Communications - It’s not a one-way street

Effective communications … wow, we can spend a lot of time on this subject.  It could be simplified to the title, but so much would be lacking and left unsaid.

Many of us having to deal with a disease that has no treatment or cure.  And, initially we often have problems explaining how we feel.  We tend to hold these feelings close to our chest often for fear that we might come across as weak or emotional or in need of help.  Many of us carry this self-made belief that we should ‘man-up’ (suck it up) and just get on with life.  

Unfortunately, holding these thoughts and feelings in is not healthy. 

• First, we need to be able to unload … de-stress … because holding these feelings inside can be harmful to your health as well as family relationships. 
• Second, we cannot always see the forest because of the trees.  We become so entangled with ‘what-if’’, ‘why me’ and ‘what are we going to do now’ that we lose sight of the end-game (in other words, how do I maintain loving and healthy relationships as this disease progresses).

Communication Tools

I read an interesting article recently in “The Costco Connection” about “Aging Wisely.”  One section was on “Simple communications tools to remember.”  The points fit well into today’s topic.  Below are the points as well as my translation.

• Take a walk in the other person’s shoes.  (Yup, it is the old walk in mile in another person’s moccasins)  Try to understand what’s going with all your family members. 
  • Translation:  Ask each family member how they see your condition and what it means to them if you cannot physically do what you used to be able to do.
• Separate the person from the problem.  Conflict is a shared problem that you can conquer together without placing blame.
  • Translation:  So often we feel this is all on us and we have let everyone else down.  We find it difficult to share our thoughts and fears.  In other words, it is all about me.  Once we can separate ourselves from the situation and look at it from a family perspective, the ‘how do we continue to grow as a family’ become more evident.
• Remember optimism.  Celebrate small successes and build on them.
  • Translation:  When we are capable of clearly looking at the situation it is not a ‘now’ problem.  Kennedy’s Disease, for example, is a slow progressive disorder.  Now that we know it is not the end of the world.  We have time to think it through and consider our options.  We have time to plan and time to talk it over with others that care.
• Break bread together.  Sharing meals together can help reestablish bonds and open communication channels.
  • Translation:  Pick the right time to talk about the subject.  Often, the best time is at the end of a meal, when the family is more relaxed and not multi-tasking.
• Regularize family meetings, phone calls and emails.  Keep everyone in the loop.
  • Translation:  Don’t just talk about it and forget it.  Many times additional questions and concerns will come up later.  It is your job to keep the communication channels open and to make the entire family feel comfortable talking about any concerns.
• Try a little humor.  It is not a funny situation, but if you can avoid taking everything so seriously, it will make it easier to discuss.
  • Translation:  Everyone must know it is not the end of the world.  Life will continue on.  Yes, things might change over time, but you are still a loving family … and that will not change.  If the rest of the family can see that you can still joke about things (i.e., your recent fall), it will help ease the tension.

 

Keeping the channels open

Keep in mind that you were the family leader before the news and you are still the family leader now.  It is your job to keep the communication channels open and to make other family members feel comfortable with asking questions or expressing their concerns.  Occasionally it might even mean saying, “I don’t know.” 

For communications to be effective, it has to be a two-way street. 

• When was the last time you had a family meeting to discuss anything including your current health situation? 
• During the meeting did you hear how the rest of the family was dealing with the news? 
• Did you make them feel comfortable when they shared their concerns? 
• Were you open and honest in answering their questions?

How can one disease have so many names?

I am often asked where did the name Kennedy’s Disease come from.  Many people, when they first hear Kennedy’s Disease, associate it with some disease  that John F. Kennedy had.

You can imagine the confusion that exists when Kennedy's Disease goes by many names including (many are the same and just arranged or spelled differently):

• Kennedy's Syndrome
• Kennedy Disease
• Spinal Bulbar Muscular Atrophy
• Spinal and Bulbar Muscular Atrophy
• X-linked Spinal Bulbar Muscular Atrophy
• X-linked Spinal and Bulbar Muscular Atrophy
• Bulbospinal Muscular Atrophy
• SBMA

And, adding to the confusion, it seems that even researchers and doctors cannot agree on one name for the condition.

To make matters worse, when some people do a little research on symptoms, they often end up with other neuromuscular disorders or motor neuron diseases including Spinal Muscular Atrophy (SMA Type IV – Adult Onset) and Amyotrophic Lateral Sclerosis (ALS).

Further, when they hear or read the word “disease”, people occasionally  associate it with something possibly contagious.

So, Why Kennedy’s Disease?

