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Thursday, December 20, 2012

I Sing the Body Electric

Kenan is taking 4-6 breathes/minute and is holding his blood oxygen levels at 98%. The normal respiration rate for toddlers, 1-5 years in age, is 20-30. It is something to be awed by, his ability to adapt, compensate then function with extreme efficiency. What Kenan is performing on a physiological level makes him a BREATHING GENIUS, a SUPER STAR, a GOLD MEDAL OLYMPIAN. If you need something AMAZING to happen today, it is him.


Saturday, December 8, 2012

Thanksgiving


Part I: I had a small rubber ball sitting in the palm of Kenan’s hand when a clonus jerk of the arm (involuntary retraction of muscle) caused him to throw it, a fair distance considering. For a split second the illusion in my heart of what he could and should be doing “ if only” was set free: MY BOY HAD JUST THOWN A BALL! It was a gift of effect wrapped in the sadness of cause and it was just for me.




Part II: Gratitude Tamsen style (stickers compliments of our local grocery). 



Part III: Leftovers

Kenan was at the Thanksgiving table but fell asleep before pie. Today is a different story. His plate includes cranberry sauce, turkey gravy and a pumpkin pie-whip cream slather. He was most motivated by the cranberry giving us alot of swallow. So proud of him.
 




Part IV: The Wishbone and Sibling-hood

Tamsen ended up with the larger side and in her big sister way, tried to crack IT in half so she could share the winning piece with her brother. Not sure what her wish was but her continued display of devotion to Kenan granted one of mine.







The Armor: Preventing Morbidity

There are the symptoms of Krabbe and then there are the side effects of those symptoms. 

Babies are born with loose joints for good reason. It’s through the process of resistance gained by kicking, rolling, crawling, and weight bearing that allows their joints and bones to set. Kenan was a strong little boy but started loosing muscle tone around 6 months. He never made it to sitting up. Because of this there are skeletal (and internal organ) consequences: his hips never set and are migrating out of their sockets, his spine is starting to curve in the direction I typically hold him, his fingers are now hypermobile (double jointed) to name a few. These orthopedics are designed to help prevent and correct misalignments. From top clockwise: hip brace worn at night to set joints, knee stabilizers that help support his lower body when I hold him in a standing position, AFO’s for foot alignment and ankle strength when standing, resting hand splints to help relax the hand muscles as well as align the bones. Each of these is worn daily and some in tandem for various durations of time.



And the reason his orthopedics rock our world: a chance to hug.



An Introduction to Kenan’s Equipment Arsenal


Meet the Food Pump: Around the time of diagnosis, Kenan started having choking episodes while feeding. As these became more frequent we elected to give him a g-tube. It is a simple surgical procedure that allows formula and medications to bypass the mouth/esophagus and go directly into his stomach. The pump lets us to set both volume and rate of flow. He currently receives 4oz over the course of 2 hours 15 minutes 4x/day, what his body can tolerate. In most photos you see Kenan with an extension and syringe off of his belly. Kenan was given a Nissan Fundoplication (a plug on his stomach) to prevent acid reflux and aspiration that can lead to respiratory infections and/or pneumonia, another side effect of low muscle tone. Because of it, he can no longer burp and has to expel air through his tube instead. When his respiration is high (for him), between 8-12 shallow breaths/minute, this is a problem as air fills his belly requiring constant maintenance. When his respiration falls between 4-6 breathes/minute, his breathing is much deeper and there is less belly air to contend with. (For anyone concerned about his breathing, he maintains O2 levels of 95% during both phases of high and low respiration.)




The Vest: This is primarily a preventative measure. There are many side effects from lack of mobility, respiratory infections being one of them. The vest gets wrapped around his torso, is set to a designated pressure, frequency and duration, inflates with air and shakes, shakes, shakes, loosening the congestion that has settled in his lungs. He receives these treatments 3X/day for 15 minutes each and tolerates them well. The downside is he spends the rest of the day working at bringing everything loosened, up, a noticeable aggravation to him and constant maintenance for his caregivers. The benefits certainly out way the drawbacks but the door to quality of life issues get opened for me with this therapy.




The Suction MachineOut of all of Kenan's equipment, this is the most depressing and poorly designed machine, especially when you are in a state of urgency, yet crucial to his well being; without it he would have choked to death 100 times over. A year ago, it spent most of the time sitting in the corner. Today, when awake, approximately every 3 minutes, Kenan needs assistance either bringing the buildup of saliva and mucus out or holding his mouth closed to help him facilitate a swallow. To keep everything as thin and manageable as possible, we keep him well hydrated with 2 oz of water between his feeds and 5oz of Pedialyte through his pump over the course of the night. The Vest and saline treatments through a nebulizer play a role in keeping everything moving, but point black, we take for granted what it means to have a body in motion.







