Abstract
The significant increment in life expectancy, associated to the existence of high-performing older adults, and the appropriate diagnosis of early dementias, lead to an uncommon scenario, of healthy parents accompanying their children with Alzheimer’s disease or another dementia to medical consultations. Here, we reported three peculiar clinical vignettes of patients diagnosed with a dementia, who were accompanied by healthy parents. This is a modern situation that tends to become more frequent, and must be properly discussed, since multidisciplinary care and specific training are necessary.
INTRODUCTION
Early-onset Alzheimer’s disease (EOAD) is defined as the diagnosis of Alzheimer’s disease (AD) in patients who present symptoms before the age of 65 years. EOAD comprises about 5% of cases of AD, and, in comparison to late-onset AD, may present different clinical characteristics, such as more aggressive course, less memory impairment, and greater involvement of other cognitive domains on presentation, commonly leading to frequent delay in diagnosis [1]. Although there is also greater genetic predisposition, sporadic EOAD (sEOAD) is still more common than familial EOAD (fEOAD). This fact, however, contrasts with the daily clinical suspicion, that is usually higher when there is a clear family history.
On the other hand, a new concept has emerged, that is of SuperAgers or high-performing older adults (HPOA), that are defined as individuals aged 80 years or older with memory abilities similar or superior to middle-aged subjects [2, 3]. The existence of HPOA, and the appropriate diagnosis of early dementias, lead to scenarios that are still rare, but which tend to become more frequent, which is the situation of healthy parents having their children diagnosed with dementia. The family impact generated by the deconstruction of the natural idea that “the children will grow up and take care of their parents in their old age” is evident and must be well understood by health professionals, so that integrated care and adequate support can be provided.
In order to illustrate this new situation, we present below two characteristic clinical vignettes describing uncommon EOAD scenarios and one more case in which a classical AD may also present an unusual, but very modern circumstance, of a cognitively healthy father with a child with AD.
CLINICAL VIGNETTE 1: “HOW CAN MY DAUGHTER HAVE DEMENTIA IF I HAVEN’T?”
A very lucid 94-year-old mother, a Japanese descendant, brought her 67-year-old daughter for an assessment to determine why she had been having memory decline and difficulty performing some daily tasks in the past three years. In the initial investigation, the patient scored 20 points out of a total of 30 points in the Mini-Mental State Examination (MMSE). She also presented signs of executive dysfunction and mild behavioral changes, causing a major impact on her functional autonomy and on her quality of life. The patient had hypertension and insulin-dependent diabetes mellitus. Diagnostic workup indicated clinical diagnosis of probable AD dementia at a mild to moderate stage.
The patient had three grown-up children and a husband, but the family had not suspected the diagnosis of AD earlier, since they had never experienced this situation with any close family member and also because of a natural reluctance to consider the possibility of someone having dementia if her parents had not.
The patient’s father died due to complications after a stroke, at the age of 72. He had hypertension, diabetes, and dyslipidemia. The patient’s mother was bilingual with Japanese as her main language, although she speaks Portuguese fluently. She has hypertension and diabetes mellitus, controlled with oral medication. Despite her age, she had no cognitive complaints. The question that the mother brought to us is certainly a philosophical challenge for every clinician who treats people with dementia: “But how can my daughter have dementia if I haven’t?”
CLINICAL VIGNETTE 2: “IS IT POSSIBLE FOR A SON TO REACH SENILITY BEFORE THE PARENTS?”
A 57-year-old man, diagnosed one year before with early-onset AD was accompanied to the outpatient clinic by his family, including his children, and by his healthy adoptive parents in their 80s. The parents did not have any neurological complaints. In addition to memory complaints, the patient had behavioral changes, which, although incipient, were causing great distress to his parents. The patient did not meet diagnostic criteria for another cause of dementia, and cerebrospinal fluid AD biomarkers confirmed the previous diagnosis.
The patient was married and had two children, who were the first to identify the symptoms. However, they doubted somebody could have AD before the age of 60. In this case, there were no major conflicts regarding the patient having an illness without his parents having, since the real family history was unknown. However, the patient’s condition was a huge burden for this family, and they raised an interesting question: is it possible for a son to reach senility before the parents?
CLINICAL VIGNETTE 3: “MY DAUGHTER NEEDS YOUR EVALUATION: HER MEMORY HAS BEEN VERY BAD!”
