Things are better (Spinal Muscular Atrophy)

Lizzy did not alarm at all last night. (Yay and the crowd roars) Think we have it figured out. It was both bipaps. Hers and the back up bipap. Lizzy told me it was her bipap not blowing out right. We got another used bipap and all went smooth last night. Thank goodness. I still didn't sleep well last not wanting to hear that buzzer soon as a dosed off but tonight I will SLEEP. I was a Pulse ox watcher last night. Thank goodness Lizzy got to rest.
I am thinking that using it on the inverter on the plane might be why we are having so many issues .I am not sure but we are waiting to find out what is wrong.

Lizzy was Fancy Nancy for the church party(2 blocks away ) last night . She looked just like her favorite story book character. Fancy Nancy by Jane O'Conner. Lizzy has been a fan since the first time we read her the book.


Well, we got the terrible news the Prison here is closing Dec 31. That is so many jobs. They are trying to think of another use for the prison.
I am so worried about this community. I hope they will find another use for the prison. Its a huge building. They Governor is closing many State Parks also. This is so wrong. http://www.DanRutherford.com/ for more details. Our favorite IL Senators web-site.

No sleep yet again.. feeding tube clog and alarms(Spinal Muscular Atrophy Type 1)

Lizzy has been feeling great but darn it every-time she doses off on her bipap at night the her pulse ox starts going off because of her o2 levels drop. She drops for a second and then back up to 98-100 O2. I cough her, suction her and she is not having any problems. I change her mask , I am using her back up bipap , pulse ox probe change and I don't know what is going on. She says she is fine every time it wakes her up too. Right now she is sleeping off bipap 3 feet from me. Her O2 level is 98 and her heart rate is 72. She is sleeping so well. No alarms off bipap. What is going on?? This has been going on for days. We sent her bipap in to be fixed and have been using the back up and still we are having issues. Is that one not working right also?
I will watch the pulse ox and think all is well then soon as I start dozing off there it goes off again ...... buzzzzzzzzzzzzz. Its like you have been zapped in your brain. zzzzzzzz (chill up my spine just thinking about it) . Then when I did everything I can think of and finally got her situated turned her over again I had my eye on the pulse ox I happened to notice her G-tube was clogged (she is vented). I had to get the clog out and then try to get some sleep. Then, I was worried her gtube was going to get clogged again and it did !!! OMG!!!! I got up again and took and cut the end off a q-tip and cleaned out her G-tube and sure enough there was something lodged in there. She had alarmed 5 times last night for a second then backed up g-tube. Its like she is 2 years old again. I am not used to this. It all started with the bipap messing up after she had gotten sick.
She is no longer having illness related issues but what the heck is going on? I need some sleep. She is also not sleeping because of it.
Her O2 and heart rate are totally back to base line. Treatments are going smooth. She says she is not sick and feels fine.
Pray I get some sleep tonight!!

Better day (Spinal Muscular Atrophy)

Lizzy is acting better today. She is not so demanding today and cranky. This illness is a mystery
The DME gave Lizzy a demo mask and it works well for her. Only thing is Lizzy dropped a few times o2 last night while sleeping again last night. No air leaks NOTHING. I would cough her suction her and she was fine. I am not understanding what is going on. Her O2 and heart rate are great off bipap she has had no fever. I am concerned and don't know what is going on. We got a used bipap from the DME till Lizzy's gets fixed. They said I was right her bipap was not working correct. She seems to do better off bipap. Her treatments today went normal. No extra junk. Great air movement. She says she has a headache but that is all. She is on every three hour treatments so I am still NOT understanding if she sound good, looks good and does great off bipap what is going on. She is on extra Breast milk and pedilite also so this is really baffling me.

On a good note. Charlie and Sophia are released from the hospital. Sophia is on her way home. Both kids are doing great.

Prayers for Logan H he is not doing well. He has his air ways start to close and they don't know why. Many prayers to them

I went to curves for my 5th time this week. I am starting to feel great and feel lighter.

