A Good Movie

 Far Guy and I are watching Vikings Valhalla on Netflix at night.  It isn't as funny as Norseman but still entertaining enough for us...it is fairly violent so it is not for the squeamish.  The special effects and wardrobe department did an awesome job on this series and we hope that there is a second season. 

I keep reminding Far Guy that Vikings are his people...as the Vikings bodies naturally developed Alpha 1 Antitrypsin Deficiency because they ate so much fish and would get worms....of course they only lived to be 40 or 50 years old so the effects of Alpha 1 were all positive ...they didn't get worms.  However they did pass their genes on down the line.  Far Guy has both genes, our daughters are carriers and we have one grandson that also carries one gene.   

A few days ago it was rare disease day...since Far Guy is lucky enough to have two rare diseases  (Trigeminal Neuralgia and Alpha One Antitrypsin Deficiency)...I thought I would at least mention it. 

Back to the movies. 

My Niece Stacy sent me this photo...she said "It must have been a good movie!" 

Dad and Mom with four of their Great Grandchildren.  ( I am guessing that the movie was The Yearling)

Far Side

Town Report

Thursday is Far Guys infusion day.  The infusion protects his lungs for 6 days...it replaces the protein that cannot escape his liver to protect his lungs.  WE are thankful for one more week.

Clinic Report:  They take your temperature when you enter the building, and ask the questions "Have you been out of the country?"  "Have you visited New York, California or Washington State recently?" " Have you had a temperature?  A cold or the sniffles?"  Then they write down your temperature on a post it note and stick it on your chest.  (Proof they did their job?)  They give you a mask to put on.  Then the gal tells you to go to registration....the gals there say "go ahead Gene you are all set Happy Thursday."

He is their every Thursday same time same place guy.

I waited outside in the parking lot...there are hearts in the window.  I called my sister and talked to her for awhile.  I crocheted.

After Far Guy was done we picked up our groceries from our online order.   Stopped by the butcher shop and bought bacon and brats.  You call in your order and they bring it out to the car.

Main Street was mostly deserted there were lots of places to park.

Minnesota goes into Stay at Home for two weeks this evening March 27 until April  10.

My other baby brother and his wife ( she who sees Robins first) stopped by to visit out on our patio. ( 6 feet apart at least)  She who sees Robins first already saw a Robin.  She brought me a skein of yarn from wally world so I can finish a project.

We are home and safe for another week   Stay safe!
Far Side

February 29 Rare Disease Day

Far Guy is a bit of an expert on Rare Diseases as he has two of them.  Seems he won the Rare Disease Lottery.

The last day is February is Rare Disease Day every year.

Far Guy was diagnosed with Trigeminal Neuralgia or TN on December 25, 2007.  Even I recall the date.  He spent two weeks in extreme pain until the medication took effect.  Trigeminal Neuralgia is often called the suicide disease as the pain is so intense and relentless.

The pain is like a cattle prod to the side of your head and then burning that is constant.   There are many different medications and combinations of medication over the years he has tried them all.  He has also had injections into the nerve and acupuncture.

Right now Far Guy is in remission and off all medications, he has a wonderful Neurologist who he sees several times a year.  We are so very thankful for a good Doctor and for remission, we hope the remission lasts forever.

Far Guy was diagnosed with Alpha 1 Antitrypsin Deficiency on May 18. 2015. 
Alpha 1  Antitrypsin Deficiency is an inherited/genetic disease.   Alpha 1 is a protein made in the liver, when you get a lung infection the protein goes from the liver to the lungs to protect them.  In people with the deficiency like Far Guy the proteins are in the liver but the wrong shape and they are stuck there so they cannot travel to the lungs to do their job.

Alpha 1 is rarely diagnosed although there is a confidential free blood test available at Alpha 1.org The blood test there is the ONLY reliable test available in the United States...sure you can go to your local clinic and have the test but don't count on the results being correct.  Everyone who has any kind of breathing problems should be tested in my opinion.  I was tested..and I am normal so our daughters got one bad gene from their Dad and one good gene from me.  Only one of our grandchildren has a bad gene.

