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Who Has Submitted?
Last Name: Duttweiler
Personal: H height 6' 5", weight 258 lbs., age 48, Marital status: married to Eileen; Military service: U.S.
Army 1967–1970; Nam 1968; Sex: male; Place of birth: Syracuse, New York
Alias: D’MAN, PeterD, D’Meister, Dutt
Where does one begin after seeing so many beautiful, creative articles already composed and presented in front of me? I have been spoiled by having the privilege of being the editor for The Alpha Atlas and seeing the contributions first hand if you will. So who the “hell” is this guy they call PeterD on-line and read about in the Alpha-1 Newsletter. Good question as I have been asking that of myself for decades.
Back in June on the 13th (Superstitious) of 1948, I was born in Syracuse, New York. I was one large baby and can remember being a thorn, if you will, in my mothers’ side as I was always one to ask why something is or was and try to take it apart to see what made it tick. I don’t recall many major respiratory problems when I was going through my earlier growing stages.
If we fast forward to 1967 that appears to be when most of my problems started. It was here that I was introduced to the U.S. Army, heavier smoking, bureaucracy and Vietnam. There never was a pulmonary function test given at the military induction physical. They said, “You’re a-okay son and we’ll make a man out of ya.” They trained me as a wireman which meant I had to climb poles, however, in Vietnam there were none. Learning the Army’s ways at an early stage. I do remember being lightly out of breath on climbing these poles, with spikes and full gear on, but thought nothing of it because of the newness of the occupation, training and at that time, “No one in their right mind would ever mention their shortcomings.” You see we were “honed” as fighting units. As strong as the weakest link and all that good stuff.
In 1968, I had the pleasure of going overseas to Vietnam for the whole year. That was the year of the “TET” offensive and probably recorded as the most active militaristically during the war. I would have times when we were attacked and enemy incoming rounds or satchel charges came into our base camp when I would lose my breath running for cover. Did I put it together then? Did it register then? No! I figured it was a natural response to fear. Mind you Vietnam or the Army as a whole isn’t exactly a place setting for a formal dinner. I can’t and won’t tell you about all the dirt, fumes, smoke, Agent Orange, fuels and extremely bad weather conditions that I was exposed to. Trust me when I say, yes, the stage was set for heavy environmental exposures.
That isn’t the best scenario for a young Alpha-1, considering the stressors of war and the Army giving out “free,” that’s right, free cigarettes in the C-ration meals. Talk about sublimation via direct and indirect motivations. These factors together (war and tobacco), along with the showering of deadly particulate and environmental onslaughts, kind of set me up to lose lung tissue.
Lose lung tissue I did. In 1974, I had (PFT) pulmonary function tests that showed my FEV1/FVC reading as 55%. That was at the age of 26 years. I showed reversibility with puffers and you know what the attending doctor said? No meds needed at present and good day, Mr. Duttweiler. There wasn’t a serum level test given nor even a basic request by this doctor to have me report back at a future date for follow up. Of course nothing’s wrong. You have 55% left, you can function. You really got to love it. On the advice of this gentleman, out the door I went!
So, I felt everything was just fine. It wasn’t. I continued to do my thing and we know that the outcome is going to be losing lung tissue rapidly, because they are taking no preventive steps to address the underlying medical issues at hand. Meshed in the seventies and early eighties, I went through the pattern of what you have seen in many of these articles: the colds, sore throats, bronchitis, and upper-respiratory infections. Each one taking a small chip out of my total capacities.
After the military, I went to college and got my Bachelor of Science in Criminal Justice with a minor in Psychology. I never really used the degree for all intents and purposes. I was a Federal correctional officer in Danbury, Connecticut and recall many instances of being short on breath. If there was trouble in the prison, you had to get to the source and to it fast. Your coworkers depended on you. To get there you would have to walk or run. Christ, if I ran, by the time I got there I wasn’t much good anyway because I was far below reserves for air left. We had to take physical body (inmate) counts fours times a day and that involves just walking and talking. I had trouble doing that due to shortness of breath! Of course the stress of the lads incarcerated didn’t add to my merriment.
I left and went to the Postal Service figuring it would be healthier on my system and my “life” overall. Dog bites versus inmates made the choice easier. So I still stayed with Uncle Sam, smartly building up time and became a letter carrier. You guessed it. Out in the elements again and the cycle intensified. The colds lasted longer, the bronchitis lingered longer and asthma was finally diagnosed. Great I have asthma. That’s why I’m sick all the time. I had to take a lot of time off from work and that didn’t please the Post Office. They don’t want to hear this “crap.” After all, you probably are just milking the job.
I went in on days that I shouldn’t have because of how I felt or the weather conditions were terrible. Either way my health was at stake. I had some routes that were quite hilly and sometimes I had to sit, anywhere, because I was hyperventilating and short of breath. Asthma, right? I would have to stop between blocks to catch my breath and then it got so bad that I would be stopping between every other house. This was sad. I wasn’t a happy camper. Again the Postal Service looked at me as shirking my responsibilities.
Finally, enough was enough. I transferred inside to work where I was met with loading docks, breathing in diesel exhaust fumes, pushing (exertion) all your Christmas letters and packages around, changes of temperature on the docks and whirling machinery kicking up the smallest microns of dust imaginable. At the end of the twelve-hour days, I could barely talk as my system was shutting down and my lungs inside felt like someone has taken them out, sprinkled cement dust on them and reinserted them. There was actual physical pain upon breathing.
