NIAID - National Institutes of Allergy Infectious Disease

The NIAID - National Institutes of Allergy Infectious Disease is the institute that has been helping my husband (Colin Benson) with DRESS Syndrome.  We are so blessed to have a strong team of dedicated health professionals in his life and caring for him.

In May of 2018 the NIAID (after much research and analysis of Colin's specific condition) offered to take Colin into their full time care and hospitalize him with a trial drug, Tofacitinib.  They were also going to try anti-viral therapy for his HHV-6 reactivation.

Tofacitinib is also known as xeljanz. https://www.xeljanz.com/  Xeljanz is primarily used for Rheumatoid Arthritis.  It is a strong and powerful drug, one that comes with a black box warning label.

Our family was made aware of this drug around the time he was hospitalized at Walter Reed Medical Military Center (WRNMMC) in December 2017.  The medical team at WRNMMC had tried various other drugs and these medicines just weren't strong enough or able to hold back his break through skin eruptions, rashes and his relapses.  Not only was his skin shedding in great volume, but his labs were showing more damage to his kidneys and liver. Additionally labs showed reactivation of the HHV-6 Virus.  It was clear that there was a lot more going on than just DRESS Syndrome.  DRESS Syndrome should go away after removing the offending drug and after several weeks of mediciations.  Unfortunately, Colin complex condition was much more severe and longer lasting.

Because his condition was so unique and the medicine that NIH was interested in using was so powerful, we took Colin to Mayo Clinic for a second opinion.  A new set of brilliant doctors were looking over his entire body, labs and medical history only to concur that his case was unique.  His doctors were all in uncharted waters and no medical treatment plan existed for his condition, nor was there a diagnosis that really explained everything happening with him.  Even still, every doctor was still trying to determine just exactly what was his medical condition.  Since DRESS Syndrome is a diagnosis of exclusion all of doctors could only do one thing, keep testing.

Some of the suggestions provided by doctors at Mayo Clinic were to continue with IVIG (Intravenous Immunoglobulin Treatment).  He previously used IVIG three days in a row while hospitalized at the Burn Unit in Washington Hospital Center, Washington DC. Additionally, Mayo Clinic doctors were able to determine that Colin's CYP2D6 Gene metabolized drugs slowly in his liver.

After visiting the Mayo Clinic in Minnesota twice, it was now April 2018 and we were ready to move forward with Tofacitinib and the Anti-Viral with NIH in Bethesda, MD.  The process took a few weeks and he was moved into their hospital for observation in May of 2018.

He stayed in-patient at the NIH (NIAID) for over 6+ weeks.  (Thankfully it was across the street from WRNMMC, so I was able to see him every day.  I was also permitted to spend the night with him several times.)

Colin's Condition - February 13, 2019

Prednisone Step Down

Medical Information:  Prednisone / Steroids 

February 13, 2019

Colin had his appointment with National Institutes of Health (NIH) to review the immune protocol and experimental medication that we hope will replace all other immune suppression medicine today, February 13, 2019.  

We were instructed to continue with his Prednisone (steroids) taper, or "step down" and his doctors put a plan together that would cover the next few tapers.

Today he is on: (Listing immune suppression medicine only)

10 mg of Prednisone 

75 mg of Cyclosporine 

1000 mg of Cellcept

10 mg of Tofacitinib

First the steroid will be reduced (February 14) and then we will reduce the cellcept by 500 mg off his morning dose (February 28) and then hopefully is everything is going well and he is able to continue tapering on schedule we plan to reduce the steroids down to 8 mg (March 14)

So, in tomorrow's medication I will reduced his Prednisone to 9 mg.  At the onset of his illness Colin was initially taking 125 mg of steroids!  That is a very high (dangerously high) amount of steroids.  He began taking this high dose back in April 2017.  Unfortunately, he has suffered damage to his eyes from long term steroid use.  He has a lot of eye pain and pressure.  I give him a lot eye drops.  We also learned that he not only has glaucoma, but also has to have cataract surgery.  This explains why he is having a hard time seeing, blocked vision, headaches, pain and burning in his eyes and also seeing starbursts. 

Doctors have been attempting to slowly taper him off this high dose of steroids, cellcept and also Cyclosporine to replace with his experimental drug:  XELJANZ XR (tofacitinib) 

Here is some information about tapering slowly from Mayo Clinic

https://www.mayoclinic.org/prednisone-withdrawal/expert-answers/faq-20057923

Medical Information: https://www.xeljanz.com

My husband has a very unusual, prolonged undiagnosed medical condition that is chronic.  They have temporarily labeled his condition as immune dysregulation and chronic "DRESS Syndrome" with a reactivation of HHV-6 virus.  

There is a HHV-6 Foundation website that contains "some" information that is helpful to us, but again there has been no one found in the world that has what my husband has.  

So this entire process has been scary, stressful, confusing and challenging.  Which is why we continue to prayer for God to watch over Colin and to heal him.  

