Day +95 Free of Implanted Medical Devices!

(for the first time in 14 months)

Day +92 IVIG & Appetite Changes

Ryan ended up requiring IVIG yesterday (see previous post for an explanation of what IVIG is). I anticipated that it would take anywhere from 2 to 4 hours, but it was more like 6 hours from the time we got to the infusion suite.  However, everything went fine and he didn’t have any reactions (some kids get the chills and shakes – or other reactions.)

This coming week is Ryan’s last week on steroids. As he tapers off, I have noticed that the last few days he doesn’t have an appetite in the morning and it borderlines on nausea.  I have to persuade him to eat something small so that his morning medications do not upset his stomach.  Even then, he only wants to eat a few bites.  Then, he makes up for it at lunch and dinner (he had two servings of spaghetti for lunch today and then was a little sad that we were out and he couldn’t have more).  I am concerned that the appetite change could be a sign of gut GVHD flaring up now that he is coming off the steroids.  I am really hoping that is not the case.

We have an exciting event coming up on Tuesday… Ryan is scheduled to have his central line removed!  It will be so nice for him to be free of tubes dangling from his chest. He will be able to take a normal bath or shower again without worrying about his lines and dressing getting wet.  It will also be the first time in 14 months that he will not have a medical device surgically implanted in his body.

The picture included in this post is one of my favorite post-transplant images of Ryan celebrating his freedom from the hospital. Brad captured it on the shore of Puget Sound about a week after Ryan was released as an inpatient. 

Day +88 Anxious to Go Home & Sad for one of our fellow Aplastic Anemia families

We are so ready to go home…  Ryan and I are both really homesick.  Ryan has been missing his kitty, Dexter (and of course his dad and sister).  Yes, we named our cat after the TV serial killer, but it seemed fitting being that our cat is also a “red head” (flame point Siamese).

The week before last, we practically begged Ryan’s team to give us a pass to drive home for just a couple of hours. I got a little bit of a speech about how it is against their policy, and that some doctors will not even consider it, but they didn’t give me too hard of a time. They decided to let us go because Ryan has been meeting his goal for fluid intake and no longer needs to be on IV hydration.  However, the doctor said, “Well, if you’re going to drive all that way, you might as well stay the night.”  I wasn’t going to argue with that!

We had to set up an emergency plan, and I had to agree that if Ryan even had a hint of a fever or anything else concerning, I would rush him right back to Seattle (or call 911).  Neither of us had slept in our own beds since May 21^st.  Select Comfort donated Sleep Number beds to the Ronald McDonald House Apartments (which is really nice of them), but after all of this time, I still can’t seem to find my “Sleep Number.”  It was heaven to sleep in my own bed!

Now that we have had a taste of home, these last few weeks in Seattle seem to be dragging along in slow motion.  Ryan’s “Day 80 Work-up” started last week and continues into this week.  This is a repeat of all the tests and exams that were done leading up to transplant, with some new tests added.  The purpose of the work-up is to review the status of his bone marrow, determine if he has damage to his body from the transplant, and to decide if he is on track to be discharged at Day +100.  So far, he is on track!

Ryan is so sheltered right now and is really missing the social interaction he normally would have at school with his friends.  The last few weeks during our clinic visits, I can see that he is just starving for interaction with people.  “Stranger Danger” has gone out the window and he has become “Chatty Cathy” with anyone who will give him the time of day.  He asks people where they are from, why they are there, whether they are a transplant patient, whether they have a Hickman line like his, and so on.

We don’t see many other kids at SCCA and the adult patients seem to get some comic relief from his 5-year old boy antics.  He has a few “regulars” that he visits with in the waiting rooms.  In fact, last week, he was asking one of his lady friends if she had a Hickman line like his.  Before she could answer, he quickly pulled her shirt collar out and took it upon himself to look down her shirt. As I was cringing and trying to explain to him that it wasn’t appropriate, the woman just laughed and said “oh, that’s okay.”  Most transplant patients have had their privacy violated so much that I think it probably really didn’t bother her. But still…

He has also somehow worked a deal with the woman in the cafeteria. By the time I pay for my coffee and turn around to usher him along, a full cup of whipped cream with a spoon has somehow magically appeared in his hands.

