Constipation is not fun

It has been almost 6 days since Bella has had a bowel movement. We were giving her half a glycerin suppository once a daywith no result. We also would stimulate her by using a rectal thermomenter but still no good result. I finally could not let her go any longer without having a bowel movement so after talking ot Brandon about it we decided to make her an immediate appointment to the pediatrician to either get her a fleet enema or some laxatives. The Pediatrician felt her belly and sure enough he felt a very hard stool stuck in her. He prescribed her Miralax for her constipation and asked that we give her double the recommended dosage just the first time to help her out then give her the regular dosage once a day everyday to keep her regular. My inly concern with Miralax was that its just a laxative; its not a stool softner so her poop was going to be forced out whole. That was going to be so painful for Bella so I knew I was going to have to research another option.

He also listened to my concern about the Minnie Button infection. I am completly happy with the results that the antibiotic Bactrim has had but I wanted a topical cream to help fight any potential fungus infection on the surface of the skin. He prescribed Nystatin which is a powder that you can rub on the infected sight it it looks red or irritated. Finally, I had a way to fight the infection from both the inside and out. Hopefully noe, i can get rid of this infection for good.

The Pediatrician brought up a shot called the RSV shot. The RSV shot (Respiratory Syncytial Virus shot) is given to babies once a month who are at high risk for severe RSV. Because lung infection is the leading cause of death to SMA children it is important for us to consider any medication that can help prevent symptoms before they start. Im glad the Pediatrician told us about this shot because I had never read anything about it. Its one more weapon is our pocket to help fight SMA! Im planning on bringing it up to Bella's Pulmonologist at our next appointment which is in 2 weeks :)

The very next day we had another appountment but this time with the GI doctor. They looked her Minnie Button and said it looked great. She had a small granuloma beginning to grow under the Minnie Button but they werent conserned. They said its so small that it isnt even an issue. If it gets bogger and begins to obstruct the Minnie Button, the doctor said they can burn it off. Kinda scary but Im glad there is something they can do about it.

I brough tup to them that I believed her constipation was caused by her formula. She was prescribed Elecare when we were discharged and for whatever reason the order was never placed with our provider. I had her on Neocate in the mean time and her consitipation just worsened. Not only does the Neocate and Elecare cause constipation but it doesnt not help her sweating at all and this has become a new issue for me. The GI doctor asked which formula would work best and I explained that the Amino Acid diet was very important for SMA babies and that Vivonex is what almost every baby with SMA uses. She spoke with the dietician and of course she said no. Vivinex is primarily used for children over 2 blah blah blah. They are not experts on SMA nor do they have all the information that I do so it irritated me that they just shot my opinions down so quickly. Im planning on calling the dietician myself and raising hell because its necessary for Bellas care to to first class. Not because I want the best of the best, but because her conditions requires it.

The GI doctor also decided that Miralax wasnt the best option for Bella because it wont soften her stool. She prescribed Milk of Magnesia (a stool softener) at 5mls 3xDay for 2 days. Then 3-4 mls once daily to keep her regular. Once Bella is back to normal and stooling once day without straining, than we can switch her back to Miralax. She explained that Milk of Magnesia will give Bella diarrhea which is what she needs to clean her out, but diarrhea os a bad thing when its not necessary. That is why we will put her back on Miralax after she's nice and emptied. lol

The GI finally placed the order for Elecare and we should be recieving it in a few days. In the mean time, she gave me three cans and I started Bella back on Elecare this afternoon. Hopefully, when I get a hold of the dietician I can get her on Vivonex and put this mountain behind me.

Bella's weight has been increasing at what her GI doctor says is a perfect rate :) She is exactly where she should be weighting in at 13.7lbs and measuring 25.5 inches! I makes me feel good that all our efforts, both her doctors and mine, are paying off!

And for some great news...Bella got accepted as a candidate for the Valproic Acid/Carnitine cliniccal trial at the University of Utah. Valproic acid with carnitine has been shown to stimulate the SMN gene that produced the protein necessary to help with motor function. It is only a therapy, not a cure. It will stop or at least slow down the progression of the disease buying us time to get the Stem Cell Therapies going and has even shown to increase strength in some candidates! I still have to fill out the Parent Consent form and email it back to the University but I don't see a problem with us participating. My dad has alrready volunteered to drive Bella and I so all the money raised buy the car wash and the Astro Burger fundraiser will go directly to that trip! We are making it happen guys! God is happy to see us working so hard and therefore he is providing! GOD IS GOOD!!!!!!

Oh yeah, I know many of you have been texting me and emailing me asking how the Astro Burger fundraiser went. It went GREAT!! We made $2,000 and left a lasting impression on many peoples hearts :) Katie Andrianos's (fundraiser coordinator) uncle knows many politicians and has expressed his willingness to help! He wants to know what kind of funding SMA research needs and what it will be used for and with that information he will do anything and everything he can to raise that money! Im on a hunt to find any and all money starved trials and research that is going on to help cure SMA so that he can get a good picture as to what they money will be used for. People have been surprising me left and right with there generosity and persistance!

This pretty much sums up the last 2 days of our SMA fight. Everyday brings a new battle and a new reason to keep going :)

God Bless,
Bren

Are you with me or not?

Our fundraising efforts have been a huge hit not just with my family and friends but with my community! Im not sure if many of you watch KTLA channel 5, but they aired the Astro Burger fundraiser that was held on Monday, August 24, 2009 yesterday at 6:30pm!! Im not sure on the final amount raised as of yet and as soon as I recieve that figure I will be sure to let you know :)

The car wash on Sunday August 23, 2009 was another success raising $363 and change! The owners of Mr. Petes have expressed there support offering us another day to hold a fundraiser! And this time, every car wash purchased also is given a coupon for a free burger!! I can't wait to start planning that car wash and many more fundraisers! Bella Mia's condition and her daily fight with SMA has been an inspiration to many and together with the help of my family, friends, and communty, we will change the course of SMA history even if its in the smallest of ways.

Nothing in my life has ever made me more humbled or proud as my fight agaist SMA and the overwhelming support it has recieved. I had initially asked for monetary support to save our home and cars and unfortunetly we already lost our home but on the bright side saved our cars. And now that my needs for Bella's comfort are being met by my dad (thank GOD for him) Im ready to give as much of my raised money, time, and heart as I can. Its time for me to answer the phone that has been ringing. My purpose is calling. Is my purpose saving my daughters life? Fighting for her life? Raising awareness for her condition? Going to medical school? As a matter of fact its all of it.

