Friday, October 31, 2008

Clinic appt.

Wednesday I had a check up appointment at Akron.The PFT showed FEV1 at 43% and 1.02 liters.Not a big improvement,but I will take it.Also,my culture showed a bit of aspergillus and another fungus (I forget what it was called).She said it's very common with CF ...I just wonder how I get this stuff!!So now I'm on an antifungal med.They did another culture yesterday.She also thought that my laryngitis was caused by the TOBI,although I did have a bit of a sore throat too,which probably made it worse.I was still squeaking yesterday,but today,I'm finally able to talk again,still not quite back to usual,but close!I go back next Wed.when they will decide if the picc will be pulled or if I'll need to keep doing the IV's for a third week.The tobra IV is now changed to every 36 hours instead of 24.I have to keep closer track of that schedule.I can practically do the IV thing while I'm asleep,I'm so used to it.At 5:55 in the morning or midnight,I AM practically doing it in my sleep!

My dad is coming home from the hospital today(I think).He had a partial knee replacement on Tuesday.He seems to be doing pretty well.My sister will stay overnight to help him for 3-4 days.I wish I could help more...Please pray for him to heal well and for my lung function to improve!Thanks.

Monday, October 27, 2008

Quietly resting

Quietly is the key word here...I have laryngitis,serious laryngitis.I haven't been able to talk other than whisper for 6 days.I don't LIKE not talking.Yes,I know I'm home by myself all day,but I do like to maybe have some conversations with my dog occasionally.And phone calls sometimes.I have been doing a lot of text messages...I think the reason I lost my voice is I started a new inhaled antibiotic medicine called TOBI,which targets the pseudomonas.It takes at least 20 minutes and it just made my throat raw,it was brutal!I have to do it twice a day for 28 days,then off 28,then back on.Hoarseness is a side effect,but this is beyond hoarse!!So I don't know if it will get better as I get used to the med.or if I won't be able to talk for 28 days.Yikes!!It's just annoying...

The IV's are going OK.The nurse was here this morning to take blood and change the dressing over my picc.She said my lungs are sounding better,so I guess it is all worth it.I go back to Akron on see what the doctor says.Hopefully I can stop the IV's as planned in another week.I'm glad I can stay in today,it looks like it could snow.I'll just curl up and keep on quietly resting...

Wednesday, October 22, 2008

Back on IV's

I had a clinic appointment last Friday afternoon at Akron. PFT's showed FEV1 down to 42% and .99 liter.Eek!For my height and weight,I should be taking in 2.38 liters of oxygen. I have been coughing more and my chest has been feeling tight the last week and was sounding crackly and wheezy again.My doctor said she felt I needed to go back on IV's,but thankfully, is letting me do them at home instead of the hospital.I was a bit depressed that I needed them again so soon,but glad I can do them at home.And I AM glad they're treating me aggressively.So,Monday morning I had an ENT appointment and after that I had to get the picc line put in again.It did not go quite as smoothly as last time!The line was in too close to my heart and making my heart do funny things,so the tube had to be pulled out about an inch.It was quite a long tiring day!

Tuesday,the home health nurse came and brought my IV equipment and meds,taking over my fridge again!I needed a refresher course in administering the meds,but it came back to me pretty quick.I am on two different antibiotics than last time...tobramycin and ceftazidime.I run the ceftazidime every 8 6am,2pm and 10pm.Each time takes 30 min.The tobra is from 1 to 2pm.I had to have blood drawn before I started and 6 hrs.later.The nurse will be back on draw more blood and change the picc dressing.They need to do blood levels because the tobra can affect the kidneys.I also have to add an extra vest and inhaler treatment in the afternoon.So the next two weeks,I'll pretty much just be doing lots of treatments,trying to get back on track again.I did have a couple of good months after the last IV's.I really felt pretty good for awhile!

Sunday, October 19, 2008

July 25 clinic/hospital

My first clinic appointment was set for July 25. My hubby went along. As soon as I checked in at the window,I had to put on a for me.I had to leave it on until I was in the patient room. I first saw the nurse,t hen the nurse practitioner,who went over my history.Then I put the mask on again and went down the hall to do a pulmonary function test (PFT's).You have to do it 3 times and they take the best one. My FEV1 this time was 48%. Quite a drop in just a few months.Then back to my room.Doctor listened to my lungs,which sounded very "crackly". Because of the PFT and lung sounds,I was told I needed to be admitted to the hospital for at least a week of IV antibiotics.The nurse took us over to the adult CF wing (connected by a walkway).She showed us around a little.There were no rooms ready right then,so they told us go have a "date" first and come back in a couple of hours.We were both hungry and I wanted to get a book and a toothbrush for the hospital!I wasn't prepared for this!So we found a Max and Erma's and a Sam's Club.Then headed back to the hospital.The rest of the day was a flurry of tons of people coming in,asking lots of questions,bloodwork,x-rays,doctor coming in,more questions,having trouble getting an IV in,finally successful.Student nurses,nurses,technicians,lab people.It was exhausting!!They had trouble getting an IV veins are very small.They had to redo it because it wasn't working in my hand,and finally after midnight they had it in right where you bend your arm and I was left alone for a few hours to sleep!

