Easter Edition

1 Peter 1:1 "Praise be to the God and Father of our Lord Jesus Christ! In his great mercy he has given us new birth into a living hope through the resurrection of Jesus Christ from the dead..."

 

Spring is here! We enjoyed watching our bluebirds busily nesting in their boxes, laying eggs.  Now we are enjoying the five baby birds chirping at the top of their lungs when mama bird brings a bug. The cool spring temperatures combined with the rain has the flowers dressed and ready in vibrant colors. Spring is a time when every creature, every living thing is getting ready, dressed up in its best form for what is to come...it's a time of celebration of new births, new life, new hopes. As we approach Easter, it is much that way with us. We are given a new birth also through the resurrection of Jesus Christ and we are given an inheritance that is being kept in heaven for us that can never perish, spoil or fade . Oh what joy, hope and comfort that brings to my heart! It sets the tone as I prepare my heart and mind for our Easter celebration. Jesus is alive, lives amongst us, and is still in the business of performing miracles.

 

Our journey with VHL continues and praise God we have more great news to report! My husband went for his routine 3 month bloodwork check and visit with the hematologist this past week. Since his polycythemia diagnosis several years ago, his hemaglobin levels stay high (around 19-20) so blood has to be removed every few months to reduce the numbers (to around 14). The current blood work revealed the number at 12 with no blood being removed over the past year. His doctor was very encouraged and extended his next check to 6 months!

 

So, as we approach our celebration of the risen living Christ, we thank him for the ULTIMATE sacrifice he made for us on the cross, but we also thank him for walking with us daily and showing himself mightily! Hallalujah! Celebrate! The grave is empty- HE is alive!

Thanksgiving Edition

Psalm 71 (NIV) 'do not forsake me when my strength is gone'

With Thanksgiving in just a few days, we tend to be more mindful and thankful for the blessings God has given us.  This Thanksgiving is an extraordinarily special one for us. It marks the one year anniversary of a procedure that now allows Gaston a much better quality of life.

Several years ago, Gaston began having pain, nausea and discomfort so severe that we found ourselves in the ER numerous times. Each time the diagnosis was pancreatitis, the doctor would give him medications for the pain to subside, hydrate him and dismiss him. Strangely though, each time the test results would show elevated liver enzymes which is unrelated to the pancreas. God put it on Gastons' heart to see a local gastroenterologist who specializes in liver problems to get his opinion.The physician was convinced it was related to the bile duct being partially blocked considering his major whipple surgery. This doctor had just returned from Mayo clinic training on how to use a scope for liver procedures traditionally done by surgery. What a blessing that would be if we could avoid more surgery!  We were encouraged!  Since Gaston had endured so many procedures in the past and was currently having no problems, he decided to wait.

At this point in our Christian walk, Gaston had been involved for many years in a prison ministry in which he is actively involved today.  Several months after seeing the doctor, Gaston was away for a prison ministry weekend and began to have the symptoms of an attack early that morning. At breakfast, the group prayed for him and his symptoms subsided. I went to the prison that evening for the closing ceremonies. When I first saw Gaston, he had a different color to his skin and appeared more pale and weak.  He told me what happened earlier in the day.  He said he felt better other than the typical fatigue brought on by all the weekend entails for them while getting very little sleep. After the closing, we came home and he became violently ill. We headed straight for the ER.

Here is where more blessings than we could ever imagine started to happen: 

Thank God Gaston had been to see the liver specialist since he was called immediately rather than the on-call physician who would have been untrained in using a scope versus surgery. For a patient without adrenal glands, it's very dangerous to be sedated for any type of procedure for any length of time much less a surgery of that magnitude. Gaston was admitted and tests showed scar tissue that had formed over time where the intestines were sewn back to the liver during the whipple procedure.  The scar tissue was causing complete blockage of a bile duct. The bile had no where to go except be released in his body causing him to be so sick. He was given IV antibiotics to prevent infection, medicine for vomiting and nausea and had to recover for a day before anything could be done. At this point, we had no idea if the complicated formation of Gastons' intestines would allow a scope to pass through and if we still were facing major surgery. I spent alot of time in scripture, talking with God, longing to hear from Him, and keeping my ears open. 

Early one morning as I drove back to the hospital, I was at the end of my own strength.  I needed a strong word from God, and I needed it badly. About that time the Christian radio station I was listening to played the Lincoln Brewster song "Everlasting God".  It recalled one of my favorite verses that I love dearly and read frequently. It brought me such comfort that I never knew was possible in such a situation.

