The Ups and Downs while we “Wait and See”

Brad captured this great picture of Ryan at Lake Sawyer yesterday.

The last two weeks have been a series of ups and downs.  Twice, Ryan’s platelets registered over 40,000!  This was so exciting!!!  Until… his numbers took a nose dive to 14,000 at each of the appointments that followed.  However, we did get to skip platelet transfusions at each of the appointments where it was at 40,000. This means he may be trending toward platelets once a week instead of twice a week.

Last Thursday was the last 40,000 count, and Ryan got to skip a platelet transfusion (although he did get another red blood transfusion).  I expected at today’s appointment that his counts would follow the same trend as the last two weeks and be at 14,000.  Unfortunately, they were at 12,000.  I know it is only a difference of 2,000 but any little thing that feels like a setback is increasing my anxiety level these days.  The count is also only 4,000 higher than when he was initially diagnosed at 8,000 at the end of June.

I am starting to get impatient and I wish we were seeing better progress.  I know that this treatment can be very slow to “kick in”.  We really have to wait until about three months post ATG treatment to get an idea of whether it is working to put him into remission.  (Three months will be mid October for those who are counting.  Just in time for Ryan’s 5^th birthday.)

Note: I have included graphs showing the trend of Ryan’s labs below.  I will try to include updated graphs about once a month.

It is so frustrating not being able to do anything but be patient and “wait and see”.  It is in my nature to want to hurry up and find something to fix this, but no matter how many hours I spend reading, researching online, quizzing the doctors, and analyzing the medical histories of other AA patients with blogs, I have to resign myself to the fact that I am probably not going to be the one who stumbles upon the cure for this dreadful disease while searching the internet or watching reruns of House.  I truly just have to be patient and take things a few days at a time.

We were finally able to get Ryan out of the house this weekend for a short hike.  He has been too fatigued this summer to do much, other than be a couch potato, do art projects, or have a few brief visits to school.  His doctor has decided to increase his threshold for red blood transfusions so that he doesn't get as exhausted and have other side effects in between transfusions (anger, loss of appetite, stomach pain, and mental changes).  This has really helped and, for the most part, he was full of energy this weekend.

We did a short hike down to Lake Sawyer and then walked the shoreline for about an hour or so before Ryan announced that he was tired and wanted to go back.  On the way back, as Murphy’s Law would have it, Ryan tripped and fell hard on his left knee.  He started crying and blood started trickling down his knee.  Within a few minutes, a large purple “goose egg” formed.  Luckily, the blood was from some surface scratches.  I could see it was watery from low platelets, but at least it wasn't gushing.  Ryan was a trooper and limped back to the car, where I cleaned and bandaged his knee.

I wonder if his knee injury could have burned up those 2000 platelets that I was expecting to have included in his count today…?  Last week, the doctor explained that platelets can get “burned up” doing their job, so I guess it is possible.  We will have to wait and see what next week’s counts look like…

What a difference a unit of blood can make!

This past week has been a little rough.  Ryan started last Thursday off in a super cranky mood.  He didn’t want to eat anything, was mad about every little thing, and just wasn’t acting normal.

He had an appointment Thursday morning, but his normal doctor was on vacation and we saw a doctor we had not met before.  When the nurse first took his temperature it registered at 101.2.  We had learned from Ryan’s regular doctor that a temperature of 101 in an immune suppressed individual should be treated “as if the fire alarm is going off.”  If he gets a fever of 101 we have to take him to the ER right away for IV antibiotics.

The nurse asked if I had brought an overnight bag.  I didn’t realize I should be carrying one in the car, but based on the fact Ryan has had two hospital stays within a month, it might not be a bad idea.  They waited and took his temperature again about a half hour later and it had gone down to 99.8.  Since his temperature dropped below the admission threshold, we were told just to monitor it and the conversation shifted to his labs.

The last two weeks in a row, his numbers had gone up slightly, so I was hopeful to see an upward trend.  However, at Thursday’s appointment all of the counts were down again.  I expected we would be sent upstairs for both platelet and red blood transfusions.  However, they only sent us for platelets and said he would probably need both platelets and red blood on Monday.  Note: We have been averaging 2-3 appointments a week, and not a single appointment has gone by where he hasn’t needed platelets.  He has been averaging red cell transfusions every couple of weeks.

