MVP Award

The doctor provided a different pain medicine but said we could only wait and see about the pain. We must wait and see for the next 6 WEEKS! When he said that, my mind flashed to a scene 6 weeks in the future when I will be in rehab for my addiction to pain killers and my new residence would be in a padded room with a special jacket. It was hard to imagine 6 weeks of pain like I had been experiencing, but I have discovered that the new pain med is much more effective than the other. I still have pain, but it is more of a nuisance than anything now. The doctor admitted that there has only been one other man who has come close to the problems I have had, but he awarded me the prize. I guess it’s flattering to be the best at something. I get the MVP award (Most Vasectomy Problems). I’ve noticed that there are no crown or cash prizes with this title. But the gist of the situation seems to be that things should get better and there is no way to diagnose any long-term complications until, well until a long term has gone by. There has been more than one man I know that swears they will never, never, ever, ever, never have this procedure done themselves now that they have heard my story. The real strain in all of this has been around taking care of Macayla. Jennifer really did an amazing job at lining people up to take care of Macayla during the two weeks after surgery. Between Jennifer, her parents, my mom, neighbors, nursing, Macayla’s teacher, and friends, I have been able to go this entire week without having to lift the kids at all and meals have been provided. God provides through all types of people and avenues. What an amazing God and what an amazing wife I have.

The Wisdom of Bob Barker

Bob Barker always ends the Price Is Right gameshow with the sage advice to spay or neuter our pets. As it turns out, we don't have any pets. So, Jenny and I figured I was the next best thing to a pet. I can be messy and at times I shed, but in my defense I have yet to chew up a pair of shoes. Anyway, we decided that I should get a vasectomy. It is hard emotionally to decide not to have children anymore, but we don't want to take the chance of plaguing another child with Batten's Disease. I was more prepared emotionally for the decision than Jennifer. If we decide to bring more children into our family it will be through adoption.
The recovery was described to me by multiple men who had gone before me down this path of sterility. "Oh, man, I maybe took one or two of my pain pills and I was fine after that." Or, "I had mine on Friday and was normal by Monday." My personal favorite, "You'll ONLY feel a tug." I felt a tug alright!! It felt like a tugboat was pulling me off the table!! I almost came off the table and the doctor was worried I was going to grab him. After the procedure at 5:30 on Friday afternoon, Jenny drove me home and as we neared the house, I felt a huge wave of nausea. We pulled into the driveway and I passed out. We assumed this was due to the pain and adrenaline from the LITTLE "tug" I had just felt at the doctor's office. We went inside and I laid down in the bed and watched a movie until about 10:30. I decided to try to use the bathroom before I went to sleep, so I got up to go and took only a few steps when pain shot through my abdomen and nausea swept over me. I turned toward the bed and the next thing I know is there are paramedics in my bedroom.
Jenny explained that I had passed out again and that I had a seizure. I've never had a seizure before. It was midnight before I was truly alert again and then the waves of pain started. It took morphine to ease it off. We left the ER at 7 a.m. with really no answers except that the passing out and seizure could have been caused by a sudden drop in blood pressure. My doctor and the ER doctor both stated that seizures can happen that way. As far as the severe abdominal pain, we have no idea. It is Tuesday and it still hurts. We are going to see the doctor tomorrow to see if he has any ideas. So much for, "I had mine on Friday and was back to normal by Monday." So, we hope to find out what would cause continuous pain in my abdomen accompanied with waves of stabbing pain.
In the meantime, we are so thankful for all of the help we are receiving from Jennifer's parents, friends and neighbors. We couldn't take care of the kids without them. God has moved people to bring us meals unbeknownst to them that our oven also quit working this week. He certainly provides. I do pray He will provide some answers as well.

ER Revisited...Almost

Macayla hit her chin last week and split it open which prompted an ER visit (see below). Last night, she was crawling and I was right there with her and she fell again. It opened up a little bit of the cut again. I put gauze on it and with the use of some bandaids, held it in place under her chin. She soaked the gauze in blood. I replaced it and slowly but surely it became soaked as well. I decided that we would run to the ER since it would not stop bleeding, but Jenny brought home some special glue that would hold Steri-strips in place. (These are just special pieces of tape made to hold skin together.) 24 hours later, the strips seem to be holding in spite of drool, drinking and eating. So, for now, no ER.

Oh Holy Night

By the way, there are some versions of Oh Holy Night that are definitely better than others. Some versions have really captured its essence while others drain it of its meaning. But one of my favorite versions can be heard at the following link. Enjoy!! Oh Holy Night
Might I add, that this link was passed on to me by a pastor who was trained in classical music. It has inspired me to consider music ministry, especially as a solo vocalist. You know it's got to be good if it could get me to sing!! ;)

