Daphne was born March 26, 2011.
We spent 4 days in the hospital treating her jaundice with phototherapy. This jaundice was due to ABO incompatibility. Daphne is type A and I am type O.
When Daphne was 3 weeks old her paediatrician, Dr. Lopatka, thought she still looked a bit jaundiced. I could not see it, or believe it because I only saw perfect beautiful Daphne. She also happened to see one of Daphne’s dirty diapers and said the poop was too light. I knew baby poop was supposed to be yellow, but didn’t realize the shade of yellow was so important. Although I thought my baby was perfect, I did the blood work the doctor ordered that very day.
The next day we were told her liver jaundice was too high and biliary atresia was mentioned to us. We had never heard of it up to that point.
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Biliary atresia occurs when the bile ducts inside or outside the liver do not develop normally. It is not known why the biliary system fails to develop normally. The bile ducts help remove waste from the liver and carry salts that help the small intestine break down (digest) fat. In babies with biliary atresia, bile flow from the liver to the gallbladder is blocked. This can lead to liver damage and cirrhosis of the liver, which is deadly if not treated.” (http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002130/)
The next day we saw a paediatric gastroenterologist, Dr. Yap; he examined her and ordered many blood tests and an ultrasound. This process took about one week. I kept hoping something would show up to rule out biliary atresia. However nothing did.
Daphne was almost 6 weeks old when she had a liver biopsy. This was so stressful to us at the time.
The next day we were told the results were consistent with biliary atresia and we met with the surgeon, Dr. Dicken, to discuss surgery. Daphne needed a procedure called a Kasai. She was to get this the very next day.
”In the Kasai procedure, the bile ducts are removed and a loop of intestine is brought up to replace the bile ducts and drain the liver. As a result, bile flows from the small bile ducts straight into the intestine, bypassing the need for the larger bile ducts completely.” (http://digestive.niddk.nih.gov/ddiseases/pubs/atresia/#procedure)
May 10 is when Daphne had her Kasai. One day shy of being 6 weeks old. It was horrible to see her after the Kasai. So little and with so many tubes and an incision across her entire abdomen…no parent wants to see that. Dr. Dicken confirmed that once he opened her up it was very obviously biliary atresia. Her gall bladder and her biliary ducts were completely fibrous tissue. It was a wonder that any bile got through at all and that her liver still looked good. We were encouraged to hear that her liver was still in good condition.
Daphne was a little trooper during her hospital stay. We held her constantly and in 3 days she was smiling more than ever. Four days post surgery she was breastfeeding again.
Six days post surgery Daphne was discharged.
Over the next while I was monitoring her poop colour like a woman obsessed. They were soon bright yellow and/or green. Diaper changing was a joy!
At 2 months of age Daphne got her first vaccines. I remember this stage with Rowan and how horrible it made me feel to do that to her. How it had been the worst thing Rowan had experienced up to that point. When it was Daphne’s turn I knew how much worst things could get. Vaccines were such a small thing when considering the surgery, biopsy, blood work, and ivs Daphne had.
At 10 weeks our happy time at home started to change again. Daphne’s poops were light yellow once more. She was admitted into hospital and treated with antibiotics. She had a mild case of cholangitis. We were in the hospital for 1 week. Although her infection went down, the hospital stay was a big blow to us. Her numbers were not going down as they should. It was starting to look like the Kasai had not been successful. (The Kasai is only successful 1/3 of the time.)
Daphne was sent home on more antibiotics and more vitamins. I was also told to start supplementing my breastfeeding by giving her a special formula, pregestemil, added to expressed breast milk.
The formula was a complete disaster. It is absolutely disgusting. Daphne hated it. She refused to take it. She really would have gone hungry instead of drink it. Since her weight gain was ok, we gave up on the formula and started giving her MCT oil in a syringe. She didn’t like that either, but it was easier to give her 3 mL of that than 3 oz of formula.
At the end of July Daphne had an ultrasound for an enlarged spleen. The ultrasound showed that Daphne had two spleens! Dr. Yap says this means she has the congenital form of biliary atresia rather than the acquired form. This is also when liver transplant was first discussed as not just a possibility but rather as a necessity.
