BMT: Isolation (Day +64)

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)

BMT: Isolation (Day +48)

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.

Clark has a new Stat lock in the shape of a teddy bear. So much cuter than stitches!

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’s around the clock medicines. This is the shorter list, since we’ve tapered a lot of medicines already.

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.

BMT: Isolation (Day +44)

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.

Super ClarkAnd 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?