Mortality

A very public advocate for PIDD issues passed away on October 6th. She had the same immune deficiency, diagnosed the exact same time age-wise (she's about 15ish years older), and seeing the same immunology practice.... We even have the same first name.

I don't know how she died, but at the end of June, at the IDF Conference, she looked robust and healthy and seemed full of passion about PIDD issues and the new Affordable Care Act provisions that would help us.

The news of this death hit me pretty hard. I see myself in her shoes and I wonder... is treatment much better for me than for her? What killed her? Will I die in a similar fashion? Who would I want to know? How would I tell them? Being that I live a good deal of my "disease life" online, I have to make sure that there's some way to let them all know when things happen to me. It's scary to think that so many of my friends exist primarily in my virtual world...

That's really beside the point of my post though. Seeing her obituary, seeing the things she's done, I'm proud to say I knew her and we were connected. I'm still frightened because I wonder if I will have a similar expiration date. I hope not.

I've Got Good News

I got some exceptional news! I have been contacting my congressman's office constantly about the Medicare IVIG Access Act and I found out that Rep. Moran has decided to sign on as a cosponsor!

Dear Ms. Miller: Thank you for taking the time to contact me regarding the Medicare IVIG Access Act (H.R. 1845). I appreciate learning of your interest in this legislation.

As you know, this legislation would establish a Medicare demonstration project to evaluate the benefits of providing intravenous immune globin (IVIG) in the home. This proposal has the potential to save billions of dollars for Medicare. I am pleased to tell you that I have added my name to the list of cosponsors to H.R. 1845.

Thank you again for contacting me.

Sincerely, James P. Moran

Huzzah! This makes me feel like I actually matter, at least a little bit, in the whole grand scheme of how things work in Washington. Maybe, just maybe, little people can sometimes have a voice.

There Are Some Things I Will Never Understand

I've been trying to recruit people to sign and pass along this petition about infant screening for SCID in Florida. It takes about 2 seconds to fill out and pass along. Or just fill out.

It has 324 signatures.

I received a notice about a friend supporting another petition. This one was about animal cruelty, specifically wanting someone to get the full punishment for hurting a dog. It's a negative petition rather than a positive one, in my opinion. It has signatures in the tens of thousands.

Apparently it is more appealing to punish someone who hurts a dog than it is to save dying babies. I don't think I will ever understand that. The kid who killed the dog will be punished in some way, maybe not the maximum sentence, but he will be punished.

Without public support, 8-10 SCID babies will die in Florida this year. The first one already died, the second is currently fighting for life.

I guess I shouldn't be too surprised. There was, after all, an animal welfare charity before there was ever one to protect children.

Hey! It's Me!

A few months ago, I went to tell my story at a briefing for interested congressional staff. The event was sponsored by the Plasma Protein Therapeutics Association. I am reminded because the briefing was written up in their summer newsletter called The Source. (Check the section called Inside PPTA)

In case you're curious, here's my story, as shared that day in March.

Good Afternoon.

First, I’d like to thank the IDF and the PPTA for allowing me to be with you today to share my story and I’d like to thank all of you for taking the time out of your schedules to listen.

I look like an average 20-something DC professional. I have the suit, the shoes, the hectic schedule, but a big part of that schedule is my Primary Immune Deficiency. I was diagnosed with Common Variable Immune Deficiency (CVID) just after my sophomore year in college. I receive IVIG treatment once a month at my immunologists’ office in Chevy Chase. IVIG is a miracle of modern medicine. At any given time I have the antibodies of 10,000 plasma donors protecting me. These donors give me an incredibly wonderful gift and I am incredibly grateful for their gift.

Looking back, there were signs when I was a child. I was always small for my age, was a failure to thrive as an infant, and had some really strange infections, including a big skin thing on my forehead... in the 5th grade. Totally tragic for me, at the time. Overall, though, I was a pretty normal kid and nothing seemed to be amiss until my freshman year of college.

I went off to Washington and Lee University, looking forward to a bright future and a great education. About midway through the winter semester, I felt terrible. I figured it was just the “winter term blahs” as we called them and that I’d be fine. I just needed to work hard and finish the semester. Spring term would be easier.

Spring term was easier, because I could sleep all the time between classes. My friends were outside, enjoying the beautiful weather, and I made my dorm room into a cave for constant napping. I finished the year and packed my bags to work at a Christian Appalachian Project camp in rural Kentucky. I just assumed I was stressed out and that’s what was making me so tired. I went to the family doctor before I left, because I felt I had a sinus infection. The doctor gave me antibiotics and sent me on my way.

I felt good for the first 2 weeks of camp. At the beginning of the 3rd week, I became delirious with fever and landed in the ER, 45 minutes away from camp. From there, the doctor admitted me. A few days later I was transferred to the ICU at the University of Kentucky where I stayed for 11 days while they ran tests and tried to get me well again, at least well enough to leave the hospital and go home to Virginia. They put me on high-powered antibiotics that finally kicked the infection and I was released without any answers about why my pneumonia was so severe.

