It's Here!!! The Happiest Day of the Year!

After eagerly waiting for this day to come, now the sun was descending toward the western horizon. In a few short hours, the sun would set, and it would be Yom Kippur! The phone rings. My mom answers it and talks to the person. When she hangs up the phone, she enters my room. "That was the infusion company. They got everything approved. They are sending out the antibiotic tonight. It will be here in the morning. When it arrives, we need to call the home health nurse. She needs to come and administer the first dose to you."

Upon hearing this, my heart sinks. My mind cries out, "No! NO! This is not good! Tomorrow is Yom Kippur. It is a Shabbat. It is a day of rest. No one is supposed to do work for me. Sending out the drug tonight means people will have to work to drive my package to a sorting facility. In the morning, a delivery person will have to drive my package to my house. Moreover, calling my home health nurse means she too will have to work when she drives to my house and administers the drug. Oh this is not good! This is not good at all! Having other people work for me means I will be breaking Shabbat."

I am too heart-broken to say anything. My mom leaves. I pray to God that if this is not supposed to happen, my package delivery will be delayed until after Yom Kippur. When the sunsets, I watch a live streaming Yom Kippur service. I try to allow the beautiful service to soothe my broken heart.

In the morning, I sleep long. When I get up, it is around 11 a.m. My antibiotics have already been delivered to the house. I open the box and fight back tears when I see the medicine neatly arranged inside. My mom tells me she has called my home health nurse. She is going to be here in the afternoon. I place the medicine in the refrigerator. I then go back to bed and watch more live streaming Yom Kippur services.

Shortly before 2 p.m., I make my way to the living room. I sit on the couch and continually apologize to God for making this day, which is the holiest day of the year, just as common and ordinary as any other day of the year. I apologize for making people work for me on this day.

When my home health nurse arrives, she conducts her usual routine. She takes my vitals, checks my PICC line and asks me a standard series of questions. When she completes everything, she takes one container of antibiotic out of the fridge. She primes the pump and tubing. Soon, she starts infusing the drug into my PICC line.

Very quickly, I am not feeling well. I develop a headache which continues to intensify. My breathing grows labored. I cannot sit up any more. I slouch to the side. I feel impending doom like the world is ending. My home nurse is busy filling out paperwork. My mom notices my abrupt change in health. My mom alerts the nurse. The nurse talks to me. My tongue is thick. It is terribly difficult to speak. My nurse grabs the emergency medicine which came with the antibiotic. She prepares the Benadryl and epinephrine. She administers both drugs. She then calls 9-1-1.

A short time later, the ambulance shows up at the front door. Thankfully, the Benadryl and epinephrine have worked their magic. I am starting to feel a little bit better. I can now speak short sentences. The emergency medical workers help me on to a stretcher. I am then loaded up into the ambulance and taken to the hospital. As we make the long drive, I continually apologize to God. "Oh, I am so sorry. I have continually violated this day. Now, I am making two more people work, and when I arrived at the hospital, I am going to be making medical personnel at the hospital work too. Oh, I have really messed up this day!" I am grateful the anaphylactic reaction along with the sedative effects of the Bendryl make it very difficult for me to move or exhibit reactions. The tears welling up in my heart never flow from my eyes.

When I arrive at the hospital, God arranges for a very kind physician and amazing nurse to tend to me. The doctor runs some tests. My mom informs the physician she just wants to make sure I am stable. She then is going to drive me nearly 400 miles to the medical facility where my doctors are located. The doctor agrees to her plan. Soon, the physician gives the green light that I can leave. I am grateful to be leaving the hospital...but I plead with my mom to not take me to another medical facility. "I am fine. Let's just call my doctor in the morning." I desperately want to get home; I want to at least try to salvage just a little bit of this happiest day of the year. But, my mom insists my physician said if I had a reaction to the antibiotic, I should come to the hospital.

