Two Years

It was two years ago today that we received notification of Macayla’s diagnosis. It was a colder and cloudy day. Today is more pleasant. But we had to take Macayla in to have her tube switched out. We finally got a Mic-Key button put in. Mic-Key feeding tubes are held in place by a balloon inside the stomach that is filled with water. It has a locking mechanism that the feeding tubes lock onto. The advantage to these tubes is that we can change them out at home. We will simply deflate the balloon and the tube pulls out and then we can insert a new one and inflate it to keep it in place. It has to be changed more often than other tubes, but it is less traumatic to her site and doesn’t require a drive to the doctor’s office. So Macayla got a new tube for Christmas. Our nurse quipped that everyone wants Mickey for Christmas, but this was not what Macayla had in mind! The tube change went great and Macayla only bled a minimal amount. Her site is healing well. We went down afterward and had a contrast study on x-ray that showed that the new tube is flowing correctly.

Much has changed with Macayla in two years. She was eating, talking, and walking two years ago. She ate cheese constantly. She loved filling up her little purses with all the toys she could fit into them. They would overflow with stuff. She takes after her mom! She liked playing with any animal. She now is immobile. She doesn’t eat or drink or talk. She is much taller and thinner than she was two years ago. She still has a great smile, a quirky sense of humor, a great memory and still loves movies. It has been a long two years in some respects, but it has been a blur. We stay so focused at times that suddenly we look up and discover that two years has passed without our full realization. Where will we be two years from now? 

Jesus' Address

Christmas has all of its cultural baggage and even controversy these days. The season (more accurately the marketing) begins at Halloween. The celebration is often warped by materialism as throngs of people step on each other to buy presents. Stores are afraid to use the term “Christmas” because it might offend, so they use the term “holiday” even though it comes from the words “holy day.” This season’s purpose has indeed become muddled. It is difficult to not get sucked into the vortex of controversy, wrapping paper, money, and stress over the people that are so hard to buy for.
As I watch Jacob get more excited about Christmas this year than any other so far, I wish I could recapture the wonder and excitement of childhood. I wish I could recapture that feeling, that “Christmas spirit” that seems so fleeting in adulthood. But Jacob’s questions and statements about Santa Clause made me realize where the true wonder lies in Christmas. Jacob said, “You have to be good if you want Santa to bring you any presents.” This concept is expressed countless times at Christmas in songs and among parents to their children. This concept departs radically from reality. The wonder of Christmas lies in the fact that the God of the universe didn’t go down a list and weigh out if we had been good enough to deserve being saved from a fallen world. The fact is, compared to a perfect and holy God, just one of our sins puts us on the “naughty” list forever. The wonder of Christmas is that the God of the universe who created us loves us so much that he came to us. He came to us in the flesh, a man, in surprisingly low economic and social status among the people 2000 years ago. The psalmist echoed this wonder when he wrote, “What is man that You (God) are mindful of him?”
Like us, the first disciples were caught up in the physical of the incarnation. The Gospel of John starts off with all of these lofty declarations of Jesus being eternal, from before time and the creator of all things. Jesus is called the Lamb of God and the Son of God. But when the first disciples meet Jesus their first question is “Where do you live?” Ravi Zacharias points out in Jesus Among Other Gods that we certainly could think of better questions to ask than the address of a man declared to be the savior of the world. The reader and the disciples get the same challenge from Jesus, “Come and see.” When one of the disciples learned that Jesus was from Nazareth, his response was “Can anything good come out of Nazareth?” Like us they were so focused on the limited portion of reality called the physical. Jesus’ address was not merely Nazareth. Jesus of Nazareth was also God the Son of heaven. Jesus’ address is at the heart of what Christmas is all about. It carries us through the materialism and controversy of Christmas. It shows us what is important to the God who is mindful of us and loves us.
When we and the disciples want to know where Jesus is from, he challenges us to “Come and see.” Ultimately, Jesus was from beyond Nazareth. Jesus was born of a virgin, conception without consummation. His origin is heaven and that does indeed tell us his worth. “Can any good thing come out of heaven?” Come and see the baby, wrapped in swaddling and lying in a manger. Come and see that life is not a material pursuit or about our earthly address. Ravi Zacharias wrote, “For the disciples, Jesus’ answer to their simple question – “Where do you live?” – was to lift them beyond race and culture, beyond wealth and power, beyond time and distance to make them true citizens of the world, informed by the world to come…He showed them the inclusiveness of His love for the whole world. But in that was the exclusivity of His truth, for which they were willing to give their lives. We have reversed Jesus’ order. We have made truth relative and culture supreme and have been left with a world in which wickedness reigns.” I pray Jesus will reign with all his wonder in our hearts this Christmas.

The Tube Plan

Macayla went back to see the surgeon again today. Her incision where the tube was placed had looked better this past Sunday and Jennifer and I were in the hospital Monday through Wednesday morning. When I saw Macayla’s incision Wednesday, I could tell it had opened up more since I had seen it last. It was draining like crazy and so I stepped up on using the special gauze they had given us (Aquacel) and changed it out multiple times a day. I called the surgeon about it and when we got into see him today, her wound had already improved. But he cauterized it with silver nitrate and we are hoping that it will look better by Monday when we will pick back up with the Aquacel. For those who are interested, we cut small pieces of the Aquacel and pack it into the incision right at the tube and it absorbs all the discharge to help keep the incision dry. If you are not a person interested in such things, sorry and I hope you aren’t reading this on your lunch break or something. In the meantime, we plan to go back in two weeks and trade out this Bard Button (that still leaks) with a Mic-Key. Our prayer is that the removal process won’t tear up her tube track and start the bleeding all over again. We pray that she will continue to do well with her feedings and get healed up soon. Overall, she is a great patient and handles all this much better than her daddy.

Love My Body

As Jennifer is recovering from surgery, I have been exposed to more than I ever wanted to know about the human body. I am so thankful that there are people like Jennifer and my sister-in-law who are willing to go into the medical field and take care of the rest of us. I get quite squeamish about much of this. But this morning, in the hospital room, God reminded me of something He said. He said that husbands are to love their wives as they love their own bodies. The implication is that we would naturally protect and care for our own wellbeing and since Jennifer and I are “one flesh” in marriage, caring for her is caring for me too. Lest we think God wants us to use a selfish motivation to care for our wives, He said in the sentence before this that husbands are to give themselves up for their wives as Christ gave himself up for the Church (and this isn’t the brick building on the corner, but the people who make up the Church around the globe). He gave his body over at the cross to bear the pain and separation from God that our sin has brought. He died a horrible and painful death in our place. I believe God reminded me of this because in the coming weeks, I must be prepared to once again give myself up to care for Jennifer. I’m not too good at this normally. Selfishness and my agendas tend to distract me from her care. I couldn’t take her place in the operating room (I am lacking the uterus necessary for a hysterectomy) but I wish I could take her pain away. I don’t understand all that she needs medically, but I pray that I don’t fail to provide what she needs holistically. I pray she recovers and that I learn more about what it means to “love my own body” by caring for her. I pray my care for her won’t fall away after she recovers. I pray this simple circumstance can be used to teach me more about the mystery and wonder of what Christ did for us at the cross. Check out Ephesians in the Bible. The whole letter is worth a read.

800 ml

Macayla seems to have turned a corner. Yesterday and today she has gotten all or almost all of the normal amount of formula she should get in a day. She is scheduled to get 800 ml of formula per day plus some free water. That may sound strange. We don’t charge her for the formula or the water, so don’t worry. We have struggled some days to get 200 ml in her after the surgery, but this weekend it got better and then yesterday and today she seemed to leap back into the groove. She will still have a GI study to see the functionality and structure of her stomach and GI but at least she’s back up to where she should be. Her home nurse, Hannah and her Grandfather Keith were taking care of her the last couple of days and they apparently have the magic touch of getting Macayla back up to speed. This was especially good since Jennifer and I have been tied up at the hospital as Jennifer recovers from her surgery. Jennifer’s surgery went well and she absolutely needed to do it according to what the surgeon found. But she’s doing well and the doctor seems pleased with her progress. We have certainly seen the sustaining power of Christ through the prayers and ministry of others. We are so blessed and thank you to all who are praying and helping. Thank you for being the hands and feet of Christ.

The Good Pus, The Bad Pus, & The Ugly Pus

Pus. According to Webster’s, pus is a yellow-white substance found in abscesses, sores, etc. consisting of liquid plasma in which white blood cells are suspended. Yuk. We took Macayla to the surgeon’s office yesterday to let them check her tube site and incision. In the last 48 hours it had not looked too good so we were concerned that it might be the beginning of an infection. The doctor said it was not an infection but that it certainly did not look as good as it should. We learned that there are two kinds of pus. Yes, there is normal, good pus (hard to imagine for those of us outside the medical profession) and then there’s pus from infection. Macayla had some pus coming out from around her site and it was the “good” kind. There is a fancy medical term for it, but it still looks like pus to me. It did not have a foul odor and her site was not warm. These are indicators of infection and, yes, “bad” pus. They gave us some special gauze-like material to pack around the tube site to help dry the incisions up. We packed the site and put regular gauze over it. All I know is that pus, good or bad, is ugly. On another note, Macayla may go back for a GI study to see how her stomach empties. Even before the surgery her stomach seemed to empty slowly and that makes it difficult to get all of the calories and volume of water she needs. It was worse after surgery so we started a medication called Reglan and so far it seems to help. Yesterday, I felt like I wasn’t keeping up with her stomach. She was empty pretty quickly between feedings. Hopefully this will solve the problem and we can skip the GI study, but if the medication is not successful we can do the study and determine if there are any structural or functional problems. Overall Macayla seems to be turning the corner as of yesterday, but I wish we could get the button on her feeding tube to quit leaking.