The Soul Felt Its Worth

Every year the radio stations start playing Christmas music more and more, earlier and earlier. I’m waiting for the year they end the broadcast day on July 4th with the “Star-Spangled Banner” and start July 5th with “Jingle Bells.” But each Christmas season I find myself scanning the radio stations for the song “Oh Holy Night.” It is my absolute favorite. It captures the essence of what Christmas (from Christ Mass) is about, what it means that God became flesh and dwelt among us. The song identifies our existence in a world that lay in sin and error pining. But when HE appeared, the soul felt its worth. This line doesn’t say that the soul wasn’t worth anything until he appeared, but it says that the soul had worth but apart from Him we can’t recognize it. We have to come to realization that God loves us so much that he was willing to put on flesh with all of its weaknesses and nerve endings and pain and not only live in that state, but die that way. He did it so we wouldn’t have to die that way, but so that we could live in glory and freedom. Our souls feel their worth when we realize that the God of the universe lived and died for us in the flesh. He values us, so we are valuable. Self-esteem is truly accomplished when we learn of God’s esteem for us. This news gives us a thrill of hope and in our weariness we can rejoice. “Fall on your knees,” the song says. For some this may seem like a demeaning demand, but for those who have truly discovered their worth as a child of God, falling on their knees is a natural response. It is actually joyful and awe-filled instead of being compelled by an unwilling sense of guilt-driven duty. The song takes us to that moment in time when God in the flesh could be observed in Bethlehem. “Led by the light of Faith serenely beaming, with glowing hearts by His cradle we stand.” We can only come to the cradle and likewise the cross by the light of faith and that faith is authored by the One that was in the cradle and on the cross. Then the song states the amazing and almost paradoxical essence of the Gospel, “The King of kings lay thus in lowly manger.” The God of the universe lying in a feeding trough!! Why? Because He loved us that much. “In all our trials he was born to be our friend. He knows our needs, our weakness is no stranger [to him].” Behold our King! This song strikes the deep chords of our hearts. Macayla’s disease, cancer, violence, our sin and the evil of this world are undeniable, but the God of the universe loves us and so much so that he lowered himself to be born in the weakness of skin and bone, to be subjected to this evil world, to be tempted in every way but free of sin. He values us. He wants to free us and the creation around us. “In his name all oppression shall cease.” We know what we and the world are like. We know what we and the world SHOULD be like. Jesus gives us faith and grace that guides us from where we are to where we should be. This faith and grace empowers us along that journey. This faith and grace transforms us from who we are to who Jesus created us to be. Indeed, let all with in us praise His holy name because that Holy Night led to Easter Sunday!

Just A Little ER Visit

Macayla was crawling in the kitchen and had a seizure and fell forward. She does this quite often and it is hard to catch her when she falls this way. Normally she just bumps her face or at worst bust a lip open, but this time, she split her chin open. She needed stitches so we took a little trip to the ER. They opted for gluing the cut closed instead of stitches. Macayla was acting as if nothing had happened and she pretty much stopped crying as soon as I bandaged her up. She didn’t cry at the ER and was content just about the whole time. This little diversion put us off schedule for giving her meds so she didn’t get her sleep meds at her normal time. It was after 1:00 am before she fell asleep. But she did well through the whole ordeal and we are thankful that this hasn’t happened sooner or more often. It is hard to balance her needs at times. She needs to stay as mobile as she can for as long as she can so we encourage her to crawl and walk with assistance. But we have to keep her safe and that is not always easy while she is mobile.

Dodge-Devouring Dachshunds

The more I ponder this, the more I am amazed at the fact that a 9 pound dachshund can totally disable a 2,000 lbs Dodge van. That is what is so annoying. My weakness is that when little things go wrong, I become a lot less Christ-like. I seem to handle major catastrophes better than little problems. It seemed that everything went downhill after the wiener attack. Because we had to drive to get another car late in the evening in the rain, that put the kids and us going to bed much later than normal. To add insult to injury, all of the excitement helped us forget to give Macayla her meds until we got back. This meant she fell asleep much later and then slept much later the next morning. This made us late, but it looked like we were going to make it out the door on time anyway. That was until Macayla had a bad diaper. So, that put us behind and we didn’t have time to make it to the lab to draw blood before going to the eye doctor. So, I got her to the eye doctor and gave her medication and some breakfast. In the meantime, the van was not repaired until yesterday because I have no way to safely secure Macayla while I worked on it until Jenny got home, but by then it was too dark outside to see. All of this along with a laundry list of other things exposed my less desirable character traits. I find myself relating to the van. Some of the smallest things can bring me to a halt. Mole-hills become mountains. Dachshunds become devastating. This is something I pray for God’s grace to overcome. But it does make me feel even more foolish to realize how much this situation has exposed this weakness. At the same time I guess I should be thankful that the Spirit is showing it to me. Maybe there is an opportunity for Him to show His strength in my weakness. In the meantime, I believe that the “Truth in Advertising” statutes of this country should require Dodge to come up with a more truthful sales campaign.

Eye Doctor

One of the major symptoms of Battens is blindness. Macayla went to the eye doctor and her eyes show little change. There is some pigment change in the retina which would correspond to any loss of peripheral vision. The doctor said that it was still so slight of a change that he probably wouldn’t be concerned about it or mention it accept that he knows Macayla’s condition and history and that is the only thing that makes it noteworthy. We had noticed that Macayla would respond less to visual stimulus in the peripheral of her field of vision. She also seems to have lost some depth perception and has a hard time finding people or objects at a distance. Her optic nerves appear to be in good shape. Basically, the doctor said that structurally the eye is good, but it is difficult to tell objectively how good Macayla’s eye sight really is since she cannot communicate what she observes. There is no way to know for sure how the brain atrophy is affecting her visual cortex. So, we just have to watch and be aware that she will obviously respond better to images that are directly in her focus. We are thankful that her vision is doing as well as it is.

