Tuesday, September 29, 2009

Strabismus Correction

Alyssa's eyes began turning in when she was 4 months old. She was prescribed and began wearing glasses when she was about 7 months. We have noticed with each bit of assistance improvement in her ability to interact not only visually but socially as well.

She had surgery yesterday on her right eye ( eye muscle correction). Her right eye was the strong eye, until her left eye was corrected in April of 2008. After April gradually her right eye began to turn in more. We wanted to correct her strabismus (crossed- eyes) to help her get the most out of her vision field.

The surgery went well. We can see a big difference already. We'll post after surgery pictures soon. Alyssa handled sedation like a champ! (whew) She is now resting comfortably on the couch. We expect that the redness and discomfort to decrease and resolve within in a week. Until then we will spoil her more than usual with hugs, kisses and cuddles.

Monday, September 21, 2009

August had been busy...

aAbove is a picture of Alyssa receiving a bolus feed for her Gastric Emptying Study. We decided to have this study performed because she began vomiting routinely after her feeds since having the G-tube. We opted for her not to have a Nissen fundoplication (This surgical procedure tightens the valve between the esophagus [canal connecting the mouth and stomach] and your baby’s stomach) when her G-tube was placed. Alyssa has mild to moderate reflux. Our hope: with proper medication management and flexible feeding rates we'll be able to better control her vomiting. We learned from the study that the rate in which Alyssa's stomach empties is within the normal range. Using this information we have made adjustments to how we feed (the rate, timing intervals etc.) Alyssa's vomiting is decreasing, I also think part of the vomiting is due to muscle weakness. There are times that a good sneeze /cough while going "poo" will bring on vomiting, to much pressure in the belly for her weak muscles to control or hold in all in. (???) It's a day to day battle. Alyssa eats some food by mouth, in which she tolerates well. Hopefully this will increase. Alyssa currently weights 17lbs and 13 ounces; she is 25 inches tall.

Alyssa saying "hello" to Dr. L prior to her MRI and CT scans. Dr. L an anesthesiologist was able to intubate Alyssa on he first go! Considering her small airway this was awesome. The less attempts means less trauma to her airway and less irritation after waking up. For her last sedation by another doctor, he made multiple attempts before deciding to go the way of a lighted fiber optic scope.

Sedation is a scary thing for us, when thinking of Alyssa's health concerns. Alyssa has had one other sedation experience and she did well, however we can't take that for granted. This time she recovered well too. My one major complaint was for the after care team. We felt as though they were rushing to get us on our way. In other words ready to send Alyssa home about 30-35 minutes after the scans, even though her respiration stats were all over the place. We had to stand our ground in waiting it out for Alyssa's respiration to return to her "normal". We use an apnea monitor and Pulse Ox machine for Alyssa, so we are aware of what her typical respiration patterns when awake and asleep, sick with a cold and well (w/T18). We know the settings, so when at the hospital her stats intermittently dipped and remained low then perked up~ they said something along the lines of oh you probably never noticed but she has slight apnea spells` don't worry, she recovers quickly...( but on at least 2 occasions it wasn't so quick). Our response "NO" Alyssa thankfully hasn't had a registered apnea episode since bringing her home last July, we know her normal and what was showing up after the studies was not it ( by the way her normal is the same for other babes her weight/size).

So she remained a little longer in recovery then typical and her stats became consistent. Could you imagine how tense/fear-filled our 1 hour drive home (longer w/traffic) would have been if we hadn't stood our ground? It sounds easy to do, but even for the most confident of people I don't think it always is; I'm just glad we were able to this time.

