DWQA QuestionsCategory: QuestionsDeveloped severe form of VLCADD in adulthood
shona Pippet asked 3 years ago
Hi, I am after some information on VLCAD in adults and how to best address the issue of exercise without causing muscle pain and stiffness.
I live with this everyday and my calves, ankles and feet seem to be the biggest nuisance at the moment. I it is like a peripheral neuropathy and can extend up to my goin when it gets bad during a hospital stay of 5 days to treat my condition which was heading towards  Rhabdo episode I realized that the dextrose drip they put me on cured the pain and icky feeling in the legs. Potassium level dived along with the CPK going up and I was hypoglycemic. They put me on potassium drips as well but my potassium is not back to it\’s normal value yet and I have been home for two weeks. Calf muscles still not co-operating well and not want to move so I am taking it easy until they are ready, I was at near total collapse before I went to the hospital I am 68, Have I passed my use by date and will the leg muscle come back to normal with careful exercise. I am investing in a motorized wheel chair for use at the shop as walking was becoming difficult be for this event. Icky feeling is back in the feet ankles and calves again and I still recovering from the hypoglycemia. I hope you can help with this as it has been going on for too longl now and I am trying to stick to the diet my Metabolic team put me on but it isn\’t helping. I have a rescue drink called SOS power not sure if I should use a little on bad days before bedtime, or go the whole hog and drink it all day without food for a day and then call the team. It is progressing and my brain seems to be affected too, it goes a bit la-la towards night, it actually got better on the dextrose drip? The Rhabdo is scary as it comes out of the blue!
Kind regards. S Pippet

1 Answers
Keith McIntire answered 3 years ago
Mr/Ms Pippet, you are certainly experiencing classic symptoms of VLCAD deficiency. There are numerous treatment options to consider including total daily calorie intake, distribution of calories across food categories and time, and additional measures aimed at decreasing the inflammation that is part of VLCADD. In addition, the new medication triheptanoin (Dojolvi) may help control your pain and rhado episodes. I’m sure you understand that I can’t provide specific advice on each of these options as I don’t know your complete history, current treatment plan, or laboratory indicators of metabolic balance. However, I suggest that you meet with your metabolic team to review your current treatment plan in detail and work to one that better controls your symptoms. I hope this helps. Dr. Vockley.