Liver for LuLu: Happy 1st Alpha-versary

Friday, February 19, 2010

Happy 1st Alpha-versary

365 days ago I felt defeated, hopeless, unsure of my baby LuLu's future.

Tonight I am elated, grateful and overcome with happiness.

Thank-you Alpha-1 Antitrypsin Deficiency. You didn't kill us, so you must have made us stronger.

xoxo

Lizzie
Alpha 1, Liver MuM

10 comments:

Christensen's said...: Thanks for sharing your story in the Salt Lake Trubine. I am so glad to hear that LuLu is doing so well. We are also patients of Dr Books and it is reassuring to know that other are dealing with simular issues as us. Thanks agian for sharing your story and the best of luck to your family.; February 20, 2010 at 6:40 AM
Jen said...: Congratulations! I'm an Alpha-1 mom too. I have 2 daughters who have ZZ Alpha-1. Gracie is 7, and Meghan is 5. Thankfully, they are doing quite well with their Alpha-1 so far.

We also have a blog at http://alphagirls.blogspot.com

Feel free to stop by. ~ Jen; February 20, 2010 at 1:28 PM
Jeannie, Jane, Angel, Mommy, etc.. said...: Just popping in to see how little lulu was doing. I am so glad that you and your family has made it this far. I know that when we were a year out from Ember being diagnosed with Biliary Atresia, it was an emotional time.; February 21, 2010 at 8:31 PM
Margaça said...: One more thing in common about our babies: Martim was also diagnosed in February (17th)!

I remember that day, the same emotions that you describe, the same fears and uncertain about my baby´s future. The fear about my daughter sufering from the same disease.

But the past and the present made us better persons. Now we know what is really important in life!

Well done Lizzie, I knew you are a special mum! Lulu has a beautifull family.

Alice ( Martim´s mum); February 22, 2010 at 4:49 AM
ALL 4 DYETS said...: I haven't kept up like I should but I am so grateful for Lu Lu's success and what she has conquered!!! You are an amazing family! I feel so blessed to even have shared a small part in your life. My heart just cries full of joy and thankfulness every time i check on your family. Thank you, thank you for touching my life, and I know millions of others. If we could only be more like you this world would be absolutely AMAZING!!
Steph; February 22, 2010 at 9:09 PM
Janene said...: Hurray! It certainly did make you stronger. Way to go!!; February 24, 2010 at 10:39 PM
Megan said...: Liz, London, Jonah, James:
I am so glad to hear how well London is doing! It truly is amazing how strong you all have been throughout this whole process. I bet London is just so excited to be back at home where she belongs, playing with Jonah, sleeping in her own bed, making messes! It truly was a joy (understatment of the year) to get to work with you all during your stay at PCMC. I feel very grateful to have been a part of your lives, and I feel immense happiness for how well London is doing. Love to all!
-Megan, Child life specialist PCMC; February 26, 2010 at 10:04 AM
Anonymous said...: Oh that's so good to hear that LuLu got better in just one year...but bad she was one of those poor people that got the sickness! Xoxo; February 27, 2010 at 3:13 PM
heymaynard said...: http://www.facebook.com/event.php?eid=322768632792&ref=mf#!/photo.php?pid=30685022&o=all&op=1&view=all&subj=322768632792&aid=-1&id=1596014071&oid=322768632792&fbid=1286414652561

This is a Benifit concert for Ashley Maynard, LuLu's Angel, our angel! Come if you can!; February 28, 2010 at 9:46 PM
Steve and Sara said...: Love you guys!!!; March 2, 2010 at 5:16 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

Friday, February 19, 2010

Happy 1st Alpha-versary

10 comments:

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