An interesting week (and somewhat worrisome)

Last week was interesting for me. Since beginning my own dutasteride trial, it was the first time in eighteen months that I felt any real weakness. The following is a summary of my daily journal for this unusual episode.

Day 1: I noticed a slight weakness in the legs when getting up and transferring. Then when I exercised, the leg extensions were very difficult. Everything else was okay, but not easy. The rest of the day the weakness was noticeable.

Day 2: I noticed more weakness in the legs and also the arms. I was experiencing some muscle tightness.

Day 3: Legs and arms are weaker. The entire exercise program was more difficult. The rest of the day I struggled far more than normal (the last 18 months).

Day 4: Still noticing the weakness. I am beginning to have some concerns. What is going on?

Day 5: Not quite as weak today. My exercise routine went okay. This is positive. Let’s hope I am on the mend again.

Day 6: Weakness hardly noticeable.

Day 7: Everything seems fine again. Full strength, no problems with exercises, no noticeable weakness in other activities. Still have some muscle tightness after exercising, however.

Day 8: Everything is fine once again.

I am taking this one day at a time again. I became so used to good day after good day, I forgot what it was like to have ‘weak’ days. Life has been pretty good since starting the dutasteride trial. The development is interesting and I hope not a trend.  I have enjoyed the ride.

Human Muscle Regrown

As soon as I saw the New York Times’ article by Henry Fountain, “Human Muscle, Regrown on Animal Scaffolding,” I had to read it. The two page article was focused on how this new technology appears to be working for some soldiers returning from the war that need rehabilitation. However, the more I read the more I wondered if this might be something available for those of us living with Kennedy’s Disease.

Excerpts from the article follow, but you can follow the above link to read the entire article.

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PITTSBURGH — In the months after a roadside bomb in Afghanistan blew off part of his left thigh, Sgt. Ron Strang wondered if he would ever be able to walk normally again.
 
The explosion and subsequent rounds of surgery left Sergeant Strang, 28, a Marine, with a huge divot in his upper thigh where the quadriceps muscle had been. He could move the leg backward, but with so much of the muscle gone he could not kick it forward. He could walk, but only awkwardly. 
 
“I got really good at falling,” he said of his efforts. And Sergeant Strang, a tall, athletic man, had to give up running. 
 
But that was two years ago. Now he walks easily, can run on a treadmill and is thinking of a post-military career as a police officer. “If you know me, or know to look for it, you can see a slight limp,” he said. “But everybody else, they go, ‘I would never have guessed.’ ” 
 
There is something else they would never have guessed: Sergeant Strang has grown new muscle thanks to a thin sheet of material from a pig. 
 
The material, called extracellular matrix, is the natural scaffolding that underlies all tissues and organs, in people as well as animals. It is produced by cells, and for years scientists thought that its main role was to hold them in their proper position. 
 
But researchers now know that this scaffolding also signals the body to grow and repair those tissues and organs. Armed with that knowledge, the new body builders are using this material from pigs and other animals to engineer the growth of replacement tissue in humans. 
 
The technique used on Sergeant Strang, though still in development, holds particular promise for some of the thousands of veterans of the Iraq and Afghanistan wars who have been maimed by explosives and have lost so much muscle from an arm or a leg that amputation is sometimes the best alternative.

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Later the article further explained the process and got into more specifics.

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The scaffolding is isolated by stripping out all of the living cells from a tissue or organ, leaving an intricate three-dimensional web of proteins and other compounds. Removing the cells eliminates the possibility that the material, of animal origin, will be rejected outright by the body when it is implanted. But the matrix does provoke a less intense immune response, Dr. Badylak said, which is necessary for it to work. “You actually need the immune system to recognize the material.” 
 
“The body can say, ‘This is not me,’ but the signals that are there are actually telling me that I need to rebuild that tissue,” he added. 
 
The matrix has to be in contact with healthy tissue, which is why scar tissue must be removed first. “If it’s put in the middle of a scar, it doesn’t remodel because it’s not exposed to the bloodstream and sources of cells,” Dr. Badylak said. 
 
