2006 Updates
JJ's Journey
December 2006 - 2 years, 1 month
My thoughts and prayers go out to the families who's children earned their angel wings this weekend and to all of those who are sick!!
December 4, 2006 - Well, it has been a long month with Jenna. We have been fighting feeding issues, stomach distention, pump rates, gas, you name it. Then yesterday Jenna started with a low grade fever in the morning and by bedtime was laboring. We put her to bed with her bipap and that helped the breathing, but she still had a low grade. Despite giving her Motrin every 4 hours, her temperature climbed to 103.6 early this morning. We took her to the pediatrician and because her lungs are clear (which is a good thing) and now has a runny nose and lots of secretion, he is calling it a cold. She is still running a 101.8 fever after 3 hours of Motrin so we will be rotating the Motrin with Tylenol. Her sats are jumping from 94-96 without bipap with periodical dips to 90, so she pretty much has it on now. Her heart rate has been climbing up to 150-160 due to the fever. Of course we are coughing her regularly and when needed. Please say an extra prayer for Jenna as we fight through this mess!
November 2006 - 2 YEARS OLD!
November 11, 2006 - Jenna is no longer doing well with bolus feeds so this past weekend we changed her over to drip feeds. I guess her tummy was just too full with boluses and making her uncomfortable. She is on the feeding pump most of the day with a couple 2 hour breaks during the day. She is actually doing pretty well with it and her sats have improved. Even her sleep has improved!
November 8, 2006 - We went to the Wiggles Sunday and Jenna just loved it! We got to meet them before the show and they were so nice! The arena made a mistake with our tickets on where we were suppose to sit, so to fix the error, they made seats for us in the front row! So Jenna had a blast and just loved it. To see pictures, click Wiggles!
Now for the bad news. Jenna is starting to desat when sitting up
(in a reclined position). It reminds me of how she was when we tried to
go out to Utah and landed in Nebraska. She had to go for blood work
this morning and when we were done and just getting in the van, she
dropped and I had to treat her in the parking ramp. Her sats fell again
after speech therapy today (she reclines in her Kid Kart for ST). Even
though she is better laying flat, she desated a couple times during nap
today too. She definitely has some extra secretions she is trying to
deal with and I think at times they are too much. We just keep coughing
and suctioning her, which does help bring her back up. Please keep her
in your prayers that it doesn't get worse and she is back to herself
soon!
November 2, 2006 - Happy Birthday Day to You! Happy Birthday to You! Happy Birthday Dear JJ, Happy Birthday to You! You are a strong little girl and continue to beat the odds. I love you very much and I am so proud of all the things you do! Love you Lots! XO XO Mama XO XO
October 2006 - 23 months old
October 24, 2006 - It's a busy week this week as we are getting ready for Jenna's BIG birthday party on Sunday. Yep, the Big 2! Of course we are doing a Barney theme, Jenna just loves him. I think we will also have the kids dress up in their Halloween costumes. Trick-or-treating is either being rescheduled or cancelled in some areas because of the snow storm. A few are still without power and it is a huge clean-up with trees and power lines still down.
Well, Jenna is doing pretty good and continues to beat the odds! She is doing well in Speech Therapy and is actually "trying" to say words, and isn't doing too bad at it either. A couple months ago, she had no interest in talking. What a turn-around for her. I think her body is adjusting to the VPA, as she is starting to sleep better at night too. Her arms seem a little stronger and she is really trying to roll when assisted. Hopefully more good will come from it.
October 15, 2006 - This past weekend, we had our first snow storm of the season here in Buffalo. We lost power Thursday evening and ended up with a couple feet of snow. Trees and power lines are down everywhere. Our power just came back on late this morning, but some will be without power for another week! Thank God for our generator and car inverter! Paul was able to hard wire the furnace to run off the generator and we were able to plug in Jenna's feeding pump, bi-pap, and pulse ox. The cough assist wouldn't work off the generator. It just wasn't powerful enough, so we used the power inverter in the van and run an extension cord into the house to give her treatments. All in all, everyone was fine and we are so glad it is over!
