Day +53 A Day in the Life of a Transplant Patient & Small Victories

Ryan’s platelet count has rebounded a little bit. They jumped up from 78K to 119K in just four days.  I felt so relieved when I found out.  I know that platelets will fluctuate and that he is doing fairly well, but anytime I see his platelets go down, I panic.  I just can’t help it.  I watched his counts go down over and over for almost a year and it was always bad news.  I am now conditioned to be disappointed to see numbers go down and it will take me awhile to get over that.

I went into yesterday’s appointment with my usual list of questions and concerns.  After not seeing an increase in platelets for a few weeks, I wanted to know if Ryan had been tested yet for platelet antibodies and, if not, I was going to request it.  If someone has platelet antibodies, it means that their immune system is mistakenly creating antibodies that attack and kill platelets.  So, my question “Has Ryan been tested for platelet antibodies?” was met with “Well his platelets are almost 120K, that’s pretty good.”  The team acknowledged once again that they know I am worried, but they want me to know that they are not worried.

Another piece of good news is that Ryan hasn’t needed any anti-itch meds in over 24 hours. He still has a slightly visible GVHD rash and, since we lowered his steroid dose again Sunday, his cheeks are a little pinker. However, so far he hasn’t had a major GVHD flare up. The PUVA therapy seems to be helping some. Being off the anti-itch meds (which also act as sedatives) has certainly increased his energy level and overall happiness. He has been a lot more loving the last couple days and has been downright silly at times.

On a non-medical note, Ryan’s latest hobby is mopping.  Yep, you read that right. He has been asking every day this week if he can mop the floor.  I figure he can’t do much damage with a Swiffer, so why not? It also gives him some additional exercise so it’s a win-win.  I am secretly hoping he will want to continue mopping everyday when we return home.

In case you are wondering what Ryan’s days are like, below is a sample of a typical clinic day (This was yesterday, Monday 7/21/2014):

6:30

I wake up and jump in the shower

6:45

Wake up Ryan and disconnect the IV used for overnight fluids

7:15 AM

Leave for Clinic

7:45 AM

Arrive at SCCA Clinic

8:00 AM

Blood Draw at Lab

8:30 AM

Take morning medications & grab quick breakfast in the SCCA cafeteria

8:45 AM

Hickman Line Dressing Change (Ryan’s most dreaded appointment every week – it hurts…)

9:30 AM

Appointment with Nutritionist:

Weight/height recorded. Nutritionist advises that Ryan needs to continue working on getting more fluids, eating more potassium rich foods and adding calcium as much as possible.  We hear for the first time that Ryan’s last chest x-ray showed some bone de-calcification, therefore, calcium and vitamin D are really important right now.

9:45

Take Psoralen in preparation for 11:15 PUVA treatment (must be taken precisely 1.5 hours before treatment to be effective)

10:00

Clinic Appointment with Transplant Team

Vitals are taken and we discuss medications, fluids, skin symptoms, lab results, ekg results, hickman line function, appetite, nausea, and so on. The de-calcification shown on the last chest x-ray is brought up again. The team re-assures us that it is reversible, but we need to continue to address it.  Note: Ryan already takes two Viactive calcium chews a day, along with Vitamin D.  The bone issue is a common side effect of transplant and the high-dose steroids – all the more reason to keep tapering him off the steroids, if possible.

11:00

Mid-day Medications

11:15

PUVA Therapy (a.k.a. “The Tanning Bed”)

12:00 PM

Lunch back at the RMH apartment. Ryan sneaks in some SpongeBob on TV while I work on Laundry.

2:00 PM

Physical Therapy at Seattle Children’s

3:30 PM

Return to the SCCA clinic to pick up new prescriptions and IV fluid bags to be used until the next clinic appointment on Thursday.

4:30 PM

Return to RMH apartment

5:30

Dinner

7:30 PM

Start prepping IV pump (gather supplies, check pump battery, program pump, attach tubing to bag and prime)

7:45 PM

Clean and flush Ryan’s Hickman line and hook up IV.

8:00 PM

Evening Medications

8:30-9:00 PM

Ryan goes to bed (by this time, Ryan is normally asking if he can go to sleep…)

10:00-11:30

Around this time, I generally go to bed, but I am so busy processing everything that I have a hard time going to sleep.  About the time I finally drift off, I hear Ryan wake up and yell from the other bedroom “Mom! I have to go pee!”  My job is to get up with him and hold the backpack that contains his bag of fluids and IV pump so that he can do his business…

Note: This week’s schedule is an improvement over last week when Ryan needed anti-itch medication around the clock, including at 11:00 PM, 2:00 AM and 5:00 AM.

