2007 Updates

JJ's Journey





December 2007 - 3 Years, 1 month     

December 31, 2007 - If it's not one thing, itís another.  Poor Jenna - she just can't "get better."  Over the weekend, she had some nasty nasal congestion and was placed on another antibiotic for a sinus infection.  So she is getting better with that and now today I had to pull feeds to 1/2 strength again.  I think the GI issues were starting to brew last week as she hasn't slept well since Wednesday night (today is Monday).  Her tummy was a bit distended yesterday and today it is totally distended.  She was crying because of it and it was pretty bad.  So unfortunately, we have to start all over again with her feeds.  Due to all the GI issues she was having at the hospital, she lost 1.8 lbs.  I guess one good thing about being overweight is that she can afford to loose that weight.  Now that she is on half feeds again, I guess we'll see what happens. 
Sending more get well wishes for Kyle and Emily as they are still in the hospital and still intubated.  My thoughts and prayers are with them and their families.

December 27, 2007 - The last couple of days, Jenna is doing better, a little better yesterday and a little more today!  She was off her bipap most of the day except for respiratory treatments and nap.  That is an improvement all in itself.  She still has a lot of what we think is nasal congestion, but her sats are generally good.

Get well wishes and many prayers going out to Emily and Kyle who are also sick with RSV.  And thoughts of little Matthew on his birthday/angel day.

December 23, 2007 - Jenna is home!  She is still pretty sick and needs total bedside care, but we figured they weren't doing anything more at the hospital than what we do at home so it was time to go before she catches anything else.  She still requires bipap and can only take a break of 1 1/2 hours at a time.  She still has A LOT of junk to get out and we are constantly suctioning, coughing, nebs, etc.  Tonight she is running a low grade fever of 100.3 and her heart rate is slightly higher than it was yesterday.  Even though she is still sick and requires a lot of work, it is so nice that she is home and we can all be together again. 

Thank you so much for all your thoughts and prayers.  They are much appreciated! 
Hope you have a wonderful holiday!

December 18, 2007 - @ 8:30 pm - Yep, Jenna was positive for RSV! And C-Diff too! Nice, huh? You go into the hospital to fix one problem and you come back with two more!  Thank God she had her two synagis shots already this year. The insurance company originally denied us and we had to fight for it. I don't know how she would have made it out of it without the shots. She is definitely on the down slope which is good, because I was really, really scared that she might not get thru it the other day. We have been weaning the 02 over night and she should be off sometime today. Her tummy is much softer and I guess we'll wait until rounds to see if we are going to attempt the feeds.

December 18, 2007 @ 11:35 am - Jenna had a real tough day yesterday. It was weird, the morning seemed better than the day before, but then by afternoon not so good.  She had extreme tachycardia with a heart rate of 180s, when sort of sleeping, it was in the 160s.  She was laboring and it took every ounce of energy she had to breathe, to a point she was shaking the bed.  This is with her bipap on and 3 liters of o2.  She spiked 104 temp and they ordered another chest xray and one of the abdomen as she was also so distended.  This xray wasn't as nice as the prior one.  It showed atelectasis and they are saying a possible pneumonia as we await respiratory cultures.  The abdomen xray showed an ileius (paralysis) of the bowel so feeds have been stopped again and she was put back on TPN for nutrition.  We are doing aggressive respiratory treatments and we will repeat both xrays tomorrow morning.  Today, she is a little more comfortable with a heart rate of 150s.

Prayers going out to all the other sick kiddos right now!  Get better soon!

Thanks MJ, for keeping our website up to date.  We really appreciate it! 


December 16, 2007 @ 10:45am - Good news is Jenna's xray looks good, no pneumonia or anything.  Bad news is she is still having problems.  Her heart rate is 170s-180s and all she does is cry.  I ended up talking the docs into giving her chlorahydrate (sp?) as she had this before without incidents.  We have been giving her around the clock pain meds and that doesn't help.  She does not have a fever and she is acting like she is just too tired to keep her breathing/sats up.  She is still on bipap, 5 liters o2 and her sats are 90-91.  Paul arranged for the girls to be with family and made it up here.  Because of the snow, I am having a hard time reaching an outside line for my laptop, but right now I am in the "family room" which has a PC, while Paul is with Jenna.  I will try to update when I can.


December 15, 2007 @ 7:30pm - Jenna started labored breathing this afternoon and was very irritable, high heart rate 150-160.  Her RR was jumping due to her tugging to breathe. We ended up doing an Albuterol neb, CPT and starting the cough assist at 20/20. Afterward, she started flowing and it was nonstop suctioning.  She continued to be irritable/constant crying and labored breathing, HR now 170-185 and sat fluctuating 93-97 and respiratory rate jumping due to her laboring.  We ended up coughing again at 30/30 after touching base with Dr. Swoboda.  We really didn't get any more junk up and she continued with the irritability/crying. The fellow in the PICU thinks it is due to pain. The resident said to give her Morphine, which concern us as we all know it can depress her respirations.  We started with a low dose (1/3 dose for her body weight).  It has calmed her down and she is sleeping - HR 150, RR 40, o2 93%.  She has briefly awoken with some whining, but then drifts back
off. We are waiting to hear back from Dr. Swoboda on if there are any more recommendations.  Please continue to keep Jenna in your thoughts and prayers.


