Project Shui for Tay-Sachs

Abba's Online Journal

This page contains various journal entries from Shui's father's online journal on

*Video or Pictures of Tay-Sachs

Project Shui Home Page

Welcome From Shui
   How Close are we to a Cure?
   *Rachaeli's Friends
   Sign up for Updates
*Bar Mitzvah Video Invite
What is Project Shui?

Thank You From Shui
   Internet Ads&Marketing

What is Shavuot?
Shavuot & Shui's Sister

What is Tay-Sachs?
Tay-Sachs Diagnosis:

      Part I

      Part II

   Genetics Made Fun
      *Lysosomes The Movie
      *Genetics 101 Video
Narrated by Alec Baldwin
   Genetics 101 Article

*Inspirational Videos

*Beautiful Faces of Lysosomal

*Tay-Sachs Talk
   *Connor Hopf Cure Tay-Sachs
   *2010 Faces of Tay-Sachs
*My Soul Sister-Molly Grace
*Our Star Dakota
   *Rachaeli's Song of Love
   *Elise-A Child Living with Tay-Sachs
   *Last Laugh
   *Meet Gavin

Tay-Sachs in the Media
Glee Sectionals
   *Grey's Anatomy Sweet Surrender
   *Law & Order SVU MERCY
21 Below Documentary
Legal & Financial Issues

   What is Wrongful Life?
   Right to Live in the United Kingdom
   How to Pay Medical Bills?
   Social Security Disability
   CHIPRA-Insurance for Kids
   Medicaid Waiver Programs
   Katie Beckett Medicaid
   Who is Katie Beckett?
 Why We Need Katie Beckett Waiver?

Eligibility for Katie Beckett
   Katie Beckett in Georgia
Katie Beckett's Going Home
   (from People Magazine 1981)

Treatment or Cure
Canavan Pioneers
   *Gene Therapy
   Jacob Sheep Sensation
   Article in Forward-Jacob Sheep
   *Stem Cell Transplants
   Enzyme Replacement
   Substrate Inhibitors
   Should I Try Zavesca?
   Letter for Insurance 
      Approval for Zavesca
   Chaperone Therapy

In-Home Medical Care
Home Health Care
   Coping Strategies
Coping with Humor w/ Shui's Dad
Rachaeli's Story
Shocked by Tay-Sachs Article
The Project Shui Family
Mommy's Online Journal
   Abba's Online Journal
   Shui's Fun Pages


New E-book Reveals Unique Holistic Strategies to Cure Acne. Discover How To Quickly And Easily Cure Acne Permanently...Even If Everything Else You Tried had Failed...Without Drugs, Without Over The Counters, and Without Nasty Side Effects – Guaranteed!
Click Here For More

Discover How to Recognize the Signs of Autism – as Well as Learn Breakthrough Strategies That Can Quickly & Easily Unleash the Maximum Potential of Someone Who Has Autism.
Click Here For More Information on Autism.

The Day My Life Changed Forever

Thursday December 4th, 2003

12/4/2003 9:41:18 AM

Today my life changed forever. I’m sure this can be said of other days, as well, though I may not have recognized it at the time.

As I continued seeing patients, I waited for the call. All day we had anxiously awaited the Ophthalmology consult, hoping Rachaeli’s eye exam would be entirely normal. Rachaeli had been in the hospital now for over a week. We still didn’t know what was causing the aspirations that led to recurrent bouts of pneumonia. The geneticist that saw her yesterday seemed concerned about a metabolic disorder. He asked about any family history of Tay-Sachs. I took comfort in the knowledge that I tested negative for Tay-Sachs years earlier, although my sister was, in fact, a carrier. While we feared the worst diagnoses imaginable, we still hoped for the best. A clean eye exam would strongly suggest that our baby did not have a metabolic storage disease; perhaps we could rule out all inborn errors of metabolism. Even that phrase is strange -- inborn “errors” -- as if God was careless or sloppy.

At 3 P.M., I was sitting in my office, counseling an adolescent female about appreciating the incredible power she has to choose her own destiny; that she truly had the capacity to “choose life”, so to speak, or, by default, choose to go on living a largely vacuous existence. When LaTisha [my receptionist] buzzed me, she insisted that the session was over. She told me I needed to call my wife. Nicole’s voice was eerily calm and emotionless. She asked if I wanted to know now or when I arrived at the hospital. The Ophthalmologist saw a bright cherry-red spot in the back of my daughter’s eyes. I immediately understood. The process of neurologic degeneration had begun. Our worst diagnosis was confirmed. I knew, with certainty, that my life just changed forever.

