Is there a reference bibliography that helps in the management of the patient?
Recently, I was assigned a task in an integrated facility, with guests mainly suffering from Alzheimer's disease, senile dementia and oligophrenia. Average age 75 years.
Together with these guests there is a 54 year old boy with Huntington’s Disease. He was an accountant. Unfortunately, there is no medical record with the history of the patient. The operators told me that he had a sister (the one who then took him to the facility and that unfortunately I have not yet had the pleasure of meeting to interview in person) and that no one in the family had suffered from this disease. I read the last certificate dating back to 2015 made by the neurology department of a Local Health Unit (ASL) in Sardinia, stating that the patient had not substantially worsened.
Well, I did not know this disease but I immediately thought that in the facility, the boy, at this time, had "more need".
The stage of the disease, I think, is advanced. The patient is always seated in the wheelchair, contained with the belt on the front. He has difficulties in expressing himself (and I can understand that... because I don't know whether to attribute his gestures to the disease or to a request for something). He often understands what I ask him if expressed in a simple way. I have noticed that he gets very nervous when he hears the noise made by other patients and when he is hungry (he eats quite voraciously). As I mentioned earlier, not knowing the disease in depth, I documented myself but I notice that there is information regarding the disease but not a bibliography that could be useful from the practical side. I thank you in advance for taking the time to answer to me.
Patients also at an advanced stage understand much more than expected. Probably the correct framework should take into account the pharmacological management that, sometimes, erroneously, impairs the motor capacity in an attempt to suppress involuntary movements. From what you describe to us there seems to be a considerable dystonia of the movement that can be a consequence of the evolution of the disease as well as the side effects of some drugs. For this reason, if you believe, you can arrange the possibility for us to see the patient in one of the clinics nearest to you by calling our toll-free number (800.388.330). As far as bibliography is concerned, we invite you first to look at our website and the educational video on the right of the homepage ('Huntington's Disease in 5 minutes'). The bibliography is extensive, mostly in English, and hardly offers practical suggestions, but we are happy to email you a "guide" to understanding and managing symptoms related to the disease, including cognitive-behavioral aspects (also in English). It is also important, in these cases, to have a family member of reference to facilitate the reconstruction of a part of the patient's life history, an aspect not to be underestimated in order to establish a good therapeutic alliance (Response by Dr. Ferdinando Squitieri, neurologist and Dr. Simone Migliore, neuropsychologist).