Two Old Men

Two old men had been friends since childhood. They had watch each other play baseball and graduate from school. They each had been there when the other got married. They each celebrated the birth of the other’s children. They each attended the funeral of the other’s spouse. For almost 75 years they had been friends and had shared so much. But even now, in their old age, there was one thing that one had not shared with the other. This one thing had not been uttered and the old man thought his friend never knew about it, but his friend did know about it. His friend knew that for years the other had been stealing from a local charity where he volunteered. Upon discovering this fact about his friend, an old man had to make a decision. He knew the stealing was wrong and wanted to say something to his friend, not to condemn or to hurt his friend, but to free his friend from a crime that had become a habit. But if he confronts his friend with the truth, will his friend go away or change his life? He must choose between staying silent and killing his conscience or saying something and possibly killing their friendship. Killing the friendship would break his heart and break the heart of his friend. But could he be a true friend if he did not share the truth? Truth that could help his friend gain freedom from a destructive pattern.
There was a time when Jesus performed a miracle and made a picnic for 5,000 people out of two loaves and five fish. This sensational event drew large crowds and they chased after him where ever he went. How many pastors and churches wish they had that effect on the general public? But Jesus turned to the crowds and said a truth that he knew many of them would not put up with. He knew that many in the crowd were there for the hype, sensation and the free lunch. But he wanted more for them. He wanted them to have the greater reality that the miracles were pointing them to. So, he shares with them that lunch only sustains them today, but he was the bread of life, bread that would sustain them forever. He said, “Unless you eat the flesh of the Son of Man and drink his blood, you have no life in yourselves.” (John 6) This is a strange way of saying it, but he knew that many in the crowd were thinking with their stomachs and not their minds and hearts. He knew that he could give them truth that would free them from destructive patterns in their lives, but they would have to think with their minds and hearts and not with their stomachs and emotions. He knew that some would walk away when he said it and I believe that broke his heart. He was not spouting off truth just to rub it in their faces. He was sharing truth that would transform their lives. He was speaking in metaphors, but they were too stuck in their physical realm to see the larger scale of reality. I believe his heart broke when so many walked away because he knew each and every person there better than they knew themselves. He knew them better than two old friends could ever know each other. But had he not shared the truth, no one would be changed. How could he say he loved anyone if he were not willing to share the truth that he knew would change their lives for the better? It was a truth that he not only shared with his words, but he shared it with his body. He shared it when he died on a cross for our sins and it broke his body, the bread, and spilt his blood, the drink. Whoever believes this truth will be changed forever. Whoever lives by this truth will not starve their soul any longer, but will have true life. This is the truth of love and not dogmatic traditions. This is the truth that brings freedom and not legalistic barriers. It is a truth that will cause division because in a fallen world where sin is present, people will either choose to separate from the truth or separate from sin. But His heart aches over those who separate from the truth.

Bard Button

We have not been thrilled with this feeding tube. It seems that if anything much thicker than cough syrup is pushed through it, the valve in the tube gets stuck open and it leaks. We have to include Diet Pepsi in the regimen of medicines each night just to clean the valve. I’ve tried to find other suggestions for how to use this thing, but it seems that most parents are reporting less than stellar performance for this particular feeding tube. We could dilute things with more water to thin them out, but she can’t handle much volume right now, so increasing the volume increase the opportunity for nausea and vomiting. That increases opportunities for aspiration and choking. So far the button has leaked both nights we have been home (of course it never did while we were in the hospital) and she has not tolerated food nearly as well as she did in the hospital. After 29 ml of formula yesterday afternoon her stomach suddenly became distended. She looked like she was 6 months pregnant. We decompressed her and got 85 ml of formula off of her. We were thankful that the decompression actually worked that time. She only got 200 ml yesterday out of her normal 800. We just pray she keeps hydrated enough and doesn’t have to go back into the hospital. We pray that this silly button starts to work properly.

Prayer: God Shows Up

Our time at the hospital was not fun and there were complications, but there was prayer. The previous entry can tell you about the complications, but this is to talk about the prayer. We had multiple people calling us to get updates to pray for us and pass on the prayer requests to others. We had at least four churches praying for Macayla and us and many individuals. This procedure was not major relative to many procedures we could face or that other children face very often. But that does not negate the need for prayer. I find that it is the more mundane difficulties and circumstances of life that wear me down. Give me a crisis and I will pray well and hold up well. I will more likely forget God in the midst of a flat tire or outpatient surgery. We had nurses praying for us. We had family and friends praying for us. We thank all of you for prayers, food, calls and support.
There were some nursing students on the floor while we were there and things worked out for Jennifer to give six or so students a mini-seminar on Battens disease, tube feeding, and patient care. Jennifer loves to educate. As the nursing students were leaving, one stayed behind and asked how she could pray for us. I was impressed a young lady her age had the courage to stay and pray with total strangers. Most college-age students would be too self conscious. She prayed for our family and our marriage. God showed up a lot over the last few days. Check out Jacob's page soon (December) to see how God showed up in his prayer time.

New Tube, No Fun

Macayla’s feeding tube was relocated on Wednesday. They removed the old tube and create a new hole a couple of centimeters over to put in a new tube. It was supposed to be a 45 minute process and we had decided to put in a tube called the “Mic-Key” since it is one we can change out at home. When the surgeon got in, he discovered that there wasn’t as much play in her stomach as we had hoped for. In order for these feeding tubes to work, they pull the stomach up against the abdomen wall. As it heals, the stomach basically adheres to the abdomen wall at that location. They had to make a bigger incision to free up the stomach a little more in order for it to stretch over better and pull up to the location of the new tube site. But this incision created a problem with the type of tube we had selected. The Mic-Key is held in place by a water-filled balloon inside and that was not going to be good next to this incision, so the doctor had to make a decision on the table and put in what is called a Bard Button. It has a more ridged flange inside. From the outside, it looks like the valve on a beach ball after it has been pushed down and is flush with the surface of the ball. It has a little flap with a stopper on it that folds over and closes it off and there is a valve inside the tube that is supposed to allow fluids to come in but not come out. We will use this for the next 4 weeks and then after she has healed we will change it out in the doctor’s office with a Mic-Key.
The surgery took an hour and 45 minutes and Macayla did well. But when she woke up from the anesthesia she threw up a bunch of blood that was left over from the surgery. She threw up bile twice more, once during a contrast study to make sure the feeding tube was working properly. It was a long day since we have to suction her every time she throws up to prevent her from suffocating. This bile and blood would have normally drained off of the stomach and into a trap that is connected to the feeding tube, but the trap was clean. Nothing was draining. We could push stuff in her feeding tube but we could not drain or decompress her. Decompression is important because it helps remove excess air and check for blood in her stomach contents. Without decompression, she would start throwing up again when we got home. We tried everything but it didn’t work and to the point we stayed an extra night in the hospital. We discovered that as we got more food and Pedialite in her stomach she decompressed better. The surgeon said the stomach needed to stretch back into position now and should work fine. Decompression has been more successful but inconsistent. But the button they had to put in her has been a pain to work with. The tube that attaches to it for feeds does not lock on so it easily comes out. We discovered this our first night home as I gave Macayla her meds. As I was pushing meds through the tube, the tube disconnected from the button on her tummy and spewed the medicines in her lap and chair instead of into her stomach. There was no chance of her shirt, pants or wheelchair having a seizure with all the meds they had on them. But we mixed up more meds gave her a dose and this time made sure the tube stayed in place. After we disconnected the tube, the button wouldn’t stop leaking. Stomach contents kept pouring out of it. The little valve inside was not working. We put the little stopper back in and to keep the stopper from coming out while Macayla slept, we put gauze and tape over it. Not ideal on a fresh surgical site. I hope things improve with this button or it will be a long 4 weeks.

Plain Fact

My four-year-old, Jacob, and I were talking about buying Jennifer a Christmas present and I asked him what he thought his mom would like. He first said that she likes food and we should get her some food. (He also suggested we get this for his eye doctor since "Dr. Tony helps us so much.")
“Mom does like food,” I agreed. “What other things does she like?”
“She likes me,” Jacob said.
I agreed again and said, “But you already are a gift to us.”
In a very matter-of-fact tone he said, “Yes, I am. God sent me and I’m a present and everyone thinks I’m cute.”
I laughed a little and he said, “What’s so funny?”
Jacob wasn’t being conceited when he stated that everyone thinks he’s cute. Many times when we are around other people, he often hears them say that he is so cute. From his standpoint, it is just the truth. It is a plain fact that he has learned because so many people around him have reinforced it. What we may mistake for conceit is simply Jacob making a plain and true statement. We may think that God is conceited when He states that He is worthy to be worshiped and praised. We may think it is conceit when God says He should be at the center of our lives. But the fact is that it is true and just like Jacob, God is not trying to talk it up to make Him feel better about Himself. He is simply sharing the facts and it is therefore not conceit or vanity. Maybe this is why Jesus tells us to become like a child (not childish) so that we can recognize truth and trust it. As Jacob’s parent, I can attest that he is pretty darn cute. As an adopted son of God and as a parent that watches God work through Jacob, I can attest that our Father in heaven is worthy to be worshiped and praised.