October 10, 2006 - We are finally settling down from our trip and things are falling back into place. Jenna is doing much better and she has started the VPA. She is having trouble sleeping at night lately... not sure if it is the new meds, teeth, or what. Hopefully we can work that out real soon too!
September 2006 - 22 months old
Thanks Jana & Kyle for the pillows! They work great and are just beautiful!
September 15, 2006 - It has been a pretty busy month with the girls starting school and Jenna and her new therapists, but everyone is well. Since the weather changed, Jenna has a few more secretions to deal with, but all in all, she is doing fine.
Exciting news! We got floor seats to the Wiggles concert for Jenna for her birthday!! She turns the big 2 on November 2 and the concert is November 5. Shhhh...... don't tell her! She is going to be soooo excited!
We are planning another trip to Utah soon. We haven't been there in so long because Jenna would get sick every time we had plans. Dr. Swoboda is going to switch her from PBA to VPA, which we want, but she should have an evaluation done first. Pray that she stays well so we can make the trip this time!
September 1, 2006 - Well August is over and so is summer. We have had some cooler than normal temps and it feels as though fall is near. Besides the weather, there are so many changes coming up this month. Big sister's Mya and Kali start school on Tuesday (Kali starts KINDERGARTEN! - Yeah!). We plan on going to the Labor Day Parade on Monday as a final goodbye to the summer and hello to a new school year.
As for Jenna, most of her therapists will be changing. Her PT had to drop her due to scheduling, and her OT is going out on maternity leave. Even her aquatic therapist will be changing We all know Jenna is not a fan of change and new people, but she will eventually get use to everyone! ☺
August 2006 - 21 months old
August 28, 2006 - Jenna has had a real good week. No colds, no respiratory issues, no nothing... Just what we like to see! She even went to her cousin Mariah's birthday party this past weekend! Everything is good in JJ Land.
August 18, 2006 - Today was a sad day..... This morning, we learned that Princess Grace earned her angel wings last night. Grace was 2 1/2 and also had SMA Type 1. She was a beautiful little girl and we will never forget her smile! Hugs and Prayers to you little Grace!
August 10, 2006 - Today is a tough day for me. One year ago, Jenna was formally diagnosed with SMA Type 1. Just remembering the terrible news and the feeling as though someone has ripped out your heart and the feeling of loosing your baby girl to some devastating, progressive disease. The feelings just come back so strong on an anniversary such as this. Over the past year, we enrolled Jenna in a clinical trial in Utah, obtained all her respiratory equipment, and she lost her swallow and went through G-Tube and Nissen surgery.
On a happier note, Jenna is feeling better and her sats are great. She is not sleeping well at night, but she is getting three more teeth including a molar.
We had our 3rd Annual SMArt Walk For A Cure this past weekend and it was great. The weather was beautiful, Jenna had a good day, and it was nice to meet so many other families. The walk was a great success and is believed to have had 400+ walkers and raised approximately $50,000. We will know more details as everyone is still settling in from our big day.
July 2006 - 20 months old
July 31, 2006 - Well, Jenna is sick again. We woke up at 2:00 am to her pulse ox alarm. She dropped to 86% and heart rate 165. She was running 102.4 temp and had LOTS of head congestion. We were able to get her back up using the cough assist and suctioning. A cool bath brought down her temp and she went back to bed. At 5:30 am, her alarm went off again with a sat of 86%. We gave her another round of cough assist and suctioning, but this time she needed Albuterol to get her back up. We already had an appointment with her pulmonologist for this morning and she did fine on the way. After we sat down in the waiting room, she dropped again. Another couple rounds of cough assist and suctioning and some Chest PT brought her back up. She dropped a couple more times while in the clinic. She stabilized and we brought her home. Her fever is staying down to about 99.9 with Motrin. She just has so much head congestion that it is just dropping into her lungs. Right now she is sleeping on Bi-pap and staying around 95%. Her lungs sound clear and nothing was prescribed.
