.  But I said it "can't be Tay-Sachs" because Eric's screen was negative.

De cember 3, 2003 (Wed). 
AM  Swallow Study - Aspiration of thin and thick fluids.  A Nasogastric tube was placed for feedings, which Rachaeli pulled out twice. 
PM Abdominal Ultrasound - Enlarged Liver.

Decem ber 4, 2003 (Thursday) - D-DAY (Diagnosis Day, Death Day - you choose).
Ophtho Consult - I was looking forward to it so we could eliminate a set of possible diagnosis.
Whole consult team came in.  Within 10 seconds the attending, with some big lens in his eye,  immediately identifies a Cherry Red Spot. 

I leave, floating down the hall, praying to take it away.  I didn't hear that. It's something else.   There's still time for Hashem to make it okay.

A resident comes out to the hall and says, "Come in mom.  You don't have to leave."  I am somehow coaxed into returning.  Upon seeing me, the attending says, "Come take a look.  It's a cherry red spot.  You see it in Tay-Sachs and one other disease, I think Sandhoff disease."
I reply, "No,no.  My husband tested negative for Tay-Sachs and my parents aren't Jewish."
"Then it could be Sandhoff...or I'll look it up and see what else it could be."

I think:  Help.  Stop it.  Change it now.  No. No. No. No.  Do you know what you just said?  Do you know what your'e doing ? You call yourself a doctor?  How could you change everything like that then just walk out - and be happy and excited! You #@$.  Do you know what kind of news you're giving me?  How could you?  You're telling me my baby is going to die!  Wipe off that smirk of academic excitement and go to @#$$!  Go to @##$you @#$$  &**((.

Do you even know what you're saying?!  How would you like it if I walked into your room and told you your daughter was going to die in the next 2 years, and I had that excited look on my face?  Would you appreciate that?  You probably wouldn't even see it.  You cold-hearte d @$$&*().  What kind of example are you setting for your residents and medical students following you around?  That you give a diagnosis of a terminal illness and walk out the door a minute later?  Nice example.  Great bedside manner.  Did you go home and tell your wife, "I diagnosed this great Tay-Sachs case today."  I hate you.  Learn some clinical skills, or stay away from people.  If you can't give a diagnosis with compassion and empathy, then don't give any diagnosis and let the team attending do it.  But don't ruin my life with that excited smirk on your face.

How do you spell scapegoat?

I didn't say any of those things (to his face) and it is possible that the conversatio n we did have was slightly different than I remember it, and I don't hate ophthalmologists (that much).  Let's just consider it a brief episode of psychosis.

C alled Latisha - Eric is with a patient; told her it was really bad; have Eric call me.

Called Mom and Dad.
Called Judi (who called MomMom and PopPop).
Called Zoya at work :  Please look up cherry red spot and find me a diagnosis that is not so bad.  Please.

Eric arrived.  Both of us in shock. Neither of us angry.  Trying to figure out the pieces of this puzzle.  Trying to figure out what this all means in the grand scheme of things.  I can visualize hundreds of my own life experiences - people, places - swirling around a funnel and disappearin g into a jug.  Does everything factor in?  Who's running this show?  It's not me.  What is the reason for this?  There is a purpose - Just what is it? Or maybe we can just turn back the clock.  Please, can we please turn back the clock?

Rachaeli is just lying in her crib smiling, with a board on her hand so the iv will stay in, and with a tube up her nose.


Dr. X cam e by.  Discussed Lysosomal Storage Diseases.  He gave us a list of 9 possible diagnosis.  Like a really bad Chinese Menu.  All were bad.  Some were worse than others.

Disc ussed Home Hospice.  Discussed Respite care.  Discussed Do Not Resuscitate orders, etc.... AHHHHHH!  STOP!  How can we be discussing DNR orders today when yesterday I was figuring out how to make a Gymboree basement?

Ca lled Elaine- Today was Danny's (her husband)  second yartzeit (anniversary of his death).  All her family in town to tell Danny stories.  Will visit after sundown.

Lot s more calls.

Dec. 5, 2003 (Fri) - brain MRI with sedation - generalized cortical atrophy? Cerebral atrophy?

