Harmonee Lynn: January 2010

Saturday, January 30, 2010

Harmonee had her Echogram on Wednesday to see why she had bradycardia. Fortunately the the cardiologist saw nothing wrong in any of the ultra sounds. For the procedure she had to have and IV placed because that had to sedate her. When we went back to the room where she was getting her echo done in the nurse gave her 3 medicines to help her fall asleep. Harmonee was so mad that she hadn't been able to eat for about 7 hours now refused to go to sleep at first. For about 20 minutes she was slow motion until finally she gave in and feel asleep. But before they were fully done with the procedure she started waking up hitting the doctors hand to get off of her. By the time we got her to post op she was screaming at everyone to give her some food. She's a stubborn girl!
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We also had to go to liver clinic earlier in the day. They took her off a her prednisone (steroid) nystatin, and smz-tmp, but added metronidazole. At least it less then before. We start Harmonee's point system to see where she is placed on the transplant list her next clinic. We are taking her blood test, and we also get to take mine to see if we officially match. Dr. Book said we should have all of her tests done in a few weeks and by June we should have an actual 'plan of action' for her transplant. Her newest trademark is healing pretty well, but her tummy keeps swelling. Her tummy measured to be 50 cm at her visit. Hopefully it goes down, it looks so uncomfortable. She proves just how strong she is every time we go see her doctors.

Sunday, January 24, 2010

Great news! Brandon (daddy) is back in the running for live donors for Harm! Technically before he wasn't 'out' of the picking. The doctor had said that his liver would probably be to big so there really wasn't a point in taking time to test him. But after having a few in detailed talks with one another we decided he might at well still ought to try. It is his daughter, and I don't think he could live with not even giving it a try. Of course if he is a match, we have to have a few more in detailed talks about income and what not since he is the only set of income coming in right now. But we'll figure something out, we always seem to do.
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Dr. Book called and wanted to see Harmonee on Tuesday this week. We also have an appointment with the transplant coordinator. This means, we are officially going to start testing for live donors. I still hope that it is me. We are eating healthier and working on getting our livers healthy for our little girl. The first time we gave her her liver it didn't work out, this time, I want it to be perfect for her. Of course I am a little scared about the surgery, and I don't know how I would get along with out seeing Layla for a few months while I'm in the hospital, then not be able to do a lot of stuff with her for a few months until I got fully healed. But I am more then willing to happily give my liver to Harmonee. I am even working on getting my degree early and finishing in June instead of September. This way I'll be able to concentrate on Harm, not worrying about everything else at the same time. I just can't until this is all over and Harmonee can get on with her life... for the most part.

Friday, January 22, 2010

This was one of my better days :)!

Our morning and bed time routine

My sleepy day

Most parents usually would be happy to have their baby's sleep most of the day. It allows them to get things done, and to catch up on rest from the midnight feedings. Of course it would be nice for a day. Harmonee has been asleep for the past 3 days. Waking up long enough to drink half a bottle or to take her medications. During her bath I caught her eyes opening a little just to roll to the white again. Moving her didn't seem to phase her the least bit. At night it is nearly impossible to wake her up, and almost not worth it, just to get not even an ounce down her. I know Dr. Book told us that some of her medicine would make her sleepy, she also mentioned that liver patients tend to sleep more often as it is. We only have a few more days on this medication, I cant wait to be able to entertain my little girl again and see her smile and hear her laugh.

Sunday, January 17, 2010

We made our first goals for Harmonee at Liver Clinic on Thursday. After looking at Harmonee's newest ultrasounds, both of her doctors predicted December or maybe even sooner. To help her reach the goals, we have changed her diet to progestamil, which is a formula to help her absorb fats, hopefully to help her gain weight and become an acceptable size. Our long term goal for her is to make it to December, the bigger and older she is the easier the transplant will be.

We have started testing for live donors. We are lucky to have so many friends and family who are willing to give Harmonee her gift. Brandon {daddy} was already ruled out of the picking. Next is me. I hope so much to be a donor so that when the time comes, Harmonee will already have a liver waiting for her.

