By Olivia Burns (guest blogger)
Bob’s foot shook with the thoughtless determination characteristic of Huntington’s Disease. His uncontrolled movement was erratic and unstoppable—that is, until Exeter rested his head on the jerking foot with equal unconsciousness. Completely disregarding the abnormality of the situation, Exeter calmed the rogue appendage…
I listened as Nila openly and eloquently spoke of her husband, Bob, and his experience with Huntington’s Disease (HD) and his most effective treatment against it: his service dog, Exeter. Mixed emotions whirled in my mind. I was thrilled to be personally hearing the only publicized account of an HD service dog, but simultaneously torn with sadness knowing the man she affectionately talked about could no longer speak for himself because of the disease. I wanted to share something comforting and smart. I wanted to show Nila that I cared and understood, but my planned discussion topics gurgled with the scientific jargon through which I had initially come to know HD. My words jumbled in inarticulate sentence fragments. Thankfully, Nila accepted them as the honest forms of interest I meant them to be. Talking with her made me realize how far I was from the isolated classroom where I initially became interested in HD.
It was my genetics and ethics professor who first explained HD to me. HD is an often taught example of a genetic disease caused by a change in a single gene. This gene is called Huntingtin, named after the doctor, George Huntington, who first documented the hereditary origins of the disease in 1872. Sometimes the Huntingtin gene contains extra DNA, which produces an elongated protein. This is considered a mutated Huntingtin gene, and it is the source of HD. Like a nosy neighbor, the long protein created from the mutant gene cannot simply mind its own business. It takes on new functions and invades areas where it doesn’t belong, specifically nerve cells. The abnormal Huntingtin protein fragments and clumps within cells, disrupting their function. Click here to watch a video that further explains the genetic cause of HD.
Typically, the disease process is slow, and symptoms are unnoticeable until midlife. Overtime, movements, decisions, and emotions are no longer controllable. The mental and physical deterioration in Huntington’s Disease can last over a decade and always leads to death. Although researchers are pursuing various cures, there is no current treatment to stop or reverse HD. Managing symptoms is the main priority. Drugs to control involuntary movements and lessen depression are often given to help with the physical and emotional impacts of HD. However, medications can worsen other symptoms, and must be regularly reviewed in order to assess effectiveness. This is where Exeter comes in; the only side-effect of the Labrador’s services is love, which he gives unconditionally to Bob and his family. He retrieves needed or knocked-over items, steadies Bob’s gate, and reduces stress.
Monitoring stress is particularly important for HD patients, because high levels of it can augment their symptoms. Bob regularly reached these levels when he was without Exeter. Before his official diagnosis, Bob got into multiple car accidents because of his yet-to-be-realized inhibited cognitive and motor skills. As if that alone wasn’t stressful enough, police found that he could not pass sobriety tests for the same reason. They would arrest him until urinalysis came back negative for alcohol presence.
Nila now recalls the accidents and arrests as obvious indications of HD, but at the time, neither she nor Bob were familiar with the disease. Typically, people presenting HD symptoms know immediately the cause, because a parent suffered similarly. Bob was adopted though; he had no family history to draw from. Therefore, he continued to live unaware, attributing his irritability, fatigue, and depression to stress.
“They call those soft symptoms,” Nila said, “but they really aren’t so ‘soft,’ because those are the ones that ruin your life.”
The personality changes started back in 2003, followed by the decrease in motor skills leading to the string of car accidents in 2006. The diagnosis did not come until 2007. “I was falling apart. I had been through so much with him already,” Nila shared. “But I knew he had to get help for himself, because I couldn’t do it for him.” At that time, Bob, Nila and their 3 sons had decided to move from Colorado to Northern Idaho. Bob and Nila wanted to start their own custom closet business. Both were experienced in construction, so it seemed not only like a good change, but one with an easy transition. “But Bob just couldn’t get the hang of the new business. He couldn’t remember how to install a light bulb.” Bob came home that day and admitted to Nila that either he was stupid, or something was wrong with him. He was open to getting help.
Because they were new to the small Idaho town, they did not have an established physician to consult. A friend recommended a local family doctor, who struggled to find a connection between the described symptoms and a physical condition. He thought perhaps it was a deficiency in vitamin D, but referred them to an excellent neurologist for further diagnosis. However, like rides in Disneyworld, the best doctors always have the longest lines. The appointment with the neurologist was not for 3 months, and Bob’s symptoms continued to worsen. Like any efficient amusement park goer, Bob and Nila went to another ride—or in their case, neurologist—to fill the time. This neurologist began treating Bob for Parkinson’s disease, but these treatments only aggravated his symptoms. Bob and Nila quickly realized the lack of progress being made and began doing their own research to find better treatment options. At an independent clinical facility, Bob took cognitive and motor skill tests evaluating his functional capabilities. At home, they turned to the Internet for information about possible diagnoses. In a blur of a few days, Bob got the results from his testing back. “The doctor came out in tears, saying that Bob should no longer be allowed to do anything. No driving, no building, not anything.” It was also then that Bob and Nila were first introduced to HD through an online article on rare neurological diseases. Everything that Bob had been experiencing, the reasoning for the poor outcome from the testing, was spelled out right in front of them.
