Neupogen

On Friday morning, we went to see Dr. Grayson in Temple.  Morgan has been in a great mood this past week and has come a long way since our last visit to Temple.   She had a lot of fun playing in the exam room and looking at the animal pictures on the walls. 

Dr. Grayson explained that the Autoimmune Neutropenia is not caused by a lack of neutrophils being made in her bone marrow.  There is actually an antibody in her bloodstream that kills her neutrophils which is why her counts are so low.  Autoimmune Neutropenia occurs in 1/500,000 and there is no known cause.  Fortunately, he said she will grow out of this by the time she is school-aged and probably sooner!!! 

In the meantime, we will give her Neupogen injections three times a week.  The Neupogen causes her body to make more neutrophils to compensate.  On Fridays, she will get blood taken in town to monitor what her levels are doing.  Hopefully in a few weeks her white blood counts will be at a normal level.  Until then, she won’t be able to play with other kids and we just need to be really careful about where we take her.   We are so fortunate for the injections as her low levels can put her at a high risk for deep skin abscesses, pneumonia and mouth sores (which she already has had). 

The only negative side effect to the injections is that it can cause some bone pain and headaches.  She was irritable after her first injection but she has two incisors coming in, so it might have just been her teeth bothering her.  Pray that her levels normalize soon and that she doesn’t have any side effects.  Thank you so much!

We have a Diagnosis! :)

I was laying on my bed praying during Morgan's nap and unexpectedly, Morgan's hematologist called.  He said he already had the results of the antibody test.  He had some excitement in his voice when he said she is positive for the antibody.  That means that her bone marrow is creating antibodies that are attacking her neutrophils.  The reason her counts are so low is that she is making neutrophils but her body is killing them off before they can mature.   It is called Autoimmune Neutropenia or Chronic BENIGN Neutropenia.  The GREAT news is that she will grow out of this by the time she is school-aged.  In the meantime, Dr. Grayson said there are some injections we can give her to help her not to get sick so much.

This is such an answer to prayer that she does not have the more serious kinds that can lead to cancer.  We have SO much to be thankful for and God has been so faithful to us!  We are also overjoyed that we do not have to do the blood draws.  We go to Temple on Friday at 10:30 to find out about the injections and to find out more about the Autoimmune Neutropenia.  We are so relieved!!!  Thank you so much for your prayers.  This is a huge weight that has been lifted off our shoulders.  We can't thank you enough for your prayers, love and support!

Rough Morning

 I just found out her bloodwork still has not changed (0-70).  

  I'm so frustrated and upset.  I found old bloodwork that said that she had neutrofils levels at 2500 at nine months.  Also it said her levels were 700 just two weeks ago - april 12.  There was another set I found from several months ago that said her levels were 900.

Morgan's new blood draws have all shown so far that there has been no change in her neutrophil levels.  I just got a call them saying that yesterday's blood draw was 64.

I got copies of the old cbc's from upa and found out that there are at least three occasions that Morgan has had neutrophil levels over 500 in the past.  In August of last year (at 9 months) her counts were 2500, which is well in the normal range.  It also shows that her level was at 793 on April 12, 2012.  I told the hematologist this yesterday and he said "At this point we can probably  assume she doesn't have the congenital and that we are looking at AIN, cyclic or transient."  He said we just need to see what all the results show in the next few weeks.

I'm having a rough morning for some reason.  These low levels this week make me keep wondering if it is congenital. 

We find out at by the end of this week what the results of her antibody tests are.  A possible explanation for the low levels is that she has the Autoimmune type.  This is where the body kind of has an allergy to her neutrophils and are killing them.  This is actually not such a bad thing bc it always goes away by the time they are 5 years old at the latest.  Also, the injections can help in the meantime.  The antibody test might tell us this.  It has a high rate of false negatives.

Morgan has been on bactrim a lot in the past and I was on it a lot when I was nursing her.  This can also cause neutropenia.  I gave her two doses last weekend for the sore in her mouth because I was desperate.  I am wondering if that has anything to do with this.

Pray that I have a peace about this and that I can rest in what the doctor said.  We are so thankful that Morgan is in a great mood and eating well.

Neutropenia 101

I didn't find out a specific number for Morgan's neutrophil level on Friday but the nurse said the percentage indicated no change.  HOWEVER, I talked to Morgan's previous pediatrician yesterday afternoon.  It turns out that Morgan has had normal neutraphil levels in the past from previous bloodwork on at least 3 different occasions (over 500). She said it seemed to go up and down.  On Tuesday, the hematologist said that the Severe Congenital Neutropenia is when her levels from birth are not capable of going over 500.  So, he hasn't confirmed it, but from what he told us Tuesday, and everything I have read, it is pretty safe to assume that Morgan does not have the severe neutropenia since she has had normal levels in the past.  This is of course a HUGE answer to prayer and I definitely am breathing easier!

I would be remiss to tell you about this experience so far if I didn't tell you how God has embraced us as we wait on test results.  The morning after we came back from Temple, I felt like the wind had been knocked out of me.  I spent some time in the Word and God was faithful to show me that Morgan will make it through this ok, that he has big plans for her and that good things will come of this!  He didn't need to tell me that but He has been so faithful to comfort me through this process.

Please continue to keep us in your prayers.  Pray that Morgan does not get sick during the next four weeks of blood draws, bc if her fever goes over 101.0, she has to go to the hospital in Temple.  Pray that God comforts Morgan during the blood draws which are going to be in her arm, instead of doing the finger prick.  Also, keep Jeremy and I in your prayers as we need to be strong for her and trust in Him.  Thank you so much for lifting us up!