As I sit here writing this note, only 8 hours have passed since the phone call. Yet this feels like the longest day ever. I have cried for much of the past 8 hours. I spent the rest of the afternoon at the hospital, after which I returned home to pretend to be normal in front of my kids as I corrected their homework.

At 7 P.M., I drove to the home of a woman whose son died at age 5. I had never spoken with this woman before, yet suddenly we were members of the same miserable club. I showed up on her doorstep unannounced. I asked if I could come in; I explained that my daughter was dying. I asked her to tell me about her son. She did. And for a brief moment, I was comforted. I needed to dilute my pain. I felt strangely better knowing she understood what I felt at that second. It was with an amazing sense of urgency that I sought this parent out. Before I spoke with anyone else, I needed to speak with someone who has been through this before me. I’m glad God made sure she was home.

At 9 P.M, M.K. [a dear friend and mentor] called. He gave meaning to this day. I asked him if God was taking away anything that I NEED. He explained that we all have a job on Earth. When we are done, we are free to go. Rachaeli’s job is almost done. We may never know exactly what it was, but, suffice it to say, it’s almost complete. When she is no longer needed here, she can (and will) go. When she leaves, I will not be losing anything I need, because she has already done her job. I know he is right. He tells me he loves me. I need to hear that.

It’s 11:30 PM now, and I’ve been back to the hospital. The baby looks just beautiful and sweet. Nicole is calm and appears together. I want someone to cry with me, but I can’t bring her down. I miss my baby. I pretend this is a dream. It isn’t. It’s just the day that changed my life forever.

Return to top


 My Daughter has an Old Soul

Saturday Night December 13, 2003
12/13/2003 9:41:33 PM

I led the davening [prayer service] at synagogue this morning. I tried to focus on the words as I sang, investing them with the painful newfound meaning they now possess. Afterwards, I let M.B. [a sensitive and spiritual friend] know that my daughter has Tay-Sachs. I remind her that when she met my daughter many months ago, she described her as an “old soul”. I asked her, today, what she meant. She says that you can look in the eyes of some people and simply see a depth; a depth that tells you that they have been here before.

I recall what M.K. said; that not everyone needs to be here for a lifetime. Some souls only need a couple of years to accomplish or complete their goals. Then, they are done. They need not stay longer than that. They have, during their short stay, achieved perfection. Perhaps Rachaeli’s old soul only needed 2 or 3 or 4 years to complete its mission. Then it will be ready to go. I think I find a bittersweet comfort here, as well.

Return to top


 Tuesdays with Rachaeli

Tuesday January 13, 2004

Tuesdays are my day at home. It’s a special treat to be able to spend time with my baby. I hate thinking that my Tuesdays with Rachaeli are now numbered.

I love that she still smiles and laughs. God, it’s incredible how beautiful she is when she smiles. I melt. It’s like an angel visiting you for 3 seconds. I’m so afraid when the smiles will go away. I know that before too long I will see her starting to slowly drift away. As her vision dims and her brain fills with fatty deposits, there will be less and less of my baby here with me. The thought of that terrifies me. Every smile is a welcome reassurance that we are not there yet. I’m so afraid of the day when the smiles are gone.

The baby loves her brothers. They make her smile the moment she sees them. Nicole is right when she says that they will never once be mad at her. They will never once yell at her. She will never challenge them, anger them, or be hurtful to them. When she leaves, they will feel pure sadness; pure sadness. Nicole says she will always be a baby. It’s true. She will never sit up; she will never walk; she will probably never say Abba again (God, I miss that sound.) But I love my baby. My forever baby. She’s my old soul. She is as pure a Neshama [soul] as you can find. Yoni [my 8 year old] reminds me that she will never commit a single sin. Never speak one angry word; never hurt another’s feelings; never break a single rule. He is wise. She is pure goodness.

