What is Pyrrole Disorder?

Pyrrole disorder, also known as pyroluria, kryptopyroluria, kryptopyrole or Mauve disorder is a biochemical imbalance involving an abnormality synthesis and metabolism of haemoglobin. It can be purely genetic, or acquired through environmental and emotional stress. It is very common in people with leaky gut, particularly after a lot of antibiotics.

How does Pyrrole Disorder occur?

All cells in your body produce waste or by-products. The by-product of haemoglobin is called hydroxyhemopyrrolin-2-one (HPL) also known as Pyrrole. People who have Pyrrole Disorder, produce excessive amounts of these Pyrroles which then bind to or stop use of Zinc, Vitamin B6 and Omega-6 fat GLA. Thus, these nutrients cannot be used by the body.

There are three main nutrients that are not properly absorbed in someone with pyrrole disorder. These are vitamin B6, magnesium, and zinc.

B6 in particular is necessary for the creation of red blood cells. It increases energy levels and proper cognitive function. A deficiency of B6 will often cause inflammation, depression, and sometimes anaemia.

Magnesium is often used as a muscle relaxant for athletes and is strongly involved in the blood sugar cycle. A deficiency can be the cause of blood sugar or appetite control issues, sleeplessness, constipation, and even nervous twitching.

Zinc is vital for concentration, memory, detoxification and good digestion, and when lacking, can lead to anorexia, leaky gut syndrome and digestive disease. In fact, zinc is required for many enzymatic processes in the body, yet 80% of the Australian population are estimated to be deficient in it.

The Cause Of Pyrrole Disorder

Pyrrole disorder is caused by the overproduction of hydroxyhempyrolin (HPL). The HPL binds zinc and B6 preventing their use by the body and causing excretion in the urine and hair. HPL is a biomarker for oxidative stress and is neurotoxic. Stress of any kind will increases production of pyrroles/HPL which in turn decreases zinc and B6. The reduced utilisation of zinc and B6 is what causes the symptoms of Pyrrole Disorder.

There is evidence to suggest that Pyrrole Disorder is a genetically based ailment as well as a disorder caused by a variety of lifestyle issues such as poor diet, leaky gut syndrome, stress and poor digestion. Statistics and research indicates that if a relative sufferers from Alcoholism, Schizophrenia, Depression, Bipolar Disorder or has suicided, there is a greater risk of Pyrrole Disorder in other family members.

Poor dietary choices and poor digestive health can lead to an increase in Pyrrole levels as a low nutrient diet will not deliver all of the nutrients a body needs much less one with the need for additional support. Leaky Gut Syndrome is common in sufferers of Pyrrole Disorder and a common factor for increasing blood Pyrrole levels. Consider the child with sensory processing, autism spectrum or other oral motor issues, who may have a very select array of foods they are happy to eat. If they are genetically at risk of Pyrrole, plus they eat a strict diet, and possibly have leaky gut, they can manifest the symptoms of Pyrrole disorder (but you’ve got to look at how to manage the underlying triggers of Pyrrole).

Alcohol, smoking, drugs and heavy metal exposure can dramatically increase blood pyrrole levels. This is why people who have Pyrrole Disorder will tend to get a worsening of their symptoms 24 to 48 hours after a big night out drinking or recreational drug use.

Research has discovered that stress increases the production of HPL in your blood, which in turn causes a worsening of your symptoms. It is a well known fact that high levels of cortisol (aka STRESS!) damages the intestinal wall causing intestinal inflammation. Both of which lead to an increase in Leaky Gut Syndrome. Studies have shown that zinc deficiency increases bowel permeability too. Leaky gut is not always from stress and diet, and can actually be due to a candida overgrowth, or other bacterial imbalances in the gut microbiome.

The main issue with Pyroluria is severe zinc and B6 deficiency.

Zinc is essential for 100’s of processes in the body and is particularly important for healing, immune function, digestion, neurotransmitter activation, physical growth, memory, insulin sensitivity and control of blood sugars, DNA replication, prefrontal cortex development, detoxification, and even skin health.

Zinc and B6 are essential for production of neurotransmitters such as serotonin (our happy hormone), melatonin (our sleep hormone), GABA (our relaxation hormone), and acetyl choline which is important for memory. They are also involved in production of our steroid hormones such as cortisol (our anti-inflammatory, anti-allergy hormone and stress hormone) and the conversion of oils in the body (fat metabolism, liver and gall bladder issues and weight control). The oils EPA/DHA but mostly GLA are found to be low in those with pyrrole disorder and are damaged by oxidative stress/free radicals/toxins created by pyrrole.

Common signs and symptoms of Pyrrole Disorder

White spots on fingernails
Hypoglycemia/sugar intolerance (a very common one and contributes to the Jekyll and Hyde personality often seen in someone with Pyrrole!)
Food and environmental allergies
Joint pains
Fatigue (often unexplained by other reasons, may appear as though it is “made up”)
Irritable bowel, constipation, gut pain
Dizziness
Insomnia
Poor memory and concentration
Poor stress control
Poor emotional regulation - episodic anger, or depression, may seem manic at times
ADHD or ODD in children

While you may not get all of these symptoms, or you may have all of them, it’s important to note that the level of symptoms can be effected by the severity of your zinc and B6 deficiencies, and also how you’re genetically designed.

