Who needs a gnome...?

Nov. 16th 2012 @ 1:41 PM

can't recall if we had any classes together, or if we ever even spoke more than a few greetings passing in the halls between classes. I do know we shared a few mutual friends, and believe she was actually friends with my high school sweetheart who also later became my first wife, Debbie. I'd had way too much fun in high school to be bothered with the likes of grades or the SAT. I honestly have no idea how I graduated on-time. I'd been thrown out of classes, summer school (who knew bottle rockets weren't allowed on the smoking patio?) and football games (smoke bomb thrown onto the field). We go through high school and on our separate ways without a thought for how our paths may intersect down the road. She went off to college, followed by, more than likely, starting a family, career and everything in between, living the "American Dream". Me, on the other hand, my saving grace was in the form of the USAF.

Fast-forward three decades, give or take; fortunately, and seemingly miraculously, we're able to reconnect with friends and acquaintances from way back simply by typing their name in a little box. (helps if they're on facebook) If you had asked me back in the day in the halls of Reid Ross who I'd thought would be there for me after all these years...well, it wasn't her.

Valerie Smith Potter is that person, in many ways. Even though she suffers from her own debilitating condition, she and her husband Clarence have gone out of their way to show their support and spread the word far and wide, advocating for my own cause and health crisis, ALS. Also known as Lou Gehrig's disease, ALS has taken many things from me, including many friends I've been fortunate to make both online and off, but it cannot stop those unexpected friends, new and old, from following their heart and making a difference, distributing hope in the form of smiles, prayer, visits and... well, I think the pictures do tell the rest of the story better than I could ever dream to.

Open and closed doors

September 13 2012 at 11:35 AM

y journey with ALS, which has progressed at a snail's pace, began with symptom-onset in late 2001/early 2002. Some see such a slow progressing "terminal" illness as a blessing, others, a curse. I've always been the glass-half-full type, and ALS lead me back to my spiritual walk with Jesus Christ; I believe He has plans for me, a purpose behind the struggle. My decision to go on a ventilator was made long ago, with a certain naivete' even, having yet to meet another vented PALS. I've known many online, but until you meet one in person, spend time with them, you can't really grasp what it's going to be like. My decision has always been based on seeing my boys grow up and get established in the world. Also, had I chosen otherwise, I don't know if my good fortune of meeting and marrying Cindy, my love, my backbone and my courage, would have come to pass. Not to mention two of the most awesome, beautiful stepdaughters, Hillary and Maura, double bonus! Boy, did I have alot to learn about teen (and beyond) girls.

Back to the subject at hand. I'd hoped to qualify for the diaphragm pacer, but unfortunately, my fvc dropped around 20% over the last two years, all the while knowing my breathing had become a little more labored, yet unaware just how bad it was. By the time Cindy and I decided to go back to Duke ALS clinic for an overall assessment as well as an EMG of my diaphragm, my fvc had dropped to the high 20% range, but my diaphragm was still showing some promising data, it was still helping me breathe on my own. Dr. Bedlack told us that Duke was starting the pacer implants, but my fvc was just too low to risk it. After consulting with the pulmonologist, we decided it was time for the vent.

All this happened so fast, and before we knew it, we were on the way to Duke Hospital for the procedure, which took place July 6th, bedside in the ICU, apparently a common practice there. The anesthetic they use for PALS is a shorter lasting one, so before I knew it, I was half awake, seeing Cindy and the girls standing over me with worried looks on their faces. Can't remember much about that night, other than overhearing the 7PM shift turnover, and the nurse's inaccurate account of my ALS history. I transferred to the respiratory/transplant ward the next day.

I was at this point on big hospital vents, and bagged for the transfer. Once I was settled in the room where Cindy and I were to end up spending the next 18 days or so, we learned that Duke was considering transferring me to the VA hospital across the street, as the VA was to provide my home vents, but they weren't willing to bring someone over to Duke Hospital to train the respiratory techs on a vent they were unfamiliar with. There was also an incompatibility issue between the VA's vents and Duke's; the Duke vents were tied in to a pager system, where the r/t would get paged if there was a problem with a vent patient. This was an unexpected mess we didn't need, so we began coordinating plans for our home health agency, PHIcare.com, to bring in their portable vent rep out of Charlotte, who would bring me a Trilogy 100, one of the smaller, more compact portable ventilators available. I believe this took place on day 3 or 4.

I feel it's important to explain that Duke, like any other teaching hospital, will have some good and bad differences as opposed to non-teaching hospitals. Morning rounds will include the primary pulmo doc, a resident, different “fellows”, director of nursing, a social worker, and unnumbered various med students in different years of school, some appearing way too young for med school. It's like the room is a phone booth, and the students are trying to see how many they can pack in. So while the primary, teaching physician listens to your breathing on one side of the bed, his resident will be doing the same from the other side while the rest of the “posse” looks on, or takes turns with the stethescope. I must say at this point that most of the ward staff are very professional and put you at ease through their genuine concern for your welfare. Then there's the few who have no bedside manner, or qualms about waking you at 4AM for blood work or just vital signs. They turn on the brightest light, don't tell you what they're doing, and never crack a smile in the process. Very unnerving.

