Studies show that Calcium-D-glucarate may help fight Gilbert’s Syndrome symptoms. Here I unpack what it is and what it could do for people with Gilbert’s Syndrome.
What is Calcium-D-glucarate?
Calcium-D-glucarate is naturally found in fruits and vegetables. It’s a combination of calcium with glucaric acid, and is similar to glucaric acid which is found in the body. It is commonly used as a dietary supplement due to its potential health benefits.
What does Calcium-D-glucarate do and why does it help fight Gilbert’s Syndrome symptoms?
Some studies show that Calcium-D-glucarate may have a positive effect on liver health and bile metabolism. It is believed that Calcium-D-glucarate may improve the detoxification process in the liver by increasing the excretion of certain toxins. Importantly for people with Gilbert’s Syndrome this includes bilirubin.
How does Calcium-D-glucarate help fight Gilbert’s Syndrome symptoms?
It’s thought that Calcium-D-glucarate stops an enzyme called beta-glucuronidase. This enzyme is responsible for breaking the bond between glucuronic acid and toxins. This lets them be reabsorbed into the body instead of being eliminated. This means that by stopping beta-glucuronidase from working as well, Calcium-D-glucarate may help prevent the reabsorption of bilirubin and other toxins.
Here’s what happens in your body in medical terms:
Beta-glucuronidase is an enzyme that plays a role in the breakdown of bilirubin in the intestines. It acts by deconjugating bilirubin, converting it back into its unconjugated form, which is less water-soluble. Normally, bilirubin that has been conjugated by UGT1A1 in the liver is excreted into the intestines, where it is further metabolised and eliminated from the body. However, in individuals with Gilbert’s syndrome, the reduced UGT1A1 activity leads to impaired bilirubin conjugation. The presence of beta-glucuronidase in the intestines can cause an increased release of unconjugated bilirubin.
The increased activity of beta-glucuronidase can contribute to higher levels of unconjugated bilirubin. This makes the symptoms of Gilbert’s syndrome worse.
Here’s what this means in ordinary words-
Calcium-D-glucurate gets in the way of chemicals that increase bilirubin and so you can deal with it more easily, if you have Gilbert’s Syndrome.
It is always important to note that the available research on Calcium-D-glucarate’s effect on bilirubin is limited. More studies are needed to establish how well it works in reducing Gilbert’s Syndrome symptoms.
Is it the right supplement for you?
Don’t forget – everyone responds differently to supplements too. You might have other conditions that mean that Calcium-D-glucarate isn’t right for you. It does other things, such as affect estrogen levels and some suggest its use for hormone related conditions :https://drbrighten.com/benefits-of-calcium-d-glucarate/. Make sure you check with your Doctor before taking it as it could affect other medications.
You can increase your levels of the glucaric acid element by eating more apples, oranges, broccoli, cauliflower and cabbage.
You can also decrease the beta-glucuronidase that may be worsening your symptoms by eating a healthy low fat diet, that includes plenty of fresh fruit and veg. Reducing smoking, alcohol and sugar may also help. Want to find out more about how your diet can help you live a happier life with Gilbert’s Syndrome? Check out this post https://gilbertssyndrome.org.uk/the-liver-diet/ You can find out more about food and nutrition to help with Gilbert’s Syndrome in the Posts ‘diet’ category here https://gilbertssyndrome.org.uk/category/diet/
If you decide you want to get a supplement, try this one. It has good reviews, although badged as for women due to its impact on hormones – you don’t have to be a woman to take it of course.
So, it sounds like Calcium-D-glucarate just might have a positive impact on bilirubin levels by potentially enhancing the liver’s detoxification processes. However, further research is needed to fully understand its effects, and it is advisable to consult a healthcare professional for personalised advice if you plan to take a supplement.
Did you know that Gilbert’s Syndrome and gallstones are linked? Studies show that people with Gilbert’s Syndrome have a higher risk of developing gallstones. Let’s reveal the details and what it means for people diagnosed with Gilbert’s Syndrome.
If you have Gilbert’s Syndrome or suspect you may have gallstones, it’s crucial to consult with a healthcare professional. They can provide personalized advice and guidance.
Researchers discovered that individuals with Gilbert’s Syndrome are more prone to gallstone formation.
Those with the syndrome may face challenges such as frequent gallbladder operations due to issues with their bile ducts.
The Link to Gallstones:
The study established a direct connection between Gilbert’s Syndrome and an increased likelihood of gallstones.
