In late June, we flew to California for Susan’s wedding. She is my best friend from college, so even though I was a little worried about the trip, I really wanted to go. It went a lot better than I expected, and I was so happy to be a part of her wedding. Jin wasn’t pleased with the lack of air-conditioning, though! Having Jenni travel with us was a huge help since I travel with so much and can’t carry anything, but we decided not to torture Luke with a weekend trip. Abbie took him to visit her family in Florida instead. He had a blast riding in a boat for the first time and was fearless on the intertube!

Luke started school in August and loved it from the very beginning. It probably helped that our friend Angie is one of his teachers; Luke has always liked her. On his first day, he rushed into the classroom without even a wave! Luke is only going two days a week from 9 until 1. Next year, though, he’ll go four days to get him ready for kindergarten. Where does the time go? He has become such a little man and his comments are so grown up! I’m amazed with how he reasons and justifies. He’s a little too clever for his own good.

In late January, Andy coordinated the second Doughnuts 4 Dough. It was cold enough that I had some trouble driving my chair and loading in the van. It’s tricky when shivers keep jerking the chair left. Thankfully, Scott was generous enough to allow us to invade his restaurant, Sushi Nami, for the event, so we didn’t have to suffer the cold. I actually had a really good time even though it was packed. Socializing in a group is usually frustrating and very tiring. Communication is complicated with more than one person since I really can’t contribute before the topic has been changed. So for the most part I just sit, listen, and feel superfluous. This morning, though, I actually felt like I was contributing to conversations, which was a nice change. I’m not really sure what was different, but it went well. Maybe I should just park next to a chair and let people come to me more often. Luke wasn’t very sociable, but I think he just gets tired of having people he barely knows handle him. It’s hard to blame him. Mom, Dad, and Jenni seemed to be entertained though. The event raised an amazing $22,469.00! Thanks so much to everyone who contributed! The money will be a huge help with buying new equipment that becomes necessary as I get weaker.

One piece of equipment I’m not sure I want is a trache. The Emory doctors have mentioned I’m getting close to needing one, since my respiratory function is hovering around 30%, but I think I’d be absolutely miserable. I’ve been reading up on them quite a bit, and some accounts are fairly positive so maybe it wouldn’t be as horrible as I imagine, but the feeding tube gave me an intense dislike for cutting permanent holes in my body.

One major change I have made is to my wheelchair. Since my arm and shoulder are pretty useless now, it’s been modified to respond to a head array rather than a joystick. I’m still adjusting after about two months, but I haven’t torn up the house too badly. My main difficulty is that it’s hard to turn and move forward at the same time. I rest my head against the back cushion to move forward and touch the right and left pads with my temples to turn, but it’s hard to keep contact with both at once. We tried moving the side pads closer, but then I kept turning unintentionally, which was even worse. As a result, it’s hard to make minute adjustments and I sometimes can’t follow a straight path easily. So I let Jin or Abbie load me in and out of the van to avoid driving off the edge. There is now a joystick for them to use behind my seat, which allows them to drive. The chair is also definitely harder to drive with a head array on concrete. Every dip seems to throw me right or left and, since making small adjustments is difficult, it is pretty frustrating. If I get aggravated, I let someone else drive.

As for my progression, I’m considerably worse. I can still take some steps to transfer, but it’s more difficult and both ankles now collapse when I walk which puts strain on my knees. Since my legs are weaker overall, I’m not able to help as much when I am situated in my chair or bed, and I worry Jin or Abbie will hurt their backs moving me. We don’t need any more complications! More neck muscles have also begun to fail, which means I can’t hold my head up for long and have trouble lifting my head from a resting position. Since I can’t take a good breath, it’s now hard to say even one word at a time. I rarely attempt to say anything to anyone besides Jin, Abbie, or Luke and even they can’t understand me most of the time. If I want to say something, I’ll usually request my computer. I’ve completely given up eating or drinking by mouth; it’s simply not worth the mess and choking spellsI It’s also hard for me to stay comfortable in my wheelchair for long; my neck, back, and knees all begin to ache after a few hours.

I’ve been spending a lot more time in bed lately since I’m most comfortable there, and over the last few months, Luke has taken to playing Lego star wars next to me after his nap. He’ll push the blanket and sheet down to the footboard to clear a space then place all his Lego bins and vehicles on the bed. The piled up blanket becomes a castle and most battles are fought on my tray table. It’s really sweet how he’ll include me in the play. Luke will pick out a character for me then ask questions as the battle unfolds: Are you Boba Fett? Did you just shoot me? Are you about to fall? I’ll answer yes or no and he’ll play my character for me. We’ll also watch Lego stop-motion movies on You Tube while he plays, but half the time it’s just background noise. Either way, I’m happy Luke has found a way to play with me!

Luke saw an older picture of me in the pool and said in a shocked voice to Abbie, “Mommy knew how to swim?” When Abbie explained that I still know how but simply can’t anymore, they started talking about other things I used to be able to do. Abbie said Luke’s surprise kept growing: “Mommy used to hold me? Mommy used to change my diaper? Mommy used to feed me a bottle? Mommy used to be able to run?” I’m very glad I wasn’t around for this conversation since I fought tears when Abbie told me. I’m happy Luke knows I used to do those things for him and was able to run and swim, but it’s heartbreaking to know that’s like a fairy tale to him. So amazing as to be almost unbelievable. He’s very optimistic, though, and will tell me I can do this or that when I “learn how to walk again.”

We all went to Disney in mid May and had a great time the first three days. A huge thanks to Susan and her friends at Pixar for the free park tickets! That was a huge cost savings. Since Michele and Ryan live closer to Orlando, they arrived first and were able to check in and coordinate the delivery of my hospital bed and tray table for my computer. They also found that Cinamin had upgraded our group to an amazing three bedroom suite with an incredible view of the animals. We saw giraffes, zebras, and water buffalo wandering right off our porch. Very cool. The suite also had a table full of crazy nice gifts. We all got personalized Mickey caps, there was an Animal Kingdom throw, a Mickey blanket, Mickey gloves, a Mickey plush doll, animal toys, star wars potato heads, stickers, a coloring book, picture frames, and so many other little fun gifts for Luke. It was overwhelming.

We spent the majority of our time in the Magic Kingdom and Hollywood Studios, since that park had Star Wars. The guest assistance pass let us skip all the lines, which was especially helpful with an almost four year old. When he complained about short delays, Michele threatened to take him through the standard line. My spoiled little boy had no idea how good he had it. I decided not to even attempt navigating my chair on my own through the parks. Uneven streets, tight turns, and oblivious crowds made it virtually impossible. I was actually able to go on the majority of the rides, though. Some would accommodate my power chair but there were others that required me to move to a manual chair. Since Disney’s chairs were made of worn out canvas with no head support, we decided to bring both wheelchairs after the first day so I could transfer into my own manual chair when needed. That worked so much better! Plus, Luke could ride along in the extra chair when he got tired. He looked like a miniature ruler on his throne. There were also a few rides I wanted to go on where Jin had to actually transfer me. We had a few dicey moments and Jin’s back probably suffered, but we made it on without ever falling.

Luke had a great time on most rides and loved the parade, fireworks, and shows, but I think his favorite was brunch at Café Mickey. He was so excited that he couldn’t stay still; he was clapping his hands, shimmying his shoulders, and bouncing in his seat. It was really adorable! Luke also kept looking around in anticipation waiting for the Disney characters to come by. He met Goofy, Mickey, Donald, Pluto, then Minnie and got their autographs. He was thrilled to meet them and posed so cute smiling on cue; we didn’t have to coerce him at all. In between character visits, Luke ate his weight in food. I haven’t seen him eat with that much enthusiasm for a while. Luke’s only compliant was that Mickey didn’t eat with us. From what we told him, he assumed Mickey would actually join us at our table.

Unfortunately, the last half of the week was spent in and out of urgent care centers. Luke had a super high temperature, complained of hurting all over, and couldn’t keep anything down. We managed some fun things for distraction like a trip to the Lego store when he seemed to feel a little better, but for the most part, he was miserable. Since the doctors around Disney had no clue what was wrong with him, we went straight to Children’s when we got back to Atlanta. By this time, Luke had a strong dislike of hospitals and needles and wasn’t afraid to let everyone know it. Luke was admitted, countless tests were run, and they finally determined he had Kawasaki’s disease. This disease can have serious consequences for the heart, but since it was found and treated quickly, Luke shouldn’t have any complications. Unfortunately, he was in the hospital for a total of five days to receive the treatment and Luke pleaded to go home for the majority of his stay when he wasn’t begging the nurses not to hurt him. Jin was pretty worn out from trying to comfort Luke. Thanks to everyone who came by with distractions! Luke is almost 100% now.

More recently, we went to Shepherd’s for an evaluation with their assistive devices group, which was facilitated by Dr. Lin who Jin met during a closing. After talking with us a while, they showed us three things that will be a big help. The first should solve our most immediate need, which is for some kind of alarm I can trigger when I need help. The X10 Palm Pad Remote is a device that they’ve modified to accept a switch and will set off remote alarms when pressed. This same device can be programmed to give me some control over my environment by allowing me to turn things on and off: the TV, the lights, the fan, etc. Very neat. The second is an impulse switch that can replace the switch I currently use to do a left mouse click when working on the computer. I press the current switch with my toe and it can be uncomfortable to stay in the same position for an extended amount of time. The muscle is also fatiguing more quickly. The impulse switch can be placed anywhere and takes a lot less effort to engage. The third device they showed us will be great for communication and will replace my current computer when I can’t use SmartNav anymore. The Tobii is an eye-controlled device that is integrated with a fully functional computer. Calibration was incredibly easy and I had no trouble using it to make selections. Once I learn where all the options are, I think it will be super fast. It’s crazy expensive, though, so hopefully insurance will cover some of the cost.

Here are some links if you want to read more about the devices:
· X10 Palm Pad Remote
· Impulse Switch
· Tobii

For those who are curious, my ABCs children book is finished, but we’re having trouble with production quality. Brian has made heroic efforts to resolve the issues, so we’ll see what happens! As for everything else, I found a description of living with ALS that so very nearly mirrors what we’re going through that I might have written it if the more specific details were swapped out. If you’re interested, you can read The Attic.

I guess a year’s worth of details can add up. I really did try to condense! Thanks to all our friends and family who do so much. I don’t know what we’d do without you!

Soon after I started feeling better, we decided to get involved with a study being conducted out of Georgia Tech’s brain lab.   We had driven down to meet with the professor overseeing the lab in December, who is actually one of my old CS professors, Melody Moore. They are trying to measure brain activity in the language portion of the brain. One project is capturing an on/off signal, which could have applications for a yes/no switch. The other project captures the range of activity so it could be used as a selector of sorts. They were both pretty cool, but Jin had mentioned a machine that would display text from thinking about sign language, so my expectations were very high and I was a little disappointed. Still it was fun to try out, and we told them we’d be interested in participating in the case study they were coordinating.

They decided I would be a good candidate, so Melody came over in mid-January with the Hitachi developer from Japan to set up the equipment for the case study. I’m one of 30 ALS patients who are recording data, but I’m still not sure how long the study will last. The initial session actually went a lot more smoothly than I expected. The majority of time was spent on calibration. We went through a series of measurements where I was either supposed to try to relax my mind to get a ‘no’ or activate my mind to get a ‘yes’. Once the different results were plotted, they tried to draw a line between the ‘yes’ results and the ‘no’ results. From then on, the location of my subsequent results determined the system’s yes/no response. I did really well with ‘no’, but had a harder time with ‘yes’. After they tweaked the shape of the line and adjusted the sensor on my forehead, though, we got a few correct responses. They left the system for me to work with, and I had every intention of being a model participant, but I was still tired from earlier and had a relapse of fevers and extreme fatigue, so we never got in the habit of collecting data.  I’m embarrassed by how little we have used the system and we have given it up as a lost cause at this point.  I feel pretty horrible about the whole thing.

