July 17, 2006
(letter Tina sent out by e-mail & Sandi forwarded to all her contacts also)
Dear Family and Friends,
I am sending you this letter with great sadness. I am very sorry to be sharing the following information through a letter, but I have been deliberating how to share the news easily and effectively without making things more difficult for me and our family. Again, I know that this news is difficult to read, but it was the only way I could share it quickly and effectively without talking with everyone personally or it spreading slowly by word of mouth. I also felt that I needed to share it now since I haven't been very good at returning phone calls, emails, sending thank yous promptly ... even though I am grateful to have so many wonderful people in our lives and am grateful for all you may have done for our girls. We are going through a very difficult time in our family's lives. I hope you will understand after reading this letter and will place us in your prayers.
Many of you know that Allyson (who is almost 6 months old now) has been having some testing done to see what is causing her to have very low muscle tone or floppiness. She began physical and occupational therapy at 3 months old, but is still unable to hold or lift her head, support weight on her legs, and has very limited movement in her arms and legs. She is making some improvement with therapy twice a week and infant massages, but not enough. She is also experiencing some raspy breathing and has a very faint cry and cough that is worrisome. After about a month of tests and lab work (MRI, muscle and nerve testing, muscle biopsy, blood and urine work and genetic testing), we learned on July 10th that she has a genetic disease called Spinal Muscular Atrophy (SMA) and probably has the most severe form. This disease affects the muscles and nerves. It can also affect the respiratory system and is often fatal at a young age. So far, Ally is doing pretty well and she is a very happy baby. She goes to therapy two times a week and we are looking into the best medical care for her. Below I'm going to list some facts found on a website, but remember that every child is different. These are the cold facts and we have lots of hope for Allyson. If we can keep her respiratory system healthy, maybe a cure will be found soon. Hope is not always easy in these difficult times, but it is all we have. We are trying our best to stay positive and stay strong for all of our girls. Kristyn understands that Ally may get weaker and sick, but not the complete severity. She also thinks Ally will prove the doctors wrong and be a strong lady one day.
After reading the facts below, if you would like to learn a little more, feel free to call (although I'm still learning myself) or here are some of the websites I have found most helpful:
www smasupport com (very friendly site mostly with information from parents - GREAT diet info and respiratory CARE info, message board)
(great info site with tons of research info and medical background, support)
If you know of anyone else who has been affected by SMA or similar condition, please let us know. We are learning quickly that the best information often comes from families. Most doctors have not experienced patients with this disease and we are looking for the best care for Allyson. Currently we have been going to Lutheran General, but we have an appointment in August with a neurologist at Children's Memorial and may go to Madison, WI to see a pulmonologist (respiratory doctor). We are also looking into the research that has been very successful in mice and has begun to be successful in children.
I know that some of you may want to reach out and help. There is not much anyone can do, but here are some things that others affected by SMA have found helpful or some things friends have suggested:
* Very important - We would love for you to be a part of Ally's precious life. She has a lot of love to share and lots of people to meet and touch with her adorable smile. PLEASE let us know if anyone is sick in your family though. Even a tiny cold can be dangerous. I've never been much of a germ person, but I will be learning very quickly. I know the winter months will be the most difficult.
* Please keep in contact with us. I know it is difficult to talk about such scary times, but now is when we need the most support. If I don't get back to you right away, I still appreciate you calling or writing, but may be having a hard day or week.
*Some of you have volunteered meals and babysitting, and I originally declined. It is difficult for me to accept help from others without being able to do it in return (although I couldn't live without my mom). These types of help, might be really beneficial as I think more about it. In this beginning period, I am spending a ton of time coping, holding Ally, trying to stay "normal" for Kristyn and Melissa, and doing as much research as possible. I could possibly use a little help here and there with meals or babysitting. If you are interested in helping, just let me (Amy in my MOMS Club or my sister Sandi 847-875-5304) know and I may take you up on the offer. I'll try to return the favor sometime. I know things will get easier, but for now it's been really hard and we may have our ups and downs for awhile. Hopefully they will mostly be "ups" and I will be writing happy updates.
I better end this very long letter. I appreciate your time and your prayers
for Allyson. Below are the facts I mentioned, but remember, Allyson is going
to be a fighter and beat as many of these odds as she can.
Love to you all,
Tina and the Krajewski Family
What Is SMA?
· SMA (Spinal Muscular Atrophy) is a neuromuscular disease passed on genetically to children by their parents.
· SMA Affects a child's muscular development. The earlier the symptoms are noticed, the more severe the type of SMA. Type 1 is the most severe, affecting children while still in the womb or shortly after birth.
· Type 1 children are usually not able to hold up their heads, roll over, crawl, sit up without support, or walk.
· All muscles are extremely weak, with the weakest muscles being the legs, upper arms and neck. Also affected are sucking, swallowing, digestion and breathing.
