You are burning my biscuits!

Recently, I had a new durable medical equipment (DME) company come to change over my services. The current company I have provides medical supplies for ventilators; however, they have very little knowledge about neuromuscular disease (NMD). The new DME company provides services only to patients with NMDs. This new company understands the needs of folks with NMD and tries hard to provide the best support for their patients. Encouraged by their knowledge about NMDs, I was excited to switch companies.

As the man from the DME company was talking to me about the ventilator and the system he would be renting me, he made the comment, "You didn't need to get a trach. I could have managed you by using non-invasive ventilation." I was a bit stunned by his boast. I have done extensive research on non-invasive ventilation and the proper settings to use. I tested all sorts of settings on my machine, trying to find the best relief from my failing respiratory muscles. Nothing worked. As I probed a bit into this man's remarks, I discovered his fatal error. He assumed because I still retained some muscle strength in my arms and legs, my respiratory muscles were not very weak. I wish I would have had the energy to explain to this man why he was very wrong in his assumption.

Mitochondrial Disease selectively attacks one group of muscles or one organ system. The disease can attack and attack and attack the organ system until it has failed or is extremely weak. The disease does not progress in an orderly fashion like many other NMDs. Most NMDs affect all other systems--speech, swallowing, muscle strength, coordination, etc.--and lastly affects the respiratory muscles.  Most folks with NMDs who get trachs are paralyzed or have so very little muscle coordination that they rely 100% on others to care for them. Many doctors unfamiliar with the randomness of Mitochondrial Disease mistakenly believe they can manage Mitochondrial disease patients using only non-invasive ventilation because the patients are usually still able to speak, swallow, and retain strength and coordination in their arms and legs. The doctors often do not recognize the severity of the respiratory muscle weakness. Unfortunately, this belief is often fatal. I have had a couple friends with Mitochondrial Disease and respiratory failure die because their doctors did feel they needed a tracheostomy. One doctor remarked, "I do not preform tracheostomies on my patients until they lose the ability to swallow." Unfortunately, many with Mitochondrial Disease and respiratory failure die while still maintaining the ability to swallow.

After the DME man left, I was stewing in his comment about me not needing a tracheostomy. Was I imagining the vast improvements I have seen with the trach--the abundant sleep I am now able to get, the increase in energy I now have, being able to eat and drink and talk while the vent forces air into my lungs, and not feeling as though I am fighting for each breath and fighting to live? I discussed this man's comments with my mom. And like a warm embrace, she comforted me with her words of wisdom. "How many patients with Mitochiondrial Disese has the DME company assisted? Did you not see the incredible concern on your pulmonologist's face when you said you would wait to get a trach? He was scared if you waited you would die. Do you trust this guy from the DME over your doctor?" Well, when she put it that way, why was I doubting my doctor and his extensive medical career? My doctor believed I would die without a trach. Whose opinion is more important, someone with extensive medical training and a very long medical career or a man who services ventilators? Praise be to God for my mom! When uncertainty strikes, she makes everything clear.

Living fearlessly in a Godless world

When my health challenges me and causes me to seek medical attention, I must leave my world of God's abundant love and face the fear-filled world. Since I rarely leave my house except to go to church and most of my friends are Christians, it is always a jarring experience to suddenly be surrounded with folks who do not know or believe in Jesus.

It seems doctors, nurses and medical staff love to tell me hopeless news. "You now use invasive ventilation. You need someone with you 24/7. What happens if you get a mucus plug or your ventilator gets disconnected or your ventilator malfunctions? You need someone by your side to assist you or you will die." As much as these folks try to "help" me with my medical situation, I always ignore their comments. I often look into their faces and wonder why they are so fearful. If something happens, God is by my side. He will lead me in the way I should go. If I am to live or die, that is up to Him. "Naked I came from my mother's womb, and naked I will depart. The LORD gave and the LORD has taken away. Blessed be the name of the LORD" (Job 1:21).

When I try to explain my lack of fear, I am often attacked. "Do you not want to live? Why are you being so difficult? You need to be fearful of getting an infection or getting a mucus plug and dying!" I often sigh and silently talk to God. "Wow, Lord. Is this how the rest of the world thinks? Why are they so full of fear and dread the day of their death? Why are they so hopeless? It makes me so sad that so many folks live this way. I pray for these folks to surrender their lives to You and take comfort in Your presence. I am so glad You will never leave me nor forsake me. I am so happy to be able to rest in You. For You say, 'Come unto Me, all you who labor and are heavy laden, and I will give you rest. Take My yoke upon you and learn of Me; for I am meek and lowly in heart. For My yoke is easy, and My burden is light' (Matthew 11:28-39). Thank You for taking my heavy burden. Thank You for giving me abundant joy and love. Amen."

"There is no fear in love, but perfect love casts out fear because fear has torment. He that fears is not made perfect in love" (1 John 4:18).

