Author: Bethany

As of October 1st, we have officially changed our medical insurance. Since Clark’s disease is so rare, we thought it was best to go with doctors that are more familiar with Dyskeratosis Congenita, as well as having a plan that doesn’t restrict us from visiting with specialists regardless of whether they are in or out of network. Our previous health care provider prided itself in “preventative care”, and I think they did a great job of that. I think educating patients on how to properly eat and exercise  is wonderful, but this focus isn’t as much help when a genetic illness shows itself.

Over the last 2 weeks we have been busy finding a new Home Nurse to help with blood draws, bandage changes and cap changes. We actually can do all of these tasks on our own (as long as both Patrick and I are available)… but the real challenge is dropping off blood at the lab.  Going into a lab filled with sick people to drop off blood with Clark is extremely high risk – especially given that we can’t even take him in to a grocery store right now. So we really need this service to help out when Patrick is at work, as well as dropping off the blood at the lab.  So far, this has worked very well, and we’ve learned a lot from the nurses that have come in to help us.

Changing insurance has also meant that we need to change our pharmacy, and medical supply company. The transition has actually run quite smoothly (with the exception of a bumpy start with the home nursing company) all thanks to UCSF making phone calls and coordinating on our behalf.  UCSF has really impressed us with their customer service, and proactive approach to helping their patients.

We are also busy trying to find a primary doctor for myself, Patrick and Connor, especially since we need to get our flu shots soon! This is not an easy task. I am literally “shopping” for a doctor by searching through our insurance companies website for the “perfect” doctor who needs to be in-network. We recognize it is a massive advantage for us to have this selection, but that doesn’t make it any easier to identify a doctor that is well suited for our needs.  That said, hopefully we can find one family doctor that will be able to see all of us!

As for Clark’s progress, he is growing, laughing, playing and trying so hard to communicate! I think he is only a few months away from saying some legible words. He can’t say Mama and Dada yet, but he is very close to saying “Yes”… right now it sounds like “Da”.  Maybe we have some Russian in our background?   One thing that is helping Clark communicate is for us to not give in. Thankfully Grandmom has been very consistent with this even when I forget! When he starts whining for something, whether a toy, milk or a cuddle, instead of doing what we think he wants immediately, we wait and make Clark attempt to say the word. As long as he tries, that’s what really matters.

Both boys are enjoying their morning walks with Grandmom around Heather Farms. Heather Farms is a local park with a long walking path, playground, multiple baseball and soccer fields, a dog park, skateboard park and a large pond with fountains.  Clark walks with his mask on, while Connor rides his bike. They love watching the turtles in the pond (which happens to have tons of cute red eared sliders)

Today was Clark’s monthly follow-up at the doctors. It was supposed to be weekly, but Clark has been doing so well, they have decided “less is more”.  It had been 3 whole weeks since our last visit!! How great is that!?

Mondays are Clark’s regular days for a bandage change, blood draw and cap change (the little caps on the ends of his broviac lines). And since his doctors appointment happened to fall on a Monday, we decided to have it all done at UCSF! This way the nurses could help with our least favorite activity.  Any guess on what our least favorite activity is??  Answer: the bandage change.

When Clark had his double lumen broviac (aka “Powerline”) placed, they held the line in with 2 stitches to prevent his line from easily being tugged out. One of the stitches fell out 2 weeks ago, and the second was tugging his skin, creating a red irritated callous. So today, during his bandage change, one of the nurses cut the second stitch, and replaced it with a stat lock.  A stat lock is a sticky clamp thing, that happens to be in the shape of a bear. (so cute!!).   So far I like it much better than the stiches, and think it will do a better job holding Clark’s broviac. That said, we’ll see how it does next Monday when we have to change it with his bandage change. :)  Adhesives and skin simply do not mix!

The doctor did a thorough evaluation on Clark, checking him for rashes, going over his medication, answering all of my questions and even making sure I had the 24 hour phone number for specific hemophilia needs.  All of this went very, very well.

