Fall is almost here and what a LONG Summer- Living with Spinal Muscular Atrophy

School has started in much of our area. Lizzy will not be starting till after labor day which is fine with us.
Lizzy is excited because she is reading some things and wants to show off to her teacher.

Its been a long exhausting summer to say the least with all that has been going on in our lives.
Paul is healing and starts physical therapy on Thursday this week. Hoping he will be able to get a job soon as he is able.
Mom is finally back to herself mentally and is walking to and from the bathroom. The nursing home that she is in is the Good Samaritan here in Pontiac. They are doing an wonderful job and she has come so far in a little over a month. The nurses and aids are fantastic. I feel so much better she is doing better.
We had a busy last week as we went to UW in Madison twice. One for the SMA Education Day and other for muscle clinic. We were on a panel about traveling with Lizzy. We have traveled all over with Lizzy that is for sure. The ER info was very good.
We met with some great families in Madison. It was two fast trips. Lizzy and I enjoyed it because we GOT OUT!!
Now, the UW Muscle clinic day. It was a great day until the end. Lizzy is stronger than last time. She had the OT awed in watching her. The PT Karen was also excited about Lizzy also. DR. Schroth said Lizzy sounded great. Big difference between the RSV hospital stay and now DR.Schroth said.
The dreaded Xrays on her back and hips. Weeeeeeeell, Lets see how to explain this. Her curve is worse but........ her hips are great . The cons of doing spinal growth rods are a serious concern. Will it help her? Maybe a little,. Will it will stop her progression of her curve? Yes, but  it will not change her rib deformity and  she will lose turning her head side to side easily and will not be able to go on her exercise ball and stretch. Is her breathing affected? NO not at this time. Christen says,"Not at this time." DR.Schroth did not come back in and talk to us after Christen and the Ortho talked.  I am afraid we have upset her. I hope we can make Lizzy's  back better like we did her hips. Compared to April when she had RSV she is better but not since last year when she had her curve checked out.
Many prayers to the Lee family . Jocelyn lost her battle with SMA http://www.jocelynpaigelee.org 
and another little girl  Skyler Jones www.blog.skylarmariejones.com  lost her battle with SMA also. We need to find a cure soon!! Too many kids have lost their lives to this devastating disease.


Keep us in your prayers. 


Thank you to all of you that are in our lives!! 


" We believe in miracles because we live with one!"


www.our-sma-angels.com/elizabeth 

Finally, a much needed break - Living with Spinal Muscular Atrophy Type 1

It has finally cooled off here just a bit.
I just finished helping Paul in the shower. He is doing much better and healing well now.The incision area has shrunk and the hematoma has gone down. He still in some pain but not nearly as bad as before. That man scared the bjeepers out of me. He is a whopping 145 lbs now! The man eats more than me! I need his metabolism. His sister Sharon is skinny like him also. She is not a diabetic though as far as I know.
He is doing much better!This was an extremely bad accident and  he is suffering from EXtreme Boredom!

My mom is doing great. Totally back to herself mentally. She was incoherent for about 2-3 weeks  I am in charge her medical and I have her off the pain meds totally during the day and only one at night when needed.. I have her on Ensure 2x daily and she is getting really great care. Gives you some peace to know she is so much better just with a few changes. She is getting new dentures and if she has to pay more than her copay my siblings are going to help. Yay!! Life is getting better.

Lizzy has discovered Skype.  She was online with  Emma and her brother Nick for two hours the other day. Then, Aubrey another SMA friend from Louisiana. It was so cute listening to her. She is so excited.

Lizzy is doing pretty well these days but I still worried about her back and ribs. Seems everything I do is not enough  in my opinion. I work on her a few times a day and I have not given up but I do NOT want her to go through a major surgery. She is not complaining at all.  We are done with swim therapy and have to come up with more goals we need to achieve to keep her in swim therapy.

We go to Madison on the 12th  for a SMA Family Day at Madison on the 13th. Christen is going to speak on traveling. Not me. this time. Then, we come back for two days and back up there again for Lizzy's Muscle clinic. That will be the biggest plan trip we have gone this year so far. Then Lizzy, B-day week in September we will be in Minnesota visiting some close friends! We can not wait!!

