Creutzfeldt-Jakob Disease and Counseling

About once every 6 months, an article pertaining to the psychosocial impact of human prion disease makes its way to the scientific literature.  I was very excited to see one of these articles towards the end of 2010 entitled, "Counselling people with Creutzfeldt-Jakob disease (CJD) and their families."  The author is Clare Morris, who I have not had the privilege of meeting but hope to some day at one of the prion disease conferences (Clare-if you are reading this, please introduce yourself!).  Clare works in the National Prion Clinic within the UK and has first-hand experience of counseling patients and their families who are affected by all forms of human prion disease.  All forms including variant CJD (vCJD), which I think is important.  

Overall, I liked the article but was a little disappointed after having read it.  My disappointment had nothing to do with what was written, but was more in line with the continued lack of data that we have in regards to this aspect of the field.  Any information that is provided on these issues, even those that are anecdotal, is very important due to the lack of information in the scientific literature regarding the psychosocial effects of human prion disease.  Like the few other articles that have been published before, this article has precious anecdotal information mixed with information regarding experiences with similar illnesses.

Why do the majority of these articles not focus solely on human prion diseases?  I think about this often and I have a few theories.  

1) Lack of data: There is little data on psychosocial aspects of the disease and/or the effects on caregivers.  One of the most data driven articles on this topic that I can think of readily comes from an article on the vCJD caregiver experience.  But still, this is not the kind of data that we generally collect to analyze in order to further assessment and treatment standards.  (NB: I have some data, but have not published it yet).

2) Lack of interest?: By lack of interest, I am principally referring to funding of projects and research support.  This is something that PrioNet has addressed and they are encouraging researchers to work in this area.  I think that this is both a brave and generous approach by this organization.  I think there is also a lack of interest on the part of researchers and clinicians regarding this data.  However, this could well be because of the disease's low incidence, funding, qualified researchers interested in the field, and assumption that the rapidity of the disease precludes any meaningful assessment and/or intervention(s).

3) Application to other, more prevalent diseases: Reading the article, one quickly realizes that information learned from dealing with those affected by prion diseases can be readily applied to other conditions that may be more prevalent yet also in dire need of information and direction.  This was apparent in the article when counseling for prion disease was correlated with young-onset dementias. The latter also has a dearth of psychosocial information contained within the literature, however; this has been thankfully changing over the past couple of years. 

4) Nothing can be done:  Sometimes people believe that nothing can be done for those affected by neurodegenerative illnesses.  In general, physicians are trained to "fix" medical problems or ameliorate them considerably.  Very few physicians treat patients that have no hope for recovery or improvement on a daily basis and this can be difficult to grasp.  However, much can be done.  The article sees the primary purpose of counseling in this population as mostly a presence of mind.  In other words, being there for the patient and family.  We stop talking, and listen.  We offer support, we answer questions, and most importantly we walk the journey with them.  How much anxiety does one get when they are first introduced to a new and life-changing situation (e.g., college, marriage, births)?  How much easier is it for someone to travel down such a lonely path while being accompanied by someone who has walked the road before?  

5) Impact: Another assertion is that the impact is minimal.  No disease is discovered, no laboratory test can be marketed, and no treatment can be developed.  However, individual lives can be affected and will be regardless of what we do.  We can only hope to make it a little less worse experience.

So that's my reflections on the article.  I applaud its being written as well as being published within the scientific literature.  Good job!

Comments

[Clare Morris]

I would be very interested to meet you and discuss the article and the need for psychosocial research regarding the experience of CJD. i have now moved on from the NPC but I am looking to do some research into the experience of living with the risk of inherited prion disease.
With best wishes
Clare Morris