My Alpha 1 story

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HI, I AM ALECIA.  I AM 69, WIDOWED, MOTHER, GRANDMOTHER AND RETIRED RN.  I HAVE ALPHA 1, A GENETIC COPD/ EMPHYSEMA, WHERE I RECEIVED A Z GENE FROM EACH PARENT. MY NEW MANTRA IS FROM "MADAGASCAR"  "YOU GOT TO MOVE IT, MOVE IT, MOVE IT!"  MY DAUGHTER SAYS TO ME, NO, YOU HAVE TO BE MORE POSITIVE AND SAY "I LIKE TO MOVE IT, MOVE IT, MOVE IT".  I SAY, BUT I DO NOT LIKE TO MOVE IT!  TO THAT SHE SAYS, NO MOM, YOU SAY IT UNTIL YOU BELIEVE IT!  SHE IS A WISE WOMAN, THAT DAUGHTER OF MINE AND IT IS TRUE, I DO NEED AN ATTITUDE ADJUSTMENT IN THAT DEPARTMENT.   I HAVE ALWAYS HATED TO EXERCISE AND THAT HAS NOT CHANGED AS OF RECENTLY.  A YEAR AGO I DECIDED THAT I NEEDED TO LOSE 10 POUNDS AS IT WAS MAKING IT HARDER TO BREATH.  IT TOOK ME ABOUT 6 MONTHS TO DO THIS AND ALTHOUGH IT DID NOT IMPROVE MY LUNG FUNCTION, IT DID MAKE IT EASIER TO DO MANY THINGS.  I STARTED TO EXERCISE BY WALKING THE MALL WITH A FRIEND A FEW YEARS BACK.  HERE IN THE GREAT NORTHWEST THE AMOUNT OF DAYS ONE CAN WALK OUTSIDE IS LIMITED DUE TO THE ABUNDANCE OF LIQUID SUNSHINE.   IT IS LIMITED FOR ME AS THE HEAT AND THE COLD SEEM TO BOTHER MY BREATHING, SO THE MALL IS THE BEST CHOICE MOST OF THE TIME.  THE MALL I WALK IS .8 MILE AROUND IF YOU WALK THE PERIMETER.  I AM NOW UP TO THREE CONSECUTIVE LOOPS WITH A REST THEN DOING ONE MORE.  I FAILED MY LAST 6 MIN WALK AT THE PULMONARY DR'S SO NOW USING MY OXYGEN TO WALK WITH AND WITH STRENUOUS ACTIVITY.  I HAVE A BACKPACK AND A SMALL OXY GO CONCENTRATOR. I HAVE USED OXYGEN AT NIGHT SINCE 2010.  I ALSO JOINED A SENIOR WATER AEROBICS CLASS IN A SALINE POOL WHICH IS FOR ARTHRITIC FOLKS WHICH WORKS WELL FOR ME.  IT IS ABOUT 94 DEGREES IN THE POOL SO CONSIDERED THERAPEUTIC. I HAVE JOINED A CHAIR YOGA CLASS FOR STRENGTH AND STABILITY WHICH IS HELPING AS WELL.  I TRY TO EXERCISE 4 OR 5 TIMES A WEEK AND WEATHER PERMITTING GARDENING WHICH IS WHAT I CALL "YARD THERAPY".  I DO NOT GET A SURGE OF ENDORPHINS AS IS OFTEN THE REWARD FOR OTHERS THAT EXERCISE. WHAT I GET IS INCREASED HUNGER AND NEED FOR A NAP!  I DO UNDERSTAND HOWEVER, THAT EXERCISE IS NECESSARY TO KEEP MY OTHER ORGANS HEALTHY AND MAINTAIN A HEALTHY WEIGHT.  I AM NOT YET TO A POINT OF "LIKING IT" BUT WORKING ON THAT AND FIND I LOOK FORWARD TO IT WHEN A FRIEND IS INVOLVED.  I AM FORTUNATE TO HAVE FRIENDS WHO DO NOT HAVE THIS DISEASE THAT ENCOURAGE ME AND GO WITH ME TO EXERCISE.  MY REWARD IS A CUP OF COFFEE, VISIT WITH MY FRIENDS AFTERWARD AND AN OCCASIONAL PRETZEL WHICH WE SHARE.  MY STORY WITH ALPHA 1 BEGAN IN 2006.   I WOULD GO TO WORK EACH DAY, PARK IN THE PARKING GARAGE AND WALK INTO THE OFFICE UP A SLIGHT RAMP, WHICH WAS ABOUT A HALF A BLOCK TO A BLOCK.  I BEGAN TO NOTICE THAT I WAS GETTING SHORT OF BREATH BY TIME I GOT TO THE OFFICE.  I STARTED TESTING MY OWN OXYGEN SATURATION LEVEL WHEN WALKING PATIENTS FINDING IT WAS OFTEN LOWER THAN THE PATIENT I WAS WALKING WITH.   I ALSO STARTED HAVING SOME SUB STERNA PAIN.  