In mid-February we went to the ALS clinic.  I was a little worried since I felt as though my breathing had gotten considerably worse.  It had become noticeably harder to force a deep breath, my breathing felt shallower in general, I had frequent headaches, and I felt tired through most of the day.  I hoped they wouldn’t recommend I wear my BiPAP during the day as well as at night.  I know that’s a likely option in the future, but I already feel partly removed from my life.  Adding a mask that will further restrict movement and interaction with Luke will make that feeling even stronger.  Surprisingly, the respiratory techs at clinic reported my breathing hadn’t deteriorated as I had expected.  I scored 53% which is actually the same as last time!  I was very surprised.  They did want me to wear my BiPAP more during the day to see if that would help with the headaches I reported, but otherwise didn’t have any other instructions.  I’m actually being good and have started wearing it when Luke naps. Regardless of my respiratory score, though, I believe my breathing has suffered over the past few months.  I’ve always been pretty aware of changes to my body, and I know it is harder to breathe.  Still it was nice not to get confirmation.

For the most part, there haven’t been many noteworthy events or any purchases of new medical equipment.  Jin did buy me a SlingBox, though, which I absolutely love.  It lets me control the TV with my computer instead of the remote; the buttons on the remote finally became too troublesome.   As for my condition, I don’t feel like there’s anything new to report, but the things I’ve been having problems with continue to progress.  For instance, it has become even more difficult to talk since I don’t have enough air to force out more than a few words at a time.  It makes me so sad not to even be able to talk with Luke easily.  He can hold actual conversations and is curious about everything he sees and hears, but I can’t really answer his questions without considerable effort on both our parts.  It’s heartbreaking when he simply can’t understand, but his patience is incredibly sweet.  Everyone else is having a harder time understanding me as well, and I’m trying to resign myself to using my laptop all the time to communicate.  I haven’t given in yet, though, because the setup makes me feel so isolated from everyone, especially Luke.  Regardless, I know I’ll have to give in soon.

I’ve also gotten to the point where I eat very little by mouth.  I’ll sometimes take a few bites at dinner for a taste and eat something here and there, but for the most part eating doesn’t seem worth the effort.  Moving food around to chew takes so much concentration and energy that half the time I don’t even appreciate the flavor.  I simply want to swallow so I can stop working so hard.  It doesn’t help to know I must be putting on quite a show for everyone else at the table.  I’m sure they don’t care, but I do.  It’s impossible for me to be comfortable with how I look while eating.  Fortunately, I truly don’t care about not eating even when I know I’d enjoy the food offered.  I’m a little shocked at myself, since I absolutely loved to eat.  I thought I would be miserable over not eating, but I’m not.  Even if I do get a craving, when I think about how much of a hassle it will be, the craving disappears rather quickly.

One symptom that I don’t remember mentioning before is something they call emotional lability.  Basically, my emotions can be a little uncontrollable and will be triggered by the smallest thing.  The crying especially has gotten a bit out of control.  When Jin read Luke “The Mountain that Loved a Bird,” I was fighting tears from the second page.  Jin caught me tearing up at the end, which of course meant I immediately lost the fight.  Luke must have been confused, since I was crying but laughing as well because I knew there was absolutely no reason to cry.  I can keep tears from actually falling the majority of the time, but I still tear up with annoying frequency.  If a commercial or show is even a little bit touching, I end up teary eyed.  And I’m fighting tears during every church service.  Other ALS patients take medications to help, but I have always been nervous about meds designed to level emotions.  I would much rather handle this without adding yet another pill to my day.  Especially since the medications must affect the entire range of emotions; I can’t see how a drug could only regulate the extremes.  I worry I’d end up feeling very little emotion at all which would be much worse than what I’m dealing with now.

Other than that, everything just seems more difficult.  My arms and shoulders especially seem significantly weaker.  Even placing my hand over my wheelchair controller or using the switch takes enormous concentration.  My neck and back ache almost constantly, it takes a ridiculous amount of effort to hold my head up while taking a drink, and I’m never quite as comfortable as I would like to be.  And I hate being weak.  I try not to dwell on it too much, though, because I know it will only keep getting worse.  It’s frightening to realize that I am currently as strong and functional as I will probably ever be.  In a few months, I may look back on today and count myself fortunate to have had so much of myself left.  I can’t explain how much I envy people in wheelchairs who are in a static state.  I think it would be wonderful to know that your current limitations are what you have to work with and work around for the foreseeable future.  Once they have their new situation handled, they can move forward. It’s maddening that after I learn to adapt, everything changes again.  There’s no time to relax and take a relieved breath.  Instead I sit waiting for the next loss and the new corresponding frustration.

Through all the frustrations, though, Luke is so much fun.  He talks in complete sentences, and hearing his thoughts is highly entertaining.  I found out that Luke’s confident he could use a plane to touch the stars if we’d buy him one that wouldn’t crash all the time.  Apparently his toy planes don’t cut it.  He’s also doing fantastic using the potty, but we haven’t tackled bedtime yet.  He’s definitely up to preschool standards, though, which is fortunate since he’ll be going to Alpharetta Christian Academy starting this August.  Luke will only go twice a week, but I’m fairly sure I’ll be sobbing on his first day.  If I weren’t certain he’d love it, there would be no way I could give up my time with him.   Luke is such a sweet little boy and loves to be helpful.  He presses the elevator buttons for me, fixes my wheelchair feet, opens doors, and does anything else he thinks he can handle.  He can definitely still be a trouble maker, but we got pretty lucky.  I’m going to miss him when we head to California for a wedding at the end of the month.  We’re not staying long and I know he’d be miserable, so Abbie is taking him to see her parents in Florida while we’re gone.  Somehow I think the beach, a boat, and fishing will make him much happier.  He probably won’t even notice we’re missing!  My little sister, Jenni, is traveling with us to help with equipment and other logistics.  She’s actually staying with us for a few months and has been a huge help taking over some of my care.  Luke has no shortage of playmates!  Even with Jenni’s help on the trip, though, I’m sure it will be interesting.  Wish us luck.

Love,

Kim

At this point, I spend the majority of my day in the power chair except for a few hours in the afternoon when I rest in bed and work on my laptop while Luke is napping.  Since my neck cramps after a few minutes at the office computer, I now almost exclusively use my laptop while propped up in bed.  The setup is pretty comfortable!  I definitely think the over-the-bed table Jin fought to buy has been a very good investment.  Since I’m getting to the point where I should start using my laptop to communicate, through, we’re going to order a laptop mount for my power chair.  We’ll see how that addition changes my routine.  I’m also trying out a new system called SmartNav to control the cursor on the computer with head movement.  There are still a few issues to work through, but I’m using it to type right now.  If I could just get dwell clicking to work, I’d be thrilled with the system!  I can still use a traditional mouse, but it is getting more difficult so I thought I’d be a little proactive.

Once we had the power chair, Jin ramped up his search for a van.  It was a very frustrating process.  One company told outright lies about available features to try and sell existing inventory.  Jin narrowed it down to two companies he trusted: R&R Mobility (http://www.rrvan.com/) and A+ Mobility (http://www.apmedical.com).   A woman from R&R drove a wheelchair accessible minivan to our house in mid July so we could try it out.  We decided to get that type of van, but since they weren’t able to get a van with the features we wanted in a reasonable timeframe, we purchased the Toyota Sienna from A+.  The only downside was that A+ is located in Augusta and we had to take my power chair along when we picked it up so they could install the hardware to secure the wheelchair base to the van floor.   We drove down on a Friday morning in early August, waking up at 4:00 to be in Augusta when A+ Mobility opened at 8:00.  Mikey was nice enough to drive us down with the power chair loaded in the back of his Sequoia, and we were actually a little early.  When they opened, we were told the part they needed to install in the van to secure the chair had not arrived but should be there soon.  By 11:00, the part still hadn’t been located.  Jin was aggravated, but he has unfortunately come to expect that dealing with something for me will always be a huge hassle.  The van wasn’t ready until after 1:30 and, even then, we had to leave my chair behind.  They installed the latch in the van successfully, but the hook that needed to be attached to the bottom of the chair wasn’t installed, since they didn’t order the right part.  We drove the van home hoping they would install the hook onto my chair correctly and deliver it to our home in working order.  Thankfully, my chair was safely delivered that following Monday and secured in the van perfectly.

Having the van has been wonderful.  It is a little strange sitting up so high, but I’ve gotten used to it.  Also since the middle seat was removed to allow the wheelchair to roll in and out, there’s a lot of room between the front seat and Luke.  I worried he wouldn’t like being so far away and that the distance would make caring for him in the car difficult, but the exact opposite is true.  Luke loves running around in all the open space, using the ramp, and pushing the button to shut the door.  And all that space means Abbie can stand in front of Luke to buckle him in then slide into the driver’s seat without leaving the van.  It also makes changing a diaper less challenging, or so I’ve been told. I think we’re all very happy with how well it has worked out.  Having the van makes getting out of the house and running errands so much easier.  Abbie never complained and was always willing to push or pull me along in the manual chair, but I didn’t like making every single trip so difficult.  Now I can even be a little helpful; the handles on the back of my power chair are great for carrying bags!  And it is wonderful being able to power myself around the store.  Luke was a little confused at first when we wouldn’t let him hold on to the chair while walking around a store, since he had become accustomed to that rule, but it wouldn’t be safe with the power chair.  I would like to avoid running over my son, especially with a chair that weighs over 300 pounds.

One concern I’d had about a ramp van was whether parking would become a little tricky; there seem to be a limited number of van accessible handicap parking spaces and sometimes the room allotted for the ramp is a bit small.  One day when we got back to the van, we saw that the car parked next to our ramp door was parked way to close for me to even attempt to get in.  Since I was expecting that to happen sooner or later, I wasn’t terribly surprised or really even angry.  I’m sure I used to encroach on handicap parking without thinking about whether I would be in the way.   Abbie loaded Luke into his seat while I rolled towards the back of the parking lot to find enough space to load.  I felt a little silly sitting in my chair surrounded by empty parking spaces waiting for Abbie to drive the van over, but the process wasn’t too much of a hassle.  At least now I know there’s a simple solution.

The only drawback of the van was the price; a handicap accessible minivan is over double the cost of a standard model.  Andy Petzinger generously offered to coordinate a fundraiser in mid October to help offset some of the cost, and it was an amazing success.  We have such incredible friends!  I was fighting tears when I saw how many of you had come to support us.  I wish I could have talked more to everyone, but I’m hard enough to understand in a quiet room.  I have almost no chance in a crowd.  It’s hard for me because I have questions I want to ask and comments to make, but I know that an attempt will lead to frustration in a loud environment.  Please know that I want to talk to you!  In fact, I would love it if you’d volunteer answers to the standard questions: how’ve you been, what have you been up to, how ’s the family, etc.  That would be fantastic!  I had fun watching the donut eating contest, though.  And Luke had quite an introduction to Krispy Kreme.  Andy was able to raise over $8,000 with the fundraiser!  Thanks so much for the donations and to everyone who participated in this entertaining event.

At the end of August, we all went to Hawaii for a week.  Luke was amazingly good on the flight down especially considering that we woke him up at 6am and he only slept an hour on the 9-hour flight.  For the most part, Luke was perfectly content playing with the two new cars Jin had given him when we boarded the plane.  The flight wasn’t nearly as difficult as I had thought it would be.  I had to move carefully to my seat and using the bathroom was tricky with Jin and I crammed into that tight space, but I expected worse.  I just wish I’d been able to sleep more!  I held Luke on my lap as we were landing so heard him whispering ‘whoa’ as he watched the scenery zip by.  I think everyone in our section heard him shout it when the wheels touched down!  Luke was thrilled when we took a shuttle bus to pick up the rental car.  Once we had picked it up and were on our way to the hotel, Luke kept saying ‘I wanna ride school bus’ every time he saw a bus.  That continued for the rest of the week.