Bile Duct Operations:
People with the syndrome may require surgeries on their bile ducts due to gallstone-related complications.
Implications for Patients:
These findings highlight the importance of monitoring gallstone risk in individuals with Gilbert’s Syndrome.
Proactive Healthcare Approach:
Doctors should be vigilant in assessing and managing the gallstone risk in patients with this syndrome.
Better Treatment Strategies:
Understanding this link enables medical professionals to develop more effective treatment plans for patients with Gilbert’s Syndrome.
The study confirmed the higher susceptibility of individuals with Gilbert’s Syndrome to develop gallstones, emphasizing the need for proactive healthcare and tailored treatment strategies to mitigate potential complications.
Gilbert’s Syndrome was first identified in 1901 by Dr Gilbert, as a benign condition causing jaundice. Since then, scientists have mapped our genome and discovered why we have Gilbert’s Syndrome. More has been explained about how the mutated gene impacts our bodies in different ways. Each year we discover new information.
As we have improved our understanding of our genes, we have also discovered that there are different types of Gilbert’s Syndrome. People from different populations have variations of Gilbert’s Syndrome.
Mutations in this gene happen in people all over the world, but to different levels depending on your background. The main impact is that people with this gene difference produce less of an enzyme – a chemical that helps process things in the body. Bilirubin is one of the substances that this enzyme processes. See moreabout bilirubin here.
One of the great things about having more bilirubin in the bloodstream is that it appears to help protect people from some diseases such as cardiovascular diseases and diabetes type II. Some scientists have suggested it could be a positive evolution.
Research shows that the effect is different depending on your heritage:
For example, individuals with Eastern Asian ancestry (i.e. Chinese and Japanese) appear to have the lowest circulating bilirubin concentrations (prevalence of GS ∼2%), whereas individuals originating from India, Southern Asia and the Middle East demonstrate significantly increased rates of GS, approximating ∼20% (Figure 2). Caucasian ethnicity is associated with a 2–10% prevalence of GS .
You can inherit Gilbert’s Syndrome from one or both of your parents. But, these are different types:
Gilbert syndrome can have different inheritance patterns. When the condition is caused by the UGT1A1*28 change in the promoter region of the UGT1A1 gene, it is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have the mutation. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
When the condition is caused by a missense mutation in the UGT1A1 gene, it is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. A more severe condition known as Crigler-Najjar syndrome occurs when both copies of the UGT1A1 gene have mutations.
Other things that impact your Gilbert’s Syndrome symptoms
The current thinking is that there are different versions of mutation of the UGT1A1 gene. Because of this and a range of other factors you might experience symptoms. This is where the importance of understanding that your genes are only ONE piece of the jigsaw comes in. The key to managing any genetic condition or trait is to understand that there are many other things that impact how it works in you.
In Gilbert’s Syndrome, of whatever type, other things will impact your symptoms. One reason few people experience symptoms before puberty is because the hormonal changes that occur at that time trigger symptoms. Hormones are one factor – from life changes to menstruation, this will impact your symptoms.
Other factors which affect your Gilbert’s Syndrome symptoms include – vigorous exercise, fasting, dehydration, a virus, external toxins, stress, sleep deprivation and many more. The pathways of the liver which use the enzyme we are deficient in as a result of this gene mutation are affected by all of these internal and external factors.
It’s worth understanding that genes are only one component of how you live your life. AND you can control many of the other components to enhance your wellbeing. But don’t forget you also benefit from the protective effects that your Gilbert’s Syndrome offers.
Find out more and stay up to date
As a member of the Genetic Alliance, a charity with a membership of over 200 patient organisations in the UK that supports people with genetic conditions, I stay in touch with the latest on support for people with genetic conditions. If you want to find out more about genetic conditions check out their website https://geneticalliance.org.uk/ You can also find out more about genetic testing in the UK.
With continued discoveries about how a gene difference can influence us, and the adaptation of science to support our health, as well as how lifestyle and lifestage are key, it’s important to stay up to date with understanding Gilbert’s Syndrome. This way you can take control of your health and happiness.
Itching is a topic that really gets under the skin of people with Gilbert’s Syndrome.
One of the most common questions I hear, from people with Gilbert’s Syndrome, is ‘does anyone else get really itchy???!’ The answer is ‘absolutely’! Many people with Gilbert’s Syndrome get itchy skin.
But WHY and WHAT can you do about it?