When It Rains Cats and Dogs, It Pours!

The rain pelted the window panes by the kitchen table where Jacob sculpted ducks, rabbits, and biscuits from play dough. Macayla was sleepy in her chair and was only half interested in the bright pink dough. I heard the rain begin to slack up a bit and as it did the sound of drops against the windows was replaced with barking and yapping. This is not an unusual sound in our neighborhood because there is a rogue pack of long-haired dachshunds that will wander onto our street from time to time. They bark. They poop in our driveway. They are annoying. I was at home with the kids by myself, so I was too busy to look outside to see what the little pack of Oscar Meyers was up too. But after about ten minutes of the constant barking, I looked outside. I see tails and short hind legs protruding from under our van near the front driver side. I ran outside and the short-legged demons of annoyance scattered. I went to the front of the van to discover they had chewed through a conduit of wires. Black, corrugated conduit and bits of wire were lying on the concrete. I pull out my keys and try to start the van. It turns over, but it won’t run! They had severed the power to the fuel pump and gauge. AAARRRGGG! In a fit of anger I so wanted to have a target practice session. I could even have the little critters stuffed and mounted in my den! But then it dawned on me, “Why were they so interested in the van?” They had been chasing something that ran up into the engine compartment and I had just turned the engine over several times! I cautiously raised the hood to find a huge, fluffy cat was sitting on top of the engine block. He hissed at me and ran off with the demonic wieners in pursuit. Lucky for him (and me) the engine belts and fan didn’t chop him to bits. Enough was enough. I called animal control for the first time in my life. ALL of the officers were in hazmat training. Hazmat training! I knew from my time in law enforcement that animal control usually only works from 8 to 5. (Since animals never cause any problems at night.) So, needless to say, the little van vandals got away. The van was dead and the rain began falling again. I didn’t let up for hours. When Jenny got home I went out into the rain to try and fix it, but it was dark and even with a flashlight, I couldn’t tell a black wire from a green wire. We had to play musical vehicles with her parents so I could get Macayla to a doctor’s appointment the next morning. But mark my words, this is not over. The animal control dispatcher reassured me that I have the legal right to protect myself and my property by any means necessary! Oh, I will miss dispatcher, I will! The bb gun is pumped up and ready!

Toxic Green

Macayla has wheels! She now has a wheelchair and she seems to really enjoy it. It is very supportive and makes her feel very secure. Jacob had to try it out of course, but Macayla did not care for him being in her chair. It is mostly black, but the frame is “toxic green.” It is a bright light green color and her name is embroidered into the seat in green as well. We are very glad that she finds comfort and security in the chair. Due to the color, I doubt we will have a problem confusing her chair with anyone else’s.
A little over a week ago, Macayla participated in my sister’s wedding. She was part of the wedding party and with Jennifer’s assistance, Macayla was actually able to walk down the aisle. I helped to conduct the ceremony, so I was at the front with the pastor as she walked down the aisle. I suddenly realized that, short of a miracle, this would be the only time I would see my daughter walk down the aisle. She did great but it was much harder on us than Jenny and I thought it would be. She and Jacob were adorable and their cousin, Rebecca, helped to bring comic relief for us all.

Reflections in Cardboard

Macayla is continuing to do well with school and she does more for her teachers than she will for us at home. She eats foods for them she won't for us. She interacts in ways there that she won't at home. She is being like most kids, she puts on a show for others but not for us. That is comforting. She's a five year old after all!
Macayla's condition sunk in the other day, as it sometimes does. These are times when the reality of her condition and prognosis comes to the surface fresh and full of pain. I started thinking about how Macayla would go to heaven before we will (barring any unexpected departure on our part!) But the thought occurred to me, "Has Macayla seen enough of Jesus reflected in me to recognize Him when she sees Him?" I know that Scripture says that one day every knee will bow and every tongue will confess that Jesus Christ is Lord. So, everyone will "know" Him when they see Him. But as one preacher said, some of those knees will be scraped as they are left no choice but to finally admit that Jesus is Lord. But at the heart of my question was that when Macayla gets to heaven will she see Jesus and say, "Oh, hi Lord, it's you. I saw your reflection in my family. How great it is to see you fully now."
But then I thought about the fact that all of us are "terminal." We will all die at some point regardless if it is at 12 or 92. Will my son recognize Jesus when he sees him because of what he saw in me? Will my wife? Will anyone who meets me? I'm ashamed to say that I'm a poor mirror. I feel about as reflective as cardboard most days. But it is the Refiner's fire that gets the impurities out of the precious metals so that they reflect the Refiner's image more clearly. I hope that I will surrender to that purifying work. I pray Jesus' reflection will shine a little more each day.

EEG Update

Macayla's EEG results are in and she shows no real changes in her seizures. The doctor said she had some myoclonis, but not enough to disrupt her sleep. She still has a large number of seizures each day. So, nothing came of it that would shed any definitive light on Macayla's twitches other than they appear to be non-epileptic. But, she is a moving target anyway and changes daily. Hopefully, we can keep her eating as well as she has been for as long as possible and we also hope she will have more nights of sleep than nights of restlessness. We tend to have one or two nights each week that keep us up till 3 a.m. because she can't seem to fall asleep (even with a sleep med and melatonin on board). So, along for the ride we go.

Side Effects Include...

