Pyrrole

Disorder

ADD, anxiety, restlessness, inattention, irrational anger or frustration, addictive tendencies, behavioural problems ... or Pyrrole Disorder?

What is Pyrrole Disorder?

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Pyrrole Disorder is a condition that causes nutrients essential to brain and nervous system function to be bound up and unavailable for use in the body by an aberrant metabolite of haemoglobin, leading to increased susceptibility to mental, emotional and physical stress. This condition causes a correlated disruption of calming neurochemicals such as serotonin (the happy, content neurotransmitter), melatonin (which regulates sleep and the 'body clock'), GABA (important for relaxation, calmness and quality sleep), and acetyl choline (vital for memory, learning and the protection of  nerves). When pyrroles are high, interference in the balance of brain chemistry means the child can't calm or self-sooth, becoming more restless, irritable,  and internally disrupted making it difficult to learn easily, becoming more and more sensitive to stress and overstimulation from normal everyday environments.

Depletion of these key essential nutrients can also lead to issues with immunity, the production of stress hormones such as cortisol, which is anti-inflammatory, anti-allergy and stress regulating, as well as the metabolism of essential fatty acids in the body, affecting brain and nerve health.

As if this isn't enough, 'Pyrrole' itself is pro-inflammatory, leading to oxidative free-radical damage to cells, nerves, brain cells and body tissues, over time damaging function.

People with pyrrole disorder need to be supplemented, particularly through periods of stress. Food sourced nutrients are not enough. When the production of these calming neurotransmitters (serotonin and GABA) are restored once more, which can take months, the person will begin to feel calmer and more settled once more, and teachers, parents, friends and employers notice a difference, a calmness and clarity that weren't possible before, namely a reduction in anxiety, hyperactivity, restlessness, impulsiveness, meltdowns and addictive behaviour.

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​Pyrrole disorder is also known as pyroluria, kryptopyroluria, kryptopyrole or Mauve disorder. It is a biochemical disorder caused by an abnormality in the sythesis and metabolism of haemoglobin, which adversly impacts the bioavailability of nutrients which are essential for many pathways in the body, particularly those affecting mood, nerve and brain function.

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What is Hemaglobin?

Hemoglobin is the oxygen carrying molecule in the blood. Normal hemoglobin handling does not affect nutritional factors. This disorder, however, leads to a dysfunctional handling of the metabolite called hydroxyhemopyrrolin-2-one (HPL) also known as Pyrrole. People who suffer from the abnormal handling of this molecule produce too much  of these bioactive HPL substances, which are then capable of binding to and inhibiing the essential role of nutrients in the body, impairing system function and over time causing the symptoms of system imbalance.

When essential nutrients are bound up by HPL, and become unavailable to the body, all of the roles those nutrients would play in metabolism and function become effected.

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What biochemical factors are impacted?

It is the disruption of neurochemicals and the increase in oxidative stress that causes the problems to wellbeing.

Pyrrole byproducts bind up nutrients important in the production of :

• Serotonin - important for a calm, happy mood, apetite and digestion, social behaviour, memory, sleep and central nervous system function.

• GABA - important for for relaxation and calm (people need alcoholto relax are often looking for more of this neurotransmitter.)

• Cortisol - an anti-inflammatory, anti-allergy and stress regulating hormone)

• Melatonin - sleep and circadean regulation (body clock)

• Acetyl Choline - a vital component of nerves and important for memory, and brain and neural protection

• Essential Fatty Acids - important for normal fat metabolism, liver and gall bladder function, weight control, brain function, and cell membrane health.

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​What are the symptoms?

Symptom presentation depends on the degree of pyrroluria. When the degree of binding is high, we are more likely to notice the behavioural, emotional (mood regulation) and learning issues associated with the condition. In children, adults and the elderly, when levels are high and unsupplemented over time, we are likely to see greater cognitive impairment, sleep disruption, immue issues and emtoional symptoms. Overall people affected by this disorder feel a great deal more anxiety than those without this issue. There is a vast amount of inner tension, they feel on edge, and may not be able to self-calm or modulate their behaviour like others. Over time there comes a neural sensitivity that may appear like their nerves aren’t sheathed and protected like normal nerves. The degree of symptoms and effects are related to their level of nutrition, as higher amounts of the involved nutrients can overcome the binding process, and support better function.