July 24, 2006 - Sorry I haven't updated in quite awhile, but it has been a busy couple of weeks. We went on vacation for 9 days and stayed at Sherkston Shores in Port Colbourne, Ontario Canada. It was great! The girls had a great time, even Jenna. She just loves Bradley Bear! He ranks up there with Barney if you can believe that one! She did real well as the weather was pretty warm. She swam quite a bit and loved taking walks and golf cart rides on the beach.
Other good news is that we finally received Jenna's new bath chair. This is great! It is a Leckey and is so functional. It kind of looks like a mini sun lounger! As for her powerchair, we still have the loaner. It is the Quickie Z500 power base with the Mini-Proportional Joystick for her Kid Kart. It will be nice once we receive her own.
Overall, Jenna has been doing well, but for the past two days, she is running a low grade fever (100.1). We're not sure what this is due to yet. Of course I am hoping it is just teeth! She had one episode yesterday while taking a nap where her pulse ox alarm went off. It is set at 90 so it must have been at least 89%, but in the minute it took to go check on her, it was up to 92% and rising. Today her sats have been 97-100! So we're really not sure if maybe she aspirated on drool or if she refluxed a bit (as this is happening a little more now that her PBA dose was increased, and yes she does have a Nissen and is on Prevacid).
Seeing we missed Jenna's appointment with Dr. Swoboda in Utah due to her being sick, we have rescheduled and are going again in a couple weeks. I am interested in what the test scores say as it has been awhile. Wish us luck that we can get there this time!
July 5, 2006 - Jenna had a wonderful week. The Zyrtec REALLY seems to be helping. The congestion is gone, her sats are good, and she is sleeping again! All is good in JJ Land!
June 2006 - 19 months old
June 27, 2006 - Even though Jenna is doing better, we were unable to make our trip to Utah this week to see Dr. Swoboda. Jenna was doing alright until we were at the airport and security had to "pat her down". As you know, Jenna does not like strangers so she got herself all worked up and the secretions started flowing again. Once we got through security, she calmed down, but still had the secretions to deal with. We tried suctioning her, but it really didn't help. After a bit, she started laboring so we decided she just wasn't well enough for the trip. We are going to try to get the lab work done here so at least we will know where she stands with her "safety" labs (CBC, etc).
We believe it is her allergies that is causing more secretions, and in return is causing her other respiratory problems. She does seem to get worse when she goes outside. We took her to the pediatrician's office this morning and they prescribed Zyrtec. Hopefully this will help and she will be the same ol' Jenna again!
June 25, 2006 - Jenna had a lot of head congestion to deal with. It was a tough week trying to keep up with it. She doesn't have much of a swallow so when the congestion would fall, it would go into her lungs and her sats would fall. We kept up with RT every 2 hours, increased her bi-pap pressure and for the most part we have made it through once again!
June 19, 2006 - Jenna had a pretty decent week last week. She wasn't sleeping well at night, but she hasn't been sleeping well for the past 3 weeks so that wasn't any different. Months ago she had trouble sleeping at night for about 3 months. We put an "egg crate" on top of her mattress and that seemed to help. This time we ended up buying some Memory Foam for her crib so hopefully that will help with the pressure points and at least make her more comfortable. Besides teething there hasn't been any other signs of why she isn't sleeping.
Now we are on the SMA roller coaster again.......... Last night was the first night her sats dropped in awhile. Her alarms went off around 1:30 am to find her at 91%. Within seconds she dropped to 86%, then to 76%. This is the lowest she has ever dropped and it was pretty scary. We gave her a Nebulizer treatment and a couple rounds of Chest PT, Cough Assist, and Suctioning and she finally went back up to 97%. She has been a little better this afternoon, but has dropped a couple times since this morning. Once before she woke up and she was at 93% and then again during nap she went down to 88% and I had to wake her up and do another round of RT. It looks like we will have to do the Nebulizer every 4 hours and the rest of RT every 2 hours. Hopefully that will help and we will get off this ride for awhile!