Stitching it directly to the muscle also ensures that it will be stretched when the muscle is activated, beginning the day after surgery, when patients start an intensive program of physical therapy. Putting a mechanical load on the matrix tells the body, in effect, that this should become muscle and not some other kind of tissue. 
 
Sergeant Strang was skeptical when he first signed up for the trial and cleared all the physical hurdles to qualify for it — among other things, patients must have some muscle remaining and enough undamaged nerves so the muscle can work. But at that point he was willing to try anything to be able to walk normally again.
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Well, after reading the article, I had to contact our resident biology professor and ask if this process might be applicable for someone with Kennedy’s Disease. Ed’s response was:
 
“It could, it would appear to depend on if there were any neurons left.  The common belief is the muscles atrophy is due to the neurons dying.  If there were no motor neurons, then it would seem that the muscle would not grow (note the need for the presence of muscle and nerves in the patient to be eligible for the procedure).”
 
Well, that wasn’t a definite “NO” anyway.

New Breed of Robotics Aims to Help People Walk Again

As you already know, I am an advocate of technology. I believe that in the not too distant future, robotics will be an efficient way to walk and will also be cost effective. Today’s article in the New York Times is another view into the future of robotics … and for those of us living with Kennedy’s Disease, hopefully our future.

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By Brian X. Chen                    Published: September 11, 2012 

RICHMOND, Calif. — When Joey Abicca pokes a metal crutch into the ground with his right arm, tiny motors start whirling around his left leg, lifting it and moving it forward. When he does the same with his left arm, the motors whir to life again and his right leg takes a step. The metallic whine is like something out of the movie “RoboCop.”

Mr. Abicca, a 17-year-old from San Diego, is essentially wearing a robot. His bionic suit consists of a pair of mechanical braces wrapped around his legs and electric muscles that do much of the work of walking. It is controlled by a computer on his back and a pair of crutches held in his arms that look like futuristic ski poles.

Since an accident involving earth-moving equipment three years ago that damaged his spinal cord, Mr. Abicca has been unable to walk on his own. The suit, made by a company called Ekso Bionics, is an effort to change that.

“It’s awesome — I love getting back up,” Mr. Abicca said before strapping on the legs during his recent visit to the company’s headquarters here. “Even just standing up straight is awesome.”
Ekso is one of several companies and research labs that are working on wearable robots made to help disabled people or to make the human body superhuman. In 2010, Raytheon released a suit for soldiers that is designed to reduce injuries from heavy lifting. And in Israel, a company called Argo Medical Technologies also makes a robotic suit to help paraplegics walk again.

Ekso says it was the first company to introduce a self-contained robotic suit, without any tethers to, say, a power supply. And though its suits for the disabled are now used only in rehabilitation centers, it is looking ahead to a day when they will let people take to the sidewalks, the shopping malls — and maybe even the woods.

Ekso, which was founded seven years ago by engineers in Berkeley, Calif., takes its name from the word exoskeleton, meaning a skeleton that is on the outside of the body. Originally financed by the military, the company collaborated with the University of California, Berkeley, and the military contractor Lockheed Martin on a device called the Hulc, which allows soldiers to carry up to 200 pounds of equipment over mixed terrain.

In February, Ekso started shipping exoskeletons that are being used in physical therapy to get people out of wheelchairs and using their lower bodies so their muscles do not deteriorate. About 15 rehabilitation centers in the United States are using the suits; they pay $140,000 for each one, along with a $10,000 annual service contract.

With a frame of aluminum and titanium, the bionic suit, called the Ekso, is battery-powered and weighs about 50 pounds. The suit is not yet at the point where a disabled person can use it independently. The batteries last three hours, at which point a physical therapist needs to replace them. Supervision also ensures that a patient does not fall over; the company said hundreds of people have walked in the suit, and none had fallen.

The Ekso suit is already going beyond just helping people walk again. The latest version released last month includes walking modes with different difficulty levels to challenge patients to make progress in their rehabilitation.

In the first mode, when a patient is first learning to walk with the suit, a physical therapist sets the step length and speed and presses a button on a computer to trigger each step. In the second mode, the patient can trigger a step with buttons on the crutches. And in the third, most advanced mode, once the patient has learned to maintain her balance in the suit, she can trigger the suit to take a step just by shifting her weight.