The reason it happens is up for speculation. It has been recognised, for over 2,000 years, that jaundice(which many people with Gilbert’s Syndrome will have at some point) is linked to itching (technically known as ‘pruritis’).
Is it the prickly nature of bile salts? Is it toxins building up in your body as your liver isn’t dealing with them properly? Your skin is your second largest de-tox organ, and if your liver isn’t doing its best, then your skin may be acting as back up. Are there histamines, serotonin or other chemicals produced by the body involved?
There’s no doubt that bilirubin build up(one of the main Gilbert’s Syndrome symptoms) above a certain level does cause intense itching, usually at the point when you can see jaundice in your skin or eyes.
Of course – it may not be your Gilbert’s Syndrome at all. Lots of things can cause the sensation of itching:
Sometimes it’s an allergic reaction, or eczema, or even a pinched nerve. Pregnancy and some medications can also cause itching. Itching can be a side effect of medication that’s not being effectively processed in your liver, as a result of your Gilbert’s Syndrome. Do keep an eye on symptoms when taking any new prescription drug and speak with your doctor if you have any unusual or unpleasant sensations. Air conditioning or your age can affect your skin’s dryness and sensitivity. You can also develop a response to a detergent, wash or perfume even if you’ve used it for years. Don’t assume it’s always Gilbert’s Syndrome.
This article provides some hints and tips to try – it is not medical advice and I am not medically qualified. Always speak to your medical professional before changing a health or medication routine, or if you are getting unpleasant side effects or symptoms.
What can I do?
Most importantly – how do you stop it when it’s driving you crazy!
Once you’ve read this article you should be better equipped to:
Deal with the liver function if that’s the root cause
Soothe and comfort the skin to reduce the itch
Give your skin the best chance to be irritant free
Look after your liver function:
If this is a Gilbert’s Syndrome related symptom, then Step One in managing a flare up of GIlbert’s Syndrome remains the same. In headlines : sleep, drink water, eat nutritious low fat, low sugar whole foods and relax. If you are being triggered by something in your environment (medication, chemical fumes etc), then remove or reduce it if you can.
Usually symptoms will last just hours or a couple of days if you can take time to manage your lifestyle. This isn’t always possible of course – you may have caring responsibilities, a shift pattern, other chronic illnesses, be travelling or under a great deal of stress. Whatever your situation, be kind to yourself and do what you can to make the most of your situation to try to include some of those elements of good self care.
You can read more about dealing with symptoms and lifestyle across this website and take the Essentials of Gilbert’s Syndrome course (find out more here) to get foundational tips to manage your life better with Gilbert’s Syndrome.
Meanwhile – let’s deal with the itch!
This is really important, before we go any further, DO NOT SCRATCH! All this does is irritate the skin and you’ll end up with a longer term problem whilst the skin heals. If you DON’T scratch then the itch will stop as your Gilbert’s Syndrome symptoms settle down (or when you remove the irritant).
If you DO scratch, then you’ll be dealing with skin that is irritated, sore and healing and will itch even MORE!
You may find, like me, that you scratch at night or without even thinking about it. Try to train yourself to notice before you damage your skin. At night, you could cover the itchy skin with clothing or wrap some fabric around it so you don’t scratch whilst asleep. Some people even wear a light pair of gloves to prevent their nails from scratching their skin whilst asleep.
You might be in tears trying not to scratch – I’ve been there. Here are some things that can help:
Nati Melnychuck unsplash
PS I have sourced examples of products for you so that you can easily find them – there’s no obligation to buy these, and you might find them cheaper elsewhere. These links may provide a small affiliate fee, at no extra cost to you, but which will help keep this website running.
Creams and ointments –
There are a number of things I have found which help my skin feel less itchy. I have other conditions which result in me having soft and sensitive skin and these help me. Creams with colloidal oatmeal, such as Aveeno, are really soothing. The oatmeal helps inflammation and provides a barrier that protects irritated skin. You can buy colloidal oatmeal as a separate powder and add it to a bath or your own cream.
Aveeno is a product works well for deep moisturisation, and has a ‘triple oat complex’ plus shea butter, which you can buy here https://amzn.to/3S4QJWj I recently bought some and it’s working really well for me.
