If you are looking for stories of entitled white women assaulting others with their privilege, this is kind of the opposite of that. My sister Karen lived her entire life with Lennox-Gastaut Syndrome and passed away on June 12 from metastatic breast cancer. The main connection to white privilege is that I acknowledge that she may have had a harder time getting adequate medical care if she were not Euro-American, and that she grew up in a family of modest means, but not in poverty. There is no cure for Lennox-Gastaut Syndrome (LGS), or usually for metastatic breast cancer. It has taken me a month since her passing to be able to write this, not least because I do not expect that many people will be interested in my sister’s story. But my sister deserves to have her story told, and if it helps to raise awareness about LGS and other developmental disabilities, so much the better. Even those with limited verbal skills have voices, and my aim is to amplify hers.
My earliest memory is of waiting outside the hospital where my sister Karen was born. I was not quite 3 years old and it’s possible this is a ‘reconstructed memory’ from hearing about it, but I can picture the rows of windows and I think it is real.
When Karen was 20 months old, she had her first seizure. My dad saw it and knew what it was because his brother Curtis had had seizures. What might seem like a probable genetic connection was never established. Curtis had fallen out of a car when he was a toddler and the family thought that he suffered brain damage, whereas Karen’s seizures had no known cause. Curtis died young in an age of rather primitive treatment for epilepsy, whereas Karen would have turned 61 on June 24. Most of our lives we had no diagnosis for her other than epilepsy with mental retardation. She had a mental age of about 5 or 7, depending on the task. It was not until Karen moved to Seattle at the age of 54 and saw a new neurologist that we got the name Lennox-Gastaut Syndrome or LGS to describe her condition. Even so, LGS is not one disease but a description for people with childhood onset of multiple kinds of intractable seizures and a slow wave EEG, almost always with cognitive impairment. There are both genetic and non-genetic causes for LGS, and Karen remains unique, with symptoms not exactly like any other person with LGS. Many affected LGS individuals are non-verbal, confined to wheelchairs, may have feeding tubes, and some die quite young. In this regard, Karen was more fortunate, though like many other LGS individuals she had daily seizures despite four different seizure medications and an implanted vagus nerve stimulator. In the last year, modern genetic testing found a mutation in one copy of Karen’s PIGO gene. Seizure disorders have been associated with people with mutations in both copies of the PIGO gene, but it is still inconclusive whether this single detected mutation was causative for her neurological disorder.
As a child, my parents tried to give Karen as normal a life as possible. She was a Girl Scout and sold more cookies than anyone else in the troop. She went hiking, swimming, canoeing, and camping with us, often on some rather long trips. She climbed Half Dome in Yosemite (with considerable help from my father). Hot weather sapped her strength and we often had to wait patiently while she recovered. Sometimes she could sleep through anything, including bouncing mountain roads that made your head hit the car roof. She loved to sing camping songs and usually remembered the words better than her siblings. She memorized songs from musicals like Mary Poppins, the Sound of Music, and the Man of La Mancha. She would help Mom make cookies and always liked to celebrate holidays.
Karen was physically strong because of her seizures, even with her skinny arms. She could grip you so hard it was nearly impossible to get her to let go, and when she was angry she could turn into raging berserker, not unlike the Incredible Hulk. Fortunately, she was usually sweet and cooperative.
Though she loved to dance and skip, her balance was always imperfect and became much worse with age, probably as a result of damage to her cerebellum from seizures. When she was 8 or 9 and we stayed over at someone’s house, she went to brush her teeth, opened the wrong door, and fell down the stairs. She could never balance on a bicycle, but in her teens she learned to ride an adult-sized tricycle. Of course she still needed someone with her at all times to help her negotiate braking and traffic. I don’t remember when her balance became a big enough concern that she started wearing a helmet routinely, but it may have been after she moved to the state school as a young adult. At the state school she lived for too long in a dormitory with much less capable kids because the staff determined it was not safe for her to be on stairs, which angered me because she had navigated stairs her whole life. Eventually, though, she found a place in an excellent group home with 5 women of similar capabilities and with no stairs. In her later life she used a walker for balance, and for the last year or two we began using a wheelchair, which I liked because we could move faster, and which she liked because she could push herself around her house without worrying about falling.
At the group home in Utah where she lived for more than 30 years, each woman had her own room and had tasks to help with housework. Sometimes they would go to work at simple jobs. There was a house with six men across the breezeway with whom they sometimes socialized, and there was a public pool close by, where my mother would take Karen swimming regularly. The house had a cat and a living room with big couches and a big TV where the women loved to watch Wonder Woman. They would all cheer with delight when Diana changed into Wonder Woman. Occasionally the women and staff would go out for entertainment such as Holiday on Ice, or a visit to the zoo, or go on trips, including to Disneyland. My mother would often go along as a volunteer to assist.