Far Guy receives weekly infusions of the protein to protect his lungs.  Right now his lung function is about 20%  He is presently on The Lung Transplant list at the University of Minnesota.  Our bags are packed and we are ready to go when ever they call.

We are very careful...we don't go very many places in the winter time.  Fragrances, smoke, cleaning solutions, fabric softner, hair spray, smelly shampoo, gasoline fumes, exhaust from vehicles all make difficult breathing more difficult.  The candles being extinguished at church...many things that a normal person doesn't even think about cause breathing problems. A simple cold can end up with a stay in the hospital.

1 in 8,000 people get Trigeminal Neuralgia.
1 in 2,500 people get Alpha 1 Antitrypsin Deficiency.

I suspect that one person getting both rare diseases is very rare.

Far Side

Wistful Wednesday: 2013

This is a photo of Far Guy and Chance before Far Guy was diagnosed with Alpha-1 Antitrypsin Deficiency.

2013

We have returned from The University of Minnesota. 

We have a few more hoops to jump through.   Pathology reports from a previous Colonoscopy and possibly a Colonoscopy.  PSA results.  They will call us when we get the go ahead for a week long visit for numerous other tests.  The Lung Transplant Specialist sees no huge roadblocks for a transplant at this time.  One day and test at a time. 

Far Guy has lost even more weight.  The Doctor was young...in her 30's.  She said Lung Transplants  are one of the most difficult transplants.   Many people don't make it through the surgery, many don't live a week.  Some die within the first year....those that make it a year live on the average of 7 years.  She was quite brutal with the facts. Recovery will be difficult because of his age.  Anti rejection drugs are hard on your kidneys, there are three forms of rejection.  Cancer is a concern when you are on anti rejection meds. 

She said something that I have never heard a Doctor say before.  " Everyone has cancer, our bodies natural immune system fights it off all the time."

Far Side

Wistful Wednesday: What we pass along

Far Guy got the results of his DNA story.

England, Wales and Northwestern Europe 69 %

Germanic Europe 17 % ( Northwest Germany)

Norway 6 %

France 4 %

Ireland and Scotland 4 %

Northwestern Europe covers a large area: Ireland, UK, Belgium, Netherlands, Northern Germany, Luxembourg, Northern France, Denmark, Norway, Sweden, Iceland, Finland, South Germany and Switzerland.  Some of these peoples were Celtic and some were Vikings.

The Vikings part is a bit interesting.  There is a Genetic connection to Far Guy.  The Vikings were eating raw fish and sometimes the fish had worms that would make you ill.  Over the years their bodies adapted and they developed a mutated gene…that gene is what causes Alpha One Antitrypsin Deficiency.   That is the good news/ bad news, you won’t get worms but your liver will not produce a protein to protect your lungs. 

Far Guy’s parents.  They were both carriers for the bad gene, one was an M (normal) and and S (deficient gene), the other was M (normal) and Z (deficient gene).  We do not know which is which.  ( If all relatives would take the free test we might be able to figure it out…as of yet few have been tested.)  Far Guy is a SZ (deficient), his sister is MM (normal) he got the bad genes and she got the good ones.   A child gets one gene from their father and one from their mother…the luck of the draw.  I am MM (Normal) so we knew right away that both of our daughters were carriers.  Only one of our Grandchildren is a carrier….four escaped and one did not.

Far Side

Port

Far Guy had surgery yesterday morning to replace the port in his chest.  His old port (his second one) was really hard to stick (it felt like trying to stab a tennis ball) and there was no blood return and his IV every week was getting slower and slower.  One call to his surgeon and we were put on the Saturday surgery list at o dark thirty.

Far Guy’s weekly infusions to protect his lungs are dependant on a working port.