A very pleasant 99-year-old man (Mr. F), a widowed retired driver with one year of schooling, was brought by a great-grandson to a geriatric consultation. The great-grandson told him that he was going to pick him up at the end of the assessment when they were to call each other by cell phone.
Mr. F was clearheaded and fully independent for instrumental and basic activities of daily living and was alone at the consultation. We conducted a global geriatric assessment, including a brief cognitive evaluation. His MMSE score was normal for the education-adjusted cutoff score for cognitive impairment. Geriatric Depression Scale score was 0, indicating absence of depressive symptoms. Upon receiving his final report and our compliments on his health, Mr. F asked us to schedule an appointment for one of his daughters (Mrs. C) to administer the same tests we performed on him: “She is the one who needs this consultation, doctor.”
After a few months, Mrs. C, aged 75 years and with eight years of schooling, came to the outpatient clinic accompanied by her father, Mr. F, already aged 100 years, and by one of her six children. Global geriatric assessment and ancillary tests confirmed the diagnosis of mild dementia due to a clinically compatible AD.
Mr. F, who is still in good health, is a very successful centenarian. He finds it surprising and quite unexpected to have a daughter “so young with AD” (as he describes), but he is happy to know that she is “well taken care of by the family and the medical team”. Mr. F has a very interesting and uncommon Brazilian name, “Felizardo”, which in Portuguese means very lucky and happy.
DISCUSSION
The cases presented illustrate a modern phenomenon of parents who have their children diagnosed with dementia. Among several factors associated with the genesis of this situation, are the recognition and proper diagnosis of people with dementia who would otherwise be misdiagnosed with a psychiatric disorder; the presence of environmental and epigenetic factors that add risk factors to genetically predisposed people; in addition to the existence of parents, called high-performing older adults. With the significant increment in life expectancy experienced by most countries in the last decades, the absolute number of people who reach advanced ages and still maintain good cognitive functioning and autonomy is also of note. Higher education, absence of cardiovascular risk factors, physical activity, and also genetic factors as the absence of the apolipoprotein epsilon4 allele, may be protective factors of cognitive decline, and may be also related to HPOA [2–4]. In addition, epigenetic factors, transcriptional regulations, and gene-environment interactions could offer a plausible explanation of why close relatives may not develop the disease or may present with the condition at such variable ages [5, 6].
The prevalence of dementia increases exponentially after the age of 65 years, usually doubling its rate at each five-year interval [7]. Hence, it is indeed much more common to see daughters and sons bringing their parents with cognitive changes to clinical consultation. The situation of a father bringing a child for consultation with cognitive complaints, usually age-related, seems a contradiction, and an inversion of the natural order of life. Moreover, the incidence of EOAD has increased nowadays, and although the etiology and genetic basis remain poorly understood, patients with EOAD are commonly excluded from observational and therapeutic studies.
In the usual circle of life, parents take care of their children until they become fully independent. Later on, it is the children’s turn to deliver care to their parents when they become fragile or when they present some illness that affects their functional independence. It is still not usual for a parent to look after a middle-age or older child affected by dementia. This situation represents an inversion of what is considered “normal” during the life cycle and is certainly a major challenge for parents, demanding capabilities of understanding, acceptance, and coping.
The professional approach of an early dementia may have some peculiarities. First, early diagnosis should be sought in an attempt to reduce initial family burden. Also, clinicians must clearly communicate the diagnosis, the perspective of evolution, and the therapeutic possibilities, avoiding vague terms or medical language. Still, it is important to understand the family context, to know if the parents are the main caregivers, since despite the relatively preserved cognitive function, parents may have physical limitations, due to their age, such as frailty syndrome, among other age-related diseases. There is a need for closeness to other family members, and, frequently, caseworkers. Finally, there are legal implications of having an elderly caregiver that must be taken into account.
In this way, although the term senility, as mentioned by one of the parents, may have a broad definition [8], it is crucial that healthcare professionals seek to educate the community that dementia is not a natural part of aging. In addition, specific training of the multidisciplinary team may be necessary to manage and deliver the best possible care within this new scenario, that may appear more frequently in the near future.
CONCLUSION
The situation of healthy parents, having their children diagnosed with AD or other dementia, has a relevant practical implication in society today. The proper care may be more difficult and preparing healthy professionals to properly recognize and understand how to manage this condition is of extremely importance, since it tends to be more frequent with the natural aging of the population.
DISCLOSURE STATEMENT
Authors’ disclosures available online (https://www.j-alz.com/manuscript-disclosures/21-5234r1).