 Second Year Tsunami

Monday, May 5, 2008 10:03 PM, EDT 

http://www.caringbridge.org/visit/rachaeli/journal/21

For the past 4 1/2 years, my mom has been stuffing letters, emails, articles, poems, etc. into her "tay-sachs" file. It's a mess and will probably never get organized. So, she is going to do the 'grab-bag' approach and randomly share things from her file in this journal.

On Sunday, December 26 (or 24), 2004, my mom, abba and I went to South Florida and left my brothers with my grandparents. I had just turned 2. My parents needed to regroup and process the previous 12 months. They also wanted to hug and cuddle with me as much as possible. We had just been through a whirlwind of a year, as the 12-24 month period in a tay-sachs baby's life is devastating. Parents watch their kids regress, but at each little plateau, they pray to keep the new status quo. "It can't get worse than this," they think. On top of all that, the parents are mourning the diagnosis, and essentially the loss, of their child. But their child is still with them. Parents are confused, conflicted, weak, tired, depressed, and worn down to the core. As they grieve the loss of each milestone, they feel the need to celebrate their time with their child. They want to make each day count and cannot imagine the day they will have to separate.

Well, when we checked into the hotel, my mom put on CNN. The great tsunami of 2004 had hit the day before and the death toll at the time was about 20,000. It seemed that every hour the new guestimated death toll would rise by another 10 or 20 thousand. It was unreal. My mom held me tight on her lap and recalled 9/11, when thousands had died before the world's eyes as the towers collapsed. My mom feels chills and heartache at the slightest thought of that day. She remembers feeling the thousands of souls rising to heaven at the same time, and each time she saw a re-run of the collapse, the same painful awe would, and still does, overcome her. And now we were witnessing the deaths of 10 - 20 - 30 times the number of people lost on 9/11. It is impossible to conceive. We watched the interview with the woman who had to choose which of her sons to hold on to and which to let go, as she clung to a tree which saved her life. It was the miracle of miracles that the son she let go of found his way to his grandmother and they all survived. But that was an anomaly.

So we sat there crying as mom was forced to imagine what it would be like to lose someone so suddenly, with no warning or preparation. While we had many losses throughout that year, we had not had the ultimate loss. We had something to be grateful for. We had time. And my mother clung to me thanking God for giving us time. She was so thankful that she was given that past year, and now, as I write this, she is so grateful she has been given the past 4 1//2 years. Most people with terminal illnesses don't live for another 4 1/2 years. We were the lucky ones. God had mercy on us. He didn't just grab our children and pull them into the sea, never to be seen again.

So three months later, on March 15, 2005, my mom wrote this poem. She is far from a poet, but the words just ended up on the page. She doesn't even like or appreciate poetry. She hopes it makes sense to somebody.

Little Tsunami

Reflections on Year 2 with Tay-Sachs

2 1/2 years ago, a tiny boom, barely a ripple in our sea

Effortless, pain-free

You entered our world, eyes wide open, mystified

Confused but Content

Calm, joyous, peaceful

13 months later, a Deluge

Backs turned, dizzy with surprise

Engulfed and overpowered

Pushed and pulled, unprepared

Deeper and deeper into the depths

Smothered, suffocated

No last gasp for air

Blackness.

Seconds, hours, days, years?

Lifeless, tossed to shore

Resuscitated, revived

Peace once again

Still in my arms

Slightly withered

But free

Breathing salty sea air, a blessing

Confused but Content

More minutes to hold, kiss, love

Like an Eternity

Thank you, God

Return to Top

____________________________________________

http://www.caringbridge.org/visit/rachaeli/journal/22

This is a story my mom wrote for me. The name and descriptions can be changed to those of any princess out there.

Princess Rachael

Once upon a time there was a little girl named Princess Rachael. Princess Rachael knew she was a princess because that's what everybody called her. When her mother came into her room each morning, she greeted her with, "Good morning my princess." When friends saw her, they would ask, "How is the beautiful princess doing?" Complete strangers would walk up to her and proclaim, "Princess, you are looking stunning today."

Princess Rachael had princess hair. It was silky brown and hung all the way down her back. Her mother told her it was a gift from God and that not everyone was born with princess hair. When her mother braided her hair into two long braids, she said, "My, my. Aren't you the most striking Native American princess?"

Princess Rachael had princess eyes. Her mother told her that they were big, brown, and almond-shaped, and that all of the most beautiful Asian princesses had eyes like hers.

