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Great Advice on Pyrrole Disorder (Pyroluria)

Discussion in 'General Treatment' started by Sherpa, Dec 3, 2014.

  1. Sherpa

    Sherpa Ex-workaholic adrenaline junkie

    Here is a great beginner's article on Pyroluria - written by Mr. Steve Thompson of Sydney Australia - and posted on the Facebook Pyroluria Group.

    A few suggestions for people who are new to Pyrrole Disorder

    First, it is important to have an accurate diagnosis of Pyrrole Disorder from a positive Urinary Pyrroles Test before you begin any treatment. You may do harm by taking high doses of the supplements required for Pyrrole Disorder if you don’t actually have Pyrrole Disorder.

    Second, the more I read about Pyrrole Disorder and other Copper related disorders, the more convinced I am that excess unbound Copper is quite significant for almost everyone with Pyrrole Disorder. For some, I believe it is even more important than the Zinc and B6 deficiencies. The toxicity of excess unbound Copper can bring far worse health problems than the Zinc and/or B6 deficiencies.

    My Recommendations:

    1. If you suspect that you may have Pyrrole Disorder:
    Don't diagnose from a symptom list.

    There are many Pyrrole Disorder symptom lists available on the internet; I won’t list them again here. But I would strongly caution against deciding that you have PD from any of these symptom lists. There are similar lists for many different disorders available on the internet, and they have so many symptoms that there is anywhere up to 90% overlap between the various lists. It would be quite possible to diagnose the same person with half a dozen different disorders using these symptom lists.

    2. Have a Urinary Pyrroles Test. A positive UP test is diagnostic for Pyrrole Disorder.

    It’s vital that the collection and transport of the urine sample be done according to the correct protocol. The sample must be collected in a container with preservative, be immediately protected from sunlight and be immediately frozen and remain frozen until it reaches the laboratory. It’s preferable that the collection be done at a pathology collection centre experienced with the correct protocol.

    If you do have to use a home test kit, make sure it includes freezer bricks and express return to ensure that the sample is frozen until it reaches the lab. If the urine sample doesn’t remain frozen, the HPL molecules degrade very quickly and may result in a false negative result.

    Stop taking any supplements that include Zinc and/or Vitamin B6 for at least three days, preferably a week, before this initial, diagnostic test, otherwise they will affect the result.

    The report may show two results for Hydroxyhemopyrrolin-2-one (HPL), the first is the actual measurement, the second is adjusted to account for the specific gravity of the urine sample, which can vary dependent upon how much you’ve been eating and drinking before the sample was collected. The result to work with is this “adjusted”, “normalised” or “corrected” HPL result, not the actual HPL measurement.

    3. If your Urinary Pyrroles test returns a low measurement, normal or negative, check that the sample was handled correctly, in particular that it was frozen immediately and that it was transported frozen and arrived at the laboratory still frozen. If the sample was collected and transported correctly but returned a low measurement, a negative result, you don’t have Pyrrole Disorder. Your practitioner will need to look elsewhere.

    4. Everything below here applies to people who have a diagnosis of Pyrrole Disorder confirmed by a positive Urinary Pyrroles Test. Don’t follow these suggestions below if you do not have a diagnosis of Pyrrole Disorder confirmed by a positive Urinary Pyrroles Test.

    5. Before you start taking supplements, have blood tests for Zinc, Copper and Ceruloplasmin. It’s not sufficient to test blood levels of Copper; you must test Ceruloplasmin at the same time.

    Most practitioners and pathology labs will usually test serum levels. Some practitioners recommend testing plasma Zinc and serum Copper and Ceruloplasmin. But testing either serum or plasma will give meaningful results. These calculations are very broad brush and give a generalised indication as ratios or percentages; the exact numbers are usually not so important.
    From these blood tests, calculate your Copper:Zinc ratio and your Non-Ceruloplasmin-Bound Copper level and ratio. The calculation methods are shown at the end of this document.

    6. Treat the Pyrrole Disorder with supplements of Zinc, preferably the Zinc Picolinate form, and Vitamin B6, preferably the Pyridoxal-5-Phosphate (aka P5P) form. Your practitioner will advise on the best dosages for you. Some practitioners like to add other supplements. Some practitioners like to use combined / compounded supplements, meaning all in one capsule, but I’d suggest individual supplements so that you can adjust the dose of each separately, and you can tell if you react to any individual supplement. Once you establish the right doses for you, you can change to compounded supplements with those doses tailored to your needs. For young children who have difficulty swallowing capsules, there are transdermal creams available.

    Always, Always, Always, start low and increase slowly, else you’ll make yourself feel even worse.

