Archives for September 2010

With Hannah’s condition getting back to the baseline she was at before she was hospitalized (and even better in some places), we decided that we need to start getting back out into the real world and doing things as a family again.

After a few attempts out  (few restaurants, shopping, Freemont Street, and a craft festival), we are realizing that it is going to be much more slow going than we anticipated.  Either that or we need to adjust our mindset as to what we can do “out” as a family.

Likes…

• Hannah loves being out of the house (hell, who wouldn’t!).
• She really enjoys looking at what is around her, especially if it is lights and people.
• She loves walking through the casinos with all the lights and sounds (that aren’t too loud for her).
• She loves the water (her grandpa held on to her while he dipped her legs in the pool, and she loved it!).
• Even though it takes quite a bit of strength for her to pull herself to sit up in her stroller, she does it the majority of the time (instead of lying back) because she wants to see what is around her.

However…

• She does not like sitting in a high chair for any longer than a few minutes without either being picked up or being entertained with her portable DVD player.  She will play with her toys at the time briefly, but that only buys us a little time.
• She cannot tolerate much heat or direct sun at all — she closes her eyes, her body wilts, and she just tries to shut down.
• She cannot tolerate loud music, especially if it is pounding loud (like Freemont Street Experience was).
• She cannot watch moving things very well because of her limited vertical and horizontal eye movements — if it isn’t in her midline vision, chances are she won’t see it unless she has time to thrust her head up or down to that area!
• She gets tired and worn out pretty quickly.  Even just an hour or so doing something stimulating is enough for her.   It is going to take some time before we can take a day trip to Sea World or Disney World or something like that — but that is our goal!

The only big drawback is having to lug around the trach suction machine and carrying our extra-large diaper bag (because of all the extra supplies).  There have been a few times that we have had to break out the suction machine during our outtings.  Most of the time she does not need it.   I don’t like having to use it in public because of the incredibly loud vaccum-like noise that comes along with it.  I don’t like the stares we get during these times, but I know people are curious — I am sure I would have been too if I saw something putting a suction tube into someones throat!

Oh and sidenote…We realize that she can STILL have breath-holding spells while be trached!   The problem we had in the car where she would get upset being in the car for a too-long-for-her time (10 minutes or an hour, it all depends), and she would start to hyperventilate or have a breath-holding spell?  We found out today — that can still happen even with the trach!   We were driving to Caesar’s Palace to take a walk around the forum shops (getting Hannah out in what looks like the outdoors but is still indoors).   She started getting really cranky about 5 minutes before we got there.  Sure enough, about 1/4 block from the entrance to the parking structure, we had to stop the car so I could get out and get her out of her car seat and into my arms.  It was only a 2-minute mommy-daddy hug-love moment before we could put her back in her car seat, and she was fine from that point in the car ride on.

But we also realized that when she throws a tantrum (possibly leading to a breath-holding spell), that we cannot hear her because she does it silently.  So during our window-shopping and people-watching walk around the Forum Shops today, we were all constantly looking at Hannah to make sure she was okay (a few times she was starting to get cranky).  Definitely does not make it very relaxing, but we will do what we have to in order to get out!

I am truly amazed at how wonderful Ethan and Abigail are when it comes to us having to limit certain activities or events because of Hannah’s reactions and limitations.  They are just so good with her, and they also keep an eye on her to make sure she is okay.   This is becoming the ‘normal’ for them around Hannah, and Ethan told me recently that he didn’t care “as long as Hannah gets to go too.”    Even my nephew (who is just a bit younger than Ethan) is starting to understand “the way it is” when we all go out with Hannah.

We have taken advantage of having nursing on Saturdays with Ethan and Abigail, as we took the two of them to breakfast and then to the Children’s Museum this weekend.   They were able to run around the two floors of the museum and play with Daddy and I without having to have our attention always focused on Hannah.   Daddy and I had a great time with them, and I didn’t feel guilty because we were only gone a few hours.  But I know that the kids appreciated having this one-on-one time with them.

I can’t wait for the weather to get cooler (still in the upper 90s to low 100s during the day) so we can start adding back things like the park and other outdoor events.

When Daddy took Hannah to Occupational Therapy on Monday, he mentioned that the OT had tried Hannah on the stairs up to the trampoline…and she did it!   She complained and cried and tantrumed the entire time, but she made it up the stairs…TWICE!

I took Hannah to physical therapy today (same place as OT), and at the end of the session, we tried Hannah on the stairs again.  Sure enough, she made it up THREE times!  It is SO much work for her.  You can see her straining her muscles to get her knees up, but she did it.   She even went backwards once (unintentionally, but she was scared to death of going into the trampoline so she was high-tailing it backwards to get away from it).

I am so, so, so proud of her.  This was one of those feats that I never expected Hannah to achieve so soon.  I sure as heck would not have had her attempt it.  But both her physical therapist and occupational therapist really push Hannah believing, as our PT put it today, “Hannah can probably do a lot more than we think.  She just never has been pushed to do it.”