More than anything Karen loved visiting family. She and Mom would take trips to see us other siblings, especially for Christmas. Karen was proud to be an aunt and loved meeting pets. She enjoyed giving gifts, though she was usually much too excited to be able to keep the contents a surprise. She enjoyed receiving clothes, puzzles, and sticker books, or anything related to Disney princesses, though as an adult she seldom played with dolls.
In 2013 Karen was diagnosed with breast cancer and had a mastectomy and started hormone therapy. Only a few months later Karen and Mom moved to Seattle to be near me, as Mom was developing more health issues and was already in her late 80s and knew she would need help with Karen. I became co-guardian of Karen and then guardian after Mom died.
Karen processed grief differently than most people. When Dad died and one of the hymns in in the memorial service had a good beat, she danced in the aisles. At Mom’s memorial service, she similarly sang exuberantly to an old favorite “I Love the Mountains”. I never saw her cry over these deaths, but sometimes she would hide her head against my chest when we talked about how we missed Mom or when she decades after the fact would tell me out of the blue “We miss Dad, don’t we?” For Valentine’s Day, she asked me what we should get Mom, and I had to explain, “Karen, Mom’s dead, we can’t get her anything” She asked again and when I gave her the same answer she looked at me in exasperation as if I were a complete idiot and said “I mean, what would we get her if she was alive?” Thus clarified, we went out and got Mom something. Last Mother’s Day, when Karen wanted to get Mom a card, I suggested flowers, thinking she and her housemates could enjoy them, but she would have none of it and I knew we needed to go pick out a card.
Karen developed a colon tumor that had to be surgically removed in 2018. Though that tumor was not cancerous, in the process of checking we discovered that her breast cancer had metastasized to numerous locations on her spine, lymph nodes, sternum and other places. Over the next 2 years, we switched to different hormone therapies and then through two chemotherapies. She never complained about chemotherapy. When Covid hit, I was not allowed to visit her at her home because of the risk to the other residents and caregivers, but I could still take her to chemotherapy, so we still had frequent visits. We discovered that Karen had a germline mutation in the ATM gene that confers a 48% risk of breast cancer in women and lower risks of other cancers in both men and women. Recently I learned that both my aunt and grandfather had nipple cancer, which might have been caused by this mutation. I do not have the mutation, and other family members will be be tested so that if they have it they can be regularly checked to catch any cancer early.
In May, Karen went the emergency room because she wasn’t sleeping or eating well and began having very severe pain in her back from bone metastases. Testing revealed she was severely anemic from chemo, and she was admitted to the hospital. At first the hospital staff told me I would not be allowed to visit, but once they realized how much help I would be in dealing with a cognitively impaired seizure patient in considerable pain, I was allowed to stay. After her Covid test came back negative, we were moved to the Covid-clean floor. It took 3 units of blood to get her back to an acceptable, but still low, red blood count. After 12 days in the hospital with dim prospects for further chemo and still poorly managed pain, she went home to hospice care. My brother came from Idaho to help. The caretakers at her home, most of whom are African immigrants whose first language is not English, welcomed us and dispensed with Covid restrictions on access for us. They worked round the clock to try to keep Karen comfortable, and to allow my brother and me to get some sleep. They fed us breakfast and we brought them chocolates. Over the next week and a half Karen stopped eating, then stopped drinking, and eventually stopped breathing,
After I posted Karen’s passing to the Facebook page for LGS families, I was surprised that over 530 people whom Karen had never met sent condolences, and one person said that she considered Karen, who was the oldest LGS person in the group, to be the matriarch of the group who gave hope to parents that their child will have a long life and be well-loved.
We had a Zoom memorial enchilada party for family members, because enchiladas were Karen’s favorite food in the last few years. My niece said that she thought Karen was the most important member of the family because of her unconditional love for family members (that she was not at all shy about demonstrating in public ways that might embarrass the recipient), and that Karen had taught the whole family to have compassion for those who are different. I count myself among those so taught.
We had a separate pizza party for the caretakers and residents at Karen’s home. Some of the caretakers had been in the room with me, holding Karen when she passed, and were quite distraught. Though some of the residents are non-verbal, it seemed clear that they all missed Karen. One wept openly.
Karen received an economic stimulus check! When her rent and other bills are paid, there will be money left, and I told my brother I thought I would donate it to the LGS Foundation. He said he was fine with that, or he would also be ok if it was donated to advance the Blue Wave, so maybe I will do some of both. Though Karen was not competent to vote and probably did not really understand what a democracy is, as someone who had relatively little control over decisions in her own life, or even over her own body, she still understood fairness, respect, and opportunity. Love of family was what she lived for. I like the idea that she has the power to contribute to saving our democracy and ridding us of someone who mocks the handicapped, promotes racism, takes freedom from children, and separates families. Karen never did any of those things, and would not approve of them. If money is actually speech, then let her voice be heard.
If you have made it this far, thank you for listening to Karen’s story.