​Functional Signs & Symptoms Associated with Pyroluria

There are many signs and symptoms that may indicate the presence of elevated Pyrroles in the blood, and whether the issue manifests medically, or in a sub-clinical way, is down to how healthy the body, diet and digestive system is, how well-nourished the person is, and the degree of pyrrole production and accumulation.    The list below are ways that Pyrrole disorder can manifest. 

Pyrrole Disorder increases a person's tendency to these characteristics, which will vary in severity of pyrrole toxicity :

• Reading difficulties

• Fluid retention

• Emotional Instability

• Poor Memory

• Frequent colds, fevers, and chills

• Sensitivity to bright light

• Frequent ear infections as a child

• Sensitivity to smells

• Severe inner tension

• Skin rashes

• Hypersensitivity to noise

• Significant growth after the age of 16

• Hyper-pigmentation of the skin

• Social withdrawal

• Substance abuse

• Inability to think clearly

• Temper tantrums

• Tendency towards iron deficient anaemia

• Intolerance to alcohol

• Tingling in the arms and legs

• Intolerance to drugs

• Intolerance to some protein foods

• Unusual smelling body odour

• Joint pain

• Unusual smelling breath

• Knee pain

• Abdominal pain

• Lack of hair on head, eyebrows and eyelashes

• Abnormal body fat distribution

• Lack of regular menstrual cycles

• Loss of appetite

• Low libido

• Amnesia spells

• Low tolerance to stress

• Anger – explosive

• Mood swings

• Argumentative - likes to argue

• Morning nausea

• Cold hands and feet

• Motion sickness

• Much higher capability in the evening than mornings

• Creaking in joints

• Nervous exhaustion

• Delayed puberty

• Overwhelmed in stressful situations

• Pale skin, poor tanning or burn easy in the sun

• Difficulty remembering dreams

• Panic attacks

• Early greying of hair

• Poor morning appetite/tendency to skip breakfast

• Elevated eosinophils (histamine / allergies)

• Preference for spicy or heavily flavoured foods

• Prone to stitches when running now or as a child

• Characteristic Body Features Associated with Pyroluria

Physical Characteristics

Some people who suffer from Pyroluria may also have characteristics manifesting in their physical appearance such as;

• If the family has dark skin, a person with pyrrole disorder will have the lightest skin in the family

• Thin / scant head hair, eyebrows and eyelashes

• Skin which is prone to stretch marks

• Overcrowded teeth in the upper jaw (unless orthodontic treatments have taken place)

• White spots on the fingernails

• Skin problems such as acne, eczema or herpes

• Poor quality tooth enamel

• Skin that appears thin

Medical Conditions that may be associated with long-term Pyroluria

• Acute Intermittent Porphyria

• Criminal Behaviour

• Neurosis/Neurotic/OCD

• ADD/ADHD

• Depression

• Post Natal Depression

• Alcoholism

• Down Syndrome

• Schizophrenia

• Allergies

• Epilepsy

• Substance Abuse

• Asperger’s Syndrome

• Learning Difficulties

• Tourette’s Syndrome

• Autism

• Violent Offenders

• Bi-Polar Disorder

• Manic Depression

• Anorexia / Bulemia

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What Causes Pyroluria?

Genetics - Evidence suggests that Pyroluria is a genetically based issue.  There is often evidence of it running in families. If a parent, grandparent, aunt, uncle, brother or sister has suffered from major anxiety, depression, Bi-Polar disorder, alcoholism, schizophrenia or there is suicide within the family, there is more likely to be an undiagnosed pyrrole disorder in the family genetically. This increases the risk of Pyroluria being passed down to other family members when the condition is supplemented during the preconception, pregnancy and post natal period.

Still relatively new, this disorder is only just being properly diagnosed, and when it is, knowing why a person is having these issues can be a great relief to both the sufferer and the family, and supplementation can be properly administered to address the symptoms and prevent the condition in the future.

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Research has discovered that stress increases the production of HPL in the blood, which in turn drives a worsening of symptoms when not supplemented.  In a US navy study conducted in 1992 a very rapid increase in Pyrrole levels occurred in volunteers who were subjected to the stress of a brief cold-water immersion. 

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What makes Pyrroluria worse?

Nutrient deficiencies - overcoming the nutrient deficit is the key to overcoming the biochemical damage associated with this tendency. When key nutrients are low, oxidative stress is high. Studies have demonstrated that zinc deficiency (which is bound up by HPl) increases the incidence and severity of intestinal permeability (colloquially known as ‘Leaky Gut Syndrome’) in animal and humans  (10-16).