December 15, 2007 @ 12:30pm - It is 12:30 east coast time and Jenna is doing fine.  Satting around 96 to 97 all night and this morning.  She has been good without the cough assist until now.  We are 3 days out now from surgery so we are going to start coughing her at 20/20 this afternoon and get her off bi-pap for small stretches at a time.  It is amazing how touchy a t and a surgery could be for our kids, but the plan for her so far has worked.


December 14, 2007 @ 8:45 pm - This afternoon Jenna started satting in the low 90s on bipap.  You can hear the junk that needs to be coughed out, but unfortunately, we shouldn't because of her surgery.  Dr. Swoboda suggests waiting 2-3 days before using the Cough Assist as it can cause bleeding and we certainly don't want that.  On the other hand, we don't want the junk sitting in her lungs and causing a possible pneumonia either.  So we tried some albuterol and at first it seemed to make her more junking as it breaks up the stuff in the lungs.  After we continued with CPT and light suctioning (can't go down the nose or too far back in the mouth due to bleeding), she eventually went back up to the higher 90's.  Right now she is hanging out around 95 which is fine.  My hopes is that she stays around here or higher so we don't have to break out the Cough Assist and risk bleeding.  If she can just hold off until tomorrow afternoon, it will be best.


December 14, 2007 @ 11:25 am - Well, it has been a long week! Jenna had her tonsillectomy and adenoidectomy on Wednesday. They had trouble intubating her because her mouth doesn't open very well, but they used a special
tube and got it in.  She did very well with surgery. She was finally extubated last night after a long day. She had some bladder and bowel issues that 
needed to be resolved and after stopping the Fentynal, she really didn't want to wake up. She was extubated to continual bipap with 4 liters of
o2, but is doing so much better this morning. o2 is down to 1 liter and her sats are staying 95%-98%, which is great. I am sure she will be off the o2
very soon. We are still unable to cough her so we are working on Chest PT, suctioning, and postural drainage. It looks like we will be there all 
weekend and our guess is for discharge on Monday.  Thank you for all the thoughts and prayers!

December 10, 2007 - First of all I would like to say "Thank You" to everyone for putting the benefit together, those who donated, and all who attended!  We had a great turnout and appreciate everyone's support.  It truly meant a lot to our family.  Thanks again!

Well Jenna's surgery is drawing closer.  She will be admitted tomorrow around noon and surgery is scheduled for Wednesday at 11:00 am.  I just spoke to Jenna's pulmonologist who just spoke to Dr. Swoboda.  Our plan is to have Jenna intubated for at least the first 24 hours then extubated to continual bipap for 2-3 days.  So she will be in the PICU at least until then.  My guess is she won't be discharged until Monday.  Please keep Jenna in your thoughts and prayers as she gets thru surgery, hopefully without incidents. 

Thank you all for your thoughts, prayers, and support!





November 2007 - 3 YEARS OLD!!          Happy Birthday

November 28, 2007 - As usual, it has been a busy week or so around here.  We had Thanksgiving dinner here and it turned out great.  Jenna got to sit at the table with us and "eat" : )  fruit and rolls.  She loved it!  Then the weekend was pretty busy too.  We went to the Festival of Trees at the Adams Mark Hotel, which was beautiful.  The trees will be auctioned off to raise money for Womens and Childrens Hospital of Buffalo.  Saturday we put up our Christmas tree and Sunday the girls had the pictures taken for Christmas. 
Also, some BIG news!  We purchased an adapted potty chair for Jenna thru another SMA family and Jenna is doing EXCELLENT!  She is keeping her diaper dry all day, except for naps.  We are so proud of her and she is of herself.  It is great. 
Jenna is now scheduled for her tonsillectomy and adenoidectomy on December 12.  Although this is usually a minor surgery, it will be a major one for Jenna.  The doctors have been great about following the SMA protocols and making arrangements.  She will be admitted on Dec 11 and TPN (Total Parenteral Nutrition) started that evening as our kids have Fatty Oxidation issues and cannot fast for a length of time.  She may end up needing to be intubated for a few days due to swelling and it may be a week stay.  Hopefully she will fly thru it without issues and we will all be home sooner. 
Get well wishes going out to Jacob and Skylar S.  Get well soon!
Our thoughts and prayers go out to the families of Anna and Evan, SMA children who recently earned their wings. 

November 19, 2007-  Jenna saw the ENT today.  He said her tonsils and adenoids definitely need to come out.  He is looking to get this done soon, after coordinating with pulmonology and anesthesia.  We gave him the proper SMA protocols for nutrition and pre and post op care.  Paul talked to the Pulmo today and he is going to follow-up with the pediatrician on these protocols as well seeing she may be admitted under them.  The ENT did tell us that there is a possibility that she be intubated for a couple days if there is a lot of swelling.  At least we know that up front and don't find out when/if it happens.  Recovery time is about 2 weeks.  This is pretty scary as any surgery for SMA kids is a big deal.  Please keep Jenna in your thoughts and prayers as we work toward and get thru surgery. 