I, like many others, laugh at the commercials for prescription drugs that promise to help cure or control so many problems we Americans have. Got toe fungus? Take this pill once a day and the little fungus gremlins will be driven out. Of course you have to be on guard for the side effects that might make you hair fall out and your colon collapse, but hey your toes will look great! The anti-depressants marketed on the tube will make you feel better but with all of the diarrhea, vomiting, sexual side effects, and headaches you will probably feel so lousy that you'll get depressed again. We certainly like to take a pill for every thing that ills us. Macayla certainly takes plenty. We take meds for seizures and other meds to combat the side effects of the seizure meds.
I think that as Americans, we are out of touch with pain. (I highly recommend Phillip Yancey's Where is God When it Hurts on this subject.) We want a quick fix for our problems. But a century ago, quick fixes for discomfort were largely unknown. They didn't have medicine cabinets full of pills to treat every symptom they may face in a given day. People were more in tune with trials and suffering. I think of our situation and think of how bad it is to have a dying child, but our circumstances certainly could be worse and there are people who experience worse. This is not to minimize what is happening to our daughter, but it reminds us that there is plenty to be thankful for. How great it is, for instance, that we have choices over what to eat at each meal, whereas some aren't sure if they will have a meal today.
Someone once wrote a letter to some Jews who had converted to Christianity in order to encourage them. They needed encouragement because their conversion had brought on persecution from the society they had left behind. This writer referred these Jews to their own history and the faithful people of God that had come before them. Near the beginning of the letter (Hebrews), the writer encourages them to know their own Scripture and the Gospel message of Christianity more clearly so that they will not drift away from it. Later, the writer refers to these historic figures in Jewish history: judges, prophets, warriors, etc. He lists some mighty things they did such as conquer kingdoms, shut the mouths of lions, became mighty in war, quenched the power of fire, escaped the sword, and put foreign armies to flight. They even raised the dead back to life. And they did it all through faith. That is exciting. This is the kind of Scripture that TV evangelists love to quote today. It reinforces the kind of power-and-wealth Christianity they adhere to. They say that God just wants to bless us and we simply recite positive mantras and smile our way to health and wealth. But the writer of Hebrews goes on to say that these same prophets of old also experience other things through faith. They were tortured, scourged, imprisoned, stoned, SAWED IN TWO, tempted, put to death by the sword (not escaping it), they were destitute, ill-treated, and lived in holes in the ground. And they did it all through faith! I wonder what positive things I could think of to say while I'm being sawed in two? I don't think I could smile much in the midst of such an event. In all these events, the writer declares that these prophets of old were approved in their faith but that they did not receive what was promised. What was promised was the redemption Christ would accomplish at the cross and the resurrection. These prophets of old did not get to witness this. But it seems that in a fallen world where suffering exists, God works in the triumphs and suffering to perfect our faith. Just as God created the world through a step-by-step process, God re-creates us in a step-by-step process. He uses triumphs to change us, but in a fallen world suffering is bound to come. Not all of the promises of Scripture are necessarily obtained completely during our lifetime. Pain, suffering and sin will still be experienced. Thank our Creator that He doesn't waste that pain. He uses it to transform us as well. But this doesn't mean that we will be forever confined to the pendulum swinging back and forth between suffering and triumph. This ancient writer calls us to fix our eyes on Jesus, the author and perfecter of our faith, so that we can endure because of what He did on the cross. He provided the ultimate triumph over sin, death, and suffering there. That is the place we go to every day of this process of re-creation to deal with both the pain and the triumphs. We can endure because we know that we partly experience the promises of the cross and resurrection now and that we will fully obtain and experience those promises later, in heaven. I cannot smile away Macayla's suffering. I can't say enough positive mantras to stop her seizures. But I can fix my eyes on Christ to see His ultimate triumph. I can cling to the hope that is only found through a blood-soaked tree and an empty tomb.
The prescription we need to take is Stauros (from the Greek word for cross). Taking up our Stauros daily will help create perspective, remove sin, produce love and transform our character. WARNING: Side effects may include trials, denial of self, and dying to old ways to rise to new life. Some people (including family) may think you are weird. Talk to the Great Physician to learn if Stauros is right for you.

Me and my big blog!

Well, after "bragging" about Macayla sleeping all night each night for a week, last night's bedtime did not come until 3:30 this morning. We couldn't get her to fall asleep for nothing! On top of that, she woke up at 7:00 a.m. I just don't get it. She seemed to be suffering a lot of dementia through the night and it seems that it kept her up. I just pray that she will sleep tonight and not be bombarded by the dementia again. So, needless to say, the pendelum swung the other way last night. Never a dull moment.
On more important news, Ella (Macayla's cousin), seems to have come through her surgery well and is recovering. We just continue to pray for healing and recovery and that her mother will also get some relief from a kidney stone that acted up in the midst of all this. Their family has certainly had their share of long nights this week.

On Our Toes!