Colin's Condition - January 10, 2019

Prednisone Step Down

Medical Information:  Prednisone / Steroids 

January 9, 2019

Today we are back" home" living in the wounded warrior barracks (Tranquility Hall) at Walter Reed National Military Medical Center in Bethesda, MD

Colin had his appointment with National Institutes of Health (NIH) to review the immune protocol and experimental medication yesterday.  A lot of medical information was shared with us concerning his T cells and tregs (Regulatory T cell)  as well as his IgG (Immunoglobulin G).  Most of the information is too hard for us to understand completely, but we understand that doctors are tracking all of his numbers as well as his HHV-6 (Human herpesvirus 6) reactivation.

We were instructed to continue with his Prednisone (steroids) taper, or "step down" 

So, in Colin's morning dose today I reduced his Prednisone to 10 mg.  At the onset of his illness Colin was initially taking 125 mg of steroids!  That is a very high (dangerously high) amount of steroids.  He began taking this high dose back in April 2017.

He is down to 10 mg of steroids today, and we expect his body to react (as it always does with each taper).  His body becomes (even more) weaker and struggles to recover.  Sometimes his skin erupts or gets very thick and actually comes off.  (It's painful for him and frightening for me).  
10 mg may not sound like much medication, but he has been dependent on it for so long that it's extremely difficult for his body to make up the difference and recover when it's reduced.

Doctors have been attempting to slowly taper him off this high dose of steroids, but his body doesn't react well to changes in medication.  

Here is some information about tapering slowly from Mayo Clinic
https://www.mayoclinic.org/prednisone-withdrawal/expert-answers/faq-20057923

This past year doctors have been tapering him off many of his immune suppressants in an attempt to replace them all with the experimental dose and use of  XELJANZ XR (tofacitinib).

Medical Information: https://www.xeljanz.com

My husband has a very unusual, prolonged undiagnosed medical condition that is chronic.  They have temporarily labeled his condition as immune dysregulation and chronic "DRESS Syndrome" with a reactivation of HHV-6 virus.  
There is a HHV-6 Foundation website that contains "some" information that is helpful to us, but again there has been no one found in the world that has what my husband has.  
So this entire process has been scary, stressful, confusing and challenging.  Which is why we continue to prayer for God to watch over Colin and to heal him.  

Christmas Plans 2018

Happy Holidays and Merry Christmas | 2018

This year we plan to visit our oldest daughter in Pensacola.  She and her husband are living near NAS Pensacola.  Our Son-in-Law is in the Air Force.  They were just married this past May. We have not seen their home yet, so we are very excited!  

Since we have a long drive from Walter Reed National Military Medical Center, we will have to stop half way.  We booked a hotel near Charlotte, NC aka: the Queen City

My parents will be traveling up from Central Florida with our youngest daughter and our two puppies: Wesley the Boxer and Princess Buttercup the Puggle.  We have reserved a pet friendly hotel in Pensacola. 

Colin's Condition - December 12, 2018

Prednisone Step Down

Medical Information:  Prednisone / Steroids 

December 12, 2018

We recently took Colin to Orlando, Fl for convalescent leave for Thanksgiving break and time with our family and puppies.  
We have returned to Walter Reed National Military Medical Center (WRNMMC) and National Institutes of Health (NIH).

The doctors have given instructions for another "step down" on Prednisone (steroids) beginning with tomorrow's dose.

Background:

At the onset of his illness Colin was initially taking 125 mg of steroids!  That is a very high amount of steroids.  He began taking this high dose back in April 2017.  His body has suffered a lot of damage from long term high steroid use.  

His last prednisone dosage of 15 mg will be decreased tomorrow (13 DEC 2018) to 12.5 mg.  
Other concerns his doctors have are for his liver and bones.  
He had an updated DEXA scan earlier this week.

Here is some information about tapering slowly from Mayo Clinic

https://www.mayoclinic.org/prednisone-withdrawal/expert-answers/faq-20057923

Doctors have been attempting to slowly taper him off this high dose of steroids, but his body doesn't react well to medical changes.  

This past year doctors have been tapering him off many of his immune suppressants in an attempt to replace them all with the experimental dose and use of  XELJANZ XR (tofacitinib).

Medical Information: https://www.xeljanz.com

Colin's Condition - updated Bone Density Scan

Bone Density Scan

Medical Information:  What is a DEXA scan (According to WebMD)

December 2018

As Colin continues his use of Prednisone, doctor's want to keep an eye on his bone health.
They ordered a DEXA scan at his baseline, in the beginning and have ordered a follow up DEXA scan this week.
According to Mayo Clinic, "Corticosteroids also can dramatically weaken bones and lead to osteoporosis".

He is currently taking supplemental Calcium and vitamin D as well as Vitamin D2

According to the Arthritis Foundation  "Prednisone, a corticosteroid drug used for decades to control inflammation, is one of the notorious culprits in bone mineral density loss."

Preemie Packing Party - Them too

We are looking for a donations and supplies for our Preemie Packing Party this December, in Bethesda MD.

Our Deadline is: December 20

Location - TBD

 

There will be a group of military caregivers and volunteers putting together care packages for new mothers and NICU babies, in Bethesda, MD.

 

We are asking local businesses to support our group effort by donating supplies for the Preemie Packing Party.

In exchange, we will be contacting local media sources and posting on social media to share our event and our supporters.

 

Please help our organization, and our babies.

 

We can provide a wish list and we are also open to suggestions!

