Mom: Life with Rett syndrome sufferer ‘heartbreaking’

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Walla Walla is our home. We love it here. Our lives are rich with children, family, friendships and the joy of living in a small, tight-knit community. As with every other family, we experience our share of blessings and heartaches.

It is with trepidation and vulnerability that I uncover and entrust to you some truths from my “real” life. My fear is, as Jack Nicholson said in “A Few Good Men,” “You can’t handle the truth.” Inevitably, the few who get close enough to catch a glimpse of what our life really is, are struck with the countenance of a “deer in the headlights,” or an opossum frozen in trouble’s wake. When people inquire, and seem to want to know what my life is really like, I often comically advise them to turn around, take their fresh, optimistic face and run away.

Strangely, I relate well with stories we see in the movies or read in books about families who appear whole and happy on the outside while harboring untold secrets within.

As many of you know, Kate, my 19 year old, oldest child of our four, and only girl, has Rett syndrome. Kate is amazing. She is pure and beautiful with a heart of gold. There are no words to describe the incredible light and joy she brings our family and those who know her well. Kate simply exudes unconditional love, honesty and acceptance. She is also: very aware of what’s going on around her, bossy, full-care, nonverbal, in a wheelchair, unable to use her hands with purpose and unable to walk without assistance. Kate has a great sense of humor. She loves a good karate chop, or when I re-enact Molly Shannon’s “I’m 50! Fifty years old, ladies and gentlemen. I like to KICK aaaaaand STRETCH!”

Kate used to be much, much happier. She used to be able to walk independently, and was much more emotionally engaged with and connected to the world around her. Kate has always had sparkling eyes that reveal incredible cognition. It is inherent to the disorder. As she has grown, Katie has matured, and has a grasp on the fact that she is unable to do so much of what she fully understands she WANTS to do. It has left her disgruntled, less engaged, discouraged and harder to reach. Kate’s happiness and level of physical ability have plummeted. It is heartbreaking to see her life whittled to such a limited circle of people and activities.

Frequent questions

Q: What is Kate’s life expectancy?

A: Rett syndrome is something that people die with, not from. Officially, sufferers of Rett syndrome die from complications due to the disorder. International Rett Syndrome Foundation’s newsletter frequently cites young women dying in their 20s due to seizures at night. Another common reason is pneumonia due to aspiration or illness. Essentially, there are no official life span guidelines, and I have chosen to no longer live in constant fear of “the day.” The highest level of health and ability in a woman with Rett syndrome I’ve seen correlated with her advanced age of approximately 50 years. (She could walk with purpose on her own, hold a coffee cup like a pro and swear in context.)

Q: How does Kate communicate? How do you know what she’s trying to convey?

A: The best way to answer this question is to compare it with the communications of a 5-month-old baby. Although he/she is unable to speak, anyone who knows them well can tell if his/her vocalizations indicate joy, anger, hunger, boredom, weariness, fear, et cetera. When you know Kate well, you just “know.” Kate is surprisingly effective in conveying how she feels. The heartbreaking part comes when Kate effectively conveys her discomfort, but I cannot pinpoint it. At present, Kate has had some unknown thing brewing for about two months. Medical tests are normal, as is her abdominal X-ray, but not her behavior. Her mounting vocalizations — which all in the inner circle agree are indicative of pain — continue, even in light of the medication we are using to combat her discomfort. Kate’s chronic verbal fuss is unsettling, to say the least. Tension is building. Watching Kate suffer severely over the years has killed a part of me, marred my faith and simultaneously opened my eyes. Being fully present in her pain — both emotionally engaged and physically present — has taken an indescribably huge toll.

As a whole, people are really quite caring, supportive, polite and kind. Good-hearted people often offer smiles of encouragement and compassion when I’m with Kate. Kate’s teachers and aides at Wa-Hi are great; her pediatrician was fabulous; her bus drivers and caregivers are amazing. Swim teachers, therapists, staff at Providence St. Mary Medical Center that have cared for her countless times, ambulance drivers, Wa-Hi student teachers and aides, Challenger baseball peers, mentors and coach, first responders, Legacy Emanuel Medical Center, Shriners Hospital, Ronald McDonald houses, local Shriners, Kate’s case manager, Wishing Star, my children’s friends, friends and family — they are all so great, so warm. Yet, very few of you are privy to the chronic realities in our home. I have worked hard to keep it that way.

