Today marks Akiva’s and my 7th anniversary. It’s not the typical way we celebrate our anniversary (dinner, wine, a sentimental gift). Instead, we are sleeping in different places out of necessity. I woke up at 4:30 a.m. to a very alert Idan who could not fall back asleep (yesterday’s very long nap seems to have affected his sleep cycle), and Akiva rushed over at 7 a.m. with much needed coffee and breakfast. The excitement of our day was not flowers or romance, but unhooking Idan from his IV pole for exactly enough time for him to race around the halls of the unit untethered in his favorite John Deer bike. Instead of going out to a restaurant to eat tonight, we will be taking turns negotiating with Idan to take all his oral meds. No, it’s not a traditional way to celebrate, but we are celebrating nonetheless. We are together on this incredible journey, and there’s as much love and romance in that brief moment catching each other’s eye as Idan’s giggle bounces off the halls of the BMT unit down as there could ever be sipping a glass of wine.
Today, Idan cheerfully listed all the occupations he will be when he grows up: an astronaut, doctor and lawyer, in that order. Boys with Hyper IgM have a median life expectancy of 24 years. Not nearly enough time on this earth, let alone enough time to enjoy the luxury of building a career (or three, as the case may be…). But this transplant has given Idan a chance to live a long and healthy life, and he has all the reason in the world to dream big. His doctors are very happy with his progress and we are starting the slow process of transitioning his IV meds to oral so we can be discharged after his ANC count reaches 500 and stays there for three days. We are expecting at least 2-3 more weeks inpatient, but we could be discharged sooner if his counts climb more quickly. No way to tell if these new cells are Idan’s old immune system making a return or the donor’s cells starting to grow until we test chimerism in a couple weeks, but it will be an important milestone nonetheless.
Today Idan woke up late and said he was very hungry. He had a big breakfast and took his morning oral meds. Then climbed back into bed and did not want to do anything but watch his YouTube clips (Once Upon a Time… Life– an educational cartoon about the body). He fell asleep in bed a half hour later and then continued to sleep for 5.5 hours. We had to wake him after 5pm so he did not sleep the whole day. I guess in true teenager form his body is doing a lot of growing (mostly his bone marrow), and he needs his rest.
During dinner (awake and starving, just like a teenage boy…), Idan insisted on playing his new favorite song over and over again. We all had to listen to Stressed Out by twenty one pilots. Every time the nurse walked in she would say “this song again? I love this song!” Teenagers!
His counts have continued to slowly come in over the past three days, and his ANC has climbed to 138. We closely watch this number as we hope to see a slow increase over the next week or two with the goal of getting to above 500 for three days in row, which will tell us that he has engrafted. We also started to wean him slowly on his pain meds and we will see how he handles the lower dose.
Tuesday will mark two weeks since transplant day, and three weeks since we checked into the hospital. We are all feeling the stress of isolation and long repeated days. It’s only fitting that we keep listening to Stressed Out! But, all things considered, everything is going well so far.
Well, it started out as another rough day, with Idan continuing to suffer with pain, itchiness, and fatigue when all he wanted to do was play. This morning we had a good conversation with the team who noticed right away (without us having to say anything) that, even if the pain was reduced from yesterday, the cocktail definitely needed more adjustment. We changed the dilaudid from one dose every 4 hours to a constant drip, and added a new drug that is supposed to counteract the itchiness that comes with opioids.
By evening, Idan’s mood had lifted substantially and we heard him laugh for the first time in several days. It’s the most beautiful sound. The extreme itchiness seems to have subsided and his skin rash from earlier in the week has also significantly improved.
Idan had his second school session today with another teacher from the community who teaches at the same school as the teacher who came last week. They have coordinated with each other and have already started planning a curriculum for tutoring sessions that are tailored to his interests and skills 🙂. Our heart is full, and we are so grateful to these lovely women who have added some structure, fun and normalcy to his daily routine.
Yes, another long day, but all in all, a good one.