Trust and leave the rest to God

Any time I make a commitment to do the Lord's work, I always pray for strength and leave the rest up to God. Burdened with the desire to host a Bible study, my trust in God has been tested many times. There have been so many time when I was overwhelmed with fatigue and had no idea how I was going to lead a Bible study. I would pray, make a large cup of very strong caffeinated tea and head off to study. Time and time again, God would show me His glory. When my breathing was too ragged to lead the study, God would raise up one of the participants to lead the study. When my brain would become lost and confused in my notes, God would impress on one of the participants to ask a question, which would often lead me back to my place in my notes. Every Tuesday night, God provided the strength, knowledge and cooperation of the other participants to make each session a success.

When I was scheduling my tracheostomy surgery, I intentionally asked to have the surgery on Wednesday. I host Bible study on Tuesday nights. A Wednesday surgery would give me almost a full week to recover before hosting the next session. Unfortunately, as hard as I tried, I was still recovering in the ICU on the following Tuesday. With a heavy heart, I had to cancel Bible study. The following week, I was very fatigued and could only mutter a few words before I started coughing and choking. My vocal cords were still very weak, and my lungs were making a lot of mucus from the irritation caused by the tracheostomy. Determined not to cancel the session, I prayed to God, "If this Bible study is supposed to happen, give me the strength to make it through it. If this session is not supposed to happen, then let no one show up for it." Before the session, I was notified by a couple people that they could not attend. Determined not to cancel, I headed off to Bible study. I waited beyond the start time of the Bible study, and no one showed up. "Ok, Lord. No one showed up. Thanks! My voice is so weak, and I am so tired. I am thrilled to be able to go home and rest."

The following week, I was making tremendous improvement with my voice. My lungs were getting used to the tracheostomy and making much, MUCH less mucus, which meant I was coughing less and choking less on the mucus. I still was having problems maintaining a strong voice without giving way to coughing spells which resulted in choking on coughed up mucus. After a short time, my voice would give out and soon you could not hear me above the sound of my ventilator. Again, determined not to cancel Bible study, I headed off for another session. This time, God richly blessed me! Somehow during the entire session, my voice was strong and clear. I had no coughing spells and never once choked on mucus! It was an incredible miracle! "O give thanks onto the LORD, call upon His name, make known His deeds among the people. Sing psalms onto Him, sing psalms onto Him; talk of His wondrous works. Seek the LORD and His strength, seek His face evermore" (Psalm 105:1-2, 4).

Navigating uncharted waters

If I was not a duck out of water before with having Mitochondrial Disease, now I am definitely in unfamiliar territory with a tracheostomy. Trachs are not very common in Mitochondrial Disease, and those who receive them are usually babies and toddlers with a very rapidly progressing form of the disease. These precious youngsters usually die by their fifth birthday. Trachs in adults are extremely rare. In a Mitochondrial Disease Facebook group with over 10,000 members, only three adults have tracheostomies. Why so few? Mitochondrial Disease in adults is usually a slow progressing, chronic disease. It causes lots of aches, pains and diseased organs. The most common cause of death in adults with Mitochondrial Disease is heart failure, heart attack, stroke and seizure. Respiratory failure is a very rare complication of the disease.

Perhaps, the lack of adults with Mitochondrial Disease and respiratory failure has been an incredible blessing. Since I have had the tracheostomy surgery, I have been reading lots of websites for people with ALS, which has the best information about respiratory failure and neuromuscular disease. As I have been reading the protocol for people with ALS, when they are in respiratory failure with high carbon dioxide levels (the state I was in before having the tracheostomy surgery), the ALS patient is given a choice: tracheostomy or cemetery. The person either has to agree to have a tracheostomy surgery, or the doctor sends the ALS patient home armed with high doses of morphine. (The morphine is given to lessen the suffering as the person dies from high carbon dioxide levels.) It was a bit frightening reading this protocol. I am glad God never presented me with this decision to make. Every doctor I have ever seen has only given me one option: You need a tracheostomy!!!

Now, I must look to the future. I must chart my course through these unfamiliar waters. I must be the explorer seeking the best way to manage my health. I must come to terms with the fact that I will have a tracheostomy for the rest of my life, and I will also need a ventilator to breathe. For the rest of my life...I wonder how long that will be? Thankfully, there are no life expectancy statistics for adults with Mitochondrial Disease and tracheostomies. Instead, I will rejoice every day that God has given me the opportunity to live another day--another day to breathe, another day to see God's abundant blessings, another day to worship and praise His name. "I will sing onto the LORD as long as Iive; I will sing praise onto my God while I have my being" (Psalm 104:33).

What's the big deal? It's just a trach.

Before having the tracheostomy surgery, I talked to numerous people about getting a trach. I cannot tell you how many people told me I should not get a trach. "They are dangerous. There are lots of complications. You will be susceptible to lots of infections." These warnings instilled a bit of apprehension in my decision to have the surgery. Would this surgery be a total disaster?

