Our "New Normal"

Jeff and I returned from Los Angeles Sunday morning (9/18) to find that Kayla had been re-intubated and put back on the ventilator. After being off of the ventilator for 43 hours her lungs were worn out and they could not support her breathing anymore. We were very discouraged and cried a lot knowing that the next step would be a tracheostomy tube or ‘trach’. The endotrachial tube (ET) in her mouth has a short life span and two months is about as long as the doctors like to have it in without worrying about it doing damage to the vocal cords and trachea.

Jeff and I enjoyed meeting all of the families at the fundraiser in LA. We got to meet several families who had babies born with Congenital Myotonic Dystrophy so it was really great to share stories and learn from their experiences. We also met many people who have the juvenile and adult forms of Myotonic Muscular Dystrophy (MMD). What a great group of people! The most interesting thing that we learned was how varied the symptoms can be even with siblings and members of the same family. This gave us hope for Kayla’s long term prognosis although she has the most severe form of this dystrophy.

Kayla was scheduled for surgery Wednesday (9/21/05) afternoon for both a trach and a gastrointestinal (GI) feeding tube. The feeding tube is placed directly into her stomach through her stomach wall. Babies with Myotonic Dystrophy usually have problems sucking and swallowing properly and there is always a fear of aspiration where food can enter the lungs and cause pneumonia and possible death. They have to be sure that Kayla has an alternate way to eat until further testing can be run on her to ensure safe oral feeding.

Both of Kayla’s surgeries went well and she is now in her own room recovering. She was moved from the Neonatal ICU to the Pediatric ICU where one parent is able to stay with her 24/7 so life is starting to get just a little bit easier. Each room has a phone, TV & VCR so we can keep up with what’s going on outside of our little world here. They also feed breastfeeding mothers so I don’t have to leave the room to find food everyday. They also have room where they can put a cot for another parent to sleep so it’s nice to both be able to stay at the hospital all night if we want to. It has been so nice being able to wake up next to Kayla and capture the moments that we have been missing over the last two months of her life. Life is feeling just a little more normal now. As Jeff and I like to say, this is our ‘NEW normal’.

Yesterday morning the doctors began feeding Kayla again and she is tolerating her food well. They have increased her milk from 3 ml/hour yesterday to 8 ml/hour this morning and 13 ml/hour this afternoon. They are working towards 20 ml/hour by the end of the day today if all goes well. Kayla has been breathing really effectively since she had her trach put in. They have moved her to the lowest possible ventilator settings and she is tolerating it well. Yesterday they tried her off of the ventilator for an hour and she did very well. They will begin weaning her for a couple hours per day and will continue to monitor her progress. The nice thing about having a trach is that they can easily take her on and off of the ventilator without any damage to her trachea or throat. It took us a little while to get used to the idea of the trach but now we are seeing the benefits of it.

Grandma Jane left yesterday (9/23) to return back to Santa Cruz after almost 3 months with us. She has been an unbelievable blessing for the three of us. Besides keeping us fed and organized, she also provided so much love, friendship and support during these difficult months and we will miss having her around. If I can be as great a mom to Kayla as my mom is to me, Kayla will be one lucky girl. We love you MOM!

Today is Kayla’s original due date (Day 59 in the hospital) so Jeff and I are going to celebrate in her room tonight. She is officially 40 weeks old today and weighs a whopping 8 lbs. 1.6 oz. She is getting cuter by the minute, especially now that we are able to finally see her face without tubes sticking out and tape all over it.

We will keep you updated on her progress.

All of our love,

Lisa

Heading Out to a Family Conference for Myotonic Dystrophy

Jeff and I are heading out to Los Angeles this morning to go to a Myotonic Dystrophy conference in Manhattan Beach tonight. We will be meeting with several families that we have been chatting with on a Myotonic chat group for the last month. We are eager to learn as much as we can about Myotonic Dystrophy so this event should be a great start.

Kayla has made some progress over the last few days. Her feeding tube was moved from her lower bowel to her stomach two days ago and she is handling the food well. Yesterday (9/16/05), Kayla's breathing tube was removed and she is only being supported by a nasal canula with pressured oxygen. So far she is hanging in there although she has had a handful of times when her vital signs have gone a little off the charts. She has recovered from the heart drops and C02 increases well and we are hoping that she continues this progress and can stay off of the ventilator. Unfortunately, if she fails this time, she will need to have a trach put in but we are feeling like she will make it so we aren't going to worry about that for now.