Lizzy starts home school in a few weeks. She is so excited!!

How about this good post for a change!!

Thank you that have help us through the tough last few months!! Thank you Shannon and Justin for dinner !!
Thank you Barb M my dear friend for helping me get through the last few months with out you I do not know I would of got through!! Thank you to the Oltman Family!  Thank you Molly and Andy for the great Ham!! Thank you all for the prayers!! Thank you for caring!! I will be sending you all thank you cards soon as I get caught up!

August is SMA awareness Month. Please light a candle to honor the kids that have lost their lives fighting Spinal Muscular Atrophy August 13, 2011
Candlelight Memorial Event to Honor SMA Angels, 7:30 p.m. to 9:00 p.m.

"We believe in Miracles because we live with one!"
www.our-sma-angels.com/elizabeth

Hot Hot Hot !! Living with Spinal Muscular Atrophy Type1

Oh my goodness I do not think its ever been this hot here for this long. Three window air conditioners on and I am still sweating buckets!!
Lizzy is doing great, Paul is healing and my Mom is doing much better!! I have come to the point that I am saying "Whatever!" to almost every new incident in my life. I seems if do not get stressed out over the situation  it becomes resolved faster.
Paul's surgery staples are healing well and no redness around the area. That is a good thing considering he has POISON IVY now too!! He is still very sore and very cranky these days too.
Lizzy has been doing the "Whine Voice" when she gets hot and tired and that has been hard on all of us too. Bless her heart, she is a great kid but when she gets that way until she falls asleep her "whiney sound" sends a screeching vibration to you inner ear and the pain is so intense.
My mother is doing so much better this week. She is coherent now and is getting her strength back. I am a head of her medical care now legally and I have them cutting back her pain meds successfully. I have them giving her Ensure in between her meals. She has an appointment on Tuesday with a dentist to get some new bottom teeth. She lost them the first part of the month and that is one the reasons she lost so much weight and was so weak. I made her appt for Tuesday to get a fitting for new ones. She was NOT being taken care of at home the way she should of been. She was being over sedated with no help feeding her.  God knows I have a "Full plate"  just taking care of Lizzy and Paul but I have managed to care for all three of them. I was with my mom on my birthday, her birthday and yesterday. Lizzy loves going out to see her. There are only 38 residents at the nursing home and Mom actually likes this nursing home much better than the one a block a way from me a lot better and SO do I. I feel much better knowing I have done what is best for her.Rachael, Robby, Lizzy Christen and I went to go see Mom on her birthday.
I am okay. Hopefully, the assistance we have gotten from the hospital will cover Paul's bills for his injuries. The amount exceeds $28,000.00. We were unable to get help through the state as we do not have a minor child or are we over the age 65. I had an interview last Thursday and we did NOT qualify for medical. We can not take the chance to put Lizzy on with us because if and when Paul does get a job and health insurance she could totally lose her coverage. She needs to keep hers. I also inquired for help with Community Action with utilities and there is no funds left to help us with that until November. Chin up!! We will get through this and we will survive.
Some more interesting things have happened to us this week but instead of saying I am" lethal " like I have been its a "What ever....
A thank you to our friends for being there for us when times are rough for others" I another SMA family was stranded on their way back home and it took 10 minutes or so after a face book post and they had help!! Amazing what SMA families can do when we work together!!

My prayers to my cousins Connie, Peggy and Billy for the death of their father and my Uncle Bill http://www.duffyfuneralhome.com/fh/obituaries/obituary.cfm?o_id=1211178&fh_id=10960  . I had lived with Aunt Ginny and Uncle Bill when my mother was ill for several months as a kid and it was one of my best times in my life.  I so lucky to have him in my life at such a young age. I loved you so much Uncle Bill as a child and thank you for all you had done for me doing that time. I remember he was an interesting person and my aunt loved him with all her heart until the day she died.  God speed Uncle Bill. He would of been 90 today. I was unable to attend the services today.
Thank you all that are in our life.
Prayers to Jacob who is in the hospital in Madison and to Julia who just had surgery and on her way home.
" We believe in miracles because we live with one!"
www.our-sma-angels.com/elizabeth

