Friday, August 24, 2012

Preparing for Emergencies

emergency preparedness
MDA’s Health Care Services had an interesting article on emergency preparedness that everyone should read whether disabled or not. Several of the links mentioned in the article are shown below.

The federal government has a website with additional information on different types of emergencies and services available for your area. The link to the website is:'s Emergency Preparedness Section.
emergency-kitThe MDA has put together a good checklist for people who have neuromuscular diseases. Not every item applies to those of us living with Kennedy’s Disease, but many apply to anyone with special needs or considerations. The checklist can be downloaded (PDF) at this link: PDF Checklist.
Another good link to always have bookmarked is the National Weather Service.  You can zoom in on your area and receive the most current updates and forecasts along with other helpful information.

If you must evacuate your residence and need MDA services, or information about local groups offering emergency assistance, you can reach the MDA by calling (800) 572-1717 or emailing They will connect you to the MDA office serving the area in which you've sought shelter.

As the Boy Scouts always say, BE PREPARED.

Tuesday, August 21, 2012

New Funding for Kennedy’s Disease Research

The MDA announced today that they are providing funds for Kennedy’s Disease research. The recipient is Dr. Andrew Lieberman of the University of Michigan.

Dr. Lieberman is a member of the Scientific Review Board of the Kennedy’s Disease Association (KDA).  He also supports the KDA and me personally in many ways.

Andy, congratulations!
The MDA announcement is shown below:


Andrew Lieberman, M.D., Ph.D.
University of Michigan

Lieberman and colleagues have identified a small molecule in cell models and in research mice that may help degrade the mutant protein that underlies SMBA and reduce the severity of the disease in people. Click to enlarge.
Andrew Lieberman, assistant professor of pathology at the University of Michigan Medical School in Ann Arbor, was awarded an MDA research grant totaling $405,000 over three years to study a new therapy approach for spinal-bulbar muscular atrophy (SBMA).
SBMA is an inherited degenerative disorder of lower motor neurons (nerve cells) that is caused by a mutation in the androgen receptor (AR) gene. The mutant protein causes toxicity associated with the male hormone testosterone, resulting in muscle weakness and atrophy in men.
Previous work established that the mutant AR protein is the cause of this toxicity, suggesting that strategies aimed at more efficient degradation of the protein should diminish disease severity.
Lieberman and colleagues found that degradation of the AR protein is tightly controlled by cellular machinery consisting of the heat shock protein 70 (Hsp70).
The investigators now are testing genetic and pharmacologic (drug-related) methods to see whether increasing the stability of Hsp70 will help it more efficiently bind the mutant AR protein and promote its degradation.
“It is our expectation that this work will help define a new therapeutic approach to SBMA and other protein aggregation disorders where degradation of the mutant protein is controlled by Hsp70,” Lieberman said.
Funding for this MDA grant began Aug. 1, 2012.

Sunday, August 19, 2012

A “what-if” I hope you never have to experience

tornadoUp until a couple of years ago, I wouldn’t have even considered the need for the below information. Since then, however, we had a series of tornados come through our area and one came within a couple of hundred yards of the house. Dozens of homes were damaged and several were destroyed on our road back in March 2011. Many neighbors ended up in emergency shelters.

We also had a forest fire that came very close to our house. With all this happening, we now feel more vulnerable.
  • What if we lost our home?
  • What if our home was damaged to the point we couldn’t safely live there?
  • What if we were forced to evacuate our home?
  • What if we lost power or other services (water and gas) for more than a day or two?
Since these occurrences, we have been doing a little more ‘what-if’ planning. Two questions, however, were not resolved.

  1. What if we were forced to evacuate our home?
  2. How would we manage with my disability? 

MDA’s Quest Magazine has an interesting article on this subject.emergency-shelter

A new federal program provides personal care aides for individuals with disabilities stranded in emergency shelters during a declared disaster

Article Highlights:
  • The Federal Emergency Management Administration (FEMA) has contracted with two national agencies to provide personal assistance services (PAS) in emergency shelters to individuals with accessibility and functional needs. Federal services are only available if the president has declared a state of emergency or disaster.
  • Personal care assistance in shelters also may be available through local and state programs. The emergency shelter manager is the key contact for all special needs services.
  • Services include assistance with eating, toileting, bathing, grooming and dressing, and assistance with catheters, injections and respiratory needs.

I would encourage you to read the article, print out a copy, and keep it in your emergency information file. I hope you never need it, but just in case … have a plan.

Thursday, August 16, 2012

Asking “How will I die?”

This article is another one in a series about becoming your own advocate.

AdvocateA blog article from David Ropeik does a nice job of explaining why it is important to plan for your death … more than what most of us do today. This thought process goes beyond having a will, a living will and a power of attorney. They are all important documents, but there is so much more to consider.

