At my two week checkup, the nurse and my mom noticed that I was jaundiced. The doctors quickly diagnosed me with biliary atresia (BA), a rare liver disease that occurs in newborn infants.
Infants with this disease have bile ducts that are either non-existent or blocked between the liver and the small intestines. Bile is a fluid that is produced in the liver and helps to break down fat. Because these bile ducts are blocked, bile is unable to properly flow from the liver to the small intestines; therefore, this causes bile to be trapped in the liver with nowhere to go. The buildup of bile in the liver causes liver damage, scarring (cirrhosis), and in the worst cases, liver failure.
I was, according to Cincinnati Children’s Hospital, “one in 15,000 to 20,000 babies” born with biliary atresia. My parents were very worried about my deteriorating health. And, my mom was very emotional because she thought she had done something wrong in her pregnancy that caused me to be increasingly sick. The doctors assured her that her pregnancy was completely normal and that it was not her fault. Nobody, not even doctors, have determined the cause of the disorder.
What is the cause of biliary atresia?
The cause of biliary atresia is unknown, but it is not genetically inherited from the mother or any other family members. Researchers at the University of California San Francisco have hypothesized that a prenatal or early postnatal event such as “infection with a virus or bacterium, a problem with the immune system, an abnormal bile component, or an error in the development of the liver and bile ducts” could trigger the disease.
One of the main symptoms of BA is jaundice. Jaundice is a yellow discoloration of the infant’s skin or the whites of his/her eyes. Jaundice is caused by a buildup of bile, a yellow colored substance produced by the liver that helps with the digestion of fat.
Bile also helps with the removal of waste products from the liver. Ordinarily, bile is moved from the liver by the biliary system of channels and ducts to the gallbladder and eventually from the gallbladder to the small intestine. Infants with biliary atresia are unable to do that which leads to significant jaundice. Jaundice generally occurs in the infant in the first couple of weeks after birth and continues to worsen as time goes by.
My first surgery
Five weeks after birth, on December 19, 1997, I underwent the Kasai procedure at Lutheran General Children’s Hospital to help restore my bile flow from my liver to my small intestines.
I did not find out until a couple of years ago that my Kasai procedure, as performed by my surgeons, was a cutting-edge surgery in 1997.
There are a couple of different ways to perform the Kasai procedure. The way my surgeons performed the surgery was very innovative, especially since few surgeries had been performed that way during this year. I am thankful my surgeons performed the innovative Kasai surgery. If they had not, then this surgery might not have been as successful as it continues to be, and I probably would have had to receive a liver transplant by now.
The Kasai Procedure is a major surgery that ultimately attaches the infant’s small intestines to his liver to replace the bile ducts. The small intestines act as the new bile ducts to release the bile from the liver.
The Kasai procedure is only a treatment. It is not a cure. There can be complications with this surgery that make it unsuccessful. The younger the infant is when the Kasai is performed, the more successful it is.
If the Kasai procedure is not successful, then a liver transplant is needed. During a liver transplant, the surgeon removes the damaged liver and replaces it with a new one from either a living or deceased organ donor. A living organ donor, with the same blood type, can give an infant with BA part of his liver. The donated liver regenerates in the infant as well as in the living donor. A deceased organ donor would give a small portion of his liver to the affected infant. A liver transplant is the most successful treatment for biliary atresia.
My second surgery
During the summer of 2017, I walked for the first time in the American Liver Foundation’s Liver Walk in Chicago.
Within the following weeks, I became very sick. Every time that I ate, I was in severe pain. When my pain became unbearable, I went to the emergency room at Mercy Hospital in Aurora, Illinois only to be diagnosed with constipation. Little did the doctors know that there was a more serious issue that was causing my pain.
Two days after my visit to the ER, I woke up in the middle of the night with excruciating pain. This pain was the worst pain I had ever experienced. I ranked the pain level as a ten out of ten.
Unfortunately, at that point in time, I was not at home because I was working a softball tournament in Milwaukee, Wisconsin. I called my parents at 5:00 A.M. to come and get me and take me to the emergency room.
My parents rushed from Batavia, Illinois to Milwaukee, Wisconsin to pick me up. As we were driving from Milwaukee, to Mercy Hospital, in Aurora, Illinois I started throwing up in the car. After we arrived at the hospital, the doctors ran several tests, and they determined that my liver was very sick and that there was a blockage. They did not know exactly what was causing the blockage between my liver and small intestines. I was transported, by ambulance, to Lutheran General Hospital where my specialists were.