My gut instinct told me that he should have had a red cell transfusion on Thursday or Friday, but I also know that they try to limit the red cell transfusions as much as possible for two reasons:

1)    If you have several red cell transfusions, your body can get overloaded with iron.  I have read this can start to happen at around 20 transfusions (Ryan has had about 5).

2)    The more transfusions you have, the more likely your body will reject a bone marrow transplant in the future (which Ryan may need).

So, Ryan got his platelets on Thursday and he headed into the weekend low on red blood cells.  I think that was a bad call.  Our little guy seemed to disappear for the weekend and was replaced by a psychotic grumpy old man in a 4-year old’s body.

Every single comment we would make to him was met with rage and the angriest glare I have ever seen on a child’s face.  For most of Friday and Saturday, he didn’t leave the couch.  He didn’t even want to sit at the table and play with Play Doh or color, which in my opinion doesn’t take much effort and he normally enjoys those activities.  Worst of all, he didn’t want to eat or drink fluids.  A few times, he would express an interest in food, I would make it, and then he would take one bite and leave the table.

By mid-day Saturday, he started going up to his room for hours at a time and putting himself to bed.  This is the same kid that normally fights going to bed at night, is up at the crack of dawn, and refuses to take naps. It is not at all like him to voluntarily take a nap.

Ryan slept most of the day Sunday.  When he would come downstairs, the littlest thing would set him off in anger and he would either curl up in a ball on the couch, or he would go up to his room in tears and sleep for a few more hours.

All weekend, I suspected he was in need of red blood, but I also thought some of the behavior could be due to side effects from his medication.  I wondered if his blood count could be so low that his brain wasn’t getting enough oxygen. I tried to do more research on the side effects of his medication (Tacrolimus / Prograf) and was only able to find a small study on the effects in children who had received kidney transplants.  There were a few kids in that study that had some psychological changes.

I put Ryan to bed Sunday night and was anxiously waiting for his appointment Monday morning.  I went to bed myself, but Brad woke me up a couple hours later to let me know that Ryan was crying and screaming inconsolably and he couldn’t figure out why.  We both sat with Ryan and tried to console him and figure out what was wrong.  I tried to rub his back and he would just angrily push me away.  He didn’t want to be touched.  Do you hurt somewhere? Does your tummy hurt? Do your legs hurt? Do you feel sick? Do you think you need to see the doctor? Does your head hurt?  All of this was met with inconsolable loud crying; a scary “I’m in excruciating pain” kind of cry.  I even asked if he could see because I thought maybe he woke up blind and it was scaring him.  I was just about to pack him into the car and take him to the ER, when I noticed he was kind of tugging at the pajama shorts he was wearing.  I asked him if his jammies were bothering him and he finally screamed “I’M COLD!!!”  Even though it was a warm night, he agreed to let me change him into winter fleece jammies (or “hot jammies” as he normally likes to call them), we put him into bed with us and then he slept peacefully through the rest of the night.

At yesterday’s appointment, his hemoglobin was 6.0.  Hemoglobin is the portion of red blood that provides the ability to carry oxygen throughout the body. A Normal hemoglobin is 11.5-13.5, so Ryan’s was about half of what it should be.  I don’t know if this means that his organs were only getting about half the oxygen they should, but based on his mental state over the weekend it wouldn’t surprise me if he at least had a significant decrease in oxygen flowing throughout his body.

After his appointment with the doctor yesterday, I came home to do some work (yes, I am still trying to work full time through all of this craziness – I don’t really have a choice) and Ryan stayed with Grandma N to receive both platelets and red blood.  Grandma N said he slept in the car most of the way home. He still seemed a little cranky when he got home and I started to worry that the transfusion hadn’t been the solution.  However, by 7:00 in the evening, it’s like a switch of energy was turned on.  He started running around the house, dancing, acting goofy; I even think I heard some singing at one point.  We had our little boy back!  What a difference a unit of blood can make!