It turned out to be a very long appointment since Clark was also given IVIG, a blood product containing antibodies to help Clark’s weakened immune system. The IVIG is slowly given through Clark’s broviac over 2 a hour period.  IVIG is actually a product that is made from donor blood.  I had no idea this was something that people are able to donate, but I am eternally grateful since this tiny dose of IVIG will help Clark get through another month. Thank you donors!!!

Clark has been on  2 immuno-suppressant drugs, Cellcept and Cyclosporin (aka Cyc) since his BMT. These help prevent GvHD. We have tapered him off of Cellcept, with his last dose administered on October 1st! It is so wonderful to be done with this medicine, as it was a bit tricky to administer. It had to be given 3 times a day, and his doses had to be at least an hour apart from receiving his 2 doses of Cyc. This created a schedule of round-the-clock medicines.  Clark has now increased his Cyc  to 3 times per day, but I have a bit more flexibility with timing since we no longer give Cellcept. And to make it even easier, the taper for his Cyc starts today! It is a very slow taper, but he will be finished by the end of November. Overall, Clark’s daily meds have become a lot more manageable now since he only takes a few in the morning and 1 in the evenings, plus one at 1am.

On a related but different topic… We just got the referral from the doctor to have Patrick and I tested for Dyskaratosis (DKC). We were told months ago that this is a genetic mutation, and not hereditary, especially since neither Patrick nor myself show any symptoms.  But this isn’t completely true.  Since then, we have spoken to a ton of specialists and have been reading up on DKC, and apparently it is something that one of us could be passing on, even though we don’t show symptoms. This is such a rare and unknown disease, that most doctors don’t really understand it.  So we’ve learned that there is a chance that one of us may be carrying the gene, or even have short telemeres, and thus could be able to pass on to our children.  So off we go to get tested.

On the home front, Clark and Connor continue to enjoy each others company. They even chase each other around the house to give hugs. It’s so wonderfully adorable, if you ask me.

Life is wonderful with the whole family together. We still have our daily tasks of medications taken multiple times a day, Clark’s eye drops, and managing the house. But it is so lovely to have our family dinners and watch the boys play “superhero” together.

Unfortunately, there are still days where I grow a massive knot in my stomach.

The other night, Clark woke up in the middle of the night. He sounded congested. My heart stopped. Was I hearing things? Is he getting sick? Does he have a fever? Do I call the hospital’s 24 hour line or wait and see?

My mind races… How on earth could Clark have caught a cold? He has been in complete isolation. And I know we aren’t lenient when it comes to isolation.  In fact, there are some things that we are overly cautious about (even more so than the doctors). For example, doctors say Clark is allowed to play outside away from crowds without a mask, but I’ve decided to keep his mask on for these outings away from everyone. And, almost every time we go out something ends up happening, whether it is a dog running up to us, a strong breeze blowing construction dust in our direction, or someone mowing their lawn. I’d rather error on the side of safety than sorrow.

In some cases, sanity prevails and I decide to wait and see. But of course, I have him sleep in our bed to watch over him! Under normal circumstances – this is something we would never, ever do. But I’m comforted by being able to listen to his breathing, watching to make sure the congestion was in my imagination.

And sure enough, once Clark stops crying his breathing is quiet and clear.

The next day he is back to being a normal boy (with the caveat that normal in our family isn’t quite like others, of course).  No signs of sickness.

Sometimes I feel like the worry could literally kill me, while other times I’m sure it is exactly what is saving Clark’s life.  Maybe that makes it “just the right amount” of worry?

It has been over a week since we left the hospital. Life at home has been a whirlwind of medications, emesis, and deja-vu sleep training. Luckily, it has also been a lot of fun loving family time.  It’s so great to be home and to be together.  I missed having Connor walk into our bedroom in the morning and jump into our bed.

We have Clark’s blood tests done every Monday and Thursday. The labs are drawn from home, through Clark’s broviac. This is not new for us, since we were doing Clark’s labs for months before his BMT.  The only pain with drawing labs at home, is bringing the blood into the labs and having to bring the boys with me (where sick people are everywhere!) . Thankfully our insurance covers us to have a home nurse for 100 visits during the year. So now we have a lovely nurse draw Clark’s labs for us, and she also takes them into the lab!