Macayla and Jacob have a cousin named Ella who is one year old and was just diagnosed with pneumonia last week. Unfortunately, the infection was not controlled by the antibiotics and she ended up with multiple pleural effusions (pockets of fluid/puss). She will have surgery tomorrow and we are praying for her and her mom and dad. We ask everyone who reads this to pray as well.
Macayla has been fighting nausea and vomiting for days now. We have been on the verge of taking her to the ER twice because she couldn't keep food or fluids down. But each time we were ready to opt for an IV and a bag of fluid, Macayla would get some water down and keep it down. So, we avoided the hospital. Another interesting change in all this is that after weeks of not sleeping much at night, Macayla has slept every night and even taken naps for a week now. We are really not able to keep up with her! Maybe we can blame it on the fact that she is female and will change her mind and methods quite often. The doctor had us reduce her cystagon dose by half because vomiting is a side effect of that medication. We did this yesterday and then gave her formula through her feeding tube slowly (5 oz. over a two hour period). This was an idea we got after talking to Macayla's grandfather who has experience with tube feeding. After that, Macayla was like a new child. She started playing and talking and that was a relief after two days of being weak and dehydrated. This morning she ate by mouth like a horse and hasn't stopped all day! We really have trouble keeping up with this girl. She doesn't sleep, she sleeps. She vomits, she doesn't vomit. She eats, she doesn't eat, she eats. She certainly keeps us on our toes!

It Figures!

After a few weeks of difficulty sleeping because of seizure-like jerking at night, Macayla has slept too well at the hospital. Her 24 hour EEG was intended to capture these spells at night, but she barely twitched a muscle. So, they decided to keep her another night to see if we could catch it. Finally, she did her thing during her nap just before discharge. We aren't sure if what she did was seizure activity or not and we won't know for a week or so, but we hope they got enough of something to determine it one way or another. She still has the seizures we are used to seeing, but just not as many outwardly. So now we wait.
She has vomited every weekend since our G-tube was placed. She threw up at home Sunday night, and then again at the hospital during the EEG. We are not sure if this pattern is due to reflux brought on by the tube or a side effect of the medication or just a change in her brain or digestion. It is mysterious. We are investigating this next week with a nutritionist and pediatrician.

Outside the City Revisited

As mentioned in an earlier post, I certainly can relate to Jonah sitting outside the city wondering what God is up to. Jonah's response was anger as is mine some days. Anger is a natural response to suffering or grief or disappointment. Anger occurs when we realize that we are not as in control of life as much as we thought. Suffering often comes through unexpected circumstances. Those unexpected circumstances throws our lack of control in our face and reveals the illusions that we have about what is really in our control and what is not.Through multiple sources (sermons, Bible study, articles, people, prayer, etc.), I have been challenged to look at suffering in some new ways. The Bible is full of Scriptures that call for us to endure. This is the same Bible that says Jesus came to bring life and bring it to abundance. How in the world does this fit together? I wonder if the abundant life is discovered in the midst of endurance. I wonder if the abundant life is discovered not only in victory, but in despair, in the kind of despair that leaves us no choice but to turn to Christ. Scripture also says that Jesus’ work on the cross is complete and that this finished work will restore us from not only sin, but suffering as well. Where and when is that promise obtained in our lives? When does the work of the cross remove death from our existence? I’m not sure I can answer that completely, but Jesus knew that his suffering on the cross would lead to Easter Sunday. He knew there was an Easter Sunday coming, but Good Friday still hurt like Hell (literally). So it would seem that the only way to be completely healed of suffering and sin is after death. Isn’t that what Christ said? We must take up our own cross daily, deny ourselves, to die to our old way and be raised to new life. Physical death frees us from the physical suffering of this world. Jesus also meant that we must die each day spiritually to free us from the sin of our lives. I believe this is not just about salvation because we “daily” take up our instrument of death, the cross, to be healed. I pray that we find the abundant life in the midst of suffering. I pray that we find our trials as opportunities to serve Christ. I pray that anger will give way to worship of a God who came from up there to down here to transform me.

Surfing the brain waves

We are going in next week for an EEG to determine if some of the changes in Macayla recently are seizure related. Every time she tries to sleep, she has multiple rhythmic jerks that flow through her body. It can sometimes take her an hour to two hours to fall asleep because of them. We think that the jerking is myoclonis and we want to see if it is indeed and if her seizures are changing form or are we dealing with something else. We all have myoclonis jerking when we fall asleep sometimes. It's that sudden twitch you may feel when you're drifting off to sleep but this is not epilepsy. Macayla, on the other hand, has multiple twitches for an hour or two and even some after she falls asleep. She has been waking up at 2:20 a.m. every morning and it takes until about 3-3:20 to get her back to sleep because of the twitching. The jerking also follows patterns sometimes. The other night she would do it in a sequence that started in her arms, then her legs, then her left shoulder, and then she would turn her head. She repeated this pattern about every five seconds for thirty minutes. The next night the pattern would be her arms then her legs at about the same interval. Jenny and I know that you don't want to be on the receiving end of one of those leg jerks! Macayla can kick!
Maybe her seizures have not been down like we thought, but that's what the EEG can tell us.

Prayer Answered with a "YES!"

We received our genetic results today and the best news is that Jacob is in the clear! We were confident from previous tests that he was not affected by the disease, but only a genetic test could confirm that 100%. Jacob only has one copy of the mutated gene (he would have to have both copies to be affected). He carries Jennifer's copy which is the more rare mutation and the copy I carry is the more common mutation. So, when and if Jacob marries, his future wife will need to test to see if she is a carrier or not. Otherwise, Jacob will not be affected by Battens disease and we praise our Lord for that! We prayed that Jacob would be free from the disease and God said "Yes."
So, we are celebrating this news today and we are excited that Jacob's condition is no longer in question.

