Late Onset (LOTS)
Tay-Sachs, Sandhoff and GM1 diseases
The mental health aspect of LOTS falls into two categories: an individual's overall emotional well-being, and psychiatric problems that are specific to some people living with LOTS.
Living with LOTS may trigger feelings of isolation, depression and frustration, as an individual must constantly learn to adjust to new developments and restrictions imposed by the disease. An active work, social and family life promotes emotional well-being. Individuals with LOTS, just like people who are not struggling with a rare lysosomal disease, want to engage in activities that reflect their needs, interests and functional level as well as enjoy activities that promote cognitive, physical, social and emotional well-being, and positive involvement in daily life.
About 30-40% of people living with LOTS experience mental health symptoms such as hallucinations, paranoia, anxiety, manic-depression, severe depression, obsessive compulsive behavior, and disorientation. Some medications used to treat depression and psychosis, such as haloperidol, thorazine, and the tricyclic antidepressants are thought to be potentially harmful to patients with LOTS, while others, including the benzodiazapines used for anxiety and psychosis, and tegretol which is used to treat mood disorders, are thought to be relatively safe. Patients should check with their psychiatrist and can contact the office for more information. Read Late Onset Tay-Sachs Disease: Adverse effects of medications and implications for treatment by Shapiro et al.
Several cases of childbirth triggering a psychotic episode in women with LOTS have been reported. This should not deter affected women from having children, but requires close monitoring.
It is important to seek help early if you suspect mental health symptoms. Generally the treating physician provides a psychiatric or neuro-psychiatric referral.
Subtle signs of cognitive problems
- Diminished memory, immediate or short-term
- Poor reasoning/judgment
- Reduced problem-solving ability
- Difficulty sequencing/organizing ideas
- Concentration problems/distractibility/short attention span
- New learning ability diminished
- Problems with numbers and mathematic computations
Seek assistance from a psychiatrist or other mental health professional when the areas of memory, thought processing and organization of thought become problematic.
Simple-at-home strategies to help cognitive problems:
Poor orientation to time and place
- Keep a large calendar visible
- Display a large, visible clock
- Post signs on walls stating location
- Post a schedule of daily routine
- Establish routines for all activities, for example place glasses in same place daily
- Label cabinets and drawers
- Keep a memory log book, similar to a diary, in which the affected person can write down and refer back to what he/she has done with whom, and when
- Keep an appointment book for social events, doctor's visits and other dates and occasions to be remembered
Reduced problem solving ability
- Discuss with family or caregiver possible problems that could occur at home. Solutions or steps to be followed should be anticipated, discussed, written down and kept in an obvious place
- Follow these set guidelines for problem solving.
In some cases it may be appropriate to appoint a guardian to assist with important decision making.