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Stress - It is a well known fact that stress damages the intestinal wall and causes intestinal inflammation. Both of which lead to an increase in Leaky Gut Syndrome.

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Poor diet & Digestiove Health -  This makes sense as poor diet and digestion leads to a reduction in the availability of essential nutrients. This creates a downward spiral as without optimal nutrient availablity the body can’t function properly, increasing body-stress, driving up blood Pyrrole levels.

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Intestinal permeability - this a condition whereby the protective barrier of the intestinal wall becomes more permeable than is desirable, allowing through into the blood larger than normal food particles, bacterial by-products, waste and toxins to pass through. Intestinal permeability is a widespread problems in sufferers of Pyroluria, and a confounding factor that drives up blood Pyrrole levels. 

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Gut issues- parasites and an overgrowth of adverse bacteria in the intestinal tract has also been linked to an increase in blood pyrrole levels as well as contributing to Leaky Gut Syndrome (17, 24). Stress increases the adherence of bad bacteria to the intestinal wall within 30 minutes (25).

Alcohol, smoking, drugs and heavy metal exposure – this can dramatically increase blood pyrrole levels. This is why sufferers of Pyroluria tend to get a worsening of their symptoms 24 to 48 hours after a big night on the grog or recreational drug use.

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How is it related to Neural Damage?

Pyrroles are classed as 'nerve poisons' (26) and as such can cause damage to your nerves, nerve cells and tissue, your brain and they can interrupt messages being sent along your nerves especially within your brain.

Heme is a substance found in your body that has an iron atom at its core. The most commonly know heme is haemoglobin, the oxygen carrying red pigment of your blood. Heme is not only found in your blood but in other areas of your body such as your liver and nerves. Your nerves metabolic activity is highly dependent on heme for their function and low levels of heme leads to a metabolic crisis occurring in your nerves resulting in neuronal (nerve) cell. The by-product of haemoglobin metabolism, Hydroxyhemopyrrolin-2-one (HPL) or pyrrole, is believed to decrease heme levels in humans. Animal studies have shown that HPL caused a decrease in liver heme, and the heme containing detoxifying enzyme cytochrome P450, by up to 55% over a 48 hour period (30). Vitamin B6, biotin and zinc are all required for the production of heme and a reduction in these nutrients results in sub normal heme levels. Heme levels are further depressed by stress and heavy metal exposure .  Low levels of heme result in an excess production of the toxic free radical nitric oxide, which can cause serious damage to brain tissue and is suspected to play a role in schizophrenia, autism and Down Syndrome.

TESTING

This is not yet a mainstream medical test. It is a functional test that is usually referred for by Naturopaths, Nutritionists and Functional Doctors. It is a urine test.

Why Isn’t Pyroluria more acknowledged by mainstream medicine?

​Unfortunately Pyroluria falls outside the realm of mainstream medicine due to the fact that the only way to rectify the problem is by improving the sufferer’s nutritional status, diet, digestion and stress levels. Mainstream medicine relies on drugs to suppress a symptom or relieve suffering and this form of treatment will not work for a person who has a Pyrrole disorder. Sadly sufferers of Pyroluria fall through the cracks and are often misdiagnosed and given medication or drugs that do nothing to rectify the underlying problem. Unfortunately these medications can lead to further deterioration of a persons health.

As you can see pyroluria is a complex health issue and whereas treatment with the relevant nutrients will help to reduce the symptoms associated with pyroluria, it hasn’t addressed the underlying causes. So any treatment protocol for pyroluria needs to not only support the nutritional deficiencies caused by an elevation of pyrroles, but to also identify and treat what has caused these in the first place. The first step is to assess whether or not you are suffering from pyrrole disorder is to get yourself tested, then you will know to what to do to manage it.

How is Pyrrole Disorder diagnosed?

Pyrrole disorder is  a clinical diagnosis, requiring a patient to have an elevated urinary pyrrole test result. They will also typically respond well to nutrient therapy with a vast improvement in their clinical symptoms within three months.

Test Results:

Patients with urinary pyrrole levels of 20 mg/dl or higher often exhibit several of the characteristic clinical symptoms. An intermediate level of 10-19 mcg/dl does not rule out pyrrole disorder because it is response to treatment that confirms the diagnosis.  Under 10 is considered normal.