It is over, he is sore and tired and a tad cranky.  Jen came to check on the elderly and to make sure we ate something.  She brought Miney and Little Elvis with her, they always cheer us up!

Chance thought it was great to have company.  I thought it was too…as I got to have a worry free nap and put someone else incharge for a bit.

Daylily

Chance is still holding his own peeing, pooping and begging for food.

Just for reference: 1st port July 2015, 2nd Port March 3 2016, 3rd Port August 25 2018

Infusion Day

Yesterday was infusion day.   I was able to bring in all the supplies and the “stuff” to infuse Far Guy in the hospital yesterday.  The Nurses asked what we needed and I said “an IV pole.”  Most pharmacies in hospitals do not carry the protein that we infuse.

Far Guy cannot skip infusions they must happen weekly to help protect his lungs. Luckily his Doctors understand this and told the nurses that we will handle the infusions ourselves.  (There are rules about hospital employees using outside medications on a patient.)

We get shipments once a month.  Last week when he was in a far away hospital, they would not ship the needed shipment because he was in the hospital…so after he was discharged I went over to the supply place pointed at Far Guy sitting in the car and said “Gimme the stuff, he isn’t in the hospital anymore.”

So we learned a lesson, never tell the supplier when Far Guy is in hospital if we are expecting a shipment. ( I called to ask them to delay shipment by one day because Far Guy was in the hospital and no one was around to recieve the shipment…it cannot freeze.)  I made arrangements the next day to have my brother pick up the shipment at our home before it would freeze…but since Far Guy was in the hospital they could not ship…some Medicare rule…and Medicare doesn’t even cover any of the  cost of the infusions.  Go figure I was fit to be tied.

Infusing in a hospital is different than here at home.  We have a routine, we do everything in the same order every Thursday.  It was an adventure in a different setting.  Far Guy has lost weight…another three pounds…his port is free floating anyway… and I had some trouble acessing his port…eventually I was sucessful.  The actual infusion went well.

A number of the nurses were very interested in learning about Alpa One Antitrypsin Deficiency in all of the hospitals we have been in.  Last night three nurses showed up in Far Guy’s room and said “Tell us more”  …so we did.

Hopefully Far Guy will get out of the hospital today, it depends on what his Doctor has to say and how his blood work looks.  ( I have not told Chance yet!)

Busy Week

We had three doctors appointments this past week, so far only one appointment for next week!  A week off for me as Far Guy can go to his Physical Therapy appointment all by himself.

Far Guy has a new Pulmonary Specialist who trained with the Doctor ( Alpha 1 Specialist) that we saw at the Mayo Clinic…so we feel that Far Guy is finally in good hands.  Far Guy is no better, but he is no worse either in regard to his Alpha1 Antitrypsin Deficiency.   The no worse part is in part due to the weekly infusions and staying away from sick people and anyone wearing fragrance of any kind including bug spray.

We had a date night last night and went out for a quick Walleye dinner and then headed over for the Spirit Lake Concert.  It was Bluegrass this week, there was a change of venue due to rain so it was moved indoors to the high school.  We stayed for half of the concert and then it got really warm so we came home and played cards with the neighbors.

We managed to up the total of Christmas Ornaments Ready to Paint: 55  Painted :3 Total 58.  This next week I will begin to paint a few more.

Far Guy is doing R and D on a new flower carving.  Here is the inspiration.

We headed to Sheilas place one night to take photos.  Sheila is attempting to carve Indian Paintbrush also.  I have an idea how I would do it…but right now I just don’t have the time to carve it…only to think about carving it. Sheila graduated from High School with Far Guy and is also in our woodcarving group.

Oh yes Community Education called and would like us to teach another soap carving class this winter. So I said yes…Far Guy and Sheila have agreed to help.

I didn’t mow the lawn this week, but I did spray a bunch of Poison Ivy along the road….It is a job I really enjoy.  I also weeded my poor pathetic flower garden that the squirrels have decimated.

We finally got about 2 inches of rain Friday evening.