But for now, Harmonee is as happy and healthy as a little girl with a liver disease could be. She is playing with Layla and today she is a normal 3 month old baby, who doesn't go to liver clinic, or have a scar across her tummy. Her disease is just a minor delay in her happy little life.

Thursday, January 14, 2010

I started this blog so friends and family could track Harmonee's progress through out her disease and transplant. I started the day we found out that her surgery didn't work, and a transplant was inevitable, maybe out of sheer determination to distract my mind away from such ideas. Of course it is nearly impossible to get such things out of your head, especially when it is happening to your own baby. It is difficult to think that I am putting my babies life into anothers hands, trusting them to make the surgery possible, that it will all go well. I don't think any parent with children who are sick and needing a transplant or any other surgery can easily hand over their child for such a serious surgery.
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The worst part about her disease is the unknown. Not knowing exactly when her transplant will come, who will the person be, that will be giving Harmonee her second chance liver, and what things may happen to her before the transplant. I have to admit, before all of this happened I was not a donor. No reasons as to why I wasn't other than the selfish not understanding. The past few weeks since she was first diagnosed has been such a life changing experience there is no way to describe it. Nothing is quite what I had thought of it before. I still don't qualify Harmonee as 'sick'. 'Sick' to me is a contagious cough or flu like symptoms. Realizing that 'sick' is a much wider range of definitions, and that Harmonee is now a definition of 'sick' has brought a broader understanding to the meaning of life for me.

What is Biliary Atresia {BA}

Biliary Atresia is a rare liver disease that only affects infants. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats.
In biliary atresia, the bile ducts become inflamed and blocked soon after birth. This causes bile to remain in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver.

What causes BA:
The cause of this disease is not known. In some infants, the condition is most likely congenital, meaning present from birth. About one in 10 babies with biliary atresia have other congenital defects. Some research indicates that an early viral infection may be linked to biliary atresia.
Scientists do know that biliary atresia is not hereditary; parents do not pass it on to their child. It is also not contagious, and it is not preventable. It is also not caused by anything an expectant mother did or did not do.

How is BA diagnosed:
Since other conditions lead to symptoms similar to those of biliary atresia, doctors must perform many tests before a conclusive diagnosis can be made. These tests may include blood and liver tests, an ultrasound examination, X-rays and a liver biopsy, in which a small amount of liver tissue is removed with a needle for examination in a laboratory.

How is BA treated:
Unfortunately, there is no cure for biliary atresia. The only treatment is a surgical procedure in which the blocked bile ducts outside the liver are replaced with a length of the baby’s own intestine, which acts as a new duct. This surgery is called the Kasai procedure after Dr. Morio Kasai, the Japanese surgeon who developed it.
The aim of the Kasai procedure is to allow drainage of bile from the liver into the intestine through the new duct. The operation is completely or partially successful about 80 percent of the time if performed early (before 3 months of age). In babies who respond well, jaundice and other symptoms usually disappear after several weeks.
In cases where the Kasai procedure does not work, the problem often lies in the fact that blocked bile ducts are “intrahepatic,” or inside the liver, as well as extrahepatic, or outside the liver. No procedure, except for liver transplant, has been developed to replace blocked intrahepatic ducts.
The Kasai procedure is most successful in babies younger than 3-months-old, so early diagnosis is important.
If the Kasai procedure is not successful, the only other option is a liver transplant. However, a suitable donor organ must be found quickly, before damage to the liver from the backed-up bile becomes deadly.

Babies with BA:
Without successful treatment, few children with biliary atresia live beyond age two. In some cases, where the Kasai procedure is completely successful, the child may recover and live a normal life. However, in most cases, even when surgery is successful, patients will suffer gradual damage to the liver. These children will need specialized medical care throughout their lives, and many will ultimately need a liver transplant.