· This type is often fatal, with the last known statistics showing an average lifespan of 8 months, 80% gone in less than a year and the majority of the rest gone by the age of 2 unless ventilation or "life support" is provided.
· **HOWEVER**, these statistics are no longer a hard and fast rule, and the more time goes by, the better quality and quantity of life these children are having!
· SMA is the Top Genetic Killer of children under the age of 2!
· 1 in every 40 people carry this gene.
· 1 in every 6,000 children are born with SMA.
· THERE IS NO CURE.
· For more specific information on all the types of SMA and what they mean, visit our SMA Info page.
Is There Hope? YES! SMA has been cured in mice! They now know what causes it, and are currently testing compounds to find the one that will work for humans with the most safety. All they need is time...and money. In the meanwhile, the children who are here now are often very short on time.
773-283-7211 or 773-981-7211
3614 N. New England – Chicago, IL 60634
July 17, 2006 E-mail Auntie Sandi sent out in response to many e-mails received after people read about my diagnosis...
Thank you for your thoughts & prayers. I am Tina’s older sister, for those of you who don’t know me. I have saved this general note since many of you have responded that you would like to help Tina & her family out in any way you can.
Tina has forwarded me the e-mails she has received since it is hard for her to get through all of them & respond.
The main things that were asked about were if we are going to have a fundraiser, if Tina & family need meals made & brought to her & babysitting.
I am going to send my comments & suggestions the same to everyone who has contacted us & you can let me or Tina know what you would like to help with. Thanks so much for your support.
It has been suggested by some that we should start an Ally fund to prepare for expenses that may come in the near future. I will start looking into details for a fundraiser. Please let me know if you would like to help with the planning, know how to go about contacting for donations, if you have ever done a fundraiser, etc. I recently went to a dinner/bowling fundraiser & am going to look into the details on how that worked. I am open for other ideas too.
Tina never really knows exactly what her schedule is like, between therapy, now trying to make appointments with specialists for Ally & the schedule of her other 2 children Kristyn & Melissa.
My suggestion for meals- is if you want to prepare a meal that can easily be thrown in the oven or frozen for another day. Another suggestion would be to get a gift certificate for a nearby restaurant that delivers to their house. I can give you some suggestions or if you prefer you can mail me a check & I can pick up a certificate for them, I am in the city by them pretty often. For dropping off anything, I would call 1st to make sure someone is home. My mom usually is home until at least 12:30 p.m. and Tina would be home mostly in the evenings. Their Home# 773-283-7211 Tina Cell# 773-981-7211
Address- 3614 N. New England, Chicago
As for babysitting, Allyson has therapy every Wed. & Fri. right now in the mornings, so Tina usually needs a sitter for a few hours. If you are ever available to watch Melissa & Kristyn for a few hours. I am actually going to start a spreadsheet on possible availability time so if you want to give me some specifics I will note them, such as you can pick up the kids & take to your house certain days, you can watch at Tina’s house certain days or to call as needed on certain days & you can let Tina know if you can help, etc… Please let me know the best way to reach you.
Sorry this became so long. I am going to save it so I can send back to others who respond so wanted to be very detailed. Talk to you soon.
If you need to call me- Home# 847-587-8578 Cell# 847-875-5304
Address- 120 Scenic Road, Fox Lake, IL 60020
July 22, 2006 Good News! - I will be sending out an Ally Update soon, but Allyson was accepted in a Clinical Study at Stanford University in CA. We will be traveling there August 23rd and every month or every 2 months for about the next 2 years. Each time we visit, she only needs to go to the clinic for about 2 hours. They recommend flying with 2 adults and flying in the day before the study and out the day after. We are looking into Miracle Flights for Kids to help pay for some of our flights. The study is very amazing and VERY hopeful. It has already been going on for 2 1/2 years, but they needed 4 more patients to complete it, to have a total of 18 patients. SMA children are given a cherry syrup drug called hydroxyurea. This drug has been approved by the FDA for 10 years and has even been used with adults and infants for chemo and sickle cell anemia. It is experimental for SMA patients, but has very little or almost no side effects. Hydroxyurea will help the SMN2 (survival motor neuron) increase production of protein and RNA which is needed greatly by the muscles. It is not a cure, but results have been promising. Type 1 patients have showed highly increased head control, more movement in their limbs, supported sitting... I also believe that as the muscles are building, respiration is much easier. If Allyson happens to get sick from SMA while doing the study, she can continue to take hydroxyurea safely. We can also post-pone some visits as needed. Most patients fly with their bi-pap breathing mask as needed.