November 15, 2007 - All in all, Jenna is doing fine.  Her tonsils are still swollen, her throat is sore, and she has a little bit of a stuffy nose, but other than that she is fine.  She has an appointment with the ENT on Monday, so I guess we'll see how things go.  We also have an appointment tomorrow with the Orthotist.  We are looking into getting new AFO's (well DAFOs) as her heels keep popping out of her current ones.  We are also looking into knee splints has her hamstrings are now contracted.  The TLSO vest is working great.  She actually "likes" to wear it!  We also have a rep from EyeGaze coming this weekend.  I can't wait to see how things go.  This is a communication device that Jenna can activate by using just her eyes!  Next week we are looking into the Quick Glance system, which is a similar device.  Busy, busy, busy!

Many prayers going out to all of the sick kiddos and of course to Grandma Gullo, who is in the hospital.  We love you Grandma!


November 8, 2007 -  Jenna had a wonderful birthday party on Sunday.  She had a Barbie theme and just loved all the stuff that went along:  goody bags, tattoos, etc.  She even had the chance to "taste" her birthday cake.  It didn't go so well, but she was thrilled!  Even now if you ask her if she had a nice birthday, she says "cake!"   So cute.

Well we finally received the results to Jenna's sleep study.  It showed 3 episodes of apnea and 10 episodes of hypopneas.  So.... I guess her tonsils are getting in the way of her breathing and sleeping.  She will go to the pediatrician tomorrow for a well-visit and RSV shot and we will be asking for a referral for an ENT.  I guess we will just see how that goes. 
Many prayers going out to the sick kiddos:  Ally, TJ, Miles, Emma, Errine, Logan, and any others I may have forgotten to mention.



November 2, 2007 - Happy 3rd Birthday Babydoll!  Today is your birthday and we wish you all the love, joy and happiness for your special day!  We are so proud of you, all that you have accomplished and gained in the past three years.  It is amazing how you continue to beat the odds, and although you are so weak from your disease, you are oh so strong in so many ways.  We love you babydoll ~ Happy Birthday!

We went to have Jenna's pictures taken this morning and they all turned out so cute!  Unfortunately, we have to wait to pick them up until the 13th.  She already opened some presents from Ms. Valerie and Ms. Chris.  I also bought her some big balloons today, which she loved.  Sunday, we are having a big Barbie birthday party for her with all the family and cousins.  It should be fun. 
Halloween was good.  Jenna was Cinderella, Kali was a Flower Child, and Mya was a Hollywood Starlet.  We took Jenna to the mall for Trick-or-Treating on Tuesday and it was nice for her to see the other kids in their costumes.  She sat with me at the door on Halloween night to help pass out candy.  She loved seeing the kids come to the door dressed up and when they would leave, she would call out, "KIDS!  KIDS!"  Too cute!
Also, I just wanted to add "Happy 3rd Birthday to you too, Mr. Kyle!"  Love Ya!


October 2007 - 2 years, 11 months

October 19, 2007 -  We finally got to the bottom of the Mic-Key button problem!  It was too small!  We saw surgery today and they resized her.  She had a 14 fr, 1.2 cm in and she needs a 14 fr, 1.7 cm!  That is a huge jump from the summer.  She went in for a bigger size in July and went from 1.0 to 1.2.  They didn't use the resizing tool then like they did today and I'll tell ya, lesson learned.  Whenever talking about changing the size, I will always insist on using the measuring guide!  She is very sore and it explains the tenderness and blood in her stomach.  They said it will take about a week to heal and will most likely have drainage and blood, poor thing!  I'm just glad she can finally start healing and feeling better.


October 16, 2007 -  Wow, it has been a long week!  I guess I will start from the beginning...

Last week Jenna was evaluated for Assistive Technology.  We saw the Dynavox and it was ok.  I personally don't think it is fast enough for Jenna as she is such a bright girl.  She was able to work it with different switches, but it just seems so slow.  You have to pick a group of items, then scan it to pick the word or phrase you want to say.  To me, that is just too slow.  So... we are going to have another company come in with an Eye Gaze system.  This is a device she looks at with her eyes and selects the item by blinking.  She tried this at the conference and did great.  So maybe this will be the one that works for her.  I guess we'll see.
Then Jenna had a big day in Rochester.  She had her c02 checked by a blood gas and everything was good.  She was numbed first and so she did really well with it.  Then she had an echo at the Children's Heart Center, then off to the sleep study that evening.  The results aren't in yet, but all and all things went well.  We were looking for any sleep apnea due to her (still) enlarged tonsils, but the RT said that would be hard to detect with a back up rate of 25 on her machine. So...  who know where we stand with the tonsils, but her bipap settings work well.
Saturday evening Jenna ended up with a low grade fever.  By Monday morning it was up to 103.8 and she was still complaining about her Mic-Key button (she has been complaining ever since I routinely changed it 2 weeks ago).  Thinking it was infected, I ended up taking her to the ER to have it checked out.  The NP looked at her Mic-Key button and stoma and everything looked fine.  After labs, urine culture, chest and abdomen x-rays, and a CT of the abdomen, we were told it was a virus and finally sent home after 8+ hours.  There was one point they thought she may have a bowel obstruction and may need surgery.  I was SO glad to find out that it wasn't, I forgot to ask what the whole Mic-Key button problem could be.   I guess after she is better, I will call surgery and have them look at it.
Today she is doing a bit better and we are keeping the fever down with around the clock Motrin. 
Many thoughts and prayers going out to all the sick kids.  You too, MJ!  Get better, out of the hospital, and back to college!! : )


October 6, 2007 - Jenna is doing much better.  She was on an antibiotic for 5 days and is really doing so much better.  I had her nurse look in her throat today and her tonsils have gone down since Tuesday too.  She said she can still see them, but they definitely have gone down. 

