I have no friggin’ clue! 

I have emailed all of Hannah’s main physicians, and a couple speculate that Hannah should be considered a priority patient because of the severity of her disease and her age, but they have no control or say in the distribution of the Cerezyme that is left.   But if they run out of Cerezyme, even if she is a priority (again, purely speculation, no proof), you can’t give her a treatment with a drug that doesn’t exist!

I’m scared to death that Hannah will miss a number of treatments and that her condition is going to go into a decline.  She worked so hard for the past 4 months worth of treatments, and we have seen such a huge improvement in her attitude, ability, and just her overall wellness.  Even though the Cerezyme doesn’t cross the blood-brain barrier, it definitely has worked for her spleen, liver, lungs, bones, and other organs.  Her previous bloodwork and reduction in spleen size is proof of that.

How does this happen?  How can a company as huge as Genzyme catch a virus that would completely shut down an entire factory?  After talking to our local genzyme rep, it does seem as they are working as fast as they can to figure out exactly what they are going to do and how they are going to handle all 5000 of their Gaucher patients (of which only a couple hundred are GD23). 

Based on this Bloomberg article, “The shutdown of the plant, Allston Landing in Boston, will cause a shortage in August of Cerezyme, a drug that treats Gaucher disease…”.   August is less than 2 months away!

Doesn’t Hannah have enough strikes going against her already with this disease?!  Please, please someone give her a break!!!!

I already have a message into my contact over at Genzyme.  I want to find out how this is going to affect Hannah’s treatment schedule and the other GD23 kids!

http://www.google.com/hostednews/ap/article/ALeqM5hYFudhNd3uLPeN-pYJsu1dVT62wwD98RSO5O4

NEW YORK (AP) — Genzyme Corp. said Tuesday it shut down a key production facility as it cleans viral contamination that has been slowing down the process for making supplies of two biotech-based drugs.

The biotechnology company said the virus, which is not harmful to people, was detected in one of six bioreactors at a facility in Allston, Mass., and as a precaution, it will shut down until the end of July to properly clean all of its equipment.

Bioreactors are used in the production of living cells, which are in turn used to make the biotech-based drugs Cerezyme and Fabrazyme. Supplies of those drugs will be constrained because of the shutdown.

Fabrazyme treats an inherited disorder known as Fabry disease, which is caused by the buildup of a particular type of fat in the body’s cells. Cerezyme treats Gaucher disease, an enzyme disorder that can result in liver and neurological problems.

Genzyme said it has confirmed that the virus was the cause of two previous declines in cell productivity at the Allston facility and another one in Geel, Belgium. The company is adding steps to increase raw materials screening and viral removal processes. Meanwhile, Cerezyme and Fabrazyme inventories are not sufficient to meet global demand.

Genzyme is still measuring the effect of the delays on Cerezyme, but the company expects temporary supply constraints to begin in September for Fabrazyme.

“The patients who need these therapies are our priority,” Chairman and CEO Henri A. Termeer said in a statement. “We are confident in the quality of the products produced in Allston and in our ability to resolve the issue affecting the plant.”

In a separate statement, BioMarin Pharmaceutical Inc. of Novato, Calif., said the contamination will not impact production of Aldurazyme, which treats an enzyme disorder and is marketed by Genzyme.

“We are unsure how quickly the facilities will be able to replace ‘lost product,’ thus we can only estimate what the value of the six-week delay is,” said Deutsche Bank-North America analyst Mark Schoenebaum, in a note to investors.

We made it through our first week of summer with Abigail home and Ethan at camp.  We survived!  And it really wasn’t so bad.  One week down, many to go!

We have a lot going on this week:

Monday:  Cerezyme treatment, all day
Wednesday:  Physical therapy at TCH
Thursday:  Orthotics fitting, Occupational therapy at TCH
Friday:  Pediatrician visit, Developmental therapy at home
Sunday:  Father’s Day and tentative Greyson’s Law Celebration (about 3-hour drive from home!)

Tomorrow should be interesting.  Abby is home tomorrow, so she is going to come downtown with Hannah and I to the hospital.  Daddy is going to meet us there (since he works 5 minutes away), and he is going to take Abby to lunch and to his office for an hour or so.  Then, he is going to bring her back to the hospital towards the end of the infusion, and then I’m going take both Abigail and Hannah home. 

Abby is SO excited to visit “Daddy’s work.”  What is it about a child visiting their daddy’s work that always seems so exciting to them?  I remember visiting my parents work when I was little, and it was always such an awesome feeling being able to go into this whole new world.  She is so looking forward to it!