 

Thank you very much for your consideration

 

Laura Benson

Laura@GamePlanLocal.com

Laura@GamePlanMediaEvents.com 

 

808-725-7749

202-670-6650

​

Go Fund Me Page

Details:
Hello, my name is Brandi. My request is quite simple, I am trying to raise money for  families in the NICU. (Neonatal Intensive Care Unit) Many of these families  are confused and scared because why wouldn’t you be? I want to help these families by taking away some of the stress, and by donating items I think would help them. Currently, there are 8 families in the hospital that I am trying to donate to. My wish is to get the gifts together before Christmas as nobody wants to or chooses to be confined to a hospital room on holidays. Please help me reach the goal so I can buy and get the packages to the families close to Christmas!
So this fundraiser is to put together bags of things such as chargers, stress balls, non perishable snacks, herbal teas, reusable bags, nex gift cards, iTunes gift cards, journals, pens pencils, headphones, kids coloring packs, body travel sets, gum, tissues, certain books to read to babies and preemie button up onesies. These are gift bags to help alleviate some of the stress off the families of the babies being in the NICU. So it’s built for the families not the babies necessarily.
My intentions are to continue offering these care packages in the future with the right sponsors and support from you all!
Thank you so much!

About Them too is a startup organization created by Brandi Lambert that will support the needs of new military families, specifically in the Neonatal Intensive Care Unit (NICU).
Brandi Lambert can be reached at:
803-280-7929
themtoofoundation@yahoo.com

2018 Holiday Mail for Heroes

The 2018 Holiday Mail for Heroes campaign through the American Red Cross and the American Red Cross - Walter Reed National Military Medical Center is here!

Every year we have a window of time for the community to send cards to our patients and wounded warriors for the Winter Holidays. The campaign has several deadlines that relate to specific holidays so please refer to the attached information.

Please note the address and attempt to correct anyone who may have outdated information. We will continue to post more info throughout the season.

Colin's Condition - November 23, 2018

CellCept Step Down

Medical Information:  https://www.cellcept.com 

November 23, 2018

Today we are visiting family and our dogs in Orlando, FL

Before leaving Walter Reed National Military Medical Center (WRNMMC) and National Institutes of Health (NIH) we were instructed to "step down" Colin's medication on November 23rd.  

So, in Colin's evening dose I will be reducing his CellCept medication by 500 mg.

He is currently taking 500 mg in the morning and 1000 mg in the evening.

This past year doctors have been tapering him off many of his immune suppressants in an attempt to replace them all with the experimental dose and use of  XELJANZ XR (tofacitinib).

Medical Information: https://www.xeljanz.com

Caregiver Appreciation Month Open Forum

November is National Family Caregivers Month

Thank you to Linda Rasnake (FRSA: the WTB Care Coordinator at Walter Reed National Military Medical Center) for putting together this wonderful caregiver luncheon today at the USO Walter Reed National Military Medical Center  (Nov. 14, 2018)

Linda made this wonderful banner for Mrs. Hollyanne Milley, wife of the Army Chief of Staff, who was present today. The banner had photos of our faces. That was such a nice personal touch. And she and her husband also made these clocks for some of the caregivers with our faces on it. Really nice! Very thoughtful

Linda is so incredibly helpful, full of support, love and encouragement. 
I thank God for her!   Even though she is supported on our base by the Army she goes out of her way to get to know each of us and doesn't turn anyone away based on which brand of military our loved one is serving.  (Mine is Navy)

She has so much valuable knowledge that helps us (NMA's and Military Caregivers) get through the difficult transitions and adjustments that happen when you go through something devastating like this. 
Especially when you are suddenly put in a position or medical crisis that changes your whole world. This enormous weight of responsibility is given to us by the military, as you are a trusted caregiver for your service member. ❤️🙏

NMA:  The Non-Medical Attendant (NMA) program is designed for seriously wounded, ill and injured Soldiers who could benefit from the presence and assistance of Family or friends. 
FRSA: The Family Readiness Support Assistant (FRSA)is an integral part of the FRG, an official Army program established pursuant to AR 600-20, Army Command Policy. Unit commanders at all levels are expected to establish and/or support FRG operations.

Colin's Condition - November 9, 2018

Prednisone Step Down

Medical Information:  Prednisone / Steroids 

November 9, 2018

Today we are still living at Walter Reed National Military Medical Center (WRNMMC) and Colin is still under the medical care and immune protocol at National Institutes of Health (NIH).

The doctors have instructed me to "step down"  Colin's Prednisone (steroids) today.

At the onset of his illness Colin was initially taking 125 mg of steroids!  That is a very high amount of steroids.  He began taking this high dose back in April 2017.

He is down to 15 mg of steroids today, and believe me as I write this - we are so worried.  Each time we taper his steroids - he flares.  15 mg may not sound like much medication, but he has been dependent on it for so long that it's extremely difficult for his body to make up the difference and recover when it's reduced.

Here is some information about tapering slowly from Mayo Clinic

https://www.mayoclinic.org/prednisone-withdrawal/expert-answers/faq-20057923

Doctors have been attempting to slowly taper him off this high dose of steroids, but his body doesn't react well to medical changes.  

This past year doctors have been tapering him off many of his immune suppressants in an attempt to replace them all with the experimental dose and use of  XELJANZ XR (tofacitinib).

