****We are not medical professionals. The information contained here, on this page, is what we do for our daughter. This is SKYLER’S treatment plan: the noninvasive respiratory protocol and amino acid-based diet. Please contact your child’s pediatrician if you are interested in starting your child on any of the protocols below. Or please email us and we will put you in contact with the appropriate medical personnel.****
9/30/04 An Update on Skyler
Hi All. Just want to update you on Skyler and her new medical protocol/treatment plan. In the Spring of 2004, after a long illness, we opted to have a tracheostomy done for Skyler. It was a tough decision to make, because Skyler had always done so well with the Non Invasive Therapies (NIV), but in the end, we really believe we made the right decision. In September of 2003, Skyler began spending more and more time on BiPap...20 hours or more a day. We believe something changed in her airway...but were told it would be a really tough thing to prove. Skyler was happy on her pappy and very bright, alert, creative and expressive...as she always has been. After awhile, though, we noticed that she began to breathe more against BiPap at night. She has always been a mouth-breather...and it seemed to become more of a concern as her heart rate would fluctuate at night and she always seemed to be restless, waking frequently. She was also teething, so we decided that maybe, over time, she would settle back in with the pap at night and begin to work with it better again.
Everything seemed to be going fine and then at the end of March 2004, she caught a really, really nasty respiratory bug and began vomiting and having diarrhea at the same time. Skyler had always been an upper airway plugger when she became ill...and it was the same in this case. She crashed several times...but seemed to be getting over the hump of it until the diarrhea and vomiting hit her really hard. We watched her carefully, but she dehydrated very quickly. We called in to have her transported into BMC for IV fluids...but when the paramedics arrived, they refused to take her anywhere other than our local hospital. Her SATs had begun to dip at that point and her HR was high, but I knew we could get her back on track if we could get fluids into her. To us, her SATs were fine. We DID run 1 liter of O2 into her to keep her comfortable...while she was on BiPap. But they still refused to take her into Boston. So, we went to our local hospital and they put in an IV. She was doing okay...and I was just wishing, at that point, that we had been able to have the IV placed at home. But they said that they were unable to do that. So, we had her transported into Boston. En route, her lung collapsed...on BiPap.
I won't go into detail about Skyler's 5 week hospital stay. It was time spent re-inflating the lung (it came up quickly after we demanded she be intubated), trying to solve pooping issues, and deciding on - and following through with - a tracheostomy. Skyler did really well post-surgery. She's still doing well. We no longer have to worry about her plugging her upper airway...and we are able to see her beautiful face ALL the time now :) We are still SO thankful that we had the NIV as a care option for our child...and we will NEVER regret taking that route for Skyler's initial care. It worked really well for her...but we knew, this Spring, that it was time...time to follow another path for our Skyler, to ensure that she remain happy and healthy.
Skyler still suffers from a lot of pooping issues. We haven't figured them out...yet. Alice, her PT, works on her intestinal tract every week...to keep it loose and pliable. We saw a GI specialist while at BMC, and he suggested giving her 3 suppositories (Fleet LIQUID glycerin suppositories) a day to keep her empty. We do this. Between 1-3, depending on the difficulty she is having at the time. Sometimes...she'll even go on her own :o) Skyler was also put on reglan and zantac during this hospital stay to help her with digestive issues. So, we're still figuring it all out...but she's doing really, really well...and she is HAPPY!
Even though Skyler is trached, we still use a lot of the NIV protocol to care for her. We have never been big into routine ...we treat her mainly as needed. Just like we do our other kids. Her meds, though, are regulated...because, of course, it's necessary to do so...
We also continue with the amino acid diet...and believe that this diet has helped her to do so well...and to enabled her to keep the functions of moving her arms, head and shoulders...and to begin moving her legs more and more...