Comprehensive list of symptoms associated with Pyrrole disorder:

Abdominal pain, Abnormal body fat distribution, Acne, Allergies, Amnesia spells, Anger – explosive, Anxiety/anxious, Argumentative – likes to argue, Cold hands and feet, Constipation, Creaking in joints, Delayed puberty, Delusions, Depression, Difficulty remembering dreams, Dramatic, Dyslexia, Early greying of hair, Eczema, Elevated eosinophils, Emotionally unstable, Fatigue, Fluid retention, Frequent colds, fevers, and chills, Frequent ear infections as a child, hallucinations, Hyperactivity, Hypersensitivity to noise, Hyper-pigmentation of the skin, Hypoglycaemia, Inability to think clearly, Insomnia, Intolerance to alcohol, Intolerance to drugs, Intolerance to some protein foods, Joint pain, Lack of hair on head, eyebrows and eyelashes areas, Lack of regular menstrual cycles, Loss of appetite, Low libido, Low tolerance to stress, Migraines, Mood swings, Morning nausea, Motion sickness, Much higher capability in the evening than mornings, Nervous exhaustion, Nervousness, Overwhelmed in stressful situations, Pale skin, poor tanning or burn easy in the sun, Panic attacks, Paranoia, Pessimism, Poor memory, Poor morning appetite/tendency to skip breakfast, Preference for spicy or heavily flavoured foods, Prone to stitches when running now or as a child, Reading difficulties, Seizures, Sensitivity to bright light, Sensitivity to smells, Severe inner tension, Skin rashes, Significant growth after the age of 16, Social withdrawal, Substance abuse, Temper tantrums, Tendency towards iron deficient anaemia, Tingling in the arms and legs, Tremors, Unusual smelling body odour, Unusual smelling breath.

The following conditions are associated with Pyrrole Disorder (and increase your risk of having it, if you haven’t gone down this path yet)

Acute Intermittent Porphyria, ADD/ADHD, Alcoholism, Allergies, Asperger’s Syndrome, Autism, Bi-Polar Disorder, Criminal Behaviour, Depression, Down Syndrome, Epilepsy, Learning Difficulties, Lung Cancer, Manic Depression, Neurosis/Neurotic, Post Natal Depression, Schizophrenia, Substance Abuse, Tourette’s Syndrome, Violent Offenders.

Body Appearances Associated with Pyrrole Disorder:

There can also be abnormalities in physical appearance including
•   If your family has black skin yours will be the lightest skin in your family
•   Lack of hair on your head, eyebrows and eyelashes
•   Skin which is prone to stretch marks
•   Teeth in your upper jar will often be overcrowded (unless orthodontic treatments have taken place)
•   White spots on your fingernails
•   You may suffer from acne, eczema or herpes
•   Your tooth enamel has a poor appearance
•   Your skin will appear paper thin

Pyrrole Disorder Diagnosis

Pyroluria is diagnosed by a simple urine test which detects KPU in urine.

Clinically speaking, I see many more women and children diagnosed with Pyroluria. It appears to be more common in women with red hair and fair skin, though there’s no research to back this up.

Pyroluria Treatment

The treatment to correct the imbalance seems surprisingly simple. It involves taking zinc, vitamin B6 and/or pyridoxal 5-phosphate (activated B6), GLA, an essential omega 6 fatty acid found in evening primrose, borage and black currant oil, Vit E and magnesium.

This can be a good start, if you don’t have personal access to individual support. It is important to remember that your body will need different levels of each nutrient, and trying to be your own practitioner can be dangerous.

Initially, if these nutrients haven’t been in the body for a while, all enzymatic processes will be increased - meaning you may experience a detox, or release a latent infection.

It is not advisable for people to self medicate as B6 & zinc toxicity can also be an issue for some people if their body still can’t utilise these nutrients properly and so close observation by an experienced practitioner is important.

For example, deficiency in magnesium reduces absorption of B6. Zinc and iron taken together will compete for the same receptors in the body. Incorrect ratios of zinc and B6 can disrupt stomach acid production, which could worsen leaky gut. There are many more reasons why self-prescribing can be a dangerous undertaking.

Other nutrients may assist include niacinamide (B3), pantothenic acid (B5), methylcobalamin (B12), manganese, vitamins C, E and magnesium. Food sources and nutritional supplements containing copper and red/yellow food dyes should be avoided. Copper is commonly high in people with pyroluria and needs to be kept in check.

Complete treatment of Pyroluria is best done with a practitioner who can get to the root cause

While the supplements will replenish many of nutrient deficiencies, it’s important to remember that Pyrrole Disorder has underlying triggers, and it is important to get to the bottom of them for a fuller resolution.

Any method to reduce stress and cortisol is a great place to start, as well as neurotransmitter function, leaky gut, latent infections, vagus nerve function, immune system function.. the list goes on!

It is important that those with severe Pyrrole Disorder symptoms have the support from a natural health practitioner who understands the disorder. It can be a fiddly process getting the perfect levels of nutrients, managing detox, and processing any other causes of underlying stress in the body.