Taking a Deep Breath..... - Living with Spinal Muscular Atrophy Type 1

As, I wrote in my last post husband had a terrible four wheeler accident on the 4th and I have not been online too much. He broke 6 ribs, collar bone, scapula,and fractured pelvic bone. He had surgery today and a plate put in in his shoulder. He is recovering but the doc said yesterday because of all his fractures it will be 9-12 weeks before he will be able to do much after extensive physical therapy.
My positive attitude left me for about 3 days after this last bit of bad karma hit us . I had to realize things happen and I  climbed out of that hole that was sucking me down in it. I am back up and realizing how I let my stress consume all my inner thoughts and control me. Never going to happen to me again.
Another,  situation has ended up in my life with my mother. She has given up and was placed in another nursing home today. Christen saw her and she said "She has never looked so bad". I shed some tears tonight but only because I am feeling guilty and my heart hurts because I was not able to be with her. I was sick during her last hospitalization .  I had sinusitis and allergic bronchial asthma. I was a really sick.  No,way I was going to give mom this illness if it was contagious. I wore a mask at home with Lizzy. Then , Paul's accident. I hope I am not the reason she gave up. That will haunt me the rest of my life. My mom has not been easy to live with since I was 7 years old. Lots of mental illnesses. I think that is why I am so strong minded because I did not want to have my family go through the life  I went through. She has been having bad pain issues since Christmas. I do not want to lose her but she is in such great pain these days. Will she regain her strength or will she die in the nursing home? I am so torn right now. I take care of Lizzy and have been taking care of my husband its been hard to get my mom the care she needs. Am, I supposed to care for her too? Should I go get her and take her home with me? She can not be just dumped off like a stray cat in the country, I need to pray deep to find the answers I seek. I do not know what to do.
Lizzy is my light she keeps me sane. She is a sweet incredible child.
Our trips for this month are as of now cancelled. That really blows. but..... what can you do? Family has to be taken care of first.
Please keep Paul and my Mom in your prayers.
" We believe in miracles because we live with one!!"
www.our-sma-angels.com/elizabeth

I know God won't give me anything I can't handle. I just wish he didn't trust me so much." Living with Spinal Muscular Atrophy

I thought the summer was going pretty smooth but seems like things keep happening all the time, Makes me wonder if in my past life if I was really bad or something. I am truly not a "WHINER" !! I know I try to stay as positive as I can but with the situations that keep finding their way to us makes you wonder. As,  Mother Teresa put it best "I know God won't give me anything I can't handle. I just wish he didn't trust me so much."  Mother Teresa was a wise woman . I have used that quote before because I think about that all the time.  I have to endure a lot the past few years. At some point we all face trying times and feel powerless to be able to get back on track again. If you give up and quit trying you will not get back there. I seem to be doubting myself more and more these days. Then, I pick myself brush myself off and continue on. Why I am I like that? I think its because of the life I have had since a child and because Lizzy helps me realize I can not just throw in the towel. I lost my arrogance from earlier years and replaced it humility the older I get .  If that makes sense.  I take each day as it comes. 


Its been a rough year. My husband does seasonal outside work like mowing, cutting trees down, weed eating etc just so, we can get by. Twelve bucks an hour these days is not the best wage in these trying times but it helps pay the bills. My mother has literally emotional drained me. This last weekend was bad with her. She is not well  but.. she  has went to the extreme recently. I think a lot is she wants the attention. With her recent mental frame of mind it makes it harder every time to deal with her. She has been as bad as a child. Well, worse !! Lizzy is a child and she does not act like my mother. I love my mom but too much is too much . Then, the event that happened this weekend to my husband was something I prepared myself for many years ago expecting it to happen  a long time ago NOT NOW. He is a rebel. No doubt why I married him was for the wild life he led. We have been married since Dec 5, 1974.He is 57 !!  He has mellowed with age. ....  I thought.   NOT!! He was out at my daughter's house and he flipped the 4 wheeler. He was taken by ambulance to our local ER and we spent 7 hours there last night. He had been laying across the lake for over an hour and half alone with out anyone hearing him whistling .  He finally found his cell phone called one of my girls and  they all ran to his side. Leaving me with Lizzy and freaking out on what was going on. He has a broken collar bone, 6 broken ribs and a fractured pelvic bone. He will be fine after weeks of healing. Yesterday,I was concerned for his life . Now today... I am concerned about how to pay the bills next month and the months after.  I get paid just a bit over $600 a month. The income tax we have been living on is almost all gone because we have lived on that also. What are we going to do? I am going to try to do his job mowing myself. Lets see. I need to lose some of this carb weight I gained . Might be fun or it might kick my hiney. 
I had made home made pulled pork, cole slaw and potato salad for Dawns on the forth where we were at when his accident happened. Since,  we were at the hospital my food was all taken home  with people!!  LOL At least they all liked my cooking. (Trying to add SOME humor to last evening.)