All of Ryan’s appointments lately seem to be taking twice as long because he has questions about EVERYTHING.  He wants to know what every button on each machine does and how everything works.  At least twice a week someone comments “Oh, you have a little engineer here.”

We have some of the test results from the Day 80 Work-up back and, so far, they are very encouraging.  Here is a summary:

Ryan’s platelet count has been over 200,000 for about 3 weeks now. His red and white counts are fluctuating, but in a decent range.

His DEXA bone density scan was normal for his age (good news, because high dose steroids can do some damage.)

He had a second peripheral blood chimerism test to determine what percentage of his cells are donor cells versus his own cells.  The test confirmed that all three cell lines are 100% donor (no trace of his old “bad” cells).

In addition, he had a bone marrow biopsy and aspirate last week.  A chimerism done by biopsy is more accurate than using peripheral blood.  The biopsy was also 100% donor.

The cellularity of his marrow from the biopsy was reported as 30%-40% with no dysplaysia (cells of abnormal shape or size).  The cellularity percentage concerned me at first, because I was expecting around 70-80%.  However, the team stated that a cellularity in this range is acceptable for only being 80 days post transplant.  Many patients at this stage are only around 10-20%.

Ryan continues to test negative for CMV, Epstein Bar (EBV), and Adenovirus. These are the three main viruses that lay dormant in a majority of the population and can re-activate in a transplant patient, causing significant problems.

A skin biopsy was done to test for GVHD.  The report stated that his skin sample was relatively normal with “a few apoptotic cells”.  Translation = there is only a trace of GVHD currently; nothing major.

His kidneys are stable and his liver is doing well (common problem areas for transplant patients).

Ryan will likely need an IVIG (Intravenous Immunoglobulin) transfusion within the next week because his IGG (Immunoglobulin G) level is low. IVIG contains antibodies collected from the plasma of healthy blood donors and will replace the antibodies that his body is not currently making. This is extremely common in transplant patients who are immune suppressed, and there is a chance that he will need some repeat transfusions until his immune system gets stronger.  It is interesting to note that a single dose of IVIG contains pooled immunoglobulin from the plasma of 1,000 to 15,000 donors. Thank you once again blood product donors!!!

I was very sadly reminded this afternoon of how lucky we are to have Ryan regaining his health. I learned that another young Aplastic Anemia fighter, Sydney Elizabeth, passed away today (https://www.facebook.com/fightwithsydneyelizabeth).  My thoughts are with her family tonight as they cope with her passing.

Day +70 Full of Energy!

While driving in the car a few days ago, Ryan announced from the back seat "Mom, I feel like I am back to my old self. I feel really good." Then, this morning I asked "How are you feeling this morning?" His response: "PLAYFUL!"

Day +68 Doing Well

It’s amazing to think that Ryan’s last transfusion of blood products was over two months ago.  This time last year, there were days where he needed multiple transfusions in a single day. Not only are his counts holding steady, they have gone up in the last couple of weeks!  His current counts are:

Platelets: 197,000 (Getting close to “normal”)

Hematocrit: 35 (Normal)

Neutrophil Count: 2830 (Normal)

The GVHD of his skin is still an issue at times, but it is much improved.  The PUVA therapy really seems to be helping.  As a result of the improvement, we have been able to continue weaning his steroid dosage down significantly.  If the current trend continues, he will be off steroids in about a month.  This is good for several reasons.  Steroids affect personality, appetite, add additional suppression to his immune system and cause bone loss.  Ryan’s last chest x-ray shows that he does have some bone de-calcification due to the steroids.  However, it is minor enough that it can be reversed by discontinuing the steroids and getting enough calcium and vitamin D.

