Sunday, February 24, 2013

A video tour of the International Space Station

The video is a 25 minute tour of the International Space Station given by the departing commander. She was leaving for earth the day this is shot. She shows how they sleep, exercise, use the bathroom, eat, perform experiments, dock, get rid of their trash, arrive and depart, and a whole lot more. Seeing everyone floating around and the commander mimicking Superman was pretty cool.

Watching this video was an incredible experience. This is the best video I have seen and it visually explains what life is like living aboard the space station. If you are claustrophobic, you wouldn’t do well.  But, the idea of not having gravity holding you down is somewhat appealing for those of us living with Kennedy's Disease.

I highly recommend the video. So, sit back and relax and see what life is really like in space.

Thursday, February 21, 2013

KDA Research Grants Available

Every summer the Kennedy’s Disease Association begins accepting research grant proposals.  The actual grant(s) are awarded in the fall of each year.

Because the KDA is relatively small and funding is limited, our focus in recent years has been to provide “seed-money” to post-doc and other young researchers who do not currently have the funding or credentials to receive funding from larger organizations such as the National Institute of Health or the MDA. This “seed-money” normally provides the researcher an opportunity to further his/her research while giving him/her time to apply for other grants

In recent years, the awarding process takes place in the fall. In the late summer, the KDA announces to all known Kennedy’s Disease Researchers that anyone interested should send in their grant requests as outlined in the proposal notification.  The Scientific Review Board reviews all applications with a focus on research projects that are specific to or could be used in finding a treatment or cure for Kennedy’s Disease.  The Scientific Review Board recommends to the Board of Directors which applicant(s) should receive research funding.  The Board of Directors notifies all candidates and awards the grants normally in October.

2013 Research Grants
The Kennedy’s Disease Association (KDA) is planning to fund one or more research grants in the fall of 2013 to further the understanding of the pathological mechanisms of Kennedy’s Disease.  The KDA projects that funding for each grant will be up to $25,000 and, in certain circumstances, could be funded for a second year at a similar amount.  Applications from junior investigators and from senior post-doctoral fellows are encouraged.

Submission requirements, dates and proposal guidelines can be found in the attached PDF: 2013 Grants-PDF or WordDoc file: 2013 Grants-doc .  If you cannot open either of these files, please let the KDA know.

Sunday, February 10, 2013

Cancer Cells Committing Suicide

I read this short article in the “Big Think” and found it interesting. If this is successful in humans, it would be a major step forward in treating some cancers.

Every time I read about chemotherapy, I remember an original Star Trek episode where Bones is talking to Spock. He explains a barbaric torture that 20th century doctors used to treat cancer called chemotherapy.

Scientists Get Cancer Cells to Commit Suicide

by Orion Jones
What's the Latest Development?
Scientists have found a special molecule that activates the body's own tumor killing system, causing the death of cancerous tissue in mice while preserving the integrity of healthy cells. "The molecule, TIC10, activates the gene for a protein called TRAIL (tumor-necrosis-factor-related apoptosis-inducing ligand), which has long been a target for cancer researchers looking for drugs that would avoid the debilitating effects of conventional therapies." Experiments showed that TIC10 had potent effects against a variety of tumors, including breast, lymphatic, colon and lung cancer.

What's the Big Idea?
The TIC10 molecule has been shown to be especially effective at triggering cell suicide in gliobastoma, a kind of brain tumor that is notoriously difficult to treat. By activating the TRAIL gene in cancerous and healthy cells, cell suicide is induced in cancer cells immediately next to healthy ones. Wafik El-Deiry, an oncologist at Pennsylvania State University in Hershey and lead author of the study, said "TRAIL is a part of our immune system: all of us with functional immune systems use this molecule to keep tumors from forming or spreading, so boosting this will not be as toxic as chemotherapy."

Saturday, February 9, 2013

CPR – the rules have changed

Being a senior citizen, I had CPR training in the Boy Scouts, the U.S. Navy, and twice by the Red Cross sponsored by my employer. I was taught to use the “A-B-C” method (airway – breath – compression). In 2010, the process was changed. The new method is spelled out below. There are some videos available on the internet as well as Red Cross courses to train you should you wish to learn the new method.

Cardiopulmonary resuscitation (CPR) is an emergency procedure, performed in an effort to manually preserve intact brain function until further measures are taken to restore spontaneous blood circulation and breathing in a person in cardiac arrest. It is indicated in those who are unresponsive with no breathing or abnormal breathing, for example, agonal respirations.