I just hope Hannah’s treatment begins on time tomorrow, because I am not looking forward to having to deal with Abigail while being tied down to an infusion chair with Hannah for more time than necessary!   🙂

Before Hannah, I would look at other parents of children with special needs, especially moderate-to-severe special needs, and think “Wow, that is a lot of work, how do they do that?  I don’t think I would be able to do that.”   But that is all I saw — the “work” that was involved in taking care of a special needs child.  For whatever reason, I never saw anything beyond that.

Here I am with a special needs child now.  I think back at that feeling I used to have, and now I get it.  You just do it.  You do what you have to do to keep your children happy and comfortable.  It doesn’t bother me that we have over a dozen therapy sessions each month or that we always seem to be going to a doctor’s office for a visit or hospital for some procedure.   I know why families fall into financial distress because of this responsibility and just accept it because the alternative of not providing every need is not an option.  It just becomes a part of life.

But there is a huge reward to all the “work” that goes in to taking care of her.

The reward is Hannah’s smile, her happiness, and her absolute and unconditional love she shows me and the rest of our family.  All the extra “work” taking care of her and researching for a treatment for her is just so worth it just to be able to cuddle with her, smell her, and watch her learn and explore at her own pace.  She truly is an intoxicating child, and I just love every minute I get to spend with her.

I look back on the past year.  This time last year, I was on what would become 2 months of strict bedrest because of pregnancy-induced hypertension (which never left after Hannah was delivered).  That entire time it was always “me” they were worried about because “the baby” looked so healthy and was doing great.  Never in a million years would I have expected what we have encountered since she was born.

There have been a lot of tears, a helluva lot of worrying, and so much fear in wondering the what-when-how-and-where of the progression of her disease.  There has not been a day that has gone by that I haven’t thought about it.  Even with each step forward, it is right there in the forefront of my mind.  It is even stroner on those days we have a set back.

But now I understand why they say “each day is a gift” because with Hannah, every day I spend with her right now is a true gift.   I have spent almost every awake hour of every day with her since she was born.   She is a true light in our lives, and she has positively affected Ethan, Abigail, Daddy, and I.  Our family and friends have fallen in love with her. 

She truly is a special, special little girl.  She is “my” special little girl.

After so many positive steps forward this past week or so, it was inevitable that we take a step backward somewhere.  

For the past three days, Hannah has refused to be spoon fed.  Flat out, not going to have it, cry when she sees the spoon and food refusal.  She has no problem putting a spoon in her mouth and munching on it for an entire family meal (she loves having a spoon in her mouth).  She has no problem putting toys in her mouth and going to town on them.  But she refuses to eat by mouth with a spoon.

We were making such progress in that area, almost getting 3 full jars of baby food in by spoon each day.  She still would never open her mouth for us to put it in, but she was letting us lower her bottom lip and maneuver it into her mouth.  But now, she won’t even do that.

The bright point is that she is not refusing food completely though.  She still gets between 25 and 30 ounces of formula a day plus 4 to 5 jars of stage 2 food in those bottles.  Peaches are her favorite.  Her weight gain has been right on track, and she is definitely not emaciated. 

She just refuses to eat by spoon. 

We start occupational therapy at the hospital next week with a therapist experienced in feeding issues, so hopefully we will make some progess in that area.

I am so surprised that Hannah even made it through physical therapy today. 

She was feeling much better after using the Florastor, and her stomach flattened down quite a bit from the gas that had built up in her stomach.  So I decided to give it a try.  I’m glad I did.  She made it through the entire 45-minute session.

Pam, her physical therapist, is absolutely awesome.  We started very slow and took quite a few “mommy breaks,” but Hannah definitely wanted to keep playing. 

We noticed two huge accomplishments today.  Hannah, while in a sitting position, actually now puts out her hand to balance herself while she leans down to grab a toy!  Yes, she is learning to LEAN DOWN AND BALANCE on her own!  This is a huge first step (now we have to get her to put out her hands when she loses balance, but one step at a time). 

The second accomplishment was using the big therapy ball — she stood up with her hands on the ball and balanced for almost 5 minutes WITHOUT her stomach or other part of the body touching the ball.  My baby girl was standing !!   I tried her out using the sofa when we got home, and sure enough, she can now stand up for a few minutes while holding on to the couch!  (Of course, we still have the falling issues since she just tilts and falls, doesn’t know how to protect herself yet).

I keep hearing in my head what Dr. Eng, Hannah’s genetics doctor, told me last week…the fact she is still making progress in her development is HUGE.  Even baby steps like this are huge milestones for us.  Because, her brain is still letting her learn and develop.  Huge, I say — HUGE!