Hugs For Lex With Hereditary Spherocytosis

We are wanting to raise awareness to educate people about HS. It's very rare blood disorder. Please read Lex's story.

When My Daughter Alexis was 3 1/2 years old and we found out that she has this blood disease. My husband is a carrier of this genetic disease. He got it from his Mother who was born in South Korea. When Alexis was born she was a preemie and had a blood transfusion within the first week of life. she came home 2 1/2 weeks later. We always knew there was something wrong with her but not sure what. Because her skin and eyes were a yellow tint. Dave was the sameway. I finally went to a hematologist and we finally figured what was the problem. I have a step son and Baby boy were tested and DO NOT have it. Christians birth compared to his sister was very different. On January 6, 2014, Alexis went into the hematology center for a check up because we had a weekend in the ER. Her hemoglobin was low and she was taken into the transfusion center. That same day she had a ultrasound done and her gallbladder was enlarged. We went back a few days later to check numbers and they good because of her blood transfusion. We did more blood testing to see if her gallbladder was affecting other organs. Test came back that it was. So we needed to do surgery sooner then later, like we have planned. We were going to take the Spleen and gallbladder out at the sametime. On January 15, 2014, Lex had surgery to get her gallbladder out. She did awesome! Recovery was great and she's back to herself. Her appetite has improved and has gained a little bit of weight! In 2 Months we go back in for spleen surgery.

WHAT IS HS?- Hereditary spherocytosis hemolytic anemia is a rare blood disorder characterized by defects within red blood cells (intracorpuscular) that result in a shortened survival time for these cells. Red blood cells (erythrocytes) normally circulate for a few months and when they die off are replaced by new erythrocytes. However, in hereditary spherocytic hemolytic anemia, the cells die prematurely. They also have low amounts of fats (lipid) in the cell membranes and an abnormally small amount of surface area. The red blood cells are sphere-shaped (spherocytic) making it difficult for them to pass through the spleen, resulting in the early destruction of these cells (hemolysis). The sphere shape of the red blood cells is the hallmark of this disorder, and this abnormality may be identified under a microscope. Hereditary spherocytic hemolytic anemia is caused by an inherited metabolic defect.

The spleen can become enlarged due to the breakdown of the abnormally shaped red blood cells. The reason for the enlargement is for the destruction of the red blood cells. The gall bladder can also be affected due to the build up of the hemoglobin in the blood due to the destruction of the red blood cells in the spleen. If the gall bladder develops the hemolytic stones, the gall bladder will most likely be removed. Its necessary to remove the spleen as well. The removal of the spleen slows down the destruction of the red blood cells, and can help the bone marrow from being in overdrive to produce the red blood cells that are being destroyed so rapidly by the spleen.

Hereditary spherocytosis is passed down from parents to children. A parent with the disease has a 50% chance of having a child with the disease. It is more common in people whose ancestors come from northern Europe