    7. If your Non-Ceruloplasmin-Bound Copper (NCC or sometimes called Unbound or “Free” Copper) level was high, above the reference range of 5 to 15ug/dL or 5 to 20% of total Copper, the Zinc supplement will help to gradually reduce that “free” Copper. Some practitioners like to add some other mineral and vitamin supplements to help remove the excess Non-Ceruloplasmin-Bound Copper safely from your body. Be patient; if your Non-Ceruloplasmin-Bound Copper level was high or very high, it will take a long time to safely remove it from your body. For those with a very high level of Non-Ceruloplasmin-Bound Copper, it could take twelve months or more to safely get that Copper down to normal levels. Please be patient.

    8. If your Non-Ceruloplasmin-Bound Copper level was high, remove foods with very high levels of Copper from your diet. There are plenty of lists of high Copper foods available on the internet. But don’t go overboard and remove essential food groups from your diet. Don’t worry about every little bit of copper in various foods. Because you’re taking regular Zinc supplements, you will not absorb most of the copper from your digestive system. Be guided by your practitioner.

    If you have Copper pipes in your house, you may like to flush your kitchen cold tap for 30 seconds first thing each morning to get rid of the small amount of Copper that’s leached into the water overnight. Use water from this cold tap for drinking, making tea, cooking, etc., not water from the hot tap as it may pick up Copper from your water heater.

    9. If your Non-Ceruloplasmin-Bound Copper level was high or very high, your doctor may suggest a Liver Function Test to check that your liver is not being damaged.
    Also, if you’re on a high dose of Zinc, your doctor may suggest a Kidney Function Test.

    10. If your Copper:Zinc ratio was OK and your Non-Ceruloplasmin-Bound Copper was not high, you may begin to feel some improvement within a few weeks by getting your Zinc and B6 back to normal. However, if your Non-Ceruloplasmin-Bound Copper level was high, it will take some time to safely remove that Copper and you may not feel significant improvement for a year or more. You may even feel a little worse when you start Zinc and mobilise excess Copper.

    11. You may like to repeat the Urinary Pyrroles Test and the Zinc, Copper and Ceruloplasmin blood tests at regular intervals to check your progress. Three months would be the minimum useful interval.

    Unlike the initial, diagnostic Urinary Pyrroles Test, for this follow-up UP Test, you must continue the supplements so that you can check that the dose is reducing the level of HPL in your blood and therefore in your urine. Some practitioners will tell you to stop supplements before every Urinary Pyrroles Test, but that’s wrong. Only stop supplements before the initial, diagnostic test.

    However, it’s not essential to do any follow-up UP test. The initial, diagnostic test will confirm your Pyrrole Disorder and will give your practitioner a good idea of the level of Zinc and B6 supplements to aim for.

    On the other hand, I would suggest redoing the blood tests to keep an eye on your changing copper levels. When you redo the blood tests, I suggest stopping the supplements for 24 hours before the blood draw to get a more meaningful result, not artificially increased by a recent dose.
    That usually means doing the follow-up Urinary Pyrroles test first and the blood tests a day or two later.

    12. Because you have a predisposition to producing excess HPL, you will need to continue to take at least some dose of Zinc and B6 for the rest of your life. You may need to increase the dose of Zinc and B6 during times of stress. And that means either emotional stress and/or physical stress such as severe infection.

    13. If your Non-Ceruloplasmin-Bound Copper level was high, I’d suggest repeating the Zinc, Copper and Ceruloplasmin blood tests and calculations every three to six months to make sure that you’re making progress in removing excess Copper.

    Also, if you’re taking a high dose of Zinc, you should occasionally redo the blood tests to make sure you’re not becoming Copper deficient. There may come a time when, if your Copper level drops too low, you should switch part of your Zinc dose to a multi-mineral supplement that includes Zinc and Copper. You will still need Zinc supplement to treat the PD, but you may eventually need Copper supplement to avoid deficiency.

    Your doctor may like to redo the Liver Function Test while you’re getting the Copper out, and the Kidney Function Test while you’re on high dose Zinc.

    A few comments about Pyrrole Disorder and MTHFR / Methylation Defects

    There is no direct link between Pyrrole Disorder and MTHFR / Methylation Defects. But there is some interaction.

    First, people place too much emphasis on the MTHFR gene alone. We have some twenty genes related to methylation and they each pull in their own direction. Our "methylation status" is the combined result of all of those genes, and or more correctly, the enzymes they produce. We need to consider all of our methylation-related genes.

    Second, Pyrrole Disorder results in deficiencies of Zinc and B6. Both of these nutrients are vital to some methylation / demethylation enzymes, and these deficiencies affect our overall methylation status. Also, the deficiency of Zinc allows our Copper levels to increase. That excess Copper can inhibit some enzymes.