The condition was named after Dr. William Kennedy of the University of Minnesota who first identified this disease in the United States.  I pulled this from Dictionary.com:

Dr. William Kennedy

Kennedy, William Robert (born 1927) American neurologist. Kennedy enjoyed a long association with the University of Minnesota's medical center in Minneapolis, rising to rank of professor of neurology. His major area of research was neuromuscular disorders. The neuromuscular disorder that bears his name was described in an article published in 1980 that he coauthored with M. Alter and J. H. Sung.

That being said, it appears Dr. Kennedy was not the first to identify the disorder.  I pulled this from Neurology MedLink:

Historical Note

Although Kennedy disease bears the name of William R Kennedy, the first reports of this disease were likely published by L. T. Kurland, who described an atypical form of lower motor neuron disease in a Japanese family (Kurland 1957). Following the reports by Kurland, Magee provided additional descriptions of patients with X-linked spinobulbar muscular atrophy in the absence of corticospinal tract involvement (Magee 1960). In 1968, Kennedy reported his experience with 2 large families at the Mayo Clinic in Rochester, Minnesota (Kennedy et al 1968). The designation “Kennedy disease” was first introduced into the French literature in 1979 (Schoenen et al 1979). The disease garnered particular interest as the first example of a polyglutamine-repeat disorder, of which there are now several other neurologic examples, including Huntington disease and several of the spinocerebellar ataxias.

Whew!  I am glad I did this research because it is all so much clearer to me. :-)

Heroes for my son

Last year I wrote a few articles about heroes in Brad Meltzer’s book, ‘Heroes for my Son’.  I recommended the book because it told stories about people that many of us do not know or only know a little about.

Today’s ‘Hero” from Brad’s book is Lou Gehrig.  The story is called, “Indestructible”.   Us old timers know about Lou as one of the greatest baseball players of all times and also because of the disease that he had … ALS.  Mr. Meltzer told his story this way:

Lou Gehrig - Indestructible

 

[Despite muscle spasms and broken bones, New York Yankee Lou Gehrig played in 2,130 consecutive games over thirteen seasons. In each of those thirteen seasons, he scored 100 runs and hit 100 RBI. His batting average of .361 in seven World Series brought the Yankees six titles. It took a debilitating and fatal disease to take him off the field, and even then he wasn’t beat.]
 
For thirteen seasons, Lou Gehrig never missed a single game.  Think of it.  Think of what happens over thirteen years...
 
He didn’t miss a game when he was sick.  Or when he was tired, or bored, or not feeling right.  Not when he was under the weather, or drained, or just wanted to take a day for himself.
 
Not when he broke his thumb.  Or his toe.  Or when he suffered the seventeen other healed fractures that they found in just his hand and that they never knew about because he never complained.
For thirteen seasons, for more than two thousand games in a row, Lou Gehrig showed up, because he never wanted to let us down.
 
The only thing that stopped him?  The fatal disease that once caused his back to spasm so badly, he had to be carried off the field at the end of the ninth inning.
 


They called Lou Gehrig “the Iron Horse.”  But he wasn’t made of iron.  He was made like us.  He just didn’t let that stop him.

I consider myself the luckiest man on the face of the Earth. And I might have been given a bad break, but I’ve got an awful lot to live for.
[Lou Gehrig, farewell speech, July 4, 1939, Yankee Stadium]

Knowing the way I feel some days and how a few broken bones effected my life, Lou Gehrig’s accomplishments both on and off the field astound me.  He was an Ironman.

When, What and How?

There is a question I am often asked and one that I continue to struggle with because I am not sure there is one right answer.  I believe there are far too many dependencies to say what, when and how.

The question:  “When should I tell my daughter or son that he/she possibly has the defective gene?”



In my opinion, there is no particular age that anyone is fully ready to hear this news.  I believe a parent should wait …

1. until symptoms appear (in the case of a son),
2. or if a son or daughter start discussing having children with their spouse,
3. or if a treatment is made available and the earlier a patient begins treatment the slower the progression.

Prior to that, what is the value of discussing this issue?  It will not change anything and will probably just make the son or daughter worry about something needlessly.

Once you think you have the above answer, you next have to tackle, “How much should I tell them?”  What level of detail is important at this time?  Should I really get into the disease or just provide an overview and be ready to address additional concerns?