I was there less than 24 hours. They discovered that I had a biliary stone the size of a golf ball near my liver that had developed over a period of 10 years. This stone blocked the passageway for digestion.
At this point, I knew I was going to have surgery. Lutheran General could not operate on my liver because it would have been too risky and I could have gone into liver failure. Only a liver transplant unit could handle my case and only liver transplant doctors could do the surgery.
Every doctor knew my disease
I was then transported, by ambulance, to Northwestern Hospital. After I arrived there, I knew I was in the right hospital because every doctor knew my disease and they were confident that they could operate on me.
Northwestern’s liver transplant doctors performed surgery and removed the golf ball sized stone near my liver to restore digestion flow. Luckily, they were able to use the same incision from my Kasai procedure when I was five weeks old.
My second surgery was performed on June 26, 2017. I was in the hospital for about a week.
The unfortunate part of my hospitalization was that I had to miss being the maid of honor at my sister’s wedding. After surgery, I had four months of physical therapy to help me get stronger and to prepare me for my return to college softball. I am currently doing well, and I am thankful I had the doctors that I did to make me better! I would not be as healthy as I am today if it were not for their expertise.
The lucky one
Because my Kasai procedure was successful, I am considered unique. According to Children’s Hospital of Philadelphia, the “overall survival with a native liver (not transplanted) ranges from 30-55 percent at 5 years of age; and 30-40 percent at 10 years of age. It is thought that approximately 80 percent of patients with biliary atresia will require liver transplantation by the age of 20.”
Biliary atresia is the main reason that children receive liver transplants. I am 21 years old and I have yet to receive a liver transplant. It is a miracle that I am one of the 20% that has yet to require a liver transplant at this point in my life.
Most infants that undergo the Kasai surgery have a transplant within three months after surgery. I am the exception to the rule. The liver transplant doctors at Northwestern Hospital that performed my second surgery said that I am in uncharted territory. They have no idea what will happen to me in terms of my liver disease because there are very few individuals that have made it past the age of 20 without a liver transplant. I am a rare case and the lucky one.
Because I know there is a chance that I will have a liver transplant in my lifetime, I encourage others to become organ donors. The ratio of viable organs that are available to wait-listed individuals varies/differs greatly.
According to U.S. Government Information on Organ Donation and Transplantation, in August 2017, 114,000 men, women, and children were on the national transplant waiting list. Of those 114,000, only 34,770 transplants were performed in 2017.
As a result of only a fraction of individuals receiving liver transplants each year, an estimated average of 20 patients die each day awaiting a transplant. The reason individuals need to register to be an organ donor is that 95% of U.S. adults support organ donation, but only 54% are signed up as donors.
Every 10 minutes another person is added to the waiting list. Just one donor could save eight lives through the donation of a heart, two lungs, liver, pancreas, two kidneys, and intestines.
The myths of organ donation
Individuals who choose not to become designated organ donors have three main reasons for not doing so:
- They believe, as an organ donor, if they were to seek medical treatment, the doctors and nurses would not work as hard to save their lives.
- They think that they would not be able to have an open casket when they die.
- They think that they are too old or too young to become organ donors.
The truth about organ donation
Nonetheless, these theories of individuals against organ donation are not true for the following reasons.
- If individuals are registered to be organ donors and they seek medical treatment, the doctors and nurses will do everything they can to save their lives. They do not focus on other lives when trying to save their lives. They are paired with a specialist that is an expert in their conditions.
- An organ donor can still have an open casket funeral. They take special care of all bodies during organ extraction and they appear the same as someone that is not an organ donor.
- No one is ever too young or too old to be an organ donor.
If individuals are under 18 years old and they want to register as organ donors, they can tell their family about their wishes.
If individuals are older and not as healthy, they are still able to be organ donors. They do not rule out organs just because they are older.
Medical professions cannot determine if organs are unsuitable until they run tests to see if they meet the required standards for an organ donor. The more organs there are available, the more lives that can be saved.
Throughout my life, I was always afraid to tell my story, but freshman year of college I had a breakthrough. I started realizing that this liver disease is a part of who I am. I need to embrace it and start sharing my story.
Even though I am an exception and one of the few that has yet to receive a liver transplant, I still want to spread awareness for all of those with my disease that had a liver transplant.
It is not difficult to be an organ donor. All individuals need to do is register to be an organ donor when they obtain or renew their driver’s license. They can also register with the national Donate Life Registry which can be done through the Health App on an iPhone.
An organ could save my life in the future, so I hope that you will take the initiative, do what is right, and become an organ donor.