So much has happened over the last two and a half months, I’m not even sure where to begin. Kayla has hit so many new milestones recently, it’s just unbelievable. Jeff and I are learning to live day by day and enjoy every moment of her progress. We know that with a disease like Myotonic Muscular Dystrophy, there is no guarantee of how long the upward trend might last. We are so appreciative of each and every new milestone that Kayla has met so far. Hopefully we won’t bore you with every detail but even the smallest changes are monumental to us.
Kayla started being weaned from the ventilator on December 23rd starting with 30 minutes at a time, 3 times a day. She tolerated that very well and we have been able to increase that by 45 minutes every 3 days over the last two months. I am so happy to tell you that Kayla is able to come off of the ventilator for up to 5 hours at a time now, up to 3 times a day. She usually doesn’t stay awake for the entire 5 hours so we have to split it up over the day but basically she is off of the ventilator for almost all waking hours during the day. We are required by the doctor to always put her back on the ventilator when she falls asleep since she breathes shallower when she’s sleeping. The doctors are worried about her oxygen saturation levels dropping or her CO2 levels rising. Kayla will be scheduled for a sleep study in a controlled sleep lab in the next month or so. They will take her off of the ventilator overnight and closely monitor her O2 saturation and CO2 levels to see if she is able to be weaned from the ventilator at night. Until then, Kayla will remain on the ventilator at night.
Over the Christmas holiday, Kayla weighed 14 lbs. 8.5 oz. As of today March 21st, she weighs just over 20 lbs. She is growing so quickly, building tons of muscle mass and getting stronger and stronger by the day. Kayla is already off of the growth charts but that isn’t such a big surprise if you’ve seen Jeff and me. Kayla is now 29 1/2" tall and is wearing 12-18 month clothes.
Kayla sat up by herself in her booster chair for the first time on January 8th. She struggled to keep her head up by herself for very long but every day she gets a little bit stronger. On January 12th she started lifting her head up a little while lying on her tummy. That is a huge accomplishment for her since, not long ago, she barely moved her neck at all. On January 19th, she started purposefully batting at her toys when lying on her play mat. She now plays with her toys and grasps them with both hands trying to put them all in her mouth. If she can get a hold of something, it always ends up in her mouth now.
Kayla went to the cardiologist on January 17th to have a follow-up EKG of her heart. She still has a slight VSD (hole) so the doctors will continue to monitor her but they are hopeful that this issue may resolve itself. Kayla has not shown any signs of heart conduction problems which are very common in Myotonic Dystrophy. The heart issues with MMD usually show up later in life but the doctors will continue to monitor her over the years. Kayla received her first official pair of foot splints (AFO’s or Ankle-Foot Othosis) since she’s been home from the hospital. The AFO’s are from Shriner’s Children’s Hospital and are used to keep her feet from dropping (or pointing). The AFO’s keep her foot at a 90 degree angle to keep her Achilles tendon stretched out so that someday in the next year or so, she will hopefully be able to walk on her own.
Jeff’s cousin Linda came to visit us from Baltimore, MD on January 25th. She stayed with us for about 3 weeks and was an unbelievable help for Jeff and me. We really enjoyed her company and she had a chance to spend lots of time getting to know Kayla and got to see her hit lots of new milestones. Kayla started sucking her thumb the day Linda arrived. She learned to hold her thumb in her mouth by using her other hand to hold it in. What a smart little girl.
Kayla celebrated her 6 month birthday on January 28th. We bought her a chocolate cake and let her pretend to blow out the candles. She seemed totally amused by the ritual until we all started eating the cake without her, and then she seemed a little frustrated. On January 31st, Kayla was playing with one of her favorite toys and she figured out how to make it spin on her own. She kept spinning the wheels on the toy over and over again and seemed proud of herself for figuring it out.