    My suggestion is to treat your Pyrrole Disorder first, get your Zinc and Copper levels back to some sort of normal. And then think about your methylation problems. They may not be significant once you get your PD under control.

    I can’t comment on the effects of Pyrrole Disorder on other disorders because I know little about them. But the deficiencies of Zinc and B6 and the excess Non-Ceruloplasmin-Bound Copper must have some detrimental effect.

    This is not meant to be a full discussion of Pyrrole Disorder, but rather a suggested protocol or sequence to follow. Some people diagnose themselves, or their children, by symptoms only. But that is quite risky and I’d very strongly advise against diagnosing by symptoms alone. If you start treating based on symptoms alone, you may just be masking some other disorder.

    Some practitioners and patients do the Urinary Pyrroles test and then supplement with Zinc and B6 without testing blood Zinc, Copper and Ceruloplasmin. But Pyrrole Disorder has some consequential problems, primarily excess Copper, and some people and their practitioners don’t follow up, except to blame something they call “Copper dumping” when the patient feels worse on supplements. That’s not sufficient; you need to investigate potential excess “free” Copper and treat the Pyrrole Disorder with the Copper levels in mind.

    Of all of the people in the Facebook Pyroluria support group who have reported a positive UP test and have also reported their blood tests, 98% had excess unbound Copper.

    I’m convinced that excess Non-Ceruloplasmin-Bound Copper is a significant problem for most people who have Pyrrole Disorder.

    Disclaimer: I am not a practitioner of any type. I am just another Pyrrole Disorder sufferer who has done a lot of reading and who has had a lot of experience, far too much experience for my liking. Before you take any action based on anything you read here, please consult a trained, qualified, experienced doctor, naturopath or other practitioner experienced in Pyrrole Disorder, mineral imbalance, Copper overload and/or related disorders. Not only will they help you with accurately diagnosing and treating Pyrrole Disorder, they have the training and experience to see other problems, be aware of the interactions between supplements and medications, monitor your progress, etc… They are also able to take an objective view, which is very difficult for someone with Pyrrole Disorder and consequent high levels of Unbound Copper.

    Calculations you can do following blood tests for Serum or Plasma Zinc, Copper and Ceruloplasmin.

    First, we need to get the three measurements into the same units, preferably ug/dL (micrograms per decilitre):
    If your results were given in g/L multiply by 100,000 to get ug/dL.
    If your results were given in mg/dL, multiply by 1,000 to get ug/dL.
    If your results were given in mg/L multiply by 100 to get ug/dL.
    If your results were given in ug/L, divide by 10 to get ug/dL.
    If your results were given in ng/mL, divide by 10 to get ug/dL.

    1) Zinc
    If your results were given in umol/L, divide by 0.153 to get ug/dL

    2) Copper
    If your results were given in umol/L, divide by 0.157 to get ug/dL

    3) Ceruloplasmin
    Results are usually given in mg/dL or g/L. Convert to ug/dL as above notes.

    Now calculate the ratios, etc.

    4) Calculate the Copper:Zinc ratio, divide Copper (2) by Zinc (1),
    Reference range is 0.7 to 1.0. (This is a ratio, not ug/dL.)

    5) Calculate the amount of Copper bound in Ceruloplasmin,
    Ceruloplasmin (3) in ug/dL multiplied by 0.003. (Ceruloplasmin is 0.3% Copper by weight)

    6) Calculate the amount of Copper not bound in Ceruloplasmin,
    Total Copper (2) in ug/dL minus Copper bound to Ceruloplasmin (5).
    Reference range is 5 to 15ug/dL

    7) Calculate the percentage of Non-Ceruloplasmin-Bound Copper:
    Non-Ceruloplasmin-Bound Copper (6) divided by total Copper (2), then multiplied by 100.
    Reference range is 5% to 20%.

    Note that reference ranges are statistical ranges given by the laboratories. They are not the optimal or healthy range. They vary slightly between different laboratories, but they are generally around the same levels. They are usually two standard deviations above and below the mean, and that represents about 95% of the population sampled.

    From Wikipedia:
    “In health-related fields, a reference range or reference interval usually describes the variations of a measurement or value in healthy individuals. It is a basis for a physician or other health professional to interpret a set of results for a particular patient. The standard definition of a reference range (usually referred to if not otherwise specified) basically originates in what is most prevalent in a reference group taken from the population. However, there are also optimal health ranges that are those that appear to have the optimal health impact on people.”
    BadBadBear and Johnmac like this.

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