Medical Information: https://www.xeljanz.com

Requesting Patriotic Tributes

In the past our goal was to support local veterans groups by bringing together the Veterans and the Defense Community as well as the General Public through Community Events, giving the general public an opportunity to appreciate our local/hometown heroes.

We conducted these events in Orlando, Florida with the support of local veterans groups, chambers, media sources and volunteers.  

Since moving away to Washington, DC and becoming a full time caregiver to my military spouse at Walter Reed National Military Medical Center, I am no longer able to carryout plans to execute these events in person - however, it would be my honor to recognize others throughout our great country by sharing their events, recognition and support of our nations heroes. 

We encourage you to post your events and appreciation, and we will help you share them.  

God Bless the USA.

Purpose of the Patriotic Tribute Blog

In the past our goal was to support local veterans groups by bringing together the Veterans and the Defense Community as well as the General Public through Community Events, giving the general public an opportunity to appreciate our local/hometown heroes.

We conducted these events in Orlando, Florida with the support of local veterans groups, chambers, media sources and volunteers.


Since moving away to Washington, DC and becoming a full time military caregiver to my navy spouse at Walter Reed National Military Medical Center, I am no longer able to carryout plans to execute these events in person - however, it would be my honor to recognize others throughout our great country by sharing their events, recognition and support of our nations heroes.


We encourage you to post your events and appreciation, and we will help you share them. God Bless the USA.

Colin's Condition - October 26, 2018

Prednisone Step Down

Medical Information:  Prednisone / Steroids 

October 26, 2018

Today we are still living at Walter Reed National Military Medical Center (WRNMMC) and Colin is still under the medical care and immune protocol at National Institutes of Health (NIH).

The doctors have instructed me to "step down"  Colin's Prednisone (steroids) today.

At the onset of his illness Colin was initially taking 125 mg of steroids!  That is a very high amount of steroids.  He began taking this high dose back in April 2017.

He is down to 17.5 mg of steroids today, and believe me as I write this - we are so worried.  Each time we taper his steroids - he flares.  That may not sound like much medication, but he has been dependent on it for so long that it's extremely difficult for his body to make up the difference and recover when it's reduced.

Here is some information about tapering slowly from Mayo Clinic

https://www.mayoclinic.org/prednisone-withdrawal/expert-answers/faq-20057923

Doctors have been attempting to slowly taper him off this high dose of steroids, but his body doesn't react well to medical changes.  

This past year doctors have been tapering him off many of his immune suppressants in an attempt to replace them all with the experimental dose and use of  XELJANZ XR (tofacitinib).

Medical Information: https://www.xeljanz.com

Colin's Condition - NIH Discharge

NIH discharge

Journal entry by Laura Benson — Jul 9, 2018

Colin was discharged from NIH tonight 

NIH admitted Colin for a specific autoimmune protocol.
He was monitored closely from May 29 through tonight (July 9) inpatient and finally discharged back to Walter Reed. He will continue to return to NIH as an outpatient three days/week.  Thankfully NIH is directly across the street from Walter Reed.  
Although he is considered an outpatient, we will be spending many days at NIH -  transporting him back and forth from the Navy base to the NIH campus.

We spent the entire first half of this year looking for other solutions or answers to "What is wrong with Colin?"  We even took him twice to Mayo Clinic in Rochester, MN for several weeks at a time.
Sadly, there were no answers, no prognosis and not even a diagnosis.  The only conclusion is that the medicine he is currently on is causing too many dangerous side effects, and unfortunately they aren't helping control his condition any longer. 
He has been constantly waxing and waning - but never healed or even managed.  He has had several relapses and hospitals stays.  And, every day is pain and discomfort for him. 

 

  
The NIH doctors had planned to taper the immunesuppressive drugs that he has been on for the past 1 1/2 years and replace them with a new experimental drug.  
The drug itself is not experimental, but using it for this purpose is experimental.  
To be clear - this drug is prescribed and used on other types of conditions, but not on someone with DRESS syndrome, Drug Hypersensitivity or the type of immune condition that has manifested in Colin. 

 

The goal during his stay at NIH was to get him off some of the meds through a slow taper.

NIH added a new drug: Tofacitinib to target his jak/stat pathway. 
They also added an antiviral medication, valganciclovir, for his HHV6 reactivation.  

So basically, add the new drugs and taper off the others.
So far - some of the taper was successful, while others were not.     

Today we were told by one of his doctors that this new drug may not cure him, but we were reminded and do understand that he is helping to further science and research and any others that may suffer similar conditions.  

Colin's Condition - Independence Day 2018

Independence Day (4th of July 2018)

Journal entry by Laura Benson — Jul 4, 2018

This year has flown by.  We have spent so much time living in and out of hotels, hospitals and traveling from Bethesda, MD to Rochester, MN to Orlando, FL.

Colin has currently been inpatient at NIH since May 29, 2018.  He agreed to try a new medication that could potentially help with his JAK/STAT pathway.  Additionally, his condition continues to wax and wane and with that he also has also reactivated the HHV6 Virus.  So, the doctors at NIH are trying to control his inflammatory response and his virus with two additional drugs:  Tofacitinib and Ganciclovir

The goal is to add the Tofacitinib and taper off the other immune suppressing medications that he has been on (prednisone, cyclosporine and cell cept).  Long term use of these medications have been causing secondary problems for Colin (dangerously high blood pressure, Cushing Syndrome, glaucoma, acute kidney injury and many others).  The less medication he is on, the better!  Side effects are nasty and they do cause a lot of damage.  

