mom's thoughts in the morning

You know, I think sometimes our expectations lead us to make up our minds about how our life will go before we remember to ask the King of Kings about any of it. I know that sounds really simplistic but that is kinda how I feel about this whole thing with the higher mestinon vs. lower mestinon and a dose of Albuterol. I sometimes assume striving for the most strength at all costs is the "right" thing to do. It seems that we may need to trade a more stronger Kyla for a Kyla who is a little weaker overall but has less bladder issues and potentially less long term side effects from high doses of Mestinon. The best thing about all of it is, she is just living life and enjoying the adventures. What a blessing. Friends and family, please pray for our family that we will have clear minds to ask the Lord what is best, and that we would have patience to see the long look down the road,

Love you all.

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Summer Fun

Kyla has had a fun day playing with her cousin Joy who is visiting for the weekend. Those two have put on a dance show, played inside, played outside on a slip'n slide and in the little kid pool.

Kyla is still on the weak side of her normal self, but she doesn't let a little weakness get her down. I would say she's functioning at about 75% of her pre-Mayo self. Once again, we've not seen any effects of the Albuterol, other than a noticeable case of the jitters this morning and afternoon.

Pictures from the zoo on Wednesday

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We are home

After another uneventful day of travel, we arrived home last night at 9:30. Thank you to all who have prayed for this trip and for Kyla. We are blessed to have had no travel problems.

Kyla is now on day 3 of Albuterol. She is taking a pill form of Albuterol. On Wednesday, she took 1 mg (half a pill) at 8 am and another 1 mg at noon. Yesterday, she took 2 mg (one pill) at 8 am and 1 mg at noon. Today, she has already taken 2 mg at 8 am and will take another 1 mg at noon. We will work her up in the next couple of weeks, with a target goal of 2 mg at 8 am, 2 mg at noon, and 2 mg at 6 pm each day. So far, the only side effect we have seen is a case of the jitters. She is a little shaky this morning after taking the pill. However, it's hard to say that we've seen any change in her muscle strength at all. Our starting of the Albuterol was at the same time that we decreased Mestion from 5mL to 3 mL 3-4 times a day, so any advantages of the Albuterol have been more than negated by the significant weakness caused by the decrease in Mestinon. We'll continue to journal the effects of the medicine changes on Kyla and hope others find this helpful. If you are a CMS patient and take Albuterol, we'd love to hear from you (neely4606@hotmail.com). We had heard of Ephedrine as a treatment for CMS, but the use of Albuterol was a new one for us.
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Going home today

We go home today. We drive to Minneapolis and fly out at 2:30 and will be at DFW at 5.

Kyla's Mestinon dose has gone from 5 mL to 3 mL 3-4 times a day on this trip. I pray that in the next week her body will adjust and we'll have our little Kyla back to where she was before we came. We would love to hear from other parents who have children with a CMS. My email address is neely4606@hotmail.com.
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The picture to the right is from yesterday.  The Internet connection here at this hotel was really slow last night, so I gave up on uploading it yesterday.

A final meeting with Dr. Engel and Dr. Selcen

We had our last meeting with the doctors today. We saw them together at 1:30. They agreed that Kyla is doing very well. They also agreed that Kyla's Mestinon dosage should be less and that we should continue trying the Albuterol. They recommended that we come back every two years or more often if we'd like.

This is Neely typing, so this is my opinion, not necessarily Sabrina's. I trust the doctors and want to do what is best for Kyla. However, we have given Kyla the lower dose of Mestinon only once last night and a three times today and she is noticeably weaker. I would say significantly weaker. I hoped and prayed we would leave here better than when we came. The opposite has happened and it's frustrating. The Albuterol seems to make her a little hyper, but doesn't seem to make her any stronger. We'll give it while to see if things change, but I'm a little discouraged right now. We just went swimming and her eyes we so droopy. It's like we've taken several steps backward.

On another note, last night we had to measure Kyla's oxygen saturation while she was sleeping. But, Kyla took it off in her sleep and there wasn't enough data to make the test legit. So, we get to repeat the test tonight. We're doing this because Kyla's oxygen saturation level went too low during the sleeping portion of her EEG yesterday. Hopefully, we'll get good data tonight and everything will be fine. We will consult with Kyla's pulmonologist at Cook's in Ft. Worth when we return if there is a problem with the test tonight.

Other than the doctor's visit, we have just played today. We went to a really cool park this morning and the girls had a blast. After the doctor, we went to a little zoo about 10 miles from Rochester. The girls really enjoyed looking at the animals. Tonight, we went to Rochesterfest and had fried food, corn, and dippin dots. I got to enjoy some Cheese Curds and Mini-donuts, which were amazing. Kyla and Laura loved their hot dogs and dippin dots.  There were a ton of people there, so it was a little overwhelming for Laura.