Here is a summary of the different kinds of neutropenia by most common first:

1.  Transient Neutropenia - Not Inherited. The neutrophil levels stay low for a certain amount of time and can be treated with injections before it goes away on its own.

2.  Autoimmune Neutropenia or Benign Chronic Neutropenia - Not Inherited.  This is where the blood's antibodies are actually attacking each other.  The neutrophil levels stay low for a certain amount of time and can be treated with injections before it goes away on its own, usually before age 3-5.

3.  Cyclic Neutropenia - Happens in 1-2/million.  Inherited.  My understanding is that she has it all her life but it tapers significantly at puberty.  This is where every 21 days she will have a 3-7 day drop in her neutrophil level where she will be susceptible to infection.  The injections make the amplitude of the sin wave higher and the waves shorter and more frequent.  With the injections, she would still have lows but she would recover faster than if she didn't have injections.  This is the one think she has based on her symptoms and the fact that my mom and I were in the hospital a lot as kids. http://www.medicine.wisc.edu/~williams/cyclicneutropenia.pdf

4.  Severe Congenital Neutropenia - Inherited. 1/million. Neutrophils don't go over 500 from birth.  We don't need to worry about this one.  :)

Blood Draw 1

Today is Morgan's first time to draw blood after the initial testing.  Basically, she will have her finger pricked today and then every Monday, Wednesday and Friday for the next 4 weeks.  We go at 2:30 today and Grammy and Pop Pop are coming along.  It probably won't be much higher than Tuesday because it hasn't been long enough.  However, if it jumps over 500, it automatically eliminates the most severe kind.  So, we are prepared for it to stay the same since it hasn't been long but God told me to be brazen in my prayers so we are praying for it to be over 500.  We will get the result back this evening or tomorrow.  If the level stays low, we still have to just hurry up and wait.  

Neutropenia

Basically, to make a very long story short....Morgan probably has a rare blood disorder called neutropenia.  There are different levels within your white blood count and the neutrofils are the ones that are the main defense against infection.  The test with Dr. Flippen showed Morgan had none.  There is no easy way to know if the 0 level was the bottom of a one week long cycle as in Cyclic N., or is just temporarily 0 with a couple other Neutropenia types OR permanently 0 as with Severe Congenital Neutropenia.  There are about 5 kinds or neutropenia. 

Best Case Scenario is that Morgan has one of the 4 temporary kinds that will just last a few years.  We would just have to be extremely careful about her catching things and she would have daily shots for a few years.  Our lives probably would not be all that different than they are now since we haven't been doign many playdates anyway.

Worst Case Scenario is that it is the Severe type which puts her at a very high risk of infections.  This is called Kostmann's Syndrome and it used to be that most did not live past their teenage years with it.  However, with dailyinjections (Gcs-f) most people live manageable lives as the shots help to raise the bodies neutrofil levels again.  I'm assuming its just finding the correct balance with the injections because its very variable on how much each person requires.  The bad news is that he said 25% of the people that get this type of Neutropenia, get AML ( Leukemia) from ages 5-15.  I read somewhere else online that the odds are 9%.

Basically, they took a lot of blood today and we are starting the process of ruling things out.  At the longest it will take 4 or 5 weeks to know exactly what she has.  If she gets an underarm fever of 101 or higher, we are to go directly to Temple and admit her.  Her dr. recommended going there instead of st. joes bc of the drama going on there with the med.  If she gets admitted in the next month, they will go ahead and take some bone marrow by putting her under and taking it out of her hip.  At that point, we'll know within 24 hours which kind she has.  We might still have to do the bone marrow test in 4 weeks to confirm other blood work.  He said that the recovery for that is nothing for little ones and that they bounce back really fast.  Of course they would put her under general anesthesia.

So, even if she has the worst kind, she can still live a manageable life as long as she does the shots daily.  Basically, with these shots, they build up her neutrophil levels so she can fight infection but we would just need to be vigilent if her fever gets over a certain level.  Its hard bc this disease is SO rare but from what I can tell, there are still people that go to college and do normal things, they just have to be careful when they do get sick.  In true Karin fashion, my first question for him was if she has Leukemia.  He said that with how the rest of her levels are, that the chance she has leukemia now would have to be very slight so that is obviously good.

For the next 4 weeks, we need to get the finger prick blood test 3 times a week to look at her levels over a scope of time.  This can be down here in town.  If her Neutrophil levels at any point get over a certain count,   it will automatically rule out the worst kind.  If not, it doesn't mean she has the worst kind, but we just need to wait and see for other tests.  In about a week we will get some results back showing if it is Autoimmune N.  This is a temporary kind also where the bone marrow is making antibodies that acutally attack the neutrofil.  This would be fixed with the GCS-F shots.  I told him she had been on bactrim for the mrsa infections and he said bactrim can at times cause another temporary form called Transient Neutropenia.  I then told him that I was on it a lot when I was breastfeeding her.  She hasn't been on the bactrim though in several weeks and he wasn't sure how much gets through breastmilk.  

Here is something I found online that kind of describes things: http://www.scner.de/handbooks/handbook_en.pdf

I have not handled this super well today but as I write this email I realize that Morgan more than likely will live a normal life.    As you can tell, I just want to know which she has so we can begin to manage it.  Please pray we find out something very soon and that I keep a positive attitude - that I won't be discouraged if her levels aren't rising like I want, in the timeframe I want.  Pray I stay focused on Him and how to best make Morgan as happy as possible during this time of bloodwork and tests.  Fortunately, her tongue scab came off yesterday and she started eating some this morning. Please pray that we handle this waiting game well.