I VOICED THIS CONCERN TO MY DR.  I WORKED AS THE ONLY RN IN A BUSY INTERNAL MEDICINE OFFICE FOR 4 DR.'S.  ONE OF THE PHYSICIANS I WORKED FOR WAS ALSO MY PRIMARY CARE PHYSICIAN. I HAD A  LUNG SCAN, PFT'S AND CHEST X-RAY WHICH ONLY SHOWED SOME SCARRING IN MY LUNG AND NODULES. SO I HAD A CT WHICH DID NOT SHOW ANYTHING CONCLUSIVE. MY OXYGEN LEVEL WAS A LITTLE LOW BUT NOT SIGNIFICANT.  I WAS REFERRED TO CARDIOLOGIST AND GIVEN A STRESS TEST AND ECHOCARDIOGRAM.  I WAS ALSO DIAGNOSED WITH REFLUX AND PUT ON MEDICATION WHICH TOOK CARE OF THE CHEST PAIN.  I SORT OF DROPPED THE WHOLE ISSUE BUT WAS STRUGGLING WITH INCREASED FATIGUE AND BY AFTERNOON WAS READY FOR BED.  THE DR'S FELT I WAS OVERDOING IT AT HOME AND VOICED CONCERNS ABOUT MY ABILITY TO DO MY JOB.  THIS WAS DEVASTATING TO ME AS I HAD BEEN WORKING IN NURSING FOR 40 YEARS AND WITH THE ONE DR. FOR 20 YEARS.  IT IS TRUE I DID HAVE A LOT GOING AT HOME, MY HUSBAND HAD CANCER AND HAD UNDERGONE CHEMO TO HIS LIVER ALTHOUGH WAS RECOVERING.  OUR GRANDSON (A TEENAGER) WAS LIVING WITH US ALONG WITH OTHER FAMILY ISSUES.  THIS HOWEVER WAS NOT NEW AND I HAVE ALWAYS BEEN A BUSY WOMAN INVOLVED WITH ISSUES WHICH TOUCHED ME OR MY FAMILY.  SO NO BIG RED FLAGS WERE GOING AROUND BUT I WAS STRUGGLING WITH THE FATIGUE.  I DECIDED TO TAKE EARLY RETIREMENT AND RESIGNED FROM MY JOB WITH A HEAVY HEART.  THIS WAS SEPTEMBER OF 2007.  I DUG IN AND KEPT BUSY TAKING CARE OF SOME HOME REMODELING AND STUFF THAT HAD GONE NEGLECTED WHILE I WAS WORKING.  I STARTED WALKING WITH MY NEIGHBOR FRIEND AND WE WOULD WALK FOR AN HOUR OR TWO, ABOUT 3 OR 4 DAYS A WEEK AROUND OUR NEIGHBOR WHICH IS FULL OF HILLS.  IT BECAME APPARENT THAT I WAS SHORT OF BREATH, ESPECIALLY GOING UPHILL.  AT FIRST I THOUGHT IT WAS DUE TO BEING OUT OF SHAPE BUT THEN AFTER SEVERAL MONTHS AND NO IMPROVEMENT I WENT TO SEE THE DR.  I WAS CONVINCED I HAD EXERCISED INDUCED ASTHMA.  I WAS GIVEN AN ALBUTEROL INHALER TO USE WHEN WALKING AND AT FIRST I THOUGHT IT WAS HELPING.  THEN THE WEATHER GOT COLD AND I DID NOT FEEL IT WAS HELPING AT ALL.  THAT WINTER I GOT AN UPPER RESPIRATORY INFECTION AND THOUGHT I WOULD COUGH MY LUNGS UP.    I WAS TREATED AND RECOVERED BUT TOOK THREE WEEKS AND DR INSISTED I SEE A PULMONARY DR.  THIS WAS IN THE FEBRUARY OF 2009.  I WANTED TO APPEASE HER AND WENT TO SEE THE DR.  OF COURSE HE WANTED A CHEST X-RAY AND PFT'S.  I ALSO HAD TO HAVE ANOTHER TWO CT'S TO FOLLOW NODULES IN MY LUNGS.  I WAS TOLD I HAD COPD.  I SAID, "HOW CAN THAT BE, I NEVER SMOKED", (WELL I TRIED TO SMOKE WHEN I WAS IN COLLEGE BUT NEVER DID LIKE IT MUCH AND GAVE IT UP AFTER A FEW MONTHS).  BOTH MY PARENTS AS WELL AS MOST OF MY RELATIVES SMOKED.  IN MY DAY THAT MEANT IN THE HOUSE AND CAR REGARDLESS OF HOW CRAMPED THE AIR SPACE WAS.  I DID NOT HOWEVER, FEEL THAT SHOULD HAVE IMPACTED MY LUNGS THAT BAD SINCE I MOVED AWAY WHEN I WAS 18.  NO ONE HAS SMOKED IN MY HOUSE SINCE.  I HAVE HAD QUITE AN AVERSION TO CIGARETTE SMOKE SINCE I WAS A CHILD.