Below: Alyssa resting...slowly waking after the studies.
Alyssa was sent home on hospice care seven days after her birth. By three months her first hospice provider was ready to pull out because she was thriving. Also it was suggested by those reading her apnea reports to discontinue her apnea monitor because she hadn't had any episodes. Yes, this sounds great but imagine our frame of mind...most of her doctors were still predicting that she would soon pass; even her very supportive geneticist cautioned us saying that Alyssa wasn't yet "over the hump". Alyssa's geneticist strongly suggested that central apnea could occur at any moment and the chances of so didn't decrease until the one year mark. So we retained the apnea monitor with the support of her pediatrician and went with another hospice provider (that didn't frown on us working with Early Intervention as well). Now at 14 months old, Alyssa has been off hospice for five months. We still use the pulse ox and apnea monitor. Below: Alyssa during her sleep study.
Oh, what a night this was. She didn't seem to mind (much) the leads, wires or even the head wrap. She was a good sport until the nasal cannula! That was too much and she howled like I never heard her before. She got so worked up that she vomited and we had to redo all the dressings. Through the night she would rest well, then seemingly remember where she was and start crying all over again.
The sleep study was ordered by her Pulmonary doctor. Alyssa has a diaphragmatic eventration, snores a bit and again with T18 the apnea concerns the study would give us good information (hopefully). We are still awaiting results from the study.
One appointment we were able to cancel was to the pediatric dentist for the casting of infantile dentures. JUST JOKING!!! This is a running joke in our household as our kids teeth rather late. Alyssa takes 3rd place (out of 4) as her rice grains popped up by the end of her 13 months. Can you see them? Yes two teeth came up and her gums are swollen, so as with her older sibs' more teeth may come in quickly. You know, I wasn't sure if I was going to see our girl with a toothy smile. Even though they are just peeking through it is a welcomed milestone!!
Below: Dr. A holding Alyssa. He's meeting her for the 1st time since delivering her last July. Alyssa seemed to remember him. She was talking his ear off, I suppose updating him on what's been going on this last year? When taking the picture I think Dr. A obliged because he remembered delivering her but I don't think her fully remembered all the events of that night. Not until after putting her down (commenting how chunky she is) and taking a glimpse of her chart. His back turned to me, he did a quick half step and said something like ..ugh given her diagnosis this is something hugh? I just smiled and said "yeah." He then took another look at Alyssa smiled asked her age again and upon my response slowly shook his head side to side with a broad smile.
In addition to the above appointments: Alyssa had a repeat visit to the Cardiologist, an annual re-visit to confirm she is cleared cardiac wise. After a brief scare/miscommunication and a repeat echo; we know she does have an innocent heart murmur and is otherwise heart healthy. Dr. K. a surgeon was consulted for her diaphragm issues and she was given the all clear. At this time surgery is not needed. She was also seen in the seating clinic at CHOP, for a feeding chair, stroller and bath chair. She was re-evaluated by her eye doctor and Alyssa is scheduled for surgery. She will have her right eye strabismus correction. This was her strong eye, but after correcting her left eye; the right is not turning in. She was seen by her nutritionist too~ whom would like to see more weight gain ( we are trying). Alyssa still has stenotic (small) ear canals, so we are going to try hearing aides. We waited it out, as this was suggested back in May (?) but we know Alyssa can hear (she does have some loss per her ABR) and wanted to give her canals time to grow. Since they haven't and she is making sounds, is interested in the world around her we want to further encourage this so we will try the hearing aides. Wouldn't it be neat if the mix of the hearing aides and some speech therapy would increase Alyssa's verbal skills? Her ENT visit brought a close to Alyssa's August appointments; told ya' a busy August!

Tuesday, September 15, 2009

Catching Up

Alyssa has been changing leaps and bounds in our eyes. Lately I've been so focused on the sad and difficult part of T18. It is time to catch up on the things that bring joy and encouragement in this journey. We are so thankful that Alyssa is enjoying being apart of our family as much (we hope) as we enjoy her.

The bright spots in our day come when she does her dastardly dog laugh (ya know~the raspy hehehehe) when tickled or if she thinks she's going to be. Yes she is anticipating the actions of others, when lying on her back she'll pull her right leg in and crosses her arms over her chest to protect herself from prying fingers, all the while smiling a wide open smile laughing. At night when the house is still, she'll babble and coo, we've had many late night conversations. I love staying up just to hear the sounds I was told would not come. Alyssa loves to play with her toy bar, batting, holding, trying to suck on the hanging toys. She sits/lays in various positions to access the toys to encourage skill development.

According to Parenting.com The 9 major physical milestones are :