July 27, 2006 Allyson went in for a swallow test today 07/27/06, pasted below is a note from Tina she sent in an e-mail:
We just got out of the Children's Memorial Hospital on Wednesday, August 2nd, after being there 6 days. Unfortunately, Allyson failed her swallow study test when drinking from a bottle and attempting to eat from a spoon. They wanted to have her admitted right away and stop all oral feedings. Because of my research, I convinced them to let me continue to breastfeed since she overall has been doing great with it, and it is very different than drinking from a bottle and a lot safer than formula. She had a g-tube feeding tube put in her stomach so that we could feed her directly for night feedings and if she gets sick or looses the ability to nurse. Hopefully this will not happen anytime soon, but we now know that we will never have to worry about nutrition. She also had a nissen done. This is when they wrap part of her stomach around her esophagus to prevent reflux, or food from going back up. All of this was done early to be pro-active. Allyson was not sick when we were admitted and is doing VERY well! She smiled almost all the time at the hospital and is recovering very well. We are VERY happy to be home with Billy and her sisters!
August 6, 2006
This is a copy of an e-mail Tina sent out on 08/06/06 to Dr. Schroth in Madison WI & a few others with some more info on Allyson's hospital visit 07/27-08/02 & al il bit about their visit to CA on 08/24:
Thanks for writing back Dr. Schroth!
I also want to thank you again for all of your help before and during our hospital stay. Sorry I called so early in the morning on Friday, but it really helped to have some SMA information before the team of doctors came around for their morning rounds.
Ally did not have her g-tube and nissen surgery until Monday, July 31st and it was done lapriscopically (because I had info from you). I was allowed to nurse her all weekend, started again Wednesday, August 2nd, and I'm still breastfeeding her now. She eats from me as her primary source of nutrition and we are giving her about 8 oz of vivonex over a 5 hour period at night. I'm still not sure if this is the best thing for her or not. The dietician at Childrens was not that sure either, but this is what she recommended starting with. There is just not enough medical evidence of what is best and the parents that I have gotten information from mostly have children 2 or older. I hope to nurse her as long as possible, but want a back up plan. I also worry if she is getting enough from me or not. We hope to get a scale soon to monitor her weight and occasionally her intake. She was also prescribed polyvisol vitamin that I am giving to her through her g-tube. Do you have any suggestions or thoughts about diet and nutrition?
Ally is recovering very well. Yes, she does have a mark from her bipap. It's so cute that you knew what that mark was from. We are just using it at night for now and doing her cough assist and suction treatment twice a day. We have only left the house twice since we were released on Wednesday, August 2nd and she seems to get very congested within an hour or so. We brought her suction with us the 2nd time and it helped a little. She sounds pretty good when we are at home, so I'm not sure if it is humidity, her being more upright in her carseat carrier, or something else. We haven't switched to the carbed yet since she seems to be doing okay, but maybe that is part of the problem. Any thoughts?
We go to CA for our first visit to Dr. Wang from Aug 24-26th. We are very excited to be part of his study with hydroxyurea. We know that it is not a cure, but hope that it will help Allyson keep some of her muscle strength for movement and respiration. This month, we get all the details again and sign the consent. Then, in September we will start the Double Blind Placebo stage. Of course, we hope to get the medication right away, but are willing to take the chance so that we can get to the Open Label stage. When you write up your studies for Type 1 SMA, which drug will you be testing out? Do you know when this will begin?
Well, I think I typed quite a long email.
Thanks again for all your time and help,
August 20, 2006
Ally had a rough weekend & Tina really thought she was going to lose her a few times. She sent out this e-mail to try to get some help & answers & did receive many responses. She also learned that she needs to have Ally flat a lot more often & probably change her diet. Ally seems to be doing much better. I pasted Tina's e-mail below.
(I'm writing to the sma support group and a few people that others have said might be able to help me with diet information).
I know that there is NO "right" answer to my question, but I would love to hear your thoughts and I truly appreciate any experience you have with this topic. I know there is a ton of debate out there, but I'm leaning toward the amino acid based diet since many, many families seem to think it works well. I think this might work well for a 6 month old too, but maybe its for the older kids or after kids start to have complications. I'm very confused and would LOVE some help.
I need DESPERATE HELP in figuring out a diet for my 6 month old daughter, Allyson (SMA type 1, diagnosed July 10th). She lost about one and a half pounds in the last month and hasn't begun to gain it back yet.
She is currently about 12 lbs and 26 inches.