I was in complete isolation for awhile because a few MRSA bugs were growing in my culture,but just a FEW...the pseudomonas were still there.Everyone on staff had to mask,gown and glove to be in my room.I was confined to the room for a few days,then I was allowed to mask up and go outside awhile,which was soooo nice.Also,the second day I was there,a picc line was put in my arm.It was sort of a surgical procedure done in my room with a specially trained nurse.She explained everything before and during the procedure.It took about 40 min.for her to set everything up and about 8-10 actually do it.The picc went in just above my elbow and ended close to my heart.She watched an ultrasound machine to guide it in.Right afterwards they brought a portable x-ray machine in to make sure it was in exactly right...and it was.The picc was so much more comfortable than the IV had they could run the IV's through it and also take blood.

I did feel weird being in a children's hospital,even though I was in an adult wing.Here's an interesting piece of trivia...I found my baby book last week.I was in Akron Children's age 2 weeks and had surgery for pyloric stenosis.Mom had saved the hospital bill.The room was $14.50 a day,total of $203,operating room was $20,labs $15,drugs $126.45,misc.$ was $368.25!That was a long time ago...You don't even want to know what the cost of THIS one week was!

I really liked my dr.I had to try different antibiotics.I got sick on cipro,so they gave me phenergan,which put me to sleep for a whole day.I slept right through my treatments and IV changes...I was OUT!They did find 2 that worked,so I was on 2 IV's 3 times a day,4 vest treatments,along with nebulizers.After 2 days,I started going to physical therapy every day(mask on,of course).I learned stretching exercises to expand my chest muscles for optimal breathing capacity.The therapist also took me outside for walks to measure my oxygen level while walking.I was kept very busy in the hospital.In between time,I could be on my laptop,read,watch TV or just rest.I needed the rest actually.Oh,and the food was quite good,compared to other hospitals I've been in. The highlight of the week was when my two sweet grandbabies came to visit!

They did a lot of tests,finding out that I am pancreatic sufficient,OK in vitamins A,E,and K but low in D.I had a bone scan done,which came out pretty normal.After one week in the hospital,I was sent home to continue on the IV's for two more weeks.That was a challenge too,but at least I could be home!

Friday, October 10, 2008

Finally,a diagnosis

In May of 2004,I was hospitalized with a MRSA infection and I missed the last two weeks of teaching.My health seemed to kind of go downhill after that.The following year in May, my doctor did a bronchoscopy,suctioned out mucus and washed out my lungs and said I really needed to think seriously about quitting teaching because my lungs look so bad.Again,I missed the last two weeks of school.And that summer,I made the decision to "retire" and concentrate on taking care of my health.He did two more bronchoscopies over the next year and a half.In theory,cleaning out the lungs and starting "fresh" is a good idea,but it is an invasive procedure and for me,it was futile.Within a day or two,I was back to my usual coughing self.The dr.also had me start using the percussion vest.After my 3rd bronchoscopy,I decided to change doctors at this point to get another opinion.A lady in our church has a lung disease called aspergillosis,and I knew she really liked her doctor,so I made an appointment with him.My first appointment with him was a year ago in Oct.He had me do my first ever pulmonary function test and my FEV1 score was 58%.He did the whole CT scan of lungs and sinuses in high resolution.He thought I might have immotile cilia,the newer name being PCD (primary ciliary dyskenesia),which is basically the same thing as Kartagener's.I googled it and researched and thought it fits me pretty well.(You know how you can google and try to diagnose yourself!)I told him I wanted to know for sure.You have to do a cilia biopsy and need a special microscope.The disease is somewhat similar to CF.After my third visit with him,he decided to send me to a CF doctor from the Cleveland Clinic because he wanted another opinion.So a couple of weeks later I went there,armed with all the info.about PCD,expecting him to set up the cilia biopsy,etc.Instead,he reviewed my history,asked lots of questions,then said his gut feeling is that I have CF,not PCD.This was before even listening to my lungs or doing an exam.It took me by complete surprise!!He sent me directly over to Akron Children's Hospital to have genetic bloodwork done,sweat test,another blood test to rule out Alpha-1 antitrypsan.There I am in a children's hospital waiting room with cartoons on TV,play table,etc. and then I was escorted to a lab room with a cute bus table to get blood drawn and arms wrapped in pretty pink polka dot wrap stuff for the sweat test.I also had to do a culture test so they could see if any bugs are growing in my lungs.