I’d had it! I left in January of 1992. I put in a compensation claim and have yet to have it adjudicated favorably. The Post Office is claiming that all my problems ended when I punched out that last day of work. That all problems temporarily accelerated on the job (both as a letter carrier and inside) and that they (Postal Service) and their cronies at the Federal Workers’ Compensation Division, have no further culpability because there are no “residuals.” No residuals you say. I can’t get the air out!
Here is my story it’s sad but true. In the end I will win. I’m convinced I’ll win, but look at what I have had to sacrifice. It has been more than five years without a dime being compensated. Why if it wasn’t for my wife, Eileen, then I would be down on the street selling apples.
The Post Office even tried to remove me from duty because of my inflictions. They sent me to their doctor for a physical for continued employment. He did a pulmonary function test and it was low but gave me clearance to continue working. I found out later that the doctor in a private letter asked the Postal Nurse if they had given me two pertinent tests. One was an IGE level and the other, you guessed it, an Alpha-1 Antitrypsin level test. They never disclosed this request by the doctor to me. I had to find out by initiating a Freedom of Information request.
My personal doctor during the latter 1980s was becoming suspicious, too. He sent me to a pulmonary specialist where we went through all the tests: serum levels, arterial gases, diffusion, the full gamut of PFT’s, and the phenotyping. My serum level was 26 and I came back the infamous ZZ. Oh how I would learn to know that letter of the alphabet . . . ZZ. I didn’t screw around either, homozygous, at that. Now what?
The year was circa 1990. They told me about Prolastin, HIV/AIDS, Hepatitis B and how that it wasn’t a cure all but take what you can, Son. I wasn’t too thrilled about all that stuff and “extra” baggage that came along with Prolastin so I said, “Let me think about it Doc. I will get back to you.” I thought about it for three years. Sound familiar?
During August of 1993 I went into my local Veterans Administration Hospital here in Syracuse, New York and asked them to start me on Prolastin. You see I had done my research and told them what I wanted. They approved it and I’ll never forget the first (almost) infusion. I had demanded that I be immunized for Hepatitis B and they said, yeah, yeah, yeah. Came around time for my first needle prick and I told the attendant that they had not immunized me for Hepatitis B and that I’m refusing the Prolastin. He said, “You can’t do that, it’s already mixed.” I said, “You might as well dump it down the sink. I’m not taking it.” This didn’t sit too well with VA Administration, but I was right, I knew it, and held my ground. I started my HEP-B immunization series the following week.
I receive 26,000 mg’s of mellow yellow every two weeks. I feel bloated as hell after being infused and remain dizzy, (Eileen says I was dizzy before Prolastin) with shortness of breath for a day or two afterwards. Other than that, no major side effects. I’m very upset about all the bad-lots of Prolastin being recalled since 12/94 due to suspect Creutzfeldt-Jakobs Disease, nicknamed CJD. I have been infused over forty times to date with bad-lots and this is definitely screwing up my party with life. That plus it really is a “crap-shoot” on whether or not Prolastin is working. Every day comes and goes and I honestly don’t feel any better. There always remains that lugging or heavy bakers’ dough feeling in the pits of my lower lungs. It isn’t normal . . . you know it and I know.
I try to stay on top of things through the Internet and communicating with my peers. I have self-educated myself both informally and formally in computers, went back to school and got my Masters degree, a supplemental degree in Labor studies from Cornell (I was active in unions as I like to help the little guy), and amazingly my Commercial Truck drivers (CDL) license. I strive to get the word out about our infliction and help coordinate the Central New York support group. I’ve been in the papers, (yes, for good things about Alphas) on the local television stations, and you will see an occasional posting from Moi’ on the Internet.
Inhaling many puffers and I don’t feel as they do a world of good, but we are between a rock and a hard spot, now aren’t we. Fought my butt off with the good folks down at Federal compensation. They decided that after three years of battling, since we haven’t given Peter that dime in compensation, the very least we can do is supply him with oxygen. I get such a warm glow . . . I take Azmacort, Atrovent, Proventil, pills and thrills and it gives me chills. My FEV/1 hovers around the lower 100s (1.15) depending on my mood, weather and how hard I feel like blowing into that mouthpiece.
My immediate family has taken the serum test and I have two sisters that are MZ carriers and a brother who has deficient ZZ alleles, as I. David, my ZZ brother, has no lung damage. I was blessed with it all in this family.
Talk about babbling on. Let me end so you can get on to reading other offerings in this project by saying that I’m very lucky to have a wife like Eileen, who tends the fields and harvests the crops. She never whimpers publicly about her added responsibilities and truthfully, she keeps this ship afloat. Thank you my dear wife, Eileen.
Other than that, everything is just fine and my attitude is overall, probably carefree, for lack of a better word. You can see the truth between these lines. I will say this, “I do love life dearly and truly appreciate what I have, for yes, it could be worse.”
It was my distinguished pleasure to be your editor for this great Alpha Atlas Anthology. Thank you and I really don’t know who that is in the picture above with the sunglasses on. A strange person would be my guess!