“The neurologist [who diagnosed Bob with Parkinson’s] refused to test for it, though,” Nila expressed with frustration. So they went back to the family physician. “At the time, there were no cases of Huntington’s Disease in the area. No doctor wants to diagnose it. It is only bad news for a patient,” said their family physician when asked to recall the moment he approved the HD diagnostic test. After Bob and Nila shared with him the parallel symptoms from the online HD article, the results from the skills tests, and the refusal of the middleman neurologist, the doctor agreed that testing for HD was the right course of action.
Just before the awaited appointment with the recommended neurologist, Bob and Nila got a call from their family physician. ”We knew it was bad news. If it was good, he would have just told us over the phone.” I asked if the diagnosis provided any sort of relief; after 4 years of inexplicable changes in personality and physical ability, they finally had a reason for it all. “No,” replied Nila without hesitation. “It was scary, because we had our three sons who were now all at risk.” Each of the boys dealt with the diagnosis differently. Only one opted to be tested himself, because he wanted to have a family. He discovered that he did not have HD, and now Nila and Bob are happy grandparents of their son’s two children. Nila said she expects her second son will be tested soon, as he looks at starting a family of his own. The youngest, in high school at the time of his dad’s diagnosis, has struggled the most. “He doesn’t want to have anything to do with Huntington’s Disease,” Nila said, the pain of a worried mother—a pain I cannot begin to fathom—audible in her voice.
Shortly after receiving the diagnosis, Bob and Nila got into the neurologist they had been waiting so long to see; he was the one to suggest getting a service dog. Bob resisted, thinking it would only draw more attention to an already demoralizing situation. “I told him that maybe he didn’t need one now, but it might be nice to have the dog for later when he could not do as many things himself. We really only thought a service dog would help with the physical stuff,” Nila figured. After all, that is what service dogs are trained for.
Companies responsible for the training of service dogs carefully research the needs of specific diseases, determine which dogs best serve those needs, and match patients and dogs accordingly. Patients therefore do not necessarily choose their dog, although there are compatibility requirements for receiving one. For Bob and Nila, this meant 3 days of “connection training” in California. Over the weekend, a group of 10-15 patients, all with varying diagnoses and needs, meet up to 30 different service dogs. Within the group of dogs, there are certain ones trained to meet certain patients’ needs, but the patients do not know which these are. Even without this knowledge, patients typically connect with a dog trained for their needs, and a dog bonds best with the patient they are trained to help. Company trainers and handlers present during the weekend actively look for this mutual relationship in order to successfully match a service dog.
Unfortunately, there can be times when a patient does not interact well with any of the dogs. “Bob didn’t connect with any of them. It was the end of the second day, and we were getting very discouraged.” It wasn’t until the third day when they brought in a dog from another facility, Exeter, that it changed for Bob. “Bob got down on the floor and went head to head with Exeter, and they just rested together and were calm. I could have cried.” They would not know if Bob and Exeter were matched until the next day. Someone else could have a better connection with the dog, which made Nila nervous. Bob did not have a doubt though. “Bob knew. It had become so difficult for him to get up in the mornings, but the day of the match, he woke up early out of excitement. He kept saying he was going to get Exeter.”
Nine years later, Bob and Exeter are still a perfect match. Bob’s physical and behavioral symptoms continue to worsen according to HD’s dismal trajectory, but Exeter has remained a constant companion through the changes. Bob’s neurologist feels that the dog’s persistent presence has helped limit the progression of his symptoms by reducing his overall incurred stress. Case in point: on a particularly bad day, Bob left the house alone, donning only board shorts in 50˚F weather. A concerned neighbor must have alerted the authorities, because minutes later police cars passed by. Seeing the cars and realizing Bob was nowhere to be found, Bob’s son rushed out of the house, winter coat in hand and Exeter by his side. The police remained in their vehicles, watching Exeter, in his service dog vest, calm Bob. This bad day was not so bad compared to Bob’s previous encounters with the police. Nila credits Exeter for diffusing the stressful situation without police involvement.
That incident is just one of countless times Exeter’s presence has led to compassion rather than judgment. HD is caused by a single mistake in a person’s DNA but its detrimental symptoms that grip the entire body, wrenching away free will. Judgments may be cast thoughtlessly at a stumbling, incoherent HD patient. A service dog can mitigate these, bringing awareness where there is misunderstanding. HD patients need to be cared for with love and respect. More than any person, Exeter knows this.
Below is a moving video of Bob and Exeter and their extraordinary relationship.
Click here to learn more about the basic biology of Huntington’s Disease.
Interviewer’s Note: I discovered Bob and Exeter’s story by accident. I knew I wanted to explore the lived experience of HD, but I didn’t know how. I came across the video of Bob and Exeter on YouTube and was inspired. What a simple yet elegant way to fill the void of information on HD with my passion for animals. However, if there was little information on those living with HD, there was even less on HD patients living with service dogs. I asked my dad, a family physician, if he had ever experienced either. Instead of answering, he wanted to see the video I referenced. As he watched, he said “I know him.” Through this series of serendipitous events, I was connected with Nila, who revealed to me that my dad was the doctor to test and diagnose Bob with HD.
I would like to thank Nila for taking the time to share her family’s lived experience with Huntington’s Disease. I also would like to thank my dad, Dr. Burns, for connecting me with Nila. Dr. Burns’ shared thoughts were permissioned by Bob and Nila.