Alpha-1 Awareness Month
May 13th, 2005 by GoldFalcon
May is Alpha-1 Awareness Month!
Learn about the disease here
Even More Here
Does someone you know have a chronic breathing problem that isnβt responding to treatment? They could have Alpha-1 Antitrypsin Deficiency, an underdiagnosed genetic disorder that can affect the lungs or the liver.
Researchers estimate that up to 100,000 adults and children in the U.S. have severe Alpha-1, and 2.5 million people nationwide may be carriers. Surprisingly, many medical professionals perceive Alpha-1 to be rare, so it is often misdiagnosed. An alarming 95 percent of people estimated to have Alpha-1 have not been identified.
Symptoms of the lung disorder include shortness of breath, chronic cough and reduced lung function. The World Health Organization recommends testing for Alpha-1 for anyone diagnosed with COPD, asthma or other breathing difficulties. Alpha-1 can also affect the liver; it causes liver impairment and failure in both children and adults, and it is the leading cause of childhood liver transplants.
*personal story*
For my own part, my diagnosis in 2002 has been something of a relief.
I had never been a partucularly athletic kid, and I struggled getting my run times down to “paratrooper level” (< 7 minutes a mile) but by 1996 was scoring reasonably well on the APFT (Army Physical Fitness Test) and was set to go to PLDC when things started going haywire. My PT scores started to fluctuate wildly, somedays I could do 80 sit-ups in 2 minutes otherdays I would struggle to get 50. My run times would fluctuate between a 12'30'' 2-mile to 15 minutes for the same distance. More than that I was struggling with severe chest pain that would last for days after a run. Come to find out I had pericarditis, an inflamation of the lining of the sac around the heart. No particular reason for it, but it wouldn't go away, after many trips to sick-call I was eventually labled a "nut" and moved from my squad leader position to the Battalion Transportation NCO's slot, where I stayed until I seperated from the unit. I felt short of breath when I ran, but no more --I assumed-- than everyone else did. I transferred to the Washington Army National Guard in 1998, where I held a full-time position as an assistant recruiter (I was an E-4(p)), I kept working at my PT, still with lots of pain.
My goal was to join the 19th SF Group (WAARNG) but eventually decided that if I was going to put myself through SFAS and the Q Course and SERE school that I might as well do it back in North Carolina as Regular Army, so back to Ft. Bragg I went. I had been to several doctors about the nagging chest pain and decreasing PT scores, all of them had said I was in perfect shape, nothing wrong. A couple went so far as to sugest psychological or psychosymatic causes. Thus, when I went back to Ft. Bragg in 1999 I decided that my pain and ever slower run times were the result of my own lack of mental toughness. I resolved to push through it all, to live up to the standard, to keep going no matter what.
Within 3 months I had collapsed on several runs and failed an APFT run. Slow run times had kept me from going back to my beloved 2nd Brigade, 82nd ABN DIV, so I was assigned to DISCOM (the DIvision Support COMmand). This was probably the lowest point in my life. The doctors could find nothing wrong with me, my chain of command thought I was goldbricking, and I was starting to believe that this might all be in my head. Maybe I was imagining it all. In August 0f 2001 I was medically discharged from the U.S. Army and rated at 15% disabled. The diagnosis was "chronic costochondritis", which my doctor told me was what they put down when they didn't know what was wrong.
He also suggested I see a mental health professional.
In February of 2002 my body reached a breaking point and I spent 5 days in the VA Medical center in Walla Walla. The fantastic doctors there took one look at my chest x-rays (x-rays that I had had probably 2 dozen times in the preceding 6 years) and said "Why, you have emphysema!". A quick blood test later and I found out that I wasn't crazy, I had a genetic condition that attacked my lungs, and had affected me since my birth.
Why hadn't the Army doctors noticed? A few reasons:
1) Paratroopers run a lot. This causes their lungs to increase in size, much like swimmers. One of the key indicators of emphysema on a chest X-ray is increased lung size, but this was a normal thing at Ft. Bragg, so it never raised any flags.
2) I was young, and even though my X-rays show that my diaphragm was almost completely flat in 1996 --indicated by the bottom of the lungs not having a concave shape--(another indicator of emphysema), the possibility that a 24 year old paratrooper could suffer from emphysema was never considered.
3) Alpha-1 Antitripsyn deficiency is not well known in the medical community and is considered a rare disease.
The fact is, the blame doesn't lay with my Army doctors (or anyone else, really), most people dignosed with Alpha-1 report that it took 6-9 years to be diagnosed. It's not an easy catch. My disability (now 60%) comes from the fact that the failure to diagnose the Alpha-1 for six years removed many more years than that from my lifespan as I was engaged daily in activities that were harmful to my lungs, activities that I could not refuse to do.
So what should you look for if you think you might have Alpha-1? I can only tell you my symptoms:
-Extreme shortness of breath when doing strenuous activity, so much so that it feels as though you are choking to death.
-Blackouts and tunnel vision while running or doing strenuous aerobic activity.
-Chest pain while doing strenuous aerobic excercise that last for days afterwards.
-Extreme fatigue.
One thing I will say, I never noticed my shortness of breath as abnormal, though I now know it was. This is the same sort of thing you might see in a person blind from birth. I have always been short of breath, so I didn't know that I was short of breath. This also complicates diagnosis. My diganosis in 2002 was such a weight off of my shoulders, even with the poor prognosis, because I knew what I had, knew that I was sick, knew that I wasn't crazy, knew that the doctors had been wrong and not me.