She had her g-tube and nissen surgery done on July 31st since she failed her swallow study test with a bottle and I knew we needed to be pro-active. Since then, I have been breastfeeding her during the day, and giving her 8 oz of Vivonex over a 5 hour period at night (although this didn't seem completely right, but was recommended by the hospital-which we all know often doesn't know what's best.) She is quickly losing the ability to nurse. She seemed to be doing pretty well on most days, but when I weigh her before and after, there is not much difference in weight, although she nursed for about 1/2 hour or more. Our pediatrician was very concerned with her low weight on Thursday (She was 13 lbs 5 oz, 2 months ago). I don't think I'm producing much with all the stress either. I'm going to try to pump some too, but I need another plan. With the pediatrician's recommendation, I tried this weekend to increase the amount of Vivonex I am giving her, but she acted very different, seemed out of it, breathed differently. We also had a pretty busy weekend and when traveling in an infant carseat, her sats kept going very low (mostly around high 80s and some even lower). Her heart rate would go really high for her around 175 and then even went as low as 40s. This is all very odd for Allyson. It seemed mostly in response to her eating about 4 oz of Vivonex directly through her g-tube in a short period (about 15-20 minutes) and then us traveling shortly after (since I try to feed her before we set out). After about 1 hr of traveling with complications and us needing to stop and take her out of her seat, we get to a location and after a short time, her sats get way better (very high 90s for oxygen and around 130s for heart).
Again, I'm still very new to all of this, and probably one of the most confusing things for me is figuring out what I should do about Ally's diet. My heart has also broken since I can no longer nurse her, or at least not effectively. I need to know that I can at least give her proper nutrition through her g-tube.
PLEASE email me directly with your thoughts or give me a call. My numbers are listed below.
I would also love to know if anyone gives their child Mannatech supplements. It sounds like a nice thing to add to a diet and I just got some information about it from Monica Shofner, a super nice SMA mom with 3 yr old Nolan.
Thanks in advance for your help,
August 23, 2006
I just wanted to let everyone know that Allyson is doing MUCH BETTER after the rough weekend. I have gotten a TON of help from other parents with SMA children who belong to the www.smasupport.com website. I really feel confident in the NEW diet that Allyson is starting, mostly thanks to Stephanie Price (and some other SMA families). I also feel better about how to position Ally so that she is most comfortable and breathing well.
On Thursday, August 24th, we leave for our first trip to Stanford, CA. We are really looking forward to meeting Dr. Wang and Tony Trela and learning more about the clinical trial with hydroxyurea. We will also be exploring a clinical trial in Utah that uses a drug called PBA or sodium phenolbuterate. This is all so confusing as we try to decide what might be best for Allyson, but the good part is that THERE IS HOPE! We will completely decide on which study we will participate in early September. I'll be sure to keep you posted. Thanks, Tina (Mom)
August 29, 2006
Hello Friends and Family,
I thought I should take a little time to write an update about our CA trip since sooooooooooooo many people are praying and thinking about Ally on a regular basis. Thank you so much for your continued prayers and support!
August 24-26th, we had an ABSOLUTELY WONDERFUL visit to Dr. Wang for the clinical trial on hydroxyurea in Stanford, CA (near San Francisco). I was really nervous about the trial and wasn't sure if it was the right thing for Ally. I NOW know that the benefits, far outweigh my initial concerns. Dr. Wang is the most sincere neurologist I have ever met and is probably one of the nicest doctors in the world. He helped me with all my concerns (flying long distances, which clinical drug might be best....) and I NOW truly believe that the CA study is the best option that Allyson has. As we have mentioned before, Allyson will have the chance to get a special drug called hydroxyurea. This drug will help her body produce protein and RNA needed by her muscles for movement and respiration. It is not a cure for SMA, but can help immensely and makes us very hopeful.
During our visit to Dr. Wang's, we also met another little girl, Kayleigh, who will be 2 yrs old in October. She has Type 1 SMA, but has made AMAZING strides with hydroxyurea. I cried in joy at almost every thing that her mom asked her to show us. Kayleigh can hold her head up for about 5 minutes, kick her legs, lift her hips, roll over, talk over 200 words, sign language about 60 words, and sooo much more. We also met her mom, Cindi and her aunts. They "sealed the deal" for us as we were deciding about being in the study. Kayleigh could not do most of these things before she started on hydroxyurea at about 9 months old. She is also working on sitting up independently and was a very happy little girl.
I want to send a SPECIAL THANK YOU to Bob Iverson and dreamline for purchasing our first tickets to CA. We will be looking to different organizations to help fund our trips, as it can get very expensive with airfare, car rental, hotel and meals. We will be having a fundraiser in February, but expenses have already begun. A SPECIAL THANK YOU to Justin Avey, Billy's boss for being sooo supportive as Billy takes off work for these very important trips. Family members will also make some of the trips with me and Ally so Billy doesn't have to take off too much work. ANOTHER SPECIAL THANK YOU to City Kids and Allyson's therapists for purchasing her a special Peg Perego stroller that includes a bassinet and laying seat. It also has a heavy duty tray at the bottom that allows us to carry Ally's respiratory equipment. The bassinet part of the stroller probably saved Ally's life. She is no longer able to be in an infant car seat since it compromises her breathing and we have had several scares in it. We will now be using a carbed and Ally's special stroller. We hope to get a wheelchair/stroller that lays flat in the near future. Allyson will not have to always be flat, but it does help with her "stomach" breathing. THANK YOU to my sisters Tammy and Sandi for watching Kristyn and Melissa and taking them to Great America to have a great time while we were away. THANK you mom for bringing us to and from the airport and for ALL your support daily. Without family and friends, we could never make it though this. I also THANK God for giving me strength to get through some of the rough days and for watching over Allyson and our family.