Transplant:
In children with biliary atresia, a liver transplant is generally not attempted unless the Kasai procedure has been tried. In cases where that operation is unsuccessful, the child may become a candidate for a liver transplant. Success depends on the timely availability of a matching donor liver. Fortunately, survival rates for liver transplant recipients have increased dramatically with improved surgical techniques and the development of drugs that help overcome the problem of organ rejection.

What does YOUR liver do for you?

How important is the liver?
The liver is a vital organ. Without it we would not be able to live. Apart from the brain, it is the most complex organ in the body. It has a wide range of functions and acts very much like a factory. One of its most amazing features is its regenerative power. Some 9/10ths of the liver can be cut away and provided the remaining 1/10th is healthy, the liver will grow back to its original size.

The liver is a processor
The food that we eat passes into the stomach via the esophagus (gullet). The food is broken down by the action of the stomach and the small intestine. The nutrients from the food are then absorbed into the blood via the vessels in the walls of the intestines. These drain to veins which lead into the portal vein which carries the blood into the liver. The nutrients are processed in many different ways in the liver. This processing of the food is known as metabolism. The final products made are used by the body for energy and growth.

The liver is a manufacturer
The liver produces a number of different substances which are vital in order for healthy growth and development. For example: 1. The liver cells produce substances which assist with the clotting of the blood, e.g.Prothrombin. 2. The liver cells make proteins which are carried in the blood. These have many functions. Albumin, for example, helps to control the distribution of fluid in all parts of the body and transports many substances to and from the liver and kidneys to other parts of the body.

The liver is a storage depot
The liver stores energy in the form of glycogen (sugar). It also stores a number of other substances, e.g. copper, iron and vitamins.

The liver is a controller
The liver plays an important part in controlling: 1. The correct level of many hormones within the body. 2. The blood sugar (glucose) levels. The liver stores glucose in the form of glycogen when there is too much glucose in the blood and releases it when the blood sugar level falls. 3. The amount of fluid the body retains and its distribution throughout the body. 4. The concentration of cholesterol which it converts into bile salts. 5. The action of many drugs

The liver is a filter
The liver removes many unwanted substances from the body. It plays an important role in controlling the harmful effects of some drugs and products of metabolism by changing them chemically before excreting them into the bile. Bilirubin (unconjugated or indirect) is produced when old red blood cells break down. The average life of a healthy red blood cell is 120 days. This bilirubin is carried in the blood stream, attached to albuimin, to the liver where it is altered into a different form (conjugated or direct bilirubin) which is then: 1. Excreted in the bile. 2. Transferred into the bile which passes via the bile ducts into the intestine and appears as brown pigment in the stool. Bile is continuously produced by the liver cells mainly by the action of bile salts. It collects in and passes out through the network of bile ducts into the intestine. Bile salts play an important role in the digestion of fats.

The liver is a defender
The liver plays an important role in fighting many types of infection. In particular, protects the body against infection which arises in the gut. With so many different and complex functions, it is not surprising that more than 100 types of liver diseases have been identified in babies and children

Harmonee's Story

My name is Harmonee Lynn Phillips. I was born October 8, 2009, I am 3 months old. When I was 2 months 1 week and 4 days old my doctor realized that something was wrong. Mommy asked the doctor why I was still yellow at my 2 month appointment on Dec. 18, 2009, we all thought I just had jaundice. But jaundice doesn’t last this long. Dr. Lloyd {my pediatric doctor} told mommy to take me to the hospital to get my blood checked. At the hospital mommy held me while my heel was pricked and the nurse took all the tubes of blood she needed for testing. After, mommy and I went home to wait for results. Layla {my big sister} and I were playing on the couch when mommy’s phone rang. I knew something was wrong when I saw mommy’s streak with tears and her voice catch as she talked into the phone. She knelt next to me and dialed my daddy’s number. She told me things were going to be okay, she would take care of me… she promised. I heard my daddy’s voice on the other end of the phone and I heard mommy repeat what the person on the phone had told her. "The results came for Harmonee's blood tests. Lloyd says it has something to do with her liver, but it’s not jaundice. He called Primary Children’s and someone from the GI clinic is going to call and set up an appointment." I didn’t want mommy to be sad, so I smiled at her to let her know I was okay, nothing was wrong. Mommy smiled back, and told me not to look so concerned. Layla gave mommy hugs and kisses, then gave me kisses after. Everything was okay. About an hour later mommy got another call. My appointment was set for Monday, Dec. 21, 2009.