A long day at the Mayo

We arrived at the Mayo at 8 am and didn't leave until 4 pm.  Kyla had a chest x-ray, urine test, and Electrocardiogram early on and Kyla did well through all of those tests.  Then came the EEG.  She did not like any part of it, and did not fall asleep, which was required in the test.  So, she had to be sedated for the last part of the test.  The nurse who administered the sedation was not eager to sedate her for some reason, so we spent a good bit of time just waiting for her.  And when she came in, she wouldn't sedate her because Kyla still had a blood test to go.  So, our EEG tech went to work and got orders for the sedation and we eventually were able to finish the test.  A 45 minute test took 2 and a half hours.  That was quite frustrating.  This is our second trip to Mayo and both times the EEG has been a terrible experience.  Anyway, after the EEG was over, Kyla had blood drawn and then we had lunch.  After lunch, we met with Dr. Selcen, a neurologist.  During this meeting, we discussed putting Kyla on another medication.  She agreed with Dr. Engel that 3,4 DAP is not worth the risk for Kyla, but she also suggested we try Albuterol, which Dr. Engel had mentioned yesterday.  This all deserves a little detail.

Kyla's problem is at the neuromuscular junction.  The nerve sends Acetylcholine to the muscle where it is received by Acetylcholine receptors.  Also in the junction is Acetylcholinesterase that cleans the Acetylcholine that remains in the neuromuscular junction (this is really a crude explanation, but it makes sense to me).  Kyla's problem is she is deficient in Rapsyn, which is crucial to proper function of the Acetylcholine receptors.  She currently takes Mestinon, which inhibits the Acetylcholinesterase, thus giving the Acetylcholine more opportunities to find the receptors.  This has been our miracle drug.  3,4 DAP helps send more Acetylcholine into the junction and has been positive in treating folks like Kyla.  There are risks with this, however, and we will not try this at this time.  Two other drugs have been clinically shown to help folks like Kyla, but both Dr. Engel and Dr. Selcen told us that it is not known why these two drugs work.  They are Ephedrine and Albuterol.  Ephedrine is almost impossible to get, but Albuterol is easy to get and seems to work better than Ephedrine.  So, we are going to start Albuterol tomorrow.  We also will try dialing back the Mestinon a little, as Dr. Selcen says our dose is too high.  We have no tests tomorrow, and will see both doctors tomorrow at 1:30.

The other big news of the day is that the EEG was normal, but when Kyla was sleeping during the test, her oxygen saturation level was low.  In fact, it was low enough that it should have woke her up, but it didn't.  So, we have a machine to measure her oxygen saturation while she sleeps tonight.  We'll see what happens with that.  

Even though it was a long day, we did have fun again tonight.  After an adventurous meal, we had ice cream and went swimming again.  Laura is finally starting to swim around a little and Kyla continues to have a blast in the pool.  

Thanks David and Stevo for the comments and your prayers.  Thanks to all who are praying for our little Kyla.  She bounces back from hard times very well and this trip has been no different.

The following slide show are pictures from the day.

  

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No Mestinon

For those who didn't know Kyla before she started taking Mestinon, check out her eyes on this video. She took Mestinon at 11:30 am on Sunday, then didn't take it again until after the EMG. This was to see more accurately how the 3,4 DAP would react with Kyla. Anyway, this video was taken before the EMG and she was pretty droopy. Follow her right eye as the video progresses. Her right eye tends to turn out when she is sick or tired. Without Mestinon, it just turns out consistently. Her mouth also doesn't close in this video. She also has a hard time keeping her head upright.

Dr. Engel

We visited with Dr. Engel today.  The above image is from the Mayo Clinic's Neurology display.  He's the guy who does a lot of research on folks like Kyla.  In our meeting with him, we discussed how well Kyla is doing.  He said the EMG looked good from this morning and that the 3,4 DAP appeared to help her a little.  However, she is doing so well, he would recommend that we not start the 3,4 DAP as the benefits do not outweigh the risks since Kyla is doing so well.  

This is Neely typing and Sabrina and I haven't fully discussed this yet, but I'll give my gut reaction to this news.  On one hand, it is great to hear that Kyla is doing so well for someone who has a Congenital Myasthenic Syndrome.  She completed Kindergarten and did really well in the process.  On the other hand, if there is something out there that will help her even more, I would love to give it a try.  She has trouble with fatigue in so many areas, such as writing, looking at the board, reading, running, and on and on.  

We have 5 more tests scheduled and meet with Dr. Engel and another neurologist later in the week.  By then, he may have other recommendations.  

Kyla is recovered from the EMG and is ready to go to the park.  Here's a few pictures from today's visit to Mayo.

Pictures from today's EMG

Kyla at St. Mary's Hospital getting ready for the EMG

What a family!

Kyla and Mom getting ready for the EMG.  Sabrina got to go and watch them start the iv before she had to come to the waiting room with me while the doctors did the EMG.

Recovery.  Kyla was pretty groggy.