My dad was talking to me last night about the Power of Intent. He expressed how my perserverance has astonished him and to see me get up every morning and continue searching, and talking, and fighting for SMA and Bella has shown him exactly who I am and he has never been so proud. Do you know what that means to me?? My dad NEVER talks like that. He told me to make this my ultimate goal. To visualize my expected outcome and expect nothing less. He belives that so long as I keep pushing, keep believing, keep praying, and keep searching any outcome that I want WILL be achieved. NO ONE to this day has told me that. No one has shown more heart or faith in me than my dad. No one except my daughter. I intend on saving Bella Mia's life. I intend on going the distance and walking the road wherever it may lead me. I intend on waking up every morning revitalized and hopeful regardless of the crap and hurdles I face. And I intend on making this happen alone if I must. Because Bella Mia is my daughter, my saving grace, my purpose and she deserves this.

To all you skeptics who look at this as a joke, as a sure failure, I say take your pessimistic ora as far away from my life as possible. I DONT NEED YOU bringing black holes into my fight for my daughter. I don't need your judgement, comments, or advice. I was raised to get up off the ground when I fall and to keep going. To never let any single person or group tell me what I can and cannot do. I have had many MANY MANY opportunities to dig my own grave and rot but I have crawln out of the pits of hell many times. When every odd was agaist me, I came out alive and successful. If there was something I could do, I did it. And this is just another mountain I am going to move.

If you are going to fight along our side its going to require working side by side with me, faith in God, and an optomist soul. Its time to make a difference. We are going to save Bella and nothing else should matter or get in the way of that.

Many of you have the ability to do more than others and thats great. But if you can't hold fundraisers, car washes, or yard sales in Bellas name a prayer every night holds the same value. I expect nothing from anyone except their commitment to fight along side with Bella.

God Bless every fighters fight. God Bless every prayers' prayer.
Bren

Made the move

Bella and I have made the move to Downey to live with my Dad. I dont wish to go into the details about what happened but what I will say is Frenchie from Grease was right all along, "The only man a girl can depend on is her daddy."

The first thing we unpacked when I got to my dads was Bellas playpen because my poor baby was out and about so late at night when shes used to being in bed by 8pm. She finally got to lay down and fall asleep at 11pm and I must say it looked soooo comfortable!! We finished unpacking the cars around midnight and then my brother Jake and I began organizing all my belongings into the livingroom. I felt so bad that Bella was sleeping in the same room that we were moving around but she didn't even seem to notice. We finished settling in at 3am! And the best part was that I finally got to shower for the first time in 2 almost 3 days!!

Bella did not wake up even once last night! She slept until 10:30am giving me the most sleep Ive had in weeks! It seems to me that her playpen mattress is too stiff for her little body so what I did was place a pillow down with her sheepskin blanket over it. Then I covered it all with a soft blanket and it looked like a little cloud had been placed in her playpen for her comfort :) Maybe this is what had been keeping her up all those other restless nights. She had been uncomfortable sleeping on a hard matress.

Now that we are home at my Dads we are both well rested and both less stressed we woke up happy and ready to have fun! I gave her a nice long bath and even put her little bath toys in her tub for her. I dressed her in the most adorable yellow dress with pink flowers and took her outside with a blanket to play. Over the last 4-5 days I have been really working with Bella to teach her how to roll over. She has been understanding what I want her to do and really trying which overjoys me! Finally today for the first time SHE DID IT!!! She was on her side playing her Lily Counting doll and she looks over her shoulder at the rattle hanging over her, decides she would rather play with that, and boom she rolled over to her back and began reaching for her rattle!!!!!!!!!!!! SUCH AN ACCOMPLISHMENT!!!!

All her doctors said she would probably never learn to graps toys, to straigten her wrist, to hold her head up, or to roll over but you know what I said....SCREW YOU!! SHE WILL AND YOU'LL SEE!! I practiced with her everyday, pushed her, encouraged her, and rewarded her and it paid off. She can straighten her wrists (the right more than left but we're getting there!!) She can grasp her toys. She can hold her head up when support her for 10-15 min. And now she's learning to roll over! My Beauty Queen has beat so many odds and still fighting more! So many people take the slightest things for granted and me...I appreciate them all because with my Beauty, I see just how precious they really are.

Her little infection is still present so know that I switched her pharmacy to one here in Downey my dad went to pick it up and Bella has started it. They took her off Cephalexin and started her Bactrim. Its only twice a day and hopefully this works :)

Also, my Sister Stephanie stopped by to see her lil Princess and it made Bellas day!! They played, hugged, and took pictures and for me it was especially nice for me because I did miss my sister sooo much!! Tonight we are going to celebrate here at my dads and we'll be cooking up a storm and even my dads favorite Upside-down Pineapple cake! CAN'T WAIT!!

Other than all this drama, I have sooo much to update you guys on with regards to the stem cell therapies and the FDA. I dont have time right now to type it allll out but maybe tonight when my Beaty Queen goes to sleep!

Dont forget guys tomorrow is the car wash here in Downey at Mr. Pete's to help raise money for SMA clinical trials! COME OUT AND SHOW YOUR SUPPORT!!!!

God Bless,
Bren

Fun and Fundraisers!

This morning we wanted to show Bella a little more fun that usual so we hung her swing on the tree outside and let her experience the feeling of flying! She is used to going out wheneverpossible and experiencing everything the world has to offer. Unfortunately, her conditon makes that difficult now so her fun is now restricted to cool places with outlets for her equipment and comfortable resting places. Good thing for her...Mama and Papa are extremely creative and nothing limits us from getting her toys and tools that will make a once boring place into a jungle fun haven. We decided to hang the tree swing that FSMA.org sent us and see if Bella liked it. Needless to say...SHE WAS AMAZED AND FOUND IT EXTREMELY RELAXING! LOL

Her first reaction was shock. She wasn't sure what was going on. She kinda looked around trying to figure out what the heck her parents had her doing now. Then she finally got the hint that it was fun time and she immediately relaxed. It looked like she was so comfortable and relieved to be ouside that she sat back and lounged in her little swing as it swayed front to back in the morning breeze of Corona under the most shady and calming tree around :)

We also got word the we have a big package waiting to be picked up and Im thinking it the wagon that FSMA.org has sent us. If it is, then this afternoon we will load her and her suction machine up in it and take her for a little stroll around the neighborhood. Ill definetly bring Rocko and Tucker too because they love walks just as much as Bella does. I might have to Mickey-Mouse an umbrella to it if it doesnt already have one so that the sun doesn't bother her but that wont be to hard.