Kayla weighed in yesterday at 7 lbs. 11 oz. and is is getting bigger by the day! Go Kayla!

We are rushing out the door but will write more when we return home tomorrow.

Love,

Lisa

Kayla Has Congenital Myotonic Dystrophy

Well, after 46 painstaking days of waiting for a diagnosis for our sweet little Kayla, we finally received word back from the doctors yesterday. Unfortunately, the news is not good. Kayla has been diagnosed with a severe form of Congenital (from birth) Myotonic Muscular Dystrophy. According to statistics, only 1 in 100,000 babies are born with this severe form of the disorder. As a general rule, the earlier the disease is detected, especially at birth, the more severe the symptoms will be. It is a multi system disorder that can affect many parts of the body. Most patients experience symptoms including muscle and respiratory weakness, mental and learning disabilities, heart problems, speech, hearing and vision problems, as well as issues with swallowing and processing food. The risk is very high that it could be life threatening especially during the first few months of life as her muscle weakness makes her more prone to respiratory failure, infections, and other ailments.

We feel that Kayla has been put on this earth for a reason and we are not willing to think about life without her right now. So far she has proven to be such a strong fighter that we have no reason to believe that she won’t continue to keep improving and teach us all about the fragility of life and its possibilities.

In the next few weeks we will be faced with making decisions regarding her long term breathing and feeding support including a possible tracheostomy and insertion of a gastrointestinal feeding tube into her stomach.

We will be meeting with the doctors this week to discuss these issues and what the future may hold for Kayla. If you would like more information about Congenital Myotonic Dystrophy, here are a few web links. http://www.mdausa.org/publications/fa-mmd-qa.html#whatis and http://www.myotonicdystrophy.org/General%20Information.htm

We are hanging in, thanks to everyone’s love and support. Please continue to keep Kayla in your prayers

Love,

Lisa

Still Waiting for Results of the Genetic Tests

Hi, Lisa here! Well, I finally returned home on Thursday night after a long and draining week in the hospital. I won’t bore you with the gory details but basically my placenta adhered to the wall of my uterus and had to be removed by hand in a two and a half hour procedure. The doctors said they had not ever seen a placenta complication like this before. I think I’m trying to break a new record for weird pregnancy complications. I’m pretty exhausted after losing so much blood but I am slowly gaining back my strength.

As far as Kayla’s progress….the doctors suggested we attempt to test Kayla off of her ventilator last week. We were warned to keep our expectations low as it usually takes several attempts for babies to come off of their ventilators before they can successfully breathe on their own.

On Tuesday (8/30/05) at 11:30 a.m. Kayla’s tube was removed and she began breathing on her own for the first time with the assistance of an oxygen nasal tube. She was able to breathe on her own until Thursday (9/1/05) at 3:30 a.m., almost 41 hours! (We have added a new picture in the Photo Gallery) Unfortunately, the last nurse who had Kayla while she was off of the ventilator had her on her back which is most likely why she failed. It is the most difficult position for babies coming off of their ventilators. Kayla’s lungs collapsed during this attempt so we will have to wait until her lungs re-inflate before they attempt to remove her vent again. The doctor will write orders next time to make sure that she is kept on her stomach until she seems strong enough to handle being on her back.

On Tuesday (8/30/05), Kayla was fitted with two foot braces. These will help keep her feet from having ‘foot drop’ related to her muscle weakness. She will be wearing them two hours on and two hours off for the time being.

On Thursday evening, (9/1/05), Kayla set a new record by holding both eyes open for over two hours. To us, this was significant as she has only opened her eyes for several seconds once or twice a day in the past.

On Saturday, (9/3/05). Kayla weighed in at 6 lbs, 6 oz, a gain of 1 lb, 7 oz since her birth. She is now 19 ½ inches long, a gain of 1 ½ inches. GO KAYLA! Jeff and I were able to give Kayla her first real bath which was so fun for the two of us.  Kayla was also moved into a new covered crib that will allow us to protect her from the harsh lights in the NICU. We were so relieved that she will finally be able to get some rest.

We are still waiting on her genetic test results to rule out Congenital Myotonic Muscular Dystrophy and expect to get them back this next week. Kayla is only two weeks away from her original due date of 9/24/05 so we are getting closer to her next schedule brain MRI. Waiting has been the hardest part of this whole experience for the two of us but we are finally getting close to possibly getting some answers.

Thanks for hanging in there with us.

Lisa