Starting ACTH

It's been a while since I've updated this blog and alot has happened.

The meds that Yusef was on for the infantile spasms just simply aren't working. His last two EEG's continued to show frequent seizure activity. Developmentally we are at a total standstill which is the most frustrating thing that has gone on. I'd say my 10 month old is still at a 3 month level, even regressing since this all began 6 months ago.

So two and a half weeks ago, we started the ACTH treatments. ACTH or adrenocorticotropic hormone is a treatment which is sometimes effectivce with IS for reasons not understood by medical science. The cost of the six week treatment is well over 100,000 dollars. No that is not a typo. It's craaazy. Apparently, Questor is the only drug company that manufactures it, and since it used to treat a rare condition, they can charge whatever they want! Well yay, for them, and thank God, thank God, we have decent prescription coverage.

These two vials costs $93,000, and there's more to come

It's quite stressfull to even have these vials in my house. I'm deathly afraid there's going to be a fire or something. Of course, even more stressfull is jabbing my sweet boy in the leg with a needle every morning.

The treatment is a six week course. We started at the highest dose 3 weeks ago, and are tapering down each week. The first week Yusef did great. He immediately became more alert and began cooing and gooing like never before. He had a light in his eye. He looked us in the face and truly studied us for what seemed like the first time. Then last week the insidious seizures started again, and he again became like a zombie, spaced out, seemingly oblivious to the world. Since then, the seizures have come and gone. One day, I'll see seizures, and the next he'll be back again, alert. He still hasn't smiled for what has seems like months. He's still never laughed.

ACTH is still considered the gold standard in treating IS, but there are still some treatment options that I'm quite interested in, namely the ketogenic diet which has shown to be as effective in some studies. There is also a drug called vigabatrin. I'm also seriously thinking about taking him to Children's Mercy in KC to get a second opinion as I fell like this Dr. has been dragging his feet with this. This has gone on now for 5 months! Why did we drag our feet so long before starting the ACTH and why did he never mention this ketogenic diet? I'm still learning about this, but I know that it will be the next step if this treatment doesn't work. I feel in my heart, it's not going to. And now I feel trapped since we're right in the middle of the ACTH and I feel guilty because I should have taken him for another opinion sooner when we weren't seeing results earlier in this journey. Then again, this could all be the angry rantings of a desperate mother.

This has been a heartwrenching experience and it isn't over yet. In some ways it feels like getting the diagnosis all over again, the same fears and grief, and in between glimmers of hope.

Make a Little Boy's Last Christmas Special

This message came from a fellow blogger Salma whose blog can be found here.
Please take the time to make a little boy's day!

I just learned about a 5 year old boy named Noah Biorkman who is in the last stages of a 2 year battle with Neuroblastoma Cancer. His family is celebrating Christmas next week and Noahs request is to get lots of Christmas cards.

Please let's make this little warrior's wish come true.

Send cards to:

Noah Biorkman

1141 Fountain View Circle

South Lyon, Mi 48178

Lets see how many cards
we can get together for this brave little boy.

Thank You and God Bless.

Please also consider posting this very urgent message to your blogs.

I just wanted to share this site A Little Extra by Connie Wenk. She takes the most extraordinary pictures of children with DS. It's such a gift to have these children in our lives and she says it so beautifully in her pictures.

Here's one:

We've Got Ourselves a Big Eater

I started feeding Yusef solid's a couple monthes back. I wanted to get him into it slowly due to his seizures and just generally being in a fog all the time. I have to admit part of it was sheer laziness on my part as feeding babies has got to be one of the most monotomous tasks I can think of. I'm always so glad when they can start feeding themselves.

But a couple weeks back I realized that this is the one thing I can control and that we can work on. To my delight, it didn't take any work at all as it seems he's just a natural eater. In fact he loves to eat anything and everything. Three meals a day, he sits down with us and indulges in rice cereal and prunes or oatmeal and peaches. So here we are, officially in the club of big boy eaters!

Yusef's First Camping Trip

My Dad's been in town all week, and as is our usual fall tradition, we went camping out on the lake. Before I had children, we would rough it in a tent and sleeping bag, etc, but nowadays we rent a cabin. It's complete with all the modern ammenities and creature comforts which makes camping with young children much more enjoyable and certainly easier.

The drive out is a beautiful one. I've often heard people say that Kansas is so boring and flat, but I find it beautiful. Sure, it doesn't have the majesty of the Rocky's or the wonders of Yosemite, but it does have a very real and subtle beauty. My favorite part of my state is the flint hills--a unique place that is the home to the nations largest remaining tract of tallgrass prairie. I'm a prairie girl alright. I just love it. These pics don't do it justice by any means.