Also, it is important to consider how your daughter or son might receive the information (maturity level).
From there you can drive yourself crazy with other questions including …

• Do I say “possibly” (or a 50-50 chance) even if you are certain they carry the mutated gene?
• Should I provide additional reading material on Kennedy’s Disease or sit with them as they search the internet for information and answers?
• Should I discuss my pending conversation with a genetics counselor asking for guidance as to the best way to explain the situation?  Or, do I break the news while we are both sitting in front of a counselor?
• Should I recommend testing or have them wait until they want to know for certain?
• If they do decide to be tested, how will this information impact their health insurance and employment now and in the future?
• If they son or daughter plan on having children, do I provide information on prenatal testing or schedule an appointment with a genetics counselor?

The above are just some starter questions.  I am sure there are more and perhaps some that are more important.

As a parent or potential parent, your opinion on this important subject is important.  Let other readers know your perspective and reasoning.  It just might help others when they wrestle with these questions.

She could teach me how to run

Several times a year I receive emails from family members that are living with Kennedy's Disease. They are normally from teenagers and young adults who have a father with the mutation. They are trying to fill in the gaps of what Kennedy's Disease is, how bad will it get, is there anything they can do to help, and occasionally asking why won't their parents talk to them about this. Yesterday I received another email from the son of a man with Kennedy's Disease. Following his question below is my answer (amplified for this blog).

"Thank you for all the time and effort put into your blog. My father was recently diagnosed with the disease and your blog was a wealth of insight, information, and comfort.

From a selfish position, I was wondering if you have any insight to what your children (or anyone else's children who have been affected by the disease whom you are in contact with) have gone through. It is difficult to accept that your father will become physically weaker over time. Any help or insight into coping with that and assisting both parents would be greatly appreciated."

The subject of "children" is an interesting and complex one.  The answer really depends upon the age and maturity of each child and how open the parents are to discussing the situation.  For example, my two grandkids (many years ago when they were pre-school and early grade school age) asked a few questions (why I could not get on the floor to play with them, etc.). When I explained the problem to their satisfaction, they just seemed to acknowledge and accept that grandpa was a little different (my wife has told me that for years). They thought I was still okay, however, because I bought them ice cream when they were not supposed to have it. 

I used a similar strategy recently for the two young children of friends who were also curious.  (Of course it does not hurt that I have a cool wheelchair and I take them for rides)

The explanation I gave these youngsters was that a part of my body (something inside me) is broken. Doctors do not have a way to fix it right now. Until they can fix it, my muscles just do not want to work the way their muscles do. I also explained that is why I use a wheelchair because it is difficult for me to walk. I told them no matter how weak my muscles became, I would always be their friend. I further explained that even though I can no longer pick them up, I still give great hugs (and then asked for one from each of them).

After listening to my explanation, our friend's daughter replied that she is a good runner and is very fast. If I wanted to, she could teach me how to run (the innocence of a child). 

I have found that the older the child is, the more difficult it is for him/her to accept the situation. I believe this is because he/she see (or imagine) the suffering the father is going through and the strain it has placed on both parents.  Additionally, older children and young adults have seen the progression of the disease over several years and the devastating impact it has had on the father.

My advice for children remains the same (changed slightly depending on specifics). In every aspect except for mobility and some strength/dexterity issues, your father is still the same man he was before Kennedy's Disease.  Yes, he is traveling through uncharted waters and he is struggling with acceptance issues and concerns for the future. However, he is still the father that raised you and he will always love you. These things will never change.  If you treat him differently or feel sorry for him, you are doing him and your relationship a disservice. 


My final recommendation is that you talk with him ... ask him questions and express your concerns.  He might not be ready to talk about it yet, but he needs to know the uncertainty/fear you are experiencing and that you want to talk with him more about this health issue when he is ready.  In addition, you mother is learning a whole new set of skills with the progression of the disease.  Do not be afraid to bring the subject up with her also.  She also needs your support also. 

Of course, this is my personal opinion based upon my experiences.  It is not a professional opinion and I have no training in this area.  If you continue to struggle with this, I would recommend some counseling.  A professional can help you come to terms with your concerns and perhaps help bridge the gap between the fears/concerns and the reality of living with Kennedy's Disease.

I hope this helps.  If you have other questions, please do not hesitate to ask.

__________________________________________

Readers, I could use your help with today's topic. Most of us living with Kennedy's Disease probably have some experience with this subject. Please consider adding your two-cents in the comments section below; or send me an email and I will add your thoughts to this article (anonymously).

• How did you explain the experience to your children (grandchildren)?
  
• What age(s) were your children (grandchildren) when they first began asking questions?
  
• What were their greatest concerns at the time?
  
• As they became older, did they want to know more about the disease? If so, what were their follow-up questions?
  
• If you had to do it over again, what would you do differently?
  
• Do you have any other recommendations for managing this situation?

I would value your thoughts on this important subject. Thank you.