 

WITH THE DIAGNOSIS OF COPD CAME THE RECOMMENDATION THAT I GO TO PULMONARY REHAB.  I HAVE ALWAYS TRIED TO BE A GOOD PATIENT SO AGREED.  WHEN I WENT TO THE INTAKE INTERVIEW WITH THE NURSE FOR REHAB SHE ASKS ME WHAT MY ALPHA ONE ANTITRYPSIN LEVEL WAS.   I TOLD HER I HAD BEEN A NURSE FOR 40 YEARS AND HAD NEVER HEARD OF THIS.  I ASKED HER HOW IT WAS TESTED.  SHE TOLD ME A BLOOD TEST.   I HAVE A HABIT OF KEEPING ALL MY RECORDS I KNEW I HAD NOT HAD THAT ONE CHECKED.  SHE TOLD ME IT WAS RECOMMENDED BY THE LUNG ASSOCIATION THAT ALL NEW COPD PATIENTS BE TESTED FOR ALPHA 1.  SHE TOLD ME I HAD THE CLASSIC SYMPTOMS AND ONE OF OUR GOALS WAS TO TEST FOR THIS.  I WENT HOME AND LOOKED IT UP ON THE COMPUTER AND WAS CONVINCED I DID NOT HAVE THIS AS I DID NOT HAVE FREQUENT RESPIRATORY ILLNESS.  I DID HOWEVER ORDER THE TEST AND ASKED MY PRIMARY DR TO SIGN IT.  SHE DID SO RELUCTANTLY AS SHE SAID SHE WAS NOT FAMILIAR WITH THIS DISEASE.  I TOLD HER I DID NOT THINK I HAD THIS AND IF I DID I WOULD GO TO PULMONARY DR WITH IT.  I STARTED THE PULMONARY REHAB AND TWO WEEKS LATER THE DR CALLED ME TO TELL ME I HAD ALPHA 1 AND WAS ZZ.  I WAS INSTRUCTED TO CONTACT ANY SIBLINGS TO BE TESTED.  I AM THE ONLY CHILD OF MY MOTHER AND HAVE 3 HALF SIBLINGS FROM MY FATHER.  I HAD ONLY RECENTLY MET THEM SO WAS A BIT RELUCTANT TO LAY THIS ON THEM BUT DID AND THEY ALL TESTED MM.  MY MOTHER DIED OF END STAGE COPD IN 2004 AT THE AGE OF 81.   MY FATHER DIED IN NOV 08 OF CHF, END STAGE RENAL DISEASE.  HE WAS A PEARL HARBOR SURVIVOR SERVING ON THE OKLAHOMA.  HE WAS NOT A PART OF MY LIFE UNTIL I WAS IN MY MID 30'S.