Her respiratory protocol is as follows:
Bagging and Suctioning (with and without O2) as needed
Coughing with In/Ex thru the tracheostomy tube as needed
Daily trach cleaning/care
LTV 950 vent...settings adjusted per her needs
Reglan two to three times/day for easier digestion
Zantac two times a day for possible reflux issues
Zyrtec once a day for allergies (she developed these, as well)
Breathing treatments as needed when she is ill
Tolerex with juices and baby foods
*protocol information last updated on 10/27/05
Skyler is bright and vibrant. She LOVES books, flashcards, etc...anything that can teach her something! She loves playing on the computer - and GAMES in general. Skyler also loves to play with dolls, rings, bracelets...girly stuff! Her favorite doll right now is Clara. Skyler's sister, Casey, bought Clara for Sky when she went to see the Boston Ballet perform The Nutcracker last year. Clara and Skyler go everywhere together. Skyler also loves Pink Bunny (he helps hold up her tubing) and Green Fuzzy Frog. Sky loves to play marbles, Barrel of Monkeys, the game Trouble...and she LOVES to draw, finger paint and use her Magna Doodle. There is SO much that Skyler can do...and we just try to keep on top of it all! We have several different switches...because Sky loves variety! Skyler also LOVES to play with Luke and Casey. Both kids have learned to care for her...they both can bag her. They are not allowed to trach suction, of course...but Casey always jokes that she be able to do that BEFORE she's a teenager. We'll see :) But Skyler LOVES when Luke does her leg stretches and Casey teaches her. She is just SO open to everything. Speech has been a bit tougher for her since she has been trached...but she's learning. She's helped us develop Skyler's Sign Language, a variation of ASL...for periods when she has a tougher time talking...and she's incredibly good at it! Skyler's always willing to try something...and we're just so proud of her...AND her brother and sister! We're very blessed...
Brief History: Skyler Ann McAdams was born on April 12, 2001 at 1:00 PM. There was nothing unusual about her birth. In fact, once I was in active labor, she was my quickest delivery out of our three kids. My pregnancy had been a really rough one...severe vomiting and unexplained retinal strokes kept us praying for months that I would deliver a healthy, happy baby. And I did. Skyler was our little “bruiser”, weighing in at a whopping 7 pounds, 11 ounces and she was STRONG...very strong. Once they placed her on me, she lifted her head and looked up at me with bright beautiful eyes. She was alert from the very beginning. And she could move...man, could she move! The only thing that troubled us was that she experienced severe tremors...especially on voluntary movements. We inquired about this over and over again for weeks and were told that it was just her underdeveloped nervous system. Our other two kids also experienced this, but not to the degree that Skyler did...and when she ceased moving at all at 5 weeks old, we were more than concerned. At 7 weeks, Skyler was admitted at Boston’s Children’s Hospital with severe hypotonia (floppiness) and it was there that she was diagnosed 5 days later with Spinal Muscular Atrophy, Type I. We were told she would live a couple months to a year...two years at the very most. Because she was diagnosed so young, it was assumed that she would be weaker, and would pass most likely within months...certainly not two years. I asked the question...“What happens if she lives to be 2?” The doctor’s answer almost makes me laugh now...he told us that they would then re-type her as a 2. I won’t go into the emotional aspects of Skyler’s diagnosis at this point. That is a story for a later time. Just know that we spent the following 3 days grieving a daughter that was still very much with us. It was at that point that we decided that we would grieve no longer...that Skyler was still very much with us. She was our daughter and she was living with a horrible disease. It was sink or swim...and we decided to jump in a boat and ROW!
Skyler’s Diet: Skyler was lucky. She was able to nurse until she lost her swallow and was hospitalized with a collapsed left lung in January of 2002. She was 9 1/2 months old. While I nursed Skyler, I followed a very strict diet: no yeast, no dairy, low protein, low wheat, and very low sugar. I also took acidophilus, to try and pass it through my breast milk to Sky, because kids with SMA have been found to have a problem with yeast. This diet worked out really well...for both of us! When Sky began to eat baby foods, I would add a bit of Tolerex to the food. It was okay for me to do this because I was nursing, not using over-the-counter formula (which can cause problems if mixed with the amino acids). We had a preventative G-Tube placed for Sky at UMDNJ in September of 2001 when she was 5 months old. I’ll NEVER regret doing this, because it made it much easier for us to deal with the situation once she began to lose her swallow. Skyler lost weight, but we were able to begin pumping food into her immediately once she needed it. We had decided from the get-go that we would follow the amino acid-based diet for Sky. We started with a blend of Tolerex (the adult form...more calories/less fat) and Vivonex (the pediatric form...more fat/less calories). We have since switched to all Vivonex. We also add baby juices, baby food and many vitamin and mineral supplements. We sometimes joke that our other two kids never “ate” this well...getting everything they needed...an then some...in one mixture!