The taper began, first with cyclosporine.  Doctors make adjustments week by week, so we have to be very patient.  Colin has to have his blood drawn and body examined daily.  We have been trying other new things as well, such as milk baths.  (He uses the hospital walk-in tub/bath and I pour an entire gallon of milk into the bath water with a solution of bleach).  
He was finally completely off cyclosporine on Sunday, but then the doctors didn't like the lab results and were not comfortable seeing his rash breaking through, so he was placed back on cyclosporine. 

So the new plan would be to try to taper his steroids.  
This can be tricky, because his body needs to wake up adrenal glands. In the beginning, Colin was on 125 mg of prednisone!!!  He was on 100 mg then 80 mg and stayed around 60 mg for several months.  It has taken well over a year +++ to get his taper down to 40 mg.  He keeps going up and down, from 80 to 100 then back to 80 and then slower on the way down. And even slower as they get lower.  It's a roller coaster for his body and his health.  He has been down on 40 mg for months now.  The doctors have attempted to go lower, but it wasn't successful. Meaning, his body can't make up the difference.  Trying to taper Colin off steroids always causes another hospitalization - because his own immune system can't wake up.  When this happens, doctors add more steroids back on and go back up, which makes the steroid taper process take even longer.

Honestly, we were told back in Spring of 2017 when this all began that a drug reaction would last 2- 8 weeks and that he would be off steroids in about six months - so much for that!!

He has been struggling with the steroid taper the entire time.  And his condition has caused multiple hospitalizations and the loss of so much skin.  So much damage to his body.  It's unimaginable.  And unceasing.  He is so very strong and patient, but it takes a lot out of him to be in constant pain and misery EVERY single day - without a break.  I have not seen Colin have one single "good day" since this all began. We do the best we can to make life worth living and enjoyable as possible - like trips to see the dogs and family in Florida.  But it's not the same when he is sick, handicapped (wheelchair bound) and has to conserve all of his energy.  It's heart breaking to watch someone you love go through this and realize there is nothing that I can do to help him.

Hopefully with the addition of Tofacitinib he can successful taper down to a lower (less damaging) maintenance dose of steroids.  And then doctors can decide which immune suppressing medicines to taper next, if possible.  

To add insult to injury, Colin's 23 year dedicated and highly successful career in the United States Navy is sadly coming to an end for him. (Too soon and NOT the way he wanted to retire; not his choice and not fair to him.)  
The Navy began his retirement process last month.  We were told to move our Florida home into storage and tie up any loose ends, so that we can focus on his Medical Review Board/Retirement while living in the Wounded Warrior Barracks (building 62 as we call it).  

Thank God that NIH has allowed Colin to remain in their hospital for over a month for supervision.  (He was admitted on May 29)  It's comforting to know that doctors have been carefully looking his body over -literally inside and out, every single day for over a month straight.

We can also thank God that He placed us in Washington DC in 2016, when Colin was given orders to DLI (Language School).  Thank God this serious illness and unknown medical condition did not happen while we were living in Northern Germany. 

We pray every day for Colin's health and strength to keep fighting through this and we thank God for putting him so close to Walter Reed National National Military Medical Center and National Institutes of Health, which are literally across the street from each other. 

We are celebrating the 4th of July in the hospital at NIH.  

Thinking of all of you and hoping you have a safe holiday. 

Colin's Condition - NIH Trials inpatient

Adding the experimental medication as well as the antiviral

Journal entry by Laura Benson — Jun 9, 2018

Colin has had a rough week.  Last weekend NIH gave Colin water pills to remove fluid around his heart.  He lost a lot of water and became severely dehydrated.  This took his very high blood pressure and brought it far too low.  Since he already has kidney injury from last year at the onset of his condition, this only made matters worse.  So the team of doctors spent the entire week getting his kidneys functioning and his blood pressure balanced with IV Fluids and medications (although blood pressure is slightly stable, it is still high).  Only then were they willing to finally let him take his experimental medication:  Tofacitinib.

He took his first dose of Tofacitinib on Thursday.  He had to wait ten days!!
Doctors continue to monitor his creatinine levels and also are attempting to taper his cyclosporine dose, slightly.  (Cyclosporine is known for causing high blood pressure and kidney problems.  Colin has been on it for over 12 months).   
He is now taking Tofacitinib twice a day.

Today the doctors introduced Ganciclovir, an antiviral. It is used to treat infections caused by viruses.  
Colin has a reactivation of HHV6, which is something we all had when we were babies.  There are known medical studies following patients that have a reactivation of HHV6 with drug hypersensitivity (DIHS).  Colin has been tested for HHV6 many times this last year.  Occasionally it shows up in blood tests, but has not been consistent.  So, the NIH doctors would like to treat it while he is being closely monitored.  He continues to donate his blood for testing that is used in this type of research.  