CPR involves chest compressions at least 5 cm deep and at a rate of at least 100 per minute in an effort to create artificial circulation by manually pumping blood through the heart. In addition, the rescuer may provide breaths by either exhaling into the subject's mouth or nose or utilizing a device that pushes air into the subject's lungs. This process of externally providing ventilation is termed artificial respiration. Current recommendations place emphasis on high-quality chest compressions over artificial respiration; a simplified CPR method involving chest compressions only is recommended for untrained rescuers.

CPR alone is unlikely to restart the heart; its main purpose is to restore partial flow of oxygenated blood to the brain and heart. The objective is to delay tissue death and to extend the brief window of opportunity for a successful resuscitation without permanent brain damage. Administration of an electric shock to the subject's heart, termed defibrillation, is usually needed in order to restore a viable or "perfusing" heart rhythm. CPR is generally continued until the patient has a return of spontaneous circulation (ROSC) or is declared dead.


Used alone, CPR will result in few complete recoveries, and those who do survive often develop serious complications. Estimates vary, but many organizations stress that CPR does not "bring anyone back," it simply preserves the body for defibrillation and advanced life support. However, in the case of "non-shockable" rhythms such as Pulseless Electrical Activity (PEA), defibrillation is not indicated, and the importance of CPR rises. On average, only 5–10% of people who receive CPR survive. The purpose of CPR is not to "start" the heart, but rather to circulate oxygenated blood, and keep the brain alive until advanced care (especially defibrillation) can be initiated.

Studies have shown that immediate CPR followed by defibrillation within 3–5 minutes of sudden VF cardiac arrest improves survival. In cities such as Seattle where CPR training is widespread and defibrillation by EMS personnel follows quickly, the survival rate is about 30 percent. In cities such as New York, without those advantages, the survival rate is only 1–2 percent.

Compression-only CPR is less effective in children (ages 1-17) than in adults, as cardiac arrest in children is more likely to have a non-cardiac cause. CPR for children is different. Please follow this link for more information:


Remember the Basics

1. Call 911 or have someone else call for you
2. Ask someone to find an AED (automated external defibulator)
3. Start chest compressions by pushing hard and fast on the lower half of the breastbone. The rate should be at least 100 presses per minute and approximately 2 inches deep. The song “Stayin’ Alive” has about a 100 beats per minute. Keep up the compressions until the AED or emergency response team arrives.


Advice from the American Heart Association

  • Untrained. If you're not trained in CPR, then provide hands-only CPR. That means uninterrupted chest compressions of about 100 a minute until paramedics arrive (described in more detail below). You don't need to try rescue breathing.
  • Trained, and ready to go. If you're well trained and confident in your ability, begin with chest compressions instead of first checking the airway and doing rescue breathing. Start CPR with 30 chest compressions before checking the airway and giving rescue breaths.
  • Trained, but rusty. If you've previously received CPR training but you're not confident in your abilities, then just do chest compressions at a rate of about 100 a minute. (Details described below.)

If trained, practice the C-A-B Process:

The American Heart Association uses the acronym of CAB — circulation, airway, breathing — to help people remember the order to perform the steps of CPR.
Circulation: Restore blood circulation with chest compressions
  1. Put the person on his or her back on a firm surface.
  2. Kneel next to the person's neck and shoulders.
  3. Place the heel of one hand over the center of the person's chest, between the nipples. Place your other hand on top of the first hand. Keep your elbows straight and position your shoulders directly above your hands.
  4. Use your upper body weight (not just your arms) as you push straight down on (compress) the chest at least 2 inches (approximately 5 centimeters). Push hard at a rate of about 100 compressions a minute.
  5. If you haven't been trained in CPR, continue chest compressions until there are signs of movement or until emergency medical personnel take over. If you have been trained in CPR, go on to checking the airway and rescue breathing.
Airway: Clear the airway
  1. If you're trained in CPR and you've performed 30 chest compressions, open the person's airway using the head-tilt, chin-lift maneuver. Put your palm on the person's forehead and gently tilt the head back. Then with the other hand, gently lift the chin forward to open the airway.
  2. Check for normal breathing, taking no more than five or 10 seconds. Look for chest motion, listen for normal breath sounds, and feel for the person's breath on your cheek and ear. Gasping is not considered to be normal breathing. If the person isn't breathing normally and you are trained in CPR, begin mouth-to-mouth breathing. If you believe the person is unconscious from a heart attack and you haven't been trained in emergency procedures, skip mouth-to-mouth rescue breathing and continue chest compressions.
Breathing: Breathe for the person
Rescue breathing can be mouth-to-mouth breathing or mouth-to-nose breathing if the mouth is seriously injured or can't be opened.
  1. With the airway open (using the head-tilt, chin-lift maneuver), pinch the nostrils shut for mouth-to-mouth breathing and cover the person's mouth with yours, making a seal.
  2. Prepare to give two rescue breaths. Give the first rescue breath — lasting one second — and watch to see if the chest rises. If it does rise, give the second breath. If the chest doesn't rise, repeat the head-tilt, chin-lift maneuver and then give the second breath. Thirty chest compressions followed by two rescue breaths is considered one cycle.
  3. Resume chest compressions to restore circulation.
  4. If the person has not begun moving after five cycles (about two minutes) and an automatic external defibrillator (AED) is available, apply it and follow the prompts. Administer one shock, then resume CPR — starting with chest compressions — for two more minutes before administering a second shock. If you're not trained to use an AED, a 911 or other emergency medical operator may be able to guide you in its use. Use pediatric pads, if available, for children ages 1 through 8. Do not use an AED for babies younger than age 1. If an AED isn't available, go to step 5 below.
  5. Continue CPR until there are signs of movement or emergency medical personnel take over.