Nearing sunset on the runway at Sheilas.

Tests

We spent a morning at the clinic in Fargo this week.  Far Guy is in a five year study.  This is his second year.  They did a CT Scan of his lungs, nothing has changed in the last year…his nodules that showed up last year are unchanged.  They appear to be non cancerous nodules that the Dr is not worried about.

Road between Lake Park and Hawley

A few weeks ago he had a Liver Biopsy; he does not have a fatty liver so no change in diet is warranted, he has some damage which is consistant with Alpha 1 Antitrypsin Deficiency. The biopsy made him sore and uncomfortable for a few days.  We are not big alcohol drinkers…Far Guy would not be alive if he drank more than one alcoholic drink a week…that is info straight from his Liver Specialist.

The test for Alpha 1 is a simple free blood test, we wish that more cousins would get tested so that we could help make two and two = four.  So far everyone seems to be afraid of the results.   I can understand that…I was afraid of the results too. Since it came from both sides of Far Guys Family…we can only guess where the lung problems were and where the liver problems are…since both are affected by Alpha 1.

Not everyone that has Alpha 1 is affected, some live normal healthy lives and don’t realize they have it.  That is the hope we have for our daughters and our Grandson. 

The weekly infusions are becoming old hat now, the whole process takes about an hour and thirty minutes.  He is getting much better at sticking himself!  He can do the entire process by himself but I usually help.  Is it fun?  No, he usually spends about two days every week not feeling well…he tires easily and just feels yucky.  BUT he hasn’t had to be in the hospital with lung infections since he began the infusions in May of 2015. 

I wrote an email complaint to the clinic this week.  We had to wait one hour for a blood draw in a perfume filled waiting room.  Four people were working, but the best they could manage was five draws in an hour.

Friday

It is the end of another week.  Not sure what was accomplished this week other than the usual…mowing, laundry, cooking…  repeat when necessary.

Thursday is the day we do the infusion to treat Far Guys Alpha 1 Antitrypsin Deficiency.  We don’t plan anything for the entire day except the Infusion and a nap. 

I encourage him to drink water until he floats away the day before and the day of the infusion.  Infusion time with the gravity drip is around 18 minutes right now…it was 14 when the port was brand new. Most days we get a blood return …but sometimes not. Every few weeks the Pulmonary Doctor has his Nurse check on us to see how we are doing.  We have the “stuff” to do an IV if we cannot get the port to flow.  Our daughter or niece can do an IV for us or we can go to the Infusion Center or to the ER.  We only have a three hour window once the “stuff” is mixed.  We mix after the port is accessed but that is no guarentee that it will gravity feed.  So every Thursday we breathe a sigh of relief after it has all been accomplished.

Far Guy can do the whole procedure by himself. He doesn’t need me to help…but he kinda likes it when I do. So far so good with the infusions, they are keeping him healthy although we still have to avoid crowds, germy and perfumy people.  The infusion solution costs $1,320,000.00 a year…it is a good thing we have insurance. It would be much more expensive if we went through the Infusion Center weekly…everyone has to get a piece of the pie.  Too bad I cannot bill someone. The infusion solution has been called “liquid gold.”

Does Chance look grumpy?  Well he was.  I had just scolded him for licking his owie which is mostly healed.  He is over his ear infection…finally.  He is sleeping more and still has troubles going up and coming down the steps and he is stiff when he goes from laying down to sitting and than walking.  He takes “stuff” for his arthritis in pill form and on his food.  In two months Mr Grumpy will be 12 years old.  He is becoming quite particular in his old age…routine is his middle name…he likes his walks but only so far and sometimes he picks one of us over the other to make that last outing at night. (He refuses to even go up the steps if it isn’t the person he wants.)  Silly dog.

Doctor Appointment

It was a warm day yesterday, the humidity hung in the air all day long.  Sometimes the humidity was so heavy it misted and droplets formed on the windshield. 

