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.

The Teacher

Sonny and Cher tried to capture the idea in their hippy way with "The Beat Goes On." The Byrds quoted it in their lyrics to "Turn, Turn, Turn!" The new year is upon us and this used to be a big deal to me in younger years, but now it simply means I have to be careful how I write the date for the next month. It used to mean new-years resolutions I would never keep. Now it means if I am not doing it already, there is nothing magical about January 1st that will make me start. It used to mean parties and the pursuit of debauchery, but now it means the pursuit of dinner, put the kids to bed and hope for a good night's sleep. It used to mean new possibilities, but now I see that any day is a new possibility and a gift from God.

We are blessed to have another year in marriage, and two wonderful kids. We are blessed to have had another year with our family. We have lost some family and gained some family. We have gained new friends. We have gained a few pounds and some gray hairs. We are blessed to know that the challenges we faced have been used by God to grow us and impact others. What does 2010 hold? The same as 2009. God will be in the midst of the circumstances that ebb and flow, change and challenge. Macayla will continue her digression. We do not know if she will see 2011 (in fact it is December 31st so 2010 is still in question), but she is full of surprises and God will be in them.

Long before Sonny & Chere or the Byrds, the Teacher captured the wisdom of ongoing time in the book of Ecclesiastes.

"There is an appointed time for everything. And there is a time for every event under heaven - A time to give birth and a time to die; A time to plant and a time to uproot what is planted..." Ecc. 3:1-2 NASB

The ancient Teacher who wrote Ecclesiastes saw the endlessness of pleasure seeking and that there truly is nothing new under the sun. He concluded rightly that wisdom is found in the reverence of God and following His commandments. My prayer for the new year, the new day, today, is that I will seek Him and follow the path He has for us. In spite of all that "changes" and repeats itself, Christ is forever! He does not change, but we need to and I thank Him for it.

Weekly Seizures

Macayla has been having grand mal seizures weekly now for the last three weeks. Today she had one after lunch and is finally passed out from the Diastat. We think that if we give her Diastat immediately after the first seizure she will pass out and not have any more. The last time I waited to give it after the second seizure (which is what you're supposed to do) she kept having them every two hours. Now, we just hope she will wake up seizure free.

Interestingly, this seizure started slowly and in a different way than usual. It was good because it gave me enough warning to get a chux pad and the suction machine ready in case she threw up at the end as she typically does. Fortunately, she did not throw up but kept having bad reflux for a while afterward. It's just more of the changes we see in her at this time of the year.

Merry Christmas in the Midst of the Yuk!

We certainly are a pitiful bunch in the Smoak house. Jennifer had a low-grade fever all last week but the docs could not figure out its cause. We think it was a sinus infection, but because of her symptoms, the hospital would not allow her to come back to work for seven days. Jacob brought home a virus from school and threw up quite a bit on that Friday. Not a fun way to start the weekend. After the weekend was over, I had the virus and twenty-four hours later Jennifer had it. Just in time for her to go back to work and she couldn't! All of our taste buds have been off ever since and Jacob still doesn't have his appetite back. Macayla has avoided it so far, but she has had some dark, puss-like ooze coming from her right ear. She has not had a fever but the ear is very sensitive and causing her pain. We started an antibiotic/steroid ear drop this week. After 36 hours the dark ooze has diminished. But we still have six more days of drops to administer. Her secretions in her nose and throat are still out of control and we have to suction her a lot as she is coughing and gagging so much. I wish I could make it all go away. Of course, none of my "old geezer" problems of gout or joint pain in my hands have ceased. My doctor wants me to get a sleep study (actually Jennifer wants me to more due to snoring!) and a nerve conduction test done as well.

Jennifer's grandmother and my grandparents are all experiencing failing health. Jennifer's grandmother is in the final stages of cancer and we are not sure how long she will be with us. My grandmother is struggling with Parkinson's and dementia among other things and my grandfather struggles to care for her.

In spite of these things, I have been more in the "Christmas spirit" this year than usual. I have to admit that past Christmases has been anti-climatic for me. As a believer, Christ is always with me and His grace is always a present gift. Don't get me wrong, I love the time with family and the wonderful feasts we have together, but the holiday itself has not been as captivating as it has been this year. I have experienced a hope and peace that indeed surpasses understanding. This is not to be confused with "positive thinking." In the midst of these things, I never consciously decided to utilized the so-called "power" of positive thinking to wade through. So many self-help gurus push people to smile their way past problems instead of actually dealing with them. No, we still have to deal with the vomit of stomach viruses, dying loved ones, and Macayla's annual end-of-the-year digression. It's just that Christ has reminded me once again that He is with us in the midst of it. He doesn't invite us to escape the struggles, but to face them; with His strength and for His glory. There was no room for Him at the little inn of Bethlehem, but there is room for Him in our hearts and minds. But so often I put him in the stable out back instead of giving Him his rightful place on the throne. The presence of His peace and joy this season has reminded me of His rightful place. Wise men still seek Him and even lowly shepherds can share the Good News.

