Liver for LuLu: Ashley, every step of the way

This beautiful Angel was given to us by our friends Matt & Amberly. It is Ashley helping LuLu to walk. Amberly even put a big bow on the little LuLu's head and I painted Ashley's hair darker :). LuLu has taken several steps and she's getting better and better everyday. She crawls/hauls like a maniac...and we are loving it. Little miracle after little miracle each and every day.

A favorite hang out.

Bath time!!

We will be seeing Ashley's family this weekend in Vernal. There is a benefit for her family and other families in Vernal facing major medical expenses. I am excited to be with Ashley's family and go to her graveside.

PS THANK YOU TO WHOEVER PRINTED OUR BLOG OUT AND SENT IT TO US. WHAT A WONDERFUL GIFT TO HAVE RECEIVED UPON RETURNING FROM THE HOSPITAL. THERE WAS NO NAME OF WHO IT WAS FROM...SO IF YOU WANT TO STAY ANONYMOUS, THAT'S GREAT, BUT IF THAT WAS A MISTAKE AND COMPANY SHOULD HAVE SAID WHO IT WAS FROM....PLEASE LET ME KNOW. I'D LOVE TO THANK YOU PERSONALLY!!! MY LOVE TO ALL WHO READ LULU'S BLOG AND HAVE GIVEN US so much LOVE AND SUPPORT. THANK YOU FROM THE BOTTOM OF MY HEART AND THE BOTTOM OF LULU'S LIVER.

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.

13 comments:

A womb for rent said...: Ok I have chills!!! What a perfect gift!!!; March 2, 2010 at 6:59 PM
Jeannie, Jane, Angel, Mommy, etc.. said...: That is such a perfect gift. I am so happy that you have the opportunity to share your precious little girl with her donor family. I can only hope that she continues to do well.; March 2, 2010 at 7:04 PM
AshleyS said...: that figurine is darling, as are the pics of happy lulu in her bath!; March 2, 2010 at 7:22 PM
Anonymous said...: The perfect gift for the perfect...BABE!!!!!!; March 2, 2010 at 7:48 PM
Karen said...: Love the little figurine....so very sweet! Lulu looks awesome!Her scar is healing so very well...love the jail (gate)pic!; March 3, 2010 at 3:05 AM
Christensen's said...: I came across you blog and was really touched by Lulu's (and Ashley's) story. I'm so glad your baby girl is doing better and that you're blessed with miracles everyday!; March 3, 2010 at 3:02 PM
Heidi said...: I too found your blog and am so grateful I was able to read the story of Lulu and Ashley. What an amazing family you are! I am so glad that your Lulu is doing so well. And what a great gift for someone to give you.; March 4, 2010 at 12:29 PM
Jo Lynn said...: What a perfect sink for your cute little one to bathe in, how precious she is!!!! Yeah, that's great that you get to spend time with this amazing family, I'm sure the bond between yours and theirs is powerful! I love that little Lulu, congrats on walking and what a sweet gift with a cute bow, precious! So happy to hear all is well in your home!!!!; March 4, 2010 at 3:53 PM
Carlye Momma said...: So touching! I have been following your blog for a few weeks now and honestly can't put into words just how inspired and touched I am every time I pop in to read. Thank you so much for sharing all of your experiences with all of us. So glad your baby is thriving! It's just a beautiful thing. :-); March 5, 2010 at 10:30 AM
Gardner said...: I too am another person touched by your story. I can tell that Heavenly Father is looking out for you and your cute family. thanks for sharing your pure testimony of the gospel.; March 6, 2010 at 1:21 PM
The Barbers said...: I am so happy to hear London is doing so so well! I don't know if you remember me but I am a RN on Infant and took care of LuLu a couple times! I am so happy to hear she is doing so well. That is truly amazing. I came across your blog and had to leave a comment. We miss seeing London on IMSU, but glad we don't at the same time :)
Love,
Lindsi Barber; March 6, 2010 at 9:26 PM
shashank said...: Here is a link to more information about the genetics of Prothrombin Deficiency that was prepared by our genetic counselor and which has links to some useful resources for those dealing with this condition: http://www.accessdna.com/condition/Prothrombin_Deficiency/718. There is also a phone number listed if you need to speak to a genetic counselor by phone. I hope it helps. Thanks, AccessDNA; March 7, 2010 at 12:12 AM
Erica said...: Wow Lulu is looking great and what a sweet figurine to have. Angel Ashly is watching over your sweet babe every step of the way. I really can't wait to see our girls out and playing together.; March 7, 2010 at 8:18 PM

Liver for LuLu

Tuesday, March 2, 2010

Ashley, every step of the way

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