We had Jenna's CPSE meeting this week too.  It looks like her services will be increasing in January.  She will have 3 days/week for PT, 2 days/week Aquatic Therapy, 2 days/week OT, 3 days/week Speech, and a Preschool teacher will come to the house 3 days a week.  It seems like a lot of services, but they are only 1/2 hour each except for the teacher which is 1 hour.  The school district tried to push us to put her in a preschool setting, but we are not ready for that yet.  Maybe next year. 
Next week Jenna is being evaluated for Assistive Technology, to help her in communicating.  She tried the EyeGaze system at the FSMA Conference and she did GREAT with it.  She was all over the computer clicking on what was asked of her.  We are going to try to steer them to that system, but we'll see what happens.  I'm sure it is going to be a big process like everything else.
We received a call a couple weeks ago from a wonderful woman named Lisa, who will be running both the half marathon and marathon in January at Disney World.  Her and her friend Kevin would like to sponsor Jenna for this event.  They also want to throw a benefit for her next month.  Any money raised by these two events will go toward home renovations to make her an accessible bedroom and bathroom on the first floor.   Thank you Lisa and Kevin for the wonderful things you are doing for Jenna.  Your thoughtfulness and generosity is much appreciated!
Prayers going out to all the sick kids:  Sophia, Errine, Emma, Zoe, Malorie, Stella, Kaitlyn, Courtney, MJ, and anyone else I may have missed.  Hope you feel better real soon!


October 1, 2007 - Please add Jenna to your prayer list!  I had to take her to an appointment today in Rochester to meet the physician who will be doing her sleep study.  She fell asleep in her EasyS on the way home and must have aspirated (yes, she was laying flat on her back).  Her pulse ox was going off so we pulled over on the side of the thruway and did a few rounds of cough assist and suction.  I couldn't get her stable so I put her bipap on.  She was satting around 96-97 so we were off again trying to get home.  She started desating again on the way, so we had get off the thruway and pull into a truck stop.  Another few round of cough assist and suctioning, I was able to stabilize her to 95-97 on bipap again and we made it the rest of the way home.  Since being home, she started a high heart rate, temp, and high respiratory rate.  She sounds like she has junk in her front lobe and I called the pulmonologist.  She is on antibiotics now and on bipap.  RR is better, but still having some peaks and dips.  As of right now (with the help of Motrin), her temp is down too.  Her HR is still a little elevated, but that is to be expected.  We would appreciate it if you could keep her in your thoughts.



September 2007 - 2 years, 10 months

September 24, 2207 - Well, I took Jenna to the pediatrician today as this weekend she was so cranky and running a low grade fever.  I was afraid her tonsils were still swollen like they were a couple weeks ago.  Sure enough, her tonsils are quite swollen with blisters.  The pediatrician said this is a virus that is going around.  I guess between the sore throat, swollen tonsils, and the molar coming in, that would make anyone miserable!  Plus, it explains all the extra secretions she has been fighting lately too.  We meet Dr. Connolly in Rochester next week in regards to Jenna's sleep study that is coming up in October.  I will have her check Jenna's tonsils then to see if they are coming down at all.  If not, I guess another appointment to the pediatrician and a request to an ENT.  On the bright side of things, Jenna's GI issues seem to better (knock on wood).  She hasn't had anymore stomach distension and for the most part sleeping much better at night. 


September 20, 2007 - Sorry I haven't updated in a while and I can't believe September is half over already.  It has been a pretty busy month.  We went on vacation in August to Sherkston and the kids had a great time.  Jenna loved meeting Bradley Bear and his friends.  Then we had the crunch of preparing for the start of the school year.

Jenna has continued to have GI issues since the beginning of August.  It is suggested that she may have bacterial overgrowth so we are trying a couple things.  So far it seems to be helping not only with stomach distension and constipation, but with sleeping as well (knock on wood).  Other than that, she is doing well.  She just had her flu shot this week and we are working on getting the monthly RSV synergis again this year.  
Prayers going out to all the sick kiddos and those who have recently had surgery:  Kaitlyn, Julia, Sophia, Stella, Emma Elizabeth, and any others we may have missed. 



August 2007 - 2 years, 9 months

August 10, 2007 - Jenna is still not feeling well, although today seems a little better than yesterday. Two nights ago she ended up refluxing her meds (give by GT) and we think she aspirated. Thank God Mya was near her as she noticed right away and we were able to turn her to the side and help get it out of her mouth. It was definitely the medicine Paul had just given her. We think she aspirated some as her temperature went up and and her sats kept dropping that night. We started respiratory treatments every 4 hours. Ms. Chris, one of Jenna's nurses, heard some junk in her back lobe so she is now on an antibiotic. Maybe that's why today is a little better than yesterday.

Today is Jenna diagnosis anniversary. Two years today we were told our little girl had SMA. No treatment, no cure. What a terrible day. Today is also my birthday. It's funny, most people in their 30s get depressed on their birthday as they are "yet another year older". Not me, I could care less about the age. It's just depressing that Jenna has SMA and has to fight so hard to live. 