Lizzy was coming down with something Saturday and since we caught it quick "nipped it in the bud sorta speak" she was doing great yesterday. She is pretty tired today because she stayed awake  last nightwaiting for me to say prayers and said "She really missed me." Sweet Child. Bless her heart.

Paul is slightly better tonight.  He wound on his pelvic bone looked a bit better. I have to clean his wounds. I did it but it took everything I had but I did it !!I dont usually do wound duty!!  I get all light headed doing any kind of thing with the skin, blood  and bone thingy..... ewww.  The docs last night are worried about pneumonia with the 6 broken ribs.. Paul says" This is about as bad as taken care of your Mom" I sad "No, Paul I am supposed to take care of you when you get hurt." This does not bother me. You are my husband. I said "I can take care of you and Lizzy no problem. " WE WILL GET THROUGH THIS!!

The Casey Anthony Verdict shocked the heck out of me. I do not believe it. Are these people crazy to let her back out on the street. Makes no sense to me. Now , they will probably make a movie about her, write a book and she will be a millionaire. Commit a crime , become acquitted and become a millionaire. I see something REALLY wrong with this picture don't you? hmmmm?  Just hmmmmmm?

Please add Paul to your healing prayers. Also, add Julia Kay who has Spinal Fusion on Thursday.

One tip: Use : 100% Pure & Natural Ubiquinol as Coq10 supplement. It gets to the blood stream faster and is better for you!! I get the gels and poke it with a lancet and put it in Lizzy's food mixture while making it. I use 50mg Swanson Brand and going to up her very soon. 
"We believe in miracles because we live with one!"
www.our-sma-angels.com/elizabeth

Summer so Far- Living with Spinal Muscular Atrophy

Its been a totally interesting  week so far. I caught Lizzy online playing poker today. Yeah, I said poker. Not sure if that is a good thing or a bad thing. I took her off the site and we found another web-site that she liked that was not poker. Too smart for her own good. She has wanted to go outside in her power chair last few days but it has been storming here.
Sunday, Oh, let me tell you about Sunday. We had a cook out and Lizzy's dad was here and Caitlyn. Christen and Brandon went to go get Corbyn at Dawn and Jeff's for the night also. Well, to make a long story short at Dawn's and Jeff's Brandon was upset and flipping disappeared. They live on a lake with 300 acres surrounding them. Brandon was slightly upset over a situation and left the door to the truck open, wallet , key to his room, inhaler and you name it he left it all in the truck. He disappeared for hours.  They drove all over in the Polaris looking for him and he was no where.  Christen looked for him for 4 hours. Lizzy told me " He did not go any where and he was fine and we needed to go to bed " Lizzy was right.. After hours of Christen,Dawn and Caitlyn freaking out he texted Christen and said he was in the truck driving home. What an ass. How selfish is a person to put others through this? I think he was in the corn field watching everyone freak out there. I was not freaking out. I was to the point I did not really care anymore.  I knew he was fine and messing with every one. Needless,to say it was around 4am when we got everyone settled down here and to bed. Again,  I say "What an ass!!  Thank goodness I do not have to live with the guy!!
Its conference time and we were are not able to attend . Its  okay we have a trip next month that will totally make up for it.
Lizzy is enjoying summer on days it doesn't rain!!
"We believe in miracles because we live with one!"
www.our-sma-angels.com/elizabeth

The Amino Acid Diet and Living with SMA - Living with Spinal Muscular Atrophy

My very good friend Mary wrote this info up to share with families to help them understand the Amino Acid Diet. This is the updated  version. Fantastic job Mary!! Thank you for doing this !! So many more babies, children and adults can benefit from this information.