Sudocrem is also soothing to skin. It’s really a universal antibacterial cream that is even suitable for babies, and so very kind to your body. The lavender in it is both soothing and has a nice odour. A little goes a long way. It tends to sit on the skin and has a white residue, so may be better for smaller areas, or under clothing for larger areas. It’s also really affordable! You can buy it in supermarkets, chemists or here https://amzn.to/3DLfHFL
Some antiseptic creams also have topical anaesthetics, such as Savlonhttps://amzn.to/3SjWq2z . I would suggest caution again, as there are many chemicals and other ingredients which your body might not process well and which could just add to the problem. Perhaps try other creams and gels first, such as colloidal oatmeal. If you have broken skin, perhaps from scratching, then this would help with both relieving the sensation and helping to protect the skin from infection.
Gels and creams with aloe vera can also be soothing. Aloe vera has anti inflammatory and healing properties. Watch out for the added ingredient of menthol though. Many aloe vera preparations are sold with menthol to help cool sunburnt skin, but menthol is poorly processed by people with Gilbert’s Syndrome and it can make you feel unwell. Other preservatives and chemicals can also detract from the benefit of using a natural plant such as aloe. A preparation such as this gel may be a good option to avoid too many added ingredients https://amzn.to/3qWdkZ8
Do test new products on a small amount of skin first though. A small percentage of users of the many aloe products on Amazon experienced red and sore skin after trying a new aloe brand. Do read reviews and decide for yourself. If you’ve already got an after sun cream with aloe vera that you’ve used before with no problems, then perhaps give it a try
Some people use hydrocortisone creams, which you can get from pharmacies /chemists /drug stores. I would suggest caution here as they can only be used for a short period. It is a steroid and works on the chemicals that cause itching and redness in the skin. Creams are designed for conditions such as eczema and rashes and can also cause thinning of the skin. (Preparations with antifungals are sold for athletes foot). There’s useful information about hydrocortisone here https://www.nhs.uk/medicines/hydrocortisone-skin-cream/ I would be interested to hear if you have used a hydrocortisone cream for an itch that is definitely just your Gilbert’s Syndrome, and whether it has worked.
Steroids (such as hydrocortisone and applied directly to skin), antihistamines and antidepressants. These can be prescribed or over the counter. I would suggest that they don’t tackle your Gilbert’s Syndrome and may not deal with the itch that relates to your liver function. They may be helpful by acting in another way, perhaps if you have an allergy, or another skin condition such as psoriasis or eczema for example. Your itching may not be down to your Gilbert’s Syndrome don’t forget. If they do help you, it would be great to hear about it.
Neem is a leaf widely available across India, Pakistan, Bangladesh and Nepal. It is also found in Africa and South America. This plant has been used in medicine for thousands of years. Neem has many properties, including antimicrobial, antifungal and anti-radical. It may even be analgesic (provide pain relief) and protect the liver (but these studies are in rats not people with Gilbert’s Syndrome). https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4791507/
There are many claims for neem as a detox tool. It can be taken as powder, in capsules, in ointments or tinctures or teas. Like many herbal preparations there are few clinical studies of the effect in people, given drug companies aren’t going to make any money from it. Please do read any claims with caution. If you have tried neem, then do share your experience.
Mint – some remedies include mint, but the principal cooling effect in mint is menthol, which is not processed well by people with Gilbert’s Syndrome. I am afraid I’ve only had negative effects from mint, peppermint and menthol myself. However, I’ve not tried mint as a herb on skin, plus I don’t seem to have problems with mint in toothpaste (probably as I try not to swallow it!), but mint tea or any food with mint gives me indigestion or makes me feel sick.
Do you have an experience with mint that you can share?
Other simple solutions that will help with itching and you to stay irritant free
Wear soft comfortable clothes washed in an environmentally friendly sensitive skin detergent. The chemicals in your detergent could be adding to your itching issues. You can find lots of chemical free products over at The Vegan Kind supermarket.
Clothes that are 100% organic cotton or bamboo, or tencel (made from renewable birch pulp) can be really good against the skin.
Try not to overheat (not that easy if you are menopausal or there’s a heat wave!), use cooling pads or gentle cloths dampened with cool water and gently pat the skin (don’t rub), for a cooling and soothing sensation.
An ice cube against the skin will cool and numb the skin, if you can tolerate it.
Do not over wash or bathe as this will damage the skin barrier and make the problem worse.
Avoid perfumes and moisturisers with chemicals that might irritate your skin. Try detergents, cosmetics and washes that are for sensitive skin. They will be less likely to trigger an itch.
