Seattle Washington
(1986-January 7, 2014)

January 6, 2014 – The below text is taken from the Caring Bridge Site for Kristen Rice

We are creating this website to let everyone know about Kristen’s current situation. We feel that she is now almost certainly approaching the end of her life. I almost got this site up and running over a month ago, when she was hospitalized with pneumonia, but that strong body of hers pulled through and I thought we might have more time. However, she now has another pneumonia, and the Hospice team tells us that we can expect hours to days. She is at home, surrounded by more love than our hearts can hold.

This has been a long journey. She has lived with Lafora Disease for 14 years, half of her life, given that her 28th birthday is less than two weeks away. What a good life she’s had, despite the relentless progression of this neurodegenerative disease over the past 14 years. She’s already given and received a lifetime allotment of love (and then some!) She’s remained cheerful and resilient … even joyful through most of the journey, despite her profound physical and mental impairment. And she’s brought immeasurable joy into our lives along with the heartache.

I’ve posted some pictures that were taken on September 22 on San Juan Island, “wheelchair dancing” with Mom and Dad to “My Girl” by the Temptations. How fortunate we were that our dear friend Charlotte, a professional photographer, happened to be there with her camera, and captured these moments for us forever.

That’s the last glimpse we’ve had of her joyful essence. Within a month of those pictures being taken, she became wracked with uncontrollable seizures. She’s exhausted, as we all are, but she’s now resting peacefully.

If you have a memory or two of Kristen you’d like to share here, we’d be most grateful. We’d like to make this time a celebration of her life, and we’d treasure your anecdotes of this beautiful, quirky little poet and philosopher. We promise to read them to her, and I believe she will hear them and they will bring her joy.

January 7, 2014 –

Kristen has gone to a place where there are no seizures. She waited for Katie to make it home at about 10:30 last night. Once Katie arrived, her breathing became peaceful. At 7 AM this morning she drew her last breath.

Thank you all for your love and support. We read all of your entries to her as they came in, and they gave us much comfort throughout the long, dark night.

With Love,
Kim, Jim, Katie, and Kristen

To leave a message of sympathy and support for the Rice family please visit www.caringbridge.org/visit/kristenrice

Biography

Kristen was a beautiful, articulate, high-achieving, 14 year old, full of fun and curiosity, when she began to experience occasional uncontrollable jerks, which we came to learn soon after, represented myoclonus.  The early manifestations were subtle, such as accidentally spilling her milk at the dinner table and breaking dishes when she unloaded the dishwasher.  We were seen by a neurologist, who sent her for an EEG and diagnosed Juvenile Myoclonic Epilepsy (JME).    We were told that this is a quite benign type of epilepsy that is readily responsive to antiepileptic medications, usually quite easy to fully control.  So she was started on Depakote, and for a few months, went on with the life of a basically normal eighth grader.  She announced that she wasn’t going to let her epilepsy be a “big deal” in her life, and her greatest concern was that she was no longer allowed to take baths in the tub.  However, we soon began to notice increasing clumsiness, and she began to have difficulty for the first time in her life in honors math.  And rather than getting the myoclonus under control with Depakote, she began to have full tonic-clonic seizures and much difficulty walking as well. This was in the winter/spring of 2000.

That’s when I got on the internet and started learning about all the terrible forms of PME.  I couldn’t let myself believe that it could be any of those horrible things, and our neurologist was still hopeful that it was simply a matter of not being on the right medication.  On the neurologist’s advice, she was admitted to Children’s hospital for a “rapid crossover” of medication, i.e. switching her from depakote to keppra over the course of 3 days rather than the conventional gradual weaning off one medication while slowly increasing the other.  (No one I have ever spoken to has ever heard of this kind of unconventional approach.)  This turned out to be a disastrous plan, landing Kristen in the ICU in a pentobarb-induced coma.  At that point we were told that we may never be able to stop the seizures and that her survival was in eminent jeopardy.  I can tell you that only a parent who has endured this experience, watching their child’s flat-line EEG while the body is being kept alive by a ventilator, can understand that depth of suffering.

The doctors were able to wean Kristen off the pentobarbitol and on to phenobarbitol so that she no longer required a ventilator and could be transferred out of the ICU.  However for 5 weeks after that she never regained consciousness, and we began to make plans to take her home and care for her in a vegetative state.  Then one day she woke up.

She didn’t talk right away, but she smiled and really connected with us through her facial expressions, so we knew she was still there.