June 10, 2006 - Jenna received a package in the mail a few days ago. It was "SPUNKY" the dog from Ethan Takacs. She just loves it. Thank you SO much Ethan and Happy 1st birthday! Please feel free to visit him by clicking: Ethan.
The last couple weeks have been tough. Those 3 teeth are still coming in (they take so long for her) and she ended up with 2 ear infections. We also found out that she has seasonal allergies, so between that and the teeth it is no wonder why she ended up with ear infections!
We go back to Utah at the end of the month. It has been so long since we have been there since she was in and out of the hospital for 4 weeks. It will be interesting to see her test scores. When we were there in January, she did not have a MUNE (Motor Unit Number Estimation) to measure her muscle strength as she got herself so upset we had to cut the appointment short. So her last one was done early November. The end of November was the beginning of her disease progression. She ended up sick with bronchiolitis, lost head control, stopped eating, became weaker, and had an NG tube placed. That is when we decided to stop the Rilutek and start the PBA. Speaking of which.... GOOD NEWS!!! Our insurance company approved the PBA for 1 year! Yeah!!! It has definitely shown increased strength. She started regaining head control, but lost it again in the 4 week hospital stay. She can now push and kick her legs, has more strength in her arms, and can bring her arm over with assisted rolling. These are major improvements! We are working on her head strength again and we have seen slight improvements. It will just take more time.
Jenna
with Spunky.
MAY 2006 - 18 months old
May 17, 2006 - Last week was a wonderful week. Jenna was feeling good and all respiratory problems were under control, feeds were going well, and things were good. This week, unfortunately, is a tough week. Jenna ended up with an ear infection and high fever. After a couple days, she seemed to be getting better and then.... boom, struck with another high fever, stomach upset, and now rash. I took her back to the pediatrician's office today to find out it is another stomach virus. Well, at least she is home this time and not back in the hospital.
Jenna went back to see the Ortho and had another full body x-ray. Her scoliosis is the same (30 degree curve) and that is good to hear. Her chest shape has changed due to her disease and the respiratory distress episodes she had. This is very common with SMA. So for her to remain the same after 6 months is great to hear. She is not due to go back for 1 year now.
As for more good news..... we have finally started nursing services! We have three different nurses who rotate 5-6 days a week. Once training is done, it is going to be such a big help! They all seem so nice, I am sure Jenna will love them!
May 3, 2006 - Jenna is having a better week this week. She is cutting a molar, so she has the congestion and secretions to deal with due to that, but.... she is home and doing well. We received her power base to her Kid Kart this week too. She is doing great with it. Even though she has trouble pushing, she was able to get it to go forward and backward. She was even doing doughnuts in the driveway! So all is well right now and we are just trying to get back into the swing of things.
This
picture is for all of you that Jenna rolls her eyes at! "JJ The
Bull-Headed Drama Queen" Enjoy!
APRIL 2006 - 17 months
old
April 25, 2006 - Well, as of right now, Jenna is still home. Even though she is doing better, she isn't 100% and is still congested. Since bedtime last night, she is having these "mini episodes." Her oxygen level will drop to 89-94% and sometimes she just keeps coughing and can't catch her breath. I have to give her Respiratory Therapy with the Nebulizer/Albuterol, Cough Assist, Chest PT, and suctioning. After the whole routine is over, her sats come back up to 98-100%. This is happened 3 times in the past 16 hours. Hopefully she will have a better afternoon/evening.
April 22, 2006 - Just a quick update.................. Jenna was discharged Wednesday afternoon and is once again HOME! She has some congestion, but overall she is doing better.