Groaning With Thanksgiving

What makes Thanksgiving more than just another chilly Thursday? There is a movie called “Bruce Almighty” where Jim Carey’s character is endowed with all of God’s power for a few days (and only for a few blocks in Buffalo, NY). The movie is irreverent in places, but so are we. By the end of the movie Bruce is transformed from irreverent to illuminate. But on the bonus features of the DVD there are deleted scenes and one of which I wish had not be deleted from the movie. Bruce gets to view suffering in the world from God’s perspective and in one case in particular the person comes out on the other side of suffering with a beautiful life. God tells Bruce that to paint a portrait that beautiful, you have to use dark colors sometimes.
In reading the creation account in Genesis, I realized that God created the night by creating light. He also created other things such as “sea monsters” as they are called in the ancient text. All that He created, even the darkness, was called “very good.” But sin entered the world and change us and creation. The Hebrew word for “sea monster” would be used in later texts to indicate evil and even Satan. Just as Satan was once an angel, the “sea monsters” that were originally supposed to worship God now represent rebellion. All of creation was affected including plant and animal life. This is not what was intended by the original, but it happened and here we are. We now have suffering and death. We have pain and strife. Humans were created to be individuals with unique personalities, but sin brought contention to those differences. Thus, we struggle with one another.
But then I turned to Romans 8 and discovered what Paul wrote. He does not compare our present sufferings to the glory that is to be revealed in us who trust Christ. In a few verses, he recaps how creation itself groans to be set free from its fallen state. Its freedom will come when all of God’s children are revealed. Like creation, we also groan for our redemption. We also discover that God, His Spirit, groans for us when we don’t know how to pray in this fallen world. Creation groans, we groan, and God groans with us for the glory that is to be revealed. Why is it this way? Hope. Since all of creation is connected, a major point of Genesis 1, when humans fell in sin we brought creation with us. God will now redeem us through Christ and with that He redeems creation. This hope is found in Christ and by its very nature hope is centered on something we can’t always see. Thank God that our hope is supported by the groans and prayers of the Spirit Himself.
It is hard to understand many times as we watch the suffering around us, as we watch death creep into our families and culture. But we are not groaning by ourselves or to ourselves. We have a God that will work all of these things out for the good of those who love Him and are called according to His purpose. If we trust Him, Paul tells us that we are already called, justified, and glorified. If we trust and surrender to Christ, then nothing can separate us from the love of Christ. No suffering, death, life, good or evil can separate us from Him. In all these things we actually become overwhelming conquerors. We may suffer and have setbacks. We may face even death. Life may be hard and our world may seem confusing. But none of that will separate us from the Love of Christ if we trust Him. That is what makes Thanksgiving more than another chilly Thursday. The early settlers of this country faced more struggles than we can fathom. But they celebrated and were thankful anyway. We can be like them and celebrate the creation that was once “very good” and hope in the glory that surpasses our present sufferings. We can even be thankful for the beautiful portrait God is creating, even if it has some dark colors, as He works all things for the good of those who love Him. As we groan, we are thankful for the hope that is in Christ.

Pictures of Joy

I caught the end of a movie as I was flipping through channels. The credits started and the accompanying music was acoustic guitar. For a brief moment, I felt lighter. The music made me pause and for a brief moment I felt free, or more accurately, above the present circumstances of life. By free I do not mean escape. I actually mean free from, or above anxiety or weariness while still being in the midst of the present circumstances. It was a moment of joy and as soon as I recognized it for what it was, it was gone. C.S. Lewis wrote a book about this called Surprised by Joy and describes similar experiences. Sadly, these movie credits reminded me that I had been neglecting joy in my life lately. Joy can come unexpectedly, but God gives us gifts and talents that can bring that joy in doses that are somewhat more predictable. I have found that I can take a decent photograph from time to time. I’m by no means as talented as some photographers I have met. I have simply discovered that with a camera I can see something, a moment, and capture that moment. I can freeze it and keep it. It can be a smile on Macayla’s face or the sun light illuminating Jennifer’s hair just right. It may be Jacob in the middle of something important to him and all the while he is unaware he’s being photographed. It may be drops of water on a leaf that sparkle like diamonds in the morning sun. It may be a landscape or a sleeping child but it can be a moment of joy and with a camera I can capture it. But I agree with C.S. Lewis. Joy is a surprise and it only lasts in doses that are a moment in length. Once the moment passes, you can never quite experience it with the same intensity as the first time. The actual experience of Joy is always in the present. But that is what makes it so reflective of eternity.
We are limited because we are caught in time. Eternity, as far as I can tell, does not mean a timeline we will move along forever. We are not simply dots on that line and everything before us on that line is the future and everything behind us is the past. I believe that what God means by eternal is a full consummation of all time into the Present. I believe that the present more than the future or past is most like eternity. I think that is why a surprising moment of Joy is only a moment. In a fallen world like ours that is so trampled by the unchangeableness of our past and the uncertainty of our future, the present is where joy is experienced. It is the present joy that points us toward God and our eternal purpose in Him. But it is that present joy that can break the chains of the past and bring certainty to our future. That is why it is important that we all discover those gifts and talents that God gave us. That is why it is important that we live our lives doing what He has called us to do. For it is through those things that God helps us experience joy and draw closer to Him as well as become the person we were created to be. That is what photographs can help me remember. Looking at a photograph may not have the same intensity of joy as the moment I actually took the picture, but it can still remind me of the joy of that moment. I can capture those little moments of eternity and they remind me of the freedom I have in Him. Not freedom from my circumstances, but freedom in my circumstances.

Not Bored

We are not bored. I just had surgery on Friday to correct some complications from my manly surgery last December. Macayla will be having surgery next week to relocate her feeding tube. Jennifer will be having surgery to correct quite a bit of womanly issues in December. Our van apparently decided it was jealous of us getting all the attention, so it decided to blow a hydraulic hose on the wheelchair lift today that will require “surgery” tomorrow.
As it turns out Macayla’s tube site is not clearing up and in spite of several months of medications, dietary changes, endoscopy, cutting, cauterizing, and new tubes Macayla still is not tolerating her feedings and we have blood in her stomach. So the only option left at this point is to relocate her tube and start over fresh. We have decided to go with a different type of tube this time. The tube Macayla currently has (a “Genie”) is held in place by a flange on the inside of her stomach. It is removed by simply yanking it out. The flange is soft and pliable but it still causes trauma to the site when it is yanked, understandably so. It is not a pleasant thing to watch and it is certainly not pleasant for her to experience. We love the “Genie” system because it is easy to use and is very close to the skin so it doesn’t dangle and get caught on anything. But this method of replacement seems to be asking for trouble in Macayla’s case. So we are going to try a “Mickey” (and yes the also make a “Minnie”) because the Mickey has an inflatable balloon on the inside that holds it in place. It will require more attention on our part as the balloon is inflated with about 5 cc of water that should be changed monthly and every few months we will have to replace the tube, but we can do all of it ourselves at home. So, we hope that she will do well in surgery which may end up as out patient this time.

Punishment

I have met parents of special needs children or parents who have experienced the death of a child. On more than one occasion, I have heard some of these parents make a statement that this happened to their children as payback for past sins in their (the parents’) lives. They feel like it was God’s way of punishing them. How do we worship a God that allows our children to die?
There are many places in the Bible where God punishes sin with death. Individuals were often struck dead by God for disobedience and laws were given in Deuteronomy that require death if broken. We are at first appalled at this. We are amazed that anyone can call God “loving” if He would do these things. However, we forget who we are and who God is. God is righteous and just. Our sin makes us unrighteous. Our sin requires justice. We recognize that law breakers should be punished in our society. We scale the punishment for the crime. God has done the same thing but He didn’t have to. There is no such thing as degrees of perfection. God is not almost perfect or mostly perfect. He is perfect. He requires His creation to be perfect. When sin entered the world, it changed not only humans but creation as well. God would have been just in wiping out creation and starting over. The most amazing thing is that He did not wipe us out. This perfectly just God did not exercise His justice completely. He actually has preserved humans and creation in spite of our sins. However, we are left in a fallen world where death, disease, and sin persist. How does God interact with sinful humans in a fallen world? Mercy.
The parents I spoke with that made the statements that they thought God was punishing them have missed the big picture. If God truly punished each of us for our sins, we would all be dead, not just our children. The miracle in a world such as this is that disease and destruction are not more rampant than they are. There is a place in the Bible that records a man being put to death for sin, but not his own. God the Son became a man, Jesus, and was put to death on a cross for our sins for he never had any of his own. God does punish for sin. He did it at the cross. God may train us, otherwise known as discipline, but He has already doled out the punishment at the cross. The death of our children is a product of a fallen world, not punishment. God the Father put that punishment on His begotten Son to redeem us and this fallen world. He has given us time to surrender to His mercy by trusting in the justice of the cross. Macayla is a victim of this fallen world but she will also be free of its destructive tendencies soon. Her eyes are losing sight now, but soon her eyes will see with full clarity the extent of God’s mercy.

