MY BABY HAS WHAT?!

My baby has what?!

The initial shock

You hear the words “biliary atresia” and, like most of us, do not know what they mean. Immediately a million thoughts rush through your minds, what is it? What can we do? What medication do we need? Why is this happening to our baby? Then the reality starts to set in as the doctor continues to talk and explain what is wrong with your baby. At first, you do not understand all that is being said- words like biliary system, bile ducts, and Kasai procedure make no sense to you. But then, phrases like no-known-cause and no-known-cure hit you like a ton of bricks. Our precious, seemingly healthy baby but with skin and eyes that are yellowing in color, lovingly stares up at us. In our hearts’ we know this baby is depending on us to help and you realize how helpless you feel. Every fiber of your being is screaming ‘this can’t be real’!

The wait begins

And then the seemingly endless days, then weeks, and sometimes, months in the hospitals have gone by. A continuous parade of doctors, specialists, nurses, and surgeons (the “team”, as they say) politely nod as they pass-by you each day. A new “normal” sets in. You are consumed with the struggle to learn and the search to find out everything you can about biliary atresia in your own battle to save your baby’s life.

You quickly learn

Biliary atresia is a life threatening condition in infants in which the bile ducts, inside or outside of the liver, do not have normal openings. The bile ducts are tubes that carry ‘bile’ from the liver to the gallbladder for storage and to the small intestine for use in digestion. Bile is a yellowish fluid made in the liver that has two main functions:

1. carry toxins and waste out of your body; and
2. help the body digest fats and absorb fat-soluble vitamins A, D, E and K.

When the bile duct is blocked, the bile is ‘trapped’ in the liver, rapidly causing damage and scarring of the liver cells (cirrhosis), and eventually liver failure. You know your baby has a tough fight to win!

Morgan's insight...

The cause of biliary atresia is not completely understood. It is not contagious and is not passed from mother to child. It is not preventable and not caused by anything the mother did or did not do during pregnancy. Researchers are concluding from studies that the autoimmune mechanisms may be partly responsible. More specifically, the characteristics of the neonatal immune response itself (including Th1 and Th17 responses which are deficiencies in the very important regulatory T-cells), activation of humoral immunity (i.e. a response involving antibodies) or autoimmunity (a “misdirected” immune response that attacks the body itself in error), may explain how and why the unique aspects of the neonatal immune system have gone amiss.

Symptoms:

• Jaundice that persists for more than 2 weeks.

• Dark or brown urine. This is from excessive bilirubin in the bloodstream that passes to the kidneys.

• Pale or clay-colored (acholic) stools. This is because there is little or no bile reaching the intestines.

• Enlarged liver that feels harder than normal, enlarged spleen.

• Poor weight gain.

Because early diagnosis of this disease is crucial for successful treatment, infants who are jaundiced after 4 weeks of age are usually evaluated for biliary atresia. Your baby’s physician may recommend some or all of the following tests to confirm or rule out this diagnosis.

• Blood tests. A liver function test (LFT) and blood clotting factors (PT) as well as a complete blood count (CBC); 

• Liver biopsy; 

• Ultrasound, to identify any clear abnormalities in the liver or any other organ; 

• Nuclear scan, which tracks bile flow from the liver; 

• Sometimes exploratory surgery is employed to examine the liver and bile ducts.

Once you receive a diagnosis of biliary atresia, a Kasai procedure will take place. This is an operation to re-establish bile flow from the liver to the intestine. During this procedure, the surgeon will remove the damaged ducts outside of the liver and then take a segment of your baby’s own intestine to replace the ducts at the spot where bile is expected to drain. This Y-shaped passageway connects to the liver and to the rest of the intestine.

Meanwhile…

The wait continues

You sit, sometimes patiently, sometimes pacing in angst, hoping to hear something, anything, from the surgical team-you can’t believe your sweet baby is having surgery. One day, you had a happy, healthy baby and the next thing you know, you’re sitting in a hospital waiting room…waiting, waiting, waiting.

When you finally get to see your baby, you are so relieved - but OMG! Tubes are everywhere, equipment is emitting strange sounds and you hardly recognize your baby! Another shocked but experienced parent said, “the more bags hanging and tubes inserted, the longer the stay” and you know this will be a long wait.

Another “new normal”

You have learned the Kasai procedure works in about 60% of children and you pray “…please God don’t let our child be in that other 40%!” If the procedure fails, then the term “liver transplant” comes into play and you cannot allow yourself to dwell on that thought!”

Again, you must adjust to another new normal life. You strain to grasp the new words, like bilirubin, ascites, and NPO, so you may research. The PICU (pediatric intensive care unit) team becomes your extended family-you know everyone’s days off, a bit about their children, their daily schedules, if they are the serious type or use more humor to get through their day. You know where the coffee machine is located, what the cafeteria special will be that day, and who to ask for a pillow or a glass of water. It is your new home!

Then, hopefully, your baby starts to get better-and with the recovery comes more to know, more to learn and more to do. You are in charge of an array of meds, each to be given at different times on different days. You think… “can I remember all of this and can I administer all of this correctly?” Panic tempers your overwhelming joy but you know you will do it correctly because that is what a parent does! Now you go home and you enjoy your baby crossing each bridge as it comes.

Knowledge is power!

Biliary Atresia is one of over 100 diseases that damage the liver, many life threatening and many occurring in infants and young children. Please be aware of those threats to you and your loved ones. Knowledge is power and time is of the essence.

REFERENCES:

The Lancet. http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(09)60946-6/abstract
Cincinnati Children’s Hospital. www.cincinnatichildrens.org/health/b/biliary
Children’s Hospital of Pittsburgh. www.chp.edu/CHP/atresia
UCSF. http://pedsurg.ucsf.edu/conditions--procedures/Kasai-procedure.aspx
U.S. National Library of Medicine. www.ncbi.nlm.nih.gov/pubmed/7472936
U.S. National Library of Medicine. www.ncbi.nlm.nih.gov/pubmed/ 26090510
International Scholarly Research Notices. Hindawi Publishing Corp. http://www.hindawi.com/journals/isrn/2012/132089/
Biliary Atresia Awareness & Research. http://www.biliaryatresia.ca/kasai-procedure/
Nature Reviews gastroenterology & hepatology. http://www.nature.com/nrgastro/journal/v12/n6/full/nrgastro.2015.74.html