Tonight we are going to make it a family movie night because Bella can't get enough of Disney movies. Im thinking Happy Feet but we'll see. My girl had quite the workout yesterday because of her two physical therapy appointments so today I wanted to make it as relaxing yet eventful as possible. So far, I think Im succeeding :)

Her "Minnie-Button" is still a little infected so the doctor ordered us a new prescription that we will pick up today. Hopefully this works because from what I hear, treating a g-tube infection is so hard because its mostly always moist :( For now, I have gauze and tape around it keeping it dry, im disinfecting it with a 1/1 solution of hydrogen peroxide and water every time she's finished eating, and making sure she doesn't hit it when she moves her arms around.

Her red marks on her forehead are still there but since we have begun using Neosporin it looks like its been helping not only the healing but the mask from sticking to her skin and irritating it. Here;s how it looks now. Not as bad as before. Geesh, she's so beautiful!!

I keep praying she stays strong and does not get sick because we are but months away from the stem-cell therapy clinical trial!!! I pray that my Bella Mia is a candidate and benefits from the treatment! Even if the stem-cell therapy doesnt fix EVERYTHING, I will be happy if she just beats this disease, survives, and is able to independant enough to move around in a wheelchair. I mean, OF COURSE i would love for her to be completely cured and chances are she just might be but ill be happy with any improvement!

• Don't forget that the car wash hosted my Jessica Perez is this Sunday, August 23, 2009 at Mr.Petes in Downey

Date: Sunday August 23, 2009 from 9am-3pm

Location: Mr. Pete's 12050 Woodruff Ave, Downey

• And also the fundraiser at Astro Burgers hosted by Katie Andrianos is on Monday August 24, 2009

Start Date: Monday August 24, 2009 at 7 am

End Date: Tuesday August 25, 2009 at 3am

Location: ASTRO BURGERS 7475 Santa Monica Blvd, West Hollywood

If you cannot make it to any event and still want to donate email, text, or call me and I will gladly add your donation to the others.

Proceeds will go towards:

• Ordering Bella her Pulse Oximetery that the insurance did not provide
• Catching up on our car payments
• The FSMA.org website that helps fund clinical trials aiming to cure or find a treatment for SMA

Together, we can make a diffence and save lives!! PLEASE COME OUT AND SHOW YOUR SUPPORT!

forever fighting,

Bren

Teaching Bella to read!!

Our first day living with Brandon's parents has definetly been a busy one. Moving things out of Brandon's brothers room, packing up the old apartment, taking care of Bella, and all the other things that come with moving. But, i must say, Bella Mia seems to be having a blast :D

She gets so much attention from her grandma and great-grandma I dont think she has time to realize that she's not at home anymore! She loves cooing back at her grandparents when they talk to her and especially is loving the one-on-one bonding time with her grandma Isa.

We just finished arranging our bedroom and its not as snug as i assumed it was going to be. And a funny thing is we found our air mattress to be more comfortable than our regular mattress lol. Brandons mom has let us borrow some sheet and a comforter and between Brandons parents, Brandon, and I we got settled in quite nicely.

Bella has been sleeping good and and keeping with her schedule but her little infection doesnt seem to be going away. We had gotten the Cephalexin and have been using it like the directions say but it doesnt seem to be taking effect very well. We decided to make her another appointment so that hopefully we can get her a topical cream to help fight the infection from the outside while the Cephalexin fights it from the inside.

We have also noticed that the facial interface for her bipap is really irritating her forhead. She has 2 red spots wear the interface rubs: her hair line and in between her eyebrows. We have tried Duoderm strips, gauze, cotton balls, and yet no good results. We are going to have to invent a little something to ease this pressure because once it breaks skin theres no going back. She MUST wear that interface everytime she sleeps and if she has an open wound its only going to irritate it more. Here's a picture for you to see the irritation. She still looks amazing though :)
So how about a cute little story about Bella to change the tone lol. When she was 4 months I began teaching her sign language. Her condition makes it very hard for her to use her motor and gross motor skills perfectly but I dont let that stop her from trying and me from teaching. I taughter her the sign for "milk" and within minutes she was understanding what I wanted her to do. We practiced it all day and sure enough she had it down! We didnt get to practice it much while she was hospitalized so last night I wanted to see if she remembered how to do it. Sure enough my little Bella Einstein did!! I showed her grandma and she was soo impressed!

Tonight I plan on introducing her to her "My Baby can Read" videos. Im going to emphasize the words she can do with her limited mobility and still try to teach her those she most likely wont be able to physically do. Babies with SMA have shown time and time again to be above average in their cognitive abilities so who cares if she can't show me what she reads, I KNOW she will have learned it and eventually when we get the treatment she will be ready to show me. There is no reason I should stop her from being stimulated and challenged. My Bella Einstein has the brain of a future president so I definitely intend on exercising and utilizing it. THANKS DAD FOR BUYING HER THESE VIDEOS!!

Keep us in your prayers and really emphasize to God that Bella be selected to participate in the Stem Cell clinical trial!

God Bless you all!
Brenda

We moved :(

It finally became clear to us that we were not going to be able to stay at our home so this morning we began packing and have officially moved out of our apartment :*(

We got a storage unit close by and for the next 3-4 months all of our possessions will be there. We plan of living with Brandon's parents and saving every dollar we get so we can pay off our debt and get back on our feet as soon as possible. I guess Brandon's right "Its not living with your parents when you have no other choice that makes you look bad, its getting comfortable and never leaving that does." And even with that said I still can't allow myself to believe it.

I feel like I just failed so bad...like Im just some loser living with her in-laws because I couldn't seem to keep things together. I KNOW IM NOT and I know that everyone at one point in time will need help from parents but why does it have to feel so bad? I see my daughter sleeping in her uncles room with her dad and I on the floor next to her and it just breaks me to pieces. I mean, I have worked so hard in my life and have beat so many odds that seeing myself like this just doesn't feel natural.