Have a great week-end

dealing with iIllness,docs, nurses and DMES (Spinal Muscular Atrophy)

PLEASE big prayers for Charlie and Sophia D. They both had surgery to today for their extension surgery for their backs.
Lizzy is doing ok BUT she kept alarming ALL last night! I coughed her or suctioned her and she would go back to 98-99 o2 . Her chest sounds GREAT. Great movement. It had to be the BIPAP. She is satting great with out bipap. I am not understanding what is going on. UGH Secretions/junk are thinner. Her Bipap sounded weird(Motor) so we had to send it in to get it fixed and they got us a used one which I just totally disinfected it . They said Lizzy's bipap was not working right. That was a hassle also. You have to call this person and then call this person to see if that person got the message. New Policy changes at the DME.....
Sorry my mood sucks today. No sleep last night .
Lizzy had started green gunk yesterday and TONS of secretions. Went through sooooo much bull yesterday getting her on an antibiotic She had low grade temp and/ is a total crab.
Lizzy's local doc was gone so the Nurse Practitioner ordered Lizzy ( You are not going to believe this one...... ) Freaken Robitussin!!! ... After I waited all day for them to call back with a script for an antibiotic. That is what they called and told me. Voicing my opinion got me no where. They know what Lizzy needs and they also usually always give us what we need for Lizzy. It took till 4:20pm yesterday to get that call.
I was so upset I called up to our specialists office only to get a recording from the number I used to call to talk to the nurse and it said they are "NO LONGER TAKING PATIENT CALLS ON THIS LINE" with no details only if you want to make an appt a number to call. I call Lizzy's specialist 's asst Lisa (she is a super great person)and she gave me a number to call. The doc on call called back and he informs me Elizabeth has what ever one else has and its not from the flu shot or she would of had symptoms the next morning after the shot. He was very "Dry" but called me in the script for Lizzy with no problem. It was such a frustrating last few days I already hate the season and its just started.
Lizzy is doing better tonight but is very cranky keeps repeating herself when you do not give her what she wants right now and will no nap today even though she feels like crude.
I did leave to go to Curves today so I did escape for an hour.

I know I am rambling. I think I am taking Lizzy to bed early and call it a night.
Any way special HUGE prayers For Charlie and Sophia.
Prayers for Lizzy and MJ too!!
Gosh I love my life some days!!!!!!

Lizzy not feeling well and a Losing start for me(Spinal Muscular Atrophy Type 1)

Lizzy is not feeling herself today. I am not understanding what's up. I hope she is not getting sick. She has been so whiney all day. We have a trip on Thursday to Madison (For OT appt) so please say some prayers for my girl that she is not getting sick. Only thing different is that I had to change her bipap mask to a golden seal from her profile light. Her mask had a leak and she would not wear any mask but this one. Her DME would not send us a new one. We have to wait a month to get her a new one. So for now this is working. Her sats have been good. I just think she is tired and feeling puny. I pray that is all.
I worry so much when Fall hits.

I did it!! I lost 6 pounds and 6 1/2 inches!! (hear the crowd roar) . OMG! I feel better too. Its not a lot but its a start . I am actually leaving the house ALONE 3- 5 days a week and going to work out at curves . I am with actually adults OUT-SIDE of my house. I have not been out (with out Lizzy ) much in almost 5 years.
Hey, I also found this video online that also helped me feel better too. http://pauleugene.com/ This guy really does a great work out. So, I can get myself back in shape doing both. I felted so "pumped" when I got done with only about a 10 min work-out. He also has some videos on You tube.
I have been doing Curves a month now and I think its the best thing I have done for me in a long time.

Time for Lizzy's treatment.
Hope you all are well!!

Speech eval , Tiarra and Tea - living with Spinal Muscular Atrophy Type 1

Lizzy had a busy week.
She had PT with Monica on Tuesday and then she had a speech eval with a new person in her life Allison on Thurday. Allison her a new speech pathologist. She said Lizzy talks very well and thinks I have done excellent with her exercises her mouth and keeping her muscles moving. Lizzy is 5 and has lost minimal facial movement. She did quick sort of IQ test on her and after some questions to Lizzy she determined that Lizzy is right on track for her comprehension levels and knowledge for a 5-6 year old. She was quite shocked Lizzy is a Type 1.
Allison was upset we have more or less slipped through the cracks and no one has (here) has thought Lizzy needed speech therapy. It is sad the life expectancy (Which was 1 1/2 -2 years old) has held up so much. Its like no one wanted to help us at times because they thought she was not going to be here any way. BUT GUESS WHAT ??? LIZZY is still here and going strong. Allison is going to help me with Lizzy's stimulation in her mouth. She said she will help me maintain her facial muscles and maybe even be able to help her more than I can do. I have worked for years keeping those muscles moving and making sure she uses them. Lizzy still does the Elvis lip curling imitation also. She made Allison laugh.
Lizzy and I agreed we think Allsion is going to work out just fine.

