The First Clinic Visit

Information Overload

Over the past week we mainly watched for head impacts and fevers, and went about our usual, day-to-day lives. Going into this appointment, we weren't sure what to expect. We knew they would be running a lot of tests to search for an explanation as to WHY his bone marrow shut down.  I was hoping for some kind of timeline or treatment plan.  

We checked in promptly at 2:00 to the hematology/oncology outpatient clinic.  They printed an ID band and strapped it on his ankle, which is not standard procedure for an office visit.  they got his stats: Height, weight, blood pressure, temp., and showed us to an exam room.  The nurse, Michelle, told us they planned to do a Complete Blood Count (CBC) lab, and then they likely other other labs after that.  To avoid having to poke him repeatedly, they place an IV on every visit.  then they can draw blood as needed, and at the ned of the visit, use the same poke to infuse with blood products, antibiotics, chemo treatments or other things that are considered "normal' in the is office.  Seeing the other patients, many of whom were bald, was a bit sobering.  I don't think that the reality that I have one of "those kids" has really sunk in yet.  

Pokes and Blood Draws

Andrew cried when the IV team arrived to put it in, but remained very still and didn't need to be restrained or held down.  (Had this been Mark, who's 4 yrs old, that would be another matter...) They drew 3 syringes of blood and then transferred those into individual tubes for each of the over 19 specific lab tests they ordered.  Over the course of the visit, they came back in and withdrew 2 additional syringes.  I kept thinking to myself, "If his blood counts are so low, he won't have any left at the rate they're going through it!"

Genetic Counselors

Andrew settled down and they brought him some milk with a straw and he snuggled into Daddy's lap while the first set of specialists came in to talk to us: the genetic counselors. These two women were experts.  They explained that there are 4 disorders commonly know to cause Blood Marrow Failure Syndrome.  Troy asked if/how that was different than aplastic anemia and they said that in this case they could be used interchangeably.  Many of the labs they ordered will screen/test to see if Andrew has any of them.  If so, they would also test his siblings for the same condition.  They went into a lot of detail about how and why they cause problems like this and ultimately said that the best treatment for this type of disease is a bone marrow transplant.

They reiterated that siblings are typically the most likely to be a donor match. to get a better picture of the family health histories, they asked us for everything we know about our children, selves, and parents and any health problems. This whole process took over an hour of conversation.  (I'm leaving out a lot of tedious details.) But I came away from this conversation with a better understanding of the "matching" process and bone marrow transplants in general.

The Doctors explain Lab Results

The next people to come in were the Doctors. One was the fellow we had met on the day of Andrew's biopsy, the other was new to us.  After introductions all around, the first thing they told us were his results from the Bone Marrow Biopsy, and the CBC from an hour before. there wasn't anthill different than their initial diagnosis, however, the tests that were sent to another lab showed something disheartening.  When they analyzed all the cells, they looked specifically for immature cells that would ultimately mature into platelets, red and white blood cells. There were essentially none of these found. the lab report stated this: "Markedly Hypocellular Marrow (5%) with decreased trilineage hematopoiesis." They essentially called his marrow "empty" because normal for his age is 60-80%. 

The doctors also explained his CBC results, especially in comparison to his results from 6 days prior. His platelets level after receiving a an infusion from a donor were 40.000.  They were back down to 8,000 in the office, so they recommended getting him another transfusion at the end of the visit.  This helps descries the chance of him having a serious bleeding incident, since platelets help clot your blood.  his red blood cells (hemoglobin) had gone up from 7% to 9.2%, which is still lower than normal, but not low enough to warrant another red blood cell transfusion. 

