Not a "Humerus" Week

Macayla's arm is broken! She was getting a bath and as we were repositioning her there was a loud pop. Macayla did not flinch or seem in distress. We checked her shoulders and hips and everything seemed fine. We commonly hear pops in her joints when moving her around. She did not show any signs of pain and even smiled when I talked to her. She seemed to be a bit sleepy, but she has continued to have "big" seizures this week and we attributed her lethargy to those. But about an hour later, our nurse checked again and noticed Macayla definitely had a break above the right elbow in the humorous, make that humerus. That's the big bone of the arm. She was reacting to it and was in definite pain. The swelling began.

Unfortunately, Macayla continued to have seizures throughout the day along with a lot of myoclonic jerks that affected her arms, head and legs. The jerks hurt the arm more. Fortunately, xray confirmed the bones were not displaced so they were still lined up. It was a spiral fracture caused by twisting. We still don't know how that happened. We are thankful for our nurse's attention to detail. She has a special-needs child of her own and understands non-verbal cues intuitively with Macayla. Now Macayla has a bright green cast on her from the armpit to her fingers. Jacob is excited to sign it once she is not so tender. But the myoclonic jerks continue and even in that arm. We are trying to keep the pain meds on board as the cast immobilizes, but it cannot stop the muscles from jerking. Our prayer today is that the seizures would stop.

The neurologist has changed her medications in hopes of getting better control of the seizures. It will take about a week to get her built up to the full dose of Depakote. We used it about four years ago and it did not help. But then she was only having petit mal seizures. Her seizures are different now and hopefully the Depakote will be a good match for them. Historically, Macayla has shown improvement with medication changes for a "honeymoon" period and then her condition returns to its previous level. The meds have never kept up with her seizures. But this girl is full of surprises so we will have to wait and see.

She is smiling and happy this morning and we will do our best to help her stay that way.

New Ground?

In the previous post, I mentioned Macayla had a seizure and was passed out. That was on a Tuesday morning. She kept having grand mals through that following Friday! Mulitple each day. Last week she was pretty clear until Saturday morning she had a short one, but it was not bad. But she has struggled more than usual with breathing and processing saliva. She gagged and choked a lot Sunday and Monday of this week. At times she would turn blue briefly. The strange thing is that a majority of those times we would not get any thing out when we suctioned her.

We also noticed she would sound raspy or gargle when breathing in but not out. So, we are beginning to think she may be losing a little tone around her airway. It may be the actual muscle tone or it could be a signal not coming from the brain properly. If it is a signal problem, it will probably return and go away several times and to varying degrees. So, if that is the case, we are going to explore what options we have to make her most comfortable through those moments. This is a common progression in Battens and other progressive disorders. One option may include using a CPAP to keep enough pressure in her airway to help hold it open. Interestingly enough, I was just diagnosed with sleep apnea and will have to be fitted for a CPAP. Macayla and I might be twins! However, there are some drawbacks to a CPAP for Macayla such as the mask irritating her face and not staying in place when she wiggles in the bed. The mask can rub and irritate the skin and cause breakdown. (Maybe I don't want one either!) There are nasal cannulas but she keeps her mouth open all the time, so those probably won't work. But, we have to observe more prior to making any steps in this direction.

She breathed better yesterday, but was exhausted from the previous two days and nights of struggling. She slept through a better part of yesterday and last night. Today, she will see her neurologist for a six-month checkup. We are hoping to learn a little bit more about what these stages can look like. We may see our genetics doctor next week. He's great to discuss these types of challenges with and what decisions we may need to make. This may be new ground or just a bad week or both. By the way, as I'm typing this, she just sneezed and it made her smile!

We are praying for wisdom and discernment and her comfort and healing.

Last January

Last January was a rough one for Macayla. This one has been much better by comparison. In fact, the nurse manager from our nursing provider saw Macayla on a visit last January and a couple of months later confided that she was not sure she would see Macayla again. So, we are thankful we have done much better this year!

But we have had the usual holiday/end-of-the-year changes. Mac had five grand mals Thursday/Friday and had another one this morning. She is passed out for now, but we'll see how she does when she wakes up. This January has had a lot less seizures and a lot less catheters than last January. But it is always difficult to assess where Macayla is on her journey. It's always new territory for us and her. We wish we could know more about what she feels and thinks. We know she responds to our voices and we can get her to smile with specific sounds and touches. She even laughs some. I just wish... it goes without saying. One day, that which we most hope for and desire will be realized. There will be no more Januaries to worry about. Faith, hope and love, all three live, but the greatest is love. Because of Christ, one day we will see fully and Macayla will be whole. Love is the greatest because that's all we will need then. Our faith will be fulfilled. Our hope realized. All that will be left is love expressed in eternal worship and fellowship with our Creator.