Our daughter, Mikylah Loxton, was born on February 4, 2009 a healthy 6 lb., 12 oz. girl. She did not show any signs of illness, but at her 2 week check-up her doctor said that he saw signs of jaundice in her eyes. Nothing to be alarmed about; most babies are a little bit jaundiced shortly after birth.
Then at about 4 weeks old we noticed that her bowel movements were white and almost clay-like. Again, we phoned the doctor and he asked us if we had changed anything in Mikylah's diet. It just so happened that we had switched her from concentrate formula to powdered. The doctor said there was nothing to be alarmed about, and that her change in diet would balance itself out.
We had company on March 29, 2009 and they said that Mikylah's skin and eyes looked very yellow. We of course did not notice this because we are with her every day, but we decided to take her to the doctor's office for him to examine her. He sent us to the North Bay Civic Hospital for blood tests on April 1, 2009. Mikylah was now 8 weeks old.
On April 2, 2009 we received a phone call from our physician saying that he had arranged an appointment with a pediatrician that same day. We went to the appointment, and from there were sent for more blood tests and an ultrasound to be done on Mikylah. We got a phone call that same night from the pediatrician, telling us that we needed to be at Toronto's Sick Kids Hospital by Sunday, April 5, 2009. She couldn't tell us anything more than that they had discovered that Mikylah's bilirubin levels in her blood were high. This of course meant nothing to us at the time because we didn't know what bilirubin was.
We got to the hospital on Sunday April 5, 2009 at around 2:00 p.m. They told us to be prepared to stay for at least one week while they performed a battery of tests, including a liver biopsy, to determine what Mikylah's diagnosis was.
April 10, 2009- Easter weekend. The teams of doctors entered our room at Sick Kids for a consultation. Mikylah looked like a pin cushion; black and blue from all the blood tests and LV. lines, and a patch on her tummy covering where her biopsy was performed. Then the doctors confirmed their suspicions.
They diagnosed our baby with a disease called Biliary Atresia. It is a relatively rare disease that affects about 1 in 20,000 babies soon after birth. The common bile duct that transports bile from Mikylah’s liver to her intestine was damaged so it prevented the bile from leaving her liver, which damages her liver tissue. They also told us that Mikylah needed an operation called a Kasai, in order to attempt to create a passage for bile flow. Time was of the essence because babies that pass the age of 14 weeks typically cannot be helped due to the damage already done to the liver. We were sent home for Easter and had to return to the hospital on April 15, 2009.
On April 15, Mikylah was 10 weeks old. Her operation was scheduled for April 16, 2009 at 8:00 a.m. We handed our daughter over to the surgeon at 7:30 a.m. that morning knowing that we would not see her until later that night. At 4:00 p.m. Dr. Wales met us in the operating waiting room and told us that "medically speaking" the operation went great. He removed Mikylah's gall bladder (it was damaged too) and the compromised bile ducts. Then he cut her small intestine, brought it up to her liver, and attached it directly onto her liver. The hope is that Mikylah's liver will empty the bile directly into her intestine.
We were allowed to see Mikylah for a minute in the observation room. We were fore-warned about how she would look. She was sleeping due to the continuous drip of morphine that was LV.' d into her hands, which were bound like boxing gloves. She had a feeding tube taped to her nose, monitor pads stuck to her body to keep track of her vital signs, and an incision that stretched from the right side of her ribs to just past the center of her rib cage. Mikylah spent 5 days in the constant observation room where she spiked two fevers and was put on a series of antibiotics. On April 25, 2009, the doctors sent us home.
Mikylah, so far, is doing okay although it's hard to determine whether or not the operation is working. She has to eat a special formula, which can only be ordered in (stores don't stock it), a concentrated vitamin which is only available in the United States and can only be ordered by Sick Kids Hospital, antibiotics to protect her liver, and a drug to help her body produce bile. She will be on these medicines for the rest of her life, and will also have to have routine blood tests.
We will be going to Sick Kids every month until Mikylah is 18 years old for check ups, and after that point, she will go to St. Michael's. Mikylah's journey has only begun. There is no cure for Biliary Atresia, but through a successful Kasai operation, it could be treated.
The success for a Kasai is divided into thirds. One third of the babies who have it carry on with "normal" lives and never need another operation; one third- the operation will work for an undetermined period of time and then mysteriously quit working, and one third-the operation doesn't work at all. In the last two scenarios, a liver transplant is necessary. The doctors have given us the information to prepare us for the possibility when Mikylah might need a transplant. Our hope is that she falls in with the one third of the babies who carry on with normal lives, and beats the odds!
Update 2
Tuesday April 28, 2009-Being home with Mikylah was a reality we weren't prepared for; a terrifying reality. There were no nurses here to help us out; no doctors to answer the thousands of questions we had. Would we hurt her when we picked her up? How do we give her a bath with the incision on her tummy? Why does she keep vomiting after her bottles?