~ fini ~
Last Name: Spink
Title: Lung Alpha ZZ
Hello, my name is Anita and I was diagnosed with A1AD in 1994. I was driving home from work by myself and for no reason had a breathing attack. I pulled the car over and passed out. When I finally woke up I went home and told my husband at the time that I needed to go to the hospital. I explained to him what had happened and to my shock he did not believe me. Well, it only took about one hour and here comes another attack. This time he had to call the EMS. I went to the hospital and they did treatments, drew blood, and set up an appt. with a Pulmonary doctor. Well, I was a very active person. I work for a fast food restaurant as a manager, went country dancing one night a week, went and did things with my children and grandchildren. I did not think anything about this at all. I did not have any more attacks not even when I went to the doctor. Know I had been smoking all this time since I was 16 years old. Here came the appt. time for the Pulmonary doctor and he ended up giving me my blood test results as to what my condition was. Well, I went into denial and kept smoking cause they were wrong. I was not old enough to have emphysema (A1AD) and really did not know what it was. So I kept smoking and going on with work and other things.
Well, my husband decided that he did not want someone that was sick or anything like that so he went his way. I met a guy who later on I moved in with but he had thirty cats and they were all show cats so that was interesting for me. I kept working until 1996 and showing cats, but the main factor in this is I kept smoking. Well, due to my own fault and denial, I kept getting worse and this guy did not believe me either, due to not really being around that much cause his company kept him traveling, so he moved me and cats out to the hill country. Well, we had one friend there that would check on me cause she knew my condition, but by then I was on total disability and could not do the things that I was used to doing. Even doing the cats’ shows and taking care of them was getting harder. But I still could not give up those darn cigs. Well, my youngest daughter started to stay with me out there cause she and her boyfriend split up and that is when she started helping me. Well, I finally split up with this guy and came back to San Antonio in 1998 and quit smoking for six months so I could be evaluated at a transplant hospital in Dallas. Well, I was honest and told them I took two puffs off a cigarette three months prior to the testing to see if I wanted to smoke. Well, the doctor put down that my heart was bad and I told him that I was smoking two packs a day. So I had to wait six more months, then reapply to the same hospital and even though I had not touched a cigarette for almost a year, they refused me by saying that my heart had gotten worse.
I was put on O2 by this hospital in 1999, with walking level of low 80s. Well, depression set in and I just did not care any more, but my kids would not let me give up. So I got onto the Internet for Medicare approved lung transplant hospitals and found that the one in Houston and was just approved in 2000. Well, I called my insurance company and told them that I wanted a second opinion and they said they did not have that hospital listed as a Medicare-approved transplant facility. I told her where I found it and she went and added it to her list of places and then sent all of my paper work to them in 2001. I then received a phone call from them to come to Houston to be evaluated in April or June of 2001, but they got all that rain and had to cancel until the end of July. So my first week was July and second week in Aug.2001. One week later got a phone call and a letter stating that I was accepted as a double-lung tx patient. I have had my ups and downs, but with the help of my children and them not letting me give up, I have come a long way. I know things can happen but with God, my new found family with other Alphas and this hospital and my children, I know that I will survive either way. The doctor here that I have is really leaving most things up to the hospital in Houston unless I get real sick, but sometimes I really have to point out things to him that sometimes he overlooks. But other than that I cannot ask for better. God led me to the Alpha site, my children, the hospital, and the doctors so if it is to be, then he will lead the way for us. And I believe that if I don’t, then I will be someone’s angel to help lead their way or my children’s way. May God Bless all of you.
Anita Spink
First Name:
Bill
Last Name: Miller
Title: Lung-affected alpha
Personal: Age: 41; Sex: Male
I had “asthma and bronchitis” from birth, with cases of pneumonia at age five and six. I lived with poor respiratory function throughout my early years. At about twelve years old, my asthma seemed to just go away. Of course the next thing I did (being the typical know-it-all pre-teen) was to start smoking. That went on till I quit at age 32. I was diagnosed with alpha-1 in 1993, after another case of pneumonia. My “lungs of a seventy year old,” coupled with the fact that my older brother had died of emphysema at age 42, led my GP to test me. My level was low, but I never kept track of the numbers from back then. I’ve switched doctors since then, and my last level (before starting Prolastin) was 21. I’ve never had a phenotype done, and I understand it’s impossible while on Prolastin. I’ve been on Prolastin since November 2001 and seem to be doing well with it. I’ve led a pretty active life, all things considered. I was an auto mechanic until I joined the Coast Guard in 1983. I served five years in the CG, honorably discharged in 1988. I was a factory mechanic from then till about 1992 when I became too sick to work full time. I made do with odd jobs until I was deemed disabled by Social Security in 1995.
I hunt and fish, as much as I’m able, though that hasn’t been much the last few years. I live by myself, so I have to do all the household chores, which keeps me busy. Thank God I’m still able to do that much. I recently saw a new pulmonologist, who said I need a lung transplant. I’ve been going through tests since January to see if I can be listed. I’ve not seen the doctors from the transplant center yet, just my lung doctor and the specialists he’s called in. I won’t know anything for awhile about when or if I’ll be listed for transplant. My last PFT showed a FEV1 of 16% expected, and I’m on 2 liters OČ overnight and with exertion. That’s my story so far, I sure hope to have more to add to it in the near future.
First Name: Noreen
Last Name: James
Title: ZZ lung
Noreen James.