I am shocked when we arrive home; my mom has almost everything I need for the journey already packed up. While we were waiting for the ambulance, she began to pack. She continued to frantically pack while I was loaded up and taken to the hospital. She then was able to arrive at the hospital very shortly after I had been examined by the emergency department physician. Seeing all the work my mom had done, I reluctantly decide to make the long journey to the medical center.

As we begin our trip, we drive westward. The sun is just above the horizon. It is still Yom Kippur. I apologize again to God for breaking Shabbat. I watch as the sun's rays sink lower and lower below the horizon--such a beautiful sunset! I continue to watch as the last light grows paler and paler. Quickly, the light fades, and the darkness takes over. Yom Kippur has ended.

This was supposed to have been the happiest day of the year. Quite frankly, it ended up to be the saddest day of the year. For the next year, this single day plagues my soul with guilt and sorrow. For one year, I have been asking God to allow this next Yom Kippur to be one dedicated to Him. Today,  at sunset, begins Yom Kippur. As much as I wish things would be different from last year, they remain very much the same. I am still fighting off Pseudomonas. I have another PICC line. I am again on IV antibiotics. I have asked God for nothing to disrupt this day. I am praying this year, this Yom Kippur, it truly will be the happiest day of the year.

(For the link to Part One click here)

The Road to...The Happiest Day of the Year

As one passes from day to day, it is hard to imagine how one decision can cause a cascade of events to occur, which continues for many weeks, months and even years to come. Last year, one small decision, which seemed rather insignificant at the time, has dramatically changed my life.

On July 10, 2018, I underwent a procedure called an ERCP. This entailed a scoped being placed down my throat, through my stomach and into my small intestine. A small, flexible scope was then extended up my duct and into my pancreas. A large cyst was found and drained. Before the procedure began, I was informed I would have to be placed on the hospital ventilator. Due to having issues in the past using hospital ventilators, I am always leery about turning off my ventilator and switching to a foreign machine. The anesthesiologist was extremely kind and very knowledgeable about ventilators. I trusted her; I allowed her to switch me to the hospital ventilator.

Within a week of having the procedure done, I noticed my sputum had changed. It now had a fluorescent hue to it along with a sickly sweet smell and taste. My heart sunk. I knew I had acquired another respiratory infection. I was fairly confident it was from the hospital ventilator and was a bacteria called Pseudomonas

A few weeks later, I went to the emergency department and was hospitalized. I was sick, but I had a clear chest x-ray, normal white blood cell counts and a low grade fever. A sputum culture was ordered. It came back positive for Pseudomonas. When the doctor informed me of the results, I was happy. See, I am sick. This is a new bacteria. I will be given antibiotics and soon be on the road to better health. The physician, however, believed differently. "This is a colonization. You do not have an active infection." I interjected, "But wait! Colonization means the bacteria is a normal part of my respiratory tract. I have NEVER had Pseudomonas in my sputum before. If I am colonized with Pseudomonas, I would have had it show up in my previous sputum cultures. I am not feeling well. Surely this is the causative agent." The physician dismissed my claim and said, "You do not have an active infection. You are not sick." The medical professional dismissed me home without any antibiotics.

Two weeks later, I was slated to undergo IVIG (intravenous immunoglobulin) treatment. I tried to come up with a reason why I should not have the IVIG, but nothing seemed to contraindicate the IVIG. Within 20 minutes of the IVIG being infused, my mast cells rejected the treatment. I developed an anaphylactic reaction. I was wheeled down the hall to the emergency department. I was given immuno-suppressant drugs and admitted to the ICU. In a few days, my health stabilized. I was given my last dose of immuno-suppressants around 11 a.m. I was discharged home in the afternoon. In the evening, I noticed I was very fatigued. I thought I was just tired from spending several days in the hospital.