Far Guy had a Doctor’s appointment.  He will be a candidate for a lung transplant if he gets worse before he is 70 years old.  Under new Federal guidelines that is the cut off age. If you are over seventy you will just die.  You may die anyway as transplanted patients live only an average of five years after the transplant.

So far the Lung Function tests are still holding steady.  The port problem will be addressed in the next week or so.  The infusions he receives once a week are helping to hold his lung function steady at 21% of normal.  He has to see another specialist to find out  exactly how much damage has been done to his liver…you see Alpha 1 Antitrypsin Deficiency not only affects your lungs but your liver also.  IF he was a drinker…more than one drink a week he would be in real trouble…luckily he drinks alchol rarely.

The appointment was early in the morning we came home and had a nap. 

Along the way home we saw this:

Lance Gloor received ten years in prison for drug trafficing through a medical marijuana dispensary in Washington State.  This is part of a movement to bring this Federal Law Suit to light…the State of Washington dropped all charges but the Feds picked it up. Apparently this “trailer” is being pulled by two individuals on their dream walk.

I wonder what is inside?  Samples? 

Rare

Today is a day set aside to acknowledge rare diseases.

A Rare or Orphan Disease is :
A disease or disorder that affects 200,000 or less people in the United States.
Usually has no cure.
Often misdiagnosed.

If you tell someone what you have more often than not they say “never heard of it.” OR “what the heck is that?”

It becomes even more scary when Health Care Professionals/ Doctors have never heard of it.  How can they help you with something they are unfamiliar with?

Far Guy has two rare disorders/diseases.

Trigeminal Neuralgia diagnosed in December of 2007.  Luckily he had a smart Doctor in the ER where he was diagnosed immediately.  In retrospect the ear pain that he used to have with no ear infection was a sign, so were the toothaches with no abscess. Trigeminal Neuralgia can be a hereditary disease…so far we know that two relatives suffered with it.  There is no magic cure, pain is “managed.” Some people are helped with brain surgery, others not so much.

Alpha 1 Antitrypsin Deficiency was diagnosed by a blood test in April of 2015.  We noticed a pamphlet in the Pulmonary Doctor’s office.  When the Doctors used to tell him that he had bronchitis and would just have to get over it they were in effect damaging his lungs.  A1AD is a genetic disorder, you can have a combination of bad genes….or you can have a combination of good and bad.  There has been some recent discoveries on the diseases beginnings.  The Vikings had a problem with all the raw meat and fish in their diet…and they got worms.  If their genes mutated they were not bothered with worms… but they had lung and liver problems instead. There is no cure, but treatments given weekly by IV replace the missing protein, so you can help fight off life threatening infections. Testing is free.

Spoons…and how many do you have?  Unlimited?  People with a rare disease have only a  few spoons available to them in any given day. ( Every activity takes a spoon)  Everyday is different…how you manage your spoons becomes a challenge.  Some days there are only enough spoons to manage day to day activities and nothing extra…

Both of Far Guy’s Rare Diseases are not readily visible.  He looks normal and reasonably healthy …well except for the oxygen that he wears some of the time.  He often gets the “Well you don’t look sick” comment.

Lack of knowledge is something that we the voice of the rare disease have to fight continually.

Decisions

Right or wrong we all have to make decisions.

We decided to send Far Guy to surgery to have his port replaced.  It will happen soon.  He asked my opinion and I think that having a port keeps him out of the clinic and all the germy people…which in turn keeps him out of the hospital.  I am not certain how many times he can have it replaced, I suggested they just put a zipper in his chest.

I am going to start logging our hours spent at the clinic/hospital.  Last week one of the nurses was coughing…we are both a tad paranoid about that. I almost suggested that she should have stayed home…but I kept my big mouth shut.

The Income Taxes are done and were sent electronically, how about that …no last minute filing for us.  Our accountant answered all our questions. Cross something off the list.  Years ago after an audit, I made the decision to use an accountant and I am glad I did. 