Our next trip is September 20-23rd. At this visit, Allyson will have a muscle test and start Phase I, the Double Blind Placebo part of the trial. She will be given a cherry syrup that may or may not contain hydroxyurea for the next 6 months. Then she will start Phase II, Open Label, in which all the children are given hydroxyurea. Of course we want her to get the real drug in Phase I, but understand it is worth the risk so that we can get to Phase II and also help the FDA see that this drug should be approved for treatment of SMA.
**Hope to see you all sign Ally's guestbook soon.
**Check out the adorable picture with Dr. Wang in the Photo Gallery.
August 29, 2006
Tribune Article on SMA
Thought some of you might want to read this article on another family we know with SMA.
**Maybe one day, we can have an article written about Allyson and other SMA children or even have a show on Oprah or a news station. We NEED to spread awareness about SMA so that a cure can be found soon.**
By Bonnie Miller Rubin
Tribune staff reporter
Published August 29, 2006
She shouldn't even
Elizabeth was a month old when her grandmother noticed she couldn't hold her head up and had floppy limbs. A doctor advised the baby's mother, Christen Huette, then 20, that some babies are just lazy and she should be patient.
By 5 months, however, no one was dismissing the suspicions.
SMA is not one of the 34 disorders routinely included in the state's screening program for newborns, though people with a familial link can ask for prenatal testing, according to the Illinois Department of Public Health. No one in Elizabeth's family had ever been diagnosed with SMA, so the family was caught off guard when a pediatric specialist delivered the grim news, based on a blood test.
Two days after the family was thrust into this strange, new world, Jeanna Huette took action. After stumbling across a blurb on the Internet about the Stanford University study, she confronted her daughter.
"I followed her into the bathroom and said, `Either we're going to California or I'm taking her myself,'" said Huette, jaw set, wagging her finger. "My daughter was grieving for this baby, and she wasn't even gone yet."
In June 2004, the three of them--none of whom had ever been on a plane--made their first trip to Packard Children's Hospital.
Now, every month, they haul six machines that help keep Elizabeth alive--including a cough-assist device the size of a vacuum cleaner--on a 120-mile trip to O'Hare International Aiport to fly to California so Elizabeth can be treated by Wang.
The Huettes also pack a special stroller, other adaptive equipment and containers for her special amino-acid diet, which includes breast milk, green beans, bananas and several supplements all delivered via feeding tube directly into Elizabeth's stomach every two hours.
The 14 patients enrolled in the hydroxyurea trial come from all over the U.S., Wang said.
A few have even relocated to Palo Alto--a move the Huettes briefly considered, since Elizabeth won't complete the trial until May 2007. "But when I saw the price of real estate, I just went like this," Christen Huette said, dropping her jaw.
As it is, finances are tight. The airfare for Elizabeth and her mother is picked up by Mercy Medical Airlift, a charitable program, but Jeanna Huette must pay her own travel expenses, about $750 a month. That's over and above the regular medical costs, which are daunting enough for Christen Huette, who earns $6.75 an hour at the local Kmart and lives with her parents.
The baby's grandfather, Herb, is a mechanic, and Jeanna Huette provides full-time supervision for Elizabeth. The baby's father does not live with the family.
In August--two months after the trial started--all the hassles paid off.
Instead of being a rag doll, Elizabeth moved her head. Next came her limbs and stronger muscle tone. Now, Elizabeth is chattering away, repeating her ABCs, counting to 10 and even managing a lip-curling, hip-swaying Elvis imitation.
The odds are still long, but the trips to California have given everyone something they didn't have before: hope. Family members slip easily into talking about Christmas, preschool and other future milestones.
"Whatever time we have has been a gift," said Christen Huette. "She's not giving up, so neither can we."
Copyright (c) 2006, Chicago Tribune
Hello Family and Friends!
I just created an email group of family and friends that might want to receive updates about Allyson once in awhile. Most of these will also be posted on her website in the journal part, but I know many of you have a special interest in how she is doing, and it's not always easy to check out her website or know when I've updated it. Feel free to share this information with others, as I didn't add everyone and don't have everyones' emails. If anyone wants to be added to this email group, just tell them to email me personally.
I want to send a BIG THANK YOU to everyone who has sent blessings to Ally or has offered some advice regarding her cold. It's good to know that we're not alone in this battle with SMA and with colds.