School and Fun

Macayla has had some interesting changes over the past two weeks. She has had significantly less seizures that we could outwardly notice. We aren't sure why. It could be caused by better absorption of seizure meds through her feeding tube than what she got by mouth. It could be from the fact that we reached our full dose of the experimental drug she has been on for Battens. Or it could simply be how her brain is being affected at this moment in the disease. Whatever it is, we like it. We know that it could go back to the way it was or worse, but we'll take what we can get.
Macayla tried out school for the first time today. She actually paid attention for 20 minutes during circle time! That's an amazing event since during these past two weeks Macayla's attention span has been about two minutes. My hat goes off to her teachers because they keep the atmosphere so energetic and interactive that it's hard not to pay attention. It will take time for them to get used to Macayla and for Macayla to get used to the schedule, but it seems to be off to a great start. Macayla even made a new friend in the class, a little boy, who really anticipated Macayla's needs and wants. He even got her to toss a ball back to him which is difficult to get Macayla to do usually. I'm so excited for her as she gets to go back to school for the first time in a while. We will go as long as she is able and it is beneficial. We thank her teachers for all that they do.

Outside the City

Macayla is playing on the floor and looking through a book about Jonah. Jonah the prophet of God who was sent to the city of Nineveh to deliver God's message. At the end of 2004, we had come to a point where God began to open the doors for us to start back in seminary. Through prayer, Jenny and I came to the conclusion that it was time to set things in motion. We put our home on the market and began comparing the seminaries we had narrowed our search down to. To my surprise, God made it abundantly clear that we were to go to New Orleans for seminary. My biggest hang up with New Orleans was the crime rate. How could I protect my family in a city where the murder rate was ten times the national average? I feared that such a decision was putting my family in danger, but Jenny reminded me that I ultimately can only protect her and the children so much because, in short, I'm not God. So, in February we submitted to the call to New Orleans and set all of the paperwork in motion. In March our home sold and in April Macayla started having seizures.
After we ran the whole spectrum of tests that could account for seizures, Macayla was normal (accept for the falling and seizures of course). Our neurologist also shared that the cause for epilepsy cannot be found half the time and the only choice is to control the seizures. At that point, New Orleans actually appeared to be promising for Macayla. There was an epilepsy center there with the Oshners Children's Hospital that seemed to have much to offer for someone like Macayla. So we went. We submitted in obedience to what God had directed us to do. We pulled up stakes and moved into a third-floor apartment on the seminary campus.
But hurricane Katrina sent us back to South Carolina two weeks later. Like most people, we thought it would be over quickly, but the flooding made that impossible. We knew it would be August 2006 before we would get back down there. But then...Macayla. During our time in exile from NO we discovered Macayla's true condition, Battens. What a relief to finally know what was causing the seizures, but how horrible it is to know what is coming.
Jonah was a prophet who was told to go to a violent city he didn't want to go to. After some rebellion and prayer, he finally went and did what God commanded. But Jonah had expected God to act after the message was delivered. The message was, "Forty days and Nineveh will fall." It was NOT "Nineveh will fall unless you repent." No, Nineveh was supposed to fall. So Jonah delivered the message and sat outside the city to watch the fireworks. But the fireworks never came because Nineveh repented. God had not acted like Jonah expected. Jonah was angry, so angry he wanted to die. I did what God wanted. I went to a wicked city after rebellion and prayer, but God has not acted as I would have expected. (Of course, I wasn't going to deliver a message of judgment, but to go and learn to share a message of hope.) So here I am. Outside the city wondering what God is up to. But I spoke to a man who recently lost his little infant girl suddenly. She died in her crib. Listening to him and his story made me realize that I stay too focused on what I think God should be doing. Instead, maybe I should be focused on what God is ACTUALLY doing.

She Can't Taste A Thing!!

Many people, including myself, associate feeding tubes with hospice situations and as a marker that the end of life is near. Macayla had a feeding tube (G-Tube) placed this week. I was certainly nervous about this as it seemed like a step toward giving up or giving into the disease. We certainly feel a desire to fight the disease and never give up hope and accepting a G-Tube seems like giving in. But this is not an accurate perspective in a case like Macayla. Macayla has to take such awful tasting medicines that I am amazed that she has taken them for a year now without rebelling sooner. Mac's tube will change her life dramatically. We can spend up to three hours trying to get her meds down some days and we usually can't get all of it in her when that happens. There have been some days when we can get them done in ten minutes, but those days are rare now. So, Macayla's surgery was necessary and a blessing. It is in fact a way to improve the quality of life she experiences now.
The surgery went very well and she is recovering well. She did throw up once during her stay in the hospital. But other than that, she did great. She is eating again, though she is suspicious of the food expecting it to be laced with medication. But she eats well when she can and when her swallowing is good. When it is not, we supplement her with tube feeding. We love that she is still eating, but that is temporary and eventually she will be totally tube fed. The most amazing thing we have already seen is that it takes literally 20 seconds to get 8 medications in her. That leaves 2 hours, 59 minutes and 40 seconds to do other things like play and eat food that is not tainted by the awful taste of medications.
We see over and over how God has mobilized and moved people to reach out to Macayla and us during these times. People in the hospital, multiple churches, family and friends have all been used by our Father to comfort and encourage. We thank you all. We even got to celebrate our anniversary in the hospital by the generous and thoughtful acts of several folks. One friend from church brought us Steak Out complete with cheesecake! Another friend at the hospital dropped off a DVD romantic comedy, popcorn, M&M's and a nice card to go on the wall to announce our 8 years of marriage. We also got a vase of flowers for the room and balloons. We ate our meal, cuddled on the "wonderfully comfortable" cot, and watched a movie. What a wonderful experience in the midst of a hospital stay! Thanks to all! Macayla is home now and we still celebrate every time we give medicines because she didn't taste a thing!