I began seeing a pulmonologist back near home at UVa who wanted to understand my pneumonia and why it was so intense, given my limited history of illness. He kept me on antibiotics for the start of the fall semester, until a few weeks before my winter break. I got pneumonia a week after I stopped antibiotics. After that, I underwent extensive testing that included a PET scan, bronchoscopy, thoracentesis to test the fluid around my lungs, and bi-monthly CAT scans to monitor a spot on one lung. That lung was later biopsied and it was found to have a massive pocket of infection the antibiotics couldn’t touch. Yes, I was still taking classes in spite of everything. My life consisted of eat, sleep, class, doctor’s appointments, and rounds of medical testing.

My doctor at the time reached his wit’s end. He’d never before seen any case like mine. He presented my case to colleagues and got no answers. As a last resort, he ran a fairly simple blood test on my antibodies and we found our answer. In his 30 years as a physician, he’d never had a patient with a Primary Immune Deficiency.

I was diagnosed with CVID and, after my fourth pneumonia in a year, I was admitted to the hospital to begin IVIG treatments. That first treatment was horrible. I had an anaphylactic reaction to the product, due to the lack of Immunoglobulin A in my system. Since then, we’ve learned to mitigate the reactions through a cocktail of pre-medication.

Thanks to IVIG, I have only been hospitalized once since I started in the summer of 2003. I was able to return to and complete college in 4 years, graduating with distinction in 2005. I have had a full-time job and an active social life. The only difference between me and most of you is that I miss work once a month to receive IVIG, am on a first-name basis with my pharmacist, and get call-ahead service in the Emergency Room.

I arrive at my doctor’s office at 7:00 AM on treatment day because I could be there for 3 hours or 8, depending on which product I receive. Right now, I’m on a product that infuses in 3-4 hours and this product makes me feel like I could take on the world. My friends lovingly refer to it as my “go juice.”

Prior to last month, I’d been on another product that isn’t as effective for me and does not keep me healthy. It also causes flu-like symptoms in me – massive headache, body aches, malaise. It’s very much like getting hit by a truck once a month and having to go to work the next day. Unfortunately, that’s all my insurance would cover at the time. I was still getting sick, but that did not influence the decisions from the insurance company, regardless of my physician’s recommendations. Fortunately, I became a permanent employee in November and have managed, 3 months of struggling later, to finally work out getting my preferred product.

Why does the product matter? Because I still get terrible infections on some brands of IVIG and I miss out on a lot of things when I don’t feel well. Because of the ineffectiveness of the last product for me, I’ve missed almost 3 weeks of work since September. I almost missed my sister’s wedding. Luckily, my fever broke sometime during the night before the ceremony and I was able to participate fully.

Getting that better-for-me product was quite a challenge and involved two and a half hours on the phone with the specialty pharmacy, insurance company, and my doctor’s office to ensure that my product would be covered, and that I would have it in time. Now, I make 3-4 phone calls a month to make sure that everything will go smoothly on treatment day. A call to the specialty pharmacy to initiate the refill and pay my copay, a call to the doctor’s office so they can initiate delivery, a call to the doctor to confirm delivery date, and a call to confirm the actual delivery of product the week before treatment. There is a lot of juggling and scheduling that goes along with having a chronic illness. It has made me a lot more calendar aware and organized. I have to plan my life around treatments. I try and plan trips to be right after treatment, so I can better ensure I’ll be healthy through the whole trip.

Without IVIG, I would spend much more time in the hospital. I wouldn’t be able to work and there’s a very good chance I wouldn’t be sitting in front of you today. IVIG makes me just shy of normal. I still get fatigued, I still get infections, but I can pretty much lead a normal life. I am very fortunate to be receiving the product I want in the setting I want. Most patients don’t have this luxury, and some can’t even get IVIG at all. For some, it’s a month-to-month insurance battle. For some, they’ve had to call state regulators and their congressional representation to get their IVIG authorized. I have friends who wound up in an ICU because their insurance decided they needed to do a trial off of IVIG for 3 months, despite the objections of their physicians. I recently learned another friend became septic due to infection while unable to receive IVIG. It’s scary to know that I could lose access in the way that I’ve seen others lose it, and even scarier to know that it would kill me. I am extremely lucky, but I wish I didn’t have to be.

It was quite a rewarding experience and I would definitely recommend this kind of advocacy to others.

Medicare IVIG Access Act

Just a quick note to encourage you to support the Medicare IVIG Access Act. Basically, this bill would fill the holes in the current IVIG benefit for Medicare recipients. This way, they'll be able to receive treatment in the home setting. The best news? Not only does it offset the costs of the program, it also offers a net savings to the federal government. Win win! Unfortunately, my community is small, so it's hard to get the attention of members of Congress... But with YOUR help, we can. Please take a moment and contact your members about this.

Thanks!

PS - The IDF supports this measure. More info can be found here.