More Blood

Macayla’s recovery was good from her procedure but last week she started having bright, red blood in her stomach. She has had a little more today. We aren’t sure what is happening since she had no indication of bleeding for almost two weeks and then suddenly it started again. We did some blood work on Friday to check levels and clotting but we anticipate that will be all normal. It is certainly frustrating when you cannot figure out what is causing a problem or how to fix the problem. She is not tolerating her food and we still have not gotten her up to full calories. We are hoping that the doctor can give us some insight soon as to what we might can do.

Scoped Out

Macayla went in yesterday for a endoscopy of her upper GI. We had been holding off on this because it is a procedure that requires general anesthesia and that always carries risk. But after trying medications and diet changes Macayla is still having blood in her stomach and she was not tolerating her feeds again this weekend. So, an opening came up in the OR and the surgeon worked her in yesterday. Her esophagus and stomach lining looked great. As suspected, the problem has been around the feeding tube. There was a significant amount of granulation around the tube site inside the stomach. They replaced the tube and removed all excess tissue and the surgeon feels like this should correct the problem. Macayla came through great and without vomiting in recovery or the ride home. She is recovering today and is definitely sore and tired. She tenses up anytime we give her medicine and was feeling it when I hooked up her feeding pump this morning. She only got 270 ml of formula in yesterday and that is compared to the 800 she should get, so she is low on energy. But so far so good this morning. I guess there is always the chance that new granulation will come back and if it does, we will be asking if there is anything else we can do. That may or may not include putting the feeding tube in a new location. Bottom line is that Macayla just seems predisposed to this problem. We told Jacob that Macayla had to go to the hospital to have pictures taken of the inside of her stomach. He said he wanted to see the pictures when they did it. We told him that we couldn’t go into the OR with Macayla while it was being done but he was insistent on seeing pictures so I told him we would ask for copies. I warned him that the pictures may be yucky and he said, “I know but I still want to see them.” He is definitely his mama’s son. The doctor sent Jacob some pictures home and we got to see the granulation tissue and the before and after shots of the site and its repair. He didn’t throw up or anything.

Peanuts of Truth and Heartbreak

I was finishing up a phone call and it was right at bedtime. Jacob asked me if he could have some peanuts for a snack. Since it was bedtime and he had eaten a lot already, I told him no and then turned my attention back to the phone. Jacob then brought the peanut jar to me and asked again and I said no. He ran off. I finished my phone call and walked into the kitchen to find him looking like a chipmunk with full, bulging cheeks. I asked him what he was eating and he responded by shaking his head “no” and mumbling unintelligibly through his full mouth “nothing.” He lied. When his mouth was clear I again asked him what he was eating and he said it was a cereal bar. When he realized I was shocked that he would eat a whole cereal bar, he lied and said it was only half of a cereal bar. I made sure he remembered that I told him no to snacks and he remembered. Then he admitted that he was eating the peanuts. Long story short, the amazing thing was that when he was caught disobeying me he not only lied about it, but he told three more lies to cover up the first lie and he did it all without hesitation.
In the scheme of things, a mouth full of peanuts is not a big deal and I really wasn’t upset at all. But this is my first experience with Jacob boldly lying to me about something. He wasn’t trying to pass it off as a joke or simply flirt with deception. No, he went all out. What took me off guard were my feelings. I know that my child will lie to me or be disobedient at times. I know that being human he will sin and make bad choices from time to time. I knew this would happen and I know this will happen again. But it broke my heart. It broke my heart to watch my son boldly lie to my face. In a microcosmic way, I got a sense of how God feels when I boldly sin. He knows I will sin. He knows I will at times betray Him, but how it must break His heart when we do. How heart broken He must have been when His friend Peter betrayed Him on the night of His trial and denied Him three times! Since God has an infinite capacity for emotion, I can’t begin to imagine how much more His heartbreak is compared to mine in the kitchen. Maybe that is why there is such rejoicing in heaven when we repent. When we seek forgiveness and turn back to our Father, that infinite heartbreak is overcome by infinite rejoicing and mercy. Jacob’s choice to sneak some peanuts and lie about it is just “peanuts” compared to some of the more costly decisions he will face later in life. At bedtime prayers, he told God he was sorry. There was rejoicing in heaven and in Jacob’s room. I must admit that it was pretty cute to see him try to lie through his bulging cheeks!

School and Tube

Macayla and Jacob have started school. Macayla is in a multiple disabilities class at a different school from last year with other children that have a wide range of abilities. She has some great teachers and there is a nurse in the room to help with medications and feedings. We wish we could talk this nurse in coming to work for us in our home, but it wouldn’t be fair to the other kids in the class! Macayla has lit up for them some and they’ve already seen her laugh. She apparently likes music class which is no surprise. Jacob is at the same school as last year but just in another class. He has stayed with a couple of his friends from before and has some new ones. His teachers are the teachers he first had when we evacuated from New Orleans so it was good he got to have some familiar faces. They have always been a good match for him.
Macayla’s stomach contents have been clear for the last two weeks except for one day. That means that we probably identified the source of blood as her feeding tube track. Unfortunately, her tube is getting more granulation around it in spite of multiple cleanings each day and the application of the cream used around it. She is just predisposed to it I guess. It is great to know that we have identified the source and that we can treat it albeit a less than desirable situation for her. We may have to use silver nitrate on the tube site as much as every other day. Silver nitrate essentially burns the granulation away and it is not fun for her or us. We can numb the site, but I can’t imagine it is all that comfortable.

Commandments

Last night Jacob asked me if I knew what commandments were. I said I did and I asked him what he thought they were to which he said they were rules. He said he learned about them at school. I asked him where commandments came from and he said, “God.” I asked him why God gives commandments and he replied, “To keep the people safe in the land.” That wasn’t the answer I expected. I thought he would say that commandments were rules to make us good little girls and boys, but he replied with a better answer. An answer that is closer to the truth.
In the Old Testament, God gave the Ten Commandments along with the rest of the Law for a much bigger reason than to provide a moral code. Unfortunately, religious folks have tended to take moral codes and even God’s commandments and make them an end in and of themselves. But God didn’t give commandments to see who could follow them the best. He didn’t give commandments to see how miserable he could make life. He gave His commandments and truth to actually protect life. As Ravi Zacharias illustrated with the game of tennis in Recapture the Wonder, the rules protect the game but they are not the point of the game. The rules prevent exhaustion and when followed can prevent strife and confrontation. To say it another way, umpires know the rules better than most, but they never win the game; only the players do. God gave his laws to protect our lives and in fact prevent exhaustion. The people Jacob was referring to were the Israelites as they were going into the Promised Land that was occupied by people who did not know God or follow Him. God wanted to protect the people from the strife and exhaustion that occur when we go away from Him. The Author of life knows the best way to live life. The Israelites are like the rest of us and they could not keep the commandments. But that’s the most amazing part of God’s commandments. They point us to our ultimate need for Him and His grace. They point us to Christ. Only in Him can we find freedom from life lived apart from God or a life lived in a futile pursuit of moral codes. The “land” was a form of rest for the Israelites. They were resting in the purpose God has for their lives. When we belong to Christ we are at “rest” in Him. We are resting or trusting in Him and He transforms us into who we are meant to be. His grace empowers us to follow His commandments and those commandments protect our new life at “rest” in Him. Thank you Jacob for the reminder.

Yanked and Burned!

We took Macayla to the surgeon’s office this morning and they pulled (yanked) her tube out and looked at the channel in her stomach. This means that they looked at the actual hole in the flesh where the tube is inserted and it was full of granulation. For those of you without feeding tubes, this is tissue that forms around the tube that can be quite bloody, goopy and nasty (those are highly technical medical terms!) It’s something that can be controlled by cleaning and using a special cream around the sight, but Macayla’s was down in the actual hole so it was hard to control. The good news is that the surgeon was able to “burn” it off with silver nitrate and hopefully, it will seal up and begin to heal over the next couple of days. He said he was quite sure that this was the source of the blood in her stomach and that it should clear up very soon. If it isn’t clear in two weeks, then we will schedule a scope.
Another bit of good news is that we were fortunate enough to get a safety bed for Macayla’s room. This is like a huge crib with high side rails and padding. It will keep her from falling out of the bed and it has an articulating mattress so we can position her better for reflux problems. This will also be helpful later with pressure sores when she is vegetative. We are so blessed the way things have been provided. How fortunate we truly are to have so many caring folks who take care of Macayla and the “things” that help make her life more comfortable and functional!

