merckmanuals mistakes

[ukbill]

Hi today I have e-mailed who I hope is the author of a page in the Merckmanuals (self-help type health manuals)

www.merckmanuals.com/home/childrens_health_issues/hereditary_metabolic_disorders/disorders_of_carbohydrate_metabolism.html


The page titled Disorders of Carbohydrate Metabolism has a common mistake that should have been removed years ago. So I thought perhaps giving him a little information might help...

I hope I have found the right one as there are two medical professionals with the same name.. confusingly.

If its not him I will email the second one.

I will keep you informed if I get a reply.

[charlie]

What is the common mistake?,

I thought it was quite a good article compared to alot of others.

[ukbill]

It is the line ". Milder attacks are treated with glucose tablets, which should be carried by anyone who has hereditary fructose intolerance."

Been as most Glucose tables are at best of questionable purity this is not a safe suggestion..

Besides which the eating of any sugar for an HFI is not a good idea.

But yes other than that it is a good relatively balanced article.

I have yet to get to the bottom of the Livestrong site.. they disclaim all responsibility for the content of the site and name some 3rd party who provide all the content to the site..

Hence confused articles and other misinformed articles which are prevalent about HFI and FM anyway I cannot say about other parts of the site.

I got a good response from the Dr (Yes I got the right one )

An email from him almost by return states..

"Thanks so much for your important inquiry and for alerting me to this concern of potentially important clinical impact.

I am working with the Manual Editors to review, and if possible and appropriate, correct this entry.

Lee M. Sanders, MD, MPH"

So a result.. and maybe a small step towards improving the general knowledge base surrounding HFI.

Hopefully he will join the forum and add his experiences to the group knowledge.

[ukbill]

update had a very unsatisfactory reply from someone who has no idea what HFI is.. below is my reply to them

On 17 September 2012 at 13:40 "Vigus, Janet" <[e-mail]> wrote:
Dear Mr Armstrong,

I forwarded your comments to the Editor-in-Chief, The Merck Manuals [Exec. Director, Medical Affairs]
"Please find attached comments that were sent into our Medical Information department concerning the Merck Manual."

He has replied to me with the comments below:

"The caller appears to have been reading the online Home Edition. I have reviewed our content, and our statements are correct. Hereditary fructose intolerance can cause the level of glucose in the blood to drop very low. Giving glucose is in fact the appropriate treatment for low blood glucose. This fact is uncontested. If, as the caller suggests, certain glucose preparations in his country do not, in fact, contain pure glucose, that does nothing to alter the accuracy of our entry on fructose intolerance. It would be dangerous to follow the caller’s recommendations to NOT give glucose to a patient with low blood glucose."

Kind regards,

Janet

Medical Services


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My Reply..
I cannot see the sense in this reply!

All the Glucose in the USA is very impure and NOT HFI safe.

Just go and look at the patents for the mass manufacture of Glucose and you will see if I'm not right?

One method of producing "confectionery" grade Glucose, which is the variety you are suggesting they take, results in a product while still able to be sold as 100% pure Glucose actually can contain up to 40% "other sugars".

No one who has not got HFI could tell the difference.. We can and it can in time consumption of this can prove fatal to an HFI person.

Glucose in the USA is made from Sweet Corn which has approximately 4% sugar in it. The whole ear is used including the germ where the Fructose resides. This is not removed in the processing.

It is made in the same vessels as HFCS (which is an additional process after the initial conversion to Glucose) and passes through the same pipes and through the same filling out system as HFCS, therefore the risk of contamination is great.

So HOW can you state its safe??

I think the advice is both flawed in fact and dangerous to give to any HFI.

If we are low in blood sugar there are two options.1/. Eat a safe Carbohydrate 2/. Wait a little time until our blood sugar rights itself naturally as it will in a couple of hours. This is the case anyway If we cannot keep food down as a result of a serious poisoning event.

How can this be described as dangerous?

We who have HFI all manage to control out blood sugar levels quite well thank you without Glucose, except for those of us who take Glucose.. who's blood sugar levels "see saw" all over the place as a result of the Fructose contamination.

PLEASE do the same research as I have done and you will see that NO commercially available Glucose is safe for a person with HFI.

Medical grade (liquid) Glucose which is made from rice starch and should therefore be safer, but again this should only be used in an emergency.

It is critically important that an HFI person is allowed to develop a Pavlovian response to sweet tastes, it is our only protection in the real world against accidental poisoning.

Constantly eating Glucose containing foods stops this happening therefore depleting our quality of life.

So there are two compelling reasons not to advise this as a "normal" practice.

In conclusion:-

With due respect this reply is written by someone who has no experience or real understanding of the condition, which is all too common in the Medical profession.

HFI is a serious condition and massively under diagnosed and is treated by the medical profession very poorly.

Please refer this up to the next level of management for safety compliance assessment as the current advise is not safe or acceptable.

Yours sincerely

William Armstrong
Diagnosed HFI in 1981


OK so I was angry all right?

They are killing us instead of helping us!

Its about time the medical profession woke up! Hence my anger.

[colormist]

I think, perhaps, they might be referring to a medical professional administering glucose (in which instance one would presume medical glucose in a hospital to be pure) and you are concerned with individuals taking off-the-shelf glucose (which has no standards for purity).