We had a great trip.  We went to a beautiful wedding, were able to visit with friends and family, and simply relaxed.   Jin and Abbie even went surfing!  Michele took an unbelievable amount of pictures, which gave Luke plenty of opportunities to practice his new smile-for-the-camera phrase: ‘cheese, crackers, and turkeeeey’.  He decided his favorite lunch sounded better than the standard and it has stuck.  I definitely missed my power chair, though, and desperately wanted my shower.  There were a few dicey moments when I was sure we were going down.  I also got pretty tired toward the middle of the trip, since I wasn’t able to use my BiPAP.  We had an entire carry-on dedicated to this piece of equipment, and I wasn’t able to use it because we had forgotten to pack the power cord!  Jin was slightly irritated.  Overall, though, it was a wonderful experience.

As for me, there have been a few changes.  For one, I stopped seeing my chiropractor in late June.  I’m sure the stretching was beneficial, but since I now spend the majority of my day rolling rather than walking, I didn’t think it made a noticeable difference in how I felt.   Plus, it had become more difficult to get to the appointment and I wanted to reclaim my mornings.  Having appointments three out of five mornings made planning anything else pretty challenging.  I also now only see my massage therapist once a week, so we have a lot more free time.

Jin and I went to the ALS clinic on October 10 and got some good news: I’ve put on an impressive amount of weight!  Unfortunately, I’m fairly certain it has all gone to my belly.  Oh well.  My respiratory numbers dropped a little to 53%, but since I had feared it would be worse, I was actually relieved by the results.

Now for the rundown on my current status:

• Continuing to use the BiPAP consistently at night.
• Using the cough assist at least once a day even though I’d prefer to throw it out the window.
• Taking Rilutek, Quinine, Lithium, and Arimoclomol prescriptions.
• Muscles in lower legs and feet spasm more frequently.
• Legs shake more frequently when they bear weight and are more difficult to control.
• Hips collapse more often when they first bear weight.
• Can walk unassisted for short distances if very careful.
• No longer attempt stairs.
• Talking is noticeably more difficult.
• Mouth fatigues quickly when eating.
• Meal sizes are very small; supplement with Ensure quite a bit using the feeding tube.
• More difficult, and sometimes impossible, to sit up without assistance.
• Arms, hands, and legs sensitive to pressure.
• Moving and lifting arms takes considerable more work.
• Shoulders very tight with poor range of motion.  Cause considerable pain if over-extended.
• Neck muscles have more trouble holding head up when leaning forward or back.
• Lower back aches from constant sitting.

I know that all sounds a little dire, but we have a pretty good system in place.  Abbie has been fantastic and my only worry is that we’ll ask her to take on too much.  I hope she isn’t regreting her decision to move in!  As for Luke, he’s definitely asserting his independence!  A few of his favorite phrases are ‘let me do it’ and ‘don’t help me’.  He is talking so well!  You would probably have better luck understanding Luke than his momma!  He’s also beginning to help me with doors, will give me bites of food if he doesn’t see someone else helping, and will try to put my shoes on.  It’s adorable how he’ll answer ‘ok, sure, sure, I’ll do that!’ when we ask him to do something like let the dogs in.  Luke’s such a good helper and an incredibly caring two year old.  He’s also all boy: daredevil, explorer, and pro wrestler wrapped in a hard-to-resist package.

Here’s a very short list of what Luke has been up to:

• Answers ‘daddy go shopping’ when asked what daddy does at work.
• Complains about being ‘itchy’ when lying in my lap and resting against my unshaven leg then states ‘daddy fix it’.
• Cries ‘I want medicine’ when his raw bum is being cleaned off.
• Says  ‘delicious’ when eating something he likes.
• Has become very curious about sounds and will say ‘I hear something!’ or ask ‘What’s that noise?’
• Says ‘I need tissue’ when his hands or face are dirty.
• Will play with cars for hours and recreates scenes from the Cars movie complete with dialog.
• Likes to help read along with books he has partially memorized.
• Repeats everything we say and, unfortunately, everything daddy says as well.

It’s incredible to watch Luke become a little boy and so much fun to know what he’s thinking about.  Even though it’s impossible to keep myself from wishing I could take a more active role, I do love the show.  Well, I think that covers most of it!  We’re looking forward to seeing everyone at the walk tomorrow.  It should be a little chilly but not too terrible, and I know all the other teams will have t-shirt envy. As always, you guys have shown amazing support!  Thank you!

Love,

Kim

I was surprised but relieved when the mic-key procedure was scheduled for June 20, even though only six weeks had passed since the PEG operation. We did really well again getting to Emory on time that morning. Traffic was light and we made it to the waiting room by 9:00 for my 10:00 appointment. One of Jin’s close friends had been an intern with Emory this past summer working in administration, so she met us there to talk with Jin while he waited. There was some confusion when the nurse first came to get me, since they had me scheduled for another PEG tube rather than a mic-key button replacement. When she mentioned that I would be staying for 23 hours, I started to get concerned and told her we were told it would be a two-hour outpatient procedure. We figured out the misunderstanding pretty quickly, though, and she rolled me back to the same ward I’d been in a little over a month before. Since they weren’t going to admit me, I didn’t need an IV. I’d recognized the nurse as the same one who blew out a vein during my last visit, so I was pretty relieved. She also hadn’t been very receptive to Jin when he warned her about my troublesome rolling veins and suggested she use the smallest needle. My relief didn’t last long. When the nurse was helping me out of my shirt, she accidentally grabbed hold of the feeding tube as well and gave it a good tug. Ouch! I’m sure she’s a nice person, but she needs to learn to slow down and pay better attention.

I was in a similar procedure room as last time by 9:45, but we were a little delayed since the nurses had not been informed it was a mic-key procedure, which meant they didn’t have the right special-order supplies. I could tell they were irritated, and I was starting to wonder if we’d have to abort. Luckily, the doctor had one in his office. Once they found the mic-key, we moved to the radiology lab so they would be able to verify successful placement of the new tube into the stomach. One of the nurses told me the room had opened for use on Monday, so we had good timing at least. The procedure was very quick and virtually painless; the worst part was the multiple needle pricks around the feeding tube to numb the area. After that, I felt a tug and heard a pop when they pulled out the old tube, felt some pressure as they threaded the mic-key tube through the empty pathway, and then they were done. It probably took less than five minutes. Once the new tube was in place, the doctor poured a solution through to watch its progress and made sure the liquid fed into my stomach. The video was pretty crazy, but everything checked out. Even though I was feeling a little dizzy afterwards, I only wanted to rest for a short time and was rolling out of the ward by 11:00. While I was resting, my new nurse went to give Jin the mic-key kit we would bring home to use for feedings. Jin seemed aggravated but unsurprised that the nurse was unable to give instructions on how to use and care for the device. She did try to be helpful, but gave Jin completely incorrect instructions on how to use the tubes. When questioned, she simply kept repeating what she had just read from the instruction manual that came with our kit. It didn’t inspire confidence. Jin’s friend requested that the nurse return to the operating surgeon and obtain care and use instructions for the mic-key and the associated tube kit. The nurse returned, but still could not answer the questions that Jin and his friend asked. She eventually gave up the pretense and told Jin to call the surgeon with his questions. It seemed odd to Jin that the name of the surgeon was not the operating surgeon who had done my initial feeding tube or the mic-key replacement (to try to keep it from getting confusing, I’ll refer to them as the Operating Surgeon and the Supervising Surgeon from now on) but to the Supervising Surgeon that Jin had initially spoken to prior to the first PEG operation. Regardless, Jin got through the first attempt to use the mic-key at home without mishap. It seemed simple enough, and I was very happy to be rid of that foot-long tube!

As Jin read through the mic-key manual that night, he discovered that the water in the balloon holding the mic-key in place in my stomach was supposed to be checked weekly. Since we needed to verify and replace the amount of water in the balloon, Jin called the number for the Supervising Surgeon the next day and left a voice mail asking how much water was appropriate. He also inquired on how to obtain a new mic-key as we read that the mic-key generally needs replacement every three to eight months. We were disappointed and surprised that we had not been given that information prior to the procedure. It wouldn’t have made a difference, but it’s something we should have been told.

Immediately after getting the mic-key, I asked the Operating Surgeon when I would be able to go swimming. I hadn’t been allowed in the pool all summer, and I was impatient to join Luke and Abbie. The Operating Surgeon answered two days, so I thought I was being responsible by waiting five before getting in the pool. That was a Wednesday. By Friday, the mic-key site had some granulation (swollen blister-like tissue) and had started to bleed and leak fluid. I wasn’t overly concerned because there wasn’t much associated pain, but Jin was very worried. He made a few calls over the weekend and again on Monday to the phone number given to him by the nurse for the Supervising Surgeon, but they were never returned.

That next week, Jin happened to be at Kimberly-Clark (the mic-key manufacturer) meeting with a client and they discussed my situation. The client couldn’t answer Jin’s questions, but he tracked down an executive VP of the medical product group (who also happened to be a surgeon). After giving her the details, she told Jin I needed to see someone about wound care immediately. Besides the immediate wound care concern raised by Kimberly-Clark, she was also concerned that the mic-key was incorrectly fitted based on Jin’s descriptions. The mic-key had been causing deep indentations on my skin instead of lying flush. This was most likely the cause of the leakage and was probably keeping the wound site from healing properly. Jin was immediately contacted by a representative of Kimberly-Clark who offered to assist him with anything related to the mic-key. The representative is also a nurse and became invaluable to Jin; she was extraordinarily helpful.

Jin made numerous efforts to schedule an appointment at Emory for someone to look at the mic-key. They simply replied that everything I was experiencing was routine. Jin continued the battle to get my mic-key issues taken seriously by the doctors at Emory, while simultaneously attempting to enlist other non-Emory gastrointestinal surgeons to treat my wound site. Every other GI doctor that Jin contacted did not want to touch me until my Operating Surgeon had ruled out infection or other issues. Through Jin’s conversations with other GI surgeons and his continued contact with Kimberly-Clark, we learned a couple interesting facts while attempting to get my wound care resolved: the mic-key was originally designed for use in pediatric patients and the mic-key procedure may have been performed too early. The first explained why all the GI pediatric surgeons could answer Jin’s questions about the mic-key while the adult GI surgeons could not. After a multitude of calls and e-mails and Jin’s friend getting her hand slapped by the Emory administration for trying to help, we were finally given an appointment to see the Supervising Surgeon the morning of July 3. Jin had also managed to get the Operating Surgeon’s number and he eventually called Jin back the evening before the appointment. He reiterated that he believed everything was normal but said he would meet us at the Supervising Surgeon’s office the next morning.

It was the strangest doctor appointment I’ve ever had. We didn’t sign in or show my insurance cards, no one asked about my history or what medications I was taking, we were the only people there since the department wasn’t usually open that early, the nurse did not have my chart, and the room we were taken to wasn’t prepped. When the Supervising Surgeon came in and introduced himself (as Jin and I had never actually met him), he acted as though he was unaware of our issues even though it had been clear he’d been actively involved in the e-mail battle leading to the appointment. The Operating Surgeon was not present and we have never heard from him again. After looking at the mic-key site, he said the fit of the 2.5 looked perfect but he would be more than happy to swap it for a 3.0 if that’s what we wanted. He continued by saying we could put the 2.5 back in if the 3.0 ended up being too long. As I looked at him in disbelief, he started explaining my options again thinking I hadn’t understood. I understood just fine, I was simply shocked by his proposed method for finding the appropriate size and his complete disregard for any pain associated with repeatedly swapping tubes. When he continued on to say the procedure was only mildly uncomfortable, I really wanted to ask him how he could possibly know without experiencing it himself.