The weekend was long and hard, wondering how serious and what was going to be said at my appointment Monday morning. Mommy woke me up early that morning, she gave me a warm bath, made me a bottle to eat, put me in my snow suit, buckled me into my car seat and put me in the car. I slept on the way to Primary Children’s and woke up in a waiting room, mommy was filling out papers, then she held me until my name was called. The nurse was nice; she smiled at me and talked to me, than she put warm jelly on my tummy after mommy took my clothes off. She put a thing on my tummy and spread the jelly around, I could see images on a computer above my head. I heard the nice nurse tell mommy it was pictures of all the organs and things inside my tummy. After about 20 minutes the nurse told mommy she was going to go get a doctor to 'read' the ultrasounds. He was a nice doctor, a heavier man with a beard, he talked to me too, said I was cute when I smiled at him. He looked through all the pictures, but stopped on a certain one, studied it and sat down. He took a deep breath and talked to the nurse for a minute before looking at mommy, he told her it was a picture was of my liver, the tissues were not all the same, they couldn't see a gal bladder, or any ducts but the GI doctor would explain more about what they had found. Mommy looked concerned, I started to wiggle and whine hoping mommy would be able to pick me up, hold me and let me tell her everything was okay. But she had to put me back in my car seat, and we went to the 2nd floor to the GI center to meet my next doctor. Mommy went to the lady at the desk, told her my name and signed me in, now mommy was able to hold me until my name was called. We went into a back room to wait for the doctor. Another nice doctor came to meet me, her name was Dr. Book. She asked mommy some thing’s about me and left again. We waited for 2 more hours. It was about 12:00 noon when Dr. Book came back in, she pulled a chair up to my mommy and showed her a picture. Dr. Book explained that they couldn’t see any of my main bile ducts or gal bladder; there were some tests that needed to be done before they knew exactly what was wrong with me. Because I was 2 months old and these types of things were suppose to be taken care of before I was 3 months, the tests were put on STAT. Dr. Book had made arrangements for me to have a liver biopsy that night at 4:00 P.M. I would stay the night in the hospital and she would come to my room the next morning to tell mommy the results of the biopsy. To prepare for the biopsy mommy had to take me to get more tests. We went to the ER first were the nurse put a catheter in me to get a clean catch urine specimen. Then I was taken to the Lab. Here they took more blood, and put an IV in my right hand. Then we were able to go to my room and wait for my biopsy.

Mommy and I arrived at room 4343, POD D, on the 4th floor of Primary Children’s at 3:00 P.M. Dec. 21. Mommy rocked with me in her arms until the time came that I had to go to general surgery for my biopsy. The nurses laid me in my crib and took me down the hall to the elevators that went to the 2nd floor with mommy next to me. She stayed with me as long as she could, until Dr. Book told her she had to leave. I wanted my mommy to stay. Before mommy left me she told me that she would be there with me, no matter what happened, she would see me when I woke up and she loved me... she promised. Mommy was there an hour later to meet me in my room, she was the first one I saw when I woke up, I wanted mommy to hold me. But they wouldn’t let her for a few more hours. When mommy was finally able to hold me, she held me all night, she slept in the rocking chair with me in her arms, and held me until the next morning when Dr. Book came in to tell mommy what they had found out through the biopsy. Little did we all know the next few minutes would change our lives… Dr. Book sat in front of my mom, she looked concerned, she had more pictures, putting her hand on mommy’s knee she told her the news… I was diagnosed with BILIARY ATRESIA Tuesday, Dec. 22, 2009. I was 2 months 2 weeks old.

