Prayers for Arthur

We started yesterday the day in the hospital excited about going home later in the afternoon.  Lillian spent the night over at her friends' Gabe and Rose (she could not have been more excited about her "sleep-over").  Arthur spent some of the night in the nursery, so we got in a little needed sleep.

Before we were discharged, Arthur got a Complete Blood Count (CBC) test as per our request to check for neutropenia.  Gwen had a similar test just before her surgery by which we learned that she had no neutrophils due to antibodies from mom that were in her blood. Neutrophils are the white blood cell that attack bacteria and fungi.  Naturally, we wanted to screen for this condition this time around. We were saddened to learn yesterday morning that Arthur's test came back showing he had neutrophil count of 100 where 1,500 is normal.  This condition is called severe alloimmune neutrophilia.  It leaves him in danger of bacterial infections. However, as in Gwen's case, there are things we can do and over time it resolves on its own (at least, that is the expectation of the doctors).

A neutrophil

Our pediatrician consulted with the Hematology department at UVA Hospital, and set up an appointment for us at the outpatient clinic in the afternoon.  We packed and got ourselves discharged as quickly as possible and drove over the mountain to Charlottesville. It was a little rough covering such familiar grounds – even to the point of finding ourselves in the same waiting room that we were for Gwen's first Cardio clinic visit post-CHOP.  We met with one of the pediatric hematologists for about 2 hours talking about what the results of this test and making decisions about what we need to do now.

The most likely diagnosis is that he has Alloimmune neutrophilia. The good news is this is most likely something that will, as stated above, resolve on its on in two to six months.  Because of our family history, because Gwen's condition was confirmed via a bone marrow biopsy (easily done during her open-heart surgery), the doctors are able to feel very confident in this diagnosis. Also thanks to Gwen they don't feel the need to even explore the risk-benefit of having a bone marrow biopisy on Arthur - as it is the best way to diagnosis this condition for sure.

The condition has to do with antibodies from the mother that are created to attack the neutrophils as they leave the bone marrow in the baby (this is caused by an incompatibility between the mother and father and arise after a first pregnancy, which is why Lil did not have this condition).  Those antibodies are passed to the baby while in the womb, and stay present for about two months.  Arthur's bone marrow can make neutrophils, but they are getting destroyed because of these antibodies (medical jargon offered below).

We decided with the hematologist to give Arthur an injection of Granulocyte colony-stimulating factor, which should temporarily boost his neutrophil count.  Gwen was also given this (although we are not sure which one, there are two kinds...long story, more questions to get answers too). She was also given another drug we decided Arthur did not need.

We'll go back to UVA on Thursday and get another CBC to learn if this injection raised his levels.   From there we might need to keep injecting him with G-CSF every day or every other day for a few months, waiting until these misguided antibodies leave his system.

The doctor's said he is doing great.  There is no outward sign that he's is having a problem.  They summed it up with, "We are treating a number."

This has an impact on our day to day life.  If Arthur has a fever or any sign of an infection, even if we suspect it is only a viral infection we will need to take him to the doctor and have him put on IV antibiotics and his white cell count will have to be closely monitored (all of that meaning he'd need to be admitted).  Since it's not easy to tell if an infection is viral (which he should be able to fight off) or bacterial, we will need to take reasonable measures to lessen his exposure to germs.

While we were going to be extra careful before this diagnosis, now we are going a step above...sadly this means no public appearances for Arthur for a while :(. Lil can still go to preschool.  We aren't ordered to in a "bubble" or anything, but we will do all we can to keep him protected.  This has us also planning flu shots and Whooping Cough booster shots (which we were already planning and as I have promoted on Facebook.  All pregnant women and parents and caregivers of new babies should be doing this anyway). 

We don't know how many infants have alloimmune neutrophilia.  The only reason we know Arthur has it is that we asked for a CBC because of Gwen.  Since most infants with this condition have only a mild case they recover from this condition without intervention.  There's not a large body of research to go on.   Each child responds to the treatments differently, so this is a watch, wait and pray situation.

Thanks for praying for this little guy – we appreciate the care and support. We are trying to take this in stride... it's a bit of a fall from how things felt on Monday. It's not the homecoming we dreamed of, but at least we have this insight and are able to give him whatever help he needs!

We are settling in back home with our wonderful little baby. Lil is very sweet to him. So far he's a very sleepy, undemanding baby.

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Here's some fun medical jargon if you are interested: 


"Neonatal alloimmune neutropenia (NAN) is an uncommon disease of the newborn provoked by the maternal production of neutrophil-specific alloantibodies, whereby neutrophil IgG antibodies cross the placenta and induce the destruction of fetal neutrophils. Affected newborns are usually identified by the occurrence of bacterial infections. 

Neonatal alloimmune neutropenia (NAN) occurs when a mother becomes sensitized to a foreign antigen of paternal origin that is present on fetal granulocytes. These fetal granulocyte antigens sensitize the mother and provoke antibody production. Moreover, immunoglobulin G (IgG) antibody readily crosses the placenta and destroys fetal granulocytes by providing opsonic assistance to splenic macrophages. Neutropenia is typically self-limiting and last for several weeks, but can persist for as long as 6 months. During this period, neonates are at high risk of developing infections. In NAN, symptomatic infants often present with delayed separation of the umbilical cord, skin infections, otitis media, or pneumonia within the first 2 weeks of life, and whereas most infections are mild, severe sepsis is known to occur."  see source