Both My Children


Both my children went to the poles on November 6th. Tamsen physically: I was impressed with her level of understanding, touched by her disappointment in neither candidate actually being there and grateful she caught one on my ballot that I had skipped. Kenan’s impact was more outreaching: I was humbled by the number of individuals considering which presidential candidate best represents him and our family, both in healthcare reform and medical research. It is profound to experience Kenan's life having influence on the very personal and sacred act of voting, that his voice was the momentum that pushed the pen to complete the arrow constituting choice. It all started by simply not wanting to bare it alone; everything that followed has proven greater. The act of advocacy need only start small.



From November 11th


Quiet Noticing.


Krabbe (Crab-A)


A simplified explanation of what is happening to Kenan: Because of the mutation he inherited from Dann and I to chromosome #14 (one copy of the mutation was passed on from each of us making a pair), his body is not producing enough of an enzyme called GAL-C. In the process of metabolism, GAL-C is required for cells to eliminate waste. Because his cells are unable to eliminate waste, it is building up in his brain creating a toxin that is destroying his myelin. The myelin is the coating that surrounds the nerve. Without it, the signal between brain and muscle cannot get through. In other words, there is nothing wrong with his cognition, his muscles, his organs, or even the signal; it is the conductor that is failing him. It begins with peripheral nerves (voluntary functions like moving your arms/legs) then moves through the brain stem (involuntary functions like swallowing and breathing). The reason Krabbe is so sever in infants:
 a baby’s brain develops rapidly during the first year of life with myelination being integral to this process. Kenan produced enough enzyme to get him through the first five months of life when his body’s demands outgrew his ability to supply. 

If there were a cure for Krabbe today, it would need to be threefold to benefit Kenan: It would need to allow him to produce the enzyme, it would need to repair the damaged myelin and it would have to remove the toxic buildup left behind. 

All Leukodystrophies affect myelin production.

Drawing of a “Brain” for Kenan by Tamsen (9/12).



Saturday, November 24, 2012

Halloween Part II

Even Better: Halloween Candy 





















Every child should have the flavorful experience of artificial lime that only Skittles can deliver. Kenan's speech and swallow therapist had the brilliant idea of dissolving them in water. Lime turned out to be his favorite. The others, when dissolved, lost what made them distinct and only tasted "sweet". 

Halloween Part I


















Kenan had his first date and with the stunning Lauren from Canada. Gabriel, our friend from Mexico, hosted a virtual Halloween Party for all of the kids. Below is a link to the parade of costumes.  I am often grateful for Facebook but even more so at times like this when we gather as a community, strengthen our bond to one another and satisfy some of the hopes and dreams we have for our children. Thank you to Gabriel's mom, Mayte, for getting us there.

https://www.facebook.com/media/set/?set=a.331727600259959.73060.221567744609279&type=1

October 27th






















Today's morning coffee: We have dragon, tiger and dolphin mom; how about Hulk Mom? Kenan spent part of his morning struggling to exhale: He'd take a breathe but the neurological signal to let it out was significantly delayed. Once he could, he'd cry and then it would start again. You do what you can to try to break through it and if all else fails and he is in too much duress there is morphine as a comfort med. My objective is to broaden the understanding of what Kenan and the kids of leukodystrophies are up against on a daily basis.

October 23rd

This afternoon we took in "100 Electric Signs", a video projection by Neal Vandenbergh at the Hyde Park Art Center. Kenan suffered optic atrophy at the beginning of this year: he can no longer track or focus and for a while had a startle response when approached. Like most with severe vision impairment, his hearing has compensated and he now is very good at following movement through sound. K's excited response to this silent piece (gratefully) confirms that he is still able to see something when in high contrast environments, similar to his reaction at night when a light it turned on. The annual Lincoln Park Zoo lights will take on new meaning this holiday season.


October 22nd

Kenan nestled amongst the prairie dunes; a pretty fall picture from this morning's walk along the south side lakefront- our attempt to get every last run, walk and trip to the playground in before the weather turns.


October 16th

























More fun today running, rolling and kicking in the fall leaves. (All verbs apply to our K-man.)

October 14th






















Two seconds later we got caught in a downpour but he loved it while it lasted.

October 12th


The Gradual Education of Tamsen

Many of you ask how Tamsen (just 4 yrs) is and how she is handling things with Kenan. Here is part of a recent conversation. It is an ongoing dialogue we’ve had since his diagnosis. This is the first time we talked about Krabbe leading to death. 