Although I understand how lucky I am to have in-laws that are willing to let us inconviniece thier home, I still am just ashamed. Im really trying to divert all my energy into Bella and getting her more options and more resources but days like today really poop me out.

Either way, Im still in Corona and thankfully I at least get to keep my in-home physical therapy and Inland Regional. In-home nursing...gone. SSI benefits...gone. I dont know what God is doing with my life but I hope it all works out in the end. It really feels unlikely considering how my life has been going over th last 2 years but nevertheless I have hope.

All the money that people have and are still raising for us will go to keeping our cars (since we have already been told they are going to repo one of them is we dont pay the entire overdue balance by this Friday). After that is settled, the rest of the funds will go to the SMA website that raises money for SMA clinical trials.

On a good note, I spoke with Dr. Wang (pronouced "Wong") from Stanford University and it turns out his hospital has a program to fly SMA patients and their parents to his clinic at Stanford free of charge. He is willing to see Bella and give his expert advice on how we and her doctors could best help her.

I, quite honestly, cant believe that one of the worlds leading experts in SMA from Stanford University is willing to help but by golly I sure am grateful. I feel sooooooooooooooo good knowing I have landed my daughter in the BEST hands out there. The experienced, knowledgeable, and talented hands of Dr. Wang!!

Tomorrow I plan on printing the papers out that Dr. Wong has emailed me regarding the free flight and setting a date.

Another highlight of my day was that I recieved two packages from the FSMA.org website and non-profits. The first package was a gift basket of SMA-friendly toys, a swing, a childrens bedtime story book , a parents book about coping with a child who has devestating life treatening disease, and a real sheap skin blanket for Bella to sleep on that helps keep away the skin break down and also keeps children that can't really move themselves around while sleeping more comfortable. They sent along with the package a letter welcoming us to the FSMA website and also stated that they were going to send us a Red Wagon for Bella to take walks in!!! SOOOO AMAZING!!!

The second package was a "Car Bed." Children with SMA find it very hard to breath if they are sitting in an up-right posotion so its recommended they be laying down when ever possible. Therefore, traveling in a car seat is out of the question. I had run across an "equipment pool" that was on the FSMA website and saw that they had a car bed perfect for Bella's size. I shot them an email telling them my story and within a week the car bed showed up at my door :D CAN YOU BELIEVE THAT!!! Talk about gracious and wonderful people! I dont know where i'd be had I not come across the FSMA website and group. This group of very intelligent and profound parents has already made a significant change in our lives and it hasn't even been a month since we were diagnosed with SMA and joined their group.

You know, after writing everything out, I guess things aren't THAT bad. I'll eventually get another house, my daughter is still alive, our dogs still love us, and we are still together as a family and making sure everyday that we don't fall into the "pitty me" cracks that surround us.

We WILL get another home of our own. We WILL get Bella the stem cell treatment. We will survive. It just takes time and I have to be patient and faithful.

Before I end this little blog I wanted to thank you guys for following our story everyday.
For sharing it with others.
For donating to help us.
For hosting fundraisers in Bellas honor
For writing us encouraging emails, comments, and letters.
And most of all for fighting along side of The Bearberena Family.

Every breath Bella takes is a gift from the breath of God and you guys make it possible with your prayers and support.

Brenda

It's always darkest before dawn

We finally got charged home on August 10th, 2009 :) We were unexplainably grateful that we were sent home not only with our baby girl but also with all the equipment that we would need to manage her care.

We ended up recieving:
The Bipap ST by respironics with the minime facial interface with the settings of 20/5

The Inexsufflator (cough assist) that the hospital had which made it easy for us to transition to with the settings +20/-30

The Nebulizer for her Albuterol

And the Suction machine

Since we've been home Bella has been as happy and giddy as can be :D She seems to be enjoying the fact that shes back in her room, sleeping in her bed, and playing with all her toys!

The first 24hrs were more managable than I had expected. I guess the hospital allowing us to be so hands-on while we were there really paid off. I have stuck to the routine that we had at the hospital which also has been great because it keeps me on schedule and doesn't confuse Bella.

She's been gaining weight steadily and we definetly are making sure she doesnt plump up because the fatter the baby the more difficult it is for them to move around. And with babies that have SMA we want to preserve as much of their strength and mobility as possible for as long as possible. We are trying to keep Bella at the 10th-30th percentile so her diet is monitored very closely. High calories/low fat is our approach.

The first night we spent at home we had a wonderful and resting experience. Sadly, I cant say the same about the rest lol. Bellas Mickey-Button (also known as the g-tube but we prefer to call it her Minnie-Button lol) got a small infection which caused enough discomfort to have her whimper just about the entire night. We tried cleaning it with a 1/1 solution of hydrogen peroxide and water but it didn't seem to help at all.

Luckly for us, we had a pulmonary appointment today so while we were there we asked the Pulmonologist to fill a prescription for some anti-biotics. Sure enough she did and now we are giving Bella Cephalexin every 8 hours 3 times a day. A side affect of the anti-biotics is drowsiness so hey...it'll help her sleep while fighting the infection! YAY!

We got lucky that we recieved the prescription early because had that infection gotten really bad and spread causing her to come down with a fever we would have been in deep waters. Any cold, even the mildest, it tough on these babies. Not because of the immune system (because Bellas is nice and strong thanks to the fact that she was breastfed since she was born) but because these babies aren't able to cough out any secretions that build up in their throat which can cause pnemonia and even lead to her demise.

Today we also chose her Primary Care Physician which was exciting but we have yet to met him. Ill decide if I keep him or not after our first appointment. I was really looking for a Pediatrician who was experienced and sensitive to the SMA disorder but it was time-sensitive that I get one. Reason being CCS (California Childrens Services) needed me to send back my application and the only blank question was the PCP (Primary Care Physician). And also, the SMA Patient Registry needed the PCP to finalize her application as well.

Housing is definetly still a big concern of ours especially because August 20th is just days away and we still have yet to come up with the money. I know many friends are doing everything they can to help raise money and Im praying that we get it in time. If we don't, then we have decided to move to Brandons parents house and put all of our belongings into storage :( TOTALLY NOT what I want to happen because its going to terminate not only in-home nursing assistance but also my SSI benefits.