Truer words could not have been spoken.  We realize that we baby Hannah so much because of her weakness and delays that we forgot to try new things that we feel are out of her reach.  It was a great wake-up call.  This is one step closer to get her to pull to a stand on the couch again and start cruising, which is where we left off before she was hospitalized.

Hannah was discharged (finally) from the hospital just a bit over 2 months ago.   Her physical recovery is absolutely amazing.  Her PT was telling me today that what she has seen Hannah accomplish in just one month is 10x more than she would have expected.

I’ve said it before, but Hannah is truly amazing.

I think a lot of my anxiety over the past few weeks has to do with the uncertainty of Hannah’s prognosis.  Seeing her disease progress, realizing that she is falling farther and farther behind developmentally, dealing with more complicated medical issues, etc.

But a lot of it also has to do with Baby Kyle.  He was a sweet little boy who was just a month older than Hannah, who had been diagnosed with Gaucher’s type 3.  He had been doing reasonably well.  He didn’t have much significant health involvement outside of developmental delays.   His mom put him to sleep one night, just as she always did.   He was full of smiles and just a happy boy.

The next morning, without any warning, he was gone.

Hannah was in the PCU at Texas Children’s Hospital when this happened.   When I found out what happened, we were just a few days shy of being transported to Las Vegas’ Sunrise Children’s Hospital.

To this day, I cannot get him out of my mind.  I always thought there would be some warning, some significant progression of this disease.   Something to give us notice that we had to prepare ourselves for what could happen.   He was doing seemingly well — what was it that happened that took him away so suddenly from his family that loved him?

Every night since when I put Hannah to sleep for the night, I worry about the “what if…”    If I am honest with myself, I know this is a large part of the reason that I take the “night shift” with Hannah, staying awake and keeping an eye on her until 3 am to 4 am each morning.

Before I decided to take this “night shift,” Hannah had already pulled out her trach part-way twice, got a mucous plug in her trach (plugs the trach so she has a hard time breathing), and has been dry multiple times.   I don’t want to take any chances that something like this could be something that would cause more problems.

But I also know that this is not healthy for me either.  I have not slept a full night’s sleep since we brought her home from the hospital.  I sleep 2 to 4 hours at a time, 2 to 3 times a day.  Ethan and Abby are used to me taking a 2+ hour nap almost every day, and they know it is because I am up with Hannah.  My day gets wasted as I am exhausted all of the time or sleeping when I could be getting things done.

But I also don’t want to waste our nursing hours on a night nurse.  Most of the time, I don’t have to do much to Hannah except maybe adjust her trach collar a few times (untangling her when she wraps it around her neck or body), suctioning her if she gets junky (which is not much except for when she has trachiitis).  I can’t justify losing our daytime nursing where we can spend time with the other kids and getting stuff done because Hannah is a constant during the day when she is awake.

Kyle, sweet Kyle.   I know your fight is over and you are free of this crappy disease.  Please, please keep an eye on my sweet Hannah, protect her, and keep her safe.   I will never forget you and your adorable smile.

Last night was a really rough night.   Hannah seemed to have developed trachiitis again, and she was just miserable overnight.   I must have suctioned her trach out about 30 times between midnight and 6 am (we rarely need to suction her at night normally).  It was thick, junky, and green — obviously another infection.

She also started throwing up her food again when we started bolusing her at the normal rate.   Mostly it was mucousy because of all the junk in her lungs and trach, so she barely got 1/2 of what she normally would today and at least half of it was pedialyte because the formula just seemed to heavy for her.    Poor Daddy got the brunt of it, as he must have gone through three Tshirts in a 2-hour period!

Fortunately, she was not in any breathing distress, so we knew it was trachiitis again.  Just like it was last month.

Good thing we had our appointment with Dr. Nakamura today, Hannah’s new pulmonologist.   I really liked the guy.  Listened, very knowledgeable, and really took an interest in Hannah by asking a lot of Hannah-specific questions.   Just the type of doctor we wanted.

Hannah slept through the entire appointment.  She was just exhausted.  For the trachiitis, he said that it is very common to have recurrence of trachiitis often with “these kids.”  Basically, you have an open hole to the lungs.  Bacteria grows in moist areas.  So he had his nurse take a trach swab so they could run cultures on it and gave us Bactrim antibiotic.

As for the “trach removal” question, he was also very open to the concept.  We laid everything out on the table including her breath-holding spells, Gaucher disease progression, etc., and he still thinks it is okay to remove it.  However, he does not wish to do it until Spring, as he is concerned about Hannah getting sick during the winter flu & RSV season and needing to be oxygenated.  Not saying that Hannah is going to get sick this winter, but more of a “what if” and a “better safe than sorry” concept.   But in the meantime, he will be working with our new ENT on the steps needed to make sure Hannah can be decannulated safely (he wants to do a capped-trach sleep study as well as the other things the ENT mentioned).