We had an appointment with Mikylah's pediatrician today. The concern with the doctors is that Mikylah is not gaining weight and therefore cannot develop normally. The doctor suggested that we get Mikylah immunized for as many vaccines as possible, or as many as her Sick Kids doctor's would recommend.
On Tuesday May 5, 2009 we were back at Sick Kids for a check up with the liver clinic. Mikylah was weighed at 4.7 kg (around 10 pounds) and was 3 months old. Through a blood test it was determined that her INR count was high. This is a test that shows how well your liver is producing vitamin K which is responsible for blood clotting. It's very closely monitored in children with Biliary Atresia because it determines whether your liver is functioning well or not. The doctor's decide to check her levels again in one week to see if Mikylah needs to be taking a vitamin K supplement. We make our way back home that night, exhausted after a 14 hour day.
Wednesday May 6, 2009. Mikylah has an appointment with our family physician to receive her standard 2 month immunizations. She weighs 4.6 kg today. Her excessive vomiting is causing her to progressively lose weight. We make an appointment for one week to get her Hepatitis B vaccine and Menjugate which aren't typically administered until 1 year of age.
Wednesday May 13, 2009. Back to Sick Kids. Mikylah had blood work done at 8:00 a.m. today. She still hates it. The doctors put a STAT advisory on it so we could get the results the same day to see how her INR levels were. She had an ultrasound at 9:30 a.m. to follow up on a blood clot that was detected in the left portal vein in her liver.
10:30 a.m.-back up to the liver clinic to get the blood results. The INR have balanced them selves for now and will be very closely watched. Mikylah's vitamin D levels are quite low, so she gets a prescription for a vitamin D supplement. She still has not gained weight so her dietician and liver team decide to increase the caloric value of her formula. This basically means more powder to less water. 11:30 a.m.-we meet with the research team.
Mikylah is part of their research program to determine what causes Biliary Atresia. Since the 1970's, Toronto's Sick Kids Hospital has had only around 250 babies with Biliary Atresia. Their studies have found commonness in some of the patients regarding the 22nd chromosome, but nothing conclusive. Being part of this group allows us to receive any findings that they make. Unfortunately, they really haven't discovered very much at this point.
At 2:00 p.m. we have a meeting with the blood clot team to go over Mikylah's ultrasound results. The blood clot has not increased in size so they are not going to take any measures to treat it at this time. They also discovered that her right portal vein showed fibrous material, but again, are not going to treat it yet. We have follow-up appointments for it in one month.
5:00 p.m. We head back home.
Thursday May 14, 2009. Mikylah has an appointment again with our family physician to get her Hepatitis B and Menjugate shots. Typically, they would be administered in one needle but because Mikylah is getting them at a younger age, and is so small, they have to divide them into a series of 3 shots. Upon leaving, we have 4 more appointments booked for the upcoming months
It is our job as Mikylah's parents to keep her safe and healthy (as it is with any parents) so we had to devise a system that would allow her body to keep her formula in her tummy and to gain weight.
This is an outline of our typical day:
3:00 a.m. 4 1/2 oz. "powered" Pregestimil formula
8:00 a.m. 41/2 oz. "powered" Pregestimil formula
9:00 a.m. 1 ml. Ursodiol (drug that helps produce bile)
10:00 a.m. 1 ml. Aquadek & 1 ml. D-Visol (vitamins)
11:00 a.m. 15 mg. Prevacid (to neutralize tummy acid)
12:30 p.m. 4 1/2 oz. "powered" Pregestimil formula
2:00 p.m. 1 ml. Ursodiol
5 :00 p.m. 4 1/2 oz. "powered" Pregestimil formula
8:00 a.m. 41/2 oz. "powered" Pregestimil formula
9:00 a.m. 1 ml. Ursodiol (drug that helps produce bile)
10:00 a.m. 1 ml. Aquadek & 1 ml. D-Visol (vitamins)
11:00 a.m. 15 mg. Prevacid (to neutralize tummy acid)
12:30 p.m. 4 1/2 oz. "powered" Pregestimil formula
2:00 p.m. 1 ml. Ursodiol
6:00 p.m. 1 ml. D- Vi sol
9:00 p.m. 4 1/2 oz. "powered" Pregestimil formula
10:00 p.m. 3 ml. Novo-Trimel Cotrimoxazole (antibiotic)
We figured out that in order to get a baby to take their medicine easily, put it in a nipple and have them suck it out of there. It works great!
Mikylah has been approved for a Trust Fund through TD Canada Trust, and there is a benefit dance being held at the Powassan Legion on Saturday June 6, 2009 for her.
Our next appointment at Sick Kids is on Wednesday June 3, 2009 with her surgeon, and again on Monday June 8, 2009 with the liver clinic to check her weight and perform blood tests again.
People always ask us how she's doing. That's a very difficult question to answer. There is a great deal of difference between the hospital's definition of "doing well", and of society’s definition of "doing well". She will always have this disease because there is no cure. We are always on a constant watch for any symptoms that would require Mikylah to be immediately rushed back to Sick Kids. So far, we've been lucky and hope to continue to be. Either way, we'll keep you posted.