Day +50 Half Way to the First Major Milestone

There is nothing magical about Day +100 other than, statistically, transplant patients have better survival rates if they make it to day 100.  It is also the time when, if Ryan is doing well, he can get his Hickman Line removed and, most importantly, we can go home. It feels good to be half way there.

Overall, Ryan is doing well for where he is in the process. Below are updates on a few random topics:

Labs:

Platelets dropped a few weeks ago from a high of 155K, but have leveled out around 78K. The team is not concerned about it.  They often see platelets decrease when a patient has GVHD. Ryan is also on some medications that can artificially lower counts.  Once he is further along and off some of the medications, his counts should start increasing again.

ANC has been in the normal range (around 4,500 most days).

Hematocrit has been steady in the 33-35 range.  The team told me at his last appointment that they had expected his hematocrit to drop because they have been taking so much blood for lab tests and research, that no person would be able to produce enough new blood to keep up.  Again, this is temporary and it is not hurting anything.  I have agreed to the additional research blood draws because Ryan had a very rare diagnosis and I think it is important to allow experts in the field the opportunity to learn from him.  Maybe they will find something in their research that will help another child in the future.  It is the least we can do to pay Ryan’s gift forward.

GVHD:

Ryan is still fighting some skin GVHD. He has been on high dose steroids now for about 35 days.  At the highest doses, he had quite a few side effects, so the team slowly began tapering him off of them.  However, once the dosage was lowered to a certain point, his skin rash and itching flared back up.  I was sure the team would bump the steroid dosage back up.  However, to my relief, they offered to try an alternate therapy called “PUVA” (psoralen + UVA treatment).

PUVA is a therapy done three times a week for several weeks.  It involves taking psoralen, a drug that makes the T cells that are attacking the skin sensitive to UVA light, an hour and a half before treatment and then laying in a tanning bed with special bulbs for a few minutes. The UVA light then “zaps” and knocks down the offending T cells.

It was really difficult to convince Ryan to get into the tanning bed.  I am sure it looks really scary to a 5-year old.  Plus, he knew it would get warm and his skin was already itchy and bothering him, so he was afraid the hot bed would make it worse. It took some real convincing, and thankfully the nurse that administers the treatment is really patient and good with kids, but Ryan finally let me lift him in and he stayed still the entire time.  He had his second treatment Wednesday and now he says it’s fun and he likes it.  I am also seeing some slight improvement in his skin already.

Chimerism:

A Chimera was a creature in Greek mythology usually represented as a composite of a lion, goat, and serpent.  Contemporary use of the term “Chimerism” in bone marrow/stem cell transplant derives from this idea of a “mixed” entity, referring to someone who has received a transplant of genetically different tissue.

At Day +28, a chimerism test is performed to analyze the bone marrow recipient’s DNA to determine what percentage is the donor’s DNA and how much of the recipient’s DNA remains, if any.  It’s not too uncommon at this stage for transplant patients to be 95-98% donor and still have some of their own DNA.  In fact, some people never achieve full chimerism, which can create a risk for future graft failure if the original cells start to multiply and take over again.

I realized the other day that I hadn’t received Ryan’s results yet, so I asked and was thrilled to hear “oh, he is 100% donor.”  Woo hoo! It’s one less thing to worry about right now.

Ryan's Story in our Local Newspaper Today

Below is a link to a story written about Ryan that ran in our local paper today:

http://www.maplevalleyreporter.com/news/266468381.html

Day +38 Decreasing Platelet Count & GVHD Flare

Last week, Ryan was admitted to the hospital because of breathing problems and a low oxygen saturation. They observed him for 24 hours. The breathing and oxygen saturation didn't change much but the CT scan of his lungs was clear, and they couldn't think of anything else to work him up for, so they released him. We are now back at the RMH apartment.

I am feeling a bit discouraged because Ryan's platelet count has started dropping. I know it is normal for it to fluctuate, but it was up to 155K a few weeks ago, then it hovered between 135K-145K for about two weeks. Then, last Thursday it had dropped to 105k and today it was 79K.