August is SMA Awareness month and the second Saturday, which is tomorrow, is the 4th Annual Candle Lighting for SMA Angels. Please light a candle at dusk and let it burn brightly in honor of those precious little ones who have gone on before us and those who continue to fight and live with SMA.


August 8, 2007 - Jenna isn't feeling well.  She started with a few extra secretions and a couple bad nights of sleep.  She woke up this morning with a cold.  She sounds nasally too.  So far it doesn't sound like it is in her chest, but her sats are averaging 96.  Some would say that is ok, but not for Jenna.  She usually runs 99-100.  I am not sucking too much out of her nose though either, which makes me think there is more to come.  And the poor thing is miserable!  I ended up canceling her therapies today.

On a lighter note, this past Saturday, we had our 4th Annual SMArt Walk for A Cure.  It was a beautiful day and we had a great turnout.  Official numbers aren't in yet, but we raised over $50,000!  We also had Katie Cooper and Tiffany Fogle from Kaydence.org come to visit.  They brought the SMA Angel Wings Awareness Quilt, which is beautiful!  Thanks for coming and a big thank you to everyone for your support!!



July 2007 - 2 years, 8 months

July 23, 2007 - Jenna is doing fine. She is sleeping better, so that is good! I talked to Brian Weaver at Bach's and he helped me with the settings and such.  We have to slowly go up on her IPAP as she has never been able to tolerate anything over 15. Maybe it has something to do with her other settings, maybe not. I guess we'll see.

One thing though, she cannot seem to handle much calcium. It is giving her heavy secretions and sounds all junky. Sometimes her sats drop because she can't handle them. So we backed off of it. We have tried this twice with the prescription (1250mg). When she didn't handle it the first time, we cut the dose in half and split that up to twice a day. She seemed to get junky again. So we went back to the 250 mg tablet we use to use before seeing the Endo. Unfortunately, this stinks because of her osteopenia.


July 16, 2007 - Jenna is doing fairly well.  We have been trying to increase her calcium, but we keep running into issues.  The first time was a high dose, gave her stomach issues and now I realize it gives her extra secretions as well.  We went off the high dose prescription Calcium Carbonate and now she is back on but we dropped the dose in half.  The secretions started again.  We are taking her off and I am going to give her the capsules of Calcium Citrate again, just a higher dose.  Hopefully her secretions will go down in another day or so.

Jenna is doing excellent in her power chair.  She has definitely shown improvement over the last couple weeks.  I will have to get a video clip of her and post it.  Yesterday, she drove all the way down the drive way and over to the neighbor's!  Then she wanted to see the dog two doors down, so once again, down the driveway and off she went!  It won't be long until she is zooming all over the place!

Prayers going out for Kyle, little Daniel at Primary, Achdon, and any other little ones under the weather!


July 8, 2007 - Well we figured out what Jenna's extra secretions were all about.  She has gotten so big over the last couple months that she outgrew her Robinul dose!  At first we thought maybe it was her allergies, but increasing the dose of Zyrtec didn't work.  Then, unfortunately, she had an episode at her cousin's birthday party where her sats dropped into the 60's.  We were able to get her back up to 99-100 after coughing and suctioning (quite a few times).  She wasn't sick, she was just pooling her secretions again.  It reminded us of the dreadful Nebraska disaster.  Same thing.  Once we cleaned her out, it was like nothing ever happened!  We increased her Robinul and everyday she seems to be doing better.


June 2007 - 2 years 7 months

June 28, 2007 - Wow!  June is just about over and I haven't updated yet!  It has just been a crazy, but good, month.  Lots going on with the girls and end-of-school-year stuff.  They both received excellent report cards and I am very proud of them!

All in all, Jenna is doing good.  She has some extra secretions the past week so we will keep an eye on that.  She is just growing like a weed!  She is SO tall and is in the 90% for height!  Big girl!

We went to the FSMA Conference in Chicago last week.  It was great.  We are so happy we went and looking forward to going again next year.  At least next year will be the East Coast, a little closer anyway.  The girls were all good, no issues, no problems.  They actually had a lot of fun.  All three enjoyed the pool, the kids room, crafts, and carnival.  Mya & Kali and a few friends did some fundraising for FSMA while we were there.  They made bracelets and neckties in the kids room and sold them to parents.  They raised almost $200!  How impressive!

We finally received our van back from being modified.  It is going to be real nice to transport Jenna in her chair.  Now to start thinking about home modifications. 

Oh, we also got Jenna's hand splints and AFO's this month too.  We are actually going to the Orthotist tomorrow for a TLSO vest. 

Well, hopefully next month I will be able to update a little more.  Just wanted to make sure we at least had an update for the month of June : ) !


May 2007 - 2 years, 6 months

May 14, 2007 - The last couple weeks have been pretty busy with Jenna, new dx of osteopenia, having dexa scans done to find out they are invalid due to the stone-age equipment used here in Buffalo (you would think we live in a small town or something).  We were going thru ups and downs with her blood sugar too.  It somehow evened itself out, so I guess that is good.  We just need to check it to make sure it still is good.  Jenna also got her first pair of AFOs.  They're pretty cute as they are purple with butterflies.  The hand splints were too big.  They tried to cut and modify, but Jenna's hands are just too small so they have to order a smaller size.  It seems like it has been never ending lately.  I'm just trying to catch-up with just about everything.
I am sending big hugs and lots of prayers to all the sick kiddos and those recovering from surgery:  Malorie, Charlie, Errine, MJ, Zoe, Hayden, TJ, and any other ones I may have missed.
And HAPPY (belated) MOTHER'S DAY to all the moms out there!