Respiratory Protocol: Jeff attended the FSMA conference 2 weeks after Skyler was diagnosed. It was there that Sallie Capen, who has since become VERY dear to us (as has her daughter, Liz, and her granddaughter, Kalair), introduced Jeff to Dr. John Bach from UMDNJ and Dr. Mary Schroth from Wisconsin. Hope had been something we were striving for, like a priceless treasure, and it was during Jeff’s meeting with Dr. Bach that he found it and brought it home to our family. Dr. Bach saw a chance for Sky...a chance for LIFE. And after much research and speaking with other parents following his protocol, we decided that we wanted to have Sky give it a try. She has done very well on the protocol and we are thankful for its existence in our lives. It has given Skyler a chance at a happier, healthier, higher quality of life! We use the following when caring for Sky:
The BiPap: this machine gives Skyler a deeper breath of air with every breath she takes on her own. It also has a back-up rate, so when Skyler is on it, she gets certain amount of breaths per minute. Skyler uses her BiPap (which she LOVES) for nocturnal sleep and naps. She also uses it as needed when ill. This machine, along with the one that follows in this list, enabled us to keep Skyler at home during her last severe illness. While the BiPap is at work, we know that Skyler’s lung muscles are being exercised...and that she is also being given a rest, so that she is better able to breathe efficiently on her own when she is off.
The In/Exsufflator: other wise known as “the cough machine” or “cough assist”. It does just what those terms imply...it assists Skyler in coughing effectively. The machine blows 40cm of air into Skyler’s lungs and sucks the same amount out. This enables us to clear her lungs, upper airway and sinuses of any plugs or bothersome secretions. The “cough machine” is also an excellent means of expanding the lungs to their fullest, and exercising the muscles that surround them.
The Suction Machine: because Skyler is unable to swallow, we use this machine to suck out secretions from her mouth and nose. This machine is a necessary piece of equipment...especially for Type I kids. It is also needed if you are going to use the Cough Machine...in order to suck out the plugs/secretions once you get them into the nose or mouth.
Pulse Oximeter: this device enables us to monitor Skyler’s oxygen saturation levels (SATs), which is highly important. If a child is unable to maintain certain oxygen levels, it can be indicative of something that might be brewing in the lungs, upper airway, etc. It is also an excellent way to see how your child is doing moment to moment...if BiPap might be needed for some extra air, etc. The “pulse ox” also allows us to monitor Skyler’s heart rate. Heart rate can also be indicative of something that might be going on...such as discomfort if the heart rate is too high.
Oxygen and Ambu Bag: ONLY AS NEEDED. SMA kids do not have an oxygenation problem, but a ventilation problem. A HUGE misconception in the medical community is that our kids need oxygen to make them more comfortable...when it is really more ventilation that may be needed.
Our Skyler!! : Our Skyler is bright and funny. She loves to laugh! With the help of her Tiger 2000 stroller, she is able to participate in almost every outdoor activity that her brother and sister enjoy: they race their bikes, we race her Tiger! Skyler is VERY chatty and loves to sing. Since she initially lost her ability to move, she has regained much of her strength. Her arms came first...within a month or so of losing her ability to use them, Sky taught herself to move them from the elbow down. This enabled her to chew her favorite infant toy...her Gumby! Since her 1 year birthday, Skyler has begun wiggling her legs again and can actually hold them in place, in a bent position, for quite a while. She can move her head from side to side and last year, she began to wiggle her head onto my shoulder in order to propel if off! Boy, that caught me off guard the first time! Sky loves to play games with her favorite colored buttons, her rings (she’s very specific on how she likes to play her games and is constantly changing the routines!), her Kelly dolls, her markers, her Polly Pockets, her Play Doh...and her STICKERS! She loves stickers...but the rule is that after the first set gets placed on paper, you need to put the sticker on her nose AND your nose and take them off from there and then put it on the paper. It gets pretty comical...all of us wearing stickers on our noses! There are too many activities to list...she just LOVES to play. Skyler’s favorite show now is Blue’s Clue’s and she actively participates in the show...yelling out the answers and finding clues! She also loves to sing and “dance” to Barney songs. Skyler is really working at her speech. She says many words...mama, up (she LOVES to be UP!), out, more (for her favorite Wheat Thins!), owl, etc. She loves to make animal sounds...and is really great at imitating...sounds, tones, etc. She amazes us every day! Of course, she’ll need some speech therapy and we’re currently in the process of finding someone to work with her...and her disability. Sky has found two switches she works well with...the Toggle when she is lying on her back and the Pal Pad for when she is on her side. These enable her to turn on/off household items and toys. Her favorite is turning the TV on and off...she thinks that is just TOO funny! But Skyler’s favorite activity is ANYTHING that has to do with her brother and sister, Lucas and Casey...they make such a fantastic trio and we are so very proud of all of them!!
Thank you to all those who have helped us in providing Skyler with a happier, healthier, fulfilling life! We couldn’t have done it without the love, knowledge and experience of our SMA Family and Friends!!!!
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