I hope that you find the tips give you some relief if you experience itching. You might have tried solutions that work for you and which are not mentioned here. Please share your experiences as it’s often the only way that other people with Gilbert’s Syndrome will find help. Please share them in the comments, @GilbertsSyndrom , or email email@example.com . I’ll share them out and we can all live better with Gilbert’s Syndrome
So you have lots of bits of information about COVID-19, but what do they add up to if you have Gilbert’s Syndrome? Well, there are a number of things I can tell you which should help join the dots and give you the big picture of where you stand when faced with COVID-19, if you have Gilbert’s Syndrome.
First of all, we have to understand what type of virus COVID-19 is.
It’s a virus similar to the flu and colds. Because it’s new no-one had it before and so no-one is immune and can catch it easily. You can catch it the same way you catch any cold or flu – the virus travels in tiny droplets pushed out from the infected person’s nose and mouth when they cough or sneeze. It either goes straight into your mouth or nose, or sits around for a while waiting to catch a ride on your body. The virus hijacks your cells in your nose, throat and lungs and multiplies. Your body then responds with the usual attack mechanisms, and depending on how healthy your immune system is, and how much of the virus you have been exposed to, you can see it off or have a more severe, possibly fatal reaction. The severe reaction is partly because the body’s immune system response goes into overdrive and the lung tissues become blocked because they become inflamed – which is why people have difficulty breathing.
Okay, so far, it’s pretty straightforward – but there are differences in how viruses can affect people. This one is pretty different where children are concerned (see below). Others can also affect the liver more – recently the pandemic of 2009, H1N1, was shown to cause liver damage: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4941665/
So far, studies show that COVID-19 doesn’t seem to cause permanent liver damage, although there can be some impact on the liver initially, which doesn’t appear related to the medication given to patients, as this extract from The Hindu, and the Lancet journal text extract below show:
Liver damage in mild cases of COVID-19 is often temporary and the organ can return to normal without any special treatment. This could be due to the state of direct infection of liver cells or could as well be due to liver cells getting caught up in the immune war between body’s immune system and the virus with chemicals produced by our body, namely cytokines.The Hindu
‘mild liver test derangement is present at baseline in many patients with COVID-19 before significant medication use. Ie. the liver is impacted before medication is given’
‘It has been proposed that COVID-19 causes direct liver injury via a viral hepatitis, but we believe that there are alternative explanations. First, the derangement of liver function is clearly mild. Second, when liver function tests for patients with different durations of symptoms are examined, there is no evidence that later presentation is associated with greater liver function derangement.’
What’s also great to hear is that: ‘worse outcomes were not seen in the 42 patients with chronic liver disease and COVID-19 who had outcome data’
The greatest problem that people will face when fighting off COVID-19 are underlying health problems which impact the immune system:
So, you should not worry more if you have Gilbert’s Syndrome as COVID-19 doesn’t appear to have an especial impact on the liver. However, just on flu and colds generally – the stress on your body can make you feel really unwell and trigger your Gilbert’s Syndrome symptoms. This is important to tell clinicians if they are treating you so that they can understand what’s happening to your body and why.
There may be other questions that we can’t answer such as – is there a link between low white blood cell counts, Gilbert’s Syndrome and fighting infection; what if some of the medication used in the treatment of COVID-19 is processed in the pathways of the liver affected by our enzyme deficiency. As it appears there is a link between high levels of bilirubin and reduced white cell count https://journals.sagepub.com/doi/10.1177/0004563214541969 (topic for another post!), you could potentially hypothesize that this is a good thing – as the overproduction of white cells is part of the excessive inflammatory response I talked about that can actually harm not heal. As mentioned above – do let your clinicians know you have Gilbert’s Syndrome and that some medication, processed in the Phase II pathways, isn’t dealt with as well.
Why are children less affected when their immune system is still developing? It’s theorized it is BECAUSE their immune system is developing that the life threatening response to COVID doesn’t occur as much in children – the adult body’s immune response includes a ‘cytokine storm’ which results in an inflammatory response in the lungs, making it hard for adults to breathe. Children’s bodies don’t yet respond as aggressively to the virus.
As age advances, the immune system undergoes profound remodelling and decline, with major impact on health and survival [81,82]. This immune senescence predisposes older adults to a higher risk of acute viral and bacterial infections. Moreover, the mortality rates of these infections are three times higher among elderly patients compared with younger adult patients . Infectious diseases are still the fourth most common cause of death among the elderly in the developed world. Furthermore, aberrant immune responses in the aged can exacerbate inflammation, possibly contributing to other scourges of old age: cancer, cardiovascular disease, stroke, Alzheimer’s disease and dementia . During a regular influenza season, about 90% of the excess deaths occur in people aged over 65.