We have started Idan on a regular dose of dilaudid to keep him more comfortable. It is always hard to know if a young child is in pain, and this is especially true with Idan, who played and smiled even when he was on a ventilator three years ago. This remains true today. He plays and insists on playing even though his blood pressure is elevated and he is grunting with every breath. He screams when using or about to use the bathroom, and writhes in bed with itchiness from his rash, but if you ask him if it hurts anywhere, he will insist he is fine. He is easily frustrated, very restless, and quite moody. But ten minutes after the dilaudid kicks in, he is breathing easy and having fun. So after a consultation with his team, we’ve decided to give him pain meds regularly instead of as needed. Unfortunately, the increase in pain meds comes with the added side effects of making him more agitated, itchy and tired. Worth the pain relief, but makes for an exhausting day for all of us. In the meantime, his team is working on finding just the right cocktail that keeps him comfortable in all respects.
Speaking of cocktails, during rounds today Idan bravely decided to tell the attending (who happens to also be the head of the bone marrow transplant division) that he does not like the taste of several of his oral medications. He went on to pronounce perfectly the ones he does not like: fluconozole, ursodial, and acyclovir. To his delight (and ours), the attending said we can switch his fluconozole to IV for now, thereby eliminating one of his most dreaded oral meds. Very proud of our 4 yr old for speaking up and for getting his team to change his medical plan.
When all is said and done, we are counting our blessings. The doctors are very happy with his progress and are doing everything they can to help us manage the side effects. Our very good friend Ashley is visiting from New York and has managed to keep him entertained all day. So grateful to all of our family, friends and all of you who have been supporting us along the way.
Day 0: Transplant Day! After much delay, Idan’s donor cells arrived and they started the infusion around 1:30PM. As you can see from the photos, there is a little bag of 160ml of stem cells from the amazing human being that agreed to donate his cells to give our son a long and healthy life. Idan was in the middle of lunch, as a true New Yorker he was eating a bagel and cream cheese, and did not let the commotion stop him (or 10 nurses singing to him). Besides bringing the cure Idan’s been waiting for we do hope the donor likes bagels!
Thank you for everyone that has shared Idan’s video. We reached our goal of 180 shares in just a few hours!!! Let’s keep the ball going – keep sharing the clip to raise awareness for Hyper IgM syndrome and the Hyper IgM Foundation.
Please Like and Share! We are hoping to reach 180 shares today in honor of Idan’s Transplant. You can support research for a cure through theHyper IgM Foundation here: http://www.hyperigm.org/donate or on Idan’sCrowdrise.com campaign: https://goo.gl/5gvS6X
Examining his CBC
It’s always the unexpected in transplant. Idan has been tolerating all the chemo well so far (but it’s still very early and the nasty side effects might wait a couple more days or weeks to show themselves), but his recovery from the central line surgery has been more difficult than it should be. He has had a lot of pain in his neck, a fair amount of bruising, and now his jaw muscles are bothering him. We are hoping it’s just a slow recovery and that there is no other underlying issue going on, but we are trying a couple things to see if it eases his discomfort. In the meantime, we are happy to now have 3 days of chemo behind us, with one of his chemo meds finished altogether today. We just started a pretty harsh immunosuppressant called ATG today, and Idan was pre-medicated with some Tylenol and Benadryl to reduce the risk of a serious allergic reaction. The ATG and the chemo continue for two more days and then Idan gets a day of rest before his transplant on July 18. Thanks for all the love, prayers and encouragement. It’s a long, difficult and unpredictable road ahead, but every day that we are moving forward we are that much closer to a cure.
Idan is doing a lot better. Thanks all for the good thoughts and prayers. If you ever wondered how Idan’s weekly infusions go, see below clip just posted to the HIGM Foundation site. Just FYI, Idan’s matter of fact approach to getting poked by three needles is not normal for a kid his agesmile emoticon We are very fortunate that he does so well with these infusions. See more here: http://www.hyperigm.org/immunoglobulin-replacement-therapy-examples/
Poor Idan has a really bad virus. Was up all night coughing and sneezing. Today at the doctors, even though he was so tired and out of it, he still insisted on giving Amanda a full check up. We still don’t know what it is, we did X-rays, virus swans and a lot of blood test. So far no clear picture. Probably a bad virus. Hoping he starts feeling better soon.