Only a parent who has experienced this with their child, can possibly understand the depth of that joy.  After several days she began to say a few words.  She surprised the neurologist who made rounds every day and always held up his pen and other objects for her to name.  When she actually named them one day, he was so excited he started jumping up and down and squealing, with tears streaming down his face.  After he left the room, Kristen said to me drolly, “He’s a real nut.”  Soon she was saying things like: “These pajamas are atrocious”, noticing for the first time the cute frog pajamas I’d dressed her in while she was in her coma.  With physical therapy, she regained the strength to walk with assistance.  Though she had walked in to the hospital on her own, when she left 5 months later, the ataxia was too severe.  She never walked again without assistance.

After that nightmare 5 months in the hospital, where her disease progressed so rapidly (no doubt greatly  accelerated by the unfortunate “drug crossover” attempt), her progression has been very slow.  She was never able to read after leaving the hospital, although, with great effort,  she was able to write the alphabet and tried to write some poetry, which she had loved to do before.  Her speech was very slow, but, with effort, she could produce whole sentences.  She could feed herself with adaptive utensils.  She could dress herself with moderate assistance, although it would take over an hour.  She didn’t know she couldn’t walk without assistance, so during this early period, there were many falls and trips to the emergency room for stitches.  If you turned your back on her for an instant, she’s stand up and over she’d topple.  We had a device under her mattress that alerted us when she started to climb out of bed at night.  There was very little sleep, even in those days.

Now, almost 8 years into this, she is in a tilt-in-space wheelchair because she is no longer able to support her head and trunk, let alone to stand or walk.  She is completely non-verbal, mostly incontinent, and completely dependent on her caregivers for all activities of daily living.  She is no longer able to swallow liquids and rarely exhibits any interest in solid food, although she can still swallow solids. She chokes more and more frequently on her own secretions.  She has a gastrostomy tube through which she receives her medications, fluids, and most of her nutrition.  Without her feeding tube, she would have died many years ago.

Kristen has grown from a teenager into a beautiful young woman, now 5’ll” tall, having finally grown into her long legs, but we’ve never seen her stand on them.  She has a mane of thick dark brown hair, but we’ve never seen her toss it the way beautiful girls do. Whenever I see a beautiful tall young woman walking down the street tossing her hair, I indulge myself in a brief fantasy that it is Kristen.  It does seem like such a waste to me.  But even more than her outer beauty, being robbed of her gifts and talents seems such a shame.  She was so full of potential, a writer, an artist, an environmental and human rights activist.  She was going to change the world.

My husband and I, and her home nurses, and social worker from the palliative care team, are beginning to have conversations about acknowledging that a time may come when we will be faced with a decision whether or not it is in Kristen’s best interest to continue keeping her alive through the gastrostomy tube.  We all agree that we’re not there yet, as she continues to light up the room with her radiant smile and to reach out with her shaky little arms to give the most wonderful hugs in the world.  But those moments are fewer and farther between, the farther we go on this sad journey.  Whenever I contemplate the world without Kristen, I have to go bury my face into her neck and smell her sweet skin.  She lives at home, and always will, and we cherish every moment with her and pray that the end is still a long, long time away.

Let me just end with a tribute to the amazing person Kristen is.  My sense is that she shares many qualities with the other children in this group, leading me to believe that God chooses only the most special and sweet-spirited ones for this type of journey.  Many other parents on the website have expressed the same kind of awe at the strength and grace of these children, who tend to accept their devastating losses without complaint, and to exude joy right up until the end, teaching everyone around them what is and is not truly important in this world.

– Provided by Kim Rice, mother of Kristen

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Parent Diaries

A Wedding Tribute
August, 2010
February, 2012
November, 2013
December, 2013

Orange, CA
(November 1, 1995 – April 17, 2014)

Our Angel left us on April 17, 2014. No more thrice daily meds. No more weekly blood tests. No more seizures.

Kelsey fell ill with pneumonia and in spite of antibiotic injections she was unable to take in enough oxygen. The ventilator in the emergency room was not enough. Kelsey died peacefully with her family and friends around her. Her fight is over and we are heartbroken.

Kelsey was born to Frank and Bridget Harris on Nov. 1st, 1995 in Orange, Ca. In her early childhood Kelsey lived in Irvine and attended UCI’s childcare centers and Springbrook Elementary School before moving to Tustin in 2004, where she finished elementary school at St. Cecilia’s Catholic School.

Kelsey attended Pioneer Middle School and ultimately Beckman High School where she was an honors/honor roll student. She was a senior and scheduled to graduate this year with her fellow classmates.