Dr. Book, made surgery plans for the next day Dec. 23, 2009, Dr. Scaif would be my surgeon. I wasn't allowed to eat for at least 8 hours before I went into surgery. My last bit of formula was eaten at 3 a.m. that morning, and I drank my last few ounces of pedialyte at 8 a.m. My surgery was set up for 1:30 p.m. Daddy came to see me before my surgery, I was so happy to see him! At 1 p.m. mommy was told that general surgery was running behind, it would be at least two more hours. Finally, at 3 p.m. after hours of not having anything to eat, they took me back to the 2nd floor where Dr. Scaif would perform my Kasai. The surgery would last 4 and a half hours. I went into surgery at 4 p.m. and finally at 8:30 p.m. daddy and mommy were called to come to the PICU where they would have to, agian, wait to see me. At 9:30 p.m. my daddy and mommy were brought to me, room 14 in the PICU. I wasn't awake but I could feel my daddys hand. I had tubes in my nose and mouth, a catheter, a heart monitor on my foot, IV's in each of my hands and another one on my foot. After a few hours daddy had to go home, but mommy stayed at the hosptial that night in a 'parent stay' room. In the morning she came to sit with me, and talk to me. About 11:00 a.m. that morning I was taken to X-RAY on the 1st floor to receive my PICC line. Mommy wasn't able to stay, but she promised to meet me back at my room in the PICU when I was done. To put the PICC line in they had to sedate me. Today was Christmas Eve.

On Christmas day, I woke up to presents from Santa, who had left them on a chair in my room the night before. Mommy opened them for me when she came to my room that morning and showed me what each gift was. That day I was also lucky enough to get my mouth tube removed. I wasn't able to make any noise, but a scratchy whisper for a few more hours, but I was happy to have it out. Today I also left the PICU to my newest room, this time I would be on the 3rd floor, on the IMSU {Infant Medical Surgical Unit}, room 3311. This room was smaller, but mommy was able to stay in there with me on a small pull out bed. That day, Daddy brought Layla to see me, and to open Christmas presents. It turned out to be a great Christmas. On the Dec. 26, my CBC came back low, at 11 a.m. I was given my first blood transfusion. My nose tube was also taken out today and I was put on oxygen. That night my heart rate dropped to 50, and my respirations to 10. On Dec. 27, 2009, my heart rate dropped to below 10. The nurses called the cardiologist who came to my room and put a ‘jacket’ on me to help monitor my heart. But today, I was also allowed to eat 1ml of pedialite every 3 hours. What a tease. On Dec. 28, 2009 I was moved to room 3387, on the 3rd floor in the CSU {Children’s Surgical Unit} because my heart rate kept dropping to an almost stopping position. In this unit they would be able to watch my heart closer. Throughout the week my heart was monitored, on Dec. 29th, 2009 an Echogram was ordered, unfortunately, after packing my IV's up and taking me to the 1st floor to the Heart department, they were unable to pick up the image of my heart because of my bandages. I was slowly being taken off my morphine and my meds that I would have to take home were being added to my diet. On Dec. 30, 2009, Dr. Scaif gave permission for me to start eating 1 ounce of formula every three hours. I was doing good with that and on Dec. 31, 2009 I was allowed to finally eat as much as I wanted whenever I wanted. My tummy was also measured and it was 53 cm. That night, daddy and Layla came to see me again, we had a good New Years together. Daddy held me, and we watched movies in my room. It was good to see them again.

On January 1st 2010, Dr. Scaif told daddy {who was staying with me over the weekend while mommy went home to spend time with sister} that I could start preparing to go home. On the morning of January 2nd 2010, it didn't seem that I would be going home that day. My jaundice count was higher than the day before and my stool was lighter then what it was suppose to be. After hours of waiting Dr. Scaif still gave me the go ahead, everything was prepared for me to leave the hospital. Mommy and Layla came and helped daddy get all my stuff packed and loaded into the car. I was finally going home!