Looking Back

It is difficult to sit and put into words the enormity of the situation in which we live day to day. My tendency is to look at the here and now and work with whatever situation is presented to me. Doing a mindless task such as laundry is an easier option than documenting the severe progression this horrible disease is taking on my daughter’s life and on my heart and soul.

Looking back over the past two years of photos help to jog my memory of how things were and how much of a change has come over Chelsea. Her overall demeanor and health remained fairly intact the latter part of 2009 and early part of 2010; which is really to say that while so much of her ability, both cognitively and physically, has been lost, she has remained stable with no major medical emergencies or illness. Life remained somewhat steady which seems such an odd thing to write, but our day to day routine has, for the most part, been very predictable.

I know Chelsea will sleep until about 11:30 a.m. which is good, but with that comes the fact that she is usually awake at night until about 11:30 p.m.; which is not so good, and makes for a very long day. In that regard, she is very much like any other teen. Most nights she sleeps well enough, although there are several weeks every month when her hormones surge, which promotes seizure activity. During these times, she will typically experience anywhere from two to six grand mal seizures during the middle of the night. These nights are brutal!

Generally she will sleep through these episodes, unless of course when she does not…and she awakes terrified of what is happening to her. Through the monitor I keep by my bedside, I hear Chelsea’s every breath and, of course, the beginnings of her seizures which generally only occur when she sleeps. These seizures are loud, frightening and often very violent that at times Chelsea has been flung over the side of her bed or even onto the floor. Howard and I run down the hall with our hearts racing and adrenaline pumping to be with her. Thankfully she has never incurred a serious injury more than an occasional bruising during these episodes. Most often after a grand mal seizure, even though she might not wake up, her breathing may have stopped and she may have turned blue for a few moments, which is absolutely terrifying. We have an oxygen concentrator available to assist her in gaining a more calm composure that will allow her to be restful again.

With our hearts still pounding, we make our way back to our own bedroom, to try to regain our composure and hopefully fall back asleep. I have gotten used to sleeping lightly and waking at a moment’s notice when I hear changes come over the monitor.

As time and the progression of this disease move forward, it has become more and more difficult to function as we always have in our home without making modifications to accommodate Chelsea’ps changing needs. Her bedroom, since she has been 3 years old, has been upstairs next to her brothers and down the hall from ours. Collin and Chelsea have always shared the bathroom closest to their rooms which was big enough for the two of them complete with a shower over the tub. However, the doorway was not quite wide enough to allow for a wheelchair, which Chelsea now needed most often for ambulation.

There were times when I would scoot her to the bathroom in a rolling office chair that way I could get her all the way close to the tub. While we did have a shower bench for Chelsea to use, it was extremely difficult and not very safe anymore since Chelsea really could not support herself well sitting up. We were in such a predicament. Daily living was becoming much more demanding on our backs and life was becoming so limiting with our present situation. We had to make changes as it was unacceptable to allow my mind to think that at some point Chelsea would not be able to get downstairs again, to go outside, to feel the sun on her face, to be where most of our daily living is done.

Once ready for the day, Chelsea and I would “live dangerously” and make our way down the 15 steps to the downstairs. Many times, I would have her lean against me as we slowly attempted the steps, always fearing we would tumble to the bottom. Sometimes I would sit behind her with my arms wrapped tightly and we would, bump, bump, bump on our behinds all the way down those 15 steps to the awaiting wheelchair. It became a silly game, although much too dangerous to continue.

Chelsea would remain downstairs until bedtime to at least be able to get on the couch, go outside in the backyard or be taken out for a walk in her wheelchair. At the end of the day Howard and I would carry her together creating a chair with our arms while Chelsea held onto our necks or Howard would scoop her up like a valiant prince carrying his princess.

It is so worrisome fearing one of us would get hurt if we continued to live this way. What if our backs gave out? What if we couldn’t maneuver Chelsea as we needed to? At this time, we could not afford a remodel but we needed to make changes so that we could offer the best possible living situation for Chelsea.

A solution to our problem came to us via a friend who shared our story with our town of Danville’s local rotary club, The San Ramon Valley Rotary. After reading Chelsea’s story in our local paper, the rotary club chose to build out our downstairs bathroom, equip it to be fully handicap accessible and adjoin it to our extra bedroom and create a beautiful living space for Chelsea as one of their main projects for 2010. This was a truly amazing act of kindness, a labor of love and generosity that changed the course of our daily life.

The San Ramon Valley Rotary Club led by designer Deborah, architect Rex, contractor/carpenter Chad, Valerie, Gordon, Tom and many others who give of their time to worthy and philanthropic causes stepped up, befriended our family and have shown us the power of community.

