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
Tuesday, December 27, 2011
Merry Christmas!
I am sure you can imagine Christmas here. I tried to push any thoughts of Neutropenia (as much as I could) out of my mind and think about how amazingly blessed we are to be the parents of this little girl. Our little family celebrated the birth of our savior and we played, and played, and played with all of Paxton's new toys.
Paxton in her new ball pit! Look at all that hair everywhere!
She loves her new sandbox! Of course, Paxton was spoiled this doesn't even capture the tip of the iceberg of toys she got. :) Hope you all had an amazing Christmas.
Tuesday, December 20, 2011
Roller Coaster
Roller Coaster of emotions. That is me. Today we had the 2nd of our 12 tests over the 6 weeks. They pricked Pax's finger and this time she did say hurt. So that sucked. But at least it still isn't anything like taking it from her arm. I gave her a "poke" present (a fairy princess wand). She wasn't too interested :) While at the lab, they mentioned that Texas Children's requested an additional test to be run, they called it a tic count. I asked what that meant and they said it measures red blood cells being made (or something). So of course that makes me panic. Why are they doing that? I thought her red blood cells were fine? Maybe something has changed, they only gave me her ANC last time, are the other counts off too now? AGGGHHHHHHHHHH!
I waited until 3:30 and had not received a phone call from the nurse telling me the results so I called. I kept my phone by my side and somehow I still missed the darn call! UGGH! So she left a message saying the ANC is now 126. Yes, that is up. NO, it is still not even close to being good. I keep hoping that this is some sort of weird "fluke" and the ANC is going to jump up to 1500. Well we are on our 5th test in 15 days and the highest is 126. Not good.
I waited until 3:30 and had not received a phone call from the nurse telling me the results so I called. I kept my phone by my side and somehow I still missed the darn call! UGGH! So she left a message saying the ANC is now 126. Yes, that is up. NO, it is still not even close to being good. I keep hoping that this is some sort of weird "fluke" and the ANC is going to jump up to 1500. Well we are on our 5th test in 15 days and the highest is 126. Not good.
Anyway, I call back and leave a message b/c I still want to know what this "tic count" is and what they are checking for. I get another call and the nurse (not quite as good at comforting me as the Dr. and dumbing things down) just said that is a reticulocyte count that is part of the CBC (complete blood count). I don't know. I am confused. I ask her what the total counts are (the WBC, platelets and RBC) and she says they are all within normal range. I bust out crying and say thanks and that's that.
So after Pax went down tonight, I just started crying, and praying, and crying and praying. The kind of praying where you fall to your knees and look up to the sky and beg for God to help. An emotional roller coaster.
Monday, December 19, 2011
Today...
Today I am in a funk. I think about the people who I would typically talk to often and haven't lately. Maybe they are tired of hearing me be depressed? Maybe they don't know what to say (which I do understand). It makes me look back and realize when someone I knew was going through something bad, all the things I said may not have been "the right things to say." The only right thing anyone could say to me right now is that she will be okay (and mean it). I think some of them just do not understand how scary this really is. They do not know the words that come up when you do a search for neutropenia. They do not understand that the words you see are a parent's worst fear. I wouldn't have ever known either.
It just really pisses me off that everyone goes about living their normal lives while we feel like ours is spinning out of control. Not that I expect them to do anything else. This is just me going through my emotions. I am praying constantly for God to fill me with nothing but love, hope and understanding. I actually feel guilty for the thoughts I have been feeling. I guess if I really think about it though I am full of love for my sweet family and full of hope that everything will be okay.
It just really pisses me off that everyone goes about living their normal lives while we feel like ours is spinning out of control. Not that I expect them to do anything else. This is just me going through my emotions. I am praying constantly for God to fill me with nothing but love, hope and understanding. I actually feel guilty for the thoughts I have been feeling. I guess if I really think about it though I am full of love for my sweet family and full of hope that everything will be okay.
Wednesday, December 14, 2011
Texas Children's Hospital
Tuesday, December 13th was our appointment at Texas Children's in Houston with our Hematologist/ Oncologist, Dr. Mona Shah. The drive was nerve wrenching. The closer we got, the tears started flowing. The place is amazing for children. However, you could just see the sadness in all the parents eyes. There were volunteers there handing out toys in the waiting area. When we sat down they immediately came up to Pax and asked her if she picked out a toy. I just kept thinking, I cannot believe we are here and my baby is one of the patients. I am grateful for the way they treat the children, I just want every child to be healthy and never have to go through anything like this. I am not sure if I am making sense or not, but if you have ever had to take your child to a cancer treatment center, you understand what I am saying/feeling.