In the middle of August Daphne’s tummy started to get very big. This is called ascites and happens due to severe liver disease. She was prescribed a diuretic which helped instantly. We even went to Banff for the weekend right after this was prescribed.
At the end of August her tummy started getting big again and she had another ultrasound. This ultrasound showed that her main portal vein was blocked. Daphne was admitted to hospital again. Dr. Yap told us that a blockage in the main portal vein means that she is no longer a candidate for living donor liver transplantation. This was a big blow to us. We were hoping to have Brad or I donate a portion of our liver to her so she wouldn’t have to suffer while waiting for a cadaver liver to turn up. This also explained why her ascites was getting bad again.
While in hospital, nurses were unable to get an IV in Daphne’s tiny veins. Since we needed to treat her ascites, Dr. Yap recommended giving Daphne a broviac. This is an intravenous catheter. It is surgically placed to administer medication and also to draw blood from. It may seem extreme to have a surgery for this. But she had such frequent blood work that the broviac was a huge relief to me. No more pokes! Now that she had the broviac they started to treat her ascites with albumin and lasix. Albumin is a human blood product produced by the liver. Albumin helps draw the fluid from her tummy into circulation and the lasix helps remove the fluid through urine.
A very good thing about this hospitalization was that since we were in hospital her transplant assessment was fast tracked. I won’t go into all the details but that week and a half Daphne underwent a great deal of tests and we met a lot of people!
Another development of this last hospitalization was that Daphne got an ng tube. This tube goes through her nose into her stomach. We use it to give her pregestimil formula continuously through the night. This should help her gain weight and also fight the ascites. I also love that it can be used to give her oral meds without her spitting them all out.
Her transplant assessment went well over all. The most stressful part was the development of the blood clot. The reason it formed is because of the condition of her liver. The blood cannot flow through the liver well; this causes a back up before the liver in the main portal vein. Because the blood is flowing slowly there, it tends to clot. Daphne was put on blood thinners once this was found, because as Dr. Yap said, if the clot was to spread it would be “very bad.” Something in his tone told me just how bad this would be. I was later to learn the very bad result would require a multi-organ transplant. No one explained this to us immediately. That was for the best I’m sure. I probably would have freaked out.
Well at one point during the assessment week it seemed that the clot might have spread, although they did say the flow had improved (confusing indeed!) Needless to say, this was another big blow to us. But we kept up with her blood thinners and further tests showed excellent flow. So much so, that there may not be a clot anymore. Unfortunately, that is not enough to rule out the presence of one, so she is still not able to have a living donor liver transplant. The good news is that the transplant she needs is liver only.
On September 9th she was listed for transplant! The following are some of the stats regarding her transplant. She is status 1. She can receive a liver from blood group A or O. Her donor needs to be between 5 kg to 60 kg. She has a PELD of 43. The average wait time for a liver is 3 months. Daphne would be the first in our region to get a liver if one became available.
Since Daphne was listed she has been put on hold on the transplant list for one day. This was because the diuretics she was on to combat the ascites caused her sodium to drop too low. Her meds were adjusted and her sodium level soon rose to an acceptable level to get her back on the list.
Daphne’s next medical event was getting a new broviac. Her first broviac would no longer draw blood. A chest x-ray showed that the broviac had come out of the vessel. Her new broviac does not look very good because of the amount of blood thinners she is on. The site is very bruised and keeps bleeding.
Last week Daphne’s albumin was low and she was having difficulty sleeping because of her ascites. She was put on albumin again. It helped but I have a feeling these albumin treatments will be a regular occurrence.
To wrap this up…. we come to today, Daphne’s 6 month birthday. Today Daphne got some more vaccinations. No more live vaccines though, that would delay a transplant. Daphne also met with the transplant team. They are very encouraged by her weight gain so far (7.7 kg today). And of course they all adore her! How could you not! She is gorgeous! But we are still anxiously awaiting that phone call telling us they have a liver for Daphne.