Mountain Tops

I apologize to those of you who have been wondering about the latest on Macayla and us. It seems that things have been so hectic and all of a sudden a month has flown by. We have been trying so many different things to help Macayla’s stomach and GI problems, that it has been impossible to keep up with all of them. We have tried different feeding schedules and medications to get the bleeding in Macayla’s stomach under control and nothing has worked. We have at least seen less frequency than before of blood in her stomach contents, but we still see it at least every three days if not every day depending on the week. This week we are going into the surgeon’s office and they will remove the feeding tube and look inside with a high-tech device; a flashlight. Really. When they pull the tube out they will be able to observe the hole in her abdomen and see if the hole itself is irritated and unable to heal due to movement. It is painful, but will be done in the doctor’s office and only takes a couple of minutes. If this doesn’t shed any light (pardon the pun) on the problem then we will have to scope Macayla’s upper GI. This will require general anesthesia which makes us nervous but we have exhausted all other options at that point. On a more fun note, we got to go to the mountains near Pigeon Forge last week with Jennifer’s parents and my mom. Macayla had some fun but seemed more upset more often during the trip. She may have just been in discomfort or she may have been confused by her new surroundings. Overall, though she did well and enjoyed the hot tub on the back porch. The kids loved feeding the ducks and birds near the Old Mill and Jacob got to go swimming a bunch. The grandparents gave Jennifer and I time to have a couple of meals together away from kids and responsibility and that was great. Overall it was a great trip and we got some great pictures of the kids and I even found an opportunity to get a couple of shots of God’s magnificent creation.

Astray

I often feel like circling the wagons. There are times when I want to keep Macayla home and not go out with her and notice all the stares. There is a difference between someone noticing Macayla and someone staring at Macayla. The stares I struggle with are the horrified stares as if Macayla is a monster. The intrigued stare as if they are looking at an alien recovered from Area 51. The “I’m going to vomit” stare because there is actual drool coming from Macayla’s mouth. Usually, I hardly notice the stares, but there are times that I just want to keep Macayla at home and away from all that is out there. It’s hard to tell if it is just exhaustion or grief or just a big mix of both. But my wife is such a good balance for my circle-the-wagons mentality. She always reminds me that Macayla has a purpose in life just like the rest of us. She needs to be out there as much as she can, experiencing what she can, and impacting the world whenever and wherever she can. My wife reminds me that those stares are opportunities to start a conversation and let people know that Macayla is a sweet little girl with a purpose. It is an opportunity to share what God is doing in her life.
I have been studying Psalm 23. It is famous for funerals, but that is not its only purpose. It is about life. It starts with, “The LORD is my shepherd; I shall not want. He makes me lie down in green pastures; He leads me beside quiet waters.” This poem starts where life as a Christ-follower starts. It starts by clearly stating who God is which indicates who we are and then it speaks of rest. The Lord is a shepherd. One who guides, protects, provides, and prepares. By sheer logic, if we call him our shepherd, we are calling ourselves sheep. Sheep are the dumbest livestock one can own. They will wonder away from the safety of the flock and expose themselves to predators as well as stray away from the shepherd and all he has to offer. The rest it speaks of is not an afternoon nap, but rest is a metaphor for trusting in who God is and in the purpose he has for our life. This Psalm was written directly out of the experiences of a shepherd and a king named David. His life taught him that people are like sheep; we have all gone astray. We have wondered away from the reality and truth of who God is, who we are, and the purposes for which we exist. Life as a Christ-follower starts and even grows by coming to grips with that reality on a daily basis. I must admit that I often don’t trust, I don’t rest, in the reality of who God is and the purpose he has for Macayla and our family. But the truth is that even when we are in the valley of the shadow of death, that doesn’t change that purpose. If anything, it’s in a dark valley that the light of Christ becomes stark. Macayla has a purpose. It may be to melt the stare of one person’s heart so that they when they look at her they won't see some strange carnival exhibit or refugee from Area 51. They will see Christ and all He made Macayla to be.

No Seizures!

Macayla’s EEG showed no seizures over the 48 hours she was hooked up. This is remarkably different from past EEG’s where we would have 100+ per day. Part of the reason is that so much of the brain has atrophied that there is less brain to cause a seizure. The other part of the equation is that her medications are controlling the generalized seizure activity. The doctor did confirm that she had some focal spikes in the temporal lobes, but overall we are celebrating that Macayla’s day is not broken up with seizures like before. This means that the episodes we have been seeing are either behavioral or pain related. In relation to pain, we will know more when we get the GI problems under control. The MRI has shown some “significant” changes again. This time the doctor said there were some abnormalities in the white matter. We aren’t sure yet what that can mean but we hope to get to view the MRI images sometime this coming week.
Macayla’s feeding is going well and we were able to get 1000 calories in her yesterday for the first time in two weeks. She has had less black or brown blood in her stomach contents over the last couple of days and she has less agitation with feeding. We are not out of the woods yet, but she is definitely on her way. He stomach contents tested positive for blood this past week so we know we have irritation but the new formula and increased protein-pump inhibitor seems to be working.
Unfortunately Macayla has stopped walking with assistance. She normally could bear weight and take steps with full assistance from us, but since we unhooked from the EEG, she stopped bearing weight on her left leg and is really not getting anywhere with it yet. Her PT and OT worked with her on Wednesday, but we did not get any good results yet. We are trying out a stander to see if it is helpful and she seems to like it. This allows her to stand safely and keep blood flow to her legs and gravity certainly helps with GI flow. Stander equals more dirty diapers!

GI, EEG, MRI

Macayla had a three day trip to the hospital for a follow-up EEG and MRI. It all went very well. Too well. She did not have any of the episodes we were most concerned about while hooked up to the EEG. As a matter of fact, she was more alert and interactive than usual. This isn’t the first time she has pulled that trick. But overall, it seems her seizures are in control compared to before. We have hardly noticed any of the seizures she used to have and after watching her on the EEG monitor, we wonder if what we think are those seizures are actually muscle spasms or behavioral. While the doctor was visiting us, Macayla had some movement in her arms that was typical of the seizures we used to see, but when the doctor looked at the EEG monitor, he said it wasn’t a seizure. So overall, seizures are down and that is good. It is most likely due to the fact that the part of the brain that was causing those has deteriorated. According to the EEG tech, there were some “focal spikes” on her EEG and that could indicate a higher probability of a focal seizure, but it doesn’t mean that a seizure is imminent. The only other possibility would be that she would be having seizures in a deeper recess of the brain and it doesn’t register on the electrodes as well. I also wonder if there is less conductivity between the brain and the electrodes since the brain has shrunk and is not as close to the skull, but I have not been able to ask that question yet. We will wait for a full report on the EEG, but overall her seizure activity is much less. After the EEG she had an MRI and we hope to hear about that in the next couple of days. As far as GI, she has yet to tolerate a full-day’s worth of calories. She gets irritated toward the end of the day while feeding. But the color of her stomach contents seems to be making baby steps toward clearing up. We have had to spread out her feedings to smaller amounts more frequently and that is what she is doing today. We are experimenting with scheduling and the mixing of formula to find what works and is efficient, but our goal today is to get 1000 calories in her. The x-ray of her lower abdomen came back normal, so we are pretty sure that it is an inflammation and not a back up. She may have become allergic or intolerant of her formula so changing to EleCare may help that. Unless she gets worse, we are holding off on anything invasive.

Don't Forget the Suction Machine!

Macayla visited the dentist yesterday for a routine cleaning and check up. They have to sedate her to work on her. She got the max dose of meds, and she never fell asleep during the appointment. She has quite a tolerance! She has already lost a couple of teeth including a front tooth and the other front tooth was pulled because it was loose. Unfortunately, the sedation affected her all day which was different from last time. The last dentist visit we sedated, but she was alert and active afterwards, but not this time. We fed her after the visit, and then headed to the GI doctor to deal with the stomach problems. When we pulled into the parking lot, Macayla threw up a little bit. She had trouble clearing for a second and to make things worse, we forgot the suction machine. Not good. She cleared, started breathing and we went in. While inside, we moved her out of her chair to change her shirt, I think, but moving her brought on major vomiting and she could not clear. It was stressful and when I asked for a suction bulb or suction machine, there wasn’t one to be found. They had to go to the 2nd floor of the office building and get a crash cart. Thankfully, Macayla was able to clear and started breathing. Once we got the crash cart, we suctioned her and she did fine. We were able to get some food in her later in the day, but at a slower rate than normal. She was groggy for the rest of the day and she slept all night without moving. I think we will remember to bring the suction machine from now on.
The GI doctor wants to increase her protein pump inhibitor and we have changed the formula we use to feed her. If we don’t see an improvement in her stomach by Friday, then we will test her stool for signs of infection that could cause irritation in her esophagus or stomach. What ever is causing it she has irritation that is oozing blood into her stomach and we hope these steps will bring healing. We had an X-ray of her lower abdomen done and we are waiting to hear if it showed any impaction or problems. She has gotten to the point that it hurts when we feed her. So we really want healing as fast as possible. This is new territory for us and we are trying to learn how to help her. The doctor is not in a hurry to scope Macayla which is good because it involves general anesthesia and we are not ready to take that risk. If none of these options help, then we will revisit what can be done and that may include procedures that are not fun, but we’ll see.