I hope this information helps you live better with Gilbert’s Syndrome!
The word “jaundice” comes from the French word jaune, which means yellow.
Jaundice is a yellowish staining of the skin, the whites of the eyes, and mucous membranes by bilirubin, a yellow-orange bile pigment. Bilirubin (read more about it here!) comes mainly from the breakdown of red blood cells, and is basically a waste product that your liver gets rid of.
Jaundice usually appears when the bilirubin in your blood is more than 3 mg per dL (51.3 µmol per L). The classic definition of jaundice is a serum bilirubin level greater than 2.5 to 3 mg per dL (42.8 to 51.3 µmol per L) as well as having yellow skin and eyes.
Your body processes bilirubin in 3 phases: prehepatic (before it gets to the liver), intrahepatic(whilst in the liver), and posthepatic(after leaving the liver). If any of these aren’t working properly you can become jaundiced.
The human body produces about 4 mg per kg of bilirubin per day, from the breakdown of blood cells. Bilirubin is then transported from to the liver for conjugation (where it needs to link up with other enzymes and chemicals so that it can be removed from the body) .
Unconjugated bilirubin (the bilirubin that hasn’t been removed by linking up with an enzyme) doesn’t dissolve in water but is soluble in fats. That means it can easily cross the blood-brain barrier or enter the placenta. The unconjugated bilirubin is conjugated (linked up with) with a sugar via the enzyme glucuronosyltransferase (the enzyme that people with GS don’t have enough of) and is then soluble in the bile.
Once soluble in bile, bilirubin is transported through to the gallbladder, where it is stored, or passed on to the duodenam. Inside the intestines, some bilirubin is excreted in the stool, while the rest is dealt with by the bacteria in your gut.
JAUNDICE AND OTHER SYMPTOMS
Some people with jaundice have no symptoms at all. But some may have an acute illness, which is frequently caused by infection, may seek medical care because of fever, chills, abdominal pain, and flu-like symptoms. For these patients, the change in skin color may not be their greatest concern!
Patients with noninfectious jaundice may complain of weight loss or itching / skin discomfort. Abdominal pain is the most common symptom in patients with pancreatic or biliary tract cancers.Even something as nonspecific as depression may be a symptom in patients with chronic infectious hepatitis and in those with a history of alcoholism.
‘False’ jaundice can happen if you eat foods rich in beta-carotene (e.g., squash, melons, and carrots). Unlike true jaundice, you don’t get yellow eyes, or changes in bilirubin level.
CAUSES OF JAUNDICE:
Unconjugated hyperbilirubinemia (this is when you’ve too much bilirubin in your bloodstream because it’s not been processed in the liver, as in Gilbert’s Syndrome) might happen before bilirubin has entered the liver cells or within the liver cell. If you’ve had an unusual breakdown in your red blood cells then there may be too much of the waste product, bilirubin, for your liver to process as normal.
This will usually result in mild bilirubin elevation, to about 5 mg per dL (85.5 µmol per L), with or without clinical jaundice. The blood might be breaking down because of a number of causes in the blood cells or your enzymes which mean your red cells have stayed alive longer than normal, and built up. Other causes include autoimmune disorders, drugs, and defects in hemoglobin structure such as sickle cell disease and the thalassemias.
Un-Conjugated Hyperbilirubinemia Gilbert syndrome is a common, benign, hereditary disorder that affects approximately 5 percent of the U.S. population. It usually results in a mild decrease in the activity of the enzyme glucuronosyltransferase. Gilbert syndrome is typically an incidental finding on routine liver function tests, when the bilirubin level is slightly increased and all other liver function values are within normal limits. Jaundice and further elevation of the bilirubin level may occur during periods of stress, fasting, or illness.
Conjugated Hyperbilirubinemia. Main causes are when there’s a blockage preventing bilirubin from moving into the intestines. Viruses, alcohol, and autoimmune disorders are the most common causes of hepatitis. Inflammation also disrupts transport of the bilirubin and causes jaundice.
Hepatitis A can cause acute onset of jaundice. Hepatitis B and C infections often do not cause jaundice straight away, but can lead to jaundice when chronic infection has led to liver cirrhosis. Epstein-Barr virus infection occasionally causes hepatitis and jaundice that resolve as the illness clears.