Jin tried to explain that we simply wanted the correct size, whether a 2.5, 2.7, 3.0, or some other length. Jin further tried to explain that based on his understanding, my complications would continue if the mic-key was fit incorrectly. When the Supervising Surgeon kept responding by saying he would do whatever we wanted, we got the feeling he just wanted to resolve the situation as quickly as possible so Jin would stop making noise with Emory. Since the Supervising Surgeon couldn’t locate a 3.0cm mic-key, we didn’t waste any more energy attempting to explain our expectations. The Supervising Surgeon then examined the wound, and while I thought he was simply continuing to clean the area, began to cauterize the site without any warning. When the pain kept escalating, I complained and his response was “Oh yeah, I’m cauterizing the site. It will probably hurt. I guess I should have told you.” Of course you should have told me! I understand that he is a surgeon, but his bedside manner needs an enormous amount of work. Once the site was examined and cleaned, he conceded that the mic-key might not be the best fit for me, but that if the next size up didn’t fit, we should go back to the original mic-key. The Supervising Surgeon then agreed to scour the other Emory campuses for a 3.0cm mic-key and said we could return the following week to try it out. In the end, the only good things that came from the appointment were learning the site wasn’t infected and getting a free set of spare feeding tube attachments. Otherwise, it was a disappointment.

On the drive home, Jin called his contact at Kimberly-Clark and confirmed our suspicion that the Supervising Surgeon’s proposed method for finding the appropriate size was not the standard product recommendation. We were told there was a measuring device to determine the appropriate tube length. I was pretty irritated. How can a doctor perform a procedure in good conscience without the proper tools? During the procedure, the Operating Surgeon was asked if the mic-key they found and installed was the correct size. His response to the surgical tech was basically “yes, she is thin, it will be fine.” At no time did he ever use a measuring diagnostic tool. Then for the Supervising Surgeon to suggest trial and error was mind-boggling.

Over the next few weeks, I continued to have problems with the mic-key site: it refused to heal and bandages showed abnormal discoloration and lots of blood. Jin was concerned enough for both of us, so I wasn’t as worried as I might have been. Through his continued dialogue with Kimberly-Clark, he learned more about the measuring diagnostic tool and measurement procedure. At his request, Kimberly-Clark delivered two diagnostic tools to the Supervising Surgeon to use during our next appointment. We waited until mid July for the appointment because Emory only had 2.5cm mic-keys which meant a 3.0cm had to be ordered. After numerous consultations with Kimberly-Clark, Jin also had a 2.7cm delivered to the Supervising Surgeon in case the 3.0cm was too long.

This appointment was just as strange as the first. The department wasn’t open, no insurance cards requested, no medical/prescription history taken, but this time the room at least had fresh paper over the exam table. His bedside manner had not improved. He removed the mic-key then cleaned and cauterized the wound again as I struggled to lie still. Then he inserted the newly delivered measuring device while I was lying down. Jin had been briefed by Kimberly-Clark: the procedure for taking measurements was to take two measurements, one lying down and one sitting up. The average would be the proper size. The measurement lying down to our surprise was 3.25cm! We couldn’t believe that I had been sized with a 2.5cm initially and that we had push so hard to be re-sized. Jin then had to heavily suggest (while simultaneously pointing to the paragraph in the product manual that came with the measuring device) that he take a second measurement with me sitting up. They sat me up and then took the second measurement. The Supervising Surgeon then asked Jin what he thought the measurement read! I understand that my husband can sometimes come across as difficult, especially when he is frustrated, but the patronizing question was completely unnecessary. Jin had actually tried to be respectfully persuasive instead of abrasive as I’m sure he would have preferred. Jin answered 3.5cm, but the Supervising Surgeon ended up installing the 3.0cm mic-key since it was the largest they had. My wound site had been tender for several days and he seemed to have little regard throughout the procedure for any pain he caused. The entire appointment seemed to irritate him. I was pretty angry with myself for crying; I felt as though I had lost to him. That doesn’t make much sense, but it’s how I felt. The Supervising Surgeon said that based on his experience, the 3.0cm mic-key looked perfect. When Jin pointed to the product manual and explained that the average of 3.25 and 3.5 was not 3.0 cm, the doctor agreed to order the next size up. He then told the nurse to make a note that maybe the 2.5cm mic-keys that Emory had in inventory should be traded for larger sizes and agreed to call Jin when the larger units arrived.

Unfortunately, we didn’t see any improvement and decided it was time to find another doctor. Now that we had ruled infection out, it should be easy to find another doctor for a second opinion on fitment instead of emergency wound care. Jin received a recommendation for one of the largest GI practices not affiliated with Emory. On July 24, Jin took the morning off to drive me to an appointment with one of their specialists who, according to his staff, was familiar with the mic-key. At the appointment, Jin explained the situation and the doctor examined the wound site. He then apologized and informed us he’d never seen a Kimberly-Clark mic-key and could not advise us on fitment. I’m glad he was candid, still it was incredibly disappointing and a complete waste of time. At least we weren’t charged for the visit.

For another frustrating week, Jin continued to call different GI practices in Atlanta and even resorted to calling the Medical College of Georgia to speak with GI nurses there. This time he wanted verification that the GI doctor had actually installed or was at least familiar with fitment of a mic-key. Then on July 30, Jin got really lucky. He was given the number to a pediatric nurse who ordered a large quantity of mic-keys for Children’s Hospital. He left a voice message and surprisingly received two separate return calls. Since the nurse he had called was on vacation, the other nurses in the practice were returning her calls. The first nurse Jin spoke with agreed to try to enlist one of her doctors to see me and scheduled an appointment for me the next day at a satellite location in Rome. Later, she called back to cancel because that location did not have the necessary equipment for fitment. Then Jin received a call from another nurse, Cindy Witkowski. Cindy immediately scheduled an appointment for me with a pediatric GI doctor that same afternoon. It seems as though pediatricians are the only GI doctors in Atlanta with experience installing mic-key buttons, probably because the mic-keys were originally created with children in mind. Over the course of this saga, Jin had spoken with numerous pediatric GI surgeons who wouldn’t agree to see me due to liability concerns (initially due to immediate wound care and then lack of malpractice insurance coverage because I was an adult). As we later discovered, Cindy’s best friend had experienced a similar nightmare in getting her mic-key sized properly. Dr. Saripkin, the pediatric GI doctor who agreed to see me at Cindy’s request, unfortunately has colleague who is currently battling ALS. Both Cindy and Dr. Saripkin were very kind. They listened to our saga and then answered all of our immediate questions. Dr. Saripkin then took the measurement and swapped the mic-key for a 3.5cm (there is no intermediate size between 3.0 and 3.5, slightly larger is much better than too tight). Swapping the tube still hurt, but it was obvious he was trying to be gentle and was at least cognizant of the fact that the procedure was painful. He later said his colleague with ALS specifically told him to never tell anyone a feeding tube swap of any sort wasn’t painful. Throughout the appointment, it was clear that he and Cindy actually knew what they were doing. Dr. Saripkin is the first doctor who was able to explain proper care and answer all our questions regarding the mic-key. It was a huge relief to know I finally had the correct size. They also gave us a couple prescriptions to help my wound site and stomach lining heal. As sad as it sounds, it was incredibly refreshing to have faith in a doctor’s competence.

I’d been waiting to send this update hoping I could report that all issues had been resolved. Unfortunately, even with the correct size, I still have granulation at times and a little discomfort, but it is much better than it was when the mic-key was too small. Overall, I’m a lot happier with the mic-key than I was with the PEG tube, and I think it was worth the headache. Jin may not agree. It’s still hard for me to understand why we had so much trouble getting it properly sized. Without a very persistent and well-connected husband, I would probably still have the original mic-key and many more complications. I’m very lucky Jin is so hardheaded and that there are kind professionals who care about the well being of the patient.

I promise I tried to condense this a bit! Congratulations if you made it all the way through.

Love,

Kim

Over Easter weekend, we went to Jamaica with friends for 6 days. It was Luke’s first plane trip and first trip to the beach, so he had a big time. The plane trip went better than expected, but the 2 ½ hour bus ride to where we stayed was miserable. We were all a little queasy, but poor Luke actually got sick! Thankfully, he didn’t seem too upset by it. The weather was wonderful, the beaches were beautiful, and Luke had a fabulous time with his daddy. Unfortunately, the mosquitoes were voracious. The nets over the beds clued me in early, but the nets and bug spray weren’t much defense. Also, Jamaica is not handicap friendly. Everywhere we went the ground was so uneven, I wasn’t comfortable walking around without a steadying hand. And there were stairs all over the place with no handrails; even in our villa. Jin got a pretty good workout every day helping me get around. I am glad we went, but I was relieved to be home. God bless ADA regulations! I could tell Luke was happy to be back as well, but he went through some daddy withdrawal.

In mid-April, Jin and I went to a mini ALS clinic to see the respiratory techs since I had a pretty bad cough during my last visit. Without my cold, I scored a 63 which was lower than my previous healthy score of 76. I still felt a little congested, though, so I thought that might have affected the reading. My weight was holding steady, so they didn’t push the feeding tube too much, but my continued speech deterioration had them ordering another modified barium swallow. Dr. Glass also wanted to have me fitted for a power wheelchair, since it can sometimes take months for actual delivery. He was worried about how often I was falling and didn’t seem to trust my assurances that I fall very well. J Throughout years of horseback riding, gymnastics, and soccer I think I’ve become somewhat of an expert, but he was unconvinced and I agreed to the appointment but warned the chair might sit next to the walker in the garage until I felt I was unsafe.

As most of you know, Jin and I went to the state capitol later that month to see the governor sign an ALS proclamation. And yes, I walked up the steps but not because I was being stubborn as Jin reported. I just didn’t want to walk all the way around to reach the ramp entrance! I figured the stairs were the lesser evil. And I’ll also admit that by the time we reached the governor’s office, a wheelchair was starting to sound pretty good. I’m sure Jin had the same thought, but he was smart enough not to mention it. By the next morning, I was exhausted. When I stumbled to bed that Tuesday night, it was obvious I caught whatever Luke and Jin had the week before. I stayed in bed all day Wednesday with a fever, stomach cramps, a headache, and an annoying cough. Every time I tried to sit to drink or take pills, I thought I would fall over. It took so much energy to try to stay hydrated and fed that for the first time, I seriously considered getting a feeding tube. Luke was really sweet, though. Abbie said he would come in to check on me, see that I was sleeping, say ‘hush’ with his finger pressed to his nose, and shut the door. What a thoughtful son.
I thought I was feeling better by the time my family arrived on Friday and even had a real breakfast Saturday morning. That night, though, I doubt I got any sleep; every breath resulted in a coughing fit. By the time I got out of bed Sunday morning, my legs were shaking so badly I couldn’t walk on my own. Every time I gasped for air, my back would cramp and I’d start coughing again. By the time Jin managed to get me to the bathroom, I felt like I was suffocating which scared me enough to make me cry. Since that wasn’t helping my breathing or the cramp in my back, I tried to stop as soon as I could.

Most of you know the rest. On Monday, I was admitted to the hospital with pneumonia and spent the rest of the week missing Luke and wanting to go home so I could sleep in my very comfortable bed. The pneumonia was almost gone by Saturday and I was up and walking around, so I convinced my doctor to discharge me. There was no way I wanted to stay until Tuesday as he’d told Jin! I was good about taking it easy the following week, and the follow up chest x-ray that next Friday showed my lungs were completely clear. In addition to being absolutely miserable, I lost five pounds while I was sick. Not good, but the lost weight helped to cement my decision about getting a feeding tube.