GI Update

As of right now, Macayla is not showing any signs of blood in her stool. Her stomach contents looked clear for the 48 hours we tried Maalox but it went brown again within 24 hours of stopping it. Sunday afternoon it was really dark and last night it was almost black. The GI doctor said that we should increase her Zegerid (a protein pump inhibiter like Prilosec) and give her another half dose at night. We will stay on this increase for about a week and see if it clears her stomach. If not, they already have an appointment in place to scope her. This process is hard because it takes time. There are several possible causes for her stomach having problems and we have to eliminate one possibility at a time. We are starting with the less invasive obviously. Our prayer is that the increased Zegerid will help her stomach and that will tell us that she is having stomach irritation that we can regulate and make more comfortable. Her neurological condition and feeding tube both provide opportunities for stomach irritation so it seems like the most likely cause of the problem. So we wait to see.

Sumo Perseverance

Jacob and Macayla were watching a Veggie Tales movie this morning about sumo wrestling that is a spoof of the Rocky movies. The lesson in the story is about perseverance. Jacob has to wear a patch on his eye two hours a day for lazy eye. He hates it and I don’t blame him. But considering the subject matter of the movie, I thought I could help Jacob and take the opportunity to apply the idea of perseverance to wearing an eye patch everyday. He didn’t buy it. Instead, he took a scarf and wrapped it around his waist and croch making a sumo “diaper” out of it and he asked me if I wanted to fight.

GI Issues

Macayla has always had problems with constipation, but she went over a week without a BM and it was the longest I’ve ever seen her go without. So on Saturday, I gave her some mineral oil through her feeding tube and an enema. But nothing happened on Saturday. I noticed something black in her feeding tube valve, so I drew some of her stomach contents out and found black blobs floating in it. We dismissed it as possibly left over blood from where we changed out her feeding tube at the beginning of the month. Sunday, the BM’s came with a vengeance but her stomach contents changed to a dark brown liquid. This cleared up by Monday, but was back on Tuesday. We already had an appointment with neurology and our pediatrician set for Wednesday. After all of the dust settled, we are testing Macayla’s stomach contents and BM’s for blood. We started giving her Maalox to try and promote healing of her stomach lining because that seems to be the most likely problem. Her stomach contents seem to be staying clear. But this morning she got very upset and began to cry. I can’t remember the last time she cried. She seemed to be in pain, but it is hard to tell. She can’t communicate anything and it’s hard to tell if it was from pain or frustration or just an emotional outburst. To complicate matters, we are weaning her off Cystagon (an experimental medication). We believe that while she has been taking this medication, it has been interacting with one of her seizure meds and keeping the seizure medication level down. As we wean her off the Cystagon we expect the seizure medication level to increase. That increase could cause behavioral changes. So Macayla’s responses to her environment are tricky to read. She could be having a seizure, be in pain, be having an emotional outburst, be reacting to changing medication levels, or she could just simply be having an appropriate response to something, like a dirty diaper. The events that have kept her up at night that resembled seizures could have been responses to a painful stomach. We will be doing an EEG soon to determine if that is the case.

Runaway Grapes of Irony

We’ve had two nights of Macayla not sleeping. She seems to be having seizure type activity that wakes her out of a hard sleep. We opted to give her Diastat last night to stop it as we did a few weeks ago. Needless to say, we are tired. Jacob, on the other hand has had two of the best nights of sleep in a long while. This morning, I really wanted to make sure Jacob got to school because his class was going to a swim park and then making banana splits. (Actually, I wanted to leave him with Macayla and I wanted to go!) So, I moved a sleeping Macayla from her bed to her wheelchair and loaded them up to go. But Jacob wanted to take the last four grapes he had from breakfast with him. He picked up his bowl and was carrying that with both hands and his bear was tucked under his arm. He was walking down the slope of the driveway while I was loading Macayla on the van lift. I notice a grape roll across the concrete in front of me and it was followed by the whining voice of Jacob. “Dad, I lost my grape!” My impatience was pretty high this morning and that was aggravated by two sleep-deprived nights. I told him to let it go and get in the van. “But Dad I wanted that one!” By this time, I am walking around to the side of the van to strap Macayla in. Jacob is whining and so worried about the runaway grape that he doesn’t notice that he is tilting his bowl. Two more grapes roll out onto the concrete. Jacob and I paused and watched the two grapes roll under the van and out of reach. The whining and crying were about to pour out of Jacob. I could see it coming like a sandstorm rumbling toward me across a desert plain. But my frustration was faster to the draw! I grabbed the last grape in the bowl put it in Jacob’s hand and then jerked the bowl and took it back into the house. Jacob in the meantime ate his grape and pouted in his car seat. I came back out and told Jacob that he better get his act together and show that he was thankful that he was going to get to go swimming and make banana splits. I reminded him of how difficult it was to get him there because I had such a “hard” night with Macayla. But the reality is that I missed a teachable moment and all I really wanted was to have myself acknowledged. I wasn’t concerned about Jacob being thankful for swimming and banana splits. I wanted him to be thankful for me, Super Dad! What a disappointment I can be. Jacob had four grapes and one rolled away. Once it was gone it was gone and there was no way to get it back. He was so worried about the one that got away that he forgot what he still had, three grapes in his bowl. His whining and concern over the one grape cost him two more. I was so concerned about the sleep that I lost and couldn’t get back. I was so concerned about the acknowledgement that I was not getting, that I missed a great teachable moment for my son. He didn’t need a lesson in “be more careful next time.” He certainly didn’t need a lesson in how well I am at pitching the adult version of a fit. He could have used a lesson in that being thankful comes when we recognize the blessings we do have and that loss can be a lesson in itself. He may have profited from the lesson that when one grape rolls away, it’s not fun, but it makes you appreciate grapes more. Here’s the fruity irony of runaway grapes. Jacob has recently learned about the fruit of God’s Spirit: love, joy, peace, patience, gentleness and self-control. I’ve been encouraging Jacob to ask God to help him be more patient and self-controlled during hard times. Jacob did not see any of those fruit in his Dad this morning. Now that he’s at school, I’ll have to wait until this afternoon to apologize to him and ask for his forgiveness. I hate waiting on that, but there’s a lesson in that too.

Melatonin

Macayla has to take melatonin to help her sleep at night. Our doctors suggested this to us. It is a natural substance that we produce in our bodies that essentially helps us slow down to fall asleep. According to our doctors, people with neurological conditions may have trouble producing melatonin properly. So we have to give it to Macayla. However, the normal starting dose form an adult is 3 mg. Macayla takes 12 mg and sometimes 15 mg. The doctors stated that this is where to start with children like Macayla. It has helped to keep her sleep more consistent, but there are no guarantees on some nights. The best thing we have found though for anyone who has a feeding tube is a melatonin capsule made by Life Time (a vitamin and herbal company) and we get it from a local health store. It can be opened and sprinkled in 20 ml of orange juice with 20 ml of water so that it can be pushed through the feeding tube. We have used tablets and crushed them, but they don’t dissolve or mix well at all. This is one of those practical things I thought might be helpful to others with children on melatonin. It’s like doubling diapers for quicker changes on the road.

What's your prognosis?

For the past couple of weeks, Macayla has struggled with her walking. She cannot walk on her own, but we have been able to assist her by holding her up and allowing her to make the walking motion with her legs. We have a gate trainer, but this has had minimal success with her. This is a device that resembles a walker that babies use when they are learning to walk, but it’s made for larger children. With our assistance, she only shuffles at best and for just a few steps. Her therapist got her to walk better on Wednesday by using a slightly different technique. I tried to learn this technique, but have yet to be consistently successful.
All of this to say, Macayla seems to be losing another ability. It is these times that we are reminded of where this is headed. We know what the prognosis is. It will be vegetative and fatal. The word prognosis is from Greek and the “pro” means prior or before and the “gnosis” means knowledge. We have prior knowledge of what will happen. We know what will happen, but that doesn’t prepare me for the grief of watching it happen. That prior knowledge is not a bypass for pain and grief. This is also on top of Macayla’s strange “episodes” that may be seizure activity where she seems to have rigid and repetitive movements accompanied by vocal outbursts and sometimes laughing. Our prior knowledge of symptoms does not compare to the actual experience. I went to a funeral this week for a young teenage boy who died suddenly. I know we will have a funeral for Macayla one day, but as I looked at those parents, I was reminded that there is no preparation for burying your child.I know more abilities will be lost. I know we have new types of seizures to experience. I know we will have feeding tube problems. I know we will have blindness. I know we have a funeral to plan. But I believe in another prognosis. I believe that Christ has her. I received that prognosis before we got our diagnosis. That prior knowledge tells me that Macayla will be with our Lord and she will be whole. She will be kept safe and will experience love and healing in its fullness. But between now and heaven, it means that we will experience Christ in our lives through all of this. A guy named Paul once wrote, “We grieve, but not as those without hope.” Hope is stirred by some sort of evidence. Christ makes himself evident in the midst of our grief through Macayla, Jacob, Jennifer, and so many people around us. Our prognosis tells us that Macayla’s life will and is impacting people around her and at the end of this phase of the journey she will be with Christ for eternity. Because of the fact that I have trusted Christ as my Lord, I have the same prognosis. The good news is that eternity has already started. Here again, the prognosis does not compare to the actual experience. I hope all who read this have the same prognosis as us.

