Spasms and spasticity

Spasm and cramps often begin early in the progression of symptoms. Simple things like tonic water (gin is optional) can help but most PALS have to move to muscle relaxants such as Baclofen. I opted for a Baclofen implant which pumps the Baclofen into my spinal fluid. It was placed under my skin during a simple surgical procedure. It is about the size of a hockey puck. A catheter connects the implant to the spinal fluid, so the medicine bypasses vital organs and goes directly to where it is most needed. I have no discomfort from the device. The medicine gets topped off every six months. The doctor can change the rate of dosage by sending signals from his palm pilot to the computer in my implant. It works very well for me. I am loose as a goose.

I wear hand splints to keep my fingers from curling up and becoming locked. I wear heel boots to deal with toe drop and my twisted ankles. Massage gives short-term relief to spasms.

How are others coping? I hear that pills can have unpleasant side effects. Any experiences with this?

Transfer devices

The nature of transfers changes radically during the progression of ALS. Transfers can cause a lot of stress for patients and caregivers when they go awry. When manual transfers become impossible, there are devices which can deal with PALS up to and including quadriplegics. I chose the “easy pivot” device at first. I have now switched to using a hoyer lift. I made the transition for a number of reasons. First, the physical therapist observed me in a transfer and said that the easy pivot was no longer appropriate because the loss of muscles in my back made it much harder for the caregiver to get me into it because I was unable to assist her. The caregiver had to pull on my arms to get me into position, and that was dangerous for me because this can lead to painful dislocation of the shoulder. It was also dangerous for the caregiver, who began to have shoulder pains.

I’ve been using the hoyer lift now for a month and it is working out well. It is not as scary as it looks in the film clip provided by the easy pivot people. Advantages: it is easier for the caregiver to use, CNAs are more likely to know how to use a hoyer lift than an easy pivot so less training is needed, it is easy to position me on the commode or in my wheelchair, repositioning is much easier, and it makes it possible to get someone up from the floor. I keep the sling under me all day, so the caregiver just has to put the loops on the hoyer lift and I’m ready to go. My lift is hydraulic rather than battery operated, and that works for me (I weigh about 170). I would recommend getting an alternate sling so one can be washed while the other is being used.

My hoyer lift was provided by hospice. I suspect that Medicare would pay for most of the cost of one.

What works for you?

Communication Devices

I knew what was coming in this area and I did a lot of research on communication devices. The ALSA assistive technology person was very helpful. Working with the VA, where I got my medical care, I had the opportunity to try out various devices. The one I liked the most was the Vanguard Vantage System (http://www.aroga.com/Com_Aids/ca_products_detail.asp?Item%20ID=2101). It gives you the choice of both a picture-based overlay and a word-based overlay. I found the picture overlay to be counterintuitive. I am told it is better for children. They are wired differently than us senior citizens. I used the WordCore overlay but I found it was quite difficult to program. Once the data was in the machine, though, it worked very well.

I was lucky to have my device provided by the VA. Does anyone know if Medicare covers this machine?

Has anyone else researched or does anyone currently use this machine? Any other devices with which you have had success?

Feeding tube

I opted for a feeding tube well before I needed it. The interventional radiology approach seems to be preferable to the older PEG procedure. It was a simple outpatient procedure that worked well for me. I have heard horror stories about PEGs that ended up in the wrong places (e.g. the small intestine). I was told that I had to have the procedure done before my lung function dropped to 50% forced vital capacity (FVC). I’ve had the tube almost 2 years and am still eating soft foods. The tube is a great help in hydration. I also take some of my meds through the tube. It is important if you don’t use the tube regularly to flush it daily with a syringe. It’s important to flush it with water after each use. I have gotten conflicting advice about the frequency of changing the tube; some say every 6 months, others say whenever it malfunctions. I’ve had it changed 2 times in almost 2 years. The first time because the inflatable bulb inside burst.

I started out with what is called a G-J tube, as recommended by my gastroenterologist. He was concerned that later in the disease I might have acid reflux which could lead to aspiration. When they do the procedure they insert both G and J tubes in the stomach, then feed the J tube down into the jejunum. I haven’t had much acid reflux so with the last replacement I opted to have just a G tube. I’d love to hear from others about their experience with the J tube. I heard from one MD that it takes longer to feed through the J tube because the jejunum is smaller than the stomach. I was also told that an RN could do the replacement of a G tube. I’m glad I had my replacement done in the hospital because there were complications.

As I proceed through the list of things that I can no longer eat by mouth (peanuts, dry cereal, steak), I have surprisingly few regrets. I love good wine and a cold beer and still drink them. I will miss them greatly when I can no longer drink them orally, but I can put them through the tube…it’s a shame my stomach doesn’t have taste buds!