Zero Immune System

Lastly, and most concerning where his neutrophils (ANC), which are the white blood cells responsible for fighting off infections.  Normal is 1.5. Thursday his were 1.2- low, but acceptable. Today they were 0.1.  And because there are not any of these immature white cells in his marrow, they said there is very little chance of seeing this number rebound. This has serious implications in day-to-day life.  The doctors explained that no only does this mean he can't fight off sickness and illness-type germs, his body isn't even able to maintain the balance of "healthy" bacteria and fungi that reside constantly in our mouth, on our skin and in our GI tract.  They prescribed 2 antibiotics and one antifungal medication to take daily that should help keep these in check.  (side note: One of these is taken 2x/day, and Andrew HATES the taste.  I have attempted 4 doses so far, each using a different technique, and only once did he not instantly spew it all out of his mouth and all over Troy and I with an emphatic "Yuck-eeee." Any medication administration suggestions are greatly appreciated.) 

One of them said, to prepare us for the inevitable, "He will likely be admitted to the hospital with an infection in the next 1-2 months while we are waiting for results.  *deep sigh*

They went on to explain all the many lab tests they were ordering, many of which were to test for conditions, other than inherited or genetic, that could explain the cause of his Bone Marrow Failure. Some include exposure to certain viruses. They ordered the test to determine his bone marrow/blood type.  This will identify what we are trying to match a sibling to.  I was shocked when the doctors said that we wouldn't really have the results to many of these for 2-4 weeks. That seems so long to wait when you just want to get your kid healthy now!

Platelet Transfusion

They said Andrew was likely to need transfusions every week, and they wanted us to come in weekly for the time being. They answered our questions, looked Andrew over and told us he was the cutest patient they'd seen all day, and by now it was after 4pm. (Maybe they say that to every parent.)and lastly, sent us for Andrew's platelet transfusion.  The office has an entire room with curtained off "cubicles" for patients who need infusions of some kind. There are 10+ cubicles.  And you get to keep your 2 year old there attached to an IV pole for the drip time, plus another 30 minutes of observation time to watch for a delayed reaction.  Andrew behaved so well for these past 3 hours, especially considering that he only got an abbreviated nap before we came.  This platelet transfusion followed the same protocols as the other 2 he'd received.  starting vitals, and then every 15 minutes until it finished.  Because platelets are thicker or stickier, they administer them at a faster rate.  So I was pleased to hear that it would take about 30 minutes compared to over 2 hours for the red blood cells. We looked at pictures on Dad's phone, then on my phone, then Andrew asked to read a book. The nurse looked for a book and could only find a Mickey Mouse coloring book.  There was no real plot and it didn't keep his interest. I was surprised when the nurse announced that he was done; I looked up at the bag of platelets hanging on the pole and it was still almost half full! I asked why they didn't give him the whole bag, and they answered that the volume is based on his weight, and an entire bag would be too many.  I thought to myself, "You could probably give him 2 full bags and he'd still have a lower platelet count than I have." I'm a loyal blood donor and it made me ache to see those blood products going unused. One of my children later commiserated, and I wholeheartedly agreed, "they should just split the blood into smaller bags and let the big people get 2 bags."  

As the 30 minute wait time counted down, in an effort to streamline things, I decided to head down to the pharmacy and pick up these new medications.  The anti fungal medicine needed pre-approval from insurance, so I only got the two antibiotics. Although I had planned ahead and las lasagna in the oven with a time bake set for the big kids, I was still anxious to get back home. It was approaching 5:30 and this pregnant lady was hungry. Also, Tim and Spencer's Wednesday night activity started at 6:00 tonight instead of the usual 7:00, so our babysitters were abandoning us.  Luckily, Troy's parents were happy un over and sty with the younger kids, and drop the big boys off at the activity. 

The visit made for a very long day, left us with a lot to think about and yet, still didn't give any kind of idea of what the next step is. I think I will be learning a lesson on waiting.  Even if all the stars align, and one of the other kids is a marrow donor match, the very soonest we could hope to do the transplant is September.  That seems like a long time to keep Andrew healthy.  And it means Baby Sister will arrive before it happens.  Fortunately, I am learning to trust God's timing and know that ultimately things will happen as they should.  Even if I don't like waiting.