Lizzy got all dressed up in her tiarra with Rachael and had tea today. It was too funny. They had tiarras on, all the bling you could imagine they both cracked me up. Lizzy was talking in this strange little pretend voice.

Hopefully this week we will be going to Madison and get Lizzy fitted for an arm brace. We will get to see Charlie his sisters and Sophia. They both have spinal surgery this week so please keep them in your prayers.

Time to go do some baking.

Its actually been so chilly here that the heat has been on at night.

We are home from CA / Spinal Muscular Atrophy

Our flight was very late last night. We didn't get home till around 12am and after Lizzy's nightly treatment routine it was 1:30am before I even got her to sleep. She still had 100ccs left in her bag when she woke up today so I let her sleep in till around 10am this morning.

The trip was the best ever as far as flight and arrangements on our stay. We went site seeing with Molly, Andy(Andrea Doebbert's aunt and uncle) , Jennifer , Jacob and Todd Goodson. We went to Pier 39 where the seal lions have made a home after the earth quake. Took pics of Alcatraz,(could see from the pier) The Blue Angels were practicing and we got to see them. (Lizzy hated the loud noise) I almost peed myself a few times watching them coming so close to hitting each other. Lizzy and I took off though when she said the noise was hurting her ears. We went to park that the Canadian geese that migrated their after the park was complete and have not returned home.
We saw San Francisco down town and their very cool houses , the Golden Gate bridge park and the Pacific ocean.
It was truly a great trip. Lizzy was something....... LOL . The night we got there it was late. I did her treatment as usual and she was doing so great. Well I got woke up from her alarming at 140 heart rate. So, I asked her" if she was sick"and she said "Lady.... I am NOT SICK!! I just want to know what is in the package up there " I said huh? and looked up where she was looking and Molly had a birthday present for her that she had not given her in a pretty package on top of a antique bureau . LOL. I laughed so hard. She finally told me she was pooping. That was the cause of her high heart rate was high.

The visit with DR. Wang went excellent and he is totally shocked as everyone how well Lizzy is doing. He said he leg movement thing she does is like something out of a ballet. Lizzy has gained more weight again and looks totally great. He spent most of his time with Lizzy and he didn't not talk much to us. He shook our hands and said she is doing just excellent and was so awed at Lizzy's movement, talking and just Lizzy in general.
I guess the media people tried to call us and told Virginia (coordinator for Wang) and told her Channel 11 news was supposed to be there. Because something had come up they canceled. We had no clue they were even thinking about doing that. Thank God we didn't have too!!! They can wait till I lose some more weight . ( I hate surprises like that) Robert Dicks the media person from Stanford came in and saw Lizzy and took pics of Lizzy and her favorite nurse for the year end report for Stanford. Robert told us Lizzy was just one the most beautiful children he had ever met. That made us feel good ans Lizzy looked at him and you could see the sparkle in his eyes. DR. Wang sent two residents in to LEARN about SMA so they got a quick detailed report from us on Type 1s and they need to understand SMA. NIV,AA diet (and breast milk) and how to help these kids stay strong by stretching etc. They loved Lizzy also.

The trip home was great except for the late flight last night

Molly and Andy made the trip so special. We stayed with them this time. Very Intelligent warm compassionate , heart warming people. They are like family to us. Their home is just beautiful.

We have to go back in March not sure when. That was something that we didn't not compromise on. No way we are flying during RSV season. We are always now taking the later cheaper flight and staying that extra day. Worked out great.
American Airlines were very accommodating on the the trip there and some of them were on the trip home. We do pretty well traveling with all 6 of Lizzy's machines.
Now just got to figure how we are going to get out there in March.

We fly out tomorrow to Stanford. Lizzy is so excited. She can not wait to see Molly and Andy . She says she wants to see DR. Wang also but tells me she doesnt need a blood test. LOL She has not stop talking all day.
We have to fly out there every 4 months to keep Lizzy on the trial med she is still on till DR. Wang is published.
Keep us in your prayers .