This is only info we are sharing and you should be followed by a diet professional when changing to any diet when you have a child with SMA. 

What is the Amino Acid Diet?

By: Mary Bodzo

This information is based on opinions and experiences compiled from parents of children with SMA and should not necessarily be relied upon as an alternative to medical advice from professional healthcare providers.

ABOUT THE DIET

The Amino Acid Diet is a nutritional approach that many SMA families believe has had a positive impact on their child’s quality of life. Using this dietary approach, milk and soy based formulas are eliminated and substituted with a low-fat, elemental free form amino acid formula. Elemental formulas are made from nutrients in their most broken down form, which require very little digestion. Tolerex and Pediatric Vivonex, from Nestle Nutrition, are the two formulas used most often. EO28 Splash, from Nutricia North America, is a ready-made, more palatable, amino acid formula sometimes preferred by children without g-tubes. Because the EO28 Splash contains a higher fat content, it may not be an appropriate formula choice for children with Type 1 SMA. These formulas contain vitamins, minerals, carbohydrates, protein and some fat. The protein is in the form of free amino acids. These amino acids are not derived from food sources. Most parents that implement this diet believe more benefit is realized with these elemental formulas, rather than hydrolyzed amino acid formulas, which contain broken down proteins from foods, such as dairy or soy. The free form amino acids in Pediatric Vivonex, Tolerex, and EO28 Splash, are in balanced amounts, and are less likely to trigger immune responses such as inflammation. They are easily digested and quickly absorbed into the bloodstream. When given frequently, in low amounts, these amino acids provide a steady source of accessible energy and may help prevent muscle breakdown.

Many children with SMA are very reactive to the protein and fat in dairy and soy formulas. Their reactions are often attributed to their SMA progression. However, when this protein and fat are replaced with free form amino acids and appropriately reduced amounts of fat, children with  SMA  experience  improved respiratory health and fewer metabolic complications during illness. Most children with Type 1 SMA have an immediate reduction in airway secretions. Constipation, which can be a major complication, is more manageable or even eliminated. Some children have improvement in strength and regained function.

Parents using these formulas vary their child’s particular diet based on tolerance, age, weight and length, and severity of SMA, but there are some basic guidelines that most children respond to favorably.

Children with SMA do not tolerate large amounts of amino acids or fat due to their reduced muscle mass. This reduction of muscle mass prohibits the proper absorption, storage and utilization of amino acids and fat, which could result in toxic accumulation in the bloodstream.

• More than approximately 2 packets of either formula, over a 24 hour period, may cause increased heart rate, arrhythmia, and/or gastrointestinal distress.
• %  of fat calories should be kept at the minimum requirement necessary for adequate essential fatty acid intake
• To avoid fat, protein, and calorie overload, these formulas should never be combined with soy or milk based formulas.
• Children should be monitored with the following laboratory testing: quantitative amino acid profile, essential fatty acid profile, complete metabolic profile

ADDITIONAL SUPPLEMENTS

Because these formulas aren’t used to supply complete nutrition in SMA patients, additional calories, vitamins and minerals must be added to the diet to ensure that their daily requirements are met. Special attention should be given to calcium, magnesium, sodium, and potassium intake.

Commonly added supplements include:

• Multi-vitamin and mineral– NanoVM from Solacenutrition.com  (may only require half-dosage)
• Additional Vitamin D3—this level should be monitored for adequate dose
• 1/8 to1/4 teaspoon salt
• Probiotic—Floragen 3
• L-glutamine powder—(200-300mg/kg/day, not to exceed 10gm.)

Tolerex contains 1060mg/pack, Pediatric Vivonex contains 774mg/pack, EO28 Splash doesn’t contain L-glutamine

Glutamine lab level should be approx. 500 on quantitative amino acid profile.

