Watching a patient slip into early dementia is unsettling
By Susan P. Burzynski, RDH, MSEd, AADH
I recently had two men tell me about the hardships of having their loved ones diagnosed with early-onset dementia (younger-onset Alzheimer’s disease) and later losing the person to the disease. A foster mom cried in my arms as she described living with her foster son who was affected by younger-onset Alzheimer’s disease.
While I have not had the experience of any family members with younger-onset Alzheimer’s disease, I certainly do have the day-to-day challenges of dealing with my parents, who are in varying stages of dementia. I treated the three patients I’m about to tell you about prior to their diagnoses. All three were the sweetest patients any hygienist would want to treat. Two women died in their early 60s, and the third is getting progressively worse.
Mary, age 50, was a happily married woman with a husband in education and two sons. When Mary started to display signs of forgetfulness, her family dismissed the signs at first. Mary loved to laugh and we had many delightful conversations. As she started to withdraw and develop anxiety, her family became more concerned. Eventually Mary refused to come in for her dental visits.
Donna, also in her 50s, started with the same signs, but she also had multiple other medical complications. She was a mom with an adult son who saw—and had to deal with—his mom changing before his eyes.
Joseph is a 59-year-old Down syndrome patient with younger-onset Alzheimer’s disease. He has changed so drastically since I began treating him 25 years ago. When I started treating him, he was a hugger and repeatedly said “I love you” during our visits. Now he is withdrawn, plaque-laden, nonverbal, and using sign language to communicate.
As you can see, none of these patients were old. Under the umbrella of dementia, younger-onset Alzheimer’s disease is not an “old person’s disease.” Imagine not remembering how to get to your friend’s house or being unable to remember your favorite restaurant. It’s a scary thought, isn’t it?
According to the World Health Organization, nearly 47.5 million people have dementia. The Mayo Clinic estimates 200,000 people are affected by younger-onset Alzheimer’s disease in their 40s and 50s. Between 2000 and 2013, deaths from Alzheimer’s disease increased 71%.1
Let’s start with the difference between dementia and Alzheimer’s disease. Dementia is an overall term. It is a symptom. Alzheimer’s disease is a common form of dementia.2 Trying to get an accurate diagnosis is frustrating to both a patient and his or her family. Oftentimes, a patient’s forgetfulness is dismissed as stress related. We’ve all had stress at home and work.
Doctors are not sure what causes younger-onset Alzheimer’s disease; there seems to be many causes. Scientists do know that there are two types of genes involved in Alzheimer’s: risk genes and deterministic genes. According to Alz.org, risk genes increase the likelihood of developing dementia, but there is no guarantee it will happen.3 The risk gene apolipoprotein E-e4, or APOE-e4, has the strongest impact. Deterministic genes directly cause the disease and anyone who inherits these genes will develop dementia.
I have Joseph on a three-month recall, and I try not to think of the time I will be informed of his passing. But until then, I will enjoy what time we have.
With familial Alzheimer’s disease, for example, the patient may have a parent or grandparent who developed Alzheimer’s at an early age. Genes APP, PSEN1, and PSEN2 play a part in the disease.4 Amyloid-beta precursor protein (APP) is a gene in the brain that directs movement of nerve cells during early development. This gene can have 50 different mutations, and the contributing factor in less than 10% of younger-onset Alzheimer’s disease.
One of the common mutations is in the amino acids that are in APP. This can lead to an increased amount of amyloid 3 peptide or to producing a longer, sticky form of peptide. When the cells release protein fragments, accumulation forms clumps in the brain, which is called amyloid plaque. These two mutations may cause the death of neurons and lead to the progressive signs and symptoms of younger-onset Alzheimer’s disease.
Presenilin-1 (PSEN1) is a subunit of gamma (y) secretase. In a patient with younger-onset Alzheimer’s disease, there can be as many as 150 PSEN1 mutations. This can be the most common cause of younger-onset Alzheimer’s disease.5
Besides genetics, family history, age, and heredity, Swedish researchers have found that teenage behavior can increase the risk of someone developing younger-onset Alzheimer’s disease. If the patient abused drugs or alcohol, the person may have a higher risk of developing a form of dementia.6 Additionally, head injuries can be a risk factor.7
We need to treat each patient with care and understanding. According to the Alzheimer’s organization, we should not make assumptions about a person’s ability to communicate because of an Alzheimer’s diagnosis. Every person’s diagnosis is different, and people are affected differently. Don’t exclude a person with the disease from a conversation. Speak directly to the person if you want to know how he or she is doing. Take time to listen to how the person is feeling, and what he or she is thinking or may need. Give the person time to respond. Don’t interrupt or finish someone’s sentences unless they ask for help.
We lost Mary and Donna way too early, as I’m sure their families agree. I have Joseph on a three-month recall, and I try not to think of the time I will be informed of his passing. But until then, I will enjoy what time we have.
Susan P. Burzynski, RDH, MSEd, received her associate’s degree from Erie Community College, her bachelor of science degree, as well as her master of general education, from Canisius College in Buffalo, N.Y. She was awarded the Sunstar/RDH Award of Distinction in 2010, a Fellowship from American Academy of Dental Hygiene (AADH) in 2011 and Excellence in Leadership in 2013 from the Dental Hygienists Association of State of New York (DHASNY). As a full-time clinical hygienist, Susan has written numerous articles for hygiene magazines, has worked as a key opinion leader for various dental companies, and volunteered her services to the veterans who have served our country.
1. 2016 Alzheimer’s disease facts and figures. US National Library of Medicine National Institutes of Health website. https://www.ncbi.nlm.nih.gov/pubmed/27570871. Published April 2016. Accessed March 20, 2018.
2. Dementia and Alzheimer’s: What are the differences? Healthline website. https://www.healthline.com/health/alzheimers-disease/difference-dementia-alzheimers. Accessed March 20, 2018.
3. Risk factors. Alzheimer’s website. https://www.alz.org/alzheimers_disease_causes_risk_factors.asp. Accessed March 21, 2018.
4. Cruchaga C. Understanding the role of APP, PSEN1, PSEN2, TREM2 and PLD3 in Alzheimer’s disease. Alzheimer’s Association website. https://www.alz.org/research/alzheimers_grants/for_researchers/overview-2015.asp?grants=2015cruchaga. Accessed March 21, 2018.
5. In Brief for healthcare professionals. Younger-onset Alzheimer’s disease can be due to genetic profile. Alzheimer’s website. https://www.alz.org/health-care-professionals/documents/InBrief_GeneticLink.pdf. Accessed March 22, 2018.
6. Researchers study alcohol’s link to Alzheimer’s. Hazelton Betty Ford Foundation website. http://www.hazelden.org/web/public/ade20812.page. Accessed March 22, 2018.
7. Graff-Radford J. Alzheimer’s: Can a head injury increase my risk? Mayo Clinic website. https://www.mayoclinic.org/diseases-conditions/alzheimers-disease/expert-answers/alzheimers-disease/faq-20057837. Accessed March 22, 2018.