Kelsey was a perfectionist in every way, as evidenced by her work both within and outside of the classroom. She loved art, helping children and sports of every kind, both as a participant and an observer. She played baseball, softball and basketball but soon determined that soccer was her primary love. She played for the Irvine Premier soccer club, ultimately earning a position as goalkeeper for Beckman High School girls soccer team. While she enjoyed watching every sport, she had a special love for Angels baseball.

In addition to her parents, Bridget and Frank, Kelsey is survived by siblings Erin (24), Ryan (23), Nolan (17) and Mark (13).

Kelsey was an incredible daughter, sister and friend and will be missed by all who were fortunate enough to have known her.

Learn about the Kelsey Harris Memorial Cup

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Biography

Kelsey’s story is tragically similar to many others you can read about on this website. She was born healthy in 1995. She is the third of five children and as she grew, we quickly began to see her individual personality. Even at a young age, she demonstrated a very demanding personality, a perfectionist in virtually everything. She was always a top flight student and as a result, a favorite among the teachers. Her favorite academic pursuits continue to be art and reading. She had natural curiosity, loved going to the beach and to the pool. Always active and independent, she was the poster child for the often heard exclamation “I can do it myself”.

The migraine headaches began when she was 12, periodically leading to tunnel vision and short blackouts. When she was 13 Kelsey began to suffer a migraine during a basketball game and quickly became completely unresponsive. She was awake and her eyes open, but she was almost catatonic, not responsive to any external stimulation. At the hospital she did not react to my presence, did not turn her head or move her eyes. Only when they performed a spinal puncture to test for meningitis did she let out a scream. I was overjoyed that she had “snapped out of it”. A few minutes later I witnessed the seizure. Only because I had suffered from epilepsy as a teen did I recognize the small shaking to be a seizure. The doctor immediately gave her an anti-convulsion drug and we were directed to a neurologist and Kelsey began taking daily medication to control the seizures. Still, we were hopeful. Certainly as my epilepsy passed, Kelsey’s would as well. Along with the medication came the modest drop in her grades. She had always been a perfect or near perfect student. However when her grades began to slip, I presumed this was due to the medications. For a bit over a year she seemed to be moving along okay. At age 14 and with two older siblings, her thoughts moved to driving. Indeed when she was 15 and had another seizure after nearly two years of being seizure free, she cried, realizing that this reset the calendar and that she was again two years away from being able to drive. By this time she was still a very good student, but not the nearly perfect student she had once been. This began our journey through what another parent has aptly described as “prescription elimination”. And the difficulties intensified. In the 10th grade she suffered more seizures, often on the soccer field where she was the goalie on her high school team.

Early in 2012 she was admitted to Children’s Hospital of Orange County (CHOC) for week long epilepsy monitoring. By this time she was 16, with a reasonably normal high school experience, goalie on the school soccer team, going to football games and dances. But her seizures were more and more frequent and her grades continued to fall. Still, we anticipated that she would outgrow the seizures and that once she was off her medications her grades would return to normal. This, after all had been precisely my experience. But her physicians indicated that the monitoring showed such widespread activity that they did not expect her seizures to end, that she would be on medication her entire life. This was the first heartbreak. Unfortunately, even with changes in her medication, the seizures continued. So too did the hospital stays. Over the six months from April through September, Kelsey spent over two months in the hospital with her physicians running all manner of tests to better understand what was happening.

We received the confirmed diagnosis of Lafora for Kelsey in mid-October. Since that day our lives have turned in a direction that I previously had not thought possible. No day goes by without tears; no day goes by without the tempering knowledge that our beloved daughter is stricken with a disease with no cure. Like the movie Ground Hog Day, we wake up every morning and we are still heartbroken. Kelsey’s cognitive decline seems to be accelerating. Our 17 year old daughter who this time last year was taking AP and Honors courses, now spends her time coloring in cartoon books more appropriately suited for a child half her age. Her normally busy schedule, once filled with school, practices, games, dates and dances is now filled with daily physical and speech therapy, weekly blood tests and twice daily medication to fight off the seizures. Still, she seems happy and joyful, greets everyone with a smile and shows her love for everyone with the sweet and innocent attitude of a much younger child. The only salvation is that she is unaware of what is happening.

All we can hope for is a miracle development that will give her back that which is being taken. All we can hope for is a miracle development that will give us back our daughter.

Bridget and Frank Harris, Kelsey’s parents