For years, I have hidden the “underbelly” from you because I don’t want to burden you or scare you off with the intensity of our reality. I WANT you in our lives. I laugh when I recall finding out my beloved former hairdresser of five years thought Kate was “just a slow reader.” Realizing the truth, she asked me incredulously, “How do I not already know this?” Laughing, I sheepishly admitted to having purposefully avoided the topic. The fact is, I want you to feel and know you are welcome, wanted and at ease when you are with me, with us and in our home. Scary, difficult things could have happened minutes before you call or come over, and I most likely won’t even mention it. There is often an air of volatility in my home due to what just happened with Kate, or could happen at any minute. I so desperately don’t want you to carry any of that. Nor do I wear Rett syndrome as a badge, constantly reference it or laud it as my identity and social club. Loathsome, but I’ve seen this happen a lot. I want you to know that we Aguilars are fun and upbeat. We love to laugh.

Things you probably don’t know

» When I’m hesitant to schedule an exact day to volunteer or do dinner, it is because I simply never know what a day will hold. I hold our calendar loosely.

» Molehills can turn into mountains quickly with Kate. Pain, a slight fever or lethargy are common indicators of disaster ahead. (Examples from the past: gall bladder removal; hospitalization with life-threatening pneumonia, and subsequent near-liver failure; a seizure that wouldn’t stop; a time when Kate wouldn’t wake up; a trip to direct admit Kate to Legacy Emanuel Children’s Hospital; a trip on an ambulance due to a fall in grade school, etc.) When difficulty simmers, I often get that sick feeling in the pit of my gut as Kate’s symptoms and tensions mount. I have accustomed myself to fearing the worst, and experience a semblance of relief with any outcome better than the worst-case scenario.

» I live by the clock, as Paul or I have to be home in order to relieve Kate’s caregivers on time for them to leave their shift, and cannot leave the house in the morning until someone is there with Kate. Most of the time, I exit an event, party or game much earlier in the evening than others, as I need to carefully budget care hours in any given month. I nearly always take a second car to evening events so that, in case of emergency or time constraint, one of us can rush home and ensure we don’t all have to miss out and leave the event.

» Caregivers are in our home much of each day. Holidays are difficult, as I try and give them those days off or schedule just a few hours. Simply put, they make my life — and time with my boys who will grow up and move out — possible.

» I feel the pain of Kate’s absence deeply when she is not with us as a family, but struggle with the truth that it’s all about her when she is. As a mother, the not-often-all-together-ness is particularly painful.

» I call Kate my pancake, as she must be “flipped” in bed a couple of times each night. With rare exception, I awaken daily to her calls.

» Kate loves the noise and chaos of the Peach Basket Classic each year. People often worry that loud noises will scare Kate. Like me, she prefers her music LOUD. If you’ve ever watched Kate’s joy and adoration watching her Grandpa Cliff cheer at one of her brothers’ games, you’d know — in the right context, she likes loud.

» While I sometimes do think of what things might have been like, had Kate not had Rett syndrome, I am able to celebrate YOUR child’s big day without sorrow. Somehow for me, purposefully and thankfully, your kid’s event, and my daughter’s disability and its effects, are apples and oranges.

» Keeping up with the care of Kate’s physical and social health, scheduling caregivers, filling out unending paperwork and getting rid of any poor-quality staff in her overall care — (a former case manager of Kate’s actually embezzled funds through Kate, among others, and was sent back to federal prison. Don’t get me started. I could tell you stories) — is like running a small business. Unbelievably time consuming, and often stressful.

» I don’t vacation much, as Kate’s needs dictate an inability to leave her for too long, and taking her with us has become something other than a break with the family. Paul will often go with the boys for a few days, or I will go with the boys, but rarely do Paul and I both go with the boys. Sometimes, we still take journeys with all six of us, but it is rare. A few days away that don’t get canceled are like pure gold. We purposefully, however, take time often to hang out with our boys. We enjoy golfing and swimming with them, and eat out far too often, as well. On Kate’s rare great days, I’ve been known to drive her around on the golf cart, and make her watch me golf. Poor kid, no wonder she’s fussy.

Kate exemplifies God’s kind of love, something we might not otherwise have ever learned or truly seen. She humbles me and blesses me daily by allowing and trusting me to serve her needs and interact with her in intimate and vulnerable ways. We do not love based on ability. Kate taught us that. I cannot and do not want to imagine life on Earth without Kate. Cringing at that possibility, I often laugh and think that, knowing Katie, she’ll probably room with us in the nursing home and live till she’s 100.

Thank you for all the kindnesses you’ve shown over the years. Thank you for the conversations you have with your children to encourage kindness toward others that are differently abled. I’m open to answering any of your questions, and honored when you ask.

Michelle Aguilar is a College Place writer, wife and mother of four.

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