So we went to Texas Children's Hospital again on January 24th to see the Hematologist. Paxton's neutrophil counts pretty much stayed in the severe range during the 6 weeks of testing for cyclic neutropenia. Most cyclic individuals are low for a short period (5-7 days) and then their counts go back to normal during the remaining 3 weeks of the month. There are some rare cases where someone with cyclic neutropenia stays severe but that is hard to identify as cyclic. So what I am basically telling you is that they do not think Paxton has Cyclic Neutropenia.
So that means more tests. So at the last visit they tested for the anti-neutrophil antibody, and two genetic tests (HAX1 and ELA2). The anti-neutrophil antibody test is to check for just that. In some people, a severe virus can cause their immune system to go haywire and their body can produce an antibody that will attack neutrophils so they cannot completely mature. This is Autoimmune Neutropenia. In most cases, children outgrow this by the age of 5. The problem with this test is there are many false negatives. So if you have a positive it is positive, but if you have a negative, it may be inaccurate and may really be positive.
The genetic tests are checking for mutations on the HAX1 and ELA2 genes. This is checking for congenital forms of neutropenia. I honestly do not know exactly what forms of neutropenia these are linked to because I am scared to death to research it. I know one form is Kostmann's. These will be more severe forms that also have much higher risks associated with them. These are forms that do not go into remission. Hopefully, PLEASE GOD, these come back normal.
So today has been two weeks since the last appointment. We were told about 3 weeks for results. Believe me, I have been checking to see if there are any results back yet. Our next appointment will be in 2 more weeks (Feb 21st). So our new routine if Pax gets a fever of 101, or higher, will be to go the hospital and they will do a blood count and a culture. She will get an antibiotic IV and will be admitted for a minimum of 48 hours to wait and make sure the culture is clear of any bacterial infections.
So as always I beg that you pray for us, and please spread our prayer requests far and wide. I will also try to start posting more happy thoughts! I know you all want to see pictures of our precious Paxton!
Love and God Bless,
K
Wednesday, February 8, 2012
Thursday, January 19, 2012
Types of Neutropenia
Honestly, I have been dreading this post. I am just so scared about Pax that I don't even want to talk about the thought of what her diagnosis is. Just putting everything in words is hard. Her counts have pretty much stayed severe. One time her ANC got to 560 but it has since dropped and stayed below 500.
So all that being said I am taking the easy way out and I am posting directly from The National Neutropenia Network (NNN) website. All credit goes to NNN: types of neutropenia.
Congenital Neutropenia
Sometimes called Kostmann Syndrome this type of Neutropenia is generally present at birth. It is usually very severe since neutrophils are often completely absent. Patients usually show what is known as a maturation arrest in the early stages of neutrophil development in the bone marrow. This means that their neutrophils rarely fully mature into the cells that are capable of fighting infections. This type is known to often benefit from G-CSF treatments.
Idiopathic Neutropenia
This type of Neutropenia may occur at any time in life for unknown reasons. The onset of idiopathic Neutropenia is possible in both, children and adults. Most patients respond well to G-CSF treatment but require long-term treatment.
Cyclic Neutropenia
Cyclic Neutropenia is another inherited type of Neutropenia. This type shows a cyclic pattern of varying neutrophil counts with a typical cycle length of 21 days. These cycles vary from patient to patient with some individuals being neutropenic during the whole cycle and others who have low neutrophil counts for only a few days and normal blood counts during the rest of the. The frequency of bacterial infections depends on the length of the neutropenic period that the patient experiences. Those who have a longer neutropenic period within the cycle suffer more frequently from infections.
Cyclic Neutropenia occurs because of fluctuating rates of cell production by the bone marrow stem cells. Other blood cells, such as platelets or red cells can also show oscillations with a cyclical pattern. Cyclic Neutropenia can occur sporadically, but there are families in which cyclic Neutropenia is inherited with one parent and more than one child affected. As in Kostmann syndrome, patients with cyclic Neutropenia also benefit from G-CSF treatment.