I spoke with Dr. Schroth (pulmonologist from Madison, WI) and went to see Ally's pediatrician (Dr. Kristi Lundblad from Lutheran General in Park Ridge), and overall I'm hoping and thinking that she will get through this cold, hopefully soon. Kristi checked Allyson thoroughly and she doesn't seem to have any ear infections or anything real serious starting. There is a small chance that she may have a small infection with her g-tube, but a culture was taken to be sure. I've been told a little leakage is normal, but we need to be sure. I put a little tea-tree on it to see if it will help. If there is an infection, it could be causing the fevers here and there. Overall, the fevers have only been spotty and go down immediately with Tylenol or Advil.
We have started Zithermax antibiotic to help prevent the cold from turning into pneumonia or something more serious.
Ally is using her bi-pap for her daily naps and a bit more in the day, in addition to at night. This seems to really be helping keep her oxygen saturations higher. I was worried about putting her on this so much because of all the foam and mucus that builds up around her mouth from the air coming pushing it out, but I'm realizing that this is a bit normal. I also worry about all the marks it leaves on her face, but we are rotating between a Mini Me and Respironics Small Mask to help with these. I now understand that when SMA children are feeling sick, they need to have that extra help with breathing from their bi-pap. Then they will have more energy to fight off the cold.
I am also doing treatments more often. I'm following the sheet on what to do when a child has a cold from Dr. Schroth. This includes chest physiotherapy, tons of cough assist, postural drainage, suction... Almost every time she is attempting to cough on her own, I use the cough assist on her. Her secretions are clear, but off and on get a little thicker.
Many SMA parents have mentioned using nebulizers on Allyson, but this is not something we have started yet. Since she is only 7 months old and was recently diagnosed, we haven't been prescribed with any. This is something I will check into, but I have heard some pros and cons, so I'm still a bit confused about them. I need to make an appointment with her IL pulmonologist VERY soon to go over this and other things. I've talked with Dr. Schroth, but also need to have her seen.
I have added a little more water into her diet. I am flushing her g-tube with more than I used to. I already make her amino acid diet with 1 package of Tolerex, 12 oz of breast milk, 8 oz of water, 1/2 jar of green beans, 1 tsp of primodophilis, 1/8 tsp of safflower oil. This mixture is lasts about 24 hours for her day and night feedings. I also give her polyvisol in the morning. Her diet is a work in progress. So far, I am giving her about 250 mL at 40ccs an hour over night and 3 ozs./90mLs about every 3 hours during the day as a bolus feeding. She seems to be tolerating it, I believe. When I vent her, nothing rarely comes up. She does have a nissen. Her heart rate seems to be staying about the same during these feedings and used to change before I started the amino acid diet.
Thanks so much for everyone's support. I don't know what I would do without all of you!!!!
PS - Our next trip to CA to see Dr. Wang is Sept 20-22nd! We are hoping and thinking that Ally will hopefully be feeling much better. I think she is already making improvements. At this visit she will begin the double-blind placebo stage of the clinical trial on hydroxyurea. She will also have her 1st MUNE (muscle) test. We are very hopeful about this study and the possibility of making Allyson stronger. We will also get to meet Elizabeth and her family on this trip.
September 13, 2006
This is something that my mom posted on SMA Support talk & she said it was ok if I share. Sandi
I'm Grandma to Allyson Krajewski that has SMA 1 and is 7 months old. Allyson lives in my home in an in-law apartment and I'm a big part of her life. Preventive care has saved Ally's life as Ally's has gotton a cold probably from her 2 yr old sister Melissa.Her Mommy asked me to watch over her and Missy as it was raining and I was asked to watch over them as she drove her older sister Kristyn to school only for about 5 minutes, 5 min that seemed like time stood still. Allyson appeared like she needed to cough and she can't as I turned her to her side and patted her back her alarms on her oxygen levels alarmed. I'm proud to say I didn't panic (Tho it was unknown how'd I react) I began to suction her mouth to clear saliva as I'd seen Mom do many times. It wasn't working and Allyson's tongue did not allow me to gag reflex to get suction from her throat. I'm not a professional just a Grandma. I tilted her body head lower than her legs. not working. my baby's eyes rolled up in her head her lips turned blue, her skin pale white as she layed lifeless in my arms 1000 thoughts rushing thru your mind I cannot loose my daughters child No numbers on the oxygen levels and heart beat down to 3!! Reflex I blew breath into her mouth not both nose and mouth the third breath snots came from her nose I suction her nose and the oxygen levels numbers for her oxygen arose her heart rate rose and our babys came back to us. My daughter walked in and assisted w/ a few more treatments of suctioning till all was normal. I cried my daughter understood and I'm thankful to God above for the strength not to panic. Now my daughter knows I can and will be here to help in all way that I can. No one knows how they will truly handle the stress I didn't know myself. Now I know my daughter needs a break from time to time even when its to take a shower I will be there I will help her care when times get tough. Family is family and when family can't or doesn't want to accept this disease our children are born with I'm sorry for them.I Love my family. Some have no one to turn to. SMA site has given our family answers,support and a way to journal our feelings and thoughts. I'm supporting all I can for this fight in hopes for a cure. I'm so proud of my daughter for the woman she is and for researching all she can for Allyson. Allyson is in the California Study. She had her 1st visit in Sept. next visit is in Oct. I am praying I have faith. Thanks for listening
September 13, 2006
October 2, 2006
I'm sending this to my SMA friends and also those of you that I promised I would send updates about Ally. I'm also posting it on the journal part of her website. Some of it only applies to the SMA support group that I belong to, but it really updates everyone on how I have been learning to cope and the best part about Allyson is at the end! Sorry I had to just type one email or I would never get it out.