Around 11 p.m. that night, I abruptly became extremely ill--violent shaking chills, high fever, confusion, nausea, pain throughout my body, etc. (For more details, click here) I struggled through the night. In the morning, my parents drove me to the large medical facility nearly 400 miles from our home. Once in the emergency department, I am informed I am being admitted to the ICU. I had a large hole in my lung due to Pseudomonas. During my stay in the ICU, the exact same doctor I had just two and a half weeks previously, who said my Pseudomonas infection was just a colonization, severely scorned me for having IVIG. "You have an active infection. IVIG weakens your immune system. You should have known better..." I really wanted to scream out, "Wait! You just told me 2.5 weeks ago I did not have an active infection!" But I refrained from pointing out the physician's error.

I spent 12 days in the hospital and acquired two additional bacteria. After a brief period of feeling better, I began to feel quite ill. Upon discharge, I was running a fever. My white blood cell count was elevated. I was coughing up copious amounts of blood. My doctor prescribed IV antibiotics; they did not seem to be working. I suffered through four days of worsening health. I finally decided to go to a local emergency department. My chest x-ray revealed the Pseudomonas was all over my lungs. I was admitted and spent more days in the ICU. Upon being released home, I am put back under the care of my doctor 400 miles away.

Since I was admitted to the hospital, his prescription for IV antibiotics was made void. My doctor decides to place me on a relatively newly approved drug. He submits the paperwork to the infusion company. I have to wait for them to process everything and get insurance approval. In the meantime, I look at the calendar. In two days, it will be Yom Kippur (the day of atonement). This is supposed to be the most joyous day of the year. After having a very turbulent summer and now fall, I wait eagerly for that glorious day, the tenth day of the seventh month on God's calendar. Oh, the day is going to be filled with lots of singing, prayer and happiness. I can hardly wait for the day to come!

(For the link to Part Two click here)

Saying so long to the 70’s

As I watch Rosh Hashanah services, I laugh when a rabbi says, “Say good bye to the 70’s. The 80’s are here.” This, of course is in reference to the start of God’s new year, which is the year 5780. The last ten years have been the 5770’s.

As I reflect back on the last ten years, it is amazing to review all the life changing situations which have arisen. Ten years ago, I was fighting to hold on to my life. My only diagnosis at the time was postural orthostatic tachycardia syndrome. I was assured if I made some lifestyle changes—a high salt diet, beta blockers and compression stockings—I would be on the road to better health. When I was given a medicine to help with my daily migraines, I suffered a stroke. It took several months to regain muscle strength and coordination. Despite my best efforts, my health continued to decline. I tried and tried to do everything possible to feel better, but the harder I tried, the sicker I became. I finally had to give up everything and go live with my parents.

For the next year I was bedridden. Any time I stood up, I blacked out. I often resorted to crawling on my stomach to the bathroom. The search for answers yielded nothing. My gastrointestinal tract was the next victim. My gall bladder became inflamed, infected and nearly ruptured before it was removed. My intestines stopped working. I first had a jejunum (intestinal) feeding tube. When that failed, I next had a PICC line and received IV hydration and nutrition. I was so grateful during this time, God lead me to the condition called Mitochondrial Disease. Now I finally knew what was wrong with me. The heartbreaking part was there is no cure. One can use supplements and herbs to support the mitochondria, but nothing will ever stop the disease. It is a chronic condition which will mostly likely continue to progress.

Skipping over the next five years which were filled with bowel obstructions, another stroke, continued muscle weakness and pancreatitis attacks, I suddenly developed difficulty breathing. I was assured it was just asthma. Two weeks later when I was in the emergency department in acute respiratory distress, I was told I needed to use a bilevel postive airway pressure (BiPAP) machine to help me breathe. Very quickly my breathing deteriorated. I soon was on death’s doorstep, desperately needing a tracheostomy and invasive ventilation. Thankfully, God moved a few mountains, and I was able to receive a tracheotomy and was started on invasive ventilation without many complications.

