Liver for LuLu: Day16 New Diagnosis (posted by Lizzie)

Sunday, November 8, 2009

Day16 New Diagnosis (posted by Lizzie)

No Butt Syndrome (NBS) Little toad is so teeny, tiny that she officially LOST her booty days ago. It was the last thing to leave after her legs and arms shrunk to twig like proportions. Yes, it's always been little, but she had one-it was really cute and hard not to pinch if you ever saw it. If anyone finds it please let us know. Meanwhile we will look to medical professionals to help her create a new one via the IV nutrition therapy that she's been receiving 12-18 hours a day and will continue to do so until her transplant.

We are hoping to get our little London home in the next few days. Our house isn't a home without her and Jones misses her dearly. I've had the "opportunity" (that's what it feels like) to be mom to Jonah today while James is with sweet LuLu at the hospital. Thank you all for your continued support. We've really felt so loved by so many.

6 comments:

Sarah said...: Lizze & James-- we feel like such heels having just learned not only of this blog, but of LuLu's situation. She is such a precious little girl, and we are praying for a successful transplant and recovery. We hope you get to have her back in your home soon! --as I'm sure it just aches to have your family apart like this.
And I don't know... but that lil' butt is still one of the cutest I've seen!
--Mo and Sarah; November 8, 2009 at 8:27 PM
nickandhalliepeay said...: Love the self Diagnosis...NBS...I can't wait to see LuLu so I can give her a little love pinch on her tiny little bum. I have faith that she will get that "bigger"(still very tiny) booty back soon. I love you LuLu!
Love,
Auntie Hallie; November 8, 2009 at 8:31 PM
Lexi Ray said...: James,

I am so so sorry to hear what you and your family are going thru. Jackson just told me and asked me to keep you guys in my prayers. My heart just aches for your wife and you and your little angel baby. Your little family will be in my constant prayers, And I will pray that you feel great comfort and peace and that your family will feel the lords arms wrapped around you. I will also be spreading the word, you can never have enough prayers.
Much Love to your little family
Alexis Ray (Theodore); November 9, 2009 at 2:36 PM
Cydnee said...: Liz, James is not the only funny one in your little family!

Sending lots of prayers and love....

Aunt Cyd, Uncle Bjorn and cousins; November 10, 2009 at 12:57 AM
Mama Williams said...: Hi Liz,
Cyd and I talked this weekend about your sweet little Lu Lu and she linked me this blog. We love your family. Hang in there Mama! You're doing great! Hugs and Prayers to you.
Julie Williams; November 10, 2009 at 6:26 AM
Jo Lynn said...: I love babies bottoms, they are so stinkin cute and I'm sad she's lost her's, but I know it will come back very very soon. She is such a strong little girl and she is always in our prayers. This breaks my heart, I have a little girl of my own and she's 21 months and it hurts to see her hurt...I can't imagine what your family is going through. Hang in there, He's watching over your little angel and your family and He hears our prayers!
Jo Lynn; November 10, 2009 at 9:55 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

Sunday, November 8, 2009

Day16 New Diagnosis (posted by Lizzie)

6 comments:

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