The Bad News:
-Alpha-1 is usually (nearly always in adults) fatal.
-Alpha-1 patients quality of life cannot be significantly improved.
-Alpha-1 is still exotic so finding a good doctor can be difficult.
The Good News
-Alpha-1 is not near term-fatal, most people can live a decade or two after diagnosis.
-New medications to slow the disease have been introduced.
-More end-stage options exist today than a few decades ago: lung reduction, lung transplant, etc...
-There is an active community dedicated to spreading awareness of this disease.
If you have it: GET YOUR FAMILY TESTED, especially parents, siblings, spouses and children.
May is Alpha-1 Awareness Month in many states across the country. The Alpha-1 Association and Foundation, two nonprofit voluntary organizations, are dedicated to identifying those affected with Alpha-1 and improving the quality of their lives through support, education and advocacy. They are hopeful these efforts will save lives and improve the quality of life for millions around the nation.
That was very interesting. I’d never heard of this before, think I might link to it. I can easily see how difficult the condition would be to diagnose.
Toni,
Thanks so much for your comment (and for your link). I try to do a balancing act here on the blog about my disease. I don’t want to ignore it, don’t want to dwell on it either (at least not on the blog). I will probably explore it further in future posts just because it is so underdiagnosed. The number one comment I hear (and the most exasperating) is “You look so healthy!” Unfortunatley you can’t tell if someone has Alpha by looking.
good story, that is, the way you tell it. Unfortunate that you have it, but your mental toughness and never-quit attitude probably masked some of severity of your situation. I can imagine how frustrated you were knowing that something is not right but not knowing what it was and pushing as hard as you could to get back to where you thought you should be…
Anyway, best wishes. There are always exceptions to the rule, and hopefully your longevity will be one.
So very sorry about your condition and that you had to suffer the added burden, prior to your diagnosis, of being told that you were fine.
If you don’t mind disclosing it, are you receiving any sort of treatment? Would you ever consider a liver transplant and/or lung reduction?
Thanks all for the kind comments.
I don’t mind discussing treatment, though I really don’t want to make it seem like I am depressed over this, or that I feel self sorry. I don’t. All things considered, it’s not a bad terminal disease to have if you have to have one. I am just trying to raise the awareness a bit, and not gloss over the very real consequences of the disease. I was so struck that the ignorance of emphysema is such that Ed McMahon, longtime friend of Johnny Carson, could say when Carson died “I knew he had emphysema but I didn’t think he was sick.” People think of it like it is a chronic cold or something. I often have to repeat “Alpha-1 Antitrypsin deficiency” to doctors and nurses because they have never heard of it.
I get infused with a synthetic Alpha-1 Antitrypsin replacement every two weeks via an IV. Takes about an hour and a half. I have 3 different inhalers that I take every day, these try to slow the progression of the disease. Lung reduction and replacement are both options and I am in the preliminary process of the lung transplant list. I want to postpone those for as long as possible because the mortality rate becomes much more a known factor (50% mortality within 2 years after transplant). Lung reduction has better mortality rates but is less effective, but is still no minor surgery. My liver is still only slightly cirrhotic so a transplant will likely not be needed.
Glad to hear that you are receiving therapy. I hope it is slowing down the disease process for you and giving you some relief.
So, what is the current thinking about trying a liver transplant (even if the liver isn’t severely cirrhotic) in an attempt to normalize the metabolism? Obviously, not a minor surgery either, but is it ever done in adults?
Would you do a single or double lung transplant?
Reminds me of an 04 that started acting loony when we were at Ft. Bragg running ROTC cadets through their summer camp. Every one of us figured he had started using drugs. But Airborne Ranger Majors on drugs just are not that common and we wondered if it really made any sense. He lucked out and encountered a Doc who thought that maybe it might be something else. It was…Lyme’s disease. One of the first of many at Bragg to be diagnosed.
Yours was more insidious and even rarer. I hope your story gets traction and is shared, because you know you are not the only one trying to “push thru the wall” when your body can’t really do it.
Hello~
Glad to hear you are doing well. I am a mom of a 2-1/2 yr old Liver-affected Alpha boy (Hunter). Even though liver-affected my son has asthma and a minor-blood clotting discorder, which dr’s are saying it’s all linked to Alpha. I am a Liver Aide on the Board of Directors for our local support group “Alpha Pack” here in Milwaukee, WI. Of course, myself, husband, youngest son and stepdaughter are also carriers, making us ineligible for live liver donation to Hunter.
Catching this terrible disease early is crucial for treatment and even survival. Hunter’s labs keep climbing and they think eventually he may end up w/a liver transplant. But we’re also keeping our fingers crossed for the gene replacement/therapy.
Just hang in there and keep strong. By keeping a strong mind and being positive, your recovery and health will overall be strong too. I try to stay strong, but there are days I break down and cry because there’s nothing we can do. Alpha is a luck of the draw and I can only hope and pray that Alphas will have more treatment and maybe cure options in the very near future. Take care!
I just came upon this site. I have a good friend with alpha 1, and am always looking for info about this condition. He was diagnosed about 6 years ago, but just started weekly infusions this past year. He’s 52 years old. I’m hoping more options will become available for all of you.
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