What I Was Going to Say...

I was going to post an update and say how Macayla has had a great, grand-mal-free week and she seems to be on the mend. I was going to write how she seemed to be more herself this week. But this morning she showed a sign of seizure activity. She started pursing her lips repeatedly and that seems to be associated with the start of grand-mal seizures. So, I'm not going to write what I was planning to write. I'm not going to say we are out of the grand-mal woods yet. For, she may be starting a weekly pattern instead of a monthly one. If I don't say what I was going to say, will it help things turn out the way that I was going to say they would? Well, I don't believe in jinx but I certainly think we can say things hastily out of our lack of knowledge only to be proved wrong by the future. So, I will just be satisfied to say that today, Macayla has started off happy with a few signs of neurological issues, but thankfully no seizures yet.

We are thankful for this amazing girl and her brother. We are thankful for the God who gave them to us. We are thankful he gave us each other. We are thankful for the amazing blessings he has put in our lives. We are thankful that he is patient, merciful, loving and just. We are thankful for how he teaches us through our children. We are thankful that he loves us as we are AND that he loves us so much he won't leave us the way we are. We are thankful for what he did at the cross and that there is no condemnation for those who are in Christ. These things are not said in haste and the future will prove them correct as the present and past already have. Today, we give thanks to the One who gave us abundantly more than we could imagine. That's what I was going to say.

She May Be Done

As of last night, it appears Macayla's seizures stopped. We will see how she is later today. She is just beginning to wake up which is a good sign. Yesterday morning she was post-ictal and would not wake up. We are getting her Lamictal switched back to the brand name instead of the generic. We actually started that last night, so hopefully we can see some improvement.

She was in a good mood last night. She laughed some and fell asleep right after we gave meds, which is the norm. She had not been doing that on the generic. Granted, there are so many variables with this disease and the brain that we can never be positive if that was the cause, but the timing of these seizures fits with the medication change. So, we will see.

Thanks to the folks who have offered up many prayers and sent messages. We appreciate it beyond what words can convey.

Further Breaking of the Pattern

You can check out the previous post on this ongoing challenge. Macayla awoke this morning with smiles and even seemed more relaxed. She started getting fidgety around lunch time, but was still very reactive and happy. When seizures are lurking close by, she tends to have increased rigidity in her arms and she purses her lips repeatedly. (I like to think she just wants to kiss her daddy, but in fact it's neurological dysfunction. Of course, Jennifer is probably neurologically dysfunctional since she actually does kiss me!) Anyway, Macayla became more and more rigid and "twitchy" as the afternoon wore on and tonight she had a new kind of seizure. Actually multiple seizures.

She became rigid in her limbs, turned her head to the right and her mouth locked into and "O" position for quite a while. She had little versions of this just before the minute or so long one. She gave little sounds out just before they started. When it was over, she became post-ictal and has been out ever since. The way this presented was different than any we have ever seen. Fortunately, she breathed well through the whole thing and never threw up. So, tomorrow we will get blood drawn to check her Lamictal levels and make sure we have enough on board. We hope she will do well through the night and stay asleep and seizure free until morning. But she is just continuing to show us that Battens is not a disease of patterns.

Broken Pattern

Macayla has had five grand mal seizures so far today. They started at 7 a.m. and she has not responded to the emergency meds like Diastat and Versed. She is passing out after them for a short while, but as soon as she wakes up, she has another seizure. She even had one while she was still asleep. Fortunately, she has not thrown up much because she hasn't eaten all day. Unfortunately, she hasn't eaten all day. These seizures started off stronger than usual and her oxygen level has stayed low for a greater portion of the seizures than normal. I wish I could snap my fingers and fix it.

Hopefully, she is finished but they have been coming every two hours and the last one was one hour and 45 minutes ago. All of this to say, she had a grand mal last week and now she's having a series this week. The pattern this summer was her series of grand mals would come once a month. Thus, we think they are hormonal. It was unusual to have these a week apart and for her to have a different oxygen saturation. But in the bigger scheme, historically, the disease has progressed between Thanksgiving and Christmas each year. She may be following that pattern. Then again, our insurance said they would only pay for the generic version of Lamictal and that started last week. In the past, the neurologist insisted she be on the name brand. We will have to get the neurology office help us to see if this minor change could cause an increase in seizures. There are so many variables and pseudo patterns in this disease, it's hard to keep up.