Autoimmune Neutropenia
This type involves antibodies in the blood that actually attack the body’s own neutrophils. This is the most common cause for Neutropenia of this age group. Although these infants lack peripheral blood neutrophils, they usually do not suffer from severe bacterial infections.
Granulocyte-specific antibodies are detectable by different immunological blood tests that can be performed in specialized laboratories. If these antibodies are identified, the patients should be kept under medical care, but may not necessarily require treatment with antibiotics or G-CSF.
Depending on the frequency of infections and the neutrophil counts, prophylaxis with an oral antibiotic may be considered by the treating physician. In some children where severe infections occur, treatment with G-CSF is indicated. In most children the blood counts normalise during the first 2-3 years.
Autoimmune Neutropenia is occasionally seen in young people (20 – 40 year age group) predominantly women and in this setting is often associated with other disorders or conditions.
If you have questions I can try to answer them. Tuesday is our last of the 12 tests. Wednesday, the 25th is our 2nd appointment at Texas Children's with our Hematologist/Oncologist. Please add us to all the prayer requests you come across. We need all the prayers we can get.
Love and God Bless,
K
So all that being said I am taking the easy way out and I am posting directly from The National Neutropenia Network (NNN) website. All credit goes to NNN: types of neutropenia.
Congenital Neutropenia
Sometimes called Kostmann Syndrome this type of Neutropenia is generally present at birth. It is usually very severe since neutrophils are often completely absent. Patients usually show what is known as a maturation arrest in the early stages of neutrophil development in the bone marrow. This means that their neutrophils rarely fully mature into the cells that are capable of fighting infections. This type is known to often benefit from G-CSF treatments.
Idiopathic Neutropenia
This type of Neutropenia may occur at any time in life for unknown reasons. The onset of idiopathic Neutropenia is possible in both, children and adults. Most patients respond well to G-CSF treatment but require long-term treatment.
Cyclic Neutropenia
Cyclic Neutropenia is another inherited type of Neutropenia. This type shows a cyclic pattern of varying neutrophil counts with a typical cycle length of 21 days. These cycles vary from patient to patient with some individuals being neutropenic during the whole cycle and others who have low neutrophil counts for only a few days and normal blood counts during the rest of the. The frequency of bacterial infections depends on the length of the neutropenic period that the patient experiences. Those who have a longer neutropenic period within the cycle suffer more frequently from infections.
Cyclic Neutropenia occurs because of fluctuating rates of cell production by the bone marrow stem cells. Other blood cells, such as platelets or red cells can also show oscillations with a cyclical pattern. Cyclic Neutropenia can occur sporadically, but there are families in which cyclic Neutropenia is inherited with one parent and more than one child affected. As in Kostmann syndrome, patients with cyclic Neutropenia also benefit from G-CSF treatment.
Autoimmune Neutropenia
This type involves antibodies in the blood that actually attack the body’s own neutrophils. This is the most common cause for Neutropenia of this age group. Although these infants lack peripheral blood neutrophils, they usually do not suffer from severe bacterial infections.
Granulocyte-specific antibodies are detectable by different immunological blood tests that can be performed in specialized laboratories. If these antibodies are identified, the patients should be kept under medical care, but may not necessarily require treatment with antibiotics or G-CSF.
Depending on the frequency of infections and the neutrophil counts, prophylaxis with an oral antibiotic may be considered by the treating physician. In some children where severe infections occur, treatment with G-CSF is indicated. In most children the blood counts normalise during the first 2-3 years.
Autoimmune Neutropenia is occasionally seen in young people (20 – 40 year age group) predominantly women and in this setting is often associated with other disorders or conditions.
If you have questions I can try to answer them. Tuesday is our last of the 12 tests. Wednesday, the 25th is our 2nd appointment at Texas Children's with our Hematologist/Oncologist. Please add us to all the prayer requests you come across. We need all the prayers we can get.
Love and God Bless,
K
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