What is Aplastic Anemia?
(The above is an informational video that explains the basics of Aplastic Anemia & introduces you to a few of the people affected by it.)
So What is Aplastic Anemia?
Medical Definition: Aplastic anemia is a degenerative bone marrow disease. It is a rare and serious condition in which the bone marrow stops producing enough platelets, red and white blood cells to keep the body healthy, resulting in an increased risk of infection and uncontrollable bleeding.
Parents Definition: A condition which causes your child to become sleepy, clingy and irritable. An instant crash course in at-home nursing for parents. Also known to make others think you abuse your children due to excessive bruising and bad attitude.
Aplastic Anemia (AA) is one of those diseases you never hear of until you or your loved one is diagnosed with it. There are around 300~400 cases diagnosed every year in the United States, around 1 in a million. It is a very serious condition that doctors are not very knowledgable about due to the rarity. Only in the last 40 years have they begun treating it and only in the last 20 years have they had any true success, mostly due to the advances in bone marrow transplantation (BMT) and immunosuppresive therapy (IST).
50 years ago, AA was considered an untreatable disease with 100% mortality. Even today, without some form of treatment, life expectancy is ~2yrs post diagnosis. On a very rare occation, some patients will get better without cause or explanation.
The causes of AA are for the most part unknown. It has been linked to many various factors including viruses, chemical exposure, chemotherapy and many others. Most cases of AA that are diagnosed are called idiopathic, meaning the cause is unable to be determined.
Josef has Secondary Idiopathic Aplastic Anemia… We think the cause in his case might have been the Epstein Barr virus (EBV), Cytomegalovirus (CMV) or possible benzene exposure.
AN EXAMPLE OF Aplastic Anemia BLOOD COUNTS:
Josef’s CBC counts from Kaiser on 2-14-2008, 2:45 p.m. & from Children’s Hospital on 2-13-2008, 5:45 p.m. + Children’s Hospital NORMAL standard…
WBC 0.8(AA) 1.3(AA)[4.0-12.0]
ANC 400 411[1500-5500] (a person is considered neutropenic when they have an Absolute Neutrophil Count under 500. Multiply the neutrophil count by the WBC count to get this amount.)
RBC 2.58(L) 2.56(L) [4.0-5.3]
HGB 7.7(L) 7.7(L)[11.5-14.5] (normally a person is transfused when this reaches 8.0 or below, depending on the hospital’s standards.)
HCT 21.5(L) 21.6(L)[33-43]
MCV 83.3 84.3 [76-90]
platelets 26(AA) 16(AA) [140-440] (normally a person is transfused when this reaches 20 or below, depending on the hospital’s standards.)
Monos 12 15.1(H)[2-12]