And that’s where my life has been for the last several years. I have continued to fight pancreatitis attacks, have had many anaphylactic reactions and have been plagued with nine or ten hospital acquired lung infections. I am still fighting one of those bacteria called pseudomonas which has kept me in the hospital numerous times this last year. (As I write this, IV antibiotics are being administered via an IV drip into my PICC line in hopes of finally eradicating this extremely resistant bug.)

The 70’s have been a time of great trial. I am praying the 80’s will be a time of great rejoicing, no admissions to the hospital and stable health. May 5780 be our best year yet! Shana tova (Happy New Year)!

Perspective: Height

Since I have been a child, I have always been tall. People constantly assumed I was older than what I was. When my parents would ask for the children’s menu or the kid’s entrance price, the employee would sneer at them and claim I was too old, even though I was only 5 or 6 years old. When I was 8 years old, I went trick or treating with my friend. Neighbor after neighbor told me I was too old for this event. Really? Eight years old was too old? But no one ever believed me when I told them my age.

After becoming severely sick many years ago, I needed to use a wheelchair for mobility. Suddenly, I appeared short. Instantly, people guessed I was much, MUCH younger than what I was. When I was approaching my 29th Birthday, a woman stopped me after I exited the car and told me I was too young to drive. She thought I was 15 years old! There have been many other instances such as this. A few months ago, a nurse thought I was 17 years younger than what I am. It simply amazes me that a person’s height has so much influence on people’s perception of age.

There is a different side to height as well. Tall people are often assumed to be more knowledgeable than short people. Before needing a wheelchair, strangers assumed I was in a management position at my job. If a dispute arose, customers would often seek me out because I looked like I was the person in charge. (The true leadership was significantly shorter than I. Even though their name badge indicated their position of authority, people would still come to me for assistance.)

Once I started using a wheelchair, I instantly found people guessed I had low level intelligence. Strangers would talk to me like I was a three year old. They would use exaggerated hand gestures and overly cheerful expressions. “Oh, isn’t it such a wonderful day. And YOU! You are so wonderful and special too!” It often is annoying, but I have learned to just smile and reply, “Yes, it certainly is a lovely day.”

The most amusing part about using a wheelchair is seeing people’s expressions when I stand up. Quickly, my appearance changes from one of being short into one of being tall. Often the person witnessing this metamorphosis stares at me in shock and awe. The person usually mutters, “WOW! You are so tall!” (When I was a very young child, I had a step stool I used to reach the bathroom faucet. It read, “Step up to be tall. Sit down to be small.” This phrase usually runs through my mind during times like this.)

For what it is worth, it is a blessing to be tall and use a wheelchair. Few people have the opportunity to view the world from both perspectives and know a person’s height has nothing to do with his age or level of knowledge.

Brave

For the last two years, a small boy has been fighting cancer. Many say the young child has courage and exhibits bravery. By his side, through the countless hospitalizations, therapies, blood draws, nuclear scans, doctor's appointments, etc., a stoic mother bravely holds her son. Her heart swells when given good news. "Your child is NED (no evidence of disease)!" She dances. She sings. She celebrates. When the nuclear scans show the return of the cancer, her heart breaks. She cries. She prays. She hugs her son.

As I watch this mother ride the highs and lows of having a loved one sick, I realize she is the one who is brave. She is the one who has courage. When her world is falling apart, she still has to push through to get her son to his medical appointments. She has to comfort her child when he is in pain. She has to endure not only her own emotions, but she must continue being a mother.

I have watched many of my friends fight their battles with various medical conditions. I know from my own experience, it is a struggle to go through so many tests and procedures. However, the patient is often lucky. When undergoing surgery, anesthesia allows the patient to slip off to sleep while the minutes or hours tick by. The family member(s) standing by in the waiting room must live through the experience in real time. If there is a complication or the procedure takes longer than expected, it is their hearts and minds which must struggle to find composure. They are the ones who must find strength to idly sit by.

When the patient recovers from an illness or surgery, the family members are the ones who often have to find the time and energy to encourage, assist and support the patient. Although recovery is not a walk in the park, I often feel it is much easier than trying to live one's own life and also have to take care of another person.