Princess Rachael had princess lips. Her mother told her they were shaped like a heart and that they were as red and silky smooth as a rose petal. She told her that because God gave her such gorgeous lips, she would never have to go to the trouble of wearing lipstick. Her mother told her that all the world's princesses were given lips like hers.

Princess Rachael knew she was a princess because she wore the most elegant dresses. Her mother could not resist buying them for her. She would come home from shopping and say, "How could I walk by such a fine-looking dress when I know my Princess Rachael would look absolutely glorious in it?"

Princess Rachael's mother would sing her a princess song:

(To the tune of "You are my Sunshine")

You are my princess

My lovely princess

From your head down to your toes

Princess hair...and princess eyes

Princess lips and princess clothes

Now the most obvious reason Princess Rachael knew she was a princess was because she had her very own sparkly princess tiara. Her mother had a special stand for it next to her bed. Princess Rachael was allowed to wear her tiara every day if she wanted. Her mother told her that most princesses only wear their tiaras on special occasions, but every day was a special occasion in Princess Rachael's palace.

There was another reason Princess Rachael knew she had to be a princess. Every once in a while, out of the blue, young children would show up at her palace to sing her the songs they learned in school that week. Princess Rachael loved being around other children, especially when they sang. She even kept a bowl of candy in her room so she could give the children a sweet when they left. Princess Rachael's mother told her that only princesses get serenaded by young children.

Princess Rachael had a bunny rabbit named Goldie that she loved so much. Her mother told her it was just natural that God would send her a golden colored rabbit. After all, don't all princesses have lots of gold and gold colored things?

Princess Rachael's mother would sing her a princess song:

You are my princess

My lovely princess

Your tiara worn with pride

Serenaded by young children

A golden bunny by your side

Princess Rachael had a throne that was grander than any other throne in the land. Princess Rachael's throne was pink and gold and always had new, shiny balloons attached to it. It had a special headrest so that even if she fell into a deep sleep, she would be comfortable. Her specially padded throne helped her to sit up straight and proud, the way all royalty sits.

Now Princess Rachael did not have only one throne. She had her grand stationary throne and she had an even grander throne on wheels. This way, she was able to meet and greet all of her royal court and visitors anywhere in her palace without leaving her throne. She could even be strolled through her gardens or into town without leaving her royal throne. Princess Rachael was special indeed.

Princess Rachael's mother would sing her a princess song:

You are my princess

My lovely princess

With a throne that holds you high

So that all the world can adore you

From deep sea up to the sky

Princess Rachael's mother loved her so much. She told her that not all mothers are blessed with princess daughters, and that it was an honor and a blessing from the Heavens Above to be given a princess daughter. Her mother told her it was a privilege and a joy to take care of a princess, and that every aspect of her life has been enriched since her Princess Rachael was born.

Princess Rachael's mother would sing her a princess song:

You are my princess

My lovely princess

Mother's heavenly gift from Above

She will guard you and protect you

And she will shower you with love

Princess Rachael most certainly felt loved.

Princess Rachael's mother would sing her this princess song:

You are my Princess

(To the tune of "You are my Sunshine")

You are my princess

My lovely princess

From your head down to your toes

Princess hair...and princess eyes

Princess lips and princess clothes

.

You are my princess

My lovey princess

Your tiara worn with pride

Serenaded by young children

A golden bunny by your side

.

You are my princess

My lovely princess

With a throne that holds you high

So that all the world can adore you

From deep sea up to the sky

.

You are my princess

My lovely princess

Mother's heavenly gift from Above

She will guard you and protect you

And she will shower you with love

By Princess Rachael's mother

Completed on May 4, 2008

Keep an eye out for :

Princess Rachael's Rabbits

Princess Rachael Donates her hair to Locks of Love

Princess Rachael Goes to the Annual Princess Conference in Tampa

Return to Top

__________________________________________

 Letters to Rachaeli, A Mother's Journal

Sunday, October 19, 2008 8:03 PM, EDT

http://www.caringbridge.org/visit/rachaeli/journal/15

I know. Three days in a row - unheard of. Before the High Holiday season ends this week, my mom wanted to post a letter that was printed in a Rosh Hashana booklet at our synagogue last year (Sept. 2007), but was originally written for the NTSAD Lifeline two years ago (2006).
______________________