Tonight's update, sick kids and Extreme Make-over

Lizzy had thick gunk come out of her this morning. I have been doing extra treatments on her to keep her secretions thinned out. No fever , no higher heart rate and she said she is fine. Her treatment lasted a hour longer just for extra coughs this morning just getting her cleared out . Then..... to top it off her cough machine quit working right. The pause button is broken so I have to use it on manual. This is the one she has had for 4 years. I think taking it on the plane and where ever we go is why it got broke in the first place, . The pause button has been broken since we got back from Stanford last trip but now it will not even work unless its on manual. UGH..... Now what? Its a Emerson not a Respironics. We can not live in fear of not having what we need to keep Lizzy healthy. I wish I had the means to get Lizzy every-thing she needs. It always something. Life is pretty good to us though. Their are a lot of people that have it worse off than us. We have it rough but Lizzy is doing okay and we seem to eat and get the bills paid. We dont have much money wise but we have family and great friends that care.
Many prayers to MJ , Conner ,Sophia D and my Lizzy. Also prayers to little Gracee. Gracee had an incident today that scared her Mommy and Daddy.

We watched Extreme Make-over tonight. Good for the Akers family.!! Lizzy watched in amazement. She said "I want to go there" Yeah Lizzy me too!! We would love just to get our house finished with updates "still in progress " let alone handicapped friendly . I am so happy for such a great family to get what they needed for their family. Wow! Great people in Ohio!! Paying off their medical bills and their mortgage? Oh My Wow, that is a miracle alone let alone a new home too. Way to go!! Bless you Akers family.
Prayers for our upcoming trip on Tuesday to CA.

Friday and anticipation.......

Courtney Rosas is in the hospital in Texas is having some issues many prayers for her. Dani Pruit has been sick with Influenza Type B. Mary Kate is still in the hospital in Minnesota intubated, Sarah Van Orden is recovering from a surgery. MJ has been sick this week. Ian is also sick. Many prayers for all the sick kids. Prayers for Courtney Smith's family also.
We fly out to Stanford this next week to see DR.Wang. I get nervous every time we fly. The anticipation consumes me until we get there. We have to do what we have to do to get Lizzy the medicine she needs. I know of two kids that went off the med and got weaker with in weeks of being off it. We are not taking that chance.
How about that that debate last night? I believe the Alaska Governor avoided some of the questions. I think she is a fighter and possibly a good public official for what she did for Alaska but to help run a whole country That scares the bejeepers out of me ...... McCain .....he will take away everything we do have for handicapped disabled children. He is not here to help families like us.

Lizzy has been very whiney today and a pray its because she is tired.

I actually worked out at Curves 4 times this week. I am going to do it ! I am going to get this weight off of me.

Hope you all have a great week-end .

Family in Iowa Needing Help

I say this article and this just upset me so badly. We have met Bill, Kris and Courtney when Lizzy was at UW the first time for her g-tube surgery in 2004. They are such a great family.

DECORAH, Iowa - Meet Courtney Smith of Decorah When Courtney was just a baby, her parents noticed she had some difficulties. Courtney was diagnosed with a neuro-muscular disease called Spinal Muscular Atrophy.
"As the years went on, it's progressed and she's got a lot of respiratory issues like, just a common cold will take her as far down as to be put on life support in the PICU," said Courtney's mom Kris.
The ten year-old can't even swallow her own saliva. Her parents and helpers work around the clock to help her lead as normal a life as possible, but this summer created a whole new challenge.
"I seen the water and looked at Bill and told him, we need to get out of here," said Kris.
Like many families, the Smith's were devastated by record flooding. Their house was ruined, but the Smiths used the rebuilding opportunity to modify their house and make it more user friendly for Courtney.
"We took out a fairly large construction loan to do that," said Courtney's dad Bill.
But just as things were looking good, a whole new problem hit.
"A few weeks ago, I unexpectedly lost my job," said Bill. "I had no indication what so ever that it was coming. We've got 2 mortgages and then the construction loan, and of course there's medical expenses."
"we need to figure out a way to make our house payments and stay here. Courtney can not live in an apartment," said Kris. "We reach a point where we don't know what the outcome's gonna be."
But they say that just like their daughter does every day, they must keep fighting.
"Whatever it takes to do it."
The Smith's say they're struggling to find new jobs and care for their daughter. Bill's sister Karen is raising money to help them get through this tough time. They say and little bit will help. If you would like to help out the Smith's, donations can be sent to:
Bill and Kris Smith
719 Decorah Ave.
Decorah, IA 52101
The Smith's can provide a receipt for tax purposes if needed. You can also learn more about Courtney's disease SMA by clicking here.