Over the next several months in the early part of 2010, mainly working on Saturdays, our downstairs bedroom and bathroom were being transformed into a beautiful living space for Chelsea. She would have her own bedroom and adjoining handicap bathroom with a wheel-in 5’ x 6’ shower that would allow us to more easily care for her.

While Chelsea could not really understand the changes to her space that were occurring around her, I think she did recognize and sense that things were different. When all was done, and her bed, being the last thing to be put in place was set up, we brought her into her new space. Her smile was so bright and her eyes wide with delight, as she took in her room and special friends that created it for her. Her new bedroom was painted and decorated beautifully with a Chelsea mural above her bed, new floors, bedding, décor and even a flat screen TV, a gift from her Grammy.

This tremendous and very generous gift to Chelsea from The San Ramon Valley Rotary Club allows her to be a part of our daily living, to be among visiting friends, and enables her to enjoy the outside. We are forever beholden to these wonderful and creative friends.

As Chelsea lay in her bedroom at night, her eyes would wander the room to take in all that was new. It was so sweet to watch her and I could tell that somewhere deep in her mind; there was thought. She had lost most of her ability to speak by this time, but with all her might, she chattered as though her words were as clear as ever. She was enchanted with her new room!

Thinking about the transition to moving her downstairs brings up so much emotion for me. I remember the summer between her sixth and seventh grade, Chelsea wanted to paint her bedroom. I told her that would be our summer project and she could choose the paint and bedding. Her bedroom had 3 large walls, and 2 angled walls and it was her wish to paint each wall a different color. She was always big on color and while this was not what I would have chosen, it was her room and her choice. She chose purple, lime green, orange, yellow and blue all to match her bedding. She loved her room, her stereo, her space, all her posters and pictures of friends. This room had been an accumulation of her childhood to the age of 14 and my heart broke when we cleared everything out to move her downstairs. This was her room that she had spent hundreds of hours playing in, having sleepovers, talking on the phone and growing up in. A room she called her own and loved and a room I knew she would never, ever enter again.

For me, those first few nights with Chelsea downstairs created such an odd discomfort. Knowing I would be upstairs and 40 paces away, I felt so far away from my little girl and remember crying by her bedside late at night as I said my sweet goodnights. “Remember, mommy is just a whisper away” I would tell her to let her know that I would hear her over the monitor if she needed me. While she barely was speaking, she still could utter “mom”…even if it wasn’t clear, I would know she needed me. Still to this day, I have not gotten used to her being downstairs.

Moving On

We had established our “new normal” being downstairs, where it is easy to get to the family room and the backyard, without taking chances on stairs. Having a handicap accessible bathroom and custom shower wheelchair has made life a whole lot easier. I don’t expect much out of each day generally, but to have a greater sense of ease about the simple things we need to do, like taking a shower is, in itself, something to smile about.

As most days are for us, Chelsea generally maintains a happy demeanor with a beautiful smile on her face. I don’t understand how or why she is this way, it is a true blessing that she is not in pain or aware of what has become of her. With all that has been lost: her physical ability, her thought process, her hopes and dreams, her future…we still smile around here every day. It might seem crazy to most, but I do not experience my daughter as sick. She is very different and extremely compromised from who she was, and is changing every day, but she is still my Chelsea. She is still my little girl that held such promise…it is just different now.

Finding laughter and joy on a daily basis with and for Chelsea and the occasional get-together with girlfriends has been my coping mechanism to be able deal with this tragedy. I did not come to this thought process easily. It has taken nearly 7 years of living a parent’s nightmare of watching my child die a little bit every day to come to the realization that Lafora is out of my control. I can let the fear and sadness take over me or I can face it head on and make our life matter, for Chelsea.

I have come to fully know how difficult life can be taking care of a child with a debilitating illness. What is not readily available to the professional “mom” as I have been over these past 25 years is where to turn when your child’s abilities begin to change. Sure, there is the internet should one decide to spend all day browsing for information but what is missing from that process is personal assessment, connection, and guidance from individuals who can help one figure out what equipment might be the best option for moving forward.

Over these past 7 years, we have been guided by amazing people who have led us through the proper channels for Chelsea to receive benefits, therapy and necessary equipment. While Chelsea’s neurologist has always been on the top of our list of most important people to keep an open line of communication, through this doctor, we have been referred to many others for direction and help, which I know is not necessarily the norm for many who suffer with debilitating and progressive issues. We would be completely lost if it were up to me alone to figure out the next step.