April 18, 2006 - Jenna had another episode this week. She had a great weekend and a good day for at least the first half of Monday. Once again, she awoke from a nap with tachycardia and hypoxia and laboring. I gave her 2 rounds of Respiratory Therapy including Chest PT, Nebulizer, Suctioning, and put her on her bi-pap. Unfortunately her sats did not come up and hovered near 90% - 93%, even with the bi-pap on. We had to call 911 again and get her to the hospital. A chest x-ray showed another spot of Atelactasis (partial lung collapse). We increased her settings on her Cough Assist and Bi-pap machines. She went another round of Respiratory Therapy in the ER and wearing her Bi-pap continuously. She ended up with another fever of 102, heart rate of 170-180, and sat still fluctuating around 90%+. With the Therapy every 2-4 hours and continual Bi-pap, she starting doing better around 3:00 am. Today she continued to do better and was released from ICU and placed on a floor. We are hoping she has a good night and can be discharged again tomorrow.
April 16, 2006 - HAPPY EASTER! Jenna came home Thursday evening and it has been a wonderful weekend. She is in good spirits and enjoying the beautiful weather we have. It was so nice to get some fresh air and go for a walk again!
April 12, 2006 - Well, once again little JJ was not ready to come home. On Friday, April 7 Jenna had another episode, but this time she was so sick. Her pulse ox monitor woke her up again from her nap with tachycardia of 170 - 175. This time she had severe bowel issues, retching, fever, it was so bad! We took her back to the ER as her heart rate continued at 170+ for 5 hours. Her temp reached 103.8 and she became dehydrated. She was once again, readmitted. It turns out this time she has a stomach virus. She must have caught it from the hospital as that is the only place she has been for the past 3 weeks. After a couple days she started to look better, but then relapsed. We had to pull the feeds again and start all over. As of today, she has worked up to half pedialyte, half formula and at her goal rate. If she progresses to 3/4 strength formula at the same rate, she will be able to come home. We are hoping it is Friday (Good Friday) and we can be home, together as a family, for Easter.
April 6, 2006 - Jenna was re-admitted to the ICU on March 29 due to tachycardia (high heart rate) and hypoxia (low oxygen level), She had multiple tests performed to figure our what was happening. A chest Xray and CT Scan showed a partial lung collapse. GI Studies (w/contrast, CT Scan, Esophogram) were performed to make sure everything in regards to her G-Tube and Nissen surgery were fine. Thankfully, these tests came back negative. Multiple cultures were also done, but for the most part came back negative. Antibiotics were started in case of a pneumonia or other bacterial infection. She started having GI and bowel problems, which continued for days. She could not keep up with loosing so much fluid she began to dehydrate. Extra fluids were administered through an IV then switch to free water through her GT.
So while the doctors tried to figure our what is wrong with her, she continued to have tachycardia, bowel problems, battle dehydration, and thick mucous, which needed to be suctioned. She was placed on Respiratory Therapy (Albuterol, Chest PT, Cough Assist, and Suctioning) every 4 hours. Every 2 hours we would also do another round of her Cough Assist and suctioning. After a couple days, the pulmonologist on call thought her problems were related to the new formula. She is placed on Reglan to help with gastric emptying and we decided to change her formula to Pediatric Vivonex. Unfortunately, the hospital did not have any and it had to be ordered. A few days later, Jenna was finally released from ICU and went to the floor. She continued to do better, started the new formula, and was discharged on April 6.