I haven't posted/emailed in awhile, but wanted to send an update (sorry it's so long). I feel terrible since I am always asking for help or advice (mostly on the sma support chat or to immediate family/friends/doctors/therapists) and rarely have time to respond to other's emails with well wishes, happy b-days, my thoughts.... I also feel REALLY bad that I'm not responding with thank yous to all those who have offered/given help or well wishes to us. I REALLY appreciate EVERYONE helping, thinking of us, praying.... My time is much shorter on the computer since I'm trying to focus on the kids and trying to keep out of a slump. I have had some rough emotional times lately. I try to stay as positive and as hopeful as possible, but I have my days where I barely have enough energy to take care of the girls. Billy has been wonderful during these times. I'm sure many of you have gone through similar times with your SMA or non-SMA families, and I probably don't have to explain myself, but somehow writing about it helps. I also have good days and they seem to be getting better again and are more often (Thank God). Ally's smile is just amazing and so are my other daughters, Kristyn and Melissa. My husband Billy is really trying to help too.
Since I get digest form of sma support chat, I try to skim quickly through the emails for things that may help Allyson or that I may be experiencing in the near future. Unfortunately since I'm still pretty new at SMA, I am still a bit overwhelmed. I love having a community of friends that can understand what I am going through and that I can look for for support, but it also makes me face the reality of illnesses, complications and even deaths. If I keep to myself, I wouldn't hear about these things, but then I also wouldn't hear about all the wonderful things that give me a ton of HOPE (birthdays, new wheelchairs, special accomplishments...) I also NEED all of you for advice and/or support. I have learned soooooooooooooooooo much from my new SMA friends and have gotten so much support from my friends, families, doctors, Ally's therapists, MOMS club.... I don't think I would have gotten this far without EVERYONE. THANK YOU again for being a part of mine, Ally and our family's life in some way! Please forgive me for not be more responsive or in touch. I'm waiting and hoping for the days that things get a little bit easier or more settled into a routine.
Well, now for the GOOD part about Allyson:
- She recovered from her cold after about a week or so with help from Zithermax antibiotic, lots of respiratory treatments, love and prayers.
- We did have TWO scares of her stopping breathing as her cold was winding down, but now have oxygen in our home for emergency use only. I also thank my mom for helping Allyson and me. I don't want any more scares, but know that it is possible to get through most of them if we have equipment and know what to do.- Our second trip to CA to see Dr. Wang for the clinical trial on hydroxyurea in September went WELL. Dr. Wang and Tony Trela are wonderful. Ally had a MUNE test to test her muscles/nerves for a starting base, gave blood and officially began Phase I of the trial. The first phase is the Double Blind Placebo part. For the next 6 months, she will receive a bottle of "medicine" to take each morning. It may be sugar or really the drug; only the pharmacist knows. Of course we want it to be the drug, but we have to take the chance in order to get to Phase II which is the Open Label phase, where all the children get the drug. In order for Dr. Wang to prove that hydroxyurea works and how well, there needs to be data for the FDA on how children do with and without the drug. Hydroxyurea is NOT a cure for SMA but it will hopefully help Ally's body produce protein and RNA needed for her muscles. Results on other children have been very promising. She will be giving blood every two weeks to be sure that everything is going fine and she will continue to visit CA every month. Each visit only takes us 3 days and we are only at the clinic 1-3 hours on one of those days.
- The hard part about our trips to CA is flying from Chicago. Ally is a real trooper on the plane but her oxygen levels do go down some on the plane. Everyone's oxygen is affected some due to the altitude and cabin pressure. We keep her pulse ox on and she lays flat on our laps to help with her breathing. We watch her carefully and suction as needed. Next time we fly, we are going to try her bi-pap breathing machine to see if it makes it a little easier on her. As the winter approaches, we hope we will be able to fly every month, but we will skip a month or so if Ally is ever under the weather. This will make the first 6 "clinical trial" months go a little longer, but Ally staying as healthy as possible is our #1 concern. She can still get the "medicine" if we have to skip a trip, but her dose cannot go up and it dosen't count as a clincal month of being on the drug and being studied. This sounds a little more complicated than it really is.