When we met Dr. Shah, the first thing she said was that she was seeing us as a Hematologist and not an Oncologist. She does not think Paxton has cancer. I need them to keep telling me this over and over because I keep seeing these horrible diagnoses. She said that 90% of the appointment was to see how Paxton interacted with her and us. They took more blood from Paxton but this time the pricked her finger. She didn't even flinch!! She sat there and watched them squeeze every drop of blood they needed out while eating goldfish with her other hand. I can not even stress the difference between that and them taking it out of her arm and 3 people having to hold her down. Paxton's neutrophil count came back as 0 (zero). I will post more info on all of this later but anything less than 500 is severe, 1500 is normal.
So we went through several questions and I probably left more confused. It was good to hear her rule out some of the things I had researched. I asked her again the chances of this being cancer and she said, "less than 1%. If she thought Paxton had cancer she would immediately be admitting us." We were given a prescription for lab work 2x a week over the next 6 weeks and we are to keep a journal of how she feels/her temps. From my research, this is a pretty standard place to start. They are looking to see if there is any type of cycle your body has on producing neutrophils. From what I understand, if they do not get answers this way, a bone marrow biopsy will be next.
If Paxton gets a temp of 101 or higher we still have to go to the hospital. They will do a CBC and a blood culture and then will decide if she needs an antibiotic shot or if we should be admitted and she should be hooked up to an antibiotic IV. We will be living a pretty sheltered life for now. We will not be able to attend church, go to play areas, or visit family for Christmas. We have to stay away from anyone who may be sick and just be very cautious of everything we do. I pray that it will only be for now and that one day we will back to a more normal life.
We left the hospital with feelings of sorrow for the parents that do have to make that long walk to get admitted because they are told their child does have cancer. We are still unbelievably scared and worried but grateful at the same time. Miss Paxton left the hospital with a hot pink band-aid and 3 new toys.
When we met Dr. Shah, the first thing she said was that she was seeing us as a Hematologist and not an Oncologist. She does not think Paxton has cancer. I need them to keep telling me this over and over because I keep seeing these horrible diagnoses. She said that 90% of the appointment was to see how Paxton interacted with her and us. They took more blood from Paxton but this time the pricked her finger. She didn't even flinch!! She sat there and watched them squeeze every drop of blood they needed out while eating goldfish with her other hand. I can not even stress the difference between that and them taking it out of her arm and 3 people having to hold her down. Paxton's neutrophil count came back as 0 (zero). I will post more info on all of this later but anything less than 500 is severe, 1500 is normal.
So we went through several questions and I probably left more confused. It was good to hear her rule out some of the things I had researched. I asked her again the chances of this being cancer and she said, "less than 1%. If she thought Paxton had cancer she would immediately be admitting us." We were given a prescription for lab work 2x a week over the next 6 weeks and we are to keep a journal of how she feels/her temps. From my research, this is a pretty standard place to start. They are looking to see if there is any type of cycle your body has on producing neutrophils. From what I understand, if they do not get answers this way, a bone marrow biopsy will be next.
If Paxton gets a temp of 101 or higher we still have to go to the hospital. They will do a CBC and a blood culture and then will decide if she needs an antibiotic shot or if we should be admitted and she should be hooked up to an antibiotic IV. We will be living a pretty sheltered life for now. We will not be able to attend church, go to play areas, or visit family for Christmas. We have to stay away from anyone who may be sick and just be very cautious of everything we do. I pray that it will only be for now and that one day we will back to a more normal life.
We left the hospital with feelings of sorrow for the parents that do have to make that long walk to get admitted because they are told their child does have cancer. We are still unbelievably scared and worried but grateful at the same time. Miss Paxton left the hospital with a hot pink band-aid and 3 new toys.
Sunday, December 11, 2011
We have an appointment!
Pax woke up today (Monday, Dec. 11th) with a cough. In my old world that would not be a big deal. In our new world, I really do not know what to do. So, I call the pediatrician and we are told to come in. She has no fever, her lungs sound okay and everything looks okay so nothing big.
We got in touch with Texas Children's and we have an appointment tomorrow! I am scared but sop thankful that we got in so quickly. We may have phone stalked them today. I called twice, the pediatrician called, and Jud called but who cares, we are in.
We got in touch with Texas Children's and we have an appointment tomorrow! I am scared but sop thankful that we got in so quickly. We may have phone stalked them today. I called twice, the pediatrician called, and Jud called but who cares, we are in.
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