Alcohol has been shown to affect bile acid uptake and secretion, stopping the normal flow through the liver. Chronic alcohol use may result in fatty liver (steatosis), hepatitis, and cirrhosis, with varying levels of jaundice. Fatty liver, the most common liver problem, usually results in mild symptoms without jaundice but occasionally progresses to cirrhosis. Hepatitis secondary to alcohol use typically presents with acute onset of jaundice and more severe symptoms.
More rare conditions that can cause jaundice: Autoimmune hepatitis traditionally has been considered a disease that affects younger persons, especially women. Two serious autoimmune diseases that directly affect the biliary system without causing much hepatitis are primary biliary cirrhosis and primary sclerosing cholangitis. Primary biliary cirrhosis is a rare progressive liver disease that typically presents in middle-aged women. Fatigue and itching / skin discomfort are common initial complaints, while jaundice happens later. Primary sclerosing cholangitis, which is also rare, is more common in men; nearly 70 percent of patients also have inflammatory bowel disease. Dubin-Johnson syndrome and Rotor’s syndrome are rare hereditary metabolic defects that disrupt transport of conjugated bilirubin.
Common drugs can also cause problems, such as acetaminophen, penicillins, oral contraceptives, anti-psychotic medication, and steroids. Cholestasis can develop during the first few months of oral contraceptive use and may result in jaundice.
Gallstones in the gallbladder are fairly common in adults. Obstruction within the biliary duct system can inflame the gallbladder, and can lead to infection. Cholangitis is diagnosed clinically by the classic symptoms of fever, pain, and jaundice, known as Charcot’s triad. Cholangitis most commonly occurs because of an impacted gallstone, which might then be removed.
Biliary tract tumors are uncommon but serious causes of posthepatic jaundice. Gallbladder cancer classically presents with jaundice, enlarged liver, and a mass in the right upper quadrant (Courvoisier’s sign). Another biliary system cancer, cholangiocarcinoma, typically manifests as jaundice, itching / skin discomfort, weight loss, and abdominal pain. It accounts for roughly 25 percent of hepatobiliary cancers.
Jaundice also may arise with pancreatitis. The most common causes of pancreatitis are gallstones and alcohol use. Gallstones are responsible for more than one half of cases of acute pancreatitis, which is caused by obstruction of the duct that drains the biliary and pancreatic systems.
The initial work-up of the patient with jaundice depends on whether the hyperbilirubinemia is conjugated (direct) or unconjugated (indirect). A urine anlysis that is positive for bilirubin indicates the presence of conjugated bilirubinemia. Conjugated bilirubin is water soluble and so passed through urine.
SO IF YOU’RE JAUNDICED WHAT DO THEY DO TO WORK OUT WHAT’S CAUSING IT? Here’s the clinical information :
First-line serum testing in a patient presenting with jaundice should include a complete blood count (CBC) and determination of bilirubin, aspartate transaminase (AST), alanine transaminase (ALT), gamma-glutamyl transpeptidase, and alkaline phosphatase levels.
Depending on the results of the initial tests, further serum tests or imaging studies may be warranted. The second-line serum investigations may include tests for hepatitis A IgM antibody, hepatitis B surface antigen and core antibody, hepatitis C antibody, and autoimmune markers such as antinuclear, smooth muscle, and liver-kidney microsomal antibodies. An elevated amylase level would corroborate the presence of pancreatitis when this condition is suspected based on the history or physical examination.
Ultrasonography and computed tomographic (CT) scanning are useful in distinguishing an obstructing lesion from hepatocellular disease in the evaluation of a jaundiced patient. Ultrasonography is typically the first test ordered, because of its lower cost, wide availability, and lack of radiation exposure, which may be particularly important in pregnant patients. While ultrasonography is the most sensitive imaging technique for detecting biliary stones, CT scanning can provide more information about liver and pancreatic parenchymal disease. Neither is good at finding stones inside the ducts.
Further imaging that may be done by a gastroenterologist or interventional radiologist includes endoscopic retrograde cholangiopancreatography and percutaneous transhepatic cholangiography.
A liver biopsy provides information on the architecture of the liver and is used mostly for determining prognosis. It also may be useful for diagnosis if serum and imaging studies do not lead to a firm diagnosis. Liver biopsy can be particularly helpful in diagnosing autoimmune hepatitis or biliary tract disorders (e.g., primary biliary cirrhosis, primary sclerosing cholangitis).