- ***We began home nursing today!!!*** With the help of our pediatrician and DSCC, Allyson qualifies for 84 hours a week of nursing (that's 7 - 12 hour shifts). We won't be using all these hours right away, but it's nice to have them there. I'm starting with about 3 day shifts and then will add on as needed and as I get used to having nurses in our house. Ally's crib is my room, so I also need to set up a separate area for her before I do any night shifts. I still feel like I should be SUPERWOMAN and do everything, but have accepted that I could use some medical help for Ally. As another mom told me, with nursing help, I can be "MOM." I can have more time to do special things with Ally and my other girls. I may actually start to cook dinner again and do some chores around the house. I will even be able to take a shower without my husband being home. I should be able to get a little more rest, so I can have a better emotional state for everyone too. I know that I need to take better care of myself but haven't had enough time. I don't plan on leaving the house and Ally for a long while, but I can more easily go to therapy and doctor appointments, as the nurse will come with us. I've learned that we will be staying in the house most of this fall and winter, but we are still figuring all that out. I did a long orientation with 2 nurses today (Nicole and Zuella) and they seem great. Nicole has already been to our house for 5 discharge visits after Ally's g-tube surgery, so it should be a nice transition. Allyson really loved smiling and staring at every move they made. Nicole stayed the full day today and Zuella will be here all day tomorrow. I might even make a real dinner.
Our next trip to CA is October 15-17th! An organization called Kid Power sponsored this trip's airline tickets. My mom is going to come with me so that Billy can work. I'm a little nervous about flying again, but hopefully things will go very smooth with her bi-pap. In the past we have flown with Southwest and this time we are flying on United. I am in the process of getting all of her machines appoved for United. It was a bit more complicated than Southwest's procedure. I have also been talking with TSA (airport security) to make going through with all her medical equipment, water, formula, breastmilk....a little smoother. Each time the security seems to have different rules or procedures. We have doctor notes, but we might be able to get something from TSA that explains what is clearly approved for them. She might even be able to stay in her flat stroller and have a gentle pat down while laying down. I'm working out the details with them after calling their 1-800 number. I'll let everyone know how it goes.
- Melissa, my 2 year old, began pre-school last week at City Kids. This is where Allyson attends therapy. It is a wonderful center and she absolutely loves it!!!! It's gonna be a little tricky getting her there as the weather gets colder, but I take one day or one week at a time around here. I'm just happy to having her playing with other children and learning.
- Kristyn, my 10 year old, is doing wonderful in 5th grade. She just earned Student of the Month! She also loves doing her cheerleading every week. She has become a little nurse and helps me out tremendously with her sisters. She is also so independent and is amazing at how she handles everything going on in our house.
- Billy's work has been amazing at understanding our need and desire to travel to CA each month. His boss has paid him "vacation" days for the last two trips to CA and days he has needed to take off for her g-tube surgery. He would be out, but I believe they will continue to pay him for any days he needs off. This is REALLY helpful since I am no longer teaching and our CA trips are pretty expensive with car rentals, hotel, food.... They are worth it though.
- I have decided to wait on doing any more Pampered Chef cooking shows for a little while. I want to be there in case Allyson has any respiratory trouble. I'm afraid to leave her after our two scares. Hopefully I'll get a little better about this since I need to get out of the house once in awhile, but I'm really enjoying just hanging out together. I hope to keep my director status, but need to meet all my goals for Pampered Chef so I am doing individual orders and catalog shows. Anyone who wants a real show, I am mostly having a team member do their show which also helps me in the long run. I don't know if I will substitute teach or not this year. I'm taking things slow.
- Ally's fundraiser planning is underway. We are having a banquet with dinner, raffle/auction and more on February 3rd. This will also be a 1 year old b-day celebration. I just hope she is able to make a small appearance since it will still be winter. My sister Sandi is the big organizer for this and they had their first committee meeting this past weekend. Our first MAJOR GOAL is to get as many donations for raffles/prizes as possible. If anyone is interested in helping with the fundraiser, can donate or gather donations, please let myself or Sandi know. Her email is firstname.lastname@example.org or phone is 847-587-8678 or 847-875-5304. We need to get as many big (and small) prizes as possible so that we can create a flyer listing some of them to pre-sell raffle tickets. Thanks in advance for any help/donations!!!!
Well, I guess that covers a ton. Sorry this is so long. I guess that shows I need to do updates more often.
Hope everyone has a great week!