A Little Protein Changes The World

We reviewed Macayla's genetic results today with the doctor and it confirms how complicated this disease really is and how difficult it is for the medical community to really define and classify it. Macayla does not produce enough of a protein in the lysosomes of her neurons (brain cells) and this means that the lysosomes can't get rid of certain waste products from the cells so the cells become damaged. Thus, her brain is deteriorating. I began to think about the actual size of the lysosomes in her cells. Basically, these lysosomes are microscopic trash cans in Macayla's cells and are unable to do their job and it has devastating results. These microscopic trash cans don't work because an even smaller part of her body, a gene, is mutated at one point. I don't know enough about DNA and genetics to do it justice, but my understanding is that there is a mutated part of a DNA molecule of a gene on Chromosome 1 inside the nucleus of the cells of Macayla's body. We're talking about something that is tiny, as in molecular, and it causes all of this! It is amazing to ponder how we are fearfully and wonderfully made.
In order for Macayla to have the disease, she had to get two copies of the mutated gene, one from me and one from Jennifer. One of her copies has a type of mutation that is more common among Batten's patients. The other copy she has is more rare. The good thing is that the more rare mutation is associated with some enzyme activity. This simply means that she may be producing some protein that helps the trash cans do some of their job, but it obviously is not enough to do the whole job. We also learned that there is no real clear-cut way to predict her prognosis. Other children with similar combinations of mutations had varying ages and symptom onsets. The other trick to this according to the doctor is that we are not really sure how other proteins being produced in the body may modify the progression of the disease. Each Batten's kid is really unique in their progression and prognosis. We will draw blood on Jacob and Jennifer and I to have it nailed down as to which mutation I have and which mutation Jenny has and see if Jacob has either.
We have been taking Cystagon for a week now and we are tapering her dose up over the next few weeks. She is taking it quite well, but it is powder that smells like old, boiled eggs. Old because there seems to be a hint of sulfer in the aeroma. YMMMMMM! Luckily, the taste and smell seem to disolve well in certain types of food such as applesauce and tomatoe sauces and this helps since she has to take it four times a day. We thank God for the strength he has put in Macayla to deal with all of this.

Medical Update July 14th

Macayla's genetic workup is back. We will go in next week to get more details, but the gist is that she has both copies of the CLN 1 gene from Jenny and I but each copy is mutated in different places. This means that Jenny and I carry two different mutations of the same gene. This helps us in isolating Jacob's genetics to see if he carries either copy or none or, God forbid, both. The genetics are very complicated and we hope to have more info next Thurs. We will also be meeting with the surgeon at the end of the month about having a feeding tube inserted. Mac's swallowing is hit and miss more often and the meds she takes often smell and taste awful. When swallowing is tuff already, it doesn't help to throw in something that tastes so bad it could make you gag all by itself. She will continue to eat even with the tube, but it will be mainly to ease her daily life on taking meds and will be a back up if she has a hard day swallowing food. It will be a full-time necessity eventually. Considering all, she has been the best patient and has really been tough.

Medical Update 6/30/06

We were able to review Mac's MRI today and thankfully the rate of atrophy has decreased compared to the previous 6 months. There is a little more atrophy in her cerebellum (the part located at the base of the brain where the brain stem comes in) but there is only "minimal" changes in the rest of the brain. This means that it has slowed down some and we praise God for that.
We should be starting on cystagon next week which is an experimental drug that has helped some children with Battens, but not cured anything. It has to be administered four times a day but we aren't sure exactly how to get it in her yet. We will also start vitamin E, B2, and B6 with this. The cystagon study as well as other studies have shown this to be helpful, though they aren't really sure why yet. The way we understand it, the cystagon helps slow down the build up of the deposits in the cells. These deposits build up until they destroy the cell and this leads to the atrophy of brain tissue. Hopefully, the cystagon will slow Macayla's down which would be great since it is slowing down some now. This is encouraging overall.
Macayla has been measured for her wheelchair and now we wait for all of the insurance paperwork to go through. The chair will be a big help and we look forward to her getting it and being more comfortable. She had her blood work sent off for molecular testing so they can map her genes and isolate the mutated genes that are causing her Battens. We'll use this info to test Jacob to see if he is a carrier or not. That's the latest.

Love is patient...

