Update on Adam Fulton

13 years old with LCHAD

It is hard to believe I haven’t written about Adam for almost 6 years! He will be 14 in December and will start high school next fall. He is now a big 8 th grader at is performing arts middle school. He is a very good student getting all As (some A+s) and Bs. He participates in Concert Choir, Men’s Choir, and Chamber Choir. He is still on a summer swim team, and although he's still rather slow, has mastered the four strokes. He also participates in one or two musicals a year at Children’s Musical Theater of San Jose.

Since I last wrote Adam has participated in several LCHAD studies around the country. He is an excellent patient and doesn’t mind doctor’s offices or hospitals. If he were skittish about either, we wouldn’t have him participate in so many studies. His participation has allowed him to travel around the country and meet the medical professionals most knowledgeable about LCHAD. Some of these studies are still accepting participants so please email the doctors, their facilities, or myself if you or your LCHAD child is interested. Some have proved extremely beneficial to LCHAD children and some others with fat metabolism defects.

In 1997 Adam entered a 5-year study at the University of Wisconsin run by Dr. Melanie Gillingham and Dr. Cary Harding, Madison (now at Oregon Health Science University) taking DHA for his retinal health. He would have a complete eye workup every year including an ERG and VEP to observe changes in his retinas. Unfortunately, there appeared to be a gradual increase of the abnormal pigmentation in his retinas, which were first observed when he was 3 years old. But his vision remained in tact.

In 1998 Adam was at Presbyterian Hospital, Columbia University in New York City having a PET Scan. Dr. Steven Bergmann is studying how fat is metabolized in the heart of LCHAD patients. Dr. Bergmann determined that fat was metabolized in Adam’s heart in the way, which he expected. We had a wonderful week in New York City pre 9/11 and visited with our son Dan who was working on the 52 nd floor of the World Trade Center. We all dined at Windows on the World on the 107 th floor.

And in 2001 Adam entered an 18-month study at Baylor ‘s Center for Metabolic Diseases in Dallas to test C7 Oil, odd chain oil. Dr. Charles Roe led this study predicting that odd medium chain oil would be better utilized for energy in the body. Adam was assigned massive amounts of this oil and every 6 months was tested for endurance and muscle strength. Unfortunately, Adam wasn’t able to tolerate a large enough amount of the oil to be beneficial. Dr. Roe later modified his protocol to only include participants less than 8 years of age as they had less trouble digesting the oil. Dr. Roe recorded some extreme improvements in muscle strength and endurance in several of his participants.

This past summer Adam was back at Oregon Health Science University in a 2-week diet study run by Dr. Gillingham and Dr. Harding for LCHAD children, which was to determine whether they would be more energy efficient on a higher protein diet. The first week the patients ate a regular high carbohydrate diet and the second week some of the carbs were replaced with protein. Adam did better on the higher carbohydrate diet and actually had a ‘hurty leg’ attack the second week. It was determined for him he could do well on a protein level about 10% less than the study.

Besides all of the studies in which Adam has participated, we have had a wonderful trip to Europe in 2002 and a trip to Japan in 2004. In Europe we made a special effort to meet LCHAD families in Sweden and Germany and Dr. Tiini Tyni, an LCHAD expert in Helsinki. Since Adam was in the C7 oil study at that time, it took careful planning to be sure he had his oil in Europe. Fortunately we were staying with four of my pen pals of 40 years and I could send the oil ahead to their homes. We never could have carried the necessary 4 bottles he needed on this trip. Staying with families or at condos on our trips make it easy to have a place to refrigerate and prepare Adam’s milk. He now takes 3 6 ounce bottles of fat free milk a day, one in the morning, one in the middle of the day and one before bed. In each bottle is 1T of MCT Oil and 1t of Carnitine. Adam takes a DHA, a multivitamin, Ester C, Vitamin E, and Lutin pill daily.

Unfortunately, Adam noticed a change in his vision this summer. After consulting with older LCHADs and medical experts, there seems to be a consensus that the abrupt change may be due to puberty. He saw his Ophthalmologist recently and he thinks that it isn’t an increase in retinal pigmentation but just that he needs glasses. We will go tomorrow to see an Optometrist. He has also been referred to a Retina specialist who he will see in December.

Adam has become very good at managing his care when he starts feeling bad, i.e. 'hurty legs’. He drinks massive amounts of sports drinks like Gatorade and in Japan Aquarius. Usually he can overcome the Rhabdomyolsis before he has Myoglobinuria and has to be hospitalized. He hasn’t been hospitalized in over 2 years. Unfortunately, though, Adam doesn’t seem to be able to control his activities so that he doesn’t have a ‘hurty leg’ attack. He becomes involved in ‘just being a boy’ and hanging out with his friends and forgets to curtail his exercise and drink lots of fluids. Maybe someday as he matures, he will be able to learn to prevent LCHAD episodes. He is fortunate to have very supportive friends who don’t make fun of his lack of athletic aptitude and strange beverages. All in all Adam is doing fantastic and is a very loving and caring young man.