April 2007 - 2 years, 5 months

April 28, 2007 - Yesterday was a very busy day.  Jenna had a GI appointment in Batavia.  She did real well and we are now trying a couple new meds for the reflux issues.  We will be adding Zantac (while still taking Prevacid and Reglan) every night and Gaviscon for acidity issues.  Hopefully these will work and she will sleep well again.  Also, Jenna hurt her leg in her stander on Thursday and when I touched it while changing her diaper yesterday morning she winced pretty good.  So we went for a few x-rays.  Good news is there was no fracture, bad news is she had osteopenia (thinning bones).  Her pediatrician is referring her to an Endocrinologist.  I assume she will be put on Calcium and Vitamin D supplements.

Also, yesterday was Mya's birthday, HAPPY BIRTHDAY MYA!!  She turned the big 8.  Can't believe it has been 8 years.  Wow has time gone by!  We went to dinner then Dave & Buster's for fun & games.  The girls had a great time.

Just want to wish all the sick kiddos out there get well wishes and hugs!  And you, Mr. Kyle, I'm watching you!  I know how you and Jenna like to stick together when sick!  Get well soon buddy!


April 24, 2007 - All in all, Jenna is doing well.  Her respiratory issues are under control and doing great.  She still is refluxing a bit at night and lately she isn't sleeping well.  We have another GI appointment on Friday, so we'll see what happens then.

Today is another sad day.  Little Miss Kalair earned her angel wings this morning.  Kalair was 7 years old with Type 1.  My thoughts and prayers are with Liz and family!


April 8, 2007 - First of all I would like to wish everyone a Happy Easter.  I can say that now as we are home from the hospital!  Jenna has been making progress everyday and we talked the ICU docs into discharging us to home yesterday afternoon.  They wanted to watch her for at least one more day, but she was satting 95% off bipap awake (lower when sleeping and on bipap) and the only thing they could do for us that we couldn't do at home was an xray.  And for being discharged to a floor, was out of the question!  So.. we ended up coming home!  This was the worst sickness Jenna has ever had.  It was very difficult to see her go through this and we are just so thankful she is home. 



April 5, 2007 - Just came home to get a couple things done and some rest.  Paul is with Jenna as she is still in the PICU.  She is doing a little better as she is off o2 and she has had a couple breaks off the bipap (1 hour at a time) and maintaining 95% while awake.  She is having issues with lying on either side now even with bipap on as her sats drop (anywhere from 73-89%).  The respiratory culture came back negative and they are calling it a cold.  She now has a partial collapse of one of the upper lobes, can't remember which one.  She is thick.  It is clear and white, but extremely thick.  You could pretty much just suction her continuously.  Respiratory treatments are still the same.  We are slightly drugging her to calm her down and get some sleep as she is pretty miserable and just cries.  The drugs worked well and she was sleeping and maintaining 91-95% when I left.  I am trying to remain positive as she is a little better than yesterday, I just hate to see her like this. 

Sorry I haven't updated sooner, but we do not have dial-up on our laptop and I couldn't get online.  I found out today that there is an ethernet plug in the parents room so I may try that tomorrow as long Paul is around.  I do not feel comfortable leaving her room without one of us being right there as I just left the room to go to the bathroom and when I came out, her alarms were going off and the nurse was running in as her sats dropped again.  I will try to keep everyone updated when I can.
Thank you so much for all the thoughts and prayers!


April 3, 2007 - Well Jenna was admitted to the PICU yesterday.  She was dx on Sunday at Urgent Care with early bronchitis and given an antibiotic.  We were able to control everything up until yesterday early afternoon.  Her sats kept falling and hovering 91-93% while on bipap.  We did treatments every 2 hours with Albuterol nebs every 4.  We were constantly suctioning and had to keep her in postural drainage.  After a little while, even that wasn't keeping her sats up.  So we surrendered and started o2.  Even on that, her sats continued to dip into the 80s and were bouncing all over the place.  We arrived at the ER and almost immediately admitted into the PICU.  She just couldn't keep her sats up with 3 liters of o2. Unfortunately, we still don't quite know what is going on.  Her chest x-rays continue to look "ok", no pneumonia, no major plugs, just some secretions.  They collected a respiratory culture this morning to check for RSV (and yes, she has been getting her shots each month).  It may be viral.  For the first time, we heard the talk about intubation and trachs.  This was very scary.  Of course we immediately paged Dr. Swoboda.  We wanted to make sure we knew what to look for etc.  Jenna's pulmo is pretty good and follows Dr. Schroth, but we still wanted to "be prepared" as it was the PICU physician talking and not him.  As of right now she is not intubated.  They raised her o2 and she has been working real hard with that and continuous bipap.  Her Respiratory Rate has been jumping all over the place.   Normally, she runs about 22-25 awake, and she is jumping from 17 to 65 to 30 to 26 to 69, etc and she cannot sleep.  She has not slept since waking Monday morning (and neither have I).  She cries a lot, cannot suck her thumb due to positioning and an IV line.  We have tried to place an IV in several different places to make her more comfortable, but we run into the same ol' problem of collapsing veins.  She would be able to lay on her left side and relax sucking that thumb, but she cannot maintain sats in that position.  She drops to the higher 80s even with o2.  Nothing really is showing on the x-ray for the left side.  After the pulmonologist watched her breathing while laying on her back, he thinks she is just loosing muscle tone on that side and it looks as though she is not taking in as much.  They are trying to slowly reduce the o2, but I am not sure how this will go.  When we take the bipap off to cough her with o2 bleeding in, she is ok.  Once we are done and have to get the bipap back on, she will drop quickly so we need to blow o2 at her just to get it back on again.  One good thing is that her co2 is within good range and they continually monitor it.  So,  I am hoping for the best and trying to hang in there.  I feel so bad for her and wish I could just take it all away.  Please say an extra prayer for our princess as we all know there is power in prayer!


