LCHAD & Eye Concerns

My daughter, age 4, has retinopathy in her eyes. It has not affected her vision at all. She gets her eyes checked every 6 months. She is taking cod liver oil, 1 ml, twice a day for her eyes. So far this seems to be working and her eyes are not getting worse. There has been no change in them since 16 months when it was first discovered. 

Melissa

LCHAD mom

My son started to have eye problems at age four. Tomorrow is his birthday. He will be seven, which by the way is always a huge celebration for us, since he wasn’t supposed to live past one year or walk or anything, and he is now an active almost seven year old! His doctor had me take him to an eye doctor and they did a test on him that was done outpatient under anesthesia, and at that time he had diminished night vision. Then this last July, I took him in for his routine eye visit and found out that he could not see very well out of the front of his right eye. I was devastated. They had warned me when he was four that retinopathy is a disease that starts at a certain point in the eye and can eventually leave them almost blind, but I still wasn’t prepared for it. So I sent out an email to this list and they connected me with doctor Harding who is doing a study with DHA & LCHAD children with eye problems. He explained to me that because these children cannot break down fat that it deposits in the eyes because they are not producing the DHA needed to break down the fat. I hope that makes sense. We went to Portland, OR, in January where doctor Harding is and we will go back in July to see if the DHA is helping at all.

Julie

LCHAD mom

Regarding the RP problems for LCHADD children. It is my understanding that most kids with LCHADD will eventually end up with RP at some point in time. Apparently, DHA (docosahexaenoic acid) is a fatty acid naturally produced by our bodies when burning fat. Because LCHADD children have such a limited fat intake, they tend to have low levels of DHA. The retina is made up of various different fatty acids and DHA makes up about 40-50% of it. So, if a child with LCHADD is deficient in DHA they will more than likely end up with RP. I am not a doctor, but that is how it was explained to me.

There are two studies currently being conducted concerning DHA supplementation in patients with LCHADD. One is being done at the University of Wisconsin-Madison, overseen by Dr. Jon Wolff. That study began in Jan. 1998 and will come to a conclusion in 2003. A newer one being conducted at the Oregon Health Sciences University, overseen by Dr. Cary Harding, started in January of this year. I know that the one in WI is not taking any more patients, but I think they still have some openings in OR. You can contact Dr. Harding at 503-494-5799 for more information.

We took our two little ones in March of this year to OR. They did some blood tests, an eye exam, visual evoked potential (VEP) test, and an electroretinogram (ERG). The eye exam and VEP were simple and non-evasive. The ERG required that the child be sedated. This meant that the kids had to have an IV to prevent a drop in blood sugars. The ERG took about two hours from start to finish. Once the testing was done, we received a powdered form of DHA that we give them on a daily basis. They each take 1/4 tsp. once a day. I just mix it in their formula…they don’t know the difference. There is no smell or taste to it. There are no side effects.

The study requires that the labs be redrawn again 3 months after the initial visit, a follow-up visit in 6 months, and then every 12 months after that for 5 years.

We were told that a majority of the children involved in the WI project showed signs of improvement. The remainder had no change at all. We figure that if they aren’t getting worse and there is a chance they will actually get better, it’s worth it.

The company that makes the DHA for the project is Martek Biosciences Corp. They do have DHA available to the public. It is the exact same thing used in the studies. We thought about just getting it from them, but we felt that if we could contribute to research, we should. You might ask your doctors about getting it that way. I am sure that Dr. Harding or Dr. Wolff would be able to give them a dosage. I know that some people give their children Cod Liver Oil. I believe the DHA from Martek is about 120 times more potent than the COD Liver Oil and it has no taste or smell or added fats.

Anyway, that is what I know about RP in children with LCHADD. Personally, I would contact Dr. Wolff, 608-263-5993 or Dr. Harding, 503-494-5799 for more information. They are the experts on this subject.

Kimberly

LCHADD mom