Last night I had the news on and there was a story about a Florida clinic owner and his colleagues being arrested in a doping scandal for supplying steroids to both professional baseball players and high school athletes.  The reporter made a comment about the players being in trouble for taking steroids.  Ryan stopped in his tracks, looked a little alarmed, and then asked “Mom, am I in trouble?”  It was really funny, and it did spark our first conversation about how you should only take drugs prescribed by your doctor (and that are needed for health conditions), not drugs that are given to you by other people or taken for reasons other than being sick.

At Day +80 SCCA starts a pre-discharge work-up.  We are excited to be starting the work-up week after next. Ryan will basically go through all of the same tests and appointments that he did right before transplant, and then some.  This will include a bone marrow biopsy and chimerism test to look in detail at his marrow for any abnormalities, and to determine if he is still 100% donor cells.  They will also check his IGG level (the level of certain antibodies in the blood, made by the immune system to fight antigens such as bacteria, viruses, and toxins). He will have a skin biopsy to check the status of his GVHD, chest imaging (CT and/or X-ray), bone density scan, an oral medicine exam, and potentially a visit to the kidney doctor.

Then, as we approach Day +100, we will make a decision regarding whether his central line should be removed.  That is normally a big event for kids because it means that they can take a normal bath or shower in more than a couple inches of water, and without having to have their chest covered in plastic.  Some transplant patients are also given permission to swim at that point (although probably only if they have a pool at home where they can control the cleanliness and who enters the water.)  I think Ryan will be really happy if he gets a few late summer days in September to splash around in his kiddie pool once we get home.

In the meantime, we are spending most of our time avoiding the public, as much as possible, and trying to follow all of the strict post-transplant rules.  There are rules such as: he cannot sit on the grass, dig in the dirt, go hiking in dense foliage, be near fresh flowers or live plants, or eat nuts that are roasted in the shell (among other food restrictions). It is difficult at times to remember all of the rules and I feel like I am constantly telling him he can’t do something or can’t have something.

However, in some aspects, SCCA is not as strict as some other transplant centers.  For example, Ryan can have fresh fruits and vegetables at home, as long as I select them and wash them well.  He is also not required to wear a mask in public or outdoors (although at times I make him wear one anyway, and I will be especially strict about it as we enter the cold/flu season this winter).  Instead of a mask, SCCA just asks that we avoid going to places where he may have close contact with people.  Restaurants are okay, but there are dietary restrictions and we have to go at non-peak times when there will not be very many people. We play it a little safer and I am very selective about where I will take him, period. I try to limit his exposure to the public as much as possible, while still letting him have some time as a kid that feels mostly normal to him.

Last weekend was Seattle Seafair and the Navy Blue Angels were in town.  Ryan got to meet a couple of them at SCCA.  That made him want to go watch them fly.  Their show is always my favorite part of Seafair, so we set out to find a place where we could watch them, but not be too close to other people. We ended up driving around Seattle and catching glimpses of them overhead.  Then, we went to the Spaghetti Factory, which was Ryan’s first time dining in a restaurant post-transplant. Ryan really enjoyed dining there.  We had a window seat right next to the train tracks, so he was able to watch them go by.  Plus the Blue Angels kept buzzing by.

I was really paranoid about germs and kept having to tell him not to touch the wall, the window blinds, etc.  Then, he ordered a “Cotton Candy LimeAid”.  I snapped a few pictures because it cleverly came with a big puff of cotton candy on the straw.  Ryan loved it.  Then, after he had already had a few sips, I noticed there were wedges of fresh lime mixed in with the ice! I had to send it back and ask that they make it without any fresh fruit.  That incident was followed by me having to take pistachio’s away from him today because it dawned on me that he can’t have nuts that are roasted in the shells.  So many rules - but all to try and keep him safe and healthy.

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.