“They accused him of being an alcoholic and he wasn’t,” said Portsmouth’s Jesse Strickland, speaking of the late Dan Kirby, a Portsmouth native who later moved to Wheelersburg, where he passed away in 2014.
Strickland said Kirby died of cirrhosis of the liver, which many recognize as a condition associated with alcoholism. But in reality, Kirby’s death was due to a fairly rare – and probably even more rarely understood – genetic disorder known as Alpha-1 or antitrypsin deficiency. The disorder is something Strickland has in common with Kirby and approximately one in every 2,500 people in the U.S.
Kirby is one of two people who will be memorialized Saturday during a fundraising walk organized by Strickly Alphas, an Alpha 1 support group founded by Strickland, which incidentally, explains the spelling of the first word in “Strickly Alphas.”
The other person honored will be Freda Hardin formerly of South Shore, Ky., who passed away due to complications from Alpha-1 last year.
According to various websites, Alpha-1 is caused by a mutation in the serpina 1 gene resulting in insufficient production of the protein antitrypsin or A1AT. Antitrypsin is normally produced in the liver, one of its functions being to protect the lungs from neutrophil elastase, an enzyme capable of disrupting connective tissue in the lungs.
For persons with the Alpha-1 disorder, levels of antitrypsin can drop to 40 to 60 percent of normal levels. In extreme cases, persons carrying a certain gene, can have less than 15 percent of the normal amount of antitrypsin.
Among other problems, as already indicated, low levels of antitrypsin can cause impaired liver function and may lead to cirrhosis and liver failure. Alpha 1 also commonly causes COPD like symptoms and can lead to serious lung diseases such as emphysema but more commonly causes shortness of breath, wheezing, asthma and related issues.
There is no cure for Alpha-1. Treatment means weekly infusions, which Strickland said can cost up to $10,000 each. In many instances, insurance can cover most of the cost. Proceeds from the annual river walk go to the Alpha-1 Foundation in Florida, a nonprofit research group. Last year, the local walk raised $10,276.
In addition to fundraising, Strickland said another purpose of the walk is to raise awareness of the genetic disorder. Still, he would not admit to any exasperation with the apparent lack of knowledge surrounding Alpha-1.
“It’s not frustrating because we know people are not aware of it,” Strickland said, adding before he was diagnosed he had never heard of the disorder either.
“The walk raises awareness for Portsmouth, where we have a high rate of the disease in our area, primarily due to carriers of the gene staying in the area and marrying one another, transferring the genes to their descendants,” Strickland told the Daily Times last year.
Now 62 and retired from the cleanup crew at the Portsmouth Gaseous Diffusion Plant in Piketon, Strickland said he was diagnosed at 54 and began receiving infusions eight years ago.
Anne Dissinger is another Portsmouth resident carrying the Alpha-1 gene who has been receiving infusions for the same amount of time as Strickland. She said she endured some shortness of breath most of her life, but the problem began to worsen in 2010. Her husband insisted she see a doctor and ultimately, she was diagnosed with Alpha-1.
According to Dissinger, living with the disorder can be difficult. For example, she can’t be around cigarette smoke or certain common chemicals. “We all have to be very careful,” Dissinger continued, adding exercise is incredibly important.
On occasion during the winter, she said she wears a mask to church, fearful of persons with colds and so on. Still, Dissinger said those with the disorder generally handle it in the same manner anyone copes with a chronic illness.
“You deal with it by just keeping moving forward,” she said.
The Alpha-1 Portsmouth River Walk is set for the Scioto County Welcome Center, 342 Second Street in downtown Portsmouth. Registration begins at 10 a.m. with the walk following at 11 a.m. Strickland said after crossing the street to the flood wall murals, participants will release balloons in honor of Kirby and Hardin. They will then take a short walk around the flood wall.
Preregistration is available online through the Strickly Alphas Facebook page. From there, follow the link to the registration page. A $25 preregistration ($10 for children) gains a participant a purple T-shirt, purple being the official color of Alpha-1 awareness, just as pink is used for breast cancer.
For the record, Dissinger and Strickland noted November is Alpha-1 Awareness Month. Although there is a national designation for the month, Ohio is the only state with a formal proclamation declaring November Alpha-1 Month. Strickly Alphas chooses to hold its walks in October out of respect for colder November weather.
Dissinger said organizers are hoping attendance at Saturday’s walk reaches 50 to 60 people. She noted for the first time, the Alpha-1 Walk will have competition from a local Alzheimer’s Walk. Both Strickland and Dissinger said the event will include raffles for unique gift or themed baskets donated to Strickland’s support group among other fundraising activities. Local musician Steve Free will once again perform.