I guess I like to delude myself when I am not ready for the candid unadulterated truth. After our diagnosis, I painted a picture in my mind that my child will live until 5. Tay-Sachs kids die at age 5, I told myself. I suppose I really knew that that was usually an upper limit, but I continued to envision 4 more years with my baby. This past Friday night, consistent with my sad new custom, I read from my stack of Tay-Sachs educational materials. This time, I opened the directory of Tay-Sachs families, a demographic list including the birth and death dates of each child with the illness. I start calculating lifespans of each. 3 years. 2 ½ years. Some even just 1 year old. I lost it. I need more time. I want my 5 years…But do I? Do I really? On some level I feel like part of me wants this over. I want to skip ahead to the misery of the aftermath of my baby’s death rather than sitting and waiting for the inevitable misery to arrive. How messed up is that? As I think this through further, I think that what I really want is to be ready. I guess I envisioned being “ready” in 4 years. I’m not ready now.

I dreamt 2 nights ago another of my famous vivid dreams. This time I had left the airport and was driving home. It seemed like I was leaving LaGuardia. Though I knew the way home well, somehow I found myself going in the totally wrong direction, along a highway called “Route 2”. I was torn between just continuing to drive, hoping I’ll find my way, versus eating the time I have wasted, turning around, returning to the airport, and starting over. While I felt like I could probably still eventually find my way home from where I was, I ultimately decided to return the place where I started, the airport, then making my way home on the route I knew. I would pay better attention this time. As I drove back towards the airport, I was clearly speeding, hurrying so as not to waste any further time. Suddenly I find myself driving through a rather frightening neighborhood, when all of a sudden a scary man appears in the periphery, throwing a rock or a hard ball straight at my windshield. I was going too fast and was too unprepared to swerve to avoid the projectile. I remember it hitting my windshield with an incredibly loud bang. I awoke.

With little analysis, I quickly realized how easy it was for me to find myself completely lost, desperately trying to find my way back home, where I knew I belonged. Though I knew the route well, I somehow ended up on the alternate “Route 2”. I did quickly realize my error and decided not to rationalize that I could still find my way back through the wrong path (or lifestyle); I knew I needed to return to what is right and go the way I know best, even if it takes longer. This dream reflected my incredible subconscious fear that when Rachaeli dies, I will not be ready. I will be on my way to getting myself situated and back where I need to be (spiritually, emotionally, priorities, etc.), and I will literally be blindsided by her death. This truly terrifies me. M.M. [a therapist and friend] says I want (and perhaps need) to control as much as possible; to be as ready and prepared as I can be. This illness is one thing I can’t begin to control. The dream reminds me of every movie where the protagonist finally sees the way out of his painful situation, and gets so close to escaping with the romanticized success he has envisioned. Then, in the very last scene of the film, he is robbed of his chance to achieve it. All the rooting and cheering suddenly ends with tragedy. I fear deeply that this is my fate.

Return to top


 Yoni's Dream

Wednesday March 10, 2004

I woke up Yoni before I left for synagogue to ask if he had any further nightmares about being kidnapped (vivid dreams run in the family). He immediately conveyed that he actually had a “great dream” that Rachaeli could walk. He said “we were playing Hide-and-Seek and she was incredible!” He explained, “It took me all day to find her, because she fell asleep while hiding under my bed”.

I love him so much it hurts.

Return to top


 Shui's Wish

Saturday Night March 27, 2004

I returned home from synagogue to find Shui [my 6 year old] sitting quietly alone at the table in our kitchen. “I wish ‘chaeli didn’t have to die,” he said. “She has such a beautiful face.”

Return to top


 Ari's Underwear

Friday Afternoon April 2, 2004

Ari [my 3 year old] sat next to Rachaeli on the bed, kissing her before Shabbos [the Sabbath]. “You’re the best big brother in the world,” I told Ari. He responded, “If ‘Chaeli gets big, she can wear underwear like me.”


Return to top

 My Precious Gems

Saturday Night May 28, 2004

The baby has been sick all week, with some wheezing, followed by increased irritability, more neurologic symptoms, and what seems like an increase in seizure activity. More Benadryl added to her Reglan cocktail hasn’t seemed to help. The same arm and neck movements that 2 weeks ago seemed like a baby trying to navigate her environment have been reduced to a collection of purposeless, even painful, choreiform movements associated with further white matter disease. Of course, nothing has really changed. It’s just that, once again, medical science has robbed me of the capacity to view my daughter’s existence as anything but pathology.