 

I HAD LIMITED INSURANCE WHEN I RETIRED DUE TO COST SO WAS UNABLE TO PAY FOR AUGMENTATION.  UPON FINDING I WAS ALPHA 1 I JOINED THE REGISTRY AND RECEIVED NOTICE OF A STUDY FOR GENE TRANSFER AT BOSTON UNIVERSITY WITH DR FLOTTE.  I QUALIFIED AND JOINED THIS RESEARCH PROJECT WHILE I WAITED TO GET ON MEDICARE AND START AUGMENTATION.  I WAS VERY IMPRESSED WITH THIS RESEARCH PROJECT AND HAPPY TO BE A PARTICIPANT.  I HAVE JUST FINISHED MY PARTICIPATION IN THIS STUDY AS IT WAS A 5 YEAR STUDY.  I JOINED THE LIVER STUDY FOR ALPHA1 THIS PAST SUMMER AND HAD A LIVER BIOPSY SHOWING STAGE 1 (THERE ARE 5 STAGES OF FIBROSIS, 5 BEING THE WORST) SO I AM PLEASED ABOUT THAT.  I HAVE PARTICIPATED IN A COUPLE ONLINE STUDIES FOR ALPHA 1 AS WELL.

 

IN AUGUST OF 2011 I STARTED ZEMAIRA INFUSIONS AND CONTINUE TO DO THEM MYSELF AT HOME.  MY HUSBAND USED TO HELP ME BUT I HAVE DONE THEM BY MYSELF SINCE HE DIED NOVEMBER OF 2013.  I HAVE TWO BIOLOGICAL CHILDREN WHO ARE BOTH SZ   MY DAUGHTERS SON IS SS.  MY SON IS BEING PUT ON OXYGEN AT NIGHT AND HIS LUNG FUNCTION IS LOWER THAN MINE BUT HIS SATURATION LEVELS ARE GOOD.  MY DAUGHTER HAS ASTHMA BUT NO COPD BUT MULTIPLE OTHER MEDICAL ISSUES.

 

I HAVE FOUND WONDERFUL ONLINE SUPPORT GROUPS FOR ALPHA 1 WHICH HELP ME A LOT AS WHEN I STARTED THIS I KNEW NO OTHERS WITH THIS ILLNESS.  WE HAVE A LOCAL SUPPORT GROUP NOW WHICH MEETS 4 TIMES A YEAR THAT I ATTEND REGULARLY. 

 

MY CONDITION CONTINUES TO PROGRESS AND NOW HAVE MILD PULMONARY HYPERTENSION.   I KEEP WORKING ON STAYING AS HEALTHY AS POSSIBLE WITH EXERCISE, DIET, EDUCATION AND SUPPORT OF FAMILY AND FRIENDS. THIS DISEASE ROBS ONE OF THE LIFE ONE WOULD LIKE TO LIVE.  I AM LEARNING TO BALANCE WHAT I NEED OR WANT TO DO, WITH REST AND MODIFICATION. IT SEEM LIKE EACH DAY IS ADJUSTING TO NEW LIMITATIONS WHICH IS A CHALLENGE FOR ME WHO HAS ALWAYS BEEN A BUSY PERSON. I AM SO GRATEFUL FOR THE HEALTH I DO HAVE AND WHAT I AM STILL ABLE TO DO.  I KNOW THAT ALL CAN CHANGE TOMORROW, SO I AM LEARNING TO PACE MYSELF AND BE MINDFUL OF MY LIMITATION. MOVING TOWARD USING OXYGEN FULL TIME IS MY CURRENT ADJUSTMENT. 

 

THANKS FOR READING MY LENGTHY STORY.                                                                     

 

ALECIA