All of the medicine that Colin has been taking for the past year are immune suppressants (Prednisone, Cyclosporine and CellCept and now Tofacitinib). All of the strong medications that he takes to attempt to control his flairs and relapses have a lot of side effects that are very concerning.  And if you've been following him - you know that he has had many break through flairs and relapses, which is disheartening.  As you can imagine, he is getting weak and tired from fighting whatever "this" is.  And as many of you have experienced yourselves, all of the medicine that Colin takes causes side effects that also must be treated. 

Thank you all for your prayers because we know God is listening. 

We have had the best doctors examine and treat Colin.  Walter Reed, John's Hopkins Center, Washington Hospital Center, Mayo Clinic and NIH and many others have been consulted from various other hospitals.  They genuinely care and are working hard at learning more about Colin's condition and why his own immune system won't kick in and fight for him.  

Colin's Condition - NIH Medical Trials

NIH Medical Trials

Journal entry by Laura Benson — May 28, 2018

Thank you for following Colin's story.  
We are writing this post the evening prior to admitting him into the hospital just a short distance across the street (writing this from Walter Reed) to the National Institutes of Health (NIH). 

Colin has been a Research Patient at NIH for many months.  NIH doctors have discovered many interesting remarkable things about Colin; how his body responds to chronic inflammation and what possible medication may be available to help control or stop his relapses.  We first brought Colin to NIH last November 2017 when he was viewed at Grand Rounds (over 70+ doctors examined Colin).  One of the Dermatologists at NIH viewed him and wanted to place Colin into a research study on DRESS/DIHS syndrome.

DRESS/DIHS syndrome should only last 2-8 weeks.  So, why has Colin been severely ill and in pain and unable to pull out of "this" for 14 months?!  The doctors don't all agree on the diagnosis.  After all, this is a diagnosis of exclusion (which means that doctors only know what he doesn't have.  They continue to test his blood and skin often to look for any answers to come up.  He has been tested for so many conditions and nothing makes sense). Some even suggest he may have a rare autoimmune disease. 

If you have been reading his updates, you will know that we have taken him to Mayo Clinic in Rochester, Minnesota (twice) for Second Opinions from several doctors.  Mayo helped discover one of Colin's gene mutations, which help doctors and pharmacists identify how he metabolizes medications and which to avoid.  Additionally, Colin also continues to have a severe skin flares and reactivation of a specific virus (HHV6) that comes and goes.  It is picked up in certain blood tests.  Not enough information is available on DIHS/DRESS Syndrome is available yet. However, the condition he suffers from doesn't only affect skin, but also affects organs.  All doctors are very concerned not only for damage to his organs from his condition, but also from the side effects of taking high doses of steroids and immune suppression medicines.  
We have been cautioned and warned for over a year about his immune system and he has been very lucky; however, they are going to begin an experimental medication on Colin at NIH...Before he agreed to do this...

Colin was granted convalescent leave to visit Florida where we celebrated some very important family milestones.  Colin's father (Randy) is also here helping manage Colin's care with me (Laura).  I was able to attend a caregivers retreat with Semper Fi Fund in San Antonio, TX and our daughters (Alexandra and Cassandra) drove up from Florida with our puppies (Wesley and Buttercup) to spend a week with Colin visiting before his admission back into the hospital.

Colin was able to participate in Walter Reed "field trips" to Rolling Thunder and later that day a tour of The US Capitol and front row seats to the National Memorial Day Capitol Concert.  It was an emotional day reflecting on our military's heroes and their sacrifice to our country and our freedom.

We were so grateful to have these moments, because now things are going to get more intense and many more prayers are going to be sent.  And we are asking for your prayers as well, please.  

NIH, Mayo and Walter Reed are all working together to help him.  His condition is very complicated, and he is very Atypical. So far Colin has only been a research patient, until now he will be an actual patient and admitted into the hospital at NIH.  The reason for his admission is so that doctors at NIH will have a controlled environment to protect him from opportunistic severe infections.  This is a real threat and one that the doctors are not taking lightly.  Colin basically has no immune system and even a cold could harm him significantly. 

NIH discovered that Colin has a mutated JAK/STAT pathway.  NIH and Pfizer discovered a medication "Tofacitinib" which has a generic name Xeljanz that is used primarily on Rheumatoid Arthritis, which targets the JAK/STAT pathway. It has never been used on someone with Colin's condition before.  So he is Patient #1 in the research on the uses of this medication. If you Google Xeljanz you will see a black box warning.  

Here is the link: https://www.xeljanz.com/ 

The plan for now is for Randy and I to stay in Colin's room on base at Walter Reed National Military Medical Center.  Thankfully we can easily walk across the Navy base and cross the street to visit Colin while he is at NIH for the next 1-2 weeks.  The hospital will give us permission to visit him and he may even receive a "Day Pass" to take short breaks away from NIH to attend his normally scheduled doctor appointments at Walter Reed.  Keep in mind that NIH is NOT a normal/typical hospital.  It is a research facility with a hospital for their research patients.  (Very different than what most people would imagine).

We will continue to update you on his condition and thank you in advance for your prayers. 

Colin's Condition - Mayo Clinic Updates and 23 years of service

Mayo Clinic Updates and 23 years of service

Journal entry by Laura Benson — Apr 10, 2018

We brought Colin to Mayo Clinic (Rochester, MN) in both February 2018 and again for a return visit in March-April 2018.  We requested a second opinion and an evaluation and comparison findings from NIH (National Institutes of Health) and Walter Reed National Military Medical Center. 