The local hospital doesn’t have a copy of our Advanced Care Directive but I will make a copy so they have it on file.   The clinic has it and I hear that soon the hospital and the clinic’s computers will be able to talk to each other…sometimes change happens very slowly in the boonies.

Do you have an Advanced Care Directive?  Do you have a Last Will and Testament and has it been recorded?  We were talking with a young widow the other day and her husband had no will…so she was making her way through a ton of paperwork.  Sometimes the decisions we don’t make can become real problems for those left behind.

Heliotrope

imPORTent

Hours just seem to slow to a crawl when you are in the hospital.

We spent most of the day there yesterday for a treatment of special DRANO and Far Guys port is still occluded…even the surgeon says it is a problem…and will possibly have to be replaced which means surgery.

For now we must learn the fine art of operating a IV Pump. If the “stuff” won’t go in well lets just give it some help and force it in.  The surgeon says “WELL of course someone will come out and teach you how an IV pump works.” 

NOT TRUE.  The pump is coming but hopefully with instructions because the Medical Supply place will not sent out anyone because Far Guy is not homebound.  If he were homebound they would send a nurse.  I suppose I can search for a You Tube…

Far Guy is fit to be tied and I am so sleepy I am having a hard time staying awake.

One more hurdle to get over.  I was never a good runner anyway…but hurdles…I would have killed myself with boob flap.

For the Birds Again

The Redpolls left just ahead of the warm front that visited us.  Eighty pounds of thistle seed later they have deserted us.

It was a week.  One difficult situation after another.

I wish I was an Ostrich…don’t they bury their heads in the sand until everything is back to normal?
Far Guy said “Hey I am proud of you, you were stressed out but didn’t fall asleep on me.”

During the infusion this week I could push saline through his port but once the IV was hung (gravity flow) there wasn’t enough of a hole in the fibrous tissue that has grown over the end of the catheter in his vein to drip even one drop.  The drop just hung there  ready to drip but was held frozen in time. Once the protein is mixed it must be administered within three hours.  His port was accessed twice last week and has been properly cared for.  His body must be working overtime to cover up the foreign object that is in his body.  We ended up at the infusion center where of course they cannot hang outside meds…but they could do an IV for him ( and I administered the protein) while they tried to access his port, he had a dose of Cathflow( he has standing orders for that) anyways that sat in the catheter about two hours with no results.

The Surgeon will see him Monday.  We have a back up plan that may work. We will see. I guess I need to become IV Certified….not something I really want to do…but I can access a port now and I thought that would be a big deal too…and it isn’t when the  port works correctly.

He has had this Bard Port-a-cath since June of 2015 seven months plus with no problems. Going to surgery every seven months seems a bit scary to me.  Not only that but what are these clot busters doing to his body and or blood?  He had clot busters on January 4 and again February 18…every six weeks?  I am not a Doctor but there must be something wrong with this picture. 

Friday his computer was corrupted by some super virus.  HP is working on it, and luckily he had back upped his computer.   This computer was brand new December 15 2015…so it is only two months old.  Did you know that warranties are voided if you have a virus.  Me neither.  He runs Virus Software daily. Time will tell  if it is repaired or not.
 

A little sunshine

Yes the rain turned to ice and now the walk is slippery…but we had some sunshine.

We almost had some color near sunset too.

I scraped on the ice some…where the snow fell off the roof and scared Chance…that snow is like cement….if I had a pick axe I might be able to budge it.  I hope for a melt…and that rain can stay away it is nothing but a headache.

Nothing is new…no big projects have been completed lately.  Same old same old…laundry, dusting, wonder what to cook and do up the dishes. We might be in a bit of a rut.

