As usual, I’ve been meaning to send this out for a while. There were only two months between my ALS clinic appointments this time, but I’ve been spending my computer time on other projects. And these updates definitely count as a project. The last one took me 1 ½ weeks to write! My deteriorating speech makes using Naturally Speaking impossible, and typing with one finger takes a little while. Lately, I’ve been using Click-N-Type which is basically an on-screen keyboard with rudimentary word prediction. It’s questionable if this is faster or slower, but I can type for longer stretches since the on-screen keyboard takes less energy. Maybe this update will only take me a week.
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 segmenthas 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 ones
still 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 ground
unless 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 and
- 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.