Tamsen (T): Why are Kenan’s legs kicking?

Me (M): It’s called “dystonia”. It’s from the Krabbe.

T: Why?

M: My explanation becomes too technical and I loose her.

A few minutes later…

T: Remember when we went to Hunter’s Hope and met the other Krabbe Families?

M: Yes.

Five minutes go by. She pulls out a rubber rat from her bin of toys.

T: Sam gave this to me. He was at Hunter’s Hope.

M: Yes. He had a sister with Krabbe.

T: But she wasn’t there.

M: That’s right.

T: Why?

M: She died.

T: Why?

M: From Krabbe.

T: Why?

M: Because that is what happens to kids with Krabbe. Their bodies stop breathing.

T: But Michael has Krabbe and he can walk. (Michael Wilson, Marshall’s little brother.)

M: Yes, that’s because he had a transplant. 

M: And Bella’s little sister, Jacque, also died from Krabbe.

T: Aw, that’s sad...but Bella is still a big sister...her baby Jacque is in her heart...and Bella is in my heart... and Michael and Sam are in my heart.

T: I made the enzyme. Kenan did not. 

M: That’s right. 

T: Mommy, want to play face painting?

At some point she will make the connection between Kenan and the loss of other siblings. My hope is that it is in her time and not that of the disease. An immediate sense of relief comes from knowing that Kenan is in her heart (something she says to each of us regularly), that she will continue to see herself as his big sister when he is gone and that she feels the same connectedness with the Krabbe community that we do. 

Thank you to Hunter’s Hope for also helping us care for our healthy child.




11/24/12: In the conversations to follow with Tamsen I've learned, from her perspective, that you are either alive with Krabbe or dead with Krabbe. She does not understand that there is a process to dying. She was 2.5 years when her was born and just 3 when he was diagnosed. So far, the symptoms that have taken over Kenan have all happened gradually lacking crisis to signal a critical turning point. 








October 11th

A year ago today Kenan was diagnosed. In spite of my acceptance, there is grappling. Today owns me.

October 3rd

















And another Mozart lover is born. No other musician elicits this type of reaction lasting the duration of the CD. In this photo he is listening to Symphony 40 in G minor.

September 28th





















Kenan saving the world from the out of control, giant, atomic ball today in physical therapy.  flipped the photo on its side out of my need for him to have more "boy fun".

Thursday, September 27, 2012

One Year Ago Today





















This is a photo of Kenan coming out of sedation from his diagnostic MRI. The images would prove inconclusive but it began the process of more than just explaining his symptoms, it led to a true understanding of our son. Two weeks later we would hear the words Krabbe Leukodystrophy for the first time. Although the disease has taken much in the past year, it has not taken him. And for that, our gratitude for all things Kenan overwhelms the words right out of me. 

Saturday, September 15, 2012

As the Smaller Things Become Smaller



Like a bolt of lighting dividing the air, this is the one sound that can separate my hope from my despair. For the few seconds that it lasts, I am suspended, free from the grip of either; I am at peace and so is he. This is Kenan's sigh: what graciously remains of his *non-pain, verbal communication. The second his sweet voice fades away, the two extremes crash back in on me. 

There will come a day when Kenan sighs his last sigh just like his smile, laugh, and cry before it, only we won’t recognize it as such. It won’t announce itself. Days sometimes weeks will go by before we realize it is gone, along with the chance to name it "Last".

*I've used the word non-pain rather than pain free because pain free as a state of being does not apply to any child with a leukodystrophy.


video

Monday, September 10, 2012

A Wish for September

September is Leukodystrophy Awareness Month and Kenan is making his wish.

Saturday, September 1, 2012

We Love Summer Series

We Love Summer #10: 8/31/12
















Everyone needs alone time: Kenan napping amongst 
the minnows at South Shore Beach. Happy Labor Day 
weekend everyone!

Friday, August 31, 2012

Drawing Time















Kenan Gets Drawing Time: Kenan suffered optic atrophy many months ago, but there is more to drawing then seeing. Markers, crayons, pastels and pencils all have different sounds, different smells, different textures and feel very different making marks on the paper.

Ice Cream Sundae





















We celebrated Tamsen's 4th Birthday with an ice cream social. This is Kenan's version of a hot fudge sundae: partially melted ice cream, thinned hot fudge and maraschino cherry juice, 8/26/12.

We Love Summer Series

We Lover Summer #9: Millennium Park -Chicago, 8/18/12. 

















Even before having kids I admired Jaume Plensa's Crown Fountain for the immediate access it offers special needs children. In addition, the Air  Show was taking place up above. Kenan took it all in with his usual alertness.