On February 4th, Kayla had her first taste of solid food. We fed her baby oatmeal! It was really cute to see her facial expressions while she was eating. She really seemed to love it. We were a little concerned the first time she had solids that she might aspirate but she tolerated the food really well. On February 12th, Kayla's cousins, Maya and Noelle came to visit. They had a chance to see Kayla for the first time since her birth and they were able to hold her and feed her a bottle. I added some pictures to her photo album for February. On February 18th, Kayla held her bottle on her own for the first time. It’s a little heavy for her but she tries really hard to hold it on her own now.
On February 24th, we had a little scare. One of Kayla’s nurses seemed concerned that her fontanels on her skull might be closing early. They usually close at around 18 months but Kayla’s seemed to be closing a bit early. We took Kayla in for a skull x-ray right away but the doctor said everything looked fine. If there had been an issue, Kayla would have needed to undergo surgery. We were so relieved to hear that there wasn’t an issue.
On March 3rd, Kayla rolled over from her tummy to her back by herself for the first time! We all celebrated and took videos of her new accomplishment. Now Kayla rolls from side to side and is almost able to roll from her back to her tummy on her own. She works so hard during her daily physical therapy sessions. She seems really determined to push herself to learn new things. It almost seems like you can see the wheels turning in her brain as she figures things out.
On March 7th, we gave Kayla her first taste of vegetables. We gave her sweet potatoes which she got so excited about. She is a huge fan of sweet potatoes and ended up wearing more than she ate. We are looking forward to slowly introducing her to other foods over the next few months.
On March 10th, we took Kayla to Shriners Children’s Hospital for a visit with her Muscular Dystrophy doctor. They evaluated her feet and were a little concerned that her ‘foot drop’ might not be improving as quickly as they would have liked so they recommended putting Kayla in serial casts for a few weeks to speed up the process. Kayla got bright pink plaster casts on both feet which she is tolerating really well. However, after we left the hospital after having them put on for the first time, we had to return within an hour because Kayla had worked one of her feet completely out of the cast. She is so strong! The doctors re-casted her feet and used a type of glue to keep her from pulling them out again. So far she has tolerated them really well.
On March 12th Kayla was weaned off of her feeding tube as a trial. She has continued to gain weight since then so she is now officially weaned from tube feedings as of today. She now completely gets her nutrition from nursing and solid foods. This is such an amazing milestone for her since I remember the doctors telling us in the beginning that they weren’t sure that Kayla would ever eat by mouth. I love when she proves the doctors wrong.
On March 15th, Kayla's Pulmonologist allowed us to start using a Passy-Muir valve (PMV) over Kayla's trach when she is off of the ventilator. This is a one-way valve that allows her to breath in through her trach but when she exhales, the air is blocked in the trach and is pushed out through her mouth passing by her vocal cords. When Kayla is wearing her PMV she is able to coo and make all kinds of sweet noises. She seems to really enjoy hearing her voice so we are hoping that over time she will tolerate it well so she can wear it more often. For now we are able to use it just 15 minutes at a time, up to 4 hours a day. Unfortunately, last Saturday, March 18th, Kayla got sick. We had to discontinue her PMV use and she was put on antibiotics. It was a little scary at first because with a trach she is unable to handle her own secretions so we have to help her by suctioning her trach a lot. She is starting to recover a little now so we are hoping she is through the worst of it. We are very lucky that she has gotten so strong over the last few months because she is now able to fight off a cold much better.
Jeff and I are hanging in there and are trying to figure out how to do our part in helping to find a cure for MMD. We have decided to take part in the first Annual National Myotonic Muscular Dystrophy Fundraiser and are now co-chairs of the event. It will be an email/mail campaign so I’m sure that you will all be hearing from us soon.
Thank you so much for hanging in with us through the last eight months. It has been such a challenging time but we couldn’t have made it through without all of your support. Now that we are settled into a routine at home, we would love to get back in touch with everyone so feel free to give us a call when you have time.
All the best,Lisa