Camp New Hope

Due to the generosity of some great folks up in North Carolina, we were able to stay in a mountain lodge for a week stocked with food and fun. The five bedroom cabin we stayed in was situated on 160 acres along a river in the mountains near West Jefferson. The camp is called Camp New Hope and was started by two brothers who wanted a place to let families with special needs get away without the financial burdens of vacation. The land was gorgeous and they keep the areas around the house and along the river front mowed (a three-day task every week). We were able to take Jacob on the canoe and paddle boat. Macayla was given a ride in an ATV that was essentially a golf cart on steroids. She loved to ride in it. My parents and little brother went with us and it was great to spend some with them away from the hustle and bustle of life. It’s hard to convey how relaxing it is to be there. The cabin donned a front porch enclosed with windows so that we could sit and have the view of the river below and enjoy the afternoon sun and breeze coming through the screens. Our hostess, Randy, went the extra mile to make sure we had anything we needed and even the things we just wanted. She made a special run to the store to get Jacob some pink ice cream and some orange sherbet just because he likes it. She even did some laundry for us!! We got to go up on the top of the mountain and get great video and pictures. We saw a lot of nature and went swimming and fishing. They have commercial coolers like the ones in convenience stores that are stocked full of sodas, water, Gatorade and candy. It was cool enough at night to have a campfire next to the river and roast marshmallows. The lighting bugs in the trees at night put any set of Christmas tree lights to shame. They made the trees seem alive with light. They hovered over the water and reflected their glow and an occasional lighting bug would crawl around in the grass at your feet and make the ground glow beneath you. The only thing that would have made our trip better was if it lasted another week. We’ll have more pictures on the photo album page soon.

Bat Pooh

We had a birthday party for Macayla and Jacob on Saturday, June 2nd. Their birthdays are only a week apart and we decided to have it all together in our backyard. Jacob wanted “superhero” party but he couldn’t decide on which hero. So, we had several. We had Mutant Teenage Ninja Turtle plates and Spiderman cups and Superman napkins and so on. Macayla on the other hand had a Winnie the Pooh party. So we had two tables, one with heroes all over it and the other graced the gang from the hundred acre wood. We had a Winnie the Pooh cookie (the large chocolate chip cookie with Pooh drawn on it with icing) and a pull-apart cupcake cake with Spiderman icing and multiple action figures stuck in the top. The kids played on the swing set and had hotdogs and cake and rejoiced when the candy poured from the piñata. It was fun. But there was one thing missing in my opinion. This was a superhero and Pooh party. Batman is Jacob’s favorite hero and I suggested that we decorate the Winnie the Pooh cookie a little more. I suggested that we use black icing and draw a Batman mask and cape onto Pooh and we could call him “Bat Pooh.” Alas, the chocolate chips may be too reminiscent of guano! If you aren’t sure what guano is, just walk inside a cave infested with bats and see what they deposit on the cave floor! My wife was not keen on my idea. It was vetoed!

"It's like an earring...?"

Fridays are the exciting (scary) days around here. Last Friday we got our first experience with using Diastat on Macayla to stop a long seizure. This Friday, we changed out the valve on Macayla’s feeding tube in her stomach. She has what is called a Genie feeding tube which is basically a button that is flush against her tummy and this button allows you to snap tubing from the feeding pump on to it. Every few months, the valve needs to be replaced. So, we replaced it and that involves pulling the tube out enough to get a clamp on the actual tubing behind the valve. This clamp seals it off while the valve is removed and a new one is put on. The valve change went great, but shortly after, Macayla’s shirt and bed were soaked and smelled like formula. We discovered that the tube had a cut in it. It was pouring out from around the valve when we moved her. At this point, we aren’t sure what to do. We’ve never experienced this before and we were not prepared. I thought that the only way to replace the actual tube that penetrated her tummy was in surgery. So, I am panicked and kicking myself because I was the one that changed the valve and may have damaged the tube with the plastic clamp. She would have to go under anesthesia which is risky. Will she come out of it? Needless to say, I felt terrible and angry. We took her to the surgeon’s office and they looked at it and said, “Yep, we’ll have to do some yankin’!” “What exactly do you mean by that?” I asked. “We just yanked the old one out and thread a new one in,” our nurse said casually. I couldn’t believe that you could just yank it out without pulling her belly open and I really couldn’t believe how casually the nurse said it. But I must remind you that I get the willies if there is a hair wrapped around Macayla’s tube. Yanking it out would make me pass out! Sure enough they just yank it out and the soft, pliable flange inside folds up and comes out. It is painful they say. Macayla didn’t flinch. Well, according to my wife she didn’t. I was outside the room because I wasn’t about to watch. They threaded another one in with a rod and cut it to fit and installed the valve. The nurse told me it is like having your ear pierced. She said that tissue had built around the tube and made a tunnel or hole that stayed open like the hole in a pierced ear. That’s all fine and dandy, but only the pin of an earring goes through the ear, not the diamond stud on the end! Macayla had a flange yanked through her belly. She didn’t flinch and she was back home and eating by 7:30 that night. We weren’t prepared, but the tubes do fail. The valves and the tubes themselves. When they do, it’s a mess and it’s scary. But it was much simpler to fix than we thought. It is painful for most kids. It was described that it felt like having the wind knocked out of you. Macayla can’t express pain properly, so we aren’t sure what she felt. But all is well and we were able learn and prepare for the next time.

Seizure

Around 4 a.m. this morning, Macayla started stirring in the bed. She’s been restless lately between 3 a.m. to 4 a.m. But this morning, she was having what we believe was a long seizure or cluster of seizures. It was different from what we have seen before. Her legs and arms jerked rhythmically and repetitively while she made some strange sounds over and over. The movements themselves weren’t as unusual as the fact that we couldn’t interrupt them. Anytime she gets hyper and jerky, we can usually interrupt it by changing something in her immediate environment. If she’s watching TV, we can turn it off and she will pause and look at it. If it’s dark, we can turn on a light and she will pause and look around. But this morning, she didn’t stop even when we turned on the light. I picked her up and she continued to do it in my arms without acknowledging me. We decided it was probably a seizure when we saw it suddenly stop and she was instantly asleep and then it started again as quickly as it stopped. The second time it stopped, she looked around and even made eye contact with me for a moment before closing her eyes and it started again. Around 4:45 a.m. we gave her diastat which is a rectal medication used to stop seizures. It is basically made to knock her out and stop the seizures. When we were prescribed the medication two years ago, we had to test it on Macayla in the doctor’s office to see how she reacted. That trial put Macayla to sleep in three minutes, but this morning it took 15 minutes. We’ve never had to use it before this morning for seizures. We are increasing some key medications to help control seizures more. In the meantime, Macayla’s digestion processes have been off for about two weeks. She has been spitting up frequently and even threw up on her birthday (not a fun way to celebrate 6 years old). She has had more difficulty staying regular as well. So, all in all, she has kept us guessing and on our toes. But we are so thankful for the blessing that is Macayla. She is beautiful and it’s amazing what she endures and still smiles.

Six

Six years ago at 7:49 a.m. in Greenwood, SC Macayla Grace Smoak was born. She came out at 7lbs 11 oz. and was pink, wrinkled and gooey with a head full of hair. All that hair compelled some of the nurses to ask Jennifer if she had a lot of heartburn during the pregnancy. Apparently there is a connection (wives tale) between a baby with hair and heartburn during the pregnancy. Jennifer did have a lot of heartburn, but it was probably brought on more by Macayla’s father than Macayla’s hair. I got to give Macayla her first bath with the help of my mother-in-law and sister-in-law and it wasn’t long before Macayla took her first nap. Jennifer did well except for her placenta not delivering and that required a quick trip to the O.R. after delivery. I did well until I watched them put a needle in Jennifer’s spine to control pain during delivery. That’s when I hit the floor for a few seconds! But all first-time Dads are allowed a floor nap in the delivery room. Macayla was an amazing adventure when she was born and I was ready to have twenty children. Macayla hasn’t disappointed us in the adventure department! She’s still my little girl and always will be. It’s amazing to take stock of all that has happened in that time. What is about to happen over the next six years? This past week I studied to prepare for a Bible study and in that process, I was reminded that Genesis records God creating the heavens and the earth in six days. God could have created everything in an immeasurable instant, but He chose to take time and bring order out of chaos. Step by step, one piece of the puzzle after the next, God put the universe together and when He was done, He saw what was complete and said it was “very good.” Six years ago, God started a process in our lives. This process will affect us for the rest of our days, but its purposes are found in a greater plan. The plan to redeem the world that fell shortly after it was created. It was no longer “very good.” Macayla is a victim of that fallen world, a world with defective genes and defective souls among other things. But through Christ, we are being re-created. Step by step, one piece of the puzzle after the next, God is re-creating us through a process and not an immeasurable instant (probably because we don’t handle sudden change too well as humans.) God promises that He works all things to good for those who love him and are called according to his purpose. The blessing of Macayla and the curse of Battens Disease will all be worked into that plan. How? I don’t know. But when it is finished, we will be able to see the re-creation and see that it is indeed “very good.”