Thursday, February 7, 2013

Oh boy, let’s eat some Soy!

Ed Meyertholen, Ph.D., our new KDA President KDA wrote the following report on a recent study. These are Ed’s comments on the research report including the potential viability of the treatment for those of us living with Kennedy's Disease.

Genistein, a natural product derived from soybeans, ameliorates polyglutamine-mediated motor neuron disease.
Research Report from Sobue’s group in Japan

soybeansEssentially, genistein is a substance found in soy and may be able to reduce the symptoms of Kennedy's Disease (KD).  This is due to the property of genistein to disrupt the association of the androgen receptor with another protein known as ARA70.  This allows the cell to remove the AR more efficiently and this, it is argued, may help relieve symptoms of KD (aka SBMA or Spinal Bulbar Muscular Atrophy).

The group did two major sets of experiments, one in cell models and one in a mouse model of KD. 

The results suggest that genistein reduces the symptoms of KD. 

Now, before we all rush out to buy soy products, understand that:
  • Mice were treated with genistein at 6 weeks, two weeks BEFORE symptoms were found to be expressed in untreated KD mice.  They did NOT test genistein in mice that already were showing symptoms.
  • The genestein did not eliminate the symptoms, it did seem to slow down the progression.  Also, it is not known if it would slow down progression after the onset of symptoms – as stated previously, this experiment was not done.  It is very unlikely that it would reverse symptoms that have already occurred.
  • The dose of genestein in the study used was 250 mg/kg/day of mouse.  This would mean a typical 70 kg (155 lb.) man would have to ingest 250*70 = 17500 mg of genistein/day.  From what I could find, soy powder has the highest concentration of genestein of all soy products; it has a concentration of 1 mg/g of soy powder.  Thus, to take in the same amount of genestein as the mice in this experiment, one would have to eat 17500 g of soy power per day – this is about 38 pounds!  You cannot eat enough soy powder daily to get the small change in symptoms seen in the mice.  If one buys the ‘purified’ (it is really just enriched) genistein, it comes in 1g tablets – one would have to take more than 17 every day – and the safe recommended dose is 2 tablets/day.   Genistein has side effects – talk to your doctor!
  • Just because genistein shows positive effects in mice does not mean it will have that effect in humans.
Conclusion: More work needs to be done before getting excited and wasting money on genistein.

Sunday, February 3, 2013

Evaluating your mobility needs

wheelchairAt some point when living with Kennedy's Disease, the time might come when you have to consider a wheelchair.  I would recommend that you take more time researching the type of chairs, configurations and options available than when you buy a car. Why … because at some point you will spend more time in the chair than you do in your car.

It is also important to consider your current and future needs. The worst thing you can do is purchase a chair based upon your current needs and find out in three-to-five years that the chair no longer satisfies your mobility needs. It becomes very expensive to have to buy another chair in a couple of years because it no longer supports your mobility needs.

I have written about the importance of seeing a ‘seating specialist’ to help evaluate your current and future needs and also show you, and allow you to test drive, the options available.

wheelchair-2Another KD’er told me about this website that has helpful information, videos and other considerations for selecting the correct wheelchair (manual or power). I perused through the site and found the videos and comparisons quite helpful. The videos help you see a chair being used and its potential limitations.
Other articles that might be of help: