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.

Colin's Condition - National Institute of Health

National Institute of Health (NIH)

Journal entry by Laura Benson — Jan 1, 2018

In November, while Colin was inpatient at Walter Reed, he was escorted to the National Institute of Health (NIH) for "Grand Rounds".  There, he was viewed by over 75 doctors.  One of those doctors, was Dr. Nagao who specialized in Dermatology and has an association with Japanese Dermatology Studies, who expressed an interest in Colin's condition.  His specialty is skin immunology and immune-mediated diseases.  At first, Colin was only going to be subjected to testing for medical and research purposes; however Dr. Nagao found his condition so interesting and similar to the Japanese studies that he asked to work with Colin on his ongoing condition alongside the teams of doctors at Walter Reed.

So the question is what is going on with Colin?  Why does he still have DRESS syndrome - when most people only have this condition for 2-8 weeks?  And what can we do to stop Colin from relapsing and cycling through phases of multiple skin conditions as well as dangerous systemic system responses?

We finally have some answers.

On Thursday December 14, 2018 our daughter Cassandra and I (Laura) took Colin to the NIH for his preliminary meeting and blood draw.  We brought samples of medication with us for testing purposes; including the medication that is suspected to have triggered the initial inflammatory DRESS Syndrome response thus beginning his whole horrific ordeal. 

We learned a lot from our first visit.  Dr. Nagao was extremely intelligent and thorough.  It is clear to us that he has experience with DRESS syndrome patients.  Just like you, we also have so many questions and concerns.  It appears that in rare cases (under very specific conditions) DRESS Syndrome can continuously flair for several years.  Dr. Nagao mentioned one patient in Japan has had DRESS Syndrome for over three (3) years!  When DRESS Syndrome has "fizzled" out and the inflammatory responses slow down the patients were left with an auto immune disease.  And, there is NO cure.

This was not the news we had hoped for.

Several weeks later we received a phone call from Dr. Nagao with the results of Colin's blood draw. Although the probability of having t-cell lymphoma is extremely low, they have discovered that Colin has too many inflammatory t-cells and unusually low regulatory t-cells (t-regs).  (Regulatory t-cells help manage inflammation).  Sadly, there is no medical treatment for t-cell therapy to increase his t-regs.  

This information was discovered by testing Colin's DNA and his genes. There is a specific gene that is affected and the root cause of this is most commonly found in Asian populations.  Additionally, the triggered response from the offending drug (antibiotic reaction) was lower than expected - but "just enough" to cause this cascading event.  When Colin's blood is stimulated with Bactrim MULTIPLE responses appear.  In DRESS Syndrome patients only one (1) dermatological response is recorded.  However, Colin has had 11 biopsies - all with DIFFERENT presentations.  (TENs, SJS, Psoriasis, Urticaria, Necrotizing Fasciitis, Ichthyosis Vulgaris, Dermatomyositis, Eczema, etc.)

We always knew Colin is a unique individual, but this is quite unreal!  The chances of this very small, unnoticeable, unpredictable anomaly in his DNA, combined with the prescription of a very common antibiotic created dangerous condition to his health.  

So what happens next? 
The NIH will test Colin's blood for hypersensitivity to polypharmacy. (Use of multiple medications)
During his visit to Orlando for convalescent leave he has flared twice.  We have had to alter his medication and begin monitoring his vital signs even closer than before.  If he is still presenting a rash when he returns to Walter Reed, he will have additional biopsies performed.