PLEASE keep us in your prayers

We also have decided to let the cars get repossessed and eventually save enough money to buy ourselves cars later.

I assume the only benefit to living with Brandons parents would be the amount of money we would save. In 3 months we would be able to pay off all of our debt and have enough money to rent a little home. But this means having to cram the three of us in a bedroom with nothing more than an inflattable air mattress, Bella's playpen, and a small amount of our clothes.

Right now it seems like everything thats important in our life just fell apart, landed in front of us in a messy pile, and God is saying "fix it."

Our childs health
Our marriage
Our home
Our cars
Our income
Our bills
and legal issues

Things could absolutely be worse. Brandon could be cheating, all three of us could be sick, all our our state funded benefits could seize, or our dogs could come down with something so I am defitnitely still thankful for alot of blessing I have. ESPECIALLY THE FACT THE BELLA IS STILL HERE! But, like any normal person I am still very saddended by everything that is slipping between my ever so clinched fingers.

I keep telling myself "Bren, It's always darkest before dawn," but man-oh-man sometimes crawling up into a little ball, hidding under the sheets, and crying until I have nothing left to cry about sure does sound good.

But then I remember why I don't. Because the WORLD'S MOST GORGEOUS, SWEETEST AND BEAUTIFUL (both inside and out) LITTLE BEAUTY QUEEN is my daughter and she deserves for me to fight a fight that has never been seen before. Her eyes tell me to keep it up, her smile reassures me that Im doing everything I could, and her little caresses across my face and her tender grasps of my hands give me the fuel to get up everyday, make more calls, research more options, and ask more questions.

If there is one blessing that God has bestowed upon me that I am most grateful for its my children and all that they bring to my life. It truely is astonishing just how much your children will transform your life for the better if you give them the chance.

As for tonight, Im giving my problems to God and letting him guide me threw this mess. Only he knows what lies in store for me and my family. He is the only one I trust right now and the only one I depend on because God never lets you down...he only make you sweat it out for a while.

I feel ashamed that I tend to seek God's assistance and miracles when Im in dier need
of him. Not to mention how greedy it is of me to expect him to deliver. But he is my father, my creator, and and my savior and he forgives. I'm in good hands with him and with my faith and his guidance, I shall overcome this mountain with the same strength, self-respect, diginity, and confidence that I have overcome the rest of them with.

Please keep us in your prayers and think of us often. A little babies life is on the line and every prayer makes a difference.

God Bless you all,
Bren

New Bipap and nasal interface: Not perfect but will work

Yesterday at 6:30pm we finally recieved the shipment of all the parts for her Bipap. We also got the approval from Bio-med her at the hospital to set up the machne in this hospital setting. We were good to go and ready to start her on the machine and schedule she'd be on at home. Unfortunetly, we were all a little upset by the fit of the mask as it looks like its going to swallow her entire face.

Companies such as Respironics who develop and sell these Bipap machines and masks sadly dont make the appropriate nasal/facial interfaces or machines for children that weigh between .7-30 kg (1-66lbs). Why? Because the market for these types of machines and masks is so small that the cost outweighs the benefit. Ethically, they know they should but financially it makes no sence to them. So, this is what they do.

The make adult "petite" sized masks that still fit and run off of the Bipap machine that is meant for someone over 30kg. These masks are so small I can promise you that there is NO adult no matter how petite they are that is going to fit this mask. Written in the instruction manual it even states "for adult use only". But guess what...they REALLY made this mask for children who otherwise would be S.O.L. when it came to requiring a Bipap machine and nasal/facial interface. Doing this eliminates the demand and necessity of developing a nasal/facial interface that ACTUALLY fits these children and the appropriately powered Bipap to go along with it.

There are rumors that these cmpanies are in the developmental stages of making these machines and interfaces but by the time they are out on the market Bella will be around 5yrs old. Either way, if the rumors are true, then it makes me happy because at least children will benefit from it in the future.

So, back to Bella lol. She is now on her Bipap and nasal interface. Brandon and the RT Educator sat down for three hours last night and mickey-moused a way to get the nasal interface to fit Bella without any leaks. It required some tegaderm and duaderm as well as a bit of interface manipulation but they were able to get it done. The interface will fit her just perfectly in a few months when her face is a little bigger but for the time being, its a size too big.
I tell ya', Brandon and I make a great team when it comes to doing things for our children. He has years of technical and fabrication experience meaning his hands can fix or make ANYTHING work. And not just that, he has such a high level of common sence thinking that he always thinks of the simplest things that we panic-ers are over looking. And me...well, Im the queen of research, options, and information. I can almost promise you that out of all the people and doctors in this hospital, I am the SMA expert. I have a 2" folder divided into sections such as potencial drug therapies, feeding and diet, and equipment and machines. I have scholarly articles on just about every topic related to SMA as well as clinical trial goals, requirements, and results. Together, Brandon and I cover all aspects of her care. Im the Brain. He's the body. And Bella is the heartbeat that keeps us going.

Bella will keep being sprinted from the Bipap machine (at two hour intervals) every few hours and continue being suctioned after the Inexsuffalator. She has such high spirits and is being such a trooper it makes us feel good about our decision to use the Non-Invasive approach.

Quickly, let me share a little story about her intellegence with you. The Inexsuffalator (or cough assist) is a machine that pushes air into the lungs and pulls it back out resulting in a cough. I helps get all the mucous and secreations out of her lungs which if left alone can cause infections or even pnemonia. This machine isn't bad for individuals who understand that they need to let the machine do the breathing for them while its in use. But its hard relaying that message to a baby. Babies will fight the machine out of anxiety and even grow to fear it. Bella already hates it. She just hears the machine turn on and immediately she pulls her hands over her mouth and gives you the saddest "please don't" eyes you have ever seen. And because her eye shape is so angelic as is...it breaks everyones heart :( Talk about associating sounds with emotions. At 6 months she already does.

As for the housing issue, we are still not sure what option we are going to take but I have hope. So many wonderful friends and family members are having fundraisers, asking for donations and even sharing our blog with others to raise awareness and ask for help that I feel so blessed to have. They are trying so hard to get us back in our home as we are and without them it would be impossible.

We ask that you keep us in your prayers and that you share our blog with anyone and everyone. All of us fighting for the same cause can make a difference in a lives of many.