April 1, 2006 – Well..... Jenna was admitted on March 22 to receive her PIC line for nutrition. That didn’t go so well. They tried 3 times in her left arm and 2 times in her right arm. They ended up putting in an IV for the time being. On March 23 she went in for surgery and while under anesthetic, they put a central line in her groin so she could receive the proper nutrition she needed (TPN). The surgery took 3 ˝ hours and everything went well. She was monitored in ICU and continued to do well. She was then discharged on March 29 and we were so glad to be going home. Unfortunately, Jenna was not. We took her home and put her down for a nap as she did not sleep well the night before. It only took a matter of 30-45 minutes and the alarms on her pulse ox machine started going off. Her heart rate went up to 175 and pulse ox went down to 92. She turned completely white and her breathing was labored. We called 911 and had her brought back to the hospital. From the ER, they re-admitted her to the ICU. Until they can figure out what is going on, she will stay in the unit.
MARCH 2006 - 16 months old
March 20, 2006 - Jenna's surgery was rescheduled for Thursday, March 23. Instead of fasting for 18+ hours, the surgeons thought it would be better for Jenna to be admitted on Wednesday and have a PIC line put in. This way she will receive nutrition throughout Wednesday and Thursday.
March 10, 2006 - Jenna received her blanket from MJ & B4SMA. Thanks MJ!!
March 9, 2006 - Well, poor little Jenna ended up with Strep Throat and was put on Amoxicillin. We were hoping that was the cause of her refusal to eat, but unfortunately it was not. She finished her antibiotic and we also found out that she has a sensitivity to Amoxil as she ended up with quite a rash all over her body.
Surgery is scheduled for March 20 and it is definitely time to get rid of the NG Tube. It, also, has been causing a lot of irritation.
The new medication (PBA) is showing promise. Jenna actually has a little more strength in her legs, is moving her arms more, and can keep her head up for 2-3 minutes at a time. All this with only a couple weeks of being on it! Hopefully there is more to come!
FEBRUARY 2006 - 15 months old
February started out good. We dropped out of the Rilutek study as it did not seem to maintain her neurological condition. We started the Sodium Phenylbutyrate (PBA) in hopes to increase her SMN2 protein and strength.
Jenna only needed her NG tube for supplemental feeding up until now. Recently, she stopped eating. She refused her bottle, food, even ice cream! She has started choking on Rice Krispies, rice, everything. We are unsure if she is hurting, protecting her airways, or if she has lost her ability to swallow. We have her on continuous night feeds with 2 small bolus feeds during the day and have scheduled her surgery for a G-Tube with Nissen for mid-March.
JANUARY 2006 - 14 months old
Jenna slowly got better, but still fought eating. This went on for the month of December and into January. We became real worried. After a trip to the pediatrician's office, we noticed that she lost 2 lbs in 2 weeks! It was time to take her to the hospital and have an NG (nasogastric) tube placed. She was given continuous feeds while in the hospital, but we were able to discharge her and bolus feed at home. She seemed to get better once her strength came back from the nutrition and we only used the NG tube for supplemental feeding.
DECEMBER 2005 - 13 months old
December was a tough month for Jenna. She ended up with Bronchiolitis for the 3rd time this year. Except this time, it hit her hard. Thank God we had the respiratory equipment or she probably would have been admitted to the hospital. Her pulse ox dropped a couple times where I thought we may have to take her in. We just continued to use her Nebulizer, Cough Assist, and VPAP to clear her lungs. It was a tough battle, but with the help of her Pulmonologist and her Respiratory Therapist, we were able to pull through it.
We took Jenna to an Orthopedist as it seemed her hips would "pop". They did a full body x-ray and it was determined that both of her hips dislocate and she has developed a 30 degree curve in her spine.
NOVEMBER 2005 – 1 YEAR OLD!
Jenna turned 1 year old November 2nd! Happy Birthday JJ!
By the end of the month, we noticed that Jenna’s head control was getting worse. She was having difficulty keeping it up while being held and sitting supported.
OCTOBER 2005 – 11 months old
Jenna continued to do well and we traveled to Utah once a month. Maybe this medicine is working?
SEPTEMBER 2005 – 10 months old
Jenna seemed to take the Rilutek well. So far she still had decent head control, was still able to eat solids and had no problems with eating.
JJ's Journey