Perhaps compromising by asking them to amend the statement to "medical-grade glucose" or something similar would be a good solution?

But still, if I were to take glucose, I know my levels would plummet 15 minutes later (if not sooner) and consuming glucose is ONLY a last-resort option. I always make sure to eat food shortly after taking glucose to prevent the yo-yo effect on my blood glucose levels.

Low blood sugar is a result of an underlying condition. Merely taking glucose is like putting a band-aid on a bullet wound.

[ukbill]

Spot on!

Except i would say its more like adding to the problem.. for a very short term gain.

Sorry if I incorectly took a pop at you colormist.. I am so very frustrated at the slow transferance of information to the medical proffesion at the moment.. I know thay cannot know everything about every condition.. but I found the other day a Foundation funding research into a condition which is less than 10% of the potential sufferers as HFI.

I suspect and fear that a lot of children die in early childhood with HFI

Having lost a child myself.. I really want to help anyone avoid such a situation.. believe me it is not something I would ever recommend to anyone.

So the question is wheres our Foundation into research?

The line in the Mecrk manual states . " . Milder attacks are treated with glucose tablets, which should be carried by anyone who has hereditary fructose intolerance."

So these would be over the counter tablets not medical grade.

Perhaps if someone else with HFI contacted Janet, the editor, we might get more response if she knows its not just me spouting off?

[charlie]

At the risk of being shot down in flames....................

I always carry glucose around with me. And I have used it several times.................. The article is a short, concise article and is obviously discussing hypoglycemia in its extreme, not just a bit wobbly. Management should be such that the sufferer can spot the symptoms before they set in and eat accordingly. However a child can't do this as Meg has demonstrated several times and has ended up in the back of an ambulance when she was younger as she has just gone too far. She has then been given glucose urgently. A few other times I know it is too late for just milk or food so I gave her glucose powder dissolved in a bit of water. I always carry a pot around just in case, just as I carry her anti-histamine and epipen incase of allergic reactions to wasp stings.

You then have to back it up with better food once they are coming round to maintain the blood sugar level and I am sure if they weren't trying to submit a concise article they would go into. So as a concise article I think they are spot on which is why I queried what the problem was.

Sorry

[Mimi]

Wow! Give 'em HE**, Bill!

Seriously, I can see both sides of this. I've never used glucose or dextrose, and I confess that this discussion makes me curious. I may have to try them and see how they taste. I've had dextrose candies, but I would never be able to just carry them around for emergencies. As we've discussed earlier, they are too addictive to resist.

In an extreme episode of hypoglycemia, I'd say use whatever works. A glass of milk and (if possible) a very safe carb like white rice would work for me. But I'm glad Charlie has found something that works well for Meg, glucose folowed by safe food. Adults can usually control their environment and get to the food they need. That's not so true for children.

The most frightening thing to imagine is that an HFI child or adult drops into such severe hypoglycemia that they become incoherent or even unconscious. A helpful stranger might mistake that for diabetic insulin shock and administer fruit juice, soft drink, candy, etc. Taking emergency glucose is a lot safer than that.

[charlie]

Bill, you told the publishers of this:

It is critically important that an HFI person is allowed to develop a Pavlovian response to sweet tastes, it is our only protection in the real world against accidental poisoning.

You will only develop your "Pavlovian response" by instant reaction, eg anaphylactic shock, violent instant vomiting or a shock of some sort. Now anaphylaxis doesn't happen with HFI, instant vomiting only happens if you consume a considerable amount - and by denying the kids sweet tastes you will never cause this to happen so this is not going to happen. So the only way is shock, when they have it shout at them? , beat them? give them an electric shock? its never going to happen.

Yes when they get to Megs age you can start to build up a sense of what they sense when they eat it, so they can learn to think about it, but it is never going to stop them completely. Everyone on this board has their "cheats", including you, when they feel the urge. Luckily for some, like you, it is small amounts. for others its sweeter and stronger.

So to tell Medical people about the glucose etc, in such a strong way on behalf of HFI-ers is very wrong, and unfortunately probably just ended up putting backs up. I actually didn't read your full post immediately as I just thought "here he goes again" but when I re-read it when I had time it worried me - big time. Its lucky actually because I would probably have done a "Tammy". The article is for parents and care givers of children if you read the header and in medical emergencies. Adults and older children can learn when they are feeling hypoglycemic but when they are younger you have less control over this, especially as they start school, go to others houses, forget to eat then overdo things, you then do need an instant fix. But as I said, follow it up well afterwards.

I do think your pavlovian theory needs rethinking,

I have posted before but will again: when Meg was a baby I decided to not give her sweets and puddings for the first few years to avoid her getting a sweet tooth for general long term health. This was before I know she had sugar issues anyway, it was just an idea I had. Maybe this saved her being even more ill as a baby as it turns out........ but the theory didn't work she has just as much of a sweet tooth as anyone else. So yes, lets educate and advise but not dictate and frighten.................... Everyone has to find their own safe level using common sense...................

[Tammy]

I very very much agree with all Charlie has said on this. If someone doesn't like the taste, good for them. It will help in the future. BUT if that dislike isn't there, it can't be learned. I still stand by my opinion/practice of satisfying a craving safely.

[Tammy]

Oh, and Charlie - - - - I take that "Tammy" reference as a compliment. lol