March 2007 - 2 years, 4 months

March 24, 2007 - It has been a long couple of months around here.  The last update included Jenna secretion problems, but I think we may have that under control now.  We have tried everything to help her out, but now have surrendered to Robinul.  You have to be careful with dosing as it can not only dry up secretions in the mouth and throat, but dry up other areas of the body too.  It also can slow the stomach down and we already deal with slow gastric emptying.  Speaking of which, Jenna is continuing to have GI problems.  Since the last update, she had a week of distention and high heart rate.  At the time we thought it was maybe a stomach bug.  Not so sure now.  We lowered her pump rate and gave her 1/2 strength formula then eased her back up to normal feeds over a week.  Then she had a good couple of GI weeks.  Now same thing is going on, distention, some gas, high heart rate.  Her stomach is getting as hard as a rock after being on feeds for a while.  It takes an hour or so off feeds for it to come down.  We saw a GI doc today (first one believe it or not).  We had to drive 45 minutes to see him in Batavia, but so much worth it.  He was great.  Really willing to work with us to find out what issues she is having.  He is increasing her Reglan dose again, and if that doesn't seem to help, we are going to try Zelnorm.  Also, she has another scintascan appointment on Tuesday to check that slow gastric emptying.  Things are SO different since the last one.  This was before the AA Diet and she had yogurt and Pediasure.   Now she is on the amino acids and slow drip feeds.  Apples and oranges.  Very curious on how they will perform this due to the slow drip and no boluses.  I guess we will soon find out. 
Many hugs and prayers going out to all the sick kids:  Ally, Shira, Julia, William, Cage, and any others I may have missed!  Get well soon!

My thoughts and prayers are with the Watts family, as little Logan (aka Peanut) earned his angel wings yesterday.  My heart goes out to you.



March 5, 2007 - Jenna has been having quite a bit of secretions and over the last couple days they have increased, become thicker, and white.  Her heart rate is elevated too.  She was having issues with her sats dropping, but we treated her Q4 with Chest PT, Nebulizer, Cough Assist, and suctioning.  Today the secretions seem better.  Over night we kind of figured out that her heart rate issues seem to be related to the feed.  I dropped the rate of her pump today and her heart rate has improved, not totally normal, but much better.  Hopefully it is a 24 hour bug and she will be feeling better real soon!  Big hugs JJ!

Hugs & Prayers to the other sick kiddos:  Lizzy, Malorie, Ava, Colin & Casey, Ryan and any others I may have forgotten to mention!  Glad to hear Mr. Kyle is feeling better ☺!


February 2007 - 2 years, 3 months

February 22, 2007 - Jenna is still having issues with her secretions.  They have actually gotten worse.  She is desating while reclining in her kid kart and she is also desating during naps and in the middle of the night with her bipap on.  We have to cough and suction to get her back up.  This happened a couple times last night and a couple times the night before and during the day a few days ago.  
If she is lying down during the day playing or watching a movie, her sats stay up to 98% - 100%.  She does not have a fever, but is extremely cranky, to a point she seems to get real frustrated/aggravated while playing with toys and for no apparent reason.  I had her in the pediatrician's office Saturday and her ears and throat were fine.  Her heart rate is good, 98-105 when awake and 79-90 while sleeping.  Respiratory rate seems normal. 
We thought maybe the Tolerex was the issue and have slowly changed back her diet to what was working (2 pks Vivonex, etc), even though it is low in calories.  It has only been a couple days off the Tolerex, but her secretions are not any better.  We lowered her total volume of fluid by a couple ounces (in case it's her slow gastric emptying) and that has not helped either.  We have also thought maybe it was her last molar coming in, but the tooth has not come in any more than it did weeks ago (teeth come in real slow) and Motrin is not helping with the crankiness.
It is disappointing that we cannot help her out and really not sure what the cause is. 



February 19, 2007 - It has been a pretty busy couple of weeks.   Exciting too!  We finally received Jenna's power chair!  We also found out that Medicaid has approved us for our minivan conversion: side entry with lowered floor!  Seeing it was more than the cap amount, it will have to go to Albany for special handling, but it was approved!  This is great news!  It really is difficult to transport her now.  She uses the EZ-On vest, which is great, but getting her chair in and out of the van is another story! 

