Gene Hanson of New London He has a disease that only affects one in 15 million people and for which there is no cure. But does Gene Hanson of New London let that get him down? No way. “Two years ago I told Shirley (his wife), ‘no matter what the future holds, I’m going to stay positive, I’m going to enjoy life.’” He also told his wife if he ever sounds like he’s getting down, to kick him in the butt. “She hasn’t kicked me yet,” he said with a chuckle.
Hanson has been diagnosed with a very rare disease called Primary Lateral Sclerosis (PLS). While this devastating disease pretty much destroys life as you know it, it isn’t fatal. Hanson, who’s a young 81-year-old, doesn’t feel sorry for himself at all. He plans to live life to the fullest and is taking one day at a time. He’s eager to talk about his disease and its progression in the hope that his story may help someone else. Back in 2005, he lost his balance, fell and broke his arm. He figures that could have been the beginning of the disease. In the summer of 2008 he started losing his strength and getting weak. “I started falling, my speech started deteriorating right away and I just had a number of problems.” That August he went to see a neurologist where he underwent all kinds of tests. “He (the doctor) knew it was a neurological disease but wasn’t sure what.” Finally in March of 2009, the neurologist told Hanson he thought it was PLS. “He said it’s going to get worst and there is no cure.” That wasn’t the news Hanson wanted to hear. He and his wife made three trips to the Mayo Clinic where he went through all the tests again. “They were probably 90 percent and better sure that it was PLS, and I’m firmly convinced that it’s PLS.” PLS is a progressive degenerative disease of the upper motor neurons, Hanson explained, where ALS affects the upper and lower motor neurons. While there is no cure for PLS, and it’s disabling, it’s not fatal like ALS is. PLS usually begins with lower-extremity stiffness and pain due to spasticity. With progression, people with PLS develop balance problems and lower back and neck pain. As the upper limbs become affected, activities of daily living become difficult to perform. Speech impairment may occur as a result of damage to the muscles involved in forming words. Swallowing and breathing may be compromised in the later stages of the disease. Hanson said when he got back from Rochester, he continued to go downhill. “It was about a year ago I started using a cane around the house, but I hated to use a cane uptown.” He said it finally got to a point where he had to use a cane. “Then it kept going down and down and down until I finally had to use a walker. That bothered me. I told the neurologist, ‘people will think I’m handicapped,’ and he said, ‘Gene, you are.’” When Hanson returned from the Mayo Clinic, he immediately got involved in speech therapy, and has been going to speech therapy for over a year. “I’ve been in physical therapy, occupational therapy, pool therapy and none of its going to cure my problem. The goal is to try and slow down the progression of the PLS.” Hanson has learned the one medication that does the most good as far as spasticity and stiffness in the legs and gives him some relief is Baclofen. “It’s pretty potent medication.” You start by taking it orally, he said, and you’ve got to get it into the spinal fluid. “By the time you take it orally and it works its way through the system it gets kind of watered down, you’re not getting enough, so they keep upping the dosage.” There are a lot of side effects, he said, noting he gets the dry heaves, he can’t sleep and there are all kinds of other problems. A friend of his in Lakeville that has the disease had a Medtronic Baclofen Pump implanted and it did him a lot of good, so Hanson asked his doctor about it. “I said ‘I’m willing to do anything,’” when he talked to him about the implant. His doctor notified St. Cloud right away since the hospital there does pump implants for some people with MS and other problems. “They tested me to see if the pump implant would help me. I went to St. Cloud in May and they gave me an injection right into the spine and then they monitor you every hour on the hour throughout the day to see if that helped you.” Hanson said you get a big improvement on the spasticity immediately, then as the day wears on, the medication wears off and you go back to where you were. “That proved that the pump would help me.” On June 23, Hanson had the pump implant. He spent six days in the hospital. Hanson described the pump as being round like a hockey puck. He said a person has to be on the heavy side or they don’t qualify because they have to bury the pump a half inch inside the surface of one’s body. A catheter is attached to the pump and goes around and back to the top of the spinal area. The pump disperses the medication and you’re getting 100 percent of the medication, where orally it gets watered down. The pump is programmed by a little computer, and the day he had the surgery they programmed it at the normal setting. “On days two, three, four and five in the hospital I felt really great and the day I was discharged I went downhill drastically. I was really weak.” Hanson was told by the doctors, “Well, yesterday you were prancing around like a young horse. You may have overdone it.” He has been back to St. Cloud 18 times to re-program the amount of medication he receives at it hard to find the perfect amount. Hanson is also working with a physical therapist and that’s helping him as well. He said the therapist is getting very aggressive during the workout. “You’d think she was a chiropractor, and I have seen some improvement. I can get in and out of bed a lot easier, and I can get into my car a lot easier.” Hanson said he can still drive his car. “People wonder when they see me struggling. In March of last year I had two braces built and designed especially for my legs. I don’t wear them in the house, but once I go out, I wear them.” He can walk better with the walker, he said, since that gives him support. It doesn’t hurt when he tries to walk, but his legs are heavy. Swallowing hasn’t been a major problem for Hanson, but it’s been starting in where all of a sudden he chokes. “I could be eating, drinking, sound asleep or reading a book. It’s not a major problem but it’s also part of the PLS.” He said the speech therapist and x-ray technician at Rice Hospital were going to run a video where Hanson would have to drink liquid, and eat certain types of food to see if there’s anything else going on and if there isn’t, then they would know it’s PLS. There are many side effects with this disease and one includes trouble using silverware or cutting meat. “Sometimes Shirley helps me out.” Using utensils is a little bit worse, he said, but the occupation therapist gave him some special silverware with large handles to make it a little bit easier. “She’s given me things to help me button the buttons on my shirt and putting my socks on. There are a number of things they can do to help you.” Hanson is part of the study being done by the Northwestern University in Chicago. The university received a special grant to study PLS and ALS to see how they intertwine. “We spent a lot of time doing the family history, it was 19 pages long. We had to send blood work down to them, and that’s kind of hanging there. I haven’t heard anymore.” Hanson said his PLS problem really affects both himself and his wife. “I used to do more things around the house to help, and I still try to do my share but it’s more and more difficult.” All in all, Hanson said, he’s not complaining, life is still great. He has a motorized chair he uses in the house and outside during the summer. He said he enjoys going outside during the summer and reading a book or visiting with people who stop by. “In the winter we’re kind of in the house.” Also, he said, they haven’t been going out as much as they used to, it’s too dangerous with the walker. “I have fallen eight times. We have a list of six to seven husky friends because if I fall it takes two good guys to lift me. I’m dead weight, I can’t help at all.” People are good about it, he said. He fell one day last March and lay outside on the ice and in the melted water. He couldn’t get up even with his wife’s help. “Finally we had to call the ambulance and they got me up on my feet.” That’s one area you have to be concerned about, he said. “If you fall and break a hip, you have a major problem.” Hanson said when he was first diagnosed, he wasn’t really shocked. “I think one time I came home and I may have broken down a little bit, but it dragged on and dragged on so you’re kind of building yourself up for it.” He said his biggest fear is getting weaker than he is right now. “If I lose any more strength, it’s either going to a nursing home or having people help, so each day I say ‘I feel a little bit better today’ and she’ll say ‘well, I don’t think you do.’” It’s so important to stay positive, Hanson said. People ask him how he’s doing and he always says he’s fine. “All in all, I’m not complaining, life is still great.” Hanson said he’s going to enjoy life as much as possible no matter what. “I have told some of my friends ‘I might be going downhill but I’m gaining speed.’” And Hanson, who describes himself as impatient, says his favorite prayer is ‘God, grant me patience but be quick about it.’”