Isaiah Chapter 40: 29-31 (NIV) says "he gives strength to the weary and increases the power of the weak. Even youths grow tired and weary, and young men stumble and fall; but those who hope in the Lord will renew their strength. They will soar on wings like eagles, they will run and not grow weary, they will walk and not faint."

 I had such a peace come over me that I KNEW it was going to be ok no matter what we were about to endure. Later that day, would be the first procedure.

After I had Gaston secured and sedated in the OR, I went to the cafeteria to grab a bite to eat while I waited. When I walked in, there stood the hospital head chef (who normally is in the kitchen at lunchtime). He happened to be a good friend of ours and ironically had served that weekend with Gaston on the same prison ministry team! God placed him there at that time at that very moment knowing how much I needed his prayers and hugs.  

• Day 1 - The first scope was unsuccessful in that the physician couldn't locate the duct due to the  malformation of Gaston's intestines so he was referred to a radiologist.
• Day 2 - It is back to the OR so that the radiologist could inject dye during a procedure to locate the blockage. This time it was located and 'tattooed' with ink so that when the permanent procedure was done it would be much easier to find. Also if the duct starts closing or has other problems in the future, the location will be permanently marked. A drain was installed for the rest of the bile to drain and to help the liver recover somewhat before the next procedure in a couple of days.
• Day 3 - Finally, a day of rest for Gaston.
• Day 4 - Another scope to remove the drain and place a plastic stent in the duct to hold it open allowing bile to flow and to allow the liver to continue to recover.
• Day 5 - We were discharged from the hospital with instructions to return as an outpatient in 6 weeks for the stent to be removed and for the procedure on the liver duct. 

Gaston did well with the stent and began to return to full health; however, the stent started causing some problems.  The liver specialist wanted to remove it and do the permanent procedure a week earlier than planned--the day before Thanksgiving. He explained to me that the procedure may be lengthy and he would also prepare Gaston for surgery in the event the scope couldn't attain the angle he needed to perform the sphincterotomy (stretching and cutting the muscle of the duct to prevent it from closing again). The procedure began.  I prepared myself for what the surgeon said would be a long wait and I began praying. After a short few minutes, the door opened. I would have been very concerned if the surgeon didn't have a huge smile on his face. The procedure was done much quicker, was less complicated than he had planned, and Gaston was returning to the recovery room!

God had his hand on that doctor guiding him to work quickly and efficiently with the least amount of stress to Gaston's body. As the different procedures were being performed, I will never forget such a Holy presence so intense during my wait.  It felt like Jesus himself was breathing on my neck.  He was so close, watching over us during this time. 2 Corinthians 12:10 says, "for when I am weak then I am strong". I lived this out that week. My own strength would have left me an emotional wreck.  By clinging to Christ, He lifted me up and He gave me the strength and the peace I needed to take care of Gaston.

So this Thanksgiving marks one year that Gaston has had no problems and is living an active, healthy life. We just had yearly checks of his kidneys and retinas and I am proud to report they are clear! As God continues to do great things in our journey with VHL, I will be back to share more with you.

Psalm 71:14 (NIV) " But as for me, I will always have hope, I will praise you more and more."

Part V

2 CORINTHIANS 12:8-10 (NIV) "Three times I pleaded with the Lord to take it away from me. But he said to me, 'My grace is sufficient for you, for my power is made perfect in weakness.' Therefore, I will boast all the more gladly about my weaknesses so that Christ's power may rest on me. That is why, for Christ's sake, I delight...For when I am weak, then I am strong."


After the diagnosis of VHL, we read any articles we could find to educate ourselves about the disease. We found out that the type of tumor removed from Gaston's pancreas typically stayed unchanged and benign so the whipple procedure had been unnecessary. In the surgeon's defense, he was doing what he was trained to do and removing what he thought at the time was a malignant tumor. Like the apostle Paul, God didn't remove Gaston's "thorn."  Instead, He used it to move us both much closer to Him in our obedience. God used this to teach us to fully rely on Him and never doubt His ways. Yes, we could have become bitter and angry with God.  However, we knew that we had solely trusted Him in the past.  In return, we received a peace that we could never feel through trusting anything other than God to get us through. A peace that passes all understanding.  So at this point, we knew that without some trials and weaknesses, we could never really grasp the power that Jesus made available to us.  Power that comes only from the firey trials we had faced and inevitably would face in our faith walk.