This was an exciting week for us as we watched Clark’s test results. To give you a better understanding, here is a “Before and After” picture;

Before

1. Platelets – would drop down to 0 when we let them fall. Clark was getting platelet transfusions weekly before BMT, and every 3 days during.
2. Hemoglobin – was constantly dropping. He was being transfused if his numbers fell below 8.0. Transfusions were approximately every 4 weeks before BMT, and every 5 days during.
3. ANC – Clark hovered around 500 before BMT (max we ever saw was 1000), and dropped to 0 during the BMT.
4. Lymphocytes – Clark was in the high- normal range, hovering around 65%.

After

1. Platelets – As of Thursday, his platelets are 194,000! This is considered normal, and Clark is now safe to ride a bike!  Damn, we have to buy Clark a bike!!
2. Hemoglobin – We are still watching this. It is possible that his Hgb is rising, considering his last 3 tests showed a slight increase from 8.2 up to 8.7. He has not needed a transfusion since being home.
3. ANC – Thursday, Clark’s ANC was 3195! This is sooo incredibly wonderful since the “normal” range is 2000- 8000.
4. Lymphocytes – These are very low, as expected, since the lymphocytes are what the chemo was trying to kill. Clark is at 8% right now.  It will take a long time for these to come back.

An then we got more good news.  The plan was for Clark to see the doctor on a weekly basis… but he is doing so well, that they told us we can skip the next couple of weeks! His next appointment is October 6th, when he needs to come in for his monthly IVIG.  IVIG is a plasma protein with antibodies to help Clark fight off infection. He will need to take this until he is able to build his own antibodies.

And, not only do we get to “skip” 3 appointments, but Clark was also taken off TWO of his daily medications! He no longer needs Ursidiol (to protect his liver), or his blood pressure medicine. Clark is also being tapered off of his Cellcept. This is one of the toughest meds to give, because it is given every 8 hours.

So much good news!  All in all, we are getting closer and closer to “normal”… Or at least a new “normal”.  It will be great to get to the point we can actually take a family vacation.  We’re LONG overdue for one of these!

 

Today marks 31 continuous days that we have been in BMT at UCSF (I’m not including the 2 weeks we were in Kaiser before BMT, that would be more like 45 days).  It has been one whole month of waking up to the sharp smell of disinfectant lingering in the air.  One whole month of eating bland, uninspiring hospital “food”.  One whole month of annoying beeping, snapping of rubber gloves, and medical staff bursting into the room asking “if I mind if they” do whatever needs to be done at the moment.

Ahhhh….  This will take years of alcohol therapy to forget.

To celebrate, I wanted to give you a bit of a refresher lesson on some of the abbreviations and their meanings.  It’s important to understand this when I tell you the good news!

There are several types of white blood cells (WBC). The ones we are focusing on are called Neutrophils and Lymphocytes. As you may recall, Lymphocytes are the ones that we needed the chemo to kill, because they would attack the new bone marrow. They will be one of the last WBC’s to start growing and being productive. This can take several months. The Neutrophils are one of the first parts of the new blood that we will see engrafting. The WBC’s are used to fight off infections, such as virus, bacteria or fungus. We need all of the types of WBC’s, but Neutrophils are a great start!

Absolute Neutrophil Count (ANC) is calculated with the WBC (total) and neutrophils. This is what we need be over 500, consistently over a period of 3 days.

Now the good news!  Today I received Clark’s CBC results fairly quickly. Clark’s ANC almost doubled from yesterday. It’s 4220!! That is 3 days at over 500 ANC. Can I get a booyah!!

Alright, now calm down – there is more you need to know!

To be fair, Clark is on a medication to help “boost” his white blood cells. The medication is called Granulocyte-colony stimulating factor (CGSF). Yesterday was his last day of CGSF, so we should see a slight dip in his ANC, but it should slowly continue to rise on its own after then.  Doctors assume a pullback to 3000 level, and then a continuous rise from there.