It is through enlightenment from others to direct me, that Chelsea receives the very best care possible. Chelsea has a social worker through Regional Center of California who has laid the ground work for nursing care, respite and references to many other agencies. Certified nurse assistants come into our home to assist me with Chelsea and also have become family friends. These amazing caring young women show us such love and devotion and I cannot imagine not having them in our lives.

Chelsea has had an amazing team of occupational and physical therapists who have worked on helping her maintain range of motion and have given me invaluable information on how to protect myself from injury in the day to day rigors of lifting, moving and caring for Chelsea. Amelia, Chelsea’s OT, wanted to be sure Chelsea kept as much physical function as could be, so she connected us to equipment specialists who would further guide us. Through these specialists, Chelsea has been fit for special hand and ankle/foot orthotics so that her ankles, feet and hands do not turn inward. Since she no longer is able to stand and support her own weight and is moving less, it is important to help to maintain her bone density, keep circulation moving well, aid digestion and maintain good blood pressure, so a stander has been added to our list of home equipment. Finally, so that Chelsea could be more easily accommodated for lifting her in the stander, getting her on and off the floor and sparing our backs from carrying her when the wheelchair is not the easiest option, we now have a home Hoyer lift that moves from room to room. These pieces of equipment along with her custom wheelchair shower wheelchair, hospital bed, suction machine and oxygen concentrator help us to give her the best care possible.

Chelsea’s medical team includes a family practitioner, neurologist, gastroenterologist, and most recently, a pulmonologist. Since Chelsea has not eaten food for nearly 5 years now and her swallow has become very passive, lung issues could become a problem and aspiration is always a threat. In order to be one step ahead of Lafora, through our pulmonologist, we now treat Chelsea with albuterol breathing treatments three times a day along with two twenty minute sessions on The Vest-Airway Clearance System. The Vest system creates a very rigorous palpitating percussion treatment for the lungs. Chelsea has a fitted vest she wears that connect to two tubes, much like a vacuum hose, that have bellows that fill with air. Once filled, this vest now connected to a small machine, palpitates at a very fast rate which stimulate her lungs. Through using the albuterol treatments along with The Vest treatment, I can see great improvement in Chelsea’s breathing as well as her ability to cough. Chelsea takes all this in stride; in fact, I think she actually enjoys it. She has figured that when she is doing the vest treatment and makes noises with her mouth the sound will vibrate, which makes her laugh…she always amazes me!

Where we are now

This past year has been one of little realization on Chelsea’s part. While there were plenty of smiles, there were also many days that were very vacant. Many days where she had little movement, no awareness, and no way to get a reaction from her and these days are hardest to bear. On such days for me, as her mom, her support, her caretaker, her everything, life feels dark, gloomy and filled with despair. It is often when I am alone for several days without extra help for Chelsea that I feel at my lowest. It takes all I have, sometimes, just to get myself out of bed knowing the hours will pass slowly, and I will feel unmotivated to handle the day to day chores of the household, and I have no options for change.

As things have changed so much for Chelsea these past couple of years, the fun and anticipation of birthdays and holidays have become something I dread. Chelsea always loved her birthday and would make specific plans for her big day and always loved the excitement of celebrating Hanukkah and Christmas with friends and family. She no longer even recognizes one day to the next, cannot express her joy at receiving a gift and has no ability to even experience the gift itself.

This past October, Chelsea turned 21. I mourned her turning of age and wondered who she would have become, where she would be going to school, what her views and opinions would be…what would life be like for our family if she were healthy?

She surprised me that day…her 21st. I never mention age to her for fear that if she had one nano-second of recall, she would be devastated to know her teen years had passed her by and that she had lost so much. I did tell her, though, it was a very special day just for her, that friends were coming over and we were going to have lots of fun. I repeated many times to her as the day progressed how great it was going to be and I showed her lots of enthusiasm as we got ready. In return she shared lots of smiles and wiggling around which is always an indicator of a good mood and that she was receptive to what was going on around her. She never ceases to amaze me and did rise to the occasion with smiles as dear friends stopped by to celebrate her. I am so appreciative to our wonderful friends who brought cake, goodies, lots of flowers and gifts for Chelsea and will always remember so fondly how those friends actually saved the day for me too. With them sharing their hearts, time and love with us, I didn’t have to face this difficult day alone.

The fall and winter holidays came and went with very little enthusiasm and without any knowing for Chelsea. She mainly had been very quiet and was not able to take in much these few months. We had the opportunity to bring Chelsea to friend’s homes to help celebrate the season but for the most part, life was quiet for our family.