While I was in the hospital with pneumonia, the MDA dropped off a loaner wheelchair for me to use. Over my recuperation week, I started to roll around the house to conserve energy and stay mobile. If I sat down on a couch or chair, I needed help to get back up. But when I was using the wheelchair to get around, it wasn’t an issue. Plus, Abbie didn’t have to worry about me falling. At one point, though, I did come a little too close to the top of the stairs when I was rolling backwards to chase Luke. It would be pretty ironic if I were seriously injured in a fall because I was in a wheelchair rather than on my feet! I’ve been more careful since.

In mid-May, Jin and I spent a day at Emory for another modified barium swallow exam and to be fitted for a power wheelchair. As I thought, I’m doing fine swallowing; everything went down correctly without any issues. The wheelchair appointment also went well. I drove the model they are ordering for me, and I was able to maneuver pretty easily. As much as I’ve argued against getting a chair, it will be good to have. I just wish getting it around would be easier; we’ve concluded that we’ll need to buy a van to transport me in the chair. It’s not a good time for this unplanned expense. Plus, we’ll be paying some portion of the obscenely priced power wheelchair and we’ll need to buy a manual wheelchair ourselves as backup and for air travel. I’ve become a pretty expensive wife.

Two days later, we were back at Emory for my feeding tube procedure. That experience has been well documented, so I won’t rehash it. Jin was at home the entire next week taking care of me since I couldn’t handle the bathroom without him. I basically held him captive all week for 10 minutes of help every few hours. One of the doctors at Emory said that recovering from a feeding tube procedure is like recovering from a gunshot wound. This makes sense since they put a hole in my stomach, but I just didn’t think it through.

Jin spent a lot of his time that week and since trying to sort out my medical equipment. Ordering a manual wheelchair and an over-the-bed table shouldn’t have required so much effort! I wouldn’t even attempt to guess how many hours he has spent navigating the medical subculture to find answers. Plus, Jin has been trying to figure out the van situation so we have a way to transport my power wheelchair once it comes in. After the warnings we were given about the slow turn-around on these chairs, we thought we’d have plenty of time to research the different types of vans. We were surprised when they recently told us the chair should be here before the end of the month! It will be good to have around the house and neighborhood until we buy a van, but now I think Jin’s feeling rushed. Our wheelchair tech actually said we shouldn’t buy anything, though, until we get the chair and can be certain our choice will work as expected.

Ever since the pneumonia and then the feeding tube surgery, I’ve been feeling more weak and shaky when I walk so have been choosing to roll myself around in the borrowed transport chair quite a bit to conserve energy and be a little safer. As an added bonus, riding in the chair lets me move around faster and means I need a little less help. I’m still able to walk and can climb steps with help, but I’m trying to be more conscientious about safety. I think I’m getting better, but Jin may disagree. If I’m going to be using a wheelchair more often, I may as well be comfortable. The powered chair will be a huge improvement over what I have now.

As for the feeding tube, Jin and Abbie have become experts. We use it about three times a day to supplement my diet with Ensure and give extra water. Since I need to take small sips of water to avoid choking, it’s hard for me to drink enough otherwise. The good news is that the incision no longer hurts and I’m gaining weight back after reaching a low of 115 pounds. Also, I have an appointment to replace the annoying, foot long feeding tube with a button flush against my stomach on June 20th. I’m literally counting down the days!

Besides all that, here’s my current status:

• Using the BiPAP consistently at night.
• Trying to remember to use the cough assist again after letting my stomach heal.
• Walking and talking with considerable effort.
• Taking Rilutek, Quinine, and Arimoclomol prescriptions.
  Planning to begin Lithium again now that I’m healthy again. I’d only been taking it a few weeks when I got sick, so we put it on hold.
• Dealing with tight, sore shoulders since they compensate for my hands and forearms.
• Worrying about my neck muscles since they tire quickly.
• No longer attempting to talk on the phone and have asked friends and family to get set up on IM.
• No longer driving.

Luke is fantastic and celebrated his 2nd birthday June 8th. He had a blast at the pool party with his friends but became a little too bold in the water for his momma’s comfort. I think Abbie may have Luke swimming by the end of the summer. J He also continues to amaze me every day with his growing vocabulary. It’s crazy to hear my baby start to speak in sentences! Luke has become such a little boy. It’s fun to watch but heart wrenching at the same time.

A few things Luke has been up to lately:

• Nods his head while asking us for something as if trying to influence our decision.
• Puts his finger to his nose, says ‘hush’, and whispers ‘sleeping’ when he sees a person or animal sleeping in a book.
• Starting to sing along to ‘You are my Sunshine’ and ‘Twinkle, Twinkle Little Star’ in bits and pieces. He has such a sweet little voice.
• Pushes buttons on his guitar while spinning in circles and bouncing up and down.
• Says ‘eww’ and ‘yucky’ when he’s getting a ‘big poopie’ diaper changed.
  Has started to point to his ‘hurt’ wanting a kiss.
• Starting to potty train and finally went pee in the potty the day after his birthday. When he finished, he looked up at us and said ‘bit more’. He seemed very disappointed when we said it was all gone.
• Says ‘No poopie, no poopie’ while he’s pooping in his diaper to avoid the potty.
• Starting to sing his ABCs.
• Completely understands most things we tell him to do: give daddy a hug, ask Abbie to put your shoes on, don’t step off the deck, etc.
• Tests us more often over obedience.

No matter how badly he sometimes behaves, though, Luke is incredibly good natured. I know I have a mother’s bias, but he’s a wonderful little boy.

Ok, almost done! I just want to let you guys know that Morris Jewelry, my dad’s jewelry store, is having a charity auction to benefit the foundation from June 19 – 21. Anyone can participate, so please help spread the word. I’ve attached their flyer and more details can be found at http://www.morrisjewelryauction.com. This website should be available soon.

Also, since everyone asks how they can help but we never have a ready answer, we have recently decided to set up a website using http://www.lotsahelpinghands.com/home/ to help us manage things. Here’s their synopsis:

Lotsa Helping Hands — a simple, immediate way for friends, family, colleagues, and neighbors to assist loved ones in need. It’s an easy-to-use, private group calendar, specifically designed for organizing helpers, where everyone can pitch in with meals delivery, rides, and other tasks necessary for life to run smoothly during times of medical crisis, end-of-life caring, or family caregiver exhaustion. It’s also a place to keep these “circles of community” informed with status updates, photo galleries, message boards, and more.

We haven’t created the calendar yet, but thought we would start allowing people to join. If you’re interested, click on http://www.lotsahelpinghands.com/c/605279/ and enter your email address under “Interested in becoming a member of this community?” to get started.

Thanks for keeping us in your prayers!

Love,

Kim

So we woke up early this past Friday morning to drive down to Emory for my feeding tube procedure. For once we were actually early! We were supposed to arrive by 9:00 and we pulled in at 8:30. I think the reason why we were able to make such good time was because I wasnʼt allowed to eat breakfast. Itʼs amazing how long it takes me to eat. Which is another practical reason to get the procedure.

By the time Jin filled out the paperwork, we only had to wait about 20 more minutes before they took me back to get prepped. Unfortunately, they took me to an open ward so Jin wasnʼt allowed to come with me. Most of the nurses werenʼt able to understand what I was saying which made Jinʼs absence doubly irritating. Because the first nurse lost patience trying to understand me, she ended up attempting to insert an IV into the back of my left hand with a regular sized needle and blew out the vein. It hurt like hell. The next nurse took the time to decipher my speech and used the smallest needle to place the IV in my forearm as I suggested and got it in one try.

I was ready for the procedure around 10:15 and was rolled down the hall to a tiny room. The two nurses and Dr. Cook had to be careful of all my attached wires as they navigated the space. Once I was hooked up to the monitors and had the oxygen flowing up my nose, I double-checked with the doctor that the diameter of the tube he was using would be compatible with a mic-key button. When he responded that it should, I asked him ʽshouldʼ or ʽwillʼ and he changed his phrasing to ʽwillʼ. I hope he’s right, because his first answer didnʼt inspire much confidence.

One of the nurses put a mouthpiece between my teeth while another pushed the drugs that would sedate me enough that I wouldnʼt fight the procedure but would allow me to stay awake. General anesthesia is not given to ALS patients because the doctors worry our weaker respiratory function will fail. Then they put an endoscope in my throat and told me to swallow. I couldnʼt help gagging and coughing as it moved down over the back of my throat. I was told the scope has a camera, a bright light, a grabber, suction, and air. They pumped my stomach full of air to press it against my skin, used the light to locate the stomach from the outside, and poked a hollow needle through the skin into the stomach. Then they put a wire through the needle, which they grabbed with the scope and pulled out my mouth. They used the wire to pull the feeding tube down my throat and out the hole. There was a plastic piece on the tube inside my stomach to keep the tube from coming all the way out and another one on the outside to keep the stomach tight against the skin. Obviously, the procedure wasnʼt pleasant but it was quick. I was back in the open ward by 10:50.

I slept on and off while they tried to find an open room. After they wheeled me over to Jin around 12:15, we were led to a room with two beds, since they still hadnʼt found a private room available. Kyle had come to the hospital at some point while the procedure was being done and came with us to the room. I was glad Jin hadnʼt been waiting all that time by himself, plus heʼd have someone to talk with once I got settled. I knew I wouldnʼt be very good company since the incision in my stomach was beginning to hurt like hell. Moving from the one bed to another made my stomach feel like it was on fire. They gave me Lortab through the feeding tube to relieve some of the pain, but I didnʼt feel as though it was very helpful. When the nurse pushed the medication, I was surprised at how high the tube had been placed. I had pictured it in my mid abdomen, but it was inserted right below the front of my left ribcage.

Shortly after I was settled, they brought in my roommate and her family. Since there were curtains between us, I never saw her, but I heard way too much. It was as though the nurses and doctors had never heard of HIPA. I learned more than I ever wanted to know about the consequences of removing too much of a personʼs digestive tract.

A dietitian came to see us a little while later but didnʼt have too much to say when she learned I would still get the majority of my nutrition by mouth. She did tell us, though, that five bottles of Ensure would meet my daily nutritional needs which is good information to have. Dr. Glass and two others came over from the ALS clinic to check on us around 5:00. I was grateful they stopped in since he found a kink in the tubing that was keeping my stomach from draining. Once that was fixed, my stomachache wasnʼt as painful. The incision was still killing me, though, so Dr. Glass ordered 2 mg of morphine. I wasnʼt impressed with this drug either. As soon as the nurse pushed the morphine, I got a pounding headache and felt nauseous. When my stomach muscles tightened in reaction, I was in more pain than before the morphine had been given.

The pain was actually manageable as long as I could relax and lie still. Since I use my stomach muscles to create volume when I speak, I was using my laptop to communicate. And unfortunately for Jin, laughing hurt so badly it made me cry. I finally wrote him an explanation to tell him that while I realize he uses humor to relieve stress, he was killing me. As soon as I would try to stop from laughing, the pain would make me cry. With every stomach muscle contraction, the pain went a notch higher and wouldnʼt lessen until I could concentrate enough to force the muscles to relax. Jin would try to help, but when he would talk or touch me, that concentration was broken. Iʼm such a mean wife; I basically told him to leave me alone. Jin probably felt like he was being subjected to childbirth all over again..

We were finally moved to a private room a little after 8:00. Since Shelley came to visit and deliver dinner for Jin while we were being moved, she had to wait for a bit so we could tell her where to go. Shelley was entertaining me with her reaction to the nurses; she wanted to find them and make them respond faster to our requests. They were slow, but weʼve learned that itʼs unwise to make a nurse angry. In the hospital, the patient is completely at their mercy. The nurse did finally arrive with the morphine. I had asked to be given the higher dose of 4 mg since I was still in pretty severe pain. I was hoping the headache and nausea that came with my first dose werenʼt related to the drug. Unfortunately, they werenʼt a coincidence, so that was my last try at morphine. I was actually pretty disappointed since Iʼd had high hopes that it would make me pain free. The drug helped a little, but it didnʼt help enough to be worth feeling miserable for 10 minutes after it was administered. I went back to Lortab.

