A local death

Mari A. Schaefer and Jason Laughlin have an article in The Philadelphia Inquirer about a mother's goodbye to her son:

Joanna Pacini reached into the coffin and touched her son's face. For the first time in years, she saw the man she once knew. Gone was the angry Joseph Pacini who had threatened police on YouTube (video, above), the brooding, irrational son who called her Satan. He was again the handsome, successful middle child she called "Joey, my Joey."
His peaceful expression contrasted with their last encounter, on 30 December 2014. She was preparing chicken cutlets in their Clifton Heights kitchen while he raged about the Pope. She told him she was tired of his rants against her faith.
"That's okay," Joseph Pacini said as he left to go to the gym. "Tonight it's going to end."
Within hours he was dead.
For his family, the 52-year-old man's death after a rush-hour confrontation with police in Drexel Hill was the latest reflection of a medical curse that has haunted their family for generations. At least five relatives have been known to have had Huntington's disease, a hereditary and untreatable brain disorder that afflicts or threatens as many as a quarter-million Americans, most between the ages of thirty and fifty.
Slowly the disease kills brain cells, causing loss of faculties, movement disorders, and emotional chaos. Rarely does it devolve into the violent paranoia displayed by Pacini, who posted online threats against police, and was fatally shot after allegedly trying to run down an officer.
But alienation and frustration are commonplace, experts say. In at least two cases, parents killed their adult children rather than see them live with the disease.
Pacini knew its devastating potential. Two years after being diagnosed in 1983, his father killed himself.
In that way, Pacini's death "is a tragic case from a clinical perspective," said Arik Johnson, a University of California at Los Angeles psychologist specializing in Huntington's disease. "If this guy had been able to see a doctor, if he had been able to get in and work with someone, this is something that could have been avoided."
In an interview last week, their first since the shooting, Pacini's eighty-year-old mother and fifty-four-year-old sister, Renee, discussed his downward spiral that ended when officers trying to arrest him concluded he was trying to run them down. They fired more than twenty shots, five of which struck Pacini.
Toxicology reports are pending, and investigators have not yet declared the shooting to be justified, but the family has no doubts. They said they had tried for years to persuade or even force Pacini to address and get help for his neurological disorder. He refused. Maybe, they say, he wanted to die.
"I think he's been suicidal for a long time, but didn't know how to do it," Renee Pacini said.
A natural charmer with good looks, Joseph Pacini was a happy baby and a mischievous kid. He and Renee were particularly close, she said. She was the first person he went to when he was in trouble. He attended Holy Cross elementary school in Springfield, Pennsylvania, and was a standout football player at Cardinal O'Hara High School.
After graduating in 1981, he focused on bodybuilding and business. By his thirties, his family said, he was earning so much as a headhunter for pharmaceutical companies that he gave his mother a thousand dollars a month.
But embedded in his genes was the disease that plagued his family. Joe Pacini, his father, was a civilian worker at the Philadelphia Naval Yard with top-secret security clearance, his widow said. At 48, he began forgetting the names of coworkers and couldn't remember why he was in meetings. He was showing signs of the same illness his mother and two sisters had, Joanna Pacini said. After 25 years, the elder Pacini left work on disability. "All he could see was his mother," she said. Two years later, he killed himself.
Suicide and depression are common among Huntington patients, experts say, though the two are not always linked. Huntington's damages impulse control, so suicides can sometimes be the culmination of just a bad day, said Amy Colcher, a neurologist at Camden's Cooper Hospital. A test to identify whether a person carries the responsible gene began to be used in 1993. Colcher thought those who tested positive would forgo having children, killing the spread of Huntington's. That hasn't happened. "The majority of them don't want to get tested," Colcher said. They feel that by the time their kids are at risk a cure will exist, she said.
Renee Pacini, who lives in North Wales, Pennsylvania, is among those who have chosen not to get tested. "I'm not going to live my life differently, at least not at this stage," she said. Her mother sees things differently. When she married, she had no idea that her husband had an inheritable malady, Joanna Pacini said. "I would never have brought children to the world knowing they could get sick," she said. They had three kids.
Debra Drakeley, Joseph Pacini's other sister, tested positive in her thirties. She prepared by looking for long-term care insurance and a nursing home. Now fifty, she is debilitated and has trouble talking, her family said.
According to his other sister and a friend, Joseph Pacini tested positive for Huntington's about seventeen years ago. He later denied it, sought no medical assistance, and made no plans for care, they said. Gradually, he changed. He distrusted authority. He lost interest in the Eagles or Flyers. He became disengaged from his nephews, whom he adored. A 2002 marriage ended after two years, when his wife feared he would hurt her, family said. The mother and sisters he was so close with became targets of outbursts and accusations. "I grieved for my brother years ago," Renee Pacini said. "He was a shell walking around."
He developed fixations. His family said he endlessly revised business plans that never progressed. He took business classes at Drexel University, but didn't graduate. In a video posted before his death, Pacini called singer Sara Bareilles his soul mate.
Previous run-ins with police included Philadelphia arrests in 2005 and 2009. In December of 2013, after he had moved back in with his mother, Joanna Pacini contacted Clifton Heights police when her son flew into a rage. She said that she told police he had Huntington's and that they recommended she contact the crisis unit at Mercy Fitzgerald Hospital. (Clifton Heights police did not return calls last week seeking information on the incident.) But, as was his pattern, Joanna Pacini said, her son calmed down. She never called the hospital. She would not evict him. But he gradually became a stranger in her apartment, either ignoring her or yelling at her.
She worked as a lunch lady at Cardinal O'Hara. He stayed home on his computer, the one he most likely used to post the three YouTube videos in which he unloaded rants, threats, and a vow that police would have to kill him if they tried to arrest him.
No weapons were found, but police say they believe Pacini, based on his video threats, was trying to draw them into a public confrontation.
Late on the afternoon of 30 December, Renee Pacini saw the news reports of a fatal police shooting of a driver in Drexel Hill. The car looked like her mother's Nissan. The driver's age matched her brother's. Reports mentioned videos.
A vice president at a software company, she quickly found the videos online. "My heart was pounding out of my chest," she said. She feared she knew the answer, but still called her mother with a question: "Is Joey home?"
As they sat last week in her North Wales house, both women said they didn't blame police. "Joey tried to kill them with his car," his mother said.
His viewing was on 4 January 2015. The family thought it would last minutes, Renee Pacini said. They were stunned when former classmates and friends from the gym came to pay respects and, for hours, shared stories about the Joseph Pacini they once knew.
The man who died last month "wasn't him," his mother said. "My son was a great guy." 

Here are some facts about Huntington's disease:
About thirty thousand people are afflicted with Huntington's disease in the United States and an estimated two hundred thousand people are at risk of developing the illness.
Every child of a Huntington's disease victim has a fifty percent chance of inheriting the gene that causes the illness. Every person with the gene will eventually develop Huntington's disease.
The disease causes the death of cells throughout the brain, but particularly impacts the basal ganglia, which plays a role in regulating emotion, thinking, and motor control.
There are support groups to aid people suffering from Huntington's disease and their families. A national organization is the Huntington's Disease Society of America.

Rico says he doesn't think he has this particular problem, but his Rants do not require police intervention...