We Love Summer Series

We Love Summer #8:  Hot Sun, Cool Air, Lakeside Napping, 8/11/12.

Saturday, August 11, 2012

New Sharon, Maine

                            
Kenan makes his float debut on the East Coast (far left). Thank you to Jamie Davis and her beautiful daughter Addilyn (second from right, Krabbe diagnosed at 6 months, now 11-months-old) for raising awareness and advocating for expanded new born screening in Maine.



Wednesday, August 8, 2012

We Love Summer Series



















            We Love Summer #7: The Hunter's Hope Family Symposium

After a long day of informative lectures on new born screening and Krabbe research, Kenan and I found a babbling brook to listen to on the grounds of the Holiday Valley Resort, NY. Although he lost his vision at 12 months, this photo distinctly portrays his receptive listening abilities and how attuned he is to his environment. 

Monday, July 30, 2012

Dusty Blonde Hair


There are the watershed moments of grief that one expects and then there are those that catch you off guard.

It is an afternoon like so many others. Kenan and I are driving through downtown during lunch hour. We are detoured by road construction from our regular route to pick up Tamsen from school. While stopped at a red light, my eyes skim over the busy cityscape then lock in on a young man waiting on the corner. He is twenty something, attractive, well dressed, with dusty blonde hair. A car pulls up beside him. He smiles and embraces the woman getting out. My mind immediately races into the future - a future for Kenan that will never be. Before me is a mirage of potential and possibility shifting in the sunlight trying to take shape. There will be no culmination of collected moments to answer my questions: What will HE look like? Who will HE be? For a few seconds more I linger on the young man at the corner until the green lights snaps me back into the intersection. I look at Kenan in the rear view mirror. He is awake now in his car seat. No one wants a parent to project onto him what he isn’t and can never be, least of all Kenan. 


Thursday, July 19, 2012

I’m Not a Singer, But I Am a Mom Who Sings


Tamsen recording her track.
We wrote and recorded a song for Kenan, a tribute, not only to him but also to big sister, Tamsen. After all, so much of why we had Kenan was for her. There is nothing like the sibling bond, that special connection that gives perspective and witness to everything that is our upbringing. Tamsen was three when Kenan was diagnosed with Krabbe Leukodystrophy. Although she has the memory of an elephant, there is no way to know what she will ultimately retain from her time with her brother. You wish you could bestow on her the concept of “relishing these moments”, but that sense is underdeveloped still and you do not want to create a void where there need not be one. Tamsen simply “is” with Kenan and that is the beauty she brings to our dynamic. The song and her contribution to it will remain as testimony to their mutual affection and love. In time, she will also understand the way she helped me. That I could give them both this gift is monumental.

Kenan's rattle toy was played as percussion.
On two separate occasions, validation was given to me: One afternoon, when Tamsen and I were singing his song, Kenan had a break through moment in his abilities and was able to make his laugh sound for us. Similarly, just this past week, as I was playing the recording for him, Kenan tried to sing. He opened his mouth and made sounds I had never heard before. These are the ultimate moments, when the exchange of gifts between parent and child change who you are.

I want to take a moment to thank our music therapist, Rachel Epley. Occasionally, someone enters your life and you know everything that led up to this point brought you two together. Without her, “Kenan” would not be. Thank you also to our sound engineer, Victor Sanders of Lakeside Media, who donated his studio, time, and artistic insight.


Lyrics and Melody: Natasha Spencer
Vocals: Natasha Spencer, Tamsen Witczak, Rachel Epley
Music: Rachel Epley, Music Therapist, Horizon Hospice and Palliative Care
Sound Engineer: Victor Sanders, Lakeside Media


Kenan lyrics

Asking too much of the words
Cause I’m missing my tune
All I really want to do
Is say “I love you”
Kenan, Kenan

Feeling backed up in a corner
Stuck on warped speed
I’m going to get us out
Fight, kick and scream for you
Kenan, Kenan

Your ten little fingers
Ten perfect toes
Not enough to take us
Where we thought we’d always go
Kenan, Kenan

This is too much
It’s not enough

Your sister marches in
Drops to her knees
Looking for that special spot
To kiss and squeeze
Kenan, Kenan

I open up the window
Looking for a breeze
She’s got you head-to-toe in stickers
Like flowers in the spring
Kenan, Kenan

Your sister takes me away from you
Then leads me right back to you again
It’s in the simple way she loves you

This is too much
It’s not enough

This is too much
It’s not enough

Kisses, Kisses
Kenan, Kenan

We love you
Kenan, Kenan

Our Keenster
Little Keenster

My Keeny, Zuchini
Our Little Monkey

Our little boy
My Little boy