God Bless,
Brenda

Could be going "home" Monday =D

During rounds this morning we recieved the update on Bellas Bipap machine. She will be getting the Bipap ST by Respironics and using the MiniMe mask. Hopefully by 3 pm today we will recieve the shipment and start Bella on her "at home" routine here at the hospital that way they can monitor her O2 saturation levels and heart rate to make sure the equipment they send her home with will work as good as the equipment she is on now.

They have also begun to sprint her off the machine because Bella is still strong enough to breath on her own and maintain high Sat's. So far we have let her off the machine for 2 hours and she did great! Today we are going to take her off the machine twice at 2 hr intervals each time. Then tomorrow will will try three times. We basically want to see how often she really NEEDS to be on the Bipap. The doctors said most likely she will only need to be on it for naps or night time because all babies breath shallower at night and since she is getting weaker we dont want her to work as hard breathing; we want her rest comfortably.

The cough assist and suction machine are basically ordered but we wont recieve those until we leave. The hospital wants to set up the machines at our home as well as perform an assessment on the overall safety of her bedroom. We also are going to recieve nursing assistance through out the day and when a family is provided nursing assistance they require that the baby has its own bedroom. It turns out getting at home nursing assistance is harder that we thought.

First the case manager has to see whether we qualify. They check every option they have to cover the cost such as Medical, CCS, or the insurance: We qualified and we have all three so none of those were a problem

Secnd, the case manager at the hospital gets in contact with an at-home nursing provider. Then, four things must be researched:
The nursing provider in your area must be located
They must have nurses with experience in babies (not just pediatrics)
They must have expeirience and feel comfortable using the equipment your baby is on
They must be available at the hours that are convienient to the family

The case manager here at Kaiser said sometimes a nursing provider will have all but one of these requirements meaning the hunt must start all over again. If everything were to go smoothly, the case manager here at the hospital said we should have assistance within a month or two.

Here is where our next hurdle begins:
*We dont know were we are going to live.

We can go back to our home until the 20th which is when the leasing office is expecting our rent. If we by some miracle have gathered enough funds to make rent, we can stay home. Then, all the at home assessments will have been useful and within a month we recieve our nursing assistance.

If we dont have enough funds, then we will have to resort to having me move in with my Dad in Downey or move in with a very kind lady who lives in Norco and offered us a room. Either way, we will need to get an in-home assessment done again where ever we decide to stay, setting us back another month in assistance. (not to mention all the other set backs I listed below that will happen if I go to Downey).

Either way, im trying to remain positive because I have heard from sooo many of my friends who are gathering donations from family, friends, co-workers, and churches. I am so humbled by everyones help and know that somehow we will make it through this battle like we have made it through battles before.

As sad as it is to say, all the experience my husband and I gained with my son and his fight for life helped to keep us strong, able, and willing. I can feel Bradley. I know he's here. I know he's helping. I know this because sometimes I get this funny feeling in the pit of my stomach at random times throughout the day that make me feel calm and assured. Its the same feeling I used to get when he would give me that deep stare. The stare that said, "Mama, don't cry. It's going to be ok." Funny how he could say so much and yet say nothing at all.

Thank you sooooo much for continuing to read these blogs and for even sharing them with others. I have recieving countless emails and text messages from friends and family informing me that they not only are forwarding our blog to others but that they are doing everything they can to help us financially and spiritually! We are truely blessed with you all and our humbles hearts thank you :D

SMA affecting more than just Bella

15 days into our battle against SMA and we are feeling more than just the medical affects of this disease. We are now struggling not only emotional by trying to keep it together but we are also struggling financially to make it day by day.

Living in a hospital is not cheap. Especially when the hospital is 45 minutes away from home.

*Brandon is on FMLA meaning he's only making 3/4 of his income
*All meals have to be purchased and weekly we are spending $140. We do get vouchers from the hospital but we only get 1 voucher of $5 for the both of us a day.
*In order to wash our clothes we have to drive home every two weeks costing us $25 in gas each time we go (thats not including the everyday miscellaneous driving). We did go a laundry mat and trust me the costs were the same lol
*All the bills such as cell phones, credit cards, and insurances still have to be paid. Recently, we stopped paying our credit cards and deferred our car payments. The deferrements are only good for a month and the credit card payments will cause collectors to start calling. Oh well, we need the money right now for more important things right?
*We cant make our rent so it looks like we are going to lose our home.

If we dont win the lottery or meet a millionaire willing to help us our life is going to take a drastic turn.

*I will be going to live with my dad in Downey with Bella. He has a 2 bedroom house and both rooms are already occupied so the livingroom would be my new home.
*I would place all my belongings into a storage except for Bellas crib and our clothes. All of Bellas machines and monitors would also have to come with me obviously.
*I lose Inland Regional (a community-based, private, non-profit agencies which help people with a developmental disability obtain services and supports that enhance quality of life and increase independence, inclusion, and normalization.) which provides us with free in-home physical therapy. I can transfer to one in LA but I will have to start from the beginning. Meaning, they will have to interview us to see if we qualify, re-access Bella, and then wait the two week period until the physical therapist has an opening.
*My SSI monetary benefit will be re-evaluated because since I'd be living with my dad, his income would be taken into consideration and most likely Id loss some

The reason I'm posting such a personal side of our struggle is because many of you so kindly have asked how you can help. As of now, all I had asked for was prayers but now, unfortunately, I must bring myself to ask for any type of monetary donation or assistance you can offer. It is soooo important that Bella has a stable home and life to go back to.

If you can help us please mail a check or money order to:
8726 Mccahill St
Downey CA 90241

or

call me so that I can give you my bank account number. This way you can do a direct deposit

I hate to have this sound like you ordering oxy-clean from an infomercial but I couldn't think of how else to say it :( I do feel so sad that it has come to the point where I need to ask for any type of assistance with money, but thats the reality of my situation. I feel compelled to try and keep things as normal as possible for my little Bella and her home is one thing that brings her peace.

Thank you in advance for helping in anyway you could. And please keep up the prayers!!!

God bless you guys!
Brenda

The First 14 Days...

