Dr Tolan info

[ukbill]

A message from Dr Tollan which will be of interest to all.

[colormist]

I saw this when it was posted on social media. This explains why I get so thirsty all the time. Also explains why I go hypo faster when I get hot and sweaty. Every where you turn there's a new danger with HFI.

[ukbill]

Less dangers now

We are all learning fast new stuff all the time.

By learning about our condition then we can live safer healthier lives as a result

It looks like we might be getting some traction in the medical research area as well

I always knew I could go Hypo when thirsty but did not know why.. now we do ..

[Stefanie (Ziba)]

Bill, Can you send him the link to my petition? I feel like he would be interested in making sure the NICUs are aware of Fructosemia. Maybe he would have ideas on who else to send it to...up to 2300 sigs.
www.change.org/u/me?source_location=my_petitions_dropdown#started

Regarding starch-- this is interesting as I have found corn starch (much like you have) to be so helpful. I wonder if certain starches are worse than others. Very exciting about possible meds. This does explain why my son who is super athletic can run for hours indoors but gets overheated outside (in sunny sunny Florida).

[ukbill]

It was not my message I have copied here from facebook.. because I thought some people might not see it on facebook.. and facebook is so very transitory.. see something one min and never find it ever again.

I will forward your petition to Dr T Cox the leading UK metabolic chap though.

[ukbill]

OK its on facebook Hereditary Fructose intolerance / HFI page I think posted by Amanda Johns Milner

[Stefanie (Ziba)]

I had so many questions mulling around this week that I emailed Dr. Tolan and he called me yesterday. Here is the summary of our phone conversation:


Are there new recommendations regarding Carbohydrate intake for HFI patients?

In his most recent paper last year, he said he cited animal model studies that show when they give mice a diet high in starches (rice, breads, pasta and potatoes), these high glucose foods may induce a pathway to produce fructose endogenously and lead to dehydration, long term damage to liver and kidneys. He gave the mice a diet that was high (80%) in these starches, but even with lower percentages it may create a chronic if not acute problem. This means avoid pediatlyte/dextrose/glucose unless absolutely necessary (i.e. severe dehydration). Remy happened to have a 103 fever at the time of our conversation, and he said it is best to just push the water. He should not get dehydrated as long as he is not consuming fructose or glucose. As for diet, he said as close to an Atkins diet as possible is the best course.
*Remy’s daily diet is dairy, eggs, herbs, oils, meat/fish, oats and crackers. Remy only like oats (lo glycemic) and crackers (less gluten and starch)…. perhaps his body is telling him something. Maybe this is why so many mention they cannot eat breads right out of the oven…?


Can I apply essential oils topically; many have proven anti-viral properties? Yes, all essential oils – even citrus- should pose no problem since they will not enter the metabolic pathways by skin absorption.


Any updates with genetic testing? Many of us were negative on the HFI test and my son even showed nothing on Whole Exome.

Recent (in the past year) data shows that 30% of those who have HFI will not show up on genetic tests because their mutation is still unknown. Whole Genome may be better, but the only definitive way to know if someone is lacking the Aldoses B enzyme is by needle biopsy. The liver won’t lie. But, even without a biopsy, he agreed that someone who has the symptoms of HFI and does well on the HFI diet, probably does have HFI even if tests are inconclusive.

I told him about my change.org petition to get Sweet Ease out of the NICU and he said he has long worried that infants were dying because of practices like this.

He also said he wished he had time to be on the proboards with us but he is happy we all have each other.

I went to Google scholar to find his 2018 articles, and this one is the most salient…gives us hope!
www.jci.org/articles/view/94427

*Note the discussion about ATP depletion in the article. Carnitine stimulates ATP and Remy has been on it for two years now. His health improved dramatically since he began taking it (even though he had already been fructose-free for 6 months).

[tummyache]

I am so glad to hear what Dr. Tolan has said about responding to the diet as being so important to the DX of HFI. I am one of those who also has negative DNA testing (23andMe) to the top 4 ALDOB snp's, no biopsy because doctors decided it was too invasive, and then said that my symptoms where just too obvious to do further testing. Anyway, the diet works for me...except when I mess up, or someone sips me "a micky"(like when a sugar slips into an injection or medication). 30% -- that' a lot of us! Full gene testing is so expensive, and even then no sure answers -- there is still so much not known yet -- I'm not sure it is worth the money. Also, for a young person, getting a formal "pre-existing condition" label of HFI right now may not be the best thing until the politics in the USA stablizes concerning health care. Something else to think about.