So far, the team says they are not concerned about Ryan’s counts.  However, when I was on the phone with the SCCA nurse last Thursday and I mentioned the count dropped to 105K she said "Yeah, we noticed that too" in a voice that had a hint of concern in it.

I haven’t had a chance to discuss today’s drop to 75K with the team yet, but if it keeps decreasing at this rate, he will need a transfusion in a little over a week. I thought we were done with transfusions!  I am really freaking out a bit and am worried that he might be having graft failure. It makes me feel sick to think about that possibility.

Also, his skin GVHD flared back up this morning, so we have stopped tapering the steroids and are keeping him at the same level for now.

Ryan has really been missing home.  His dad and sister stayed with us this weekend.  It was a surprise to him and it really lifted his spirits.  However, today he was talking about how much he misses his cat, Dexter.  He tried to convince me that we should “stop by the house” (as if it were close).  I had to remind him that we are not allowed to leave the city until summer is over, at the earliest.

Day +22 Exciting Day! Ryan got to breathe fresh air for the first time in a month.

Ryan's platelet count was 6 digits for the first time today  (114,000) and his ANC has climbed into the normal range for his age (2684).

The transplant team decided to slowly start weaning him off steroids. Also, there was talk about a possible discharge mid-next week. And... We unexpectedly received a 4-hour pass out of the hospital today!!!

The funny part is that we didn't know what to do at first. We have to avoid the general public, but we don't get access to our apartment until Monday. We ended up going with Grandma Terry and Grandma N to a nearby park. We had to take several precautions - Ryan had to be covered in sunscreen head to toe, he had to wear a hat, and he also had to wear a mask as he was wheeled through the hospital to avoid germs from anyone sick we might pass in the hallways. We took Purell, anti-bacterial hand wipes, and Clorox wipes. Most importantly, we had to make sure there was significant distance between Ryan and anyone else at the park (especially kids). It actually worked out.

My favorite part of the day was seeing Ryan get excited at the park and start running for a few seconds here and there. It is hard to believe that only five days ago he couldn't even stand up for more than a few seconds!

I am continually amazed at the miracles I keep witnessing. Every morning, the first thing I do is ask for a copy of Ryan's labs. I feel like a kid on Christmas morning who can't wait to open gifts. Then, I stare in amazement at the numbers I am seeing.

For a year now, the numbers on Ryan's labs always went down. It got to the point where I was really just interested in seeing his numbers so that I would know what type of blood product(s) to expect him to get that day, how long we would be at the clinic getting transfusions, and how careful we had to be that week to avoid hemorrhage and infection.

Ryan has had over 50 blood/platelet transfusions, each taking several hours. Today, it finally sank in that he may never need another one!

Day +19 & Day +20 Increasing counts & Imaginary Parasites

As far as pain goes, each day has been incrementally better for Ryan.  He hasn't had any Morphine or Oxycodone since yesterday morning and today he walked a little bit.  It was even his idea; he didn't have to be bribed or coaxed into it.

In addition, today was the first day that all three types of blood cells showed increases on his labs. Today's counts:

Platelets: 65,000

Hematocrit: 30.2

ANC: 1665

With his ANC being over 500 for several consecutive days, his ability to take pills, and the fact that he is able to eat and drink some, he would have met the criteria for hospital discharge this week, IF he didn't have acute GVHD. With the GVHD we are not going anywhere just yet. The steroids will continue for the next 9 days and then, if they think the GVHD is under control, they will start to wean him off. If it does not get under control, I have no idea how long we will be here. At a minimum, I think we probably have two more weeks inpatient.

However, I have been asked to get our housing arrangements in order because, at some point soon, they will probably start giving us "passes" for a few hours out of the hospital and we will need to have a non-public (germ and virus free) place to go.

Being that we do not live within 15-30 minutes of the hospital (I wish), we need to stay in Seattle. Luckily, some transplant apartments that are run by the Ronald McDonald House opened up and I was able to go select one. I am not sure why, but I was picturing in my mind that they would be kind of run down, outdated and smell bad.  I was surprised that it is actually a fairly new building (11 years old). Also, in the last few weeks, they have been in the process of updating the open units, so I was able to pick out a 2-bedroom with new flooring, paint, and all new furniture donated by Lazy Boy. I will be moving some of our stuff over there next week, which should also make our room at the hospital feel a little less crowded. And, my car will no longer look like a homeless person is living in it. It currently has folded clothes for both of us stacked along the back seat, and I have been keeping stuff like cases of bottled water, extra toys, even a small trampoline of Ryan's in the back. I go out to the parking lot occasionally and grab whatever we need.