Mayo Monday

Well, test one went as planned with a couple of additions we didn't know about initially. She actually had the EMG while on a dose of the new 3, 4 DAP (which is in pill form, and Kyla swallowed it!). The doc, Dr. Krum, said he saw an improvement in muscle response during the test. Praise God! We see the CMS doctor, Engle this afternoon. He should be able to tell us more then. Thanks for the prayers. She was a trooper during a not so pleasant test! God is good. More later today.
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Mayo Sunday

Today, we went to the Underwater Adventure Aquarium at the Mall of America. The girls had a blast going through the aquarium 3 times plus they got to pet the Stingrays and small sharks (no teeth!). Kyla also got to ride a roller coaster with me and the Log Ride with her Pa. About midafternoon, we headed south to Rochester and we went swimming in the pool at our hotel. All in all, another great day of travel, with Kyla continuing to say this is the best vacation of her life. We start at the Mayo Clinic tomorrow, with a 6:45 am appointment for an EMG.
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Mayo Trip Saturday

We flew to Minneapolis today on the beginning of our Mayo adventure.  Both Kyla and Laura did exceptionally well on the plane.  When we arrived, we checked into our hotel and headed straight for the Mall of America.  Kyla really liked Lego Land and riding a few rides in Nick Land.  Laura also rode a couple of rides and loved them both.  Pa and I brought the girls back to the hotel where we are trying to get Laura to sleep.  Sabrina, Meemaw, and Aunt Clarissa stayed at the mall and we'll go get them later.  All in all, it was a very smooth day of travel and spirits are high in the Twin Cities.  

Tomorrow, we will experience the Underwater Adventure Aquarium at the Mall of America before heading south to Rochester.  Praise God for a great day today!

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Getting ready for the trip!

Well, here it is a few days before the trip and the girls and I are beginning to get into a Summer routine, finally. Last week was fun but hectic with VBS and Sports Camp. This week we have cleaned a bit, played a bit, and rested. I am taking some classes at night this Summer to get my Visual Impairment Endorsement. That includes Braille class. WOW! That about says it. We are excited about the trip. I had a talk with Kyla yesterday about what was going to take place starting next Monday at Mayo. She was a little teary and that is understandable. She still amazes me with her spunk and determination. She is a brave girl! We are working on Psalm 46:1 this week. Kyla and I decided that we need God to be our "refuge and strength" all the time, but next week at Mayo might be some of our "times of trouble." :)
Love you all!
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Here are a few pics from the backyard...

Mission Quito

I spent June 1-8 on a mission trip to Quito, Ecuador. This has nothing to do with Kyla, but if you are interested, you can check out our blog from the trip by going to http://www.missionquito.wordpress.com/ and clicking on Team Deportes. It was an incredible trip.
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Mayo Trip Upcoming

We are preparing to go back to the Mayo Clinic. Sabrina’s parents and sister are traveling with us as we are all flying to Minneapolis on Saturday. We’ll drive down to Rochester on Sunday and will start our appointments at Mayo a week from tomorrow. Kyla is scheduled to have an EMG on Monday, June 23 at 6:45 am and will meet with Dr. Andrew Engel later that day at 1:15 pm. On Tuesday, she has an Electrocardiogram at 8:20, a chest X-ray at 9, blood tests at 9:30, and she will also get to pee in a cup for a urine specimen. On Wed., she has an EEG at 9:30 and an appointment with Dr. Duygu Selcen at 1. We fly home on Thursday.

My prayer for this trip is that Kyla's tests will all be fine and that she will get to start a new medication called 3,4 DAP. This medication has been known to cause seizures, so a clear EEG is a must. Also, I pray that if she gets to take the medication, that it will not cause seizures. On the one hand, Kyla's quality of life is great now, so we're hesitant to mess with what she has going on now. The other side of it is that if there is anything we can do as parents to improve her strength and improve her quality of life, we want to do that. So, we're praying for wisdom and guidance and discernment as we go.
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VBS & Sports Camp

Kyla had a big week last week, attending Vacation Bible School and Faith Sports Camp at our church. She started every morning at 8 and lasted everyday until 5 pm. For a "normal" child, VBS and Sports Camp is a long week, and it was especially tiring for Kyla. But she did incredibly well. She was tired when we got home everyday, but she bounced back well. She also really enjoyed learning Bible verses, singing songs, and hanging out with friends. In Sports Camp, she learned to dribble a basketball, did well hitting a baseball, and showed great improvement in all the sports by the end of the week. We haven't played a lot of sports with Kyla because they don't come easy to her and we all get frustrated too easily. Sports Camp was a good opportunity for her to have fun playing and learning. Kyla will never be a star athlete, but I do want her to learn to have fun being active and playing. As she gets older, I want her to make choices to be active and have fun playing games and sports and exercising. This was a great week for her because she just had fun being active and it didn't bring her down.
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