Far Guy has his Neurologist’s permission to decrease his Tegretol…he is down to one tablet a day now with hopes to be Tegretol free in a month.   The Aralast that he is infused with every week for his Alpha 1 Antitrypsin Deficiency has made a difference in his Trigeminal Neuralgia pain OR his Trigeminal Neuralgia is in remission after eight years.  We wish we knew which one was responsible.  From my perspective, I think Aralast could be a cure for Trigeminal Neuralgia pain…but I am not a medical researcher or even a Doctor… I am just  an observer.  Recently I read about a study being done with Tegretol as a medication to fight off the liver damage in Alpha 1 Patients.  I have a cousin who is in Medical Research, he is working on trying to find a researcher or two that might be interested in researching a connection with these two rare disorders.

Frosty Trip

Yesterday it was frosty and foggy.  It warmed up to 19F or –7C eh!  A warm front moved in.  There were patches of freezing rain. 
Wonderful time to have to drive to a big city for a Doctors appointment.  Especially an early morning appointment.

The highline wires lead the frosty way.

The roads were actually pretty decent and sanding/salt trucks were out.  We made our appointment in plenty of time and were called in earlier than our appointment…no waiting.   The new Pulmonary Dr K.  is going to be alright I think, he explained some test results and prescribed two doses of antibiotics to be kept on hand.    The liver scan showed some liver damage because of the Alpha 1 Antitrypsin Deficiency…not a surprise.  We got to see the MRI of Far Guy’s lungs…and where his port was placed.

He answered all our questions about Far Guys port.  We have been having problems with the port.  We were taught at the infusion center to check for a blood return before giving the infusion.  Lately blood returns have been few and far between.  Two weeks ago when I was gone Far Guy went to the infusion center where the gals checked his port for a blood return and couldn’t get one either, he received a medication called Cathflow.   It is a potent blood thinner…it usually takes a half hour to break through a clot or fibers at the inside end of a part…well it took an hour to finally get a blood return.  Since then we have not had a blood return….even though Far Guy is well hydrated (think floating away)and he does everything but stand on his head.  Dr.K said “What is the intention of your port?  To check for blood flow or to administer medication?   If it is to administer medications and they still flow through the port then everything is okay. IF at some point we cannot get anything to flow through the port then we will get a Radiologist to feed a wire through and clean it out.”  Alrighty then…we are supposed to keep keeping on. 

Far Guy can do his infusion from start to finish, both with me sitting across the room         ( when I was ill and germy) and then when I was taking care of Jen he did it all by himself and Facetimed me to show me that it was going okay other than he got no blood return. 
The infusions are given weekly on Thursdays…the protein only lasts 5.9 days in your system.  He had more lung function tests yesterday, he is holding at 21% function.   That is good news…not that the 21% is anything to celebrate but the fact that it is holding means that the infusions are doing what they are supposed to do = protect the lungs.

We were both really discouraged yesterday when the CDC came out with the blanket statement about antibiotics and how they are over used.  To a person with undiagnosed Alpha 1 and bronchitis…without antibiotics to fight the infection your lungs will be destroyed bit by bit…part by part…that is what happened to Far Guy…the medical community failed him. If he had been properly diagnosed even three months before he was it would have made a difference…a diagnosis a year sooner and perhaps he wouldn’t be on oxygen.

Bottom line, if you know someone with Emphysema, Asthma or COPD…they should be tested for Alpha 1…it is a free test…and can save someones life.

Far Guy’s funny for the day was as we were driving away…his Oxygen Bottle slid around in the back seat, making the oxygen tubing pull at his nose…he said bad word a couple of times right out loud and ripped the nasal cannula off.  I told him to calm down…he said no one realizes that where the hose goes so does the nose.

Adam: Basketball and A Gene

We traveled up north for Adam's basketball tournament.  Have I said before that Adam is quite tall for his age?  Well he is and with some practice and a bit more confidence he could control the game.

This is a basket during practice, he is number 31.

He made several baskets during the game, none of which I caught on camera ...sometimes it is just so exciting to watch! 

Yes he is  real tall.

The ref pointed out the height difference before the jump ball. 

He he is a head and a ball taller than everyone else. C 'mon Adam give the little guys a chance!