Facing, and Planning For, How You Will Die (and why we don't.)
David Ropeik
This blog writes about how we perceive risk, and how those perceptions often don’t match the facts. We’re more afraid of some things than we need to be, and less afraid of some things than we ought to be, and those misperceptions – what I call the Perception Gap - get us into trouble. One of the things we should certainly worry more about, is dying. Not whether we will die, of course, but how. Given all we do to manifest control over how we live, it’s stunning how few of us have made any plans for the nature of how we will leave this mortal plane.

Most of us won’t die from the threats that seem to worry us the most. We will not be murdered, die in a plane crash, or succumb to the effects of climate change. Environmental contamination, terrorism, swine/bird/the next big flu…each may in fact kill a lot of people. But it is a pretty sure bet that most of us will simply get frail, and sick from heart disease (#1 cause of death) or cancer (#2) or chronic lower pulmonary disease (#3) or a stroke (#4), and need progressively more and more medical care while experiencing progressively less quality of life, even progressively less and less actual lucidity or even consciousness. No need to get morbid with the details here. Suffice it to say you and I will make the long goodbye more with a increasingly care-dependent whimper than with a neat clean final bang.

Yet, as likely as this is for most of us, and as commonly as we have watched our elders pass away under these conditions, few of us have made any plans for this part of our lives. As worried as we are about dying, we are not worried enough about how we will die. That Perception Gap poses all sorts of risks, not just for us, but for our families and loved ones who will have to deal with a lot of serious decisions and questions, simply because we haven’t worried enough to ask them in advance.

Whenever we feel like we have control, we are less worried. You probably think you have control over all those unpleasant-to-think-about end-of-life questions, like what should happen if you are unconscious and need machines to keep you alive, or when to say enough is enough when you’re so sick and frail that more and more medical care is having less and less effect fooling mother nature and postponing the inevitable. All you have to do is sit down right now and think through your preferences, and write them down, and assign someone to be your decision maker if you’re not able to make the call yourself. You have the ability to decide. You have control.

But if you’re like most of us, myself included, you have not had this hard conversation with yourself. You have not faced these difficult questions. You have no end-of-life plan, no health care proxy. Why? In part, because you know that whenever you want to, you can. That sense of control let’s you off the hook from having to face some scary unpleasant ugly stuff. That produces a Perception Gap, the risk that exists because your sense of control has allowed you to worry less than you should about a risk that you and your family are one heart attack or car crash or stroke away from having to deal with.

Advance-Statement-Source-BMA - 1995

Below are additional documents and information that will help you through this advocacy process.
  • Another good read is End of Life Decision Making. The first paragraph of the article sums up the article: Americans are a people who plan. We plan everything: our schedules, our careers and work projects, our weddings and vacations, our retirements. Many of us plan for the disposition of our estates after we die. The one area that most of us avoid planning is the end of our life. Yet, if we don't plan, if we don't at least think about it and share our ideas with those we love, others take over at the very time when we are most vulnerable, most in need of understanding and comfort, and most longing for dignity
  • One other PDF document, End of Life Decisions, does a nice job of explaining some of these decisions such as ‘do not resuscitate’ (DNR) and ‘do not intubate’ (DNI).

Tuesday, August 14, 2012

Eighteen Months – Dutasteride Update

avodartIt seems hard to believe that it has been eighteen months since I began taking dutasteride (Avodart). Since my last report I have averaged 95 minutes for the every-other-day long exercise program and 18 minutes for the short ones. I have exercised every day for the last 3½ years … it sure sounds like an addiction to me. Since I began taking dutasteride I have also been keeping a daily journal of my experiences. I enjoy reading about my initial days of taking it.

I continue to feel very positive about dutasteride. For me, it works, and people that know me also continue to comment on how it appears to be helping. One of the most significant benefits has been the consistency of life I have been experiencing. Where I use to have up and down days (strength/weakness), the last eighteen months have been level (steady as she goes) except for a couple of times where I had a cold or some other health issue.

For example, in June I had an allergic reaction to something and the doctor gave me a steroid shot. For about thirty hours after the shot, I noticed a slight weakness. It taught me to always explain to any doctor how Kennedy’s Disease works and what to watch out for in regards to potential adverse reactions when treating unrelated conditions.

strength-testMore important, I feel as strong or as close to as strong as I did eighteen months ago. I have seen no degradation in my capabilities. For that, I am most thankful.

Saturday, August 11, 2012

Take care of yourself

The blog article titled “What you owe your loved ones” has several key points. I wrote about “arranging your affairs” last week. This week I want to address another topic from the article.

taking care-feetTaking care of yourself is a responsibility. You take care of yourself to minimize the stress and concern of your loved ones. For those of use living with Kennedy’s Disease, we learn early on in the progression that we are ‘high maintenance’. And, most of the stress we cause our loved ones is because of our falls and because we try to do something we shouldn’t be doing in the first place. Far too often we let our ego or our stubbornness get in the way of good sense.

Several of my bad falls (sprains, broken bones or concussions) were the result of me not thinking of the worst case scenarios before attempting something. Today, I would like to think that I consider most of the possibilities before starting something. Occasionally, I still miss a scenario. The only reason I know I missed one is because it happens.

Enjoy the topic below and follow the link above to read the entire article.