On December 6, 1996, I had a respiratory flu. I was 34 years old and had just quit smoking. The flu lasted over two weeks. My family doctor referred me to a specialist, a Pulmonologist. His diagnosis was that I had asthma. X-rays showed I had bulla on the lungs, large thin air sacks that were once healthy alveoli. The bulla signaled that I had emphysema, but because I was at such a young age, it was seen as no great danger. No specific tests were ordered. My doctor felt confident that my difficulty in breathing was due to asthma. Thus asthma it was, and I happily accepted it. Four years passed without any relief from the inhalers. By December 1999, I approached my family doctor and said, “I still can’t breath, I want to see a specialist, . . . again!” After an insurance-referral nightmare, I was sent to see the same Pulmonologist I saw in 1996. At first, he thought a condition I had been diagnosed with earlier in life, Mitral Valve Prolapse, was sending mixed messages to my brain. Instead he decided to order a PFT, Pulmonary Function Test. I sat there in this icy PFT chamber as he looked over my readings. After a moment of silence, he turned to me and said, “OK, it looks like we’ll need to set you up for more tests.”
Since that fateful PFT, my doctor opened the floodgates that sent me awash into a sea of doctors, technicians, and cold offices. A specific test was also ordered to confirm a condition my doctor heard about at one point in his busy life. It was a condition called Alpha-1 Antitrypsin Deficiency, A1AD, where an enzyme produced in the liver is missing to protect the lungs. Well, after three months of waiting, the results were in. My blood serum was at 20mg/dl. A risk-free zone from emphysema is typically between 150–350mg/dl. I had A1AD. Oh, well, it didn’t sound too bad. I figured a few good potent vitamins every morning would make up for what I was lacking. It wasn’t until my pulmonogist sent me to meet with my thoracic surgeon, in April of 2000, that the risk of what I had came into the light. It was my day of dawning, so to speak, thus the reason for the nickname I graced him with, “Dr. Sunshine.” He placed my X-ray and CT scan up on a light board to show me my lungs. They were gigantic. I’m only 5' 9" on flat ground and my lungs were so big, I could have donated them to a 7' person. The bulla at the base of each lung, nearly reached the top of the pelvis. My diaphragm was squashed flat. Even my heart presented itself as being pushed farther over to the left to compensate for the lack of room in the chest cavity.That was a defining moment. I stood there frozen. I wasn’t quite sure how to focus on what he was telling me. Weren’t the moon, the stars and the sun all in their normal place when I woke up this morning? Did God change something when I wasn’t looking? How could it be that I was in the advanced stages of emphysema at the age of 37?An inner tornado began to rip through my thoughts.
Outwardly, I was still composed. That was until my surgeon said the words, “Lift and separate.” He wasn’t talking about a support bra. He was talking about surgically opening my ribcage to get to my lungs for an LVRS (Lung Volume Reduction Surgery). I went deaf, momentarily. Say what? I fumbled nervously through my bag looking for sanity. My hands became like ice. At that moment even a root canal seemed like an easier procedure to handle. With my shaken nerves, I was ready to ask him, “The local bar is where?” Since that day (I’m happy to say) I’ve had several less “white-knuckling” appointments, due to the fact that a doctor finally took me aside to explain to me what I was dealing with. It’s good to have the right battle gear to wear, especially when you’re told that Goliath is coming around the corner. I was no longer in the dark, but ready to take on the challenge in the clear sunshine.And then there’s my super hero. I bring him with me to most of those “white-knuckling” doctor visits, . . . he’s my Lar, my husband. Without him giving me a little push now and then, I wouldn’t even have contemplated creating a website called Spiderspun. He sets my mind at ease and tells me everything will be all right. And I believe him. Noreen James . birthdate 4/12/62 . female . ZZ lung . diagnosed April 2000 . AAT level 20mg/dl . FEV1 22% as of 2/28/03 Self-infuse Prolastin by I.V. (no port or pic). 3 LVRs 2000 through 2001.
FirstName:
Pam
LastName: Hawver
Title: Liver-effected Adult MZ Alpha
Pam Hawver and her Grandson Dean.
I was diagnosed in 1991 with Alpha-1 and an MZ phenotype. I was told at that time not to worry and that I would probably never have any problems from it. The doctor made me feel like there wasn’t anything to be concerned about so I pushed it out of my mind. I also suffered from sleep apnea and blamed my exhaustion on that. I slept with oxygen for almost two years and finally had surgery for the sleep apnea in December 1995. I was still exhausted all the time, but kept pushing myself to get through each day. In October 1997 I started having problems with rectal bleeding. After many months of tests, I was sent to see a gastroenterologist who then referred me to the MUSC Transplant Center and told I would probably need a liver transplant. By that time I was suffering from ascites, an enlarged spleen, hypertension, and esophageal varices among other things. I was treated four times for esophageal bleeds over the next year or so. I was in and out of the hospital over the next two years. I was able to maintain on meds until November 1999 when, while in the hospital, they wanted to go ahead and work me up for transplant. The only problem was that I had to wait until after January for my insurance to change so that the work up and subsequent transplant would be covered. I convinced the doctors that I had to wait since my current insurance wouldn’t cover anything to do with a liver transplant. They didn’t like it and didn’t want me to wait but understood my reasoning.
I was finally evaluated the first week of February 1999 and put on the liver list for transplant on March 3, 2000. I was called in April as a stand by, but was sent home that same evening. I was again called on May 26, 2000 at 11 p.m. to get to the hospital and this wasn’t as a stand-by this time. This was the real thing. I arrived at the hospital around midnight and was up all night having tests and being prepped for the surgery. My liver transplant started at 8 a.m. the next morning and lasted only six hours. I was taken to recovery and the tubes were all removed before I woke up. I was doing great. The only problem was that my new liver never kicked in. After 24 hours, it still wasn’t working. Three times over the next two days they performed a plasmapheresis (plasma exchange) to see if they could “jump start” my new liver. I had already been put back on the list for a new liver and given 72 hours to find one or die. Well, it finally worked and my new liver kicked in and started working and has worked wonderfully ever since. For a short time while in the hospital after transplant I had a problem with blood sugar but that went away before I left the hospital. A year after transplant, I had to be put on blood pressure meds. Then in March 2001 I joined a health club and started working out. By July of that same year, I was totally off the blood pressure meds and feeling wonderful. As of today I am off all meds except Neoral and synthroid. I feel so blessed and try to appreciate each and every day that’s been added to my life. I still work out 4–5 times a week. I went to the Transplant Olympics in 2002 and plan to go back again in 2004. I also participate every April in the 8-K Cooper River Bridge Walk. I am healthy and blessed. I have also had the opportunity to meet my donor’s mom. She’s a wonderful lady. I don’t know what else to say except that I am enjoying life to the fullest and I don’t take much for granted anymore.
Pam Hawver
First Name:
Tom
Last Name: Shirrell
Title: ZZ
Wedding 1973 then . . .
and now, Tom and Sue 2003.
Tom Shirrell—diag ZZ 1996 at 21% FEV1. No other known family members affected. Elected not to use Prolastin (still not sure whether that was wise or not). Listed February 1998 for single-lung transplant at McGuire Veterans Hospital, Richmond, VA. Went to Status 7 (inactive) for a couple of years, reactivated in October 2002 and was bumped up to bilateral in June 2003.
I’m not O2 24/7, but at night and periodically as I battle infection after infection, pneumonia after pneumonia. I work full time (sort of), but I co-own a business and sit at a desk all day long. I’m waiting anxiously to join the growing list of second chancers! I cannot remember what it’s like to be able to breathe!
Tom in Indy
Last Name: Draisker
Title: ZZ Relief Webmaster
Personal: Age 65; Sex: Female
Three generations of sisters: (from left to right) granddaughter Amy (5), daughter Jo, daughter Brenda behind me, and sister LaRetta holding granddaughter Kara (2) June 2007.
As an Alpha, I feel I’ve been lucky in several ways. First, I was diagnosed as an Alpha before I was diagnosed with asthma. My shortness of breath started when my family and I were stationed in Adana, Turkey. Our tour of duty was curtailed because the boy of my fraternal twins (second Alpha-related blessing) was diagnosed with cerebral palsy. We were sent to Andrews Air Force Base for his sake. I went for a check up before restarting an exercise program. I was referred to the pulmonologist after my lung X-rays came back and he made the diagnosis from the bell-shaped lungs and lowered diaphragm. This was in 1981, I was 39 years old, and my FEV1 was 49%. The only treatment was theophyline and an inhaler and life expectancy was 50 to 55. My maternal grandfather died at age 53, after being hospitalized with pneumonia and had asthma most of his life, so I believed it.
While we lived in Falls Church, VA, I applied to be in the original research group at NIH, but was considered too healthy. When yard work made me breathless and working full time and doing the housework exhausted me, I packed up the three kids and moved to the Phoenix, Arizona area (third lucky break). We brought what possessions would fit into our station wagon (only one medium rock from my middle daughter’s collection) and my brother’s Toyota truck. I never regretted the move.
After about a year there, I managed to start a freelance business (fourth lucky move) and was able to be at home with the kids. This really helped when my son had his third surgery and had to be in casts for six weeks. About five years later, I lost my two major accounts within three months and thought it was bad luck. However, I got a job at Arizona State University just before my twins graduated from high school (fifth great opportunity). Since I was an ASU employee, they only had to pay one-fourth of the tuition and they both graduated from college (and I was there, sixth unexpected milestone).
My goal then was to see my first granddaughter (and now Amy’s sister Kara) grow up. My next goal is to retire. I can’t get full benefits until I’m 65 plus. With 1942 as my birth year, that’s the Federal retirement age. Also, for ASU I need the years to accumulate enough points. Since I was 65 on March 6, I’ve still got until December 6. In the mean time, I intend to find a way to keep working. My FEV1 is 25% now and it’s getting very hard, but I want to draw at least one Social Security check.
I attribute my advanced age on the good genes I got from my Dad. He was 93 years young on June 29 and still living on his own in a retirement village. Additionally, I have well-honed survival skills. I also believe that the very mild winters, no yard work, and just plain stubborness helped immensely. I also have supportive children, son-in-law, and “baby” sister (59 on March 7). I was forced to go on disability by my employer mid-March 2005 and moved to Springville, Utah to be near my granddaughters (and their mother and father, of course). I do volunteer work for the Children's Justice Center in Provo, Utah and the Senior Citizins Center in Springville (where I also play cards most days of the week).