God has kept two sections of Scripture biding for my time lately. These two pericopes have been orbiting my heart and mind as of late and they swirl slowly past so that I have time to look at each one separately as well as together. I have been trying to see how they compliment each other and why. The first passage is Philippians 4:4-7 and the second is 1 Corinthians 13. In Phil 4, Paul tells the Philippians to "rejoice in the Lord always, again I say rejoice." In this life, how do we rejoice in the Lord? How do we rejoice in the Lord that allows terrorism and Battens disease? How can I be "anxious for nothing" as this text later says? It says in everything by prayer and supplications with thanksgiving let your request be made known to God and the peace that surpasses all understanding will guard your heart and mind in Christ Jesus. Well, I've prayed. I've prayed and made requests. I've prayed with thanksgiving. But I'm still anxious. I still worry. Macayla is still sick. Why? I mean I did exactly what the text said to do many times over and I still have worry and not much peace. Maybe I don't have enough faith. Or maybe this is not a formula for ridding the world of anxiousness. Maybe I shouldn't take Philippians 4 for anxiousness like I take aspirin for a headache.

I was making a request known to God the other day. I prayed for patience with Macayla as we fought to get her meds down. Parents often pray for patience. I do all the time. But no patience seemed to ever come my way. Then it happened. I was driving, Macayla was yelling in anger at me over something I couldn't figure out and Jacob was making his requests and supplications known to ME about staying home for the day, getting his apple juice, and watching Batman all at once. My nerves were in the cheese grater and all I could do was pray for patience. The answer: "love is patient. love is kind." 1 Corinthians 13 was spoken to me. I then realized I needed to stop praying for a byproduct and start praying for the source. Love is the source of patience. The source of kindness. Love is not jealous, it doesn't boast, it is not arrogant. Love does not keep account of wrongs suffered. Love is, in other words, the source of contentment. Love bears all things. This word in the Greek for "bears" carries the image of thatching a roof to cover and shelter from the elements. Love shelters. Love is the source of all this and God is the source of Love. God is Love.

The word rejoice in Phil 4 is translated from a Greek word that is a root for the word "grace." Love was freely given by God to us on the cross and resurrection. It is this grace that makes it possible for me to rejoice and to love. 1 Corinthians 13:13 is engraved inside my wedding band. It isn't there as some quaint, poetic reminder that I "love" my wife. It is a reminder inside a never ending circle of the never ending love of God. A reminder of his eternal faithfulness. This is so I can go to the source. God is the only source I can go to that can supply me with the kind of love my wife needs. The kind of love Macayla and Jacob need. To supply me with patience, kindness, contentment, forgiveness, and the ability to rejoice in Him always. It is through this grace that I know the Lord is near guarding my heart and mind from worry. I pray that I continue to see the source more and more fully. I pray for his love.

Then I heard our pastor reference Romans 8:35-39 in the sermon and this gave such great assurance about Christ's love. "Who will separate us from the love of Christ? Will tribulation, or distress, or persecution, or famine, or nakedness, or peril or sword?...But in all these things we overwhelmingly conquer through him who loved us." Paul writes that he is convinced that there is nothing (death, life, angels, demons, present, future, powers, height, depth, or any created thing) can separate us from the love of Christ. This love, this source is present in my life only because of Christ and only because he is so faithful to pour it out in grace.

Jesus Wept

In 2005 we discovered that Macayla was having seizures. Throughout most of the year we were hopeful that we would discover the cause of the seizures but we were confident we could gain control of them with the right combination of medications. A few days before Thanksgiving, I heard a sermon on John 11 where Jesus raised Lazarus from the dead. We always focus on the raising from the dead part. We know the outcome of the story so it is easy to skip over some important elements of it. As I listened to this sermon, I was reminded that Jesus waited before coming to Lazarus and Mary and Martha's aid. He could have stopped Lazarus from dying, but he didn't. He didn't even have to go to Lazarus' home to do it. He could have simply willed Lazarus to be healed and it would have been so. But he stayed at a distance and allowed the sickness in Lazarus' body to take its course. I was reminded that Jesus was in control of the situation. When he did finally show up on the scene, Mary and Martha both acknowledge that Jesus had the power to prevent their brother's death but that he didn't. I knew as I listened to this sermon that Macayla was dying. I knew God was preparing me for the worst news a parent will ever hear. This was at Thanksgiving and a MRI in December confirmed that we were in the realm of a fatal disease. It would be the week after Christmas that we would confirm the name of the disease, Battens.
But John 11:35 is the shortest verse in the Bible, "Jesus wept." Jesus comes to the tomb of Lazarus who was 4 days dead at this point and Jesus wept. He didn't weep because he morned the loss of a friend. He knew that he was going to raise Lazarus from the dead before he ever got news of the illness. He wept at all of the grief that death causes in our lives. First of all, death was not included in the original design of our world. Our sin brought that about. Second, death doesn't just kill someone, it grieves those who loved the deceased. Jesus wept because it didn't have to be this way. He wept over the grief of those who loved Lazarus like he did.
I weep for Macayla often and Jesus weeps with me. Macayla is a victim of a fallen world where disease and death exists. But that's not what was intended and that's why Jesus came. He came to restore what was intended in his creation. He came to bring us back into fellowship with him. God is not a puppetmaster in the sky just toying with us. He loves us. He loves us so much that he weeps with us and I bet his tears are bigger than ours. He loves us so much that he crossed the gap between him and us. He brought "up there" down here.
Jesus could heal Macayla and totally restore her brain and body. But he is the resurrection and the life and He can take care of Macayla better than I. Even if she is not healed and dies (which only a miracle could stop) Jesus will take care of my and his little girl. Jesus not only wept but he later suffered and died because of our fallen world. His resurrection, unlike Lazarus', overcame death and sin. It provides life, real life for those who trust Him like a child.