If you’ve been diagnosed with Gilbert’s Syndrome, then you’ve most probably been told that your bilirubin levels were high. It’s one of the most common ways of determining that a patient has Gilbert’s Syndrome, and usually happens after general blood tests.
But what is Bilirubin, what does it do and will it harm us?
Bilirubin comes from the blood when blood cells become old, and die. Hemaglobin, the substance in blood that carries oxygen, is broken down to heme and globin and is passed onto the liver. Bilirubin is formed from the heme part of hemaglobin, and is a bright greenish yellow colour.
The bilirubin is not water soluble when it gets to the liver, and needs an enzyme to become so, then it can be easily passed out of the body. The water soluble substance now passes through the gall bladder as bile and into the small intestine where it continues to be processed by bacteria in the intestines, and is passed out through the faeces and urination.
This enzyme that makes the bilirubin water soluble is called glucuronyl transferease, known as UGT for short, and is what people with Gilbert’s Syndrome are genetically lacking. This means that the bilirubin is not ‘conjugated’ or converted so that it can easily leave the body.
The enzyme also uses blood sugar to help in processing bilirubin, which is one reason why missing meals can affect you. It’s best to ensure you have regular small meals, and cut down on unrefined sugar and carbohydrates like white bread and pasta, so that the amount of enzyme you have can always have a stable supply of blood sugar to do its job.
There is always some bilirubin that is in the blood but this is chemically different from the ‘conjugated’ bilirubin. GS sufferers however, have a lot more of this ‘unconjugated’ bilirubin because it hasn’t been disposed of as it should through the usual process. It’s the difference between the 2 types that tips off those conducting the lab tests on your blood that you have GS.
When bilirubin builds up in the blood at more than around 2.5mg/dl, then the skin and eyes become discoloured with yellow, otherwise know as jaundice. There are a number of reasons bilirubin builds up in the blood other than the lack of the enzyme, and it’s important to rule those out before diagnosing Gilbert’s Syndrome. For example the patient’s bile duct could be blocked, or they could have a type of anaemia, or hepatitis.
Drugs can also affect the measurements of bilirubin, and many can increase it such as steroids, some antibiotics, antimalarials, codeine, diuretics, MAO inhibitors, nicotinic acid, oral contraceptives, and others. Drugs that decrease bilirubin measurements include barbiturates, caffeine, penicillin and high dose salicylates.
You’re likely to be diagnosed between the age of 10 and 30. Males are more likely to have Gilbert’s Syndrome than females, and there are some slight differences in the gene depending on racial background. Many people will not even notice they have it.
People with Gilbert’s Syndrome are often diagnosed after a blood test shows a high level of bilirubin in their blood. You may have gone to the Dr with an unrelated problem, or have presented with a number of symptoms such as feeling tired all the time, feeling sick, jaundice (yellow skin or eyes), abdominal pain, IBS, lack of concentration, and generally feeling unwell. At least 30% of people with Gilbert’s Syndrome may have no symptoms at all.
The Dr will first rule out other liver problems, usually through liver function tests on your blood which will show if the liver is damaged as well as checking the level of bilirubin, and provided you have no other easily identifiable problem you may be diagnosed with Gilbert’s Syndrome.
Sometimes tests are conducted before and after a 48 hour fast as this seems to really raise the bilirubin levels in people with Gilbert’s Syndrome. Bilirubin levels in people with Gilbert’s Syndrome can change over time, and so may be missed if they happen not to be raised at the time of the blood test. Bilirubin is measured in millimoles per litre (umol/L). Total serum bilirubin higher than 17 umol/L is outside the normal range. The bilirubin typically fluctuates in the range of 20 – 40 mmol/L although normal values may occur, and results as high as 80 mmol/L may be seen in patients who have not consumed food for several days. Gilbert’s Syndrome is the most common cause of raised bilirubin levels, however there are rare and more serious conditions such as Criglar-Najjar Syndrome which is inherited from the same gene.
Very rarely a liver biopsy may be performed to rule out other liver conditions, and you may also be offered genetic testing.
Unfortunately, after diagnosis you are likely to be offered little help to deal with your symptoms. However, if you are experiencing nausea, IBS, or other problems you may be prescribed medication that can help. For other symptoms, well, we’re here to help and hopefully some of the information on this website will help you live better with Gilbert’s Syndrome.