Patients learn to walk in the robotic suits surprisingly quickly, said Eythor Bender, chief executive of Ekso Bionics, who previously worked at Ossur, a company that made artificial limbs. “People who come in haven’t walked for years and years,” he said in an interview. “They are walking on their own in two days.”

Yoky Matsuoka, the former head of innovation at Google and now vice president for technology at Nest, which makes a smart thermostat, said the time was right for exoskeletons to graduate from science-fiction fantasy to commercial reality. Battery technology has improved significantly, materials like plastics and carbon fibers have gotten more lightweight and durable, and robotic systems have become easier to control, she said.

“In the last 10 years, the evolution of some of those materials and some technologies allows us to make robots that really stay human-safe and human-friendly,” Ms. Matsuoka said.
However, the cost of such devices for medical use could still be an obstacle, she said, because such specialized equipment sells in smaller quantities, making it difficult to bring the price down. She said that wider use by the military could help.

At some point, the Ekso suit may have to clear some regulatory hurdles. The current version of the suit is exempt from regulation, but if the company introduced one for personal use at home, it would probably have to gain approval from the Food and Drug Administration, said John Tugwell, director of regulatory affairs at Ekso.

Ekso is hoping that the suits will, in the next few years, really start to go places.
Russ Angold, a founder and the chief technology officer of the company, predicted that exoskeletons, like today’s smartphones, would slim down and get more powerful and affordable, becoming part of everyday life.

“The dream at the end of the day is be able to walk into a sporting goods store, like an REI, and pick up an exoskeleton,” Mr. Angold said. “They’re like the jeans of the future.”

IGF-1 Continues to Show Promise

GOOD NEWS on the Research Front

 

MDA’s Quest Magazine just published an update on IGF-1. Maria Pennuto, a previous recipient of a KDA Research Grant and MDA funding, provided the following update to reporter Amy Madsen.

I have written about IGF-1 in several previous posts because of its potential. We hope to hear more promising news at the upcoming KDA Conference and Educational Symposium the second week of October in New Orleans.

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SUMMARY

• Mice with a disease resembling spinal-bulbar muscular atrophy (SBMA) that were treated with an insulin-like growth factor 1-based compound had better motor function, slower weight loss, improved muscle health and increased survival time.
• Recent studies have shown that IGF1 works in SBMA by reducing toxicity caused by a mutation in the androgen receptor (AR) gene; it also helps promote muscle and nerve growth.
• The new findings suggest that IGF1 potentially may be effective in ameliorating the disease.

Mice with a disease resembling spinal-bulbar muscular atrophy (SBMA, or Kennedy’s disease) that were treated with a compound based on insulin-like growth factor 1 (IGF1) had better motor function, slower weight loss, healthier muscles and longer survival time than mice that received an inactive substance, an MDA-supported research team has reported.

The research team also found that SBMA mice that overproduce a muscle-specific form of IGF1 have a milder disease course than mice that produce less of the protein.

The new findings suggest that IGF1 potentially may be effective in ameliorating the disease via at least two mechanisms:

• directly reducing toxicity caused by a mutation in the androgen receptor (AR) gene by changing the way the androgen receptor protein behaves; and
• generally promoting and regulating skeletal muscle growth, and improving survival of muscle-controlling nerve cells called motor neurons.

The research team reported its findings online Aug. 29, 2012, in Molecular Medicine. To read the full report, see IGF-1 Administration Ameliorates Disease Manifestations in a Mouse Model of Spinal and Bulbar Muscular Atrophy. MDA supported Maria Pennuto at the Italian Institute of Technology in Genoa, Italy.

Findings show IGF1 compounds hold therapeutic potential
 
The researchers treated SBMA mice with a compound called mecasermin rinfabate (brand name Iplex), which is a combination of IGF1 and IGF1 binding protein 3. (The binding protein helps IGF1 last longer in the body.)

Male mice were randomly assigned to receive abdominal injections of the IGF1-based compound at a daily dose of 15 milligrams per kilogram of body weight, or an inactive substance. To mimic the typical clinical setting (in which a diagnosis of SBMA occurs after symptoms appear) the researchers began injections when the mice were 10 weeks old, after disease onset, and continued through 20 weeks.