The evidence of our training became apparent in the year of 2005.  We celebrated our 15th wedding anniversary with an Alaskan cruise. We were at the airport excitedly waiting to board the plane when we received a call from Gaston's doctor.  The grim news delivered was that the annual CAT scan revealed a mass on the spinal cord. No further explanation or details could be given until the radiologist could take a closer look at the scan. Without God's grace and mercy, we would have spent our trip full of anxiety and fear. On the contrary, we both had such a calmness and assurance within us that we knew could only come through the Holy Spirit living in us. We went on our trip without much thought of the news we had just received. Upon our return, Gaston talked with the neurosurgeon who was in charge of the tests. The tests revealed a small hemangioblastoma (a benign tumor characteristic to VHL).  He explained that every other year a follow-up MRI would be necessary to monitor the growth. It has been slow growing since the diagnosis five years ago to present time.

This past summer, through the VHL.org website, we found out about a neurosurgeon at MD Anderson in Houston who is very well versed in these types of spinal tumors in VHL patients. The surgeon has removed many of these specific tumors on the spine. This past June, we went for an appointment with him for a second opinion and review of what our local neurosurgeon found.  We came home very encouraged!  We thank God for putting this doctor in our path. He warned us of the seriousness of the removal of this type of tumor.  Extreme caution must be used because these tumors are formed from many blood vessels.  As you might imagine, many blood vessels signifies a very bloody removal. Such a removal could easily become a surgery nightmare lest the issue is addressed going into the procedure. "Addressing the issue" would be accomplished by the neurosurgeon making an incision and freezing the tumor.  Then, the tumor is removed by chipping away the frozen tissue, thus causing less trauma to the area while controlling the bleeding. Should the tumor grow to a symptomatic size, the surgeon agreed to do the surgery.  Symptoms might include numbness of hands or feet or a tingly sensation in the arms and legs much like electricity. We continue to pray that God will keep the tumor unchanged and stop its growth.

We are constantly reminded as we battle VHL that we are not immortal and that we are totally dependent on God alone. We have also been greatly humbled through this experience.  We are reminded that we should 'consider it pure joy'...whenever we face trials of many kinds because this testing of our faith develops perseverance.  'Perseverance must finish its work' so that we may be mature'.  (James 1:2-4 NIV)  God doesn't allow circumstances to make us miserable.  He knew us before we were born and He knows what it takes to draw us close to Him. So, with that said, I praise Him and give all thanks to Him for the trials He has put before us. Otherwise, we may not have gotten the privilege to know Him as our personal Lord and Savior.

Part IV - No Small Miracle

James 4:8 NIV "When we draw near to God He draws near to us."

In the year 2000, Gaston's endocrinologist had enough medical pieces to the puzzle to suspect Von Hippel Lindau (VHL) disease. Genetic testing confirmed it. Now we knew we were dealing with (at that time) a very rare disease with little information published . Since VHL is inherited, Gastons' mother, father and two brothers also had to be tested. All tested negative so apparently Gaston is the one it begins with in his family tree.

At this point, we began a tremendous amount of Internet research and found what we could about VHL, the best way to treat it, and where else in the body to screen for other tumors. This is when we located the website, vhl.org . It became a very valuable tool for us as well as Gastons' team of doctors (which at this point consisted of a primary care physician, an endocrinologist, a urologist, and a neurosurgeon). Through this website, we found out the tumors can show up in the retinas of the eyes and in the hearing canals.  So, a retina specialist and an ENT (ear, nose, throat) came on board with the rest of the medical team.

Additionally, we found out VHL patients have a 75% risk of developing kidney cancer so cat scans of both kidneys are done annually. In 2002,Gaston's annual cat scan showed a suspicious spot on one of his kidneys. That night at church, our friends gathered around us, placed hands on Gaston, and asked God if He would in His great mercy heal Gaston. A few days later when a follow up ultrasound was done to determine what treatment would be necessary, there was nothing there! The doctor was baffled as he looked at the cat scan that earlier showed a spot. We weren't baffled.  We knew exactly what had happened. God restored full health to that kidney. He had performed a modern day miracle!  The miracle showed us loud and clear He had a plan for Gaston's life. Gaston had not completed his mission here on earth just yet.