So we have to wait another 5 or 6 months to hopefully get the trach out.  I’m bummed, but obviously it is probably not in Hannah’s best interest of safety to have it done sooner than later.   I’m just concerned at how Hannah is going to do during the next few months.

In the meantime, he ordered us a real pulse oximeter and oxygen concentrator for the house.  He could not believe that we did not have oxygen at home (the old pulmonologist would not write for either the pulse ox or oxygen).

We could sure use the oxygen and pulse ox tonight.  Hannah is still really junky (although I am suctioning a bit less than last night), and her O2 sats are hovering in the low 90s and upper 80s because of all the congestion (based on our little hand-held pulse ox we purchased because we wanted something!).  Not enough to go to the ER with, but I will definitely be up through the night keeping an eye on her.

If all goes like the last time she had trachiitis, she should start feeling much better after her second day of antibiotics.   Hope so!

We had our 2-month followup with our local neurologist today.

It went exactly as I thought it would.  Then again, in all honesty, I went in there knowing exactly what was going on, what she was going to say, and what medication changes I think would work best for Hannah.

She put Hannah back on the Keppra at twice a day dosing (1.5 mL) instead of originally her three times a day dosing.  TCH docs originally put Hannah on the Keppra for seizure control, but it is also used as a third line of defense drug for movement disorders.  For Hannah, who has no seizure disorder thankfully, it was a natural process to wean her from that drug last month.  However, we noticed that her movement disorder really worsened once she was off the Keppra.

She kept Hannah’s Haldol and Klonopin doses the same.  Daddy and I don’t believe she is getting any benefit from the Haldol, but we agree with our neurologist that we need to see how Hannah does with the addition of the Keppra before we remove the Haldol.

As far as Hannah’s left eye nystagmus…yep, that is what it is.  Sadly, we learned that it will affect her vision in that eye because with the pupil shaking left-right, so does her vision.  That must be so dang annoying for her.  There is no treatment for nystagmus.  And in true “Hannah fashion,” our neurologist has never seen nystagmus in just ONE eye before — it is always both eyes affected.

We are going to take a video of Hannah’s eye movements and send it to Dr. Fitzgibbons, Hannah’s neuroopthalmologist at the NIH, to get his opinion on it.

The Keppra has one other benefit that Daddy and I personally like.  If we do get the trach out and Hannah has another one of her breath-holding spells when she throws a tantrum, at least she will have some line of defensive against having another seizure like she did back in May (that started the whole hospitalization, even though it turned out that was just a minor thing in retrospect!).

Friday is “Nakamura Day.”  He’s our new pulmonologist.  Talking about getting the trach out.   Keeping my fingers crossed that Hannah is stable enough to get it removed!  (Though it would not be tomorrow, but at least we could take the next steps!)

Sometimes I tell myself that I would be better off talking to a therapist about the things going through my head. But then I tell myself that I already know what the therapist would say. It is not that I need to talk things out, I just need other things to fall into place.

This past week, I have been going through a lot of issues that seem all be weighing me down — jealousy issues, frustration issues, disappointment issues, anger issues, fear issues, etc…major reality check, in what seems like, all areas of my life. These issues are taking the forefront in my mind, and it is really bringing me down. It isn’t just related to Hannah’s condition, although it is a good-sized portion of it. It seems to encompass so many different areas in my life. Much of it also sprouts from our financial instability at this time.

I don’t do the “why me” thing. It is more of the “I just don’t get why it is that…” thing. Similar, probably. But not exactly…at least in my mind.

Admittedly, I am just not a happy person these days.   Heck, I have not been a happy person for most of 2010.  There are certain things that I hope that once Daddy finds a job and we have an income that is more than our expenses that other things will come into a better light. There are also certain things I know that I will never understand “why” or “how come.”

I’m in my early 40s now, and my life is passing me by. I want to start to have fun again. I want to want to feel like a woman again (because right now, I am last on my list). I want to have some freedom to do the things that I love to do – scrapbooking, theatre, going to events. I want to have a job that I am truly passionate about and love to do, not one that I have to accept because we need the money. I want to have some sense of normalcy in my life. I want to not dwell and get depressed on why certain people are the way they are.

I want to be a better mom, better wife, better friend…a better me.

I want to be happy again.

I keep telling myself I need to “get over it” and move forward. But I feel hopelessly stuck — There are just too many things I need to work on. I feel like there are just too many strikes against me — some I brought on myself, some brought on from the outside. I know better too — but it is almost as if there are too many things to try and change that I don’t know where to start, so I do nothing.

I need life to cut me a break and give me some hope.   Give me some good news that will help get our family back on track.   Something that feels as if we are going in a positive direction finally.

I just need some help getting those first steps taken so I can move forward.