I also try to keep the Alpha2Alpha web site updated and now typeset and edit the Alpha-1 Advocacy Alliance newsletter, the Register. I jope to help revise their web site also as I love editing and typesetting (which is a lot of what it takes to do a web site). All-in-all, this helps keep me busy and not sitting around feeling sorry for myself.
I met my first Alphas in person through the local Phonenix support group (Alphazonies). I also was phenotyped at the Alpha-1 Association Conference we hosted. Because I had infused prolastin the day before, the results said I was an MZ. The pulmonologist I had at that time took me off prolastin because of a shortage. He didn't even bother to look at the tests my previous pulmonologist did to get me on it about a year before. When I retested six weeks later, my Plasma level was 4.5 micromoles/liter and I was most definitely a ZZ. (On the letter confirming my ZZ status, someone had handwritten, “Get in touch with the patient immediately!”)
So, that’s my story and I’m stickin’ to it. Take care everyone and remember good things can happen to good people, too.
Mary Fran (excuse any typos, I’m an editor, not a typist)
Last Name: Richmeier Brown
Personal: Age: 42, when she left us; Sex: Female
As I look at all the web sites and information that has been published since mom was diagnosed with Alpha of the lungs, I am amazed. Shortly after she passed, I tried to write a high school research paper on the disease and could not find anything but a few pamphlets here and there. I am very grateful that this disease is better known today and there are support groups for this trying disease.
As far as I can tell mom was born healthy and happy like any other child. She grew up with same way. If I recall right, I believe she began smoking when she was 16 or 17. It was the in-thing back then and, of course, she thought it made her lose weight. After giving birth to me, at the age of 25, her health rapidly deteriorated. She still continued to keep her house immaculate and take care of her drunken husband, even though she no longer had energy to do these things. I often found her taking long naps in the afternoons.
My little brother came along in 1985 and everything went downhill from there. She continued to smoke even though she was short of breath. My dad left for good, leaving mom with the two of us kids to support without having the health or ability to actually work. I remember going with mom to see these doctors and listening to her ask them, “What is wrong with me?” They diagnosed her with asthma and emphysema. She was too young for the second. The local doctor did not give up and searched for answers. He remembered that he had done a thesis paper on the Alpha disease so began researching it. We went from 1985 to 1991 not knowing what was wrong with mom. She finally was sent to see Dr. Sandhaus in Denver, Colorado, where she was diagnosed with Alpha-1. After reading the stories, I am not sure what the letters, ZZ and MM, stand for, but I will do my research to find out. Dr. Sandhaus put her on weekly Prolastin treatments, but I believe in my mom’s case it was too late.
Mom’s health continued to fail. She had more bad days than not. We couldn’t even put her on the transplant list because there was just no way of paying for the $300,000 operation and follow-up care. We did many fundraisers hoping to raise enough money to give her a future with her kids, wrote all the churches in Kansas asking them to do one fundraiser in mom’s name. We did receive some donations, but not nearly enough to save mom’s life. After one final call to 911 and a week living on machines, mom said goodbye at the young age of 42.
I am a carrier of this disease, I was told that if I should have children with someone that is a carrier, there is a chance I could pass it on to them. I have decided that no matter what, I am going to live life, not only for me, but for mom, who didn’t get to live it long enough.
Mom, I love you, always will. I just know that you are watching out for all of us down here, your beautiful granddaughter included.
Tiffeny Brown
LastName: Eckert
Personal: Age: 35; Sex: Male
My name is Henry Eckert. I’m 35 years young. I concider this my fourth chance at life and I plan to make the best of it. When I was a younger man in school, I was the fastest student in the city of Kenosha, Wisconsin. I was a good athlete, loving both track and field and football.
At the age of 15, I was in the construction trade and at 24 I became a garage door installer. I would climb 30 feet high on extension ladders to install springs, lifting heavy commercial doors. I never had trouble breathing. When I was 29 years old I was diagnosed with testicular cancer. The doctors removed my left testicle due to a malignant tumor. I went through radiation treatments. I was sick to my stomach but healed enough to go back to work, doing the same job I was doing before the cancer. After returning to work, I found my job harder to do. I was short of breath and fatigued but figured it was just from the radiation and healing. Well, that lasted until November 2000. I ended up in the hospital with a severe lung infection. They told me I had phenomia. My family doctor then told me I had asthma. He prescribed an inhaler (combivent) I felt better for a little while but then got sick again in May 2001. An on-call doctor at the hospital looked at my x-rays and told me he wanted to run some blood tests, but not to worry.
The next day he came in my room and told me I had a hereditary disease called alpha-1 antitrypsin deficiency. He told me I lost tissue in my lungs and every time I get sick I lose more. He basically told me that I didn’t have any elastin (rubber-bands) in my lungs. My lung volume dropped drastically. Before the cancer and radiation I was fine. My doctors and I can’t prove it, but we think the disease was sped up from the radiation treatments. The radiation disturbs the white blood cells which is a big part of alpha-1 disease. So... I went back to my local pulmonologist, who put me on an infusion called Aralast. He said it would slow my disease down. I finally gave in and left my job as a garage door installer in March 2004. My lung capacity at the time was only at 15%. Through my union I went on disability, later to social security in September 2004.