Our next hurdle with VHL came a couple of years later when blood tests revealed elevated hemoglobin levels. Polycythemia was the diagnosis. This is when the gene that determines when the bone marrow has produced enough red blood cells becomes defective. Bottom line: Gaston's body no longer knows when to quit making blood. At this point, a hematologist joined the ever-growing team of doctors. Gaston sees him every three months for blood work.  When the hemoglobin levels reach a certain level, a phlebotomy (a large needle is placed in his arm and excess blood is removed) is performed. What a blessing that this too can be done in a manner not so invasive. The danger of too much blood in the body is that the blood thickens which can cause blood clots, high blood pressure, stroke and even heart attack.

As God tells us in Jeremiah 29:11 (NIV) "I know the plans I have for you says the Lord. They are plans for good and not for disaster, to give you a hope and a future." Yes, Gaston and I have times of emotional trials with the ugliness of this disease.  Sometimes, we question "Why us, Lord?" However, we have fully trusted God to lead us and he has not let us down so we will continue to be obedient servants. Gaston's disease has allowed us the privilege of experiencing Jesus Christ on a closer, more intimate level than we ever thought possible.

I have more miles to cover in our journey and I hope you will continue the walk with me. I also pray that anyone going through an illness or whatever situation you might be in will find some comfort in traveling with us....

Part III

DeAnn Arnold Photography

Romans 5:8 (NIV) "But God demonstrates his own love for us in this: While we were still sinners, Christ died for us."

Following the surgery, there were still more issues to be resolved. Some issues resulted from the absence of adrenal glands.  Doctors had to find the correct dosage of the replacement hormone that adrenal glands normally produce- our bodies use adrenaline in times of stress and illness. During the process of finding the appropriate amount of prednisone that agreed with his body, Gaston developed osteopenia (pre-osteoporosis) in his hips and spine.  He began taking daily bone-building drugs to increase bone density which is monitored through annual bone density scans.  He's been in a normal range for the past two years.  We also visit the emergency room routinely for IV meds when Gaston gets any type of virus that keeps him from holding down his replacement hormone meds. Without adrenaline to help his body cope with the stress, he will go into shock and the situation becomes serious.  So, the next step was the purchase of a medic alert tag stating 'adrenaline deficient' in the event Gaston was in an emergency rendering him unconscious or unable to communicate with paramedics or medical personnel.

Another hurdle during the the first few years after the surgery was frequent visits to the ER for bowel obstructions. The surgery left scarring through his intestines which occasionally made it hard for food to pass through normally. The food would literally get blocked, causing alot of pain, nausea and vomiting. After several years though, the body slowly adjusted and this problem ceased.  Taking pancreatic enzymes before any meal or snack also helps him to digest his food.  The part of his pancreas that secretes this enzyme was also surgically removed. Aren't we blessed to have the medical research and drugs available now? Gaston would have no quality of life if it weren't for these being made available to us.

During this part of our journey, our relationship with our Lord Jesus continued to grow by leaps and bounds! I teach aerobics at a local hospital wellness center where the ladies are the sweetest most loving group I've ever been around. One of them suggested a weekly Bible study after class. Without hesitation, I agreed.  This was where I found out what an adventure it is to study God's word! The deeper I dug into His word, the hungrier I got to learn more. Gaston and I both couldn't wait to wake up each morning to read our Bibles before starting the day. Gaston began attending a mens'  morning Bible study weekly, so we both were finding something to replace that empty place in our hearts that had longed to be filled. We began to lose interest in our old way of living life and we wanted to live this new life that we had discovered in Christ Jesus.  We wanted to spend time with our church friends.  As it says in Romans 12:2,  we no longer wanted to "conform to the ways of the patterns of this world" but "to be transformed by the renewing" of our minds so that we would "be able to test and approve what God's will is, His good pleasing and perfect will."

Part II

In the first part of our journey, I mentioned a suspicious spot found on Gastons' pancreas during earlier tests taken prior to the first surgery. When Gaston was in the operating room for the second surgery, tests showed a tumor in his pancreas.The surgeon felt confident it was most likely malignant and decided to do a whipple procedure to remove the tumor from the head of the pancreas.

The Whipple procedure involves removing the appendix and spleen, disconnecting the intestines from the liver to reach the pancreas, and removal of the tumor. Then, placing all of the internal organs back in the cavity. This meant reconnecting the intestines in a different formation which left the organs in shock from being moved and handled so much. The surgeon also removed what was left of the adrenal glands.