So then we had to wait a few weeks for the test results.It was June 4th...The doctor called me with the news.My sweat test was not terribly high,he thought it was a false positive,the Alpha-1 test was negative,as he had suspected.But the culture showed pseudomonas areuginosa,a very common CF bug colonized in my lungs.I've learned that once you have this,it's almost impossible to get rid of.The genetic testing showed that I have one mutation that is very common in 70% of CFers,called delta F508.The other gene mutation is not as common.So all of a sudden I learn that I do indeed have cystic fibrosis.It was a bombshell...I had seriously thought everything would come back negative.It was not what I wanted to hear!But on the other hand,I finally had a diagnosis.Now I KNOW why I am like I am.

I went back to see him in a few weeks.He went over all the tests and talked about the medicines I will probably be on and why.He said he wanted me to go to the Akron CF adult center.He said the doctors there are excellent and it is an accredited CF center.Also,it's the closest center for me.So he was going to talk to the doctors and set up an appointment for me.It seemed to take forever and I wanted to get going and get started on the right treatment.My chest had been aching all spring and summer and I had no energy whatsoever.Also my joints were hurting really bad,especially my knees,fingers and shoulders(joint pain can be related to CF).So I was very ready for the appointment.Finally,it was set for July 25...

And now I'm off to celebrate my perfectly imperfect life,because it's my birthday!

Sunday, October 5, 2008


This might be boring,but it's part of my life...After the bowel obstruction surgeries before my wedding came a surgery to remove severe endometriosis,a mastoidectomy (major surgery for an ear infection),more tubes in the ears,then a sinus surgery with a new ENT (ear,nose,throat) doctor to correct a deviated septum and drill some holes somewhere in my sinuses for more drainage.Not fun!Then the lobectomy...removing a good part of my left lower lung.The thinking there was that if they removed the worst of the bronchiectasis,it would "cure" my lung problems.I got pneumonia after surgery...It was a long recovery from that one.A few years later,myOB/GYN found enlarged ovaries,so I needed another surgery for possible ovarian cancer...turned out to be endometriosis again.I had been referred to a specialist in Akron for this.I told him about my lung problems and that I get pneumonia whenever I have general anesthetic,so they did breathing treatments before and after surgery.This time, my lung collapsed.He came in and said,boy you weren't kidding about your lungs and sent in the pulmo team.In the late 90's I had to have a foot surgery done and I requested the "twilight"sedation for that.That worked better...Next was another sinus surgery in 2001.I had switched ENT doctors because I hadn't liked the other one at all,bad bedside manner.So this is my 3rd ENT.He mentioned that I might have something called Kartagener's syndrome,but he did research on it and said no,probably not,because my organs would be reversed if I had it.It kind of made sense to me though,because with this disease,the cilia hairs in the sinuses and lungs don't work right,so you have excessive mucus build up and sinus problems.When he did the sinus surgery,he used the twilight sedation at my insistence.He had never done that with a sinus surgery before,but he agreed to it,with the condition that if I needed to be put "under" at any time during the surgery,he would do that.But I tolerated the lighter sedation OK.I also started going to an allergist/pulmonologist about this time,because I thought maybe I should be tested for allergies again.Negative on the allergies,but he did find that I had acid reflux.He put me on Prevacid,which helped a lot,I could eat again!He also did bloodwork and found my IgG levels to be low,so he thought my immune system was deficient,so I started getting IV gammaglobulin treatments once a month at Aultman hospital.A CT scan showed more bronchiectasis had developed on the right side of my lungs.Now I know I have bronchiectasis,chronic sinusitis,acid reflux and my immune system doesn't work properly.So I'm thinking,OK,I'm just a very weird person!Why am I like this??I didn't know anybody else like me.I was on antibiotics several times a year for flare ups,coughing,not a lot of energy,feeling yucky a lot of the time,but trying to lead a normal life like everyone else...Just so this doesn't sound too depressing,keep in mind that this was over a span of years and I DID have times where I felt good too.Next up,the rest of the story...