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Apr 3, 2018 Latest post:
Apr 11, 2018
Ben was diagnosed in 2009, at age 14, with Adrenoleukodystrophy (“ALD”), a genetic neurological disorder similar to multiple sclerosis. It is caused by a rare defect in the X chromosome. A defective protein in the blood causes a build-up of acids, which strip away the covering, myelin, on nerves in the brain and/or the peripheral nervous system. This de-myelination leads to nerve damage that can affect mental and physical functions in the body.
There are many types of disease caused by the ALD gene. Young boys who are affected by the disease (Cerebral ALD or CALD), which starts in the brain, usually die within 3-5 years of onset. The adult-onset version, also called Adrenomyeloneuropathy (AMN) initially affects the peripheral nerves, starting to impact the feet and work its way up to the waist. Many people with AMN do not have brain involvement and may live full lives though their legs weaken and lose their function over time.
Ben had a brain MRI at age 14 for un unrelated condition. The MRI revealed a pattern in the brain that indicated demyelination (damage to the nerve covering). A genetic test was definitive for ALD.
For years, the disease seemed to be paused, and numerous MRIs showed no signs of advancement. Ben had no symptoms until he was almost 23, in the summer and fall of 2017. Just after he started his first job after graduation from the University of Southern California, as a banker in Los Angeles, he started losing feeling in his feet and lower legs and had difficulty concentrating. He was forced to take disability leave in October. He has some difficulty walking.
Like most people with ALD, Ben also has a related condition, Addison’s Disease, in which the adrenal glands stop functioning. Ben takes daily prescription medications, so his body can respond to stress and infections. Ironically, just as the ALD was affecting him in October, Ben’s prescription for Addison’s was unexpectedly interrupted for 36 hours, which could have been fatal. It is not clear how much of Ben’s difficulty in performing his job was caused by the gap in his prescription intake.
Complicating matters further was our inability have Ben seen by ALD specialists immediately. His ALD doctor of nine years switched to academia. Very few doctors specialize in the treatment of ALD, and they are far from us geographically. We have spent the last four months in a dizzying maze trying to schedule travel, appointments, find new experts, and plan for treatment. We have also been dealing with his bosses, the human resources department at his bank, the private disability leave administrator, two Blue Cross insurance companies, and several new doctors.
When Ben saw his new ALD team at Stanford in late November, they were relatively pleased that his condition was not life-threatening. At that point it seemed the only treatment, a bone marrow transplant, would not be necessary given his condition. A successful transplant stops the progression of symptoms but does not restore any lost mental or physical function.
However, in early February 2018, the transplant team in Minneapolis recommended the bone marrow transplant. Since his original diagnosis, Ben has been seen several times at the University of Minnesota for neuro-psych testing and transplant evaluation. The doctors there have extensive experience and an impressive record of performing hundreds of transplants for ALD and related conditions. Ben’s endocrinologist of nine years gives them his highest endorsement. We are now planning for the transplant.
This development has obviously created additional stress on all of us. The current tentative start date for the transplant is the first week of April. The process takes up to six months, until approximately October. Ben will be hospitalized for 2-3 months. Then he will be required to stay within 30 minutes of the hospital, in the Cedar-Riverside neighborhood in Minneapolis, for an additional 2-3 months. It is possible he could relocate near a hospital out of state before the end of six months, depending on his condition.
A transplant would resupply Ben with blood that does not damage the myelin. However, a bone marrow transplant is risky because doctors kill the immune system with chemotherapy before introducing the marrow from a donor. When successful, the transplant generates new healthy blood and a new immune system. The ALD will likely advance somewhat during the transplant process, because Ben’s body will be weakened.
The doctors are optimistic about Ben’s chances of surviving the transplant (90 percent) and of stopping the progression of the disease.
Ben's parents, Ken and LuAnn, are planning to spend significant time in Minneapolis (LuAnn will live there while Ben is undergoing treatment). Ben's sister Annie will also be spending time with Ben in Minnesota, as will Ken's girlfriend Shelly. The family has made plans for lodging.
We appreciate the support of our friends and relatives during this challenging time.