The first visit (Feb. 19) brought many questions and interesting information to light, but no answers.  We left hopeful that we would be able to return and continue on with a possible cure or treatment plan in sight.  Our flight to and from Mayo Clinic was very challenging.  Moving Colin from wheelchair to seat and pushing him through the airport was very rough on him.  It took him many days to recover.

We returned to Bethesda on March 1.  After a few days of rest (and our first nor'easter) we took Colin to NIH for a follow up visit.  NIH, using a RNA sequencing, had discovered that Colin has a JAK3/STAT1 pathway that could benefit from treatment.  NIH would like to start Colin on Tofacitnib(which has a black box warning).  This drug is normally used on Rheumatoid Arthritis, and never used before on anyone with Colin's condition. So we were hesitant to start treatment.  We would like to learn more about the drug and what other treatment options exist before moving forward.

Later that evening we recieved a phone call from Mayo Clinic explaining that Colin's genetic testing revealed that he had several mutatued genes, CYP2D6 and others - which may explain how he metabolizes medication and it's affect on his body.  So, he may be predisposed to drug hypersensitivity.  We spend the next several weeks learning about cytokines, JAK/STAT pathways and something about IL2 and CD25 receptors, and many other new medical terminology - all very confusing, but significant and vital to understanding what is wrong with Colin.

Throughout that week we visited other departments at Walter Reed and got further confused! And scared!  This trial medicine is a very powerful immune suppressing drug - Colin would need to be monitored very closely.  It also costs a fortune and may not be covered.  The side effects are terriying.  But so is not treating him.  We must do something, but need help finding out what is best.

We were allowed two full weeks of Convalescent Leave from his command and drove to Florida to visit our family and puppies.  Colin stayed indoors and mostly in bed the entire time.  He was just too wiped out.
We then drove from Orlando, FL to Rochester, MN stopping halfway in between, along the way.
He began seeing Mayo Clinic departments on the 26th of March.  (Quite exhausted from all of the travel.)

Colin was having another relapse during this time.  He was in a lot of pain, his skin was scaling, red and inflamed, he said it felt like it was burning and he lacked energy to move about.  He was also experiencing new symptoms, more fatigue and confusion.  We nearly had to take him to the emergency room; fortunately, I had been trained in burn and wound care, so we were able to sucessfully control his skin flare in the hotel room with ointments and wraps.  And at every medical appointment we shared our concerns from his confusion, fatigue and pain.

During our stay at Mayo Clinic, we received more interesting information from NIH which was revealed to us over the phone, Colin's blood has reactivated HHV6 virus - read below for why this is significant.  
NIH and Walter Reed would like to start administering antivirals, in addition to the Tofacitinib.  Antivirals are highly toxic.  Ganciclovir is considered a potential human carcinogen, teratogen, and mutagen.
Tofacitinib is required by US FDA to have a boxed warning on its label about possible injury and death due to problems such as infections, Lymphoma and other malignancies which can arise from use of this drug
Here is an explanation on HHV6 viris:
It is thought that HHV-6 reactivates in response to the altered immune environment that arises during the adverse drug reaction, and consequently, a strong anti-viral immune response is triggered to combat the infection. In turn, immune cells attack the cells containing HHV-6 and infiltrate the infected organs, causing widespread damage

Colin had originally been diagnosed with Atypical relapsing and remitting DRESS or DIHS
DIHS, (the criteria esablished by the Japanese group to identify drug reaction is different than the  Eurpoean model of DRESS).  
The scoring system is called SCAR = Severe cutaneous adverse reactions.  (specific to drug hypersensitivities). 
SCARs includes four other drug-induced skin reactions, Steven Johnson syndrome (SJS); Toxic epidermal necrolysis (TEN), Stevens-Johnson/toxic epidermal necrolysis overlap syndrome (SJS/TEN); Colin had ALL of these!
We had Mayo Clinic test his blood while we are here, and he did NOT have the live virus. (???) How is that possible?  Some doctors say that HHV6 can come and go, just as his skin erruptions wax and wane.  Colin is in a constant state of this inflammation and no doctors or hospitals can figure out what "it" is and how to make it stop.

Here are some other terms:
DRESS = Drug reaction with eosinophilia and systemic symptoms  DIHS = drug-induced hypersensitivity 

The most common cure and fix to DIHS/DRESS Syndrome is plain and simple = remove the offending drug!  We did that, over 12 months ago!!  So why on earth does he still have it?  How is that possible?
So the argument is really - Does Colin even have DRESS/DIHS?
And 12 months later, we are always being asked if we care what the cause is or what started all of this - or do we just want to work on various possible experimental treatment options?

That is what we are desperately pleading for help with - how can we make this stop?
Why does Colin have to suffer any longer with constant flares and pain every day.  He is not allowed any pain medication - because Colin is susceptable to drug hypersensitivities.

We DO want to know what caused this and why.  We feel that it is important to know this critical information, so that if one day he is finally in remission, that we don't accidentally cause this to happen again!  We aren't looking for a band-aid; we want a cure and a safe treatment plan.  Currently Colin's organs are pretty beat up from all of the steroids and immunosupression medication he has been taking for the past 12 months.