So, as my sweet little friend Lincoln has left this world to enter eternity, I think about his mother. I think about his father. They must continue forth in this world without their firstborn son. They must continue being husband and wife. They must continue being a parent to their son, Bridger.

When I think about those who are brave, I think about all the family members who assist their loved ones through various trials in life. May we celebrate and strengthen each other as we support one another in kindness and love.

"Be strong and let your heart take courage, all you who hope in the LORD." (Psalm 31:24)

From surrounded by sewage to singing hallelujah, what a joyful miracle!!!

I often tease my parents, "When the cats are away, the house falls apart." It seems every time my parents go away on a trip something happens to our house. Examples include the dishwasher breaking and leaking water all over the kitchen, ants invading our house, the garage door malfunctioning, a swarm of thousands of bees taking up residence in our home, etc., etc. My parents often deny anything out of the ordinary happens whilst they see the world. I, however, beg to differ.

On Sunday, I notice my toilet is making odd sounds every time I flush it. Sunday evening, after I flush it, I suddenly hear a LOUD explosion! (Thankfully, I had just used the toilet, or I am pretty sure I would have had an accident!) I open up the toilet lid and see the toilet did not flush. I retrieve the plunger and start plunging away. Nothing seems to happen. I look over to my shower, and to my horror, sewage is bubbling up from the drain! There is about 2 inches of murky water covering my shower floor. I am concerned at this point. But, not knowing what else to do, I leave the bathroom. A half hour later, I return to the bathroom. There is still sewage in the shower, and now there is water leaking from the base of my toilet and is all over my bathroom floor! I grab a mop and bucket and start mopping up the water.

When the urge next arises, I decide to use my parents' bathroom. Thankfully, that toilet flushes. It appears that sewage is not backing up into my bathroom. However, I still use their bathroom sparingly as I do not want anything to add to the sewage mess in my bathroom. The sewage from the rest of the house seems to be draining directly into my bathroom. Any time I use the kitchen sink, all that water finds its way to my shower floor.

Henceforth, I cannot use the dishwasher. I cannot use the clothes washer. I cannot rinse any dishes. I cannot dump anything down any of the sinks. Additionally, the sewage is really smelling up my bathroom...and the bathroom is adjacent to my room. I have to keep the bathroom door closed and run the exhaust fan in an attempt to keep the sewage smell from entering my room.

I reuse dishes and try to keep my clothes clean to avoid accumulating laundry. My dad said he will look at the problem when he returns from his trip...he will be home the end of September.

I try to keep a positive attitude and try to stay on top of the cleanup from the water which seems to constantly be leaking from my toilet. I use an empty 32 ounce yogurt container to scoop water out of my shower and into a bucket to prevent the sewage from breaching the shower and cascading on to my bathroom floor. Between mopping the floor and collecting water from my shower, I haul about 100 gallons of sewage out of the house and dump it into the grass. My arms ache, and my hands are red and blistered.

Almost exactly 72 hours after the sewage pipe clogs, it instantly opens up again! I am in the bathroom scooping out water from the shower into a bucket. Suddenly, I hear the toilet make an add sound and then it flushes. I then look to my shower. Immediately, the sewage makes a mad dash for the shower drain. In about 10 seconds, my shower is sewage free! I am in disbelief! I am trying to send praises up to God, but my shock from the entire situation leaves my brain in a jumbled mess. I reach over to turn on the shower. I watch with glee as the water flows freely down the drain. Next, I flush the toilet. I have NEVER been so thrilled to watch toilet bowl water effortless leave my toilet and clean water instantly reappear.

My friends, I do not know if any of y'all have ever had sewage issues in your life, but for me, this experience makes me extremely grateful I live in this country at this time in history. What a glorious invention indoor plumbing is. What a kind, caring and merciful God we serve!

