Monday, January 13, 2014

MEET EZRA: RECENT FAQS


There's a quote, "Be careful who you open up to. Only a few people actually care, the rest are just curious." While we count it a blessing that our sweet little boy's story has touched your heart and appreciate all of you who care, we're actually okay with curiosity too. Because amidst our own heartaches and challenges, we know that this is an opportunity to spread awareness about Noonan Syndrome and JMML (bone marrow transplants, feeding tubes, and the like)...and that's a good thing! 

A few oncology appointments ago, our doctor shared that one of their fellow MD's had done a presentation on Ezra to the oncology/hematology department. This kid is famous! She also told us that while this fellow was doing his research he stumbled upon our blog and showed it to her. We had the opportunity to meet this doctor at our latest appointment and he had nothing but glowing reviews for the blog (insert us blushing and saying an awkward thank you here). Apparently it is a top hit on Google when you search "Noonan Syndrome and JMML." Even though I realize this is a completely public forum to be sharing Ezra's story, learning about our new audience gave me quite the dose of stage fright! However, knowing that we have helped put more of this unique information out into the world for all to see (doctors and strangers alike), encourages me to keep sharing Ezra's story. At least until he's old enough to tell me to stop, that is :)

Anyway, it's been a while since I've written a nice, meaty Ezra update, so I thought I would devote this post to answering some FAQ's and maybe answer some other questions that you've been curious about.

How is Ezra doing?

He's doing pretty good. He had a very nasty cold come on right after Christmas, but it continues to improve, with just a little bit of congestion lingering. We know that keeping him home and continuing to breastfeed has really helped protect his fragile immune system and we are incredibly thankful for the ability to do these things for him. Also, he has gone from puking daily, multiple times a day, to only puking once in over two months! We cannot even begin to explain what a relief this has been. Forceful vomiting and oversensitive gag reflex are apart of NS, but it is like a switch has been flipped with Ezra, and those are a thing of the past. Hopefully the worst of that is behind us (although, we hear it can come and go).

Why is Ezra mostly housebound?

Even though Ezra is not currently on any chemo regimen, we still consider his immune system to be compromised because of the JMML. Also, because of the chemo he's had in the past, he hasn't been able to be vaccinated. He had his very first round of shots before being diagnosed with JMML, but hasn't been able to receive any more...including his flu shot. Because of this, we plan to keep him home at least through flu season, and then we will reevaluate. He obviously makes trips to the children's hospital/lab, and we have made a couple quick trips to the store, etc, but it is very important that we avoid any crowded places or anywhere Ezra could come into contact with someone who might be ill. We don't want to take any unnecessary chances or any steps backward in his body's ability to recover from JMML.

How does mama cope with being housebound?

Quite well actually. Thankfully I am a homebody and I love being at home with my boys. But, I am human, and I can get a bit antsy/stir-crazy at times. I try to stay focused on the fact that this is temporary...Ezra will get better and someday we will get to reenter society. I also have an amazing support system. It goes without saying that my husband and our families bend over backwards to help me in any way they can. But also, in the height of the scariest times with Ezra, a couple dear friends of mine (who happen to be nurses and can't be too scared off by anything I have to share with them about Ezra) became bound and determined to get me out of the house...and I will be forever grateful to them for being there for me when I needed it most!

What determines whether or not Ezra will go back on chemo?

There is no cut and dry answer, but essentially, it has to do with Ezra's overall health, the size of his spleen, and his blood counts. Right now he has a cold like I mentioned, so his counts are a little high, but his spleen is only very slightly enlarged. His counts have been a bit elevated since early December, though, so we have gone back to getting blood work every two weeks. Not fun at all, but this is obviously something that has to be monitored very closely.


At Doernbecher's last week.

Will Ezra ever have to undergo a bone marrow transplant?

We want to say with 100% certainty that the answer to that question is 'No,' but it is not 100%. There is no guarantee that Ezra's JMML will self-resolve. However, we have never heard of a child with NS not self-resolving, so we choose to believe that no, Ezra will not have to undergo a bone marrow transplant. It is a much happier frame of mind to live with when we are operating under that assumption!

Why does Ezra still have the feeding tube in his belly if you are no longer using it?


First off, I'm happy to report that our GI is in support of our decision to stop using the tube and the feeding choices we have made for Ezra over the last couple months. But, there are no guarantees that we're done for good. She is pleased that Ezra has been able to grow as well as he has breastfeeding alone, but if we start to see his growth plateau, we may have to implement some overnight tube feeds again. And, if he ever has to go back on chemo, we will be able to use it to administer his medications which is the most important factor of all. We hate that he has it, it is a pain to take care of/keep healthy, and we are constantly worried about him pulling it out (and everything that would go along with that), but he had to go through SO much to get it surgically placed, and we would never, ever want him to have to go through that again! We can't wait until it's gone...but for now, it has to stay, just in case.


Well folks, I think that checks off quite a few boxes of what inquiring minds would like to know. We'll have to save the rest for another time. We continue to be humbled and thankful for your continued support, prayers, and well wishes. This little guy has come such a long way…we hope for continued improvement and more good news to report in 2014! Thank you for following us on this journey.

4 comments:

joe said...

Thanks for the update. It is helpful for all of us to hear your thoughts. I know it is not always easy, but know your mom, who is there daily will continue to support your family and i will also. Lots of love. Continued prayers daily are essential. God can make him well. Dad

dmj said...

Elizabeth and Josh, thank you so much for the insight. I appreciate that you do this. As I pray it gives me specific issues to appeal to our God. And I can only speak for myself, but as you share with "everyone" how could one not be humbled and see in awe how God IS listening. Also as it definitely not easy for all of you your bravery and strength is such a testimony. I love you guys and it is an honor to know you! God bless you all!!

Jonathan Hansen said...

Thanks for keeping us up to date, cousin. We continue our prayers for our precious cousin as well as for your family. Love you all.

Anonymous said...

Elizabeth, I don't know if you remember me or not. I remember you from church in Green Bay. I do know that God will help us through some very hard times. I was heart felt by your blog and will be praying for you and your family. Bob Delzer