• Based on lab reports, the amino acids L-carnitine and L-arginine are often supplemented.
• Additional essential amino acids should be added as indicated by a quantitative amino acid profile.
• CoEnzymeQ10

Although most children seem to tolerate Tolerex best, parents must add LOW amounts of essential fat to this formula. To accomplish this, there are several different options successfully used by parents. Over the last couple of years, it has become increasingly common for parents of children with Type 1 SMA to add breast milk to their children’s Tolerex. For many, this has made a tremendous difference in their children’s growth and wellness, especially their gastrointestinal health. Based on their essential fatty acid profile lab reports, approx. 8-10 oz/day of additional breast milk meets the children’s essential fatty acid requirement. More breast milk is sometimes given when an extremely reactive child cannot tolerate other calorie sources, such as baby foods and juices. If a child’s mother can’t supply breast milk, many families report a positive experience acquiring it from local donors, signed off as healthy by their doctors, or donor milk banks. For more information go to milkshare.com and eats on feets on facebook. There are many SMA caregivers willing to share information on acquiring breast milk from donors. Questions can be posted on SMA family on facebook, SMASpace.com or SMAsupport.com.

If breast milk is not an option, a commonly used fat supplement is 1/4 to1/2 teaspoon of safflower oil added to the Tolerex mixture. Some children also tolerate 1-2 grams of evening primrose oil and/or 1-2 grams of an Omega 3 supplement. Some parents find that a combination of Tolerex and  Pediatric Vivonex works well for their child. It can’t be stressed enough that all fat supplements should be added slowly, in very low amounts, to check for tolerance. When used exclusively, Pediatric Vivonex and EO28 Splash should contain ample fat for most children.

Almost all dietary fat is used by muscle. Because SMA patients have very reduced muscle mass they have an extremely low tolerance to dietary fat. When the percentage of calories given as fat exceeds 10%, many children with SMA experience flushing, high heart rate, increased secretions and delayed stomach emptying with reflux and vomiting. Because our children experience improved health and quality of life on a VERY LOW FAT diet, it is necessary to verify that their essential fatty acid requirement is adequately supplied. This should be monitored with an essential fatty acid profile laboratory test.

INSTRUCTIONS ON ADMINISTERING DIET

These formulas should be diluted as much as possible (more than directed) with water and other fluids, such as breast milk, juices, rice milk, almond milk, and fruit and vegetable baby foods. These will also increase calories, potassium, and other nutrients. 16 ounces of fluid for each packet of formula is a well tolerated dilution for most g-tube fed children. Those drinking their AA formula should dilute as much as possible with water and juices. Adequate hydration is extremely important, and recommended daily intakes should be met.

Additional Food:

In addition to the elemental formula mixtures, fruits and vegetables should be consumed.  Those with Type 2 or 3 SMA usually tolerate some grains. The formula, fluids, supplements, and fruit and vegetable baby foods should be mixed in a blender and refrigerated. Because children with Type 1 SMA are often extremely reactive to changes in their diet, foods and supplements should be added gradually, one at a time, to check for tolerance.

Children receive the most benefit from this diet by greatly reducing or avoiding animal protein foods. Children following the AA diet correctly are receiving adequate protein from the amino acids in their elemental formula, and can easily be overloaded if they are also eating high-protein foods. Most children tolerate 1gm/kg to 1.5gm/kg of amino acid protein/day. They should never be fed high amounts of amino acids over a short period of time.

CLOSING

Although children are affected by their SMA differently, the recommendations stated earlier have proven to be the safest and most effective way to implement the AA diet. Parents must take responsibility for monitoring and problem solving when placing their child on this diet. Unfortunately, due to the lack of study on nutrition in SMA, many medical professionals are unfamiliar with this dietary approach and often overload our children with fat and/or amino acids. Networking with other SMA families that are successfully using the AA diet and following the above guidelines can be an invaluable source for information on the benefits and proper administration of this diet.

Personally, I have had success with this diet for my daughter Krista, a lovely 21-year old, who was diagnosed with SMA Type 2 when she was 8 months old. We placed Krista on this diet over 16 years ago and have been very pleased with her wellness and stability. There are many other children with SMA that have also experienced more quality and length of life from following the AA diet as stated above. The feedback from their caregivers has played a significant role in our ability to adjust and improve the AA diet. I would like to thank all of you who have contributed by sharing your experiences and knowledge over the last 16 years. It has been a joint effort!  A special thank you to Jeanna Huette, who contributed greatly to the revision of this paper.

"We believe in miracles because we live with one!!" 

www.our-sma-angels.com/elizabeth