With the discontinuation of the pain meds, I had hoped that some of Ryan's itching would stop. Not so. In fact, he is still taking regular doses of both oral and IV anti-itch meds.  He doesn't know it, but I am also putting Aveeno anti-itch lotion on him. When we first arrived, the hospital initially gave us Eucerine lotion. Ryan let me put it on him once, but he didn't like that it made his skin feel greasy. With the skin GVHD, the doctors recommend lotion so that it is not aggravated by dry skin. Ryan absolutely refused to even let me put the Aveeno lotion on a small test area, so I had to resort to tricking him. The hospital gives us little travel size bottles of Johnson & Johnson baby wash. I emptied one of them out and filled it with the Lotion. He sits on the shower seat while we first wash him with the baby wash. By then, he is so busy shivering and complaining about being cold, that I can quickly put the lotion on as if it were body wash.  I think he might be starting to catch on, because today he noticed that I didn't rinse it off and was complaining that he felt slimy.

Unfortunately, the anti-itch meds seem to be losing their effectiveness.  That, coupled with a serious bout of "roid rage" after tonight's steroids were administered, made for a pretty unpleasant evening. Ryan's behavior toward the nurses and CNA's was horrible. He wouldn't cooperate when they were trying to take vitals and he was making really rude comments.  It is so embarrassing when he gets that way. I feel like the worst parent ever with my out of control child.  However, the nurses keep telling me that they see it all the time with kids on steroids.  I haven't worked up the nerve yet to say "yeah, but isn't my kid the worst you have seen?.." I'm not sure I want to know the answer.

He had a really bad itching episode tonight and was really upset that he couldn't have anymore anti-itch meds because it was too soon after the last dose. He was rolling around in bed and screamed "I am so itchy! This room is full of parasites!!!" Thankfully, his Benedryl and Zofran kicked in shortly after that and made him tired enough to fall asleep.

Day +18 A Little Better

Today was a little better than yesterday, but still a challenge. The Attending Physician this morning said that Ryan’s pain could be due to the steroids.  However, he must have the steroids for the GVHD.  The earlier you intervene with steroids, the better the outcome, and less chance that the GVHD will get worse or become a long term issue (chronic GVHD).  The good news is that if Ryan responds well, they could start tapering him off the steroids as early as next week.  In the meantime, it is a constant battle to try to get ahead of the pain and itching with Morphine, Oxycodone, and anti-itch drugs. It is quite the exercise in prediction and timing to keep him comfortable.

Another piece of good news is that Ryan’s ANC doubled again overnight and is now at 1288.  It is pretty impressive to see it go from zero to 1288 in only four days.  In addition, he has made a few platelets on his own as well, and his count has increased from 23,000 to 30,000 in the last few days.  His red cell count has decreased a little bit, but it is normal to have the white blood cells recover first and to have red cells and platelets lag behind.  I am pretty happy to see any movement with the platelet count this early and I am not yet concerned with the drop in the red count.

The “Art Cart” came around today and I picked out a wooden train kit with paints for Ryan.  I figured I would stash it away until he was feeling better (i.e., in his right mind), but to my surprise, when he saw it he said “I want to put that together now.”  With the steroids, I really didn’t think he would have the patience.  I was sure that there would be all sorts of issues with the project that would send him into a rage.

However, he agreed to get out of bed and sit with me on a mat on the floor to put it together.  There was no rage, but I did get scolded several times for not holding the paint right and not sitting the right way.  Apparently, he wanted my legs curled in a different direction for some reason.  I tried to accommodate his crazy request to avoid conflict, but one of my legs eventually fell asleep.  We did make it through most of the train project.  We are just a few wheels and some paint short of having it complete.  Maybe tomorrow…

Something finally made Ryan smile later in the day.  There is a guy that comes through the halls every few days standing on a floor cleaning machine (looks like a cross between a Segway and a Zamboni with a scrubber underneath it). He noticed that Ryan was watching him through the glass doors of our room so he drove over in front of us and did several “donuts” in front of us while waving to Ryan.  Ryan even did a courtesy wave back.