In order to be there for your loved ones you need to be the best person you can be. 

Taking care of yourself means:
  • You make it a priority to rest and get enough sleep every night.
  • You exercise regularly and eat the healthiest foods you can afford.
  • You give yourself time alone to meditate or reflect and renew if and when you need to.
  • You allow yourself to work on projects you’re passionate about and have as much fun as you can.
  • You foster relationships with the people you care about and respect.
These are the basic steps but you can add anything that energizes you and enhances your inner and physical wellbeing.

Now, since you know I am an exercise addict and have realized the benefits of exercise in maintaining my strength, I need to reemphasize the number two bullet:
  • You exercise regularly performing ‘smart’ exercises that improve and maintain your strength, flexibility and general health.
And, even though it is insinuated in the last bullet in the article above, I feel it is important to add:
  • You are ‘open’ and ‘honest’ with your loved ones. You keep them informed and share your feelings including your fears. This open and honest relationship nurtures the relationships of those you love. It also frees you from wallowing in your own negative emotions that can lead to depression.
The key message is to show your love to others by taking care of yourself.
taking care-love

Monday, August 6, 2012

I’m addicted

Okay, I admit it, I am addicted to endorphins. 

Wikipedia explains that Endorphins ("endogenous morphine") are endogenous opioid peptides that function as neurotransmitters. They are produced by the pituitary gland and the hypothalamus in vertebrates during exercise, excitement, pain, consumption of spicy food, love and orgasm, and they resemble the opiates in their abilities to produce analgesia and a feeling of well-being.

Every day I exercise and every other day my long program goes 90-105 minutes on average. When I finish my workout I feel so good … a high. Life is good, everything appears more positive, and I sense I am more in control.

No, it is not a “runner’s high” like I use to experience, but it is still good and quite enjoyable.

One of my favorite activities after I finish exercising is to take my dog for a walk through the woods and around the lake. I am always amazed how different the world looks after exercising. It is more sensual and enjoyable probably because I am breathing better, seeing and smelling more, and just more relaxed … free of stress.

endorphins-happyIf I am going to be addicted to something, I definitely want it to be endorphins.

I read this the other day and liked it: 
You are only one workout away
from a good mood.

Thursday, August 2, 2012

Affairs to Remember

I read an interesting article the other day titled, “What You Owe Your Loved Ones.” by Manal. It had three sections and for today’s article I focused on the section on organizing your family and financial affairs.

financial planA few years ago I wrote a financial planning guide about preparing for early retirement. Manal’s article discusses some of the topics in the guide plus some other considerations. I cannot emphasize enough how important it is to ‘put your house in order’. It could be one of the greatest acts of love you will ever do for your family.

Arrange your affairs
Right now I feel that not only do I have an obligation to leave my finances in the simplest form possible but also all of my affairs. The more organized and clear you are about what would happen after you’re gone, the easier it is for your family to deal with it—and that is an act of love and kindness on your part that they will appreciate.

To organize your life start with the following actions:

A. Simplify your finances. I will write about this in more detail in the future but for now focus on these two steps:
  • Prepare a list of your bank/investing and retirement accounts. Write down the access codes and details that someone would need to know.
  • Create another list of the bills you pay and online access if needed. Add to that list any loans/mortgage you have.
Between those two lists someone should be able to tell what you own and owe. Don’t leave these documents on your computer. Keep a physical copy, preferably with your will.

B. Simplify your possessions. If you have a lot of stuff that you think would be a pain for your loved ones to deal with, get rid of as much as you can now. Use the usual system of throw out, give away, or sell.

If you want to keep some stuff to give away after you’re gone, organize such items in a way that can be easily picked up and make sure to include the instructions in your will.

C. Prepare a will. This is something I did a few years back. It was tough towill do I admit. No one likes to think about death and its aftermath. But if we want to be honest with ourselves, it’s going to happen no matter what. The sooner we accept this fact the better.

In your will you will address the disposal of your possessions, name your executor and beneficiaries as well as specifying funereal arrangements. Your will can be modified any time.

If you don’t have an overly complex estate, you can buy one of those legal kits and use it or you can work with a notary public. If things are more complicated, spend some time and money working with a professional to get your affairs in order.

D. Consider a living will. I’m not sure what the legal term would be in other countries. Also, this document may not be legally enforceable. But it can give your loved ones clarity about your wishes.
living-willIt basically states what you’d want if you ever need life support and if you don’t wish to be kept alive by artificial means.

This is one of the toughest documents to sign, but it’s a relief for you and your family. They’ll appreciate you absolving them from making one of the most painful decisions.

You may also add a power of attorney naming someone to act on your behalf if you don’t have a next of kin. Please note that a power of attorney won’t be valid after death. It’s useful in situations when you’re alive but incapacitated.

Arranging your affairs is something that might trigger pain and fear. But it is worth it; you’re doing it to make life easier for others around you. It’s a wonderful act of kindness and love. Remind yourself that the peace of mind is worth the pain of getting a few things done now.