I tried to have Jin go home to get a good nightʼs sleep and see Luke, but he wouldnʼt listen. He wasnʼt impressed with the nurseʼs response time. Poor Luke; he didnʼt see mommy or daddy Saturday morning. Thank goodness Abbie has volunteered to take care of him until Jinʼs mom arrived to take over.

As it was, I ended up being very glad Jin had refused to leave. Getting to the bedside toilet hurt like crazy, but at least we had a system. It would have been a lot more painful if the nurses had attempted to help me on their own. Unfortunately, Jin was exhausted the next day. He said he didnʼt sleep until around 4:00 because he was worried about me. Plus, he was freezing since the nurse didnʼt give him bedding. I guess he didnʼt want to bother her so waited until the tech showed up to check my vitals at 5:00 to ask about sheets and a blanket. I wish Iʼd been paying better attention.

Overall, I felt much better on Saturday. Standing from the bed still hurt, but it didnʼt make me want to cry anymore. I could also move with less pain while lying down and I could talk more easily. The neurologist who came to talk with us that afternoon said the pain should ease off in a few days but wouldnʼt be completely gone for a week or two. Our day nurse, Carolyn, was great and showed Jin how to use and flush the tube. It didnʼt seem too complicated.

We thought Iʼd be able to leave early afternoon, but they wouldnʼt discharge me until Iʼd proven I could tolerate feeding through the tube. Since they wait 24 hours after the procedure before attempting food, the first try should have been around 10:30. The nurse delayed this, though, because I felt nauseous about an hour after she gave me water and Lortab through the tube. Carolyn said it was probably caused by trapped air in my too full belly. I felt better after she opened the tube to release the air. At 1:30 she talked Jin through feeding me half an Ensure, and we went through the process again about three hours later.. Both times I felt a little queasy, but it was pretty mild. I kept quiet since I wanted to go home.

Shelley came by again mid afternoon and went back out to get Jin a late lunch and to pick up some powerade for me. She left around 5:30 when Carolyn told us we could go home. Surprisingly, we were walking out the door an hour later. Wright had stopped by while we were packing up the room so helped make our escape a little more efficient. Since transferring to the over-sized wheelchair/luggage cart already had me feeling a little queasy, the side-to-side wheelchair ride and elevator trip had me fighting back nausea. I thought I was feeling better by the time I was loaded into the truck, but I had to ask Jin to pull over after driving just a few miles. Luckily, I didnʼt get sick and we made it home without incident. I canʼt even imagine how badly that would have hurt.

Jinʼs mom had just finished feeding Luke dinner when we pulled up to the house, so I was able to spend some time with him before I needed to lie down. As usual, Luke didnʼt seem to miss me at all even though I missed him like crazy. I know I should be pleased that heʼs independent and feels secure, but he could miss me a little.J I felt loved later, though, when he brought his favorite doll, Raff, to my bed and gave me goodnight kisses.

Even though Emory hospital was a little sub par, I took home some goodies. The wedge pillow for under my knees and the cushioned booties helped me sleep great last night. Iʼm sure the Vicodin probably helped as well. The incision site still hurts quite a bit, so I’ve been hanging out in bed today. I have my laptop set up next to me, which should keep me from getting bored when Iʼm not napping. I ate an actual dinner last night and breakfast this morning so feel like Iʼm doing pretty well. Plus, Jin has been showing off his new feeding tube skills by supplementing my diet with Ensure. Iʼll hopefully gain the lost weight back quickly.

As usual, this note is longer than I intended! I hope it wasnʼt too graphic for any of you. Prayers for a speedy recovery would be greatly appreciated!

Love,

Kim

As some of you may have heard, Kimberly was checked into North Fulton
Regional Hospital’s Neuro Intensive Care Unit on 4/28/08. She asked me
to write out this update to quell any fears.

Last Tuesday we had an eventful morning at the state capital. We went to
see Governor Purdue sign a proclamation regarding ALS. As usual,
Kimberly was a stubborn (insert your own appropriate word) and refused
to go to the handicap ramp to get into the capital building. Instead she
not only walked from the parking deck, but walked up the entire set of
stairs into the state capital. I’ve included the pic of us and some of
our friends with the Governor and the proclamation.

The walk up to the capital from the truck combined with the walk up the
stairs pretty much wore Kimberly out. Upon our exit, I ended up carrying
her back down the stairs and leaving her with a friendly capital guard,
while I went to fetch the truck. We then went out and had a great lunch
with some friends. Kimberly was pretty much beat from the activities and
had a low key afternoon. Sometime thereafter she most likely caught a
virus, possibly the flu. Luke had been throwing up the week before and I
had been dead in bed with chills and fever over the previous weekend.
Kimberly had the usual headache, fever, sweats, nausea, etc. She was
pretty weak, didn’t eat much and was unable to walk unassisted. Luckily
Abbie was a super trooper and helped nurse Kimberly back into shape. By
Friday, she seemed to be getting better. On Saturday she began having a
slight cough. By Saturday evening, every breath that she took turned
into a cough. Kimberly was up all night with a raging fever and vicious
hacking coughs. By Sunday morning, she had a severe pain in her back and
stomach from the non stop coughing. By Sunday evening she could barely
sit up or take a shallow breath without going into coughing convulsions.

On Sunday evening, I contacted Kimberly’s primary care nurse Shanon, who
contacted Kimberly’s primary care physician Dr. Hall. They called in a
prescription to the 24 hr pharmacy. This allowed her to finally get a
few hours of sleep. However, every 3 hours she would begin the coughing
fits as the prescription wore off. Monday Kimberly was lethargic,
nauseous and sweating profusely. We went to see Dr. Hall to allow him to
evaluate Kimberly’s breathing and her general state of being. Dr. Hall
was concerned with Kimberly’s oxygen levels and wanted a chest xray. He
sent us to the Emergency Room. At the ER, Kimberly’s chest xray
confirmed Dr. Hall’s fears of Pneumonia. They took multiple blood
samples, and then started IV antibiotics.

Evidently it was a banner day at North Fulton Regional. In our 7 hours
in the ER, I saw multiple car accident victims, laceration (read knife
wounds) victims, almost had a tub of vomit spilled on me, etc. Laura was
nice enough to bring Kimberly some Powerade and a baked potato (since
they didn’t want her to eat and only had diet gingerale to drink).
Shelly was nice enough to bring me and Kimberly’s sister, Jenni, Chinese
food. They finally admitted Kimberly into the Neuro Intensive Care Unit
around 10pm Monday evening. After 2 rounds of fluids and antibiotics,
Kimberly started to perk up and become more responsive. As it is an ICU,
I was not allowed to stay with her.

Kimberly was moved from the ICU on Wednesday to General Population. They
moved Kimberly to the room directly in front of the nurses’ station so
they could give her the additional attention. Kim was taken off IV
fluids prior to the move downstairs. A standing order was given for
nurses to check on Kimberly every hour and offer fluids. The nurses
downstairs are just as nice, but the ratio patient to nurse is a bit
higher. The facilities in General Population are a bit more weathered.

On Wednesday morning, Kimberly looked and felt better. Her speech was
relatively clear and she was very lucid. Kimberly was given a cheese
burger but it tasted like a hockey puck, so Jeff brought us Taco Bell
for lunch. Mid afternoon Shannon and Kristin came by to relieve me so
that I could go home and get Kimberly her own clothes. Elizabeth and
Jake also dropped in to check on her. By mid-afternoon, Kimberly was
looking a bit ill and her speech had becoming unintelligible. I
requested that the IV be re-inserted. I explained that although Kimberly
could swallow, she needed someone to offer a drink to be able to
swallow. They promptly re-inserted the IV and began fluids. Later
Tracey and Jerry dropped in and Heather brought us ice cream. Kimberly was
tired and decided to take a nap.

Heather and I ran out to get dinner and pick up a web cam so that
Kimberly could see and communicate with Luke. After dinner, Kimberly was
looking much better. I set up the web cam and attempted to connect with
Abbie at home with no avail. I thought there may be a server restriction
at the hospital and I finally gave up. I intended to spend the night
with Kimberly (rules are a bit more flexible due to being in General
Pop). Kimberly kicked me out and made me go home so that I could play
with Luke in the morning.

Thursday morning, Abbie and I got the web cams working at home. Luke
wanted to come visit his mom, but Kimberly and I still didn’t want to
expose him to this environment full of germs. Kimberly called the house
and asked me to bring her more clothes. Her speech was flawless. Since
Luke couldn’t visit, Luke and Abbie traced his outline on a poster board
and gave it to me to give to Kimberly. I stopped in at the office and
checked the Firm. It seems like Heather, Michael, Lindsey, Kareem, and
Heather are holding down the fort. So I picked up lunch for Kimberly and
headed back to the hospital. Kimberly looked great. She had been up and
moving around and was dressed in her own clothes. Her speech was almost
completely clear. I attempted to establish a web cam link with Abbie at
home to no avail. Later her PA said that the chest x-rays were looking
better. He hoped that she would be able to leave by Saturday. Jake and
Erin came for a visit and brought a smoothie for Kim. Buzzi came by and
brought a milkshake. The Pulmonary Doc came while Kimberly was taking a
nap. He said that she was headed in the right direction, but was
concerned that she could relapse quickly. He said if everything kept
heading in the right direction, she could go home by Tuesday. According
to the Pulmonary Doc, it could easily take 2 to 3 weeks to completely
clear up and he didn’t want to take any chances due to Kimberly’s
inability to cough up the goo from her lungs. Kimberly’s dinner
consisted of turkey and stuffing. As I have now also become her taste
tester, I took one bite and we agreed that we should save the money and
simply stop ordering meals. Luckily Tracey and Jerry called and took our
dinner order. After dinner Jerry attempted to use his crazy computer
skills to make the web cam work, however he also determined that there
the necessary port on the hospital’s servers were blocking us from
success.

Thankfully, Kimberly was released from the hospital on Saturday, 5/3/08
after convincing the doctor she was well enough to go home even
though her right lung still showed signs of some pneumonia. Kimberly
continued on oral antibiotics while resting at home. The follow-up
chest x-ray on 5/9 gave great news, showing that the pneumonia was
gone.

Jin and Kimberly thank everybody for their thoughts, prayers, and well
wishes.

In early October, I started sleeping with a BiPAP (http://www.wisegeek.com/what-is-bipap.htm). I know it helps me breathe better at night, but I have a hard time keeping the nasal pillow apparatus in place since I tend to shift quite a bit when I sleep. I probably average about 6 hours a night. I really do try to keep it on as long as possible, but when I accidentally knock it off center, the air flow is constricted and I feel like I’m suffocating. When I can’t fix it myself and don’t want to wake Jin, I yank it off. In case you’re curious, a nasal pillow device is a type of mask that seals against the outside edge of each nostril rather than around the entire nose. If you ever want to look extra sexy, I highly recommend wearing one. I’m sure Jin would agree.

To help maintain my respiratory function, I’m also still using the cough assist. Jin tattled at the ALS clinic I went to in mid November, so I was given a lecture by the respiratory techs. They want me to do a session twice a day. I really have no excuse, since a session only takes about 5 or 10 minutes. I’ve gotten better, but I need to be more conscientious. Even with my less than ideal use of equipment, though, I scored better on my breathing at clinic. I went from 66 back up to 74! Since this was the first time I did better rather than worse, I was pretty excited. I had also maintained my weight, so I was 2 for 2. I’m fairly certain my weight held steady because I had decided to ignore the empty stomach rule I had been following for the Rilutek. I had been willing to follow the rule until I was told the medication is crushed and mixed with food when patients begin having difficultly swallowing pills. I was pretty irate when I heard that little tidbit.

Since my speech has significantly deteriorated, the Emory folks were concerned about my swallowing and ordered a Modified Barium Swallow in mid October. According to a website I found, “The Modified Barium Swallow is an x-ray procedure that obtains views of swallowing function that are then recorded on videotape. The Modified Barium Swallow is useful in determining the presence or absence of aspiration of material into the airway and assessing the movement of material into and through the upper portion of the esophagus.” The procedure was quick. I simply had to eat and drink different consistencies while they recorded a video x-ray. The video was interesting to watch and thankfully showed everything working as it should. With the swallowing test results, my improved respiratory score, and my maintained weight, the feeding tube is on hold for a little while at least. Of these three, though, my respiratory function is the deciding factor. I thought swallowing would be the most important factor, but respiratory function determines whether the surgery to insert the tube can be performed safely. Based on my last numbers, though, I seem to be in good shape.

The ALS gala we attended in October went really well. I watched the video a few times before we went hoping to keep myself from crying while we were at the event, but I wasn’t able to pull that off. Oh well!
My segment has been uploaded to the foundation website if you’re interested. When the video was taken, I remember thinking how much I disliked the way my face looked when I spoke and how unclear my speech had become. After recently watching the video again, I was surprised at how much my speech and my facial control has deteriorated since July. It’s not encouraging.

In October, I also started a new medication to help relieve some of my muscle cramping. I’m happy to report that the quinine has been working great! I get the occasional leg cramp in the morning, but they are no longer a daily occurrence and they aren’t nearly as painful. Unfortunately, my hands and arms still cramp fairly often with use, but they seem to be less severe. Since the quinine is the only drug I’m taking that I know for certain is helping, it’s a little irritating that it’s banned in the US. I’m ordering the pills from Canada! Add this to the list of things I never thought I’d do. I’m also still taking Rilutek and the experimental drug Arimoclomol. At the end of December, we doubled the Arimoclomol dosage to match the most recent clinical trial and I’ve continued to tolerate it well.

I mentioned in my last update that Jin was going to start a foundation. Most of you probably already know about the website, but in case you don’t it’s http://www.kimkimfoundation.org/. Susan Fisher and her boyfriend Julian did a wonderful job designing it. Thanks again you guys! In addition to details on the foundation, the site also has my history and updates. We’re trying to plan our first official fundraiser for the Spring, but even without an event we have had generous donations from so many of you! I’ve been amazed by your support. Speaking of which, the ALS walk in November was a huge success! You all helped me raise $6,266 and our team raised over $14,000!! What a wonderful contribution to the ALS Association. Thank you!

One major change we made in December was hiring a new nanny for Luke. We are very grateful for the job Ellen did for us; she was wonderful with Luke and was a perfect addition when I began needing help caring for my son. Unfortunately, she had a fairly long commute and wasn’t able to commit to the additional hours we needed as I became less comfortable watching over Luke by myself for an hour or so in the morning. Abbie began working for us a few weeks before Christmas and became a favorite for Luke right away. The fact that she and Luke got along so well, helped me feel less guilty for making such a big change to his world. Abbie has been with us about three months and is doing a fantastic job taking care of our family. I’m hopeful she’ll put up with us for a while!

As for me, I’m still walking and talking although both continue to get a little more difficult. To help make walking “long” distances a little safer, I’ve borrowed a rolling walker from the ALS Association. It’s a bit of a hassle to load the walker in and out of the car and I don’t use it often, but it’s good to have the option. There is also one benefit I hadn’t considered: Luke has fun riding on the seat. If that continues, I might be able to help “carry” him once in a while. So far, the novelty hasn’t worn off. I can still walk upstairs if I go slowly and use the handrail, but I don’t go down without help and will choose to use an elevator if I have the option. I also continue to eat the same kinds of food as before and am usually able to be independent, but I’ve decided it’s smarter to accept help when it’s available since the process can be exhausting. I try my best not to be self-conscious. If I eat an entire meal on my own, I really think the effort burns about half the calories I take in. That‘s obviously less than ideal when trying to maintain weight.

As I mentioned, talking has become harder for me. A noisy room makes it especially hard to be understood. But again, please don’t pretend to understand me. I’ll usually know if you do! If you know there’s no way you’ll understand me, please tell me. Trying to speak clearly takes a lot of energy; I’d much rather stop making the attempt than to keep trying for no purpose. With that said, I would of course prefer to be understood. You can help me by repeating what you think you heard. That way, I may have the option to only repeat a part of what I said. Also, please don’t let me keep talking if you don’t understand. These days, I pause quite a bit for feedback and to take a breath to continue speaking. If you’re unsure if you understood, repeat what you think I said. I’m sure you get the idea! If I take a deep breath before trying again, I’m not sighing in frustration. Sometimes a deep breath helps me speak more clearly. Hopefully, whatever I’ve said will make the effort worthwhile, but I’m not promising anything! I think I’m even harder to understand on the phone, so I’ll usually have Jin or Abbie make the call. Please don’t take it as an insult.

Beside all that, here’s the new laundry list:

• Knuckles on left hand are tender and hurt to fully bend.
• Right hand’s thumb, middle finger, and ring finger are only onesstill working ok.
• Range of motion in ankles and wrists has worsened.
• Legs seem generally weaker and less coordinated.
• Cannot stand unassisted if seated too low or on the groundunless I hold on to something.
• Right knee is starting to pop back a little when I walk.
• Knees ache after standing or walking for long periods of time.
• Calf muscles and hamstrings are extremely tight.
• Stumble over my toes more often.
• Climbing in and out of bed more difficult.
• Shoulders are considerably weaker which makes eating, driving,etc more difficult.
• Head falls back more easily.
• Notice considerably more spasms in arms and along entire back.
• Muscles in face and jaw fatigue more quickly when talking andeating.
• Tongue has lost function.
  • Much harder to move food and pills around in mouth.
  • Need to open my mouth to move food under teeth.
  • Big bites are difficult to deal with.
• Top lip has less function.
  • Cannot reach lip with tongue or bottom teeth.
  • Cannot seal mouth as well around a straw.
  • Harder to rub lips together.

In mid February, I was halfway through this update when Emory called to schedule my next clinic. Since my appointment was scheduled on March 7, I decided to wait to finish this e-mail so I could include the information from that visit. Unfortunately, I’m still getting over a cold that is now entering its third week. The lingering cough made the respiratory test very difficult; every time I’d take a deep breath to begin, I’d start to cough. We did manage to get three readings, but I wasn’t able to take a good breath so my highest score was 58. I know this isn’t accurate, though, so it doesn’t worry me. My weight is holding steady; I actually gained a pound. That was good news. They also suggested that using my hand splints more often while sleeping might help relieve the pain in my knuckles. I’ll try to wear them and see if they help. I’ve been avoiding the splints because they make my arms very heavy and cumbersome. I don’t sleep as well with them on and I know Jin doesn’t!

I was also able to get some help figuring out how to use my new Words+ software with a mouse. The Words+ software should help me type faster and will also generate speech from text if I need it for communication. Right now, though, I’m using a new OnScreen keyboard program that is much more user-friendly than the original one I was using. It also has better word prediction. Since I can only use Words+ on the new laptop, I’ll continue to use this program on my other computer. Another topic we discussed at clinic was lithium. Dr. Glass said he was actually pretty impressed with the results in an Italian study, so I have an appointment with my primary care doctor to talk about the protocol. You can read about the study at http://alslithium.atspace.com/ if you’re curious.

Ok, on to a much happier topic! Luke is turning into such a little boy. Two weeks ago he graduated to a big boy bed and he has been doing great! For the most part, he’ll stay in bed until we get him up and we’ve not yet found him asleep on the floor. So far, it has gone a lot smoother than we could have hoped for! He’s definitely still a full-blown toddler, though. We’ve started using timeouts, and there have been a few mornings where Luke has spent the majority of his time sitting on a chair. But overall, he’s still a very cheerful little boy and incredibly sweet. Luke has also been talking a lot more! Sometimes he sounds like a little dictator: up, down, now, no, outside, come on, more, push, spin. He definitely knows what he wants! He probably has at least 30 words in his vocabulary right now. Luke surprises me every day with what he knows; it’s impossible to keep up.
More cute things he’s been up to:

• Leans down toward R2D2 and says ‘R2’ to try and make him dance.
• Puts finger to mouth and says ‘ushhh’ along with the old lady in
  Goodnight Moon.
• Asks for one more book, cookie, cracker, etc by holding up his
  index finger with his arm outstretched or putting his hand near
  his cheek and saying ‘one’.
  * Helps unload groceries by standing in the cart then signs and
  says ‘all done’.
• Surfs on the back and side of carts or helps push when he
  doesn’t want to ride.
• Gets in his red car and asks us to ‘push’.
• Plays hide-and-seek by running after the person trying to hide.
  Says and signs ‘more’ after he finds us.
• Says ‘beep beep’ when pushing his car around the house.
• Signs ‘thank you’ by blowing a kiss.
• Gives a hug or kiss when we ask if he’s in an accommodating mood.
• Says ‘two’ when we ask him to say one.

As usual, this e-mail is much longer than I intended. I just have one more thing I’d like to talk about and then I’ll be done. Based on a few comments I’ve gotten, I think some of you are worried I might lose my faith. Don’t worry. I know God loves me. I believe He has a plan for my life. I’m not too thrilled with how this plan seems to be playing out, but that doesn’t alter my faith. I get sad and angry and frustrated at my situation, but I do my best not to let those feelings monopolize my day. It helps to remember I have a wonderful husband who loves me. I have a beautiful, healthy son who gives the sweetest hugs and makes me laugh every day. I have family and friends who offer tremendous support. And while I definitely believe it is in God’s power to lift this from me, I need to acknowledge it may not be in His plan. This doesn’t mean I’ve given up; we’re still praying for a happy ending, and we sincerely appreciate all the prayers being said on our behalf!

I’ll try to send updates more often, but don’t expect too much.

Love,

Kim

I am no longer going to have delusions on how often these updates will be sent! I’ll probably get them out after I go to the ALS clinic, which means about every four months.

We’ve definitely had a lot going on in the last few months. I hope you’ll make it through this e-mail! My cough assist machine was delivered mid-June, and I was very good about using it until I got sick a few weeks ago. Then once I was feeling better, I wanted to hold off until after the ALS clinic so I would get a true measurement on how my breathing is doing. I understand that may only seem logical to me! I’m trying to get back in the habit now.

I decided to quit acupuncture in July, but I’m now getting a massage twice a week and am still going to the chiropractor. I tried to stick with it (pun intended J:-), but as my muscles continued to atrophy, the procedure became pretty painful. Since I could never tell if it was actually helping and the stress of anticipating pain wore me out, I gave myself permission to quit. My week is a bit happier now! I also decided to swap out the Chinese herbs with more traditional supplements; my acupuncture guy couldn’t seem to keep track of which herbs he was giving me. It didn’t inspire much confidence.

In early July, I also agreed to be a part of a video the ALS association was putting together for a fundraiser being held on Oct. 12. I’d give the details, but they’re already sold out! I wasn’t sure how much of the interview they would be able to use, since I’m pretty incomprehensible when I cry, but the editors did a good job. We got a copy in the mail a few days ago; I’ve never liked myself on film, but Luke looks adorable in it! I’m still trying to figure out how I feel about having subtitles added while I’m speaking.

The most exciting part of July, though, was finally completing work on the elevator!! It’s been so nice to have the house back and not have to worry about what Luke might find on the ground. I’ve been trying to limit my use of it, though, and only ride when I’m feeling really unstable. Unfortunately, I am feeling unsafe on the stairs more often than not. Luke loves it, though, and seems to think it’s a cool toy we got just for him. Getting him on is usually pretty easy; coaxing him off is another matter entirely! The elevator has also been helpful with Chamblee. Our poor little arthritic dog has a lot of trouble with the stairs.

As some of you may have heard, we were pretty worried about Jin these past months. He started seeing some severe atrophy in his forearms and shoulders and began to drop things more often. Since these symptoms matched some of my early warning signs, we were understandably concerned. It also made us rethink my situation since the likelihood of a young married couple both having ALS is astronomically small. We decided to follow up with a contact my friend provided and were able to get an appointment at John Hopkins in mid-August for both Jin and I. Thankfully, the doctor said Jin does not have a neurological issue. It’s a good thing. I feel like I’m handling things ok, but having Jin diagnosed with something serious may have broken me. She couldn’t explain the atrophy and clumsiness, but basically told him to stop obsessing and start working out. J As for me, she agreed with Emory on my diagnosis. My progression seems to be a textbook case, so I wasn’t surprised and didn’t get too upset. I only started to cry after she had me try out a walker. That quickly turned to irritation, though, when she mentioned anti-depressants. What is it with doctors? Four out of five who have seen me crying offer me drugs. I understand they want to help, but I consider crying a healthy outlet. They should be more worried about my mental state if our conversations didn’t make me cry! We only had appointments on Thursday, so we had the rest of the weekend to catch up with friends who live in the area. Those visits definitely made the trip worth it! I missed Luke more than I can say, but I knew he was in good hands. Ellen, dad, and Jin’s mom worked in shifts and kept Luke very happy. When we got home on Sunday, it didn’t seem like we were missed at all.

I’m still taking Rilutek and the experimental drug Arimoclomol and continue to tolerate them well; although their effectiveness is up for debate. Dr. Glass prescribed a muscle relaxant in May, but I only took that for a month. It made me tired and didn’t seem to lessen the twitching or help with the muscle cramping. They suggested a few other meds and some vitamins I can take to try and alleviate some of these symptoms, but I haven’t started them yet. I’m a big believer in trying one new thing at a time and I haven’t decided what to try first. I’ll decide soon, though, since the cramping is getting a bit out of control. My legs give me the most trouble and usually cramp in the morning when I wake up; my hands, arms, and abs cramp during the day if I hold a position too long. The muscle spasms are also becoming more frequent and are sometimes strong enough to cause my hand or entire arm to move. I’m also starting to feel them along my back and quite a bit more in my facial muscles. That worries me since I feel like I’ve already lost a lot of facial expression and it seems like the spasms precede loss of function. That could just be my paranoia, though.

Beside what I just mentioned, here’s the new laundry list of what’s going on:

• Walking is more uncoordinated and my knees tend to hyper-extend more easily, which causes my legs to collapse forward

• Walking down stairs more difficult; requires more concentration since knees/legs feel more unstable.

• Increase in saliva causes me to drool more often.

• Must talk very deliberately to be understood.

• Harder to move food around in mouth.

• More difficult to sit up from lying down.

• Writing has become very labor intensive.

• Feel dizzy when I sit up.

• Legs and arms tighten and flex when stretching or when I first stand.

• Become out of breath at times when speaking.

• Facial muscles fatigue more quickly during conversation.

• Shoulders and hands are incredibly weak.

• Holding toothbrush up more difficult due to weak shoulders.

• Showering is exhausting.

• Must steady right hand with the left when applying makeup.

• More difficult to take a deep breath.

• Eating requires considerable more energy.

• Avoid foods that are hard to handle (soups, salad, deli sandwiches).

We went to the ALS clinic on September 21 and they confirmed that my breathing is not doing well. I wasn’t too surprised since I’ve noticed a change, but I was surprised at how low I scored. I’ve gone from 102 to 97 then 82 and now 66! They then had me retest lying down, I took and scored 33. I’m still not crazy about the idea of being hooked up to something at night, but I’ll do just about anything to be around for Luke. If that means having a mask over my face at night and coming to terms with the damn feeding tube they talked about, I’ll fall in line. Dr. Glass doesn’t think I need the feeding tube yet, but he’d like me to get used to the idea before I actually need it. I’m obviously less than thrilled at the prospect.

For now, though, I have decided to do something about my walk and get fitted for a leg brace as recommended by the physical therapist. She said it should help me walk without looking as though I’m hyper-extending my left knee. I actually had an appointment last Wednesday to have a mould made of my leg so they could create the brace and picked it up yesterday. It does improve my walk, but it takes more energy to stand and walking downhill is a bit tricky. I’m sure it will get easier, though, as I get accustomed to it. As for my hands, I was given hand splints by the occupational therapist to wear at night to keep them from contracting; I’ve been waking up with my hands balled into fists. I think I’m tolerating them ok, but I have woken Jin a few times in the early morning impatient for them to be taken off. And while Jin has already been adjusting covers quite a bit, he’s now officially on cover duty. Hopefully, I won’t drive him too crazy in the wintertime.

While we were at Emory, Jin also talked with Nicole and Glass about the various fundraising events to get an idea about the different groups. Jin’s been a little frustrated by the lack of organization. During the conversation, we found out the clinic and ongoing research is self-funded. I think Jin is going to create a foundation and put his rolodex to good use. It will be good for him to have a focus and goal; I know it’s hard on him to feel so helpless. I have such a wonderful husband.

Luke is still wonderful as well even though he has fully embraced being a toddler. He can throw a fit with the best of them and loves to repeatedly shake his head no. I think his cousin showed him that last trick. J My sister-in-law, Javiera, and my niece and new nephew were here for the first two weeks of September. I had a great time getting to know the kids, and Luke seemed to enjoy having a playmate once they worked through their differences! Luke has also been attending GT football games and does a great job clapping for our team. He’s been running and stomping around the house and bounces to the beat when he hears music. He’ll even dance to Daddy’s cell phone. J It’s been amazing to see him beginning to understand so much more of what we say. Although that can cause a bit more irritation as well when we know he understands and chooses to pretend otherwise! His first “word” was oh-oh back in July and he’s since added dada, no, and (finally!) momma. He also pants when he sees the pups, so we consider that his word for dog. J Luke has done really well with signing; he’s learned “all done”, “more”, “milk”, “drink”, and “shoes”. We’re working on “please”, “socks”, “hat”, “on”, “off”, and “outside”. I think he’ll catch on quick.

A friend from soccer has organized a team to join the ALS walk being held November 3rd at Centennial Olympic Park. Registration begins at 8:00. Jin, Luke, and I are planning to walk, and we’d love for you to join us or donate to the event. You’ll find the details at http://walktodefeatals.kintera.org/faf/r.asp?t=4&i=226925&u=226925-187788351&e=1221144405. From there, go to “My Team Page” then “Join Our Team” to join the walk. We hope to see you there!

Also, I’d like to tell you about a video you can view on-line. It’s really well done, and each time the video is viewed, a donation is made to help find a cure for ALS. Please pass it on!

http://www.whatkindofworlddoyouwant.com/videos/view/id/382360

Your prayers and encouragement mean so much to us. The support you’ve given has been amazing!

Love,

Kim

I went to my second ALS clinic at Emory on the 25th of May. The only real information we got out of the visit is that my breathing is not doing as well as it has in the past. On my first visit to Emory in November of last year, I scored a 102 on the respiratory test. In January, I was down to a 97 which was still considered excellent. During this past visit, my lowest score was a 77 and my highest was an 86. The trend obviously is not a good one and it had the respiratory technician a little concerned. She said the 80s are considered average for someone of my age and height, but the fact that I keep dropping could be a problem. She mentioned that ALS patients who begin respiratory therapy earlier seem to do better overall and wanted to get me qualified for some device to use at night. I ended up with scores that put me right above the range for qualification. Oh well, I’m really not looking forward to sleeping with something attached to me anyway. J Associated with my respiration is a weaker cough. I noticed this a few months ago when I had a cold; it was very difficult for me to cough and clear my throat. I’m sure I sounded very ladylike in my attempts. While I did not qualify for whatever device they wanted me to use at night, they did have us order a cough assist machine to use twice a day in order to keep my lungs clear. I am not very pleased at having to use such a thing, but it will supposedly keep my lungs healthier and decrease the chances of pneumonia. Those are obviously good benefits so I’m going to get over it. The Emory doctor also prescribed me a new muscle relaxant medication to use at night. I have been waking up with some cramping in my legs and he thinks the muscle relaxant will help with that. He also thinks it may help smooth out some of my movements and to lessen some of the twitching. Supposedly, the only side effect is drowsiness so he told me to introduce it at night first and work up to taking it three times daily. We’ll see how it goes.

Considering that a year ago I hesitated to take Tylenol when I had a headache, I am shocked to find myself in the possession of two separate weekly pillboxes: one for my prescriptions and the experimental drug and the other for supplements and Chinese herbs. I have surprised myself at how willing I am to try my “alternative” pillbox, but I have reasoned that they are probably not doing any harm and could possibly help, so why not? I am still not having any noticeable side effects from the experimental medication. The blood work that has been done monthly also shows that my kidney and liver function are still normal. While I am obviously grateful for this, the medication has also not had the positive results we had hoped for. We had hoped the medication would slow or halt progression of the disease. There is really no way to know whether or not it has helped slow progression since we have no idea where I would be if I were not taking it, but the progression still seems fast to me.

While on the medication, I have noticed new muscle spasms along both arms, my neck, around my mouth, and down my feet and legs. My shoulders and legs are also considerably weaker which make daily tasks and walking more difficult. After taking a pretty bad tumble down my front porch steps attempting to walk the dogs, I am trying to be better about determining what I should and shouldn’t do. This is hard, though, because I want to continue to do all that I can for as long as I can. It seems an injury is required before I’ll concede. I do try to be very deliberate in what I do in order to avoid accidents; I think my grandmother might be able to beat me down the stairs at this point! Besides my jittery walk, the most annoying new development over the last few months is that my right hand has followed my left in terms of atrophy. I still have good use of my middle and ring finger on the right-hand which helps in some tasks, but writing has become very challenging. Holding a glass and utensils has also become harder, but it is still doable.

One of the harder changes to adjust to concerns my speech. I have always loved to chat with friends and family and meet new people, but I have found myself growing a little shy since I don’t like the way I sound sometimes when I talk. It definitely feels as though my speech has worsened in that it takes more effort to speak clearly and sometimes that effort does not pay off. I am trying not to let the slurring bother me, but I am not there yet. Please do not feel badly if you need to ask me to repeat myself; I would much rather repeat what I have said and be understood. Also, as a warning, I find it almost impossible to make myself understood when I am upset. If I stop speaking for a while, I am probably just trying to get control of my voice.

I know that all sounds pretty bad, but I’m actually doing okay. I am still walking and talking and enjoying watching Luke discover the world. Ellen is now coming to help during the weekdays from 10 to 7 and has become invaluable. Luke adores her, which makes it easier for me to leave for my appointments. I am still doing acupuncture and seeing a chiropractor and massage therapist every week. My chiropractor wants me to take yoga as well, but I have not been willing to add yet another appointment to my week. I may have limited control over the progression of this disease, but I do have some say in how much I am willing to give over to it. I want to be proactive and do what I can, but not at the expense of all of my time. I’m still trying to find a good balance.

As for the house, we are in full construction mode on the elevator enclosure. The add-on looks like it’s going pretty well, but the order on the elevator itself was delayed so it will not be ready until July. I am sure I’ll be fine until then, but it’s too bad this process will be so drawn out. Poor Luke probably does not remember what quiet sounds like. He seems to be handling it very well though; he actually likes to watch a lot of the activity in the backyard. It’s his personal TV show.

Today is Luke’s first birthday! He took his first steps on Mother’s Day and has become a pretty good walker since then. Yesterday, he was speed-walking through the house and clapping his hands very pleased with himself. He has also become pretty adept at using a spoon and fork, although he obviously gets very messy! He loves it though; Luke’s a very independent baby.

I guess that’s it for now. I’ll try not to let another four months go by before my next update. Thanks to everyone for the prayers! We definitely need a little help on this one.

Love,

Kim