Now that I have had the surgery and have recovered, let me say this surgery has been a piece of cake. In terms of pain, I do not think my pain level ever was above a 6-7, with a 10 being extreme pain. In terms of surgeries I have had gall bladder removal, pancreas surgery, intestinal feeding tube placement, ruptured ovarian cyst removal and wisdom teeth extraction--all been far more painful than this procedure. The biggest obstacle to overcome at first is trying not to move. With a freshly placed trach, the slightest movement of the tube causes a lot of irritation in the respiratory trach which leads to coughing spells. The coughing spells dislodge mucus which then can clog your airways.

In terms of having a trach, the care and maintenance of it is quite simple. The trach has an inner cannula which needs to be taken out and cleaned 1-2 times a day. Cleaning is easy. I pull out the cannula and throw it in a hydrogen peroxide and water solution. I use a brush to scrub away any mucus. I then leave the old cannula in the solution for several hours until I need it for the next cannula change. In the meantime, I insert a clean cannula into my trach.

If I feel as though there is mucus in my lungs, I have a suction machine. A thin flexible plastic tube is inserted into my lungs. When the tube starts making me cough, I turn on the suction machine and start pulling out the plastic tube. The suction machine does an excellent job at clearing away mucus deep in my lungs. Since I still have a good cough reflex, most of the mucus in my lungs is coughed up. I simply unhooked the ventilator tubing from my trach, place a Q-tip in my trach hole and cough. Mucus comes shooting out and gets caught by the Q-tip.

As far as other things which need to be done, the humidifier must always have water in it. When the water is low, open the humidifier and pour in distilled water. I also have a neck tie which holds the trach tube in place. This should be replaced once a week. Lastly, the ventilator tubing which needs to be changed once every two weeks.

There are many things I have been warned to stay away from--smoke, aerosols, perfume, sick people, crowds, etc. However, since I have a very limited life due to severe fatigue, this has been relatively easy.

When compared to other medical devices, having a ventilator and trach has been relatively easy. I used to have an intestinal feeding tube. I constantly had issues with the feeding tube leaking. (Let's just say, partially digested feeding tube formula mixed with intestinal juice is VERY smelly!!! Moreover, the feeding tube leakage burned any skin it came in contact with.) I also once had a peripherally placed catheter. The constant pain associated with this tube in my arm was mind-numbing. The line required constant attention--cleaning, saline flushes and heparin flushes. My line developed an infection and blood clots. I still remember vividly the day I was able to get the line pulled. Hallelujah! That nightmare is over!

Perhaps getting a trach might be overwhelming for some folks, but for me, the few extra minutes it takes to care for my trach is nothing compared to the tremendous aid the trach provides in helping me breathe. Praise be to God for the tracheostomy procedure and invasive ventilation. It is such a blessing to be able to breathe with ease! "My mouth is filled with Your praise, declaring Your splendor all day long" (Psalm 71:8).

Adventures in God's Garden

A few weeks ago, my parents bought a hummingbird feeder and a poll to mount the feeder. Unfortunately when they set up the feeder outside my window, it was too windy. The feeder whips back and forth in the wind, spilling the liquid bird food. The bird feeder was taken down and placed on a chair on the patio. Today as I was lying in bed, I was gazing out the patio window. Suddenly, I saw what appeared to be a dragonfly pause in front of the window and then fly toward the bird feeder. I thought to myself, "My! That was an odd looking dragonfly." I suddenly realized, "No! That was a hummingbird coming to get a drink from the bird feeder! Yahoo! How exciting!!!"

A little while later, I needed to go outside to the front pouch to check on my mom's plants. Upon entering the pouch, I heard what sounded like a tiny weed eater a short distance from me near the garage. I looked and there flying around some flowers was a hummingbird! Wow! Praise be to God!

As I was surveying the plants, my heart sank when I turned to one potter. My mom had planted some squash seeds in a planter with store bought soil. One beautiful plant came up and thrived. Unfortunately, the soil was infested with gnat eggs. Very soon there was an infestation of thousands of gnats. My mom tried everything to get rid of the gnats, but there were simply too many of them. She bought new soil and transplanted the plant. Unfortunately, the plant did not survive the move. It shriveled up and died within a few days. Despite this, my mom said to keep watering the plant until it was completely brown and decayed. I kept watering it, but yesterday, it was evident the plant was very dead. Today as I looked mournfully at the planter, I suddenly saw a healthy young seedling sprouting in the planter. Can this be!? Yes! A new squash plant is growing! Thank you Lord! What an incredible blessing and great gift for my mom! She will be so excited!

Next to the squash planter, there is a beautiful basket planter filled with lots of different flowers. Yesterday was quite warm. When I checked on the plants, this basket of flowers was very wilted and looked as though it was at death's doorstep. I gave the planter a big drink of water and prayed the flowers would come back to life. Today, as I gazed at the basket, I was filled with delight. The basket looked GORGEOUS!!! The flowers were perky and vibrant in color. Was this the same basket of flowers!? Thank you Jesus for bringing the flowers back to life!

"I planted, Apollos watered, but God was causing the growth. So then neither the one who plants nor the one who waters is anything, but God who causes the growth" (1 Corinthians 3:6-7).