Now more good news! Clark’s platelets increased again. Yesterday he was at 38,000, and today his is up to 52,000! This is all on his own! No booster medication, no infusions… Just bone marrow doing its job.  With the increase in platelets, the doctors decided it was safe to take Clark off of his synthetic Factor XIII. He was receiving Factor XIII because of his hemophilia. Since platelets and his factor level both play an important role in stopping or preventing bleeding, Factor XIII was a good alternative while his platelets were dangerously low.  Now that his platelets are up, no more alternative necessary!

We aren’t the only ones amazed at the progress.  The doctors are also amazed at how well, and how quickly, Clark is progressing. He is progressing so nicely, that they even decided to do his Chimera test EARLY! (this is very very surprisingly good news).  The Chimera test is just a simple blood draw to find out what percent of his blood is his “old” blood, and what percentage is his “new” blood.   This is a test usually done several weeks later than now. But… the doctors are so confident (read: “soooooooo confident”), they are gambling that the new bone marrow must be the source of all these extremely high numbers.  The sample is shipped across the country to the Mayo Clinic, so we won’t have results for approximately 10 days.

And that leads us to our final piece of good news… Since Clark has met ALL criteria for going home, there apparently isn’t anything left to do, except go home.  So that’s the plan! The doctors have decided to discharge us.  We will be packing up and leaving the hospital Thursday afternoon. This is significantly early than we expected!

Today was not as boring as some of our previous days. Clark woke up bright and early, ready to play. Things were moving along nicely, until about 8:30am when he started throwing up.

Yesterday, Clark stopped taking Zofran, an anti-nausea medication that he had been taking every 8 hours for the last 4 weeks. We decided to stop it because Clark has been doing so well, and we didn’t think he needed it anymore. Now we have the Zofran on open order as a need-to medication. Maybe he just needs to be weaned off of it?

With Clark’s nausea, and also the fact that Clark woke up too early, Clark was not in the best mood. We played by the window for about 30 minutes before Clark’s 1st temper tantrum. I didn’t want him flailing around and falling on the floor, so I had to take him back to the crib. He continued to scream at me until he wore himself out. Then we just cuddled until he was happy again.

One MAJOR benefit to Clark not taking Zofran, is that it was his last medication taken through IV. Because of this, Clark was unhooked from his lines! Freedom! (Said in a yelling sing-song way). He will be “Hep locked” every 24 hours. Meaning that they will administer 2 mL of heparin into his broviac lines to keep them from clotting. This is easy, and something we will be doing ourselves once home.

Now, onto the latest ANC news. Clark is at 2440 today!! Whaaaat!? This is in the “normal” range! My immediate reaction: “Umm, doc, are you sure you didn’t calculate that wrong? Like maybe off by a decimal point? Did he go down to 240?”

No! His ANC increased that much!

Let’s talk about some more numbers. Starting with platelet counts. Since we found out about Clark’s Aplastic Anemia a few months ago, he has been having regular CBC’s. These showed that on their own, Clark’s platelets would drop down to 0. He wasn’t producing enough (if any) to keep them at a safe level. In a normal, healthy person, platelets are between 150,000 to 400,000 mcL.

Since arriving at UCSF, Clark’s platelets continue to drop. Once they reach 30,000 or less, Clark usually has a platelet transfusion to bring them back up. Before chemo, he was receiving a transfusion about every 7 days, and since chemo, he has had a transfusion approximately every 3 days. Just last week, Clark’s platelets started to drop at a slightly slower rate.

Then, at day +17, Clark’s platelets dropped to 24,000. The plan was to give him a platelet transfusion the following day. But on day +18, they magically jumped up to 38,000! And now today, they are holding up at 38,000!  Does this mean his new marrow is producing platelets now? Most likely, but it is still early to tell.

Things are getting super exciting… Rumor has it, we may be discharged soon!  More to come…

Drum roll please….  Today, Clark’s ANC reached a whopping 740!

