Liver for LuLu: Sayonara PCMC...til tomorrow that is.

Wednesday, May 19, 2010

Sayonara PCMC...til tomorrow that is.

New trick-head stands. Hate that it's on the hospital floor...but, what can I do? Whatever floats her boat at this point.

London's scans went well today (hooray!), no blockages. Blood flow, kidneys, spleen, LIVER all look good. Q. Book is chalking her liver inflammation to the Epstein-Barr virus. So they will let us leave today, with another medication (Valcyte) to control the EVB as well as lower her immunosuppressent drugs to help fight the virus. We will go back to the hospital early in the morning for more blood work and she'll be followed closely (as always).

Dr. Book is so amazing. She is always calm, cool and collected. She always knows what to do and how to do it and how to get it done quicker than anyone else could. I have such respect and love for her and her team. We couldn't have been placed in better hands than hers. It will be a sad day in Utah for liver families when that woman retires. I declare a Holiday called Dr. Book Day--I'll let y'all know when it is and what to do...Sometimes the only peace I can find in all of this and what is ahead is that she will be there to take care of us.

xoxo

lizzie

alpha-1, liver mum

Congrats Harmonee on being so brave and strong. I can't believe these tiny little bodies with huge spirits can endure so much. We are SO proud of her and can't wait to see the new life her new liver brings.

5 comments:

Megan said...: London, Liz, James, and Jonah,

I'm so glad to hear that London is not rejecting her liver! I've been praying hard for her. She is such a tough little girl. I tried to stop by and see you guys when you were here in the hospital but yesterday London was sleeping, and today you had already gone home! I'm glad it was such a short stay, sorry I missed seeing you all. You can always have someone at the hospital vocera me when you are here, I would love to come and say hi!

-Megan, PCMC child life specialist; May 19, 2010 at 2:54 PM
Vic, Linds, and the girls... said...: London is so darling!! I have been terrible at commenting, but still love reading her updates. I am so glad to see that everything went well with her checkup. You are in our prayers..Hope you guys are doing well!!; May 19, 2010 at 8:19 PM
Rogers Neighborhood said...: Yeah Lulu. You are amazing. Keep accepting your beautiful liver. Canyon does the same head stands. It's hilarious.; May 20, 2010 at 10:56 AM
Margaça said...: Great to see it is not rejection! Hope the EBV will be negative soon.

It is so dificult to know that our babie´s life will always be like this...

Love to you and your family!

Alice (Martim´s mum); May 21, 2010 at 2:34 PM
Annie said...: I've been out of town this week and have just caught up on your blog. Ugh. What a week for you and yours. I'm SO glad that things are well after all. And that you're home. Don't know how you do it all. Bless you. xoxo; May 23, 2010 at 4:22 PM

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Welcome to liverforlulu.blogspot

Meet beautiful, brave, tough-as-nails London (aka "LuLu"). She's been fighting Alpha 1 Anitrypsin Deficiency since birth. It's a genetic disease that causes liver disease and sometimes lung disease later in life. She received a new, clean, healthy, pink, perfect, beautiful liver January 15th 2010 thanks to the generocity of a family willing to donate their childs organs to give life to others. She is LuLu's Angel. We thank every person who takes the time to look at LuLu's blog. I am so happy that many now know what Alpha-1 is. I am grateful that some may change their veiws on organ donation because of London's life that she's been living with such dignity, strength and grace. Feel free to email me, elizabethbarnum(at)hotmail(dot)com with questions or comments.

What is Alpha-1?

Alpha-1 Antitrypsin Deficiency (Alpha-1) is a condition that is passed from parents to their children through their genes.This condition may result in serious lung and/or liver disease at various ages in life. For each trait a person inherits, there are usually two genes and one gene comes from each parent. People with Alpha-1 have received two defective alpha-1 antitrypsin genes. One defective gene came from their mother and one from their father.There are many types of defective alpha-1 antitrypsin genes. The most common abnormal genes are called S and Z. Normal genes are called M. A person who does not have Alpha-1 will have two M genes (MM). People identified with Alpha-1 most commonly have two Z genes (ZZ). Current evidence suggests that there are about 100,000 people with Alpha-1 (ZZ) in the United States. Another deficient gene combination is SZ, although people with this gene combination are less likely to get lung or liver problems than those with two Z genes. (lulu is ZZ)

Alpha-1 occurs when there is a lack of a protein in the blood called alpha-1 antitrypsin or AAT that is produced by the liver. The main function of AAT is to protect the lungs from inflammation caused by infection and inhaled irritants such as tobacco smoke. The low level of AAT in the blood occurs because the AAT is abnormal and cannot be released from the liver at the normal rate. This leads to a build up of abnormal AAT in the liver that can cause liver disease and a decrease of AAT in the blood that can lead to lung disease.

The most common signs and symptoms of disease caused by Alpha-1 are: Shortness of breath, wheezing, chronic cough and sputum (phlegm) production (chronic bronchitis) reacurring chest colds, eyes and skin turning yellow (jaundice), swelling of the abdomen (ascites) or legs, vomiting blood (from enlarged veins in the esophagus or stomach) decreased exercise tolerance, non-responsive asthma or year-round allergies, unexplained liver problems or elevated liver enzymes and bronchiectasis.

Alpha-1 has been identified in virtually all populations and ethnic groups. It is estimated that about 1 in every 2,500 Americans have Alpha-1. Individuals with Alpha-1 may remain healthy throughout their lives. Early diagnosis and avoidance of risk factors such as cigarette smoking, can help prevent Alpha-1 from causing disease. An estimated 20 million people have one normal and one defective AAT gene. People with one normal gene and one defective gene (for example MZ) are called “carriers”. (james and liz) Carriers may pass the defective gene on to their children. Alpha-1 can lead to lung destruction and is often misdiagnosed as asthma or smoking-related Chronic Obstructive Pulmonary Disease (COPD). Alpha-1 can’t be diagnosed by symptoms or by a medical examination; you need to get a blood test to know for sure. Alpha-1 is the most common known risk factor for emphysema and COPD. About 3% of all people diagnosed with COPD may have undetected Alpha-1. Alpha-1 can lead to liver disease. The most serious liver diseases are cirrhosis and liver cancer. The World Health Organization (WHO), American Thoracic Society (ATS), and the European Respiratory Society (ERS) recommend that all individuals with COPD be tested for Alpha-1.

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.

Liver for LuLu

Wednesday, May 19, 2010

Sayonara PCMC...til tomorrow that is.

5 comments:

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