Expert: Michalene Peticca - 2/4/2008

Question
QUESTION: Mom is fully in stage 6 and we care for her at home with aides. She has developed myoclonic episodes that begin with decreased consciousness in the last several months. Last week during one of these episodes her eyes deviated up and to the right, she locked her jaw (breaking a lower denture)and had an ashen appearance with decreased pulse and very shallow breathing. The spells last around a minute, sometimes more sometimes less and leave her exhausted. I called the neurologist and she does not think this is related to LBD but that she should have a 'cardiac' work-up. I am a nurse and I find that difficult to swallow....have you seen this type of occurrence before? She had a recent CT, EKG, and EEG which did not show any abnormal seizure like activity. Even if this is related to cardiac or the LBD, she is a DNR and we have just been providing supportive care during the episodes. Any thoughts would be appreciated. She is not on any neuroleptics and on a minimal twice daily dose of exelon. Tenormin, dyazide, KCL, norvasc, and low dose asa make up the rest of her meds. No changes to these for quite some time.

ANSWER: Hello Diane,

Thank you for writing.  You offer a complex situation that, hopefully, we can work toward some ideas and maybe answers.

Not knowing your mother's exact historical data, i assume that she has been dx: with Lafora body disease.? If so, then we assume this is the basis for her degenerative dementia state that she is currently in.  

I believe your question refers to the seizures that she is having and if they are r/t to the LBD or some type of possible cardiac d/o.  

My thoughts....it can't hurt having a "cardiac workup" especially with the diverse amount of cardiac meds. that she is currently taking. (I think i would question why the number of different heart meds?) Perhaps she is on too many or a contradiction is occuring?  

Nonetheless, i think it is..unfortunately, quite likely that the # of seizures is going to increase as your mother's brain continues to deteriorate from the dx: LBD.  Yes, we see that a lot ....especially in the very late stages...it can go very quickly.

The question you pose about the DNR is strictly a personal question that i leave to families.  I always ask families "what would your mom/dad/loved one want?"  Most know...and i say, well, then do what you think THEY would want.    Many say, "well, then i feel like i am 'killing them' if i don't do anything" and i say, "it is a PROGRESSIVE disease whether or not your choose today to do anything...the disease will STIll progress..it is that simple".  

So, i hope this sheds some light on thoughts.  I know you will are doing the best you can for your mother and with your education, will make you days the best they can be.

Best Regards,

Michalene Peticca, M.A.

---------- FOLLOW-UP ----------

QUESTION: Thank you for your quick response. What mom has is Lewy Body Disease. I misspoke on one med, it is toprol not tenormin, but the diazide, norvasc and toprol are for hypertension and she's been on them awhile, as with the exelon. We did make an appt for her as the neurologist requested. As you so rightly point out there is a fine line between what is right and what is appropriate for this time in her life.

So I guess I have an ugly question, it is partly to prepare myself and my brothers for what is coming. I knew she would deteriorate, but didn't think at all about the seizure type activity. If I read your response correctly, we can expect to see increasing episodes like it ending finally with her in stage 7. We are prepared and understand about the food and fluid piece. Since she doesn't want tubes of any kind, we are prepared for that point when she can no longer take nourishment or water orally.

Answer
Hi Diane,

Yes, unfortunately each person that goes through the disease experiences different variations of deterioration.   Most, however, are like your mother and deteriorate quickly because of the seizure activity that occurs in the late stages.   (i imagine it is like ice building on telephone wires that just begin to break)

It is wonderful that your family is involved and care.  I think as you prepare yourself for decisions, two thoughts should prevail:  1.  This is a deteriorating disease that no matter what, is NOT curable and any interventions (even from the very beginning) only sustain or slow the deterioration...there is NO cure.  That's not to say, i'm for or against tube feeds, etc....i'm for choice based on an individual's wishes; however, many families forget the terminal aspect of these diseases.      2.  Comfort is the key word from here on...whatever it takes, whatever your mother wants/needs to be at ease.  

I wish you some peace in knowing your doing what your mother would want.

Best Regards,
Michalene Peticca, M.A.