Adam

This was the last game of the tournament.  All the boys had been playing since 8 AM, after this game was over (they lost by 4 points) they were headed off as a group for some food. 

They played a really good game.  We were glad we could be there. 

This week we learned that Adam is our only Grandchild that is a carrier for the Alpha 1 Antitrypsin Deficiency.  ( Everyone else tested MM which is normal.)  Adam is a MZ the same as his mother.   If he or his mother should have problems in the future the Doctors will be able to treat them right away before lung deterioration begins.  It is our hope that they will live a full, happy, normal life and the one itty bitty tiny gene won't make a difference.   Was I sad ?  Yes I was...both Far Guy and I prayed long and hard that the grands would ALL be spared....four were and one wasn't.  Yes I think it is unfair...but I am not in charge...God is. 

Second Snow, Alpha1 and TN

Yes it snowed, but it mostly melted.

Chance and the snow along the edge of the woods.

I didn’t even move the snow shovel to the front door yet.  My sister in law has hers all ready but she lives further north than me!

Alpha 1 Update:

Far Guy has a new Pulmonary Doctor, his last one went into Sleep Medicine so we were without a Doc for a bit.  The new one is from Poland, he speaks English quite well and is easy to understand. (He went to Medical School in Iowa) I think he will be okay, Far Guy got a Kenalog Injection..it seems to help with his shortness of breath. We now have antibiotic and steroids on hand in the event that Far Guy gets sick …we are to begin the medications immediately…the instructions were IF AFTER FIVE DAYS HE IS NOT BETTER HE NEEDS TO BE IN THE HOSPITAL.  I like that I have a hard and fast rule to follow.

The every Thursday Infusions are going well for the most part.  They are not as scary as they first were…Far Guy is working on getting the needle sticks down to one good one…he has done four sticks and two sticks…so he is improving.  I made a step by step list of how things are done so he can refer back to it if need be.  Part of the problem is that his port moves around in his chest a bit…so you have to make sure you anchor it.  He had Lynn at the infusion center check it out and she agrees..the port moves.  The infusions always tire him out.  We stick pretty close to home Thursdays and Fridays…only going for a short ride with the dog.

900+ people a year have to give plasma so that this treatment can continue for just one individual.

I have been in contact with my cousin Kurt who is involved in Medical Research. I also mentioned this observation to the new Pulmonary Dr as his wife is a Neurologist.

Since beginning the infusions with the Aralast for the Alpha 1 Antitrypsin Deficiency the Trigeminal Neuralgia pain is much less.  Is there a connection?  Our regular Neurologist says  “No they are not related.”  I am not so sure…how many patients are there out there that have both of these rare disorders?  1 out of 15,000 people get Trigeminal Neuralgia and 1 out of 5,000 people have Alpha 1 Antitrypsin Deficiency.

What are the odds that one person would get two rare disorders?  What are the odds that the treatment for one of the rare disorders would make a difference in the other?

I don’t begin to understand all this…but perhaps it will benefit someone else someday.

What we pass along

We have been waiting for our youngest daughters genetic test results for the Alpha 1 Antitrypsin Deficiency.  She is an MZ…the M from me and the Z from her Dad.  We were hoping/praying that she was an MS like her sister. Her count is low on the MZ scale…she is just 3 points above her Dad. 

HOWEVER since she knows her status she can seek treatment if necessary before her lungs or liver are damaged.  She needs to have a liver function test at least once a year. Since she leads a really healthy lifestyle she has a lot going for her.  Many people that are MZs have no problems….from what I have read it is like the roll of the dice.

There is nothing we can do…nothing we can do or say to make it all better.  We can only be good listeners and give advice to the best of our ability… and pray.

“If you pray why worry and if you worry why pray.”

If we had known years ago before we had children that they would be carriers…would we have had children?  Probably…but we didn’t know…just as we didn’t know if she would have my hair or her fathers…

Jen and her dad  September 2015

Obviously she has my hair!