Welcome family, friends and visitors,

I decided to begin a blog that I will update as much as possible with information regarding our families battle agaist SMA (Spinal Muscular Atrophy) that has affected my daughter Bella Mia. I bet your wondering "What is SMA?" Spinal Muscular Atrophy (SMA) is a motor neuron disease as well as the number 1 genetic killer of babies. The motor neurons affect the voluntary muscles that are used for activities such as crawling, walking, head and neck control, and swallowing. It is a relatively common "rare disorder": approximately 1 in 6000 babies born are affected, and about 1 in 40 people are genetic carriers.
SMA affects muscles throughout the body, although the proximal muscles (those closest to the trunk of one’s body - i.e. shoulders, hips, and back) are often most severely affected. Weakness in the legs is generally greater than in the arms. Sometimes feeding and swallowing can be affected. Involvement of respiratory muscles (muscles involved in breathing and coughing) can lead to an increased tendency for pneumonia and other lung problems. Sensation and the ability to feel are not affected. Intellectual activity is normal and it is often observed that patients with SMA are unusually bright and sociable. Patients are generally grouped into one of four categories, based on certain key motor function milestones.

My daughter Bella Mia was diagnosed with SMA type 1 on July 21, 2009. I had her tested because since birth I noticed a few odd little things. For example, the day she was born I noticed fasciculations with her tongue when she would cry. I also noticed she sounded congested and when I brought my concerns up to the doctors I was tld this was normal and would go away with time. He said she might just be a heavy breather. Fair enough, right?

At two weeks of age, I noticed she wasn't trying to lift her head at tummy time nor was she kicking her feet around much. I brought this up to the pediatrician at her first well-baby visit and the pediatrician said she might just be a late bloomer and even a little lazy. I accepted her advice but something inside wasn't satisfied. A few days after that appointment I began noticing Bellas weists were dropped the majority of the time. I decided to get a second opinion from another pediatrician and finally someone began noticing these little abnomalities as well. She recommended I see a nuerologist just to be safe. So we did.

The first nuerologist was a little unsure of what it could be and ordered a plethera of blood work and CT scans as well as physical therapy. Again, i didn't feel he was right either so I decided to get a second opinion from another nuerologist because I felt like the more eyes that can see her, the better chance I have of someone getting it right. Plus, I wasn't about to have my daughter get sucked dry to complete his blood work orders. Especially because he wasn't even sure what he was look for.

Our first appointment with the second nuerologist went well. The nuerologist said he saw a bit of low tone but nothing too concerning because since she was only 2 1/2 months. He didnt expect her to move all that much anyways. He said he thinks she had a classic case of wrist drop caused by a viral infection she caught as around 3 weeks old. He explained that what could have happened was that her antibodies fought the infection off which is why we didnt notice anything. Unfortunetly, her antibodies fought so hard they accidentally damaged the nerves in her wrist. He stated that the nerves would repair themselves over time and in the meantime we should continue physical therapy and also he recommended we see Occupation Therapy to order her some splits for her little wrist. We felt relieved and went ahead and did as he said.

At 4 months, she had already had 1 1/2 months of physical therapy and I was not seeing much of an improvement. I assumed after having physical therapy 3 times a week, not to mention constant stimulations by me at home, she would at least be holding her head up at tummy time. Sadly, no. As a matter of fact, her strength was not improving at all, it was diminishing. I began noticing her suck was growing rather weak causing her to take in less milk than before. She grew tired quicker and more and more milk would leak out of her mouth when she nursed. By this point I was scared and decided to contact the nuerologist again. He saw her for a 2nd timea but this time at 4 1/2 months. He said he definetly did see low tone now and he too was growing worried. He decided we test for SMA. He said the chances of it being SMA are slim because it is such a rare disease, but nevertheless he said we should rule it out. We got her blood drawn and 10 days later I recieve a phone call from the nuerologist asking me to come in to discuss the results.

I was already expecting to hear the news so I had gone into the meeting with ideas to battle this condition and support her in any way I could. He was so stubborn, negative, and not willing to hear any of our ideas. I was saddened and scared so I decided to take matters into my own hands. On July 21, 2009, he admitted Bella for failure to thrive because she was loosing weight due to he inablilty to swallow. I immediately began researching the first steps I shloud take to be proactive about her condition. I decided to go with the g-tube because I knew this condition would only make her weaker as time went on so there was no sence in me being unrealistic and hoping she might get stronger and eventually eat orally again. While researching, I came across many clinical trials that had been complete by a doctor at Stanford Univeristy. I was thoroughly impressed with what I had read and I decided to contact his office and try to get advice from an expert. I knew my chance of getting him to take my call were slim, but I decided to try. After all, my daughter deserves this! I left a message and less than 24 hrs later he personally returned my call and guided my through the overwhelming decisions I would need to make.

He explained that the new Standard Care for this condition is not widely recognized by hospitals so he gave the link for me to physically print it out and provide it to the doctors. He also explained that this disease is sooo close to getting a treatment that will give these children a normal quality of life. He said if we could just keep Bella going using his recommended Non-Invasive Approach, there should be no reason why she shouldn't be around to benefit from it! I was filled with such hope and did just as he said. I vigorously researched this NIV approach and got a list of all the necessary equipment I would need to manage her care.

On July 24th, 2009 we had a meeting with 9 doctor, case managers, specialists, and surgeons (Nuerologists, Orthopedics, Genetisists, Gastroenterologist, Pediatric Surgeon, and thats just to name a few). They explained that they would like to place a gastrostomy tube and we agreed. I then explained to them that I felt the Nissen Fundoplication procedure would benefit her because it would help keep the reflux that commonly causes aspiration in children with SMA down. They said that they didn't want to because they usually perform that surgery if its absolutely necessary but if I wanted it, they would. I was shocked that they weren't planning on perforning the Nissen! What were they going to do, wait until an emergency situation arose then perform another surgery?? Not to mention that she would be weaker by that point in time and might not be able to ever be taken off the ventalator!!! Either way, they wen't with my suggestion so I was pleased! I also expressed my concerns with her home care and explained to the doctors all the machines that we would need at home to manage her. They aggreed and said they would provide us with everything we needed and the meeting ended with everyone happy.