  • Smiling (8 weeks): Alyssa was on target with this. She surprised her geneticist, when flashing her a smile at an appointment.Just to confirm it was a social smile and not an "accident" Alyssa did it several times in response to Dr. S.
  • Rolling over (2 or 3 months) Flipping from back to front often takes until around 5 months because it requires more coordination and strength. Alyssa began rolling to one side at about 4 months and to both sides by 7 months. Her roll to the left is stronger. She is not flipping back to from yet. She requires guidance to get a full roll, this is just the placement of her arm so it doesn't get "stuck" under her body. She is still learning how to tuck properly and not to arch incorrectly. She does love to roll, we work on it often for this reason.
  • Grabbing (3 or 4 months) Alyssa began grabbing about 6 months. However wasn't truly consistent and focused until about 8 months. Again this is a skills she continues to develop as she favors one hand/side over the other.
  • Hugging (5 months) Alyssa was a snuggler from the day she was born, but hugging with intent...hmmm not yet. Due to her low tone it was hard to hold her upright facing my body without support. So she hugged me in this position whether she wanted to or not. In the last 2 months she has developed enough head control that I can hold her in this position without additional support, so maybe I'll get a natural hug real soon.
  • Playing peekaboo (6 months) Alyssa loves peak-a-boo. It has been such a good game for her. She anticipates the cause and effect actions of the game. It'll be more fun for her as she develops skills to take the game to the next level( sounds like I'm talking pro ball).
  • Sitting up (8 months) Alyssa is beginning to sit unsupported. She can do so briefly intervals of 45-50 seconds between repositioning. In other words she sits, she collapses, is helped to gain good positioning and repeats until tired. This is wonderful to see, as once again we were told this or much of the other skills she has gained were not to be expected.

Alyssa's physical skill development is in the range of a 4-7 month old~very scattered. Some of this is due to muscle weakness, it is difficult for her work against gravity. When things are modified she is able to do more in one position than in another. We've noticed that with weight gain, there is skill gain. As we address her vision issues positive changes occur as well.

Alyssa's social development my guess is around 7 months. Her speech development is delayed which may be related to hearing issues. We'll see how this turns out as we address her hearing. Alyssa loves to watch people, smiles, mimics, and likes bright colors. I have been playing her a mix CD of Classical music and baby babbling/cooing ( we made it ourselves). The hope: to inspire language. Who knows the true benefit, but she does enjoy listening to it and will babble back to her recorded "friend". In the last few days she has begun to babble more to people outside of our household, it is nice to see her connecting to the world around her.

Alyssa receives physical therapy and occupational therapy each 4 times a month. She also has specialized instruction (learns how to play) 2 times a month . We would like to add speech therapy. Crawling (6 to 10 months) Pulling up (8 months) Walking (10 to 18 months) Alyssa at 14 months is not doing any of the above now..but ya never know~ it could happen someday. Parenting.com indicates that there is a 2 months window in either direction for skill development. If the child is beyond that 2 month period, she would be considered delayed. So yes, Alyssa is delayed. I do see her making changes/gains everyday and that is what matters most to us. Alyssa is 14 months old and delayed as much as a year behind her true age. Yet she is happy, interactive and much loved. Not bad in our opinion.

Why then do we do what we do?
Easy because we love her and like anyone of our other kids we want her to aspire to her full potential ~ whatever that maybe. I am confident that we will know when enough is enough. Alyssa has a fiery personality and I have no doubt that she won't hesitate to let us know her limits.

Monday, September 14, 2009

Showing off

A 6 minute video of Alyssa during various PT and OT sessions from the summer. Alyssa is steadily making progress. More updates to come!!

Thursday, September 10, 2009

First Day of Preschool

Today is my older daughter's first day of preschool. Wow, she's on the younger side; a mere 3 years old but we thought it might be good for her considering our current lifestyle. I am sure she'll come to really enjoy it, she loves learning and is a social butterfly. No tears this morning for the drop off, quiet an accomplishment when this is her first school experience aside from Sunday school.

I said no tears right? Well not on her end nor on mine until I got home. I think is was a long overdue watershed. A part of me crying because she's growing up but there was more emotion connected with it. I felt somewhat caught off guard, heck really off guard by it. The first few days/weeks after Alyssa's birth I cried A LOT. My body and/or minds way to adjusting to things. Seriously I cried at the mention of simple things like a word~"blanket"~ ooohh Alyssa cuddled in the same blanket as "X"~ and the tears came. After a few weeks of this I was fed up with the crying and willed myself to keep a stiff upper lip.

I was still emotionally connected but wanted to face the world with dry eyes and a measure of resolve. Faith directed resolve but with less or hopefully no tears. It worked too. There was the oddball ocassion like, Alyssa coming out of surgery A-okay and Celebrating Alyssa's first birthday that I did cry but then who wouldn't?

Today though I did one of those gut wrenching balled on the bed, can't catch my breath either pass out or gasp for more air cries. You know the kind that rocks your body and soul to the core. It was a good 10-15 minute water shed; and then I stopped wiped my eyes and tried to decide what to do with the rest of the "me" remaining before having to go pick up my girl.