Interactive

For several weeks now, Macayla has been much more interactive and it has been great to watch. But she is also experiencing more hyper episodes that involve a lot of screaming or laughing. Her body gets quite rigid and she flinches and jumps very suddenly during these episodes. We met a young boy recently that suffers from gelastic seizures which are known to cause emotional outbursts such as laughing or crying. We are trying to catch these moments on video to send to the neurologist for review. We shall see, or I guess, he will see and then tell us if there is anything to it. I wish we could say that Macayla is just having a good time, but it certainly seems that she is distressed when these events happen. It also causes her to spit up more and since her swallowing is disorganized, that can be difficult for her and cause her to choke. So we hope to learn more soon. We are excited over the consistent level of interaction she has maintained. It gives us more glimpses of our little girl. One of the neatest things is when she makes eye contact with me in the rearview mirror (something she hasn't done in almost a year) and begins to giggle. That will brighten your day in a hurry.

Ruts

It’s amazing how ruts work. When I was first learning to drive, we lived along a well traveled dirt road. After a good rain, the road would be rutted from the many cars that traveled it. Once the dirt road would dry out, the ruts would be hard and formed and they would alter how you drove on it. If you’ve ever driven down heavily traveled dirt roads, you know what it’s like to feel your vehicle suddenly shift and drop into ruts made by those who have gone down the road before you. If it is a dirt road you lived on, then chances are you made those ruts. No matter how hard you try to avoid them, eventually you will feel the car jolt over and drop in the ruts and steering becomes a frustrating experience. The ruts act as tracks and this is why there aren’t steering wheels on train engines.
All this to say, I get in ruts. I’m in a rut now. Ruts are those old patterns in life that seem to haunt us and as we go down the dirt road of life that we live on, we fall back into the ruts that we made the last time we traveled that way. I’ve tried turning the steering wheel to make my car jump out, but it is no use. I recently thought of the title of a song “Jesus Take the Wheel” and that’s exactly what I want Him to do. So, I slid over to the passenger seat and offered the driver’s seat to Jesus, but He slid me back over behind the wheel! He told me that if this metaphor put Him at the wheel, then I would be free of having to make choices since He would be making them all for me. That would not do. I am left to make choices and do my part. He said that He would go up ahead at the appropriate spot in the road and get his road crew started. It’s a family-owned road crew as it turns out. A Father and Son and Spirit team and the Father has many adopted children that He employs as well. They would get some of the ruts out up ahead, but for now His grace was sufficient. I honestly don’t like that answer. That means that I have to continue through the discomfort my situation for now. A man named Paul had some affliction that he prayed to God three times to remove and God gave him the same answer, “My grace is sufficient.” This same Paul also wrote that he did not “consider that the sufferings of this present time are worthy to be compared with the glory that is to be revealed in us.” Paul stated that God works all things out for the good of those who love Him, those who are called according to His purpose. Those He called are justified and those who are justified are glorified. So, there’s something to look forward to, I guess. But this is going to require some perseverance. Where does that come from? Oh, yeah the grace. It comes from God’s gift of grace that has saved me through faith. It is that grace that has re-created me in Christ for the good works that God prepared beforehand so that I might walk in them. I guess in keeping with the metaphor… “so that I might steer in them.” As it turns out, sometimes we have to follow the ruts until a better spot in the road comes up. If you don’t keep the car moving, it can get bogged down and stuck up to its axles. God has prepared the good works ahead and He’s using the ruts now to get me there. Maybe staying in the ruts is preventing me from sliding off into the ditch not to mention there may be a few good works to do while in the ruts. Whatever the reasons, I pray that I can rely on that grace and pray without doubt for God’s wisdom as I navigate the frustrating ruts of this life. It’s amazing how much you can learn about life from dirt roads. Don’t even get me started on the dusty days or the ripples that form and rattle your fenders off! Guess you have to drive it to understand.

Life

I had a birthday recently. On that day, we met with our pediatrician, geneticist, and a pediatric intensive care doctor to discuss what to do on the day of Macayla’s death. This may sound morbid or strange, and that would be because it is morbid and strange! The discussion itself is not the morbid and strange part, but it is morbid that we even have to have that discussion. It is strange that on the day my family was thinking about the day I was born, we had to begin thinking about the day Macayla would die. But, it is a necessary and healthy thing to do for our family. Macayla’s disease will kill her. In her case, she may not come through a bout of pneumonia or a car wreck like she would have if she were not affected by Battens disease. The most likely cause of death will be from a pulmonary arrest as the airway becomes floppy because her muscles around her airway lose tone. It can be brought on by a major seizure as well. Nevertheless, unless she is healed on this side of heaven, she will die. But the same is true for us all. The decision we have to make for Macayla is to what degree we would intervene when her body begins to fail. If she were to have pulmonary arrest, would we intubate (insert a tube through the mouth to the lungs to help air flow)? Would we place a trach tube in her neck? Once we start those interventions, how long will we continue them? The outcome of our meeting with some special and caring doctors was that we are more educated on when and how those interventions are done and we are forming our decision now. I would like to encourage all families to think through those decisions now, even if you are healthy. It prepares you and your loved ones to respond to tragedy. It doesn’t make the tragedy any easier, but it at least prepares us on some levels. It also puts decisions in place so that if something happened to Jennifer and me, her parents would have decisions made for them as they take over Macayla’s care. The doctors told us that if we chose not to intervene with intubation or a tracheotomy that it will be difficult to go through those last moments with Macayla as she struggles for air. If we chose to intervene fully for as long as possible, that it makes those decisions for us, but it won’t change the disease or the outcome. It will only delay it. We’ve already intervened in that we placed a feeding tube to provide her nutrition, but this pales in comparison to the other interventions we must consider.
We know that Macayla will be healed. It may be here with us or it may be with Christ in heaven, but she will be healed. There are times when technology is a wonderful blessing that saves lives and provides quality of life for many, but I don’t believe that we can always hold to the idea that we must use technology to delay death. We may have the means to do so, but is that why we should do it? There is one being in this universe that has the means to keep someone alive. God could not only keep Macayla alive, but He could completely reverse the disease itself. It makes me realize that God has the means to prevent death, but He chooses not to very often…or does He? God is not evil and neither is a family that may decide in a situation like ours to not intervene. Likewise, God can heal and it is good for a family if they decide to do full interventions. At the heart of this decision is understanding reality. If we know that reality involves much more than what we can see with our eyes and that life is more than breathing oxygen and pumping blood, then we are more equipped to make decisions that promote life. Life is also in a realm that we cannot see with our eyes. This is not to say that we shouldn’t treat people like Macayla and their symptoms. This is not to promote assisted suicide. But we must be aware that Macayla’s life does not end with her last heartbeat. It will in fact take a giant leap forward. God may call us to intervene more than we would think for a special reason, or He may call us to allow the disease to take its natural course. Either way, I fall back on the promise that He made to me before our diagnosis: “Macayla will die, but I’ve got her no matter what.”

Keepin' On

Macayla has been busy lately. The most exciting thing today was that she once again demonstrated she can communicate. At therapy she used the switch devices to communicate that she wanted to go down the slide by pushing the button that said “go!” It was exciting and everyone in the room was cheering her on. What a blessing! This past weekend, we went to the mountains in north Georgia for a wedding. It was a beautiful place and the kids loved it. We stayed in a house that overlooked a river and Jennifer and Macayla took a nap out on the porch with sound of the river below serenading them to sleep. Jacob and I explored the slope above the river and went climbing and exploring. A couple of weeks ago, Macayla participated in the Special Olympics. She did a beanbag toss (as in the small beanbags, not the kind you sit in. She’s strong, but not that strong.) She did a ball toss and a relay race. It was such a blessing for me to see all of the families in Anderson that have special needs and abilities. We enjoyed it and I would suggest it to anyone to go and be apart of it. These kids and families impact others in unique ways. Macayla is full of surprises and we are so thankful for her and all of the people who pray for her and us. She was born for the glory of God.

Big Macs for Mac

Big Macs for Macayla! No, not the double-decker cheese burger from McDonalds. It’s a big, green button. Macayla’s therapist has accomplished something that none of us have been able to do. She has taught Macayla a new skill! We have been frustrated and sad, because Macayla has not been able to learn new skills but only able to lose skills. We tried to teach her sign language and she only picked up one sign (“more”) and that was over a year ago. She cannot do that sign anymore. We have tried using flash cards to teach her a new system of making choices. This would give her the option of choosing a card with a picture on it that represented something she wanted. For instance, she would be able to choose between a card with a picture of the TV on it and a card with a picture of a slide on it. She only mildly warmed up to that system and that was short-lived. We tried using Big Macs which are large, round buttons, usually brightly colored, that are designed for kids with speech issues and coordination issues. These are helpful because the whole top of the device is the button and this makes it easier for the children to activate it. They can contain recorded messages so that when the button is pushed the message is heard. They can also be made to activate just about any electrical device such as toys, TV, fans, music, etc. Macayla has never gotten into them until last week. This is the switch device I referred to in the last entry. Macayla went to therapy this week and she immediately recognized the Big Mac (which in this case was a smaller, yellow version) and tried to activate it to go down the slide. In other words, Macayla did not have to be retrained to activate the button to go down the slide. She learned a new cause-and-effect skill!! As with saying a sound for the word “go”, this may be hard for some to appreciate, but it is huge. Macayla learned a new system to communicate and used it. That’s exciting!! It may only last today, but we will take it. Now, we look to getting some Big Macs for Mac!