The operation was a success, but reality set in when the surgeon came out to explain the procedure to me in detail. He told me Gaston would be critical and possibly would not live the next 24 hours. He also told me that chances were great the organs would be damaged to the point of a permanent colostomy or at the very least diabetes. Gaston was on a ventilator and had so many drains, machines and medical devices connected to him I hardly knew him when I walked into the room. Those of you who know Gaston, know what a fighter he is.  Plus, he had a God that had big plans for him and wasn't about to give up on him! After fighting through the critical status, he was sent to a hospital room where the doctor would round twice daily. The physician would listen with his stethoscope for that much anticipated "gurgling" noise which was indicative of the organs waking up and functioning. This went on for over a week until that day when the room burst into cheers of joy (even the doctor was tearful and shouting). The Great Physician had brought Gaston through the worst part and now the healing could begin! Oh and by the way, Gaston did not have a colostomy nor is he diabetic.

Once food was introduced and his digestive tract began to return to normal, we were released to go home. Pathology revealed a non-malignant glucoganoma tumor in the pancreas, but the surgeon was confident he made the right decision in removing it considering how little was known about these tumors at the time. It was extremely rare to have the two that Gaston had in his adrenal glands and pancreas together. There was only one recorded case in Japan so Gaston made the medical records. His doctor recommended yearly screenings to keep a check for more tumors. Research showed these types of tumors can manifest as malignant tumors in the kidneys so he also goes for regular screenings with an urologist.

Gaston and I were transferred (through his job) to another city at this point. The doctor who did the surgery had studied under and consulted the endocrinologist where we moved so he was quite familiar with our case from the start (we started to see that God even traveled with us where we went!) At this point, we both were feeling weary and empty. We knew deep down inside there was something more than the life we were leading. We began attending church more regularly and also joined a Sunday school class. God used our teacher to light that fire inside us to learn more about Jesus through reading our Bibles. I will never forget when we decided to give up our daily newspaper and read our Bibles during that morning time. At first, it was strange and such a struggle ( I watched the clock more than read the Bible) but then the Holy Spirit started helping us understand it. We started to see changes in our hearts, we started doing things and feeling things we couldn't explain. The Holy Spirit moved into us and started a mighty work in us that cannot be explained other than God did it.

This was just the beginning of a long road of medical issues and problem solving. It was also the beginning of Jesus coming on board in our lives. Our minds are being renewed and transformed daily to accept what is ahead and having that reassuring knowledge that as children of God, it's going to be OK.

Introduction

We pray this journal ministers to the hearts of those with VHL and also to the families who support them.


The Beginning

We all hear those familiar words in the wedding vows "in sickness and in health" and "til death do us part," but for many we skip past those and pay more attention to the celebration that surrounds the joining of man and wife as one. The day after our honeymoon, Gaston went into the hospital, endured a 13-hour surgical procedure, went to CICU for 3 days, went to a regular room for the next 6 weeks before being released to go home still in a very weak state with more recovery to come. When the surgeon came out of the OR and told me the next 24 hours were very critical and Gaston may not make it, suddenly, those words in the wedding vows became etched in my mind and will forever.

In preface to this surgery...several months before our wedding, tests revealed tumors in Gastons' adrenal glands called pheocromocytoma.They cause the blood pressure to rise, secrete excess amounts of adrenelin that causes anxiety, headaches and palpatations. He had surgery to remove the tumors and the surgeon left slivers of adrenal glands to hopefully keep Gaston from having to take replacement hormones. That worked for awhile and he felt great, but just before our wedding the tumors grew back on what was left of his adrenal glands.  Tests also showed a suspicious spot on his pancreas so more surgery would be scheduled as soon as we returned from our honeymoon. So, we had our beautiful wedding and left for our honeymoon knowing when we returned he would have more surgery. Little did we know what an adventure and lifestyle change God had in store for us!

We both grew up in church, had been "introduced" to God, but we never knew Jesus personally as our Lord and Savior. We showed up in church occasionally, sat in the pews with stares on our faces, just going through the motions. We were living to please the world and our own sinful desires at this time in our lives. Our lifestyle involved drinking alcohol until we passed out and were much too hungover for church on Sunday mornings.

And so begins a journey of God's miracles and a new faith being born in Gaston and myself that continues to deepen more each day. We hope through telling our story of dealing daily with Von Hippel Lindau will encourage and strengthen the faith of others with this disease. What first was thought to be very rare, they're now finding out is much more common than they thought.  So, there are more and more people trying to process a diagnosis of VHL.

Please check back frequently as I will share how God and HIS perfect timing brought two lost souls into the Kingdom with His mighty works glorified through a disease called VHL.