Way to go Clark! I was surprised, amazed and so full of joy when I heard the wonderful news. When we get 3 days with ANC above 500, lots of things change.  Here are a couple examples…

• Connor can come visit (actually come in the treatment room), as long as he doesn’t have any signs of a cold.
• Patrick and I can eat and drink in the treatment room.  No longer do we have to sneak out into the anteroom for a coffee or a snack.
• Clark can start to be prepared to return home. He won’t be released right away, but they will definitely start preparing to send him home.  And in our case, this may happen fairly quickly given that he is eating and drinking normally, and has most of his medication orally.

Putting the boys together again raises some “new” issues, but we’re lucky in some respects.

Since the boys were born, I have been on-time with their immunizations (we still space them out when we administer them, we just don’t “deny” them any). There is still a scare (depending on who you talk to) that the vaccines themselves can cause harm, such as autism or mercury poisoning. Some parents still refuse to vaccinate at all!  After spending hours reading both points of view, Patrick and I decided that the benefits of getting the boys immunized outweighed those of forgoing them.

I am very glad we chose this path. Right now, Clark has the immune system of a baby born 4 months premature. When we “wiped out” Clark’s bone marrow with chemotherapy, we also effectively “wiped out” the vaccines in his blood.

As some of you are probably aware, you can’t give a baby all their vaccines right away. During the time an infant is born, and when you can give them their first vaccine, the baby is completely exposed.  Normally this timeframe is covered by receiving their mothers antibodies and supported immune system while breast feeding.   That won’t be an option for Clark, since I stopped nursing him 8 months ago.  The good news is that we don’t have to wait as long as when he was an infant, and we can “fast track” his immunizations (and still keep them spaced out).

The reason I am so glad the boys are both up-to-date with their shots, is because of Connor. Clark is not supposed to play, or be near other children that haven’t been immunized. Common illnesses like measles, chicken pox or whooping cough could kill Clark if he was exposed.  We are lucky that Clark’s risk of catching these, or more deadly diseases, is greatly minimized due to the fact that Connor won’t be exposing them to Clark.

Similarly, none of us (Patrick and myself included) have ever had the flu shot. This year will be our exception. Since neither Patrick, nor myself work with the elderly or other immune compromised people, we didn’t feel it was necessary. When it comes to getting the flu, we had the mentality of “if it doesn’t kill you, it will only make you stronger!” Since we are now caring for an immune compromised child, we have to become much more practical about these things.

Another full day today…

Clark was entertained by the speech therapist and the physical therapist! The speech therapist helped reinforce sign language with play. Using the sign while saying the word gives Clark the option to sign if he doesn’t know how to say it. With time, he will naturally start saying the words instead of signing. We had lots of success with signing with Connor, but Clark appears to be stronger in his comprehension, which has led him to require signing less.  But now that he is trying to communicate back to us, signing is becoming very important to allow him to “talk” to us.

Physical therapy is working on having Clark walk around, squat, balance and bend over. All of which Clark seems to be thriving in. Any excuse to get out of the crib and do something, looks to be of great enjoyment to Clark!

I’m sure you are all impatiently waiting to hear about Clark’s ANC (at least I was)…so I won’t keep you any longer. Today Clark had an ANC of 370! Much higher than Clark’s 140 yesterday…..but, this is still nothing to get excited about, yet. With most patients on the recovery side of a BMT, there doesn’t seem to be a common trend when it comes to their ANC rising. Some kids numbers increase strong and steady, some take a while to get going, while others tend to fluctuate or plateau before reaching 500.

Having an ANC of 500 or greater, helps reduce Clark’s risk of infection. A “normal” ANC is 1500 to 8000/mm3, so we still need to be extremely careful to prevent Clark from getting sick. This is one of the reasons why Clark will still be in isolation for 4-6 months after we leave the hospital.

500 to 1500 is considered the ‘safe” zone. Meaning Clark is still prone to infection, but his body is able to still fight back. There are some perks to Clark’s ANC reaching 500. Such as, Connor will finally be able to visit in the room! Homemade food can be brought in for Clark and we can eat in the hospital room! Clark will also be able to walk around the BMT unit with a mask on! All exciting bouts of freedom which all of us will enjoy.