Our time here at Mayo is coming to a close.  I am tearfully typing this journal entry, knowing that we will leave with no clear understanding as to what is wrong or how to stop it.  

I leave you with this important fact to keep in mind, Colin's service to our country:

Colin joined the service in 1995.  Today marks his 23rd year serving our country as an Aircrewman in the United States Navy.  Through out his career he has enjoyed many opportunities and traveled the world to exciting and dangerous places.  He has advanced in his skills and training and promoted to Senior Chief.  He has much to be grateful for concerning his career - and you can see this from his pride and many devoted years of service - and from what others say about him professionally, and those that know him personally.  
What you may not see is how this illness has negatively impacted him, not only in his health but how it may be the (premature) end to his military career.

Colin's Condition - 2nd Visit to NIH and another relapse

2nd Visit to NIH and another relapse

Journal entry by Laura Benson — Jan 18, 2018

Colin has been getting progressively worse (again).Not only is his skin inflamed (again), but he feels terribly crummy, he is out of breath, very lethargic and extremely weak.

At his last NIH visit for Human Trials (case study) the doctor noted a flair up during the exam portion of Colin’s visit. The primary goal of that appointment was to collect blood and skin for testing and research purposes.  Colin has volunteered to donate blood and skin to science at the NIH to help find a cure for DRESS syndrome. 
Thankfully, we are blessed with an accomplished doctor who has the most experience and continues to study and researches DRESS syndrome patients, specifically in Asian communities. Colin went "home " (back to the Wounded Warrior Barracks at NSA Bethesda) to rest and recover (from the extensive daisy chain biopsy sites as well as the process of transporting him from the Navy Base to the NIH Campus). 

He was at the NIH on Tuesday, and every day thereafter he has gotten worse. Not only has his skin begun to erupt and manifest several different dermatological presentations, but he continues to feel more and more lousy.

I started to panic, as I sent texts and called doctors, from a feeling of Déjà vu, as I have “been there before” and felt (from experiencing this with my husband, many times now) that we may have to take him to the Emergency Room. There is only so much that I can do for him in our room/unit.  Some relapses we struggle to care for him in our unit, while others are so severe that he needs IV and medical attention 24 hours a day.

We ended up speaking with doctors and medial professionals (Walter Reed’s Dermatologists, Internists, Nephrologists and his case manager, and I even reached out to the NIH, since they were the last to see him in clinic) to problem solve and contemplate what the source of this new relapse was. Especially since a new medication was introduced last Friday (due to extremely high blood pressure).

Ultimately, he needed to be seen right away.  So, we went to several clinics at Walter Reed early this morning to get some eyes on him and determine what would be best and safest for him.

 We are attempting to avoid a hospital admission today, but there is no guarantee that won’t be coming.  The doctor noted that he is relapsing and it is coming on strong, so we have to keep a careful watch over him.

There is a fine line in determining what is truly best for him, given his unique condition.  Admit him to give him IV fluids, medications and medical personnel yet how do we prevent Colin from possibly contracting a Staph infection or c.diff. or worse.  According to the medical professionals, we have to avoid a hospitalization if at all possible.  Each time he is admitted he picks up something from the germs in the hospital and has no way for his body to fight. (due to his suppressed immune system)

The decision to send him back to the Wounded Warrior Barracks was made to keep him away from other sick people who he would be exposed to if admitted. (We were told that this hospital has seen many admissions; mostly with RSV and the flu.  Colin's immune system could not handle that right now).
Instead, he will be under my care and observation and confined. 
Thankfully we can walk to the hospital from the barracks and the base has transport, if needed.

They ordered several blood labs and bed rest and now he is back in his room, resting. 

In lieu of hospitalization, they determined that they have to increase his steroids today to help him fight off this recent flair up. (He was hoping to continue to step down, and get away from steroids and the side effects that come along, i.e. diabetes, mania, weight gain, brittle bone, stomach ulcers, liver and kidney disease, Cushing's Syndrome, just to name a few.
He also was given a steroid shot under the skin, I'm sure that was terribly uncomfortable and painful.

The doctors have also added new medications to the list of drugs he already takes.  Unfortunately, each time we attempt to taper him off his meds he relapses and the doctors end up having to bring the dose back up again and sometimes add others.

Additionally, they are transitioning him to a brand new immunosuppressant drug and planning to wean him off the one he has been taking since May. The current drug has lost its effectiveness and also has dangerous side effects when used long term such as pancreatitis and high blood pressure.

For the next week he will be taking both immunosuppressant drugs together, (along with the steroids and all others that he takes for all of the side effects that he is experiencing). They will taper one off as they increase the other. They need time to evaluate how he reacts to the new one without withdrawing completely from his original medication. But, this is a fragile process. It seems each time they add a new medicine, he has a reaction.  

Because, Colin's condition keeps him in a state of constant hypersensitivity 

Just another day in the life of his chronic DRESS syndrome condition. 

The NIH is testing him many things, among the tests ordered is one for something called Cytokines, to get information on how his body reacts and responds to inflammation. He is very much a science experiment and involved in these extensive human trials on chronic DRESS syndrome.

It’s terrible they don’t yet have a cure!

Hopefully the study that Colin is contributing to (on his own free will) can help find a cure for any others that have to go through something as horrible as this.