Within minutes of the nurse leaving this morning, I found myself again unable to soothe my dying child. As each wave of seizures comes, I’m greeted with a humbling reminder of how little I can do to stop the process that is ravaging her brain. I now feel her whole body jerk in my arms, like she’s being shocked by some sadistic force, each jerk sending her into another round of unconsolable tears. I wonder if she can see me. Am I any comfort to her at all? Her crying reinforces my deep seated fear that I am entirely powerless to help my daughter. Her mother comes in to relieve me. Rachaeli settles down. I’m both hurt and relieved.

At synagogue, Yoni and Shui behave beautifully. They leave for groups, and I am seated alone until J.A. [a dear friend and neighbor] arrives. J.A. turns and asks how we’re doing, how the baby’s week went. I suddenly start crying, something I’ve done frighteningly little of since returning from the Tay-Sachs conference in April. I apologize for reacting like this and try to regroup. The Rabbi starts his talk and I pack my things and leave. I’m too emotional to listen. I find an empty classroom and start to cry. It’s a good cry, even tinged with some anger. There’s an emotion I really haven’t made much room for so far. I spend 30 minutes crying before I regroup and pick up the kids. I feel good that I gave myself this time.

At home, Ari sits with me on the couch after lunch, as his brothers play outside, his sister sleeping upstairs. Nicole is reading at the table.

“Why is ‘Chaeli going someplace for a long time, Abba” he asks.

I immediately feel like I know exactly what he’s asking. I ask him to explain his question, anyway.

“Why is she going someplace for a long time?” he asks again, somewhat more impatiently.

“Going someplace…you mean, like to Shamayim [Heaven]?” I ask him.

“Yeah, Abba. To Shamayim. Why is she going?”

“Because she is sick inside her body,” I try to explain.

“When will she go away?” he then asks.

“I don’t know, Ari. I guess when God decides that He wants her back, He will take her up to Shamayim to be with Him.”

“How will she get there – to Shamayim?” he asks.

“God will take her there Himself,” I say.

“But God doesn’t have any hands, silly Abba,” Ari says.

I do my best to explain how it works, though I realize midway through my explanation that I have many questions of my own about the whole process.

“You know when ‘Chaeli is in Shamayim, she will be able to walk, and talk, and run, and play tag,” I explain to him.

“I want to play tag with ‘Chaeli,” he says.

“You will one day. When you are very old and you die and you go to Shamayim, you will see ‘Chaeli again and she will be so happy to see you and play tag with you.”

“How old, Abba? Like when I’m 4?”

“No, Ari,” I explain. “When you are very old you will go to Shamayim and you will see Chaeli again.” And as I explain this to him, I am overwhelmed with the awareness of how incredibly confusing all of this must be to a 3 year old. Why does he go to Shamayim when he’s very, very old, but his sister goes when she’s still a very little girl? Again, more questions I can’t answer for myself, much less for my 3 year old.

After lunch, I settle down to read some passages from Lisa Aiken’s book, Why Me, God? about the “Jewish response” to suffering. The passage about Rabbi Meir and his wife Bruria comforts me. Upon finding their 2 sons dead, she asks her husband what to do about some gems a man entrusted her to hold a number of years ago. “He now wants them back”, she explains, and asks if she’s obligated to return them, even though she’s grown quite attached to them after taking care of them for years. “Of course you must return what’s not yours if the owner is asking for them back,” Rabbi Meir explains. She then shows him the bodies of their 2 sons and reminds him of his words minutes earlier.

I read that Rav Nachman also lost several children to Typhus. Ms. Aiken quotes a Tefila [prayer] he wrote, asking for the capacity to better understand Hashem’s [God’s] plans. I think I connect with these sentiments.

It seems that we all come to accept the reality of our losses; our comfort and healing comes through being able to make some sense of these events. I’m still stuck on why she has to seem to be suffering though. I can much more readily accept my suffering than hers.

At Synagogue tonight, I’m standing near R.B., who, at 21 or so, puts her head on her father’s shoulder and says, “I love you, daddy”. I want to cry again. More things I’ll miss out on ever hearing from my daughter.

It’s late and Shabbos draws to a close. It’s been a heavy one. I’m not sure who opened the window, but I feel it’s time to close it some. We’ll see if my children let me.

