3/28/17

NIH: Part II

Good morning. 5:30. I'm awake and here with your daily (ha) NIH update. I'm currently waiting for the blood stick to fully draw me from my half conscious state and back into the world. My room is empty and quiet, a welcome departure from the city hospitals, where privacy and sleep are nearly impossible.

I still can't sleep here, but that's not the hospital's fault.

I did some decorating yesterday, a lame excuse to buy all the dumb stuff at the gift shop I'm sure my parents would refuse me when they come. Take, for instance, this doll of Teddy Roosevelt. I mean, which other hospital could possibly sell a tribute to our 26th president? Not Mount Sinai, that's for sure.

It's only my second day here but I promised updates and though they may be as dry as the four graham crackers I just shoved in my mouth, hang in there.

On Sunday, I said goodbye to my girls and headed to Metropark, bound for DC.

I barely made it off the train in time due to an ill-advised moment of Instagram stalking. I I basically crowd surfed above an angry mob toward my suitcase, just in time to stand clear of the closing doors and enter the gorgeous Union Station.

I got into a Lyft with a deaf driver who did not appreciate my attempts to finger spell direction and finally texted me to stop trying to use ASL. I responded with my newest sign: bullshit. JK grandma!

I made it to the National Institutes of Health after a thorough security check and settled into a far off wing they'd resuciatated for patients after a water main break. I was thrilled when they called it the Ebola unit because that meant no roommates for me. And since my last roommate was fond of crapping the bed literally every 45 minutes, I gazed upon the empty bed next to me with teary eyes.

So: why the f am I here again? Didn't I spend most of February in the hospital, not losing weight despite vomiting everything? Aren't I amazing about not being bitter motheruckingsonofabitchskinnybastards

I came down to begin a drug called ruxolitinib. Thie drug has recently been used in a handful of patients with my STAT1 genetic mutation. The hope is the drug stops some of the mouth sores, the infections and the general malaise and fatigue that awaits me every day. It'd prevent aneurysms and generally save me for a bit. Sadly, it would not rid me of Type 1 Disease. That bish is here to stay. Dammit.

Unfortunately, for the last few weeks, I've had extreme jaw and mouth pain. Yesterday I saw the dental clinic. The doctors there decided I need IV antibiotics and the experimental treatment I traveled here for will have to wait until my mouth heals.

This means that my two week stay may be more of a three week stay which a huge bummer because I'm on day 2 and I've already anger colored all my coloring books in a manner in which suggests I need occupational therapy.

So the plan is to get a line in today and go from there. I'll also meet with pre-anesthesia and tomorrow they'll Michael Jackson dose me while they pull my last remaining teeth left.

In the mean time, thanks for the love! 

Love you all, in vary degrees of appropriateness.

PS: many of you have asked for my NIH address:
NIH address: Kelly Bergin National Institutes of Health 9000 Rockville Pike Hatfield Building , Unit 5NE, Room 5-2412 Bethesda, MD 20892

2/10/17

What Happens Now: the NIH Special Edition Blog

Last week at the National Institutes of Health, my team of doctors met with my parents and me for a wrap-up meeting. It had been an incredible week, full of great attention to each of my symptoms. We sat down after 5 days and listened to the team of doctors and their plan to treat the genetic mutation I and 100 other people in the world were born with. Yes, only 100-200 other humans on this planet have this mutation! I always knew I was special. *Here's more about Primary Immunedeficiency disease: http://primaryimmune.org/about-primary-immunodeficiencies/

The short summary of this fatal condition is scary. Some with this mutation have severe disease and as a result, have died as children and young adults. Those with mild disease have lived into their 50s. And those with moderate disease fluctuate in their prognosis. I have a moderate/medium fry sort of disease, perfectly mediocre, just like my grades in high school.

There is no cure but there is a bone marrow transplant option. It's as close to a cure as possible, which is cool, BUUUUUT there is a significant risk of terrible side effects, and my doctor (an expert in BMT and Stat1 GOF) has seen patients die of infection after getting new bone marrow. A bone marrow transplant requires the immune system of the sick patient to be completely replaced by their donor's, but it doesn't fully erase the mutation. However, the transplant may end up being necessary for me. 

A BMT would require at least 60 days inpatient. And then I would temporarily live down here for awhile in patient housing as I recover. Because of the nature of my disease, a bone marrow transplant is riskier than it is in most cancers. There's the risk of graft versus host disease and death if the transplant does not take and a new immune system fails to thrive. Scary stuff, I know! It's possible I won't need one if these new treatments work but it is absolutely something that is on the table and on a 6 month to a year timeline. The risk in waiting is that I could continue to pick up infections that have irreparable damage. Patients with this mutation also have an increased risk of cerebral hemorrhage and they found a tiny aneurysm that is too small to worry about for now, but it will be followed up every 3-6 months with MRA scans.

The good news is that we don't have to jump to a BMT right away. But first, we're gonna try a drug given to multiple myeloma patients. That's where we will begin. I'll be back inpatient for a week this month to see if this experimental treatment might delay the transplant.

In other news, my organs are all in decent shape which is great. Nothing is an emergency right now, and everything is going to be super closely monitored. My doctor told me a bucket list is a good idea but also told me not to freak out and think i'm about to die. Which I totally only do once a day hour minute WHATEVER MA

On my last morning the psychology team came in to see how I am dealing with the news. It's probably the denial but I feel OK about it. Obviously the mortality rate scares me but if it works, my life would dramatically improve. Nothing can reverse some of the damage that has been done, and type 1 diabetes is here to stay. But it would likely rid me of the constant infections and inflammation that wear me down and keep me in the hospital. I'm very grateful to have the team that I have, here at NIH and at Mount Sinai. One of my NY doctors, Edith, even came down for the day yesterday to see what they had in mind and to see how I was doing. I feel extremely confident that we aren't rushing into this. The timeline gives me generous amount of time to travel and see a few things in case I'm isolated from the world for 3 months.

To sign up to be a donor, please visit BeTheMatch.com. The initial test is just a swab. If you do match, the procedure is unpleasant but not terrible. Plus you'd be saving a life. MYYYYY LIFE! (JK.)

And to help support my personal bucket list and the First Descents organization, which has brought so much joy and meaning to my life as things have gotten harder, please visit my fundraising link. I plan to walk a 5K and I'm excited to raise money for my favorite organization! https://support.firstdescents.org/fundraise?fcid=441725

As always, thank you so much for your love and support. I have so much to live for and I will fight, fight, fight as long as I can.


Love,
Kelly