Before the procedure, I was only using the BiPAP when in the bed, or on road trips. I know we made it very clear that my intention was to do the same with the vent, but driving that point home to the medical team was not easy. What was easy was staying in the bed, which meant staying on the vent. We had to push the issue to even get me out of the bed and into my chair, and we had to wait until PT come in to assist with that, as if Cindy had never transferred me to my chair from the bed before. Sure, I was weak from being in the bed for days, but I quickly regained my former leg strength for transfers. Once I'd made it to my chair, we could start “trach trials”, which is when they remove the vent circuit and put a cool-mist blowing collar over the trach to keep it from drying out. I'm actually wearing one now while I write this. A visit to my local VA hospital last Thursday got me a Passy-Muir (speaking) valve and a change in trach size. The PMV also allows me to drink my coffee and sweet tea, and try soups etc. by mouth, which I have yet to do. The PMV is simply a one-way valve on the trach that only lets air in, then forces the exhale out the mouth and nose, past the vocal cords. So it took from the day of the procedure until last Thursday, almost two months, for me to be able to talk (voice is better than it was before) and have anything by mouth. This is only when the air has been let out of the cuff. I tried doing what they call “leak speech” (they let just a little air out of the cuff to see if you can speak with a little of the vent air escaping past the vocal cords) with the speech path in the hospital, but she couldn't get in to see me until the day of discharge, and all I cared about then was getting out of that place. Wasn't too fond of the sensation from that.

Trachs typically have to be changed every month. When in the hospital, the initial trach will be stitched in place to facilitate proper healing, less aggravation etc. This first trach is typically removed and replaced after the first week. Mine was day 10 I believe, July 16th. I should have had another change around August 16th., but when my home health nurse attempted it, we discovered it had adhered to my trachial wall, so she stopped as soon as she felt resistance. Had to go to the local VA to have the pulmo change that one, a somewhat unpleasant experience; hurt coming out, and Dr. Frankenstein (had to be every bit of 75) shoved the replacement in with every bit of care as he used taking out the stuck one, none. Cindy about had a conniption when she watched the RN pull the new trach out of the box and packaging without gloves, and we never saw her wash her hands. The doc was wearing gloves. The downsize on Thursday was unexpected, done after the VA speech path appt for the PMV and swallow study (not barium, just water with green food coloring to see if I would aspirate any out the trach). Seems the medical standard is to put in a size 8 Shiley trach, and change it in size or type as necessary when changed monthly.

I'd heard a lot about trachs, and devoured about everything I could find online about getting a trach, the care required etc. However, I was pretty much caught off-guard with the suctioning. Part of my ALS symptoms is my hyper reflexes, and the only way I can describe the feeling of having the suction tube poking around inside my lungs is that I feel like an inmate in an electric chair when the switch is thrown; eyes and jaw clenched, tongue-biting and completely out of control of my actions from the waist up. Pretty unpleasant, but I don't think that's the case for all vented PALS, that's just my hyper reflexes, having lasted this long with ALS.

The hospital will make a pretty big deal out of ensuring you have an adequate support and care system in place before they'll even do the procedure, or before discharge from the hospital. Cindy being an RN, and doing most of my care in the hospital herself, put them at ease concerning training the family members etc.

Final thoughts, I was fortunate to have my eyegaze in the hospital for communication, but I also highly recommend you have an established method of communication with your family caregiver(s) that doesn't require referencing a talking board you have to dig up every time you want to communicate when the eyegaze isn't available or practical, like in the ICU.

Mega-update

August 17 2012 at 2:00 PM

ell, the challenges of life with ALS just may have gotten the better of me for this last 11 months. Seems like the BCI study was years back. I was so excited to be a part of that study, but ended up dropping out after Cindy and I realized the time and effort needed for the study, along with my frustration from difficulty I had with consistent accuracy proved too much. I felt bad for dropping out for a while after, but the VA project rep we came to know pretty well during the study came to see us at Duke after the tracheotomy. From what she said, my experience with it was much the same with the other participants she worked with.

That was last September/October, and I don't even know where to start with everything since then. Maura finished her senior year at Jack Britt in January, Hillary finished her esthetics course at FTCC and passed her state certifications with flying colors. She starts a new job next week at a medical spa. Maura leaves in the morning for her freshman year at Western Carolina in Cullowhee. Shane has been working the security firm at the mall, had hopes for a position at the iphone store, but I guess they pulled the rug out from under that hope. He's done alot of talking about going to truck driving school. Scary thought, alot of responsibility, but you just have to support em no matter what. Vance has left the beach life of throwing pizzas and landscaping, and just arrived yesterday in Florida, Tampa area, to help a friend with a trailer park maintenance job. May the good Lord watch over them all!

The biggest news for us Re: ALS is that my respiratory condition snuck up on us and took me out of contention for the diaphragm pacer. Went about two years without a good fvc test, and the change, with my slow progression, has been almost unnoticeable. Around the early part of the year, I began to feel more tired all the time, taking longer naps etc, but didn't really link it to reduced vital capacity. Cindy and I knew I was overdue an updated fvc test, and had found out that Duke was going to start the diaphragm pacer implants very soon. However, I missed the bus on it altogether. One of the requirements for approval for the implant is for a minimum fvc of 45%. Two years ago I was right around 50%. I'm sure you can see where this is going; I had a tracheostomy done at Duke Hospital July 6th. I spent 18 days there, and Cindy never left my side more than the time it took her to go down to the cafeteria or check on the van across the street at the VA parking deck. You know those hospital room chairs they have for family etc. that recline? She wouldn't even recline when she slept in it to be able to keep an eye on me. That was the longest in-patient hospital stay ever for me. I know it could've been worse, but I was more than ready to get out of there. I have to give credit to staff of the ward I was in, 7800, which is the respiratory and transplant ward. So many caring people, going above and beyond. God bless all nurses!