As always I’m interested in others sharing stories with best practices!

Hospice for PALS

I enrolled in hospice in March 2007 even though my breathing function was still fairly good because I strongly believe that the palliative care services they offer are a great comfort to ALS patients. People make a big mistake by viewing hospice as reserved for people in their last few months of life. The word hospice is highly emotive in our culture along with expressions like “dead man walking.” As a result, people often wait too long before enrolling. I think that’s a mistake.

Our local hospice provides a weekly visit from an RN; 3x weekly visits by a CNA who helps with my bathing; a monthly visit from a MD, PT, and social worker; and an occasionally visit from a chaplain. Hospice provides such supplies as bandages, enemas, chuks (paper sheets to sit on), some meds and equipment (hoyer lift, hospital bed, etc). Our hospice offers a 24-hr emergency number to call; it is staffed by an RN who can walk you through any situations that may arise after business hours. That's a great comfort for my wife. And Medicare covers hospice expenses, so we have no copay. The people have been absolutely wonderful and they are an important part of my support team. The last time the MD visited I noted that I had been with hospice for more than 6 months. I asked him how much “overtime” I would be allowed; he said I could be in hospice as long as I’d like to. Most people think of hospice as something for the last 6-12 months of life, but no one knows how long they have and, as long as your condition deteriorates, you continue to be eligible. PALS don’t always deteriorate at the same rate, but we all know that this is not a disease that you recover from.

Pets for PALS

Have you noticed that almost every edition of the NBC evening news has a “creature feature”? So why shouldn’t this blog have a creature feature also? Susan and I have a thing for orange tabbies. Tigger (pictured here) is our fourth. He is an 18-pound alpha male with a highly complex personality. I have always believed that somehow animals know when you’re sick and take steps to offer sympathy, consolation, and closeness. Tigger seems to think that it’s his job to defend me from all forces of darkness, including caregivers. He sits with me in my chair by day and sleeps with me by night with his front paw draped protectively over my leg. He gives me great comfort. I welcome interesting creature features from others in the ALS community.

The DNR Dilemna

Many of us, in putting our personal affairs in order after diagnosis, establish a living will and advance directives covering procedures that we may be confronted with. The Do Not Resuscitate (DNR) statement dictates what lifesaving measures may or may not be taken in an ambulance and/or hospital. Most PALS end up choosing not to undergo long-term mechanical ventilation (vent with tracheostomy). Every state has a different approach and language in the form that all of us should have displayed in our house describing our preferences. There is a high variability in the description of the various resuscitation measures (defibrillation, intubation, etc.). In Virginia, the form does not make a distinction between various types of resuscitation. The Catch-22 for me is that the way my DNR is written, I have to rule out all forms of resuscitation; this means that if I had a heart attack, the EMTs would not be allowed to defibrillate. If I opt for resuscitation, they can do any procedure they want. Research shows that about 5 % of PALS end up trached and attached toa vent; half of that group got their vents, without prior informed consent, when they went to the hospital for a serious medical condition.

I would very much like to hear from other PALS about whether or not they are aware of this dilemma. My hospice doctor tells me that the only way to avoid my dilemma is to not get in the ambulance or go to the hospital. Heart attack victims, for example, often get intubation as well as defibrillation.

More (gasp!) on bowel issues

Thanks for the good responses to my TNT recipe. I know that everybody’s situation down there is different; therefore I do not offer my solutions as a golden rule. As George Bernard Shaw said, “Do not do unto others as you would have them do unto you; their tastes may differ.” The same goes for bowels. I will visit this area again when I do my series on best practices. I know you’ll be waiting with bated breath!

Tony's TNT - The Recipe

Ingredients
1 cup raisins
1 cup pitted prunes
1 cup figs
1 cup dates
1 cup currants
1 cup prune concentrate (juice)

Combine contents together in food processor or blender to a thickened consistency. Store in the refrigerator between uses.

Administer 2 tablespoons twice a day (morning and evening). May increase or decrease dosage according to frequency of bowel movements.

Nutrition:
2 tablespoons
61 calories
137mg potassium
8 mg sodium
11.9 g sugar
0.5g protein
1.4g fiber

We add more prune juice to thin out the mixture and make it easier on the blender.

This recipe can not be used through a feeding tube. "Don't try this at home"

Source: The Journal of Geriatric Nursing. Volume 28, Number 2 Article titled" "Pilot Study of the feasibility and effectiveness of a natural laxative mixture."

Recently I have needed to supplement the TNT with a dulcolax suppository to get things moving. The TNT works from the top down and the dulcolax from the bottom up. A match made in heaven.

I'm interested in hearing what others have found effective. I'm especially curious to hear from those of you who rely entirely on a feeding tube.