In December 2003, I was told by my pulmonologist, that I would be put on a waiting list for a transplant. Well, that never happened. I asked him about it many times but he kept telling me to just hang in there and see what the aralast did. I asked him “why” he didnt want me on the list. He was very discouraging, telling me that transplant patients only last for about three years. I got upset and went home. I got on my computer and started searching for transplant hospitals in my area. In April 2004, I found UW Madison Hospital in Madison, Wisconsin, said to be the best. I called the pulmonologist there, Dr. Richard Cornwell. The soonest appointment I could get for an evaluation was in June. I went back to Madison in June to see Dr. Cornwell, he asked who referred me to him. When I told him I found him on the computer, he said I needed a referral from another doctor to see him. So, I told him that my local pulomonlogist didn’t want me to have a transplant but I gave him the name anyway for the referral. I just prayed he would be all right with that. That was my only chance at survival. I was still taking the aralast infusions, waiting to die, not knowing. Then, I went through the evaluations and was put on the transplant list in September 7, 2004. I still excersiced three times a week with my oxygen on. At the time, on three liters of O2 everyday, all day.
On February 16, 2005, 4:30 a.m. my home phone rang. My bedroom is on the second floor, and I knew I couldn’t make it down to the first floor in time to get it. Then my cell phone rang seconds later. My stomach got really weak, I started sweating, I answered the phone. It was the nurse from the transplant team at Madison Hospital. She said, “Are you sitting down?” I started to cry. I thought I was dreaming. She told me that they had a set of lungs that were a match for me. I would be coming to Madison but go back to sleep. Ha! Ha! How was I supposed to go back to sleep! I woke up the next morning and started packing, telling everyone that I might be going. At 7:00 a.m. they called back and said they were sending an ambulance to get me. By 8:00 a.m. the ambulance was there with a stretcher waiting for me to go. I told them that they wasted their time with the stretcher, I was walking out to the ambulance! They laughed and got me loaded in. I called everyone on the way to Madison to let them know I was on my way. When I got there, they put me in a gown and talked to me about the transplant. They said it could only be a dry run. When I went into surgery, I prayed to God. I made peace with myself. I was ready to take the risk of not waking up from the surgery.
Well, I did. When I woke up, I had a tube in my throat. I knew right away that I had new lungs. I COULD BREATH! I looked up and thanked God for sparing my life again. I thank God everyday for waking up and being alive. Before the transplant, I was only at 12% lung volume. Two days after surgery, I was at 85% lung volume. I stayed in the hospital for 10 days. The nurses had me up in my chair pedalling the day after my transplant and walking a little with the help of a wheelchair. The doctors would have let me out of the hospital in five days, but they found a pin hole in my left lung. They had to install a small tube in my airway to help my lung heal and stick to the wall of my chest. That was my only complication. My surgeon, Dr. Robert Love, told me that there were only two people on the list that fit the lungs. The other person didn’t match well enough for some reason. I was very lucky to recieve a bilateral lung transplant. The only thing the doctor could tell me about my donor was that he was a big man with a huge chest. I’m not a big guy, but because of the disease and the stretching over time, I needed a larger set of lungs. My donor was a near perfect match. My lung volume now is at an astounding 118%. I excercise 3–4 days a week.
In January 2006, I found out I had testicular cancer again. They removed my other testicle. Again, I had a malignant tumor. The doctors said I caught it in the early stages of stage 1. I was very lucky not to have to go through radiation again. I feel that my cancer came back because of the immune suppressant drugs I am taking. My cancer is gone. I feel great! I feel God has blessed me once again at another chance at life. I’m back to installing garage doors. I’m still in the union, enjoying my family (seven kids between my fiance and I). Life is great! I thank God everyday for my life and my family. I have no regrets, even if I only live another year, it was worth it! I wrote a letter to my donor family to thank them for this wonderful gift, they haven’t written back yet. I would understand if it were too difficult for them. Even before this experience, I was a donor, because of this experience, I truly understand its importance. Anything I can donate when I go, will be donated.
I can say this because, I was waiting to die before I got on the list. If anyone reading this is undecided about getting a transplant, I say, the risk is worth it. It’s worth another chance at life. Just be strong. Thank you for reading my story.
Henry Eckert
Bilateral lung transplant 2-16-05 due to alpha-1 antitrypsin deficiency
Alpha-1 ZZ
LastName: Bedford
Title: MZ
My name is Gord Bedford. I am a 36 year old male living in Toronto awaiting a liver transplant.
I am now the second generation that has fallen prey to alpha, as my mother had a liver transplant 6 years ago. My story is probably similar to many other Alphas, but maybe there is some difference that may help someone along the way.
The first sign of there being anything wrong with me was my right foot swelling to three times its normal size. My first step was to get to the emergency room of a local hospital, where the doctor immediately assumed it was a deep vein thrombosis (DVT), however the ultrasound came back negative. After he read the ultrasound report, the doctor told me to go home and keep my foot elevated while taking 1200 mg of Advil. Not feeling confident with this theory, I went the next day to another local hospital, where once again DVT was the assumed prognosis, after yet another ultrasound of my leg, and another negative report. The second doctor once again sent me home and advised me to see my GP. At my GP’s office, he ordered some blood work, and told me it would be a few days. When he got the results, I was informed that my liver counts were slightly elevated and he wanted me to see a Gastroenterologist. In Canada, to see a specialist takes time, and unfortunately four months passed before I got in to see the specialist, at which time my leg and foot were even more swollen. The specialist looked at my family history, even noting my mother had Alpha-1, so he ordered more blood work. When the results came in, he explained that my liver counts were slightly elevated and my foot was actually suffering from edema, for which he prescribed spironolactone. The spironolactone seemed to help a bit at first; however, there was a nasty surprise that went along with it. I now had grown fully sensitive breasts.
