Sadly, nearly all of those diagnosed with Biliary Atresia will require a liver transplant at some
point their lives, over 60% within the first five years of life. Without a liver transplant they will not
survive. Biliary Atresia is the leading cause of liver transplants in children.
By definition, a liver transplant is the replacement of the diseased liver with either a full of half liver that comes from another individual. Due to the risks of liver transplant both during and after the operation they are only done as a last resort when the liver is no longer responding to other forms of treatment and therefore the patient is in liver failure. Cirrhosis, without a liver transplant, is a fatal condition.
Liver failure can be due to many causes. It can happen suddenly (acute liver failure) or over a long period of time, months or years,(chronic liver failure). In Biliary Atresia the liver failure is chronic.
Once you are an active liver transplant candidate you will be placed on the waiting list for a new liver, everyone on this list is listed according to their blood type, body size and how ill they are.
Once officially listed you will receive a score that determines your need for a new liver, the score will be based upon your blood results and the complications you are experiencing throughout your wait. The score is called the PELD (pediatric end stage liver disease) or in adults the MELD (model of end stage liver disease). This score priorities each individuals need for a new liver, patients with the highest scores are transplanted first. The sicker you become, the higher your score and priority for transplant will become. Those with acute liver failure have the highest priority as they are often critically ill.
The wait for a new liver is often very difficult emotionally and mentally, the individual is dying from liver failure, waiting and hoping for a second chance at life. This is obviously very hard and draining on not only them but everyone close to them so it is important to have adequate support throughout this time.
With Liver transplants, and kidney transplants, the new organ came come from either a deceased or live donor.
Criteria for live liver donation:
The donor must be doing this voluntary. They will be not allowed to donate if they are found to be under pressure or guilt, or if they are being paid for their donation.
It is important to let your family know your wishes as they ultimately have the last say, even if you were a registered an organ donor.
Once the transplant is complete the patient will go to the intensive care unit where they will be
carefully monitored by nurses and doctors, especially in the first 12-48 hours. Once the patient
is stabilized the breathing tube can be removed and they will be slowly transitioned into a
regular hospital room. Typically the average patient spends 2-4 weeks in the hospital, however
some remain in hospital much longer and a few are released after a week. It all depends on
how sick you were prior to transplant and if any complications have arose afterwards.
Risk during and after the liver transplant:
As with any operation there are risks, and complications can occur. Some of the potential
complications during and after are:
• Blockage of the blood vessels and/or bile ducts to the new liver
• Blood clots in the liver
• Leakage of bile
• Initial lack of function of new liver
It is important to know that the newly transplanted liver may not function for a brief amount of time after transplant, or it may reject. This is somewhat normal in that the recipients immune system is reacting to what it perceives as a threat and therefore it attacks the new organ. In order to allow the new organ to survive and to prevent post transplant liver failure anti-rejection medications must be taken as they trick the immune system into accepting the liver transplant. These medications however make the recipient severely immune compromised.
In some patients however the anti-rejection medications don’t work and the body ultimately rejects the new organ to such an extent the recipient requires another liver transplant in order to survive. With Biliary Atresia we have seen many individuals require 2 or 3 liver transplants. Post liver transplant failure can happen weeks, months or even many, many years after the initial transplant.
Symptoms of rejection can include, but are not limited to, the following:
• Jaundice (yellowing of the eyes and skin)
• Dark urine
• Fatigue and/or irritability
• Abdominal swelling and/or tenderness
A liver transplant gives an individual a second chance at life, but it is important to note however that a liver transplant is not a cure and with biliary atresia you are merely trading pre-transplant problems for post-transplant problems. Some of the post transplant complications can include:
Despite the anti-rejection medication the immune system attacks the new liver causing it to stop working properly, this occurs in approximately 1 out of 3 people. In most cases it is treatable but in some another transplant is required.
Due to the anti-rejection medication you will be more vulnerable to infections.
• Kidney Failure
Occurs in up to 1 out of 3 people post liver transplant, typically due to the anti-rejection medication you are taking. Doctors will closely monitor your kidneys post transplant but if they do fail dialysis and/or a kidney transplant will be needed.
• Graft Failure
Simply means the new liver is not working properly and although treatment can stabilize the body for a short while the only ‘cure’ is another transplant. This occurs in about 1 in 10 people and is one of the most serious complications.
Those who have had a liver transplant are at a greater risk of developing certain types of cancer such as cervical cancer, non-melanoma cancer and melanoma skin cancer. Another form of cancer, PTLD (post-transplant lymphoproliferative disorder) affects the white blood cells in those who have had an organ transplant. The risk of this is about 1 in 50. For this reason you will be occasionally screened for cancer.
To register as an Organ Donor
Liver transplants, or any transplant for that matter, save the lives of people who otherwise would die waiting for a second chance. Organ failure does not discriminate, it could so easily be someone you love or know and it could so easily be you. Often the only thing shielding you from this is pure luck. Biliary Atresia specifically has NO cause and affects tiny, innocent babies.
One organ donor can save the lives of up to 8 people and enhance over 75 more. An organ donor is a hero in the midst of a tragedy. Please take the time to register as an organ donor:
Canada –Site to register in Canada
America – Site to register in America
Australia –Site to register in Australia
New Zealand –Site to register in New Zealand
United Kingdom –Site to register in the United Kingdom
Pictures of just Biliary Atresia Fighters post transplant and new/old livers, warning these photo’s are graphic.