Diagnosing Alzheimer's in People with Down's Syndrome
A recent message from a reader of Our Alzheimer's asking about the symptoms of Alzheimer's in someone with Down's syndrome (DS) has prompted me to write this Sharepost. It is true that the symptoms are the same as in someone without DS; memory loss, weight loss, apathy, personality changes, loss of conversation skills, increasingly poor mobility and dependency on others in activities of daily living. The possibility of compromised communication skills is bound to be influential but it is the presentation of symptoms, lifestyle, the richness or otherwise of care, and co-existing medical conditions, in some people with DS that can make recognition and diagnosis more difficult.
Down's syndrome is caused by a genetic defect in chromosome pair 21. It is not known why people with DS have a higher risk of developing Alzheimer's disease in middle age. In the past, Alzheimer's in people with DS was less of an issue. In the 1920s, for example, life expectancy was just nine years. By 1949 lifespan increased to 12 years, and by 1985 it was 35 years. Now, people with DS live an average of 55 years or more, but it is from about 40 years of age that symptoms of Alzheimer's can be identified (proportionately a much younger age group than in people without DS).
Interpretation and Understanding Deterioration in Behavior
I can draw on my own experience of the way behavior changes by referring to a 42 year old man (I will call him Wayne) who lived in a residential care home. In his earlier years, Wayne had a fulfilling and largely enjoyable life. He'd worked as a kitchen assistant in another care home where, under supervision, he could carry out simple tasks. He had an ongoing close relationship with a fellow female resident.
Wayne began to need prompting in issues of personal hygiene. Staff then found him increasingly difficult to manage. He no longer wanted to work, clean himself or his room. He rarely spoke to staff and residents, pushed people out of his way, shouted and was generally uncooperative. Staff put this down to the loss of his primary relationship (his girlfriend returning to live with her family), loss of confidence and general changes in his attitude to life. For short periods of time some aspects of his behavior would improve minimally although he never returned to work. It was when he started to be incontinent, sometimes fecally, that staff realized something physiological was happening to Wayne. He was taken for a medical evaluation and later diagnosed with Alzheimer's.
In the context of Alzheimer's disease Wayne's behavior was understandable. He pushed people out of the way so that he could quickly hold onto tables and other furniture as his mobility began to deteriorate. He shouted because he was probably frightened, confused but unable to express himself. He could no longer carry out familiar tasks so did not do them. With better staff understanding, new increased levels of nursing input and care, more emphasis on support Wayne's behavior gradually seemed to improve. As his Alzheimer's disease worsened so his behavior changed too and he became sadly quiet but well looked after.
Problems with Staff, Medical and Nursing Cover can Affect Recognition of Alzheimer's
In some residential settings, staff changes, poor medical cover, a high turnover of nursing and caregiver staff, poor staff education and weak managerial leadership can all contribute to lack of recognition of a disease status. Changes of behavior can be overlooked for months and even years. It is not difficult to imagine the knock on effect as staff disregard the signs of brain damage and consider behavioral change as relating to some lack of personal control.
The lack of recognition of early symptoms of Alzheimer's in people with DS can also be missed even when they live at home. DS is more common in mothers over the age of 40 years, so by the time the offspring enters middle age their parents are often over 80 themselves, so they may not notice or see the significance of behavioral changes for a long time.
Difficulties of Diagnosis
Another difficulty is that many people with Down syndrome cannot be evaluated by standard psychological tests. Poor communication skills make diagnosis difficult until the more gross symptoms emerge and tests for other diseases are excluded.
Deteriorating Co-Existing Conditions
People with DS are often born with congenital heart problems. They are susceptible to obesity, cataracts and with increased aging, susceptibility to thyroid and cardiovascular dysfunction. Skeletal problems such as osteoporosis, arthritis and muscular disorders are also issues associated with age. All these factors can complicate the picture in recognizing the symptoms of Alzheimer's.