*Hashem = name used in lieu of "God"
**Shamayim = Heaven
______________________






This Rosh Hashana, perhaps more than ever, I plead with Hashem to inscribe my sweet daughter, Chana Rachael bas Nechama, in the Book of Life. I understand and accept that, with Tay-Sachs disease, my almost 6 year-old child will need to soon return Home.
I trust that when the time comes, I will be ready.
But today, I am not.
Some people have quietly asked why I try so hard to keep Rachaeli here on Earth, when Shamayim is portrayed as so indescribably wonderous a place. After much thought to this question, I retrieved a letter I wrote to Rachaeli last Rosh Hashana (actually 2 RH's ago). I believe that, perhaps, my answer lies therein.
_____

My Dearest Rachaeli,

I'm so lucky to have you. Your are my perfect baby, beautiful in every way. I haven't been sad for a very long time. I feel like I'm living the best years of my life with you here, always next to me. After all, you are with us, and it is because of you that I am at home full time, allowing me to more closely enjoy your 3 brothers growing up while I care for you.

I'm going to say it again; these are truly the best years of my life. I don't spend as much time questioning why you are sick anymore. I often don't think of you as sick, unless you are running a fever or clearly have some other "additional" illness. You are my Rachaeli, and my Rachaeli happens to have Tay-Sachs disease. We have known about it for almost 3 years now (Now almost 5 years). It is a part of all of our lives; I believe we have adjusted and continued living. Your brothers haven't missed a homework assignment or a day of school because of your illness. They continue to be superstars in school and on the soccer, baseball, basketball and football fields. If anything, you have made them stronger, brighter, and more sensitive; we love you and thank you for that.

Yo ur abba and I have made changes. I stopped working, even though I still have medical school loans to pay off. But we are surviving. Your abba is working hard, and he misses you tremendously when he is at work. It is difficult for him, but I think he is also doing better.

I'm not sure how or why Hashem has allowed us to cope as well as we have. We admittedly had an extremely difficult 6 to 12 months of mourning your diagnosis and prognosis. Our emotions followed the textbook descriptions of the grieving process, although the "phases" of our grieving never seemed to coincide. Our common sense and self-control kept us from fighting excessively or calling it quits. Our faith and trust in Hashem kept us solid.

You see, your abba and I never questioned "why" Hashem did this to you or us. We were never angry at the lab for its inaccurate test results. We were never angry at our doctors or at ourselves for not being more scrupulous about our medical histories. We were never angry about having a dying child. We were just sad, overwhelmin gly sad. When you love someone so much, you never want them to leave you. You just want to hold them tight. You never want them to feel pain. You only want them to experience comfort and warmth. Your abba was always fearful that you were in pain, that you were suffering. I think he realizes now how comfortable you truly are.

My dear Rachaeli, I often wonder about Hashem's plan when it comes to children with terminal illnesses, or children dying suddenly at a young age. I have come up with many silly theories, some of which are quite comforting. One hope is that another child is not getting sick because you are still here on this Earth, occupying one of the "sick children slots". Another hope is that the effect multiplies each day you are here, so that your "sick child slot" is actually occupying multitiudes of potential slots, keeping hundreds or thousands of other children well.

I have also imagined that the positive energy that accompanies your presence on this Earth is expanding. The more it expands, the more likely it will interact with the energy accompanyin g another sick child's will or strength to survive. This energy continues to expand and interact with more positive energy. The positive potential is endless.

You see, Rachaeli, your presence in our lives, in our community, and on this earth has meaning. Your presence has created kindness, empathy, and good deeds where it may not have existed before. It has elicited pure goodness out of people and has created warmth in hearts that may have been perpetually cold. It has raised awareness in all who have seen or been privileged to hold you -- an awareness that may be indefinable or intangible, perhaps an awareness that may not be tapped into again for many years to come. Your mere presence has given people strength, vision, perspective , and humility.


Rachaeli, you have enhanced all of these qualities in me. You have allowed me to engage in numerous acts of chessed, or loving kindness, each and every day. You have made me a stronger, more loving person, and a better mother. Thank you for giving me these "best years of my life".

All my love,
Mommy

Return to top