The researchers evaluated body weight, motor function and survival in the mice. They found that, when compared to the placebo-treated mice, those treated with IGF1 had:

• less body weight loss;
• significantly improved grip strength; and
• longer survival time (by an average of approximately three weeks).

In addition, the researchers found that the IGF1-treated mice had markedly reduced muscle shrinkage (atrophy) and degeneration.

The study results provide a basis for further study of IGF1-based compounds as potential therapies for people with SBMA.

The Contented Cow

There is an old saying, “A contented cow gives better milk.” On the farm, it was definitely true and as a kid I learned early on the importance of taking care of our milkers.

The dictionary defines “Contented” as:

• Satisfied or accepting of the way things are
• Feeling comfortable with your situation in life

This morning when my dog and I were on our early morning walk I felt contented. I was not thinking about the past or concerned about the future. I was living in the moment and enjoying every minute of it.

For a most of the walk I focused on this feeling (enjoying the moment). At some point I began to understand that contentment had nothing to do with “being happy”, but had everything to do with being comfortable with (accepting of) my life.

Then I asked myself, “Why did I feel this way?” I realized that it was probably because of what happened the previous day.

• I spent most of the day with my wife … shopping, working on a project, and going to a birthday party.
• Spending several hours of quality time with family.
• Watching family videos that were 20+ years old.
• Not spending any time on editing my novel or performing any KDA work.

If the contented cow gives better milk, then a contented person is an accepting and loving beneficial presence in this world. There is not any attitude or issues. There is only living in the moment.

It is now ten hours later and I still have that feeling. Life is good and I feel loved.

How to handle Stress

I receive this email from my brother today and it came at a good time.  Yes, it is simple, common sense advice, but it never hurts to be reminded once in a while.

Those of us living with Kennedy's Disease know how stress can exacerbate our symptoms.  We need to be aware of our tolerance for stress and act accordingly. 

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A young lady confidently walked around the room while explaining stress management to an audience. As she raised a glass of water, everyone was sure she was going to ask the ultimate question: 'Half empty or half full?'

However, she fooled them all.

"How heavy is this glass of water?" she inquired with a smile.

Answers called out ranged from 8 oz. to 20 oz.

She replied, "The absolute weight doesn't matter. It depends on how long I hold it. If I hold it for a minute, that's not a problem. If I hold it for an hour, I'll have an ache in my right arm. If I hold it for a day, you'll have to call an ambulance. In each case it's the same weight, but the longer I hold it, the heavier it becomes."

She continued, "That's the way it is with stress. If we carry our burdens with us all the time, sooner or later, as the burden becomes increasingly heavy, we won't be able to carry on." 

"As with the glass of water, we have to put stress aside for a while and rest before picking it up again. So, as early in the evening as you can, put all your worries aside and focus on your family and friends. Don't carry the stress around with you through the evening and into the night. Then when we're refreshed, we will find it easier to carry the burden.

Why am I not posting as often?

I have been negligent in publishing articles recently.  My three articles a week goal has suffered since I began editing my book.

Several years ago I wrote a novel.  It is almost 100,000 words.  When I wrote the last chapter I needed a break and put it on a shelf in the closet.  I didn’t look at it again until late last year. 

I joined the Chattanooga Writers Guild a little earlier that year and realized it was time to dust off the novel and edit it.  Since the beginning of this year I have edited two chapters a month.  I am now 45% through with the edit and these edits have been reviewed by certain members of the Guild. 

Editing is hard work and very time consuming.  Some chapters have taken up to a week to rewrite and edit.  Then, after the chapter is reviewed, it takes another couple of hours to make other recommended changes.  Combine these activities with my KDA responsibilities and other normal life activities, I haven’t focused on the web blog as much as I would like.

When I started this blog, I wanted to focus on quality and not quantity.  I’ll try to do better in the months to come, but my priorities, at least for the moment, are still elsewhere.  In the meantime, you might want to read some of my earlier articles if you only started reading this blog in the last year or so.  There are a lot of good articles on a variety of subjects including some on my personal battle with Kennedy’s Disease.

And, I promise you that if anything of interest pops up, I will still be posting new articles … just not as many for the time being.