Apparently deer really like the prairie as well because we hit one on the way in! Thank God my dad was driving and didn't panic and no one got hurt. Well, no humans got hurt, the dear deer didn't fair as well. The front left hand side of the van sustained considerable damage, but amazingly after filing a police report for insurance purposes, we managed to drive out of there and onto the lake!
The van



Actual deer hair in the rim of the tire. I cannot believe it didn't blow.



It was a cold and dreary day, but as soon as we got to camp we made a campfire of course!



Our cabin and the view off the front porch




We went to the playground and let the kids run off some energy.

Here's Yusef with Mommy all bundled up and cuddling in his sling.





Later, it was time for some serious hot dog roasting



And after dark, s'more makin' of course. I like my marshmallows nice and dark, like they literally have to catch on fire to be considered done!



The kids having a blast with their glow sticks



I would have loved to have been able to stay more than just one night, but we had to get back to reality, school, and life. So that's it. Our annual family camping trip, and Yusef's first, with many more to come, inshallah.

First the Good News

The Good News:
Somaya is doing much better. We spent 6 hours in the ER Friday night getting IV antibiotics. They also gave her some morphine because she was in so much pain and Tylenol for the fever. The doctor said she could be admitted to give her another dose of antibiotics in 24 hours or we could go home with our current oral antibiotics and some Lortab. I didn't want to spend any more time in the hospital so I opted for going home and thankfully she's doing fine now--back to her old feisty self.

The Bad News:
Yusef's EEG still shows frequent seizure activity. We saw the neurologist on Monday and he thinks it's definitely a good sign that we're not seeing clinical seizures so he wants to give it 6 more weeks and redo the EEG. If at this time, Yusef is still showing seizure activity, we'll go ahead with the ACTH treatments. This also explains why we're not seeing any progression in his milestones and the limited interaction.

ACTH (adrenocorticotropic hormone) is generally a very effective treatment for infantile spasms. However, it comes with a whole host of scary side effects. Everything from irritability, to gastrointestinal bleeding, to sepsis.

The Dr. said these would be given weekly, and that I could do these at home. He knows I'm a nurse and I have worked with him before in the professional setting. I'm assuming Yusef will need a PICC line, which again, I've cared for many, many times.

Kudos to all you Mom's out there who've had to care for your little ones at home with NO medical training. I remember when Zainah had her Hickman and later a PICC line, how stressful it was caring for them, even with lots and lots of experience. I can't even imagine how intimidating it would be for someone learning everything for the first time.

Part of me just wants to get this over with and go ahead with the treatments. I want to see my my boy advancing and working on all his milestones again. Sigh. Patience is a virtue I guess. . .

Oh yeah, and now Zainah is sick with stomach cramps, vomiting, and fevers. Blah. I mean, can we catch a break with these kids. Sometimes, you just gotta laugh so you don't cry.

Update on Yusef's Infantile Spasms

It occured to me that I never really gave an update on Yusef's progress on his meds. I'm happy to say that the second med we started seems to have done the trick and he's been seizure free for the last several weeks. He's on Kepra 3 times a day and Zonegram twice a day.

I have noticed that he seems to be a tad more sleepy since this all started and he hasn't progressed at all developmentally. From what I've learned from other mom's this is to be expected and often happens with the IS. In fact, I think it's safe to say, he's even regressed on a lot of his gross motor milestones. He won't roll over like he was before, and rarely ever smiles. He seems to avoid social contact and sometimes refuses to even look at us and interacts rarely. Our therapist seems to think it's a sensory thing caused from the seizures and meds. However, just the other night I was thrilled when I got several sweet smiles out of him!

We've got an appointment Monday with the neurologist and a follow-up EEG--I'll definitely bounce it off of him and see what he says.

Hopefully we'll be actually be able to make it to the appointment since big sis Somaya is fairly ill and might have to be admitted into the hospital for IV anitbiotics. She's suffering from a UTI that doesn't seem to be responding to the meds. Her pedi thinks there is a good possibility it's turned into a kidney infection in which case she'll have to be admitted. We do have vesicoureteral reflux in the females in our family. It's essentially what happens when the urine backs back up from the bladder up the ureter and into the kidney. It can cause chronic infections. Zainah and I both had it. Somaya has never been screened for it since she's never had a UTI before. Right now we're just waiting to see if her fever returns tonight and if it does we're to take her to the ER. Her last dose of ibuprofen was at 2:00 and it's now 6:30 so we'll see what happens here in the next few hours.

Oh yeah, our buddy walk is tomorrow and unless Somaya makes some type of miraculous recovery, I doubt we'll be able to go. I'm really bummed about this. I was so excited. It seems as if every time our local DS chapter has some big event, one of the kids is sick or there's something else going on. Sigh. I dunno, maybe Somaya will be okay. That's what I'm praying for right now--and my sanity--it's been a rough couple of days...