Return to top


The Same 

Tuesday July 22, 2008

It’s been a really long while since I’ve written anything. I’m not even sure why I’m writing now. It’s not as if anything is different. In fact, it’s beautifully, perfectly, miraculously…the same. Rachaeli inhales; Rachaeli exhales. Sometimes she gives us a sweet soft sigh. Nicole tends to her every need. I kiss her; I sing to her. I squeeze her like she is the most precious gift in the world. Each day with Rachaeli is beautifully, perfectly, miraculously… the same. I could ask for nothing more.

This morning, I got to thinking about my relationship with God. I think about this theme often, though rarely quite like today. Today I contemplated the nature of my desire to truly know God; to experience the kind of closeness and connectedness that effaces any of the interpersonal loneliness I sometimes struggle to ignore. As I strive for the closeness of that connection, I find myself wanting to latch on, to feel the tangible presence of God’s love. Like a young child for a parent, there is an all-encompassing comfort in the security of that physical connection. It is difficult to elucidate what makes it so powerful. It's simply unlike any other bond.

Today I observed a small child sitting quietly with her stuffed animal in the waiting room of my office. The plush monkey clearly was a favorite. She clutched the small, tattered toy next to her chest as she waited for her mother to return. I watched her stroke its fur. Several times she kissed it. She held it tightly, as if this transitional object was the key to her parent’s return. It was clearly this child’s temporary connection to her mother. This soft, small, inanimate object was infused with such love and the intense capacity to connect this child to her caretaker. I immediately understood the message.

As my perfect angel has lost more and more of her physical capacities – her speech, her sight, her laughter, even her ability to move on her own – she has become my precious, perfect, plush toy – my perfect connection to my Parent in Heaven. Rachaeli is the most wonderful gift – a fragile, perfect soul that requires only my nurturance and love. God has sent her to us to care for her, to love her, to cradle her, and to comfort her – but also to comfort us. She is His gift to us -- my tangible connection to my Creator. This is my chance to come closer than ever to touch, to feel a piece of God. As I squeeze and kiss and stroke this perfect little being, I am transported to a level of spiritual connectedness that allows me, even for just a short moment, to physically, tangibly feel God’s love. He has given us the perfect transition object – the sweetest symbol of His closeness to us – that I can ever hope to be blessed to touch. I am awed by the beauty of this opportunity, as I hug my Rachaeli more tightly than ever. She gives me a sweet, soft sigh, then inhales deeply and exhales again. Today is beautifully, perfectly, miraculously…the same. I could ask for nothing more.


Return to top


 Every Precious Breath

Monday December 28, 2009

It’s been almost 18 months since my last entry. My little angel is seven. Since her diagnosis, I never believed I would be able to write those words... Seven… What a beautiful gift God has given us – and allowed us to keep far longer than I ever permitted my post-diagnosis dreams to conjure.

As I look at her sleeping beside me, I am awed by the angelic presence of this little jewel. But I also feel a quiet fear overtake me. It’s a not an unfamiliar fear, but one I haven’t felt in quite some time. Several days ago, Rachaeli began breathing differently. Her respirations are now slow and shallow. There are longer periods without any breaths at all -- punctuated by the sound of the monitor alarm indicating that her oxygen level has fallen dangerously low. I watch this with unease, fighting to suppress what I know this pattern signifies. This is different for Rachaeli. It’s not a pattern I’ve seen before. One poignant reminder that we are inching towards exhaustion. A sad sign that my baby is growing weaker.

I suddenly crave yesterday -- and every Tay Sachs day before that – when each breath was a sweet word of comfort. I worry that tomorrow I will miss today.

I’ve allowed myself to bask in the wonderfulness of the status quo – a perfect island of sameness that appeases all my fears of letting go. But now I feel myself being pulled back into fearfulness, as I struggle to find ways to prevail. Its pull is as strong as my greatest defenses. I wish I could be strong like Rachaeli. Or like her mommy. I am not.

I watch Rachaeli breath again…then pause. I wait for what feels like an eternity until her next breath. I wonder if she is visiting God. If He is hugging her so tightly she can barely breathe. If each apnea is a Godly hug. I see suffering, but she feels love. I am briefly comforted as I contemplate this imagery. Then I wait for the next breath, and wait…quietly wishing that God wouldn’t squeeze her quite so tight.


Return to top

Website Builder