Another four months passed, I was at the specialists office again where I was informed I was suffering from nonalcoholic steatohepatitis (NASH), without even taking a liver biopsy. I explained again about my mother’s transplant and he seemed none too worried, stating that I needed to increase the dosage of spironolactone, which I was not happy with. I explained about the breasts, and he simply said it’s only temporary and that my liver would last another 20 years.
Ahead two months and, with my fiancé, we traveled together to the specialist and I argued vehemently for a liver biopsy, stating I was not comfortable with his diagnosis and felt the liver biopsy was the only way to be sure. He factually stated that there is no way I could have Alpha-1, because I was only a carrier and not the proper phenotype (for which he never tested for). After several minutes of humming and bandying about, he agreed. The test was set for another six weeks. He once again stated I was in no danger of needing a transplant, that my liver would last another 20 years.
Finally after having the biopsy and waiting another month to get my appointment, my fiancé and I arrived at his office only to hear our worst fears come to life. I, in fact, do have Alpha-1 and am not simply a carrier any more. I was also told that I needed to be listed on the transplant waiting list as soon as possible as my liver counts had taken a turn for the worse. My fiancé and I looked at each other and it was all I could do not to collapse onto the floor. It had been a year since my foot swelled and only now am I being told what is actually wrong with me. The specialist explained that my mother had the proper phenotype, but my father did not and I was only a carrier. Now I learn that carriers can also be afflicted with Alpha-1 and it is a much swifter ride down the slope of poor health. I was extremely overweight at 290 pounds and am now only 170. Some loss is from proper diet and exercise, most from wasting away.
After learning all I could from him, I accepted what was ahead of me as best I could. My mother was devastated to learn of my condition and, sadly, my father passed away a few years before. Since being diagnosed, I have spent the majority of my life in and out of the hospital. Paracentesis is a weekly occurrence for me, where an average of 7–8 liters (or almost two gallons) is drained at a time. Friday mornings are my regular days for the procedure and, by the time three days have passed, I am already three shirt sizes larger from the fluid. Knowing that I have refractory ascites and being unable to live a normal life is something I have to accept. When the fluid builds up, I need to sleep elevated as breathing is a chore. Walking is difficult as my hemoglobin is constantly low, even after treatments of Albumin, and my blood pressure averages 100 over 60. I have had three infections from the paracentesis, regardless of how carefully the doctor cleans and sterilizes the site. Each time the infection seems to get worse. The last bout had me in the hospital for two weeks with my hemoglobin down to 72 and my blood pressure bottoming out at 90 over 40. Sitting up was a chore, never mind walking. A fever of 102.1 which lasted two days and several bouts of vomiting were extremely difficult on my system.
Luckily for me during one of my trips to the local hospital I was cared for by an incredible physician. He has gone above and beyond the norm for me, ensuring that he keeps me going until the day I can be blessed with the gift of life—the gift of a new liver. He constantly checks up on me and performs my paracentesis, making sure that I am okay before I go home. If I get an infection, he meets me at the emergency room, admits me and manages my care. The original gastroenterologist has managed to get me on the waiting list for a new liver and I see him only in a maintenance role now. He monitors my results from the local physician, and ensures that I have my pager turned on at all times ready to receive a gift.
Through all this, I was so angry over the misdiagnosis, length of time for proper diagnosis, and what felt like complete lack of concern from the original gastro doc. Over time, I have managed to forgive him and accepted the fact he is only human and entitled to make mistakes. While an earlier diagnosis would have had me listed sooner, and perhaps already been transplanted, I firmly believe that everything happens for a reason. My mother is my primary caretaker and wow she is amazing. She is six years post transplant and you would never know it. She is in almost perfect health and more than willing to help me in this dark time. I miss out on most things in life, no longer able to work, ride a bike, go for walks, or play with my nephews, all just plain ordinary everyday things. These days I am happy if I can summon the energy and wind power to head out for a couple of hours to a movie, market, or just to the park to enjoy some fresh air. At first I was too proud to use a walker, but now I am only too happy to have one.
While I am not sure how my story will end, I am certain of one thing. I have lived a short but happy life. Many times over I have been blessed with the small things which often go unnoticed. While my body is failing, my heart and mind are never going to surrender. For a while I was forgetting things, traipsing off in conversations, and found myself unable to make decisions or draw conclusions. Learning that this was encephalopathy provided me with medications that aid in the removal of toxin from my blood. As long as I take the medications before it gets bad, provided I notice, the episodes can be minimized.
For everyone out there who has either been misdiagnosed, or accused of being an alcoholic, or simply fallen through the cracks, remember you are not alone. Yes, I am afraid of not receiving a liver transplant; in fact, I am afraid of actually receiving one. Yet it is so important to not dwell on the negative, always find something that puts a smile on your face, and remember that no one can take away your smile.
I thank you for reading my story, and promise to update it as it goes along.
Gordon Bedford, November 6, 1969, Toronto Ontario Canada
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Updated November 18, 2007