In a small home with 6 people, there isn't a lot space for alone time; let alone time for alone time. Never would I want my kids to see me that vulnerable. So now I get the merit of Preschool. My mom's guilt for sending my oldest "young" daughter out in to the wild lands of Christian Preschool has diminished.

Bottom line even though I didn't want to acknowledge it: I needed a good cry. By the by Alyssa slept soundly the entire time. Thank God for the big and small things~

Thursday, September 3, 2009

Inspired by the Botts Family

"Better, far better, with Christ to be, living and loved through eternity."~Poet Unkown ~

Right now I'm driving down the T18 highway in the backseat of a friends car...Brenda Botts. Gods at the wheel and Brenda along w/Bri (Jerry and Ashley) are alongside in the passenger seat. The view from the back is alittle obstructed but from back here the road feels hilly and narrow and it's hard to see where we're being led.

I'm feeling motion sickness and wondering how I can get comfortable. You can see this isn't the most comfortable ride for them as a family, but they are doing what they can to make the most out it. The Botts family, they are enjoying their togetherness. They are glad to have a trusty car, and a reliable driver. They're happy that they can focus on what's happening in the moment and allow their driver to worry about what is ahead. They aren't even wearing seat beats! Trusting that God will get them to their destination safely.

Me, here in the back.. I want to lean forward and strap them in. I want to ask the driver to slow down, or stop for bit so I can catch my breath. But I'm just along for the ride, a stow away waiting for the rise of the next hill, dreading the decline..wondering if there will be a clear view of what's ahead.

The Botts family have been kind in letting me stow away here in the backseat, they share bits of their front seat treasures with me. They share their morsels of food (encouragement) and fresh water (faith in a living God), even their medicine ( big doses of T18 reality) willingly. Being back here on this trip is helping me to prepare for when it's my(our) turn in the front seat.

How blessed I am that God is sustaining the Botts family so much so that they are willing to share their experience. How blessed are they to have such a loving and compassionate God. Brenda and her family are teaching me, and are examples of how God does work within. God is good. Even when the end is near He is good.

Yesterday Brenda wrote on Brianna's blog: "Pray, pray, pray for comfort for Brianna. The time is near, very near. I guess from this posting you can tell the doctor appt. didn't go very well. Her heart has deteriorated rapidly. He said she has less than 14 days to live, that's two weeks, less than 336 hours, less than 20,160 minutes.....and that was earlier today. Followed today with in part:Bri is still with us. We are all wiped out. Last night was so bad that we were praying for Jesus to take her in his arms. There was no comforting her, even with medication. But once again our God showed mercy and opened up a room at the Hospice House (less than 5 miles down the road)...

As I posted days ago on our wndrfllymdeblog :
Last year I frantically looked up T18 to get answers. The doctors hadn't given us the official diagnosis for Alyssa yet, it was by a chance that I gleaned this was something they were looking into...after coming to honor page after honor page of precious babies held in memorial I found Brianna Botts. A baby girl (16 month old) living with T18! I watched the news clip of the Botts family story, saw the pictures of this cute little girl and thought "wow, look at that miracle." I contacted Brenda right away, and in what I've learned is her innate kind way..she reached out to me immediately. Brenda has guided me along this journey with a mixture of zeal and restraint, always sharing the realities of T18 with a gentle candor, for this I am always grateful.

Brianna does give hope, she gave it to me in the wee morning hours on July 16th 2008. Brianna has overcome much in her life and there is no one on this earth to say she will not overcome more. If you've made it this far in my rambling then I ask for you to fervently pray for Brianna and her family..pray for their hearts, spirits and minds to be graciously prepared for what the Lord has in store for Brianna. Hope remains and I pray that God who has been merciful to the Botts family will continue to sustain them."

It's a week past that post entry and God has been good to the Botts family to Brianna. I share this all becuase I need to...I can't tell you what Brenda's favorite color is or how she takes her coffee. Yet I feel we share a bond of friendship, and I am grieving with her, Jerry and Ashley.

God commands Christians to join in the suffering of others :
"Weep with those who weep" Romans 12:15

You have done that for our family and now I am asking you to uphold the Botts family. Pray as they have requested, or visit Brianna's site briannagiveshope.blogspot.com and leave an encouraging message. I know they would appreciate your prayers. Sadly the reality of T18 is now being seen...Brianna at 2 years was thriving only months ago and now is preparing to be with Christ. The sadness is not for where Bri is going it is for those left behind and their missing their beautiful spirited loving girl/sister.