On Monday, July 27th, 2009 Bella went in for surgery. Everything went perfect in surgery but once she was in recovery, we did notice a bit of retractions in her breathing. After ording Chest PT and doing a few days worth of percussions, she seemed better. The day we were being discharged, we were told by the doctors that all the were going to get to manage her care at home was the pump that we would need to feed her threw her tube. We were flabbergasted! WHAT HAPPENED TO THE AGREEMENT WE ALL MADE IN THE MEETING! If anything, we neeeeeeeded the suction machine but, nope, Kaiser denied it. We went home a little upset but we figured they knew better than us if she needed all the machines we had requested.

We spent 27 hrs at home and guess what...to no ones surprise, we ending up back at Kaiser because she was retracting and it seemed like breathing was getting difficult for her. Also, her secreations had gotten so bad we could not handle them with the blue bulb. We neeeeeeeded a suction machine. We were back at the hospital, now in the ICU. The finally put her on a Bipap machine like I had request and i couldn't believe the Bipap machine they had. It looked like it was from the 80's and the nasal pronges were sooo long they made her nose bleed!!! I had had enough! I researched all my options to get her transfered to a childrens hospital. Let me just say it was a battle in itself but we got it done.

Hurdle 1: Of course Kaiser did not approve transferring her and covering all the expenses. They denied both of our attempts.

Hurdle 2: I applied for CCS (California Children Services) and was going to try through them. Unfortunety I would have had to wait for 10 days to see if we even were approved by them. I didn't want to wait that long because the nature of this disease is the children get progressively worse. There is no time line, things can take a turn from the worse at any given second so time was the enemy.

Hurdle 3: My next idea was to apply for Medical and drop her from the Kaiser policy. I applied for Medical, got it expedited due to her condition and approved. Then i realized that if I dropped her from Kaiser, another insurance provider might not cover her due to her pre-existing condition. After calling my husbands insurance company and seeing if they would allow us to go from our Kaiser to a PPO due to life circumstances and still have her covered, they agreed and did the switch for us! YAY!!!!!!! SUCCESS!!! We got CHOC on the phone and transferrred her!

On July 31st, 2009 we arrived at CHOC (Childrens Hospital of Orange County) and we're soo proud of our accomplishment thus far! Kaiser had only seen 2 cases of SMA in the 60 yrs of experience that our 8 doctors had combined. CHOC had seen 5 cases in that last 6 months! Kaiser did not have a bi-pap machine, they did not have a cough assist for her size, and they also did not have the VEST to help with the chest physiotherapy. CHOC had everything!!! With in minutes of arriving, they started her on a bi-pap and immediately we could see her comfort level rose!

Within 3 days we already have set a "plan" with the doctors for her care. It is as follows:

Bipap machine will be provided by Apria. Apparently, it is a Bipap machine used for people over 30kg (66 lbs?) but its the Bipap machine that Dr. Wong (a leading expert in SMA from Stanford Univeristy) suggested. So far we have encountered problems with the mask size. They decided not to do a full mask bc it wld be impossible to handle her secretions. But the nasal mask has also been a problem bc she's in the akward stage where shes not a newborn but she also isnt a toddler. The nasal mask is either clear from bothering her eyes but it almost swallows her upper lip or we move it up to clear her mouth but then the air leaks out of the mask into her eyes. We now have a smaller nasal mask with a less-intense machine and Bella seems soo much happier and rested. She doesnt look like she struggling to breath at all :)

The Cough Assist was ordered for us. Again, it will be provided by Apria and the make and model wont be determined until my daughter has been fully evaluated. Either way, these are basically all the same, the only difference would be the settings and intensity of the machine.

A O2 saturation monitor was ordered but im not sure exactly what it looks like yet. Im pretty sure it will just be a little sticker-like wire that will go on the bottom of her foot or on her thumb similar to the one they use at hospitals.

And lastly...A suction machine (which is sooooo helpful bc the little blue bulb was almost useless as it couldnt keep up the secreations!)

I know these machines are vital for their comfort and survival but the right machine with the right apparatuces will make all the difference which is why I'm doing so much research on which machines, masks, and accessories other families have used. We are determined to find the equipment that fit her like a glove :)

As for her formula, she was breastfed from birth until we recieved the diagnoses. We had to begin feeding her special formulas with a higher concentration of medium chain fatty acids than long chain fatty acids because they are easy for her to digest. At first we had her on 22 calorie Neocate. But after reading about the Amino Acid diet and all the other formulas such as Pediatric Tolerex or Vivonex, I began researching the best one for her. I did read the Vivonex help make the baby less sweaty, causes less tacky saliva, less fussiness after feeding, higher O2 numbers, and a lower heartrate. She seem to be tolerating Neocate well but she is still very sweaty. She sat's fine, never has fussiness after feeds, and doesnt have a high heart rate, but her sweating is a lil bad :( I brought my concerns up to the nutritionist and she seems to believe that Elecare will be the best for Bella until she can further research the advantages to Vivonex or Tolerex. She said that Vivonex and Tolerex were normally given to children over theage of 1 so being that Bella is only 6 months, she wants to make sure it will be ok and easily tolerated by her.

I also got in contact with California Stem Cell Inc and found out more information about the stem cell clinical trial. I wanted Bella to be a candidate bc she qualifies based on her condition and the fact that we live only 20 minutes away from the city in which it will be held but unfortunately they choose candidates strictly from the SMA patient registry and it goes by the first come-first serve basis. The first 8 candidates on the registry that qualify based on the criteria will be selected. Thats not to say that the first 8 WILL be selected because if they do not fit the crieria they will skip that name and go on the next one. Either way, CSC Inc said that if all goes well with the FDA's approval and no major hurdles come their way, they should begin pulling candidates by mid January. THERE'S HOPE!!!!!!!

So thats where were are today. Tomorrow is another day and more phone calls will be made so I can make sure I have all my options presented to me so that I make select that one that works best for Bella. We are going for the NIV approach but then again, if worst comes to worst and we are left dealing with "should we trach her and keep her with us for a while longer hoping that a treatment will be out" or "should we make peace with our fate and let her go," we will without doubt trach her. This fight is just beginning but at least this time we have a diagnosis, we have a way to manage the condition, and we have a little light at the end of the tunnel due to all the clinical trials and the success they have been having thus far! All we can do till then is make the best and most educated decisions and pray to God because ultimately its his decision.

God bless all those who have to live with SMA both directly or indirectly,
Brenda Barberena
Bradley and Bella Mia's PROUD AND FIGHTING Mother!!!!
Brenda