We are dealing with secretions again.  I am not sure exactly where they are coming from, either diet, teeth, or what.  We switched her over to Tolerex and the secretions started again.  So we are slowly going back to Vivonex to see if it is a Tolerex issue.  She is also teething her last (thank god) molar.  We'll see. 
We had a WNY FSMA Valentine's Party yesterday.  There were crafts, pizza, desserts, and an ice cream social.  Even a build-a-bear fundraiser!  The kids really seemed to have a good time.
I also met with Administration at our local children's hospital regarding improving neuromuscular care in WNY.  We are going to form a Parent Advisory Council with hospital staff, medical professionals, and parents to improve the quality of care for our children.  I know it will take time, but hopefully our families will be given current information about these diseases, diet information, and up-to-date treatment options.  One thing Jenna has taught me is that you have to have hopeSo I hope we can improve the care here and that a newly diagnosed family will have the right information on choices and care for their child.  I hope that when the next child ends up sick and needs to go to the hospital, they will not be dismissed because they have SMA (or another neuromuscular disease).  I hope that when the next family decides to put their child on the AA Diet, they are given the right amount of amino acids and not end up sicker than they first were.  I guess I just hope for quite a bit for our children and our families, especially proper care. 

Prayers going out to all the sick children:  Lia, Issac, Aschdon, and any others I have missed! My thoughts are with all of you!


February 5, 2007 - All in all, Jenna is doing well.  We are still playing around a bit with diet, but I have a feeling it will be a never ending process.  She has been pretty healthy, so that is great.  We are waiting on her powerchair, which we should have Wednesday, yeah!  We put in for van modifications, but haven't heard anything yet. 

Happy Birthday to KALI!  Her birthday is tomorrow and she turns 6!  Big girl now!  Plus, she ended up getting glasses a week ago and now she has a loose tooth!!  WOW!  Lot's going on around here.

I want to send big hugs and prayers to the Postma family!  Little Issac is in the hospital and he can use all the extra prayers right now.  My thoughts and prayers are with you!


January 2007 - 2 years, 2 months

January 25, 2007 - Well we never made to the GI appointment.  Jenna's bipap woke us up that morning as the power went out, which sounded an alarm.  We looked outside and we ended up with quite a bit of snow and thought it wasn't worth an hour ride to see the GI doc.  So... we rescheduled.  It is probably a blessing in disguise as we just started the process of switching from Vivonex to Tolerex.  Jenna was down 200 calories and I am sure the GI doc (whom we have never seen before) would have thought we were crazy, even though we know what are plan is.  I will be nice to finally switch over and not have to worry about the calories.  So far we have only added a 1/2 pack and she is tolerating it just fine.  Tomorrow I plan to add another 1/2.  I tis amazing as to the changes already.  We dropped some Vivonex and fat and her heart rate went down (not that it was high to begin with), she doesn't have secretion issues anymore, and she is sleeping all night.  She also is MUCH better with her GI issues, not gassy, distended, nothing.  It's just amazing!!
Jenna's VPA has increased as her levels continue to be low.  It was quite a jump, but hopefully it will put her were she needs to be.  I think it is making her a bit tired, but that should even out once her body gets use to it.  We'll check her levels and safety labs again in a few weeks. 
Get well wishes to all the sick kids out there, especially Alex and Matthew!  My thoughts and prayers are with the family!


January 15, 2007 - Please pray for little Alex as he has been in the hospital with pneumonia for the past 7 days!  My heart and prayers go out to the family.

Jenna is doing better with her secretions.  It turns out that dropping some Vivonex in her diet has helped tremendously.  We have been trying to add some foods to make up calories, but it just isn't working.  We will most likely have to switch to Tolerex as it is higher in calories.  Other than that, things are going fine.  We go see the GI doc this week, so that will be interesting. 

We received Jenna's "Auntie Jenny Hat" last week.  It is just beautiful.  If anyone is interested, proceeds go to SMA Support and you can find them at Auntie Jenny Hats.



January 7, 2007 - Good News!!  Jenna's power base to her Kid Kart has been approved!!  This is great news and now it can finally be ordered!  We have only been working on it since May!  So it should only be a matter of a few weeks for it to be in!  We have been practicing with the loaner and she really isn't doing too bad!  We are trying to figure out which side her joystick should be on as her left side is stronger than her right side, even though she is right handed and initiates everything on the right.  Whichever one it is, I guess it doesn't really matter.

As for her cold, she seems to be doing better, but she is just having such an awful time with her secretions.  We have been giving her Benedryl as it is the only thing that helps.  The secretions got better, so I backed off.  Well, by the end of the day, she had so many secretions again that her sats started dropping, even while sleeping on bipap.  So... we are back to Q6 for the Benedryl.   She is cutting her last two molars and part of each tooth has broken the skin.  I guess we need to try to figure out if the extra secretions are due to teeth or if she should be put on something to help her manage them.  Ever since the Utah/Nebraska disaster in September, Jenna is on and off with being able to control them.  She will do well for a bit, then can't handle them (I.e. reclining in her kid kart or just moving her position drops her sats and we have to cough and suction to help her out).  She has an appointment coming up with the pulmonologist, so we'll see what happens then.



2006 Updates