Collin, Chelsea’s brother, came home from Oregon, for 5 days during Christmas week, which was wonderful and we were able to spend special time together. While Collin spent lots of time being with Chelsea, watching TV, reading to her or just trying to let her know he was near, she couldn’t show him much in return except for one very special moment. Christmas Eve I prepared a special meal for us and we devoted the night to just being together as a family. While I was fixing dinner, Collin was lying on the floor with Chelsea, music was playing and there was a comforting peace in the air. Chelsea looked directly at her brother and said, “I love you, Collin”. We all heard it, and Collin sweetly replied, “I love you, too”. This was HUGE to us, as Chelsea had hardly been sharing even single words for quite a long time now. Upon hearing her sweet voice saying those beautiful words, I burst into tears. This clearly identified to me that Chelsea was fully aware of the love around her and wanted us to know it. This was a treasured moment and a little bit of a holiday miracle.

2012

As the New Year begins, I know there will be a medication change to Chelsea’s regime as previously discussed and arranged with her doctor. For the past 7 years, lorazapam has been a staple drug used in conjunction with Chelsea’s anti-seizure meds to help curb anxiety and myoclonus. For the most part, lorazapam had worked quite well for her. However, a new drug, clobazam, the same drug family as lorazapam, had just been released January 3rd by the FDA for use in this country. This drug has shown good results with treating myoclonus and better aids in seizure control as well as promises a longer acting ability in one’s system. Upon recommendation from our neurologist, we decided to begin the 3 week long process of weaning off lorazapam and weaning onto clobazam.

Over the next several weeks, as lorazapam was being decreased and finally eliminated and clobazam was being increased to full strength into Chelsea’s system, this transition has taken her further and further away from us. When previously she maintained a gentle non-stop quiver in her hands and feet, now large jerking myoclonus was prominent often throughout the day. When before she would awaken late morning, and be so, for the remainder of the day into late night, now she naps throughout the day and when awake, rarely wanted to open her eyes. Before her eyes would gaze forward and be alert to enjoy TV or see people’s faces, now her eyes were fixed in an upward, off to the side glare, that had no focus. Before Chelsea could kick and move about in bed or on the floor and now she rarely moved at all. During the month of our experiment with clobazam, Chelsea did not experience grand mal seizures which was a relief, but at what cost? It was breaking my heart to see her disappear and be so lifeless like this. It was apparent, this new medication was not the right fit for Chelsea.

So as we did the beginning of January, we now begin February with reversing the process of weaning off clobazam and back onto lorazapam. It had been a long and draining month and I was not looking forward to what lie ahead. As the next few weeks progressed, life slowly came back to Chelsea and on the third week of this reverse transition, so did her beautiful smile.

As the clobazam left her system entirely, something akin to a little miracle occurred…Chelsea began talking again! One morning, as I do every day, I went into her room and said my good mornings to her. She had a cute grin on her face as I spoke to her and upon lifting her eye mask to see if she was really awake, she said, “hi mommy!” and flung her arms open wide and threw them around my neck. Of course, I burst into tears as she hugged me tight as she used to do…such a wonderful feeling it was. It was the best morning greeting, I’ve had in years!

She continued to become more and more talkative, even saying a couple words at a time, and often repeating what I’d say to her. She delighted her dad when he came home from work one night with a big “hi dad”…this sweet greeting was the talk of the office next day. Very much like a toddler, she says “no” a lot when asked if she’pd like something, but then she’ll surprise us with a nod or a “yes” when asked something else. It has been quite amazing. I know she’s thinking and her mind is a bit clearer. She has able been able to follow simple instructions, almost without hesitation. I would ask her to kick off her blankets or pick up her arms…and she could do it. She also gained strength in her legs and core. She was able to assist when transitioning from her bed to her wheelchair. When put on the floor as we do daily, I’d turn around and sometimes find her sitting up…all proud as she was.

We have laughed a lot these past couple of weeks with Chelsea. She has said some pretty funny things, even though they are just a word or two at a time. I don’t know how to explain what has happened, but it has been a glimpse back at how she was about two years ago. I have missed her spunk, humor and energy immensely and to have a tiny bit back these past few weeks has been a lovely treasured gift.

I don’t know how long this new found energy and cognition will be with us. Life feels easier when she is more present; there is more purpose to our day and more to smile about.

As we venture further and further into unknown territory with this horribly progressive disease, I can’t begin to really know how life will be. I can only speculate the immense pain that lie ahead. Our life is emotionally hard with a heavy feeling of constant loss and sadness. This stress affects every aspect of our being and no matter how I try taking a few hours off to be with friends or do something fun…this feeling never ever leaves; only hides itself for a few moments.

As we continue to support the researchers in their study of Lafora and its affects, we know great strides and progress are presently being made and that HOPE for treatments are not far from a possible reality. We can only hope and pray that it will be soon enough for our children.

Always with HOPE,

Linda