Chloe's first days on Soliris

[Candace Mulcahy]

Linda asked me to post on Chloe's first days on Soliris...

As a little background - Chloe is 10 months, diagnosed on January 22nd, getting near daily ffp/cpp, which seemed to be working...except that her tiny little body couldn't handle the high levels of fluid. She was fluid overloaded during an outpatient infusion two weeks ago, which sent her into her latest hospitalization. Chloe's doctors, Drs. Listman and Schurman at Golisano Upstate Medical Hospital in Syracuse, have a couple of other aHUS kiddos. They had been talking with Alexion and the insurance company for several weeks, and got the approval through when her creatinine kept creeping up despite the infusions; she was in a delicate balance with the need for plasma and the fluid overload. Approval came on Thursday, the same day she had the vas cath re-inserted.

Chloe's aHUS seems to smolder rather than come on full power - her first episode crept up over three weeks or so, and subsequent relapses have been quicker, but still not as brutal as some of the other kids' experiences. She seems to respond pretty quickly to the plasma infusions.

She received packed red blood cells Wednesday, Thursday (after she lost a bit during surgery), and Friday (during dialysis). She began pheresis on Thursday prior to dialysis, but shortly into the procedure, her calcium dropped and she was fluid overloaded when they replenished it. They moved right to dialysis. Other than that, she has never been pheresed.

She started Soliris on Saturday - 300 over 35 minutes. No reactions, and today's labs were:

hemoglobin: 10

hematocrit: 28.3

platelets: 335

BUN: 32

creatinine: 1.6 (2.2 on Thursday, but she's been dialized once a day since Thursday)

Her bp has been in the 120s to 140s, with the help of nicardipine (drip) and labetalol (pushed) via IV.

She's had a couple of wet diapers since Soliris - prior to that she was dry since Thursday morning.

[Phyllis Ann Talbot]

So glad for even the teeny improvements Candace! To throw in our two cents - Hyde is also on Duocal - but he isn't on a feeding tube (fingers crossed he hasn't ever) but we add this to his milk, juice, or water - it is apparently really tasteless b/c otherwise trust me - Hyde would know! He was also on lipids during the 'bad' times in PICU. I also had meant to share our story after I read about the fluid overload - Hyde had a very similar story back when he first got sick but instead of blood product it was his first time trying IVIG - they sent up the wrong concentration from the pharmacy so it was double the fluid it was supposed to be. I knew it and questioned it and remember sitting there crying b/c I just KNEW it was too much fluid for him but apparently that stuff is liquid gold so he either got that or nothing. Long story short it was too much fluid and we ended up with a long journey with fluid on his lungs, etc. etc. Just know how you feel on having to be there b/c you feel like you know the most about your little one and obviously care the most. Just know that there is light at the end of the tunnel. Yell if we can do anything from GA. My sister is in Milford, PA and keeps telling me she wants to drive up and help - let me know and she can be there in 2 hrs ;-)

[Cheryl Biermann]

Yeah Chloe! Guess what? Nathan is still on a calorie additive, it is called Duocal and it has more calories, it is added through his formula...he is still on a feeding pump. It is wonderful that she is keeping her forumula down-with her receiving the Soliris so soon, maybe she will never need a feeding tube! I can't tell you how wonderful it would be to see my son eat enough so that he could grow as well as survive - without a tube!

[Candace Mulcahy]

Chloe is still being dialyzed every day. Her blood pressure has come down a bit and has been more manageable on Lisinipril, Isradipine, and IV Nicardipine. She's currently on formula with polycose, and is eating 2oz every 2 hours and is keeping everything down! The frustrating thing is that when it's not the disease that is getting these kids, it's the treatment.
Chloe's labs continue to look good. The Soliris seems to be keeping the disease at bay. We're praying that her kidneys wake up soon - her ultrasound showed edema, but there was urine in her bladder and blood flow through her kidneys. I'm hopeful for those small signs!

Thanks for your great ideas, and for sharing your experiences. It helps to have people "in our corner."

[Jodi Kayler]

Oh yeah, I forgot about Niphedipine and Lasix (that Cheryl mentioned). Niphedipine was a lifesaver. It would immediately drop BP within minutes but only last about 1/2 hour. It is not a solution but this med really did help as we got other medication doses fine-tuned.

[Cheryl Biermann]

Hi Candace, like your daughter and Jodi's son, Nathan has had bouts of extremely high BPs after fluid overloads due to all the blood products, he had a peritoneal tube put in as a baby to help him deal with the fluid, but it took three weeks to get him from PICU to TCU. His bp was maintained by a drip of Labatolol along with Norvasc, Propanolol, Mindoxidil and Niphedipine when all else failed and of course, Lasix. This incident had him in ICU for about six weeks. Also, when his kidneys were fighting for their last chance at life, he also had to be put on lipids, (he was five), he was just skin and bones, but the lipids really, really helped, since like
Coen, he couldn't keep anything down. Wonderful about the Soliris! God bless you in your struggles, we'll continue to keep your family in our prayers.

[Jodi Kayler]

Hi Candace, My son, Coen, went through his most violent episode lasting 4 months in the hospital when he also was 11mos old. We faced the same fluid, BP, and weight loss issues. I remember him being skin and bones and just wanted something to drink. It is heartbreaking to watch. We found the only combination of BP meds that worked on him where Labetalol, Cozaar, Norvask and Lisinipril (sp?). Coen still (even after remission with Soliris) remains on this same mix of meds but we have been able to reduce each one over time. Many of the other kids on this forum are on a similar cocktail.

Because of his severe weight loss as a baby, we also got to the point where it was better for him to temporarily put him on Lipids (IV food/calories) and dialyse him more. This helped give him constant nutrition (that he couldn't throw up) and give his kidneys a break while they healed. We did give the Lipids much slower than a typical dose because of fluid and BP issues but it really did give him energy to heal.

I'm just sharing our experience just in case anything can be of help. Sounds like if you can just get over this hump and dial in the right BP med combo, she's on the right track. Best of luck to you and your family.

[Amy Swarbrick]

We will keep our fingers crossed that the soliris will continue to work:)

[Melissa Hearn]

Thanks for the update Candace! Phyllis told me the other day that you finally got the insurance approval for Soliris. Yeah!!! Very happy to see the report of wet diapers. I am praying that awesome lab results and lower bp is right around the corner. Give little Chloe a kiss from Bryan. :)

[Linda Burke]

Thanks for the update, Candi - we hope that Chloe's next set of labs show improvement over the days, with accompanying better blood pressures. Hooray for wt diapers!