"Shout joyfully to the LORD, all the earth.
Serve the LORD with gladness;
Come before Him with joyful singing.
Know that the LORD Himself is God;
It is He who has made us, and not we ourselves;
We are His people and the sheep of His pasture.
Enter His gates with thanksgiving
And His courts with praise.
Give thanks to Him, bless His name.
For the LORD is good;
His lovingkindness is everlasting
And His faithfulness to all generations."
(Psalm 100)

Mitochondrial Disease Awareness

What are mitochondria?

Mitochondria are tiny powerhouses located in every cell in the body except red blood cells. They are responsible for producing approximately 90% of the energy needed to sustain life. Mitochondria are unique. They have their own DNA. Unlike regular DNA which is inherited from the mother and father, mitochondrial DNA is only inherited from the mother.

Many believe since mitochondria make their own DNA, it is only the mitochondrion's DNA which creates disease. This is not true. It takes about 3,000 genes to make a mitochondrion. Only 37 of those genes are encoded by the mitochondrion's DNA. The other genes needed to make a mitochondrion are encoded for by the cell's nucleus and exported to the mitochondria. Thus, mutations in either the cell's DNA (inherited from the mother and father) or the mitochondrion's DNA (inherited only from the mother) can cause mitochondrial disease. Thus, Mitochondrial Disease can be passed down from one's mother, father or both parents.

What is Mitochondrial Disease?
When mitochondria begin to fail, the body does its best to conserve energy. High energy demanding organs such as the brain, gastrointestinal tract, heart and muscles are the most affected systems. Symptoms arising from decreased energy to these organs include seizures, strokes, heart arrhythmias, an inability to properly digest food, difficulty walking, etc. As more and more mitochondria fail to produce energy, symptoms increase in severity. If enough mitochondria fail in one organ system, organ failure can occur.


Mitochondrial Disease is extremely difficult to diagnose since it presents differently in every person. People with the same gene mutations may vary from having no symptoms to being bed ridden and on life support. Mitochondrial Disease is often suspected when three or more organ systems are involved.

Mitochondrial Disease is not one specific condition. Rather, it is a general term applied to thousands of various medical illnesses. Some of the specific illnesses have names such as MELAS (myopathy, encephalopathy, lactic acidosis and stroke-like episodes), CPEO (chronic progressive external ophthalmoplegia), Leigh's Disease, etc. Other conditions are extremely rare and have no names. They are simply referred to by the genetic mutation which causes the disease.

Mitochondrial Disease Facts
Mitochondrial Disease is a chronic (long-lasting) and progressive disease. There are no cures for this illness. There are supplements such as carnitine, Co-Q 10, vitamins, minerals and herbs which help support the body. However, there is nothing which can prevent the progression of the disease.

Approximately one in 5,000 people have a Mitochondrial Disease.

Every year, more children die from Mitochondrial Disease than from all childhood cancers combined.

Although Mitochondrial Disease is most often seen in children, adult onset is becoming more and more common. This is partly due to the increased awareness about Mitochondrial Disease. Many adults in the past, who have had non-specific symptoms affecting multiple organ systems, were often told they were making up their condition or were simply cast off into other generalized conditions such as chronic fatigue syndrome and fibromyalgia. As more and more in the medical community are recognizing Mitochondrial Disease as a medical condition (and the internet makes it possible for patients to do their own research), Mitochondrial Disease is being diagnosed more frequently in the adult population.

The Future
Although there are no cures for Mitochondrial Disease, the future does look promising. Children and adults are being diagnosed sooner with Mitochondrial Disease which means symptoms can be detected and treated faster. Supplements have made a huge improvement in slowing the progression for many folks with Mitochondrial Disease. Also, advances in other areas of medicine such as portable home ventilators, home IV fluids, IV nutrition (TPN), feeding tubes, etc., have made it possible for many with Mitochondrial Disease to continue living.

Will there ever be a cure? Only God knows. Until then, may we raise awareness, support one another and try our hardest to make each day our best day yet.