Cystic Fibrosis: A Personal Story About Experiences with Ports

When was the first time you were introduced to a port?

In 1991 I was hospitalized for yet another lung infection. At that time I was 26 years old and had already been in the hospital more times than I could remember. As usual my veins were burning up from the antibiotics at the rate of about 1 a day. I had tried home IV's but the access was too much trouble. The home nurses could not get a line in and would send me to the ER.

You want to see the staff of the ER clear in a hurry? You should have seen them vanish when I showed up every day with my poor bruised and battered arm. One nurse would have to go on break; the others suddenly all had to attend to urgent matters. Inevitably some new house officer would get pushed up by everyone else to give things a stab, and a stab and a stab.

So, I decided staying in the hospital was better. On this stay my regular doctor was on vacation and the guy covering for him suggested that I get a port before I went home. I was not too sure about the idea and really wanted to wait until I could talk to my regular doctor. But, the nurses and house officers asked me at least twice a day if I was ready or not. Finally I said yes.

Please note here people: it was the first week of July. This is the week new residents come on board in the hospital. My recommendation is that you should never have elective surgery that week. Let them all get used to things and then get your procedure done.

Anyway I won't thrill you with all the details of the surgery but one interesting thing was that I was not fully anesthetized. I had enough anesthesia so that I could not speak but not enough so that I could not feel pain. I could hear everything but because there was a sheet over my face, they could not tell I was in pain. YIKES!!! They were talking about what they had for lunch! When I got to recovery I still could not speak but let out primordial screams.

Do you use the port?

No, I don't. At the hospital they trained me how a nurse is supposed to access the port. They showed me the "huber" needle. It is a needle that is about an inch long that is bent at a right angle. Tubing and a clamp come out of it. The port itself is a hard rubber disk with a stainless steal backing.

When they access it they are supposed to start with washing their hands and then creating a sterile field including using sterile gloves.

How did it feel?

At first it hurt to puncture the skin but as it got used scar tissue built-up and it was actually the most comfortable of all the IV's I have ever had.

What is daily maintenance like with a port?

The first time I ever had home IV's with the port, I had a new nursing agency. It was the agency my insurance had picked out; at that point I did not have a preference of agencies or nurses.

This nurse came in and she did not wash her hands. She was not planning on washing them but I made such a fuss that she finally conceded. Then she had me lay down on the couch to access the port. This was not the way they did it in the hospital but I went along with it.

The nurse kept on jabbing me and it kind of hurt, but because the port was new it did kind of hurt anyway. But...it still seemed to hurt a lot more than when they did it at the hospital. The nurse said my breast tissue was in the way. Yeah, right! She then got another new needle and tried jabbing away again.

Finally, she said it was in but it sure did hurt. The nurse hooked up the Tobra and let it run.

YOWEEEEEEEEE!!!!! Something was really wrong. She was nowhere near the port. She had just stuck the needle randomly into the area of the port and now the toxic burning Tobra was just eating into my tissue.

No more Mrs. Pleasant. I ordered her to get out of my house NOW! She wanted to fiddle with things some more. Then she wanted to sit and write an incident report. I told her to get out or I would really give her something to write about.

I would go to the clinic and see what the hell she had done. The supervisor came the next day with this crappy nurse. I would not let the nurse into the apartment again though. Nope. Done with her. The supervisor stood up for the woman because she was inexperienced. Too bad, I did not need that.

We are human and make mistakes but the incompetence started with her not washing her hands. Hello, nursing 101.

What do you think has kept you alive so long?

I am always kind of on guard that things be done correctly so that I do not get any more infections.

In 1995 I had to go the ER one night for a bowel obstruction. I live alone and almost always go to the ER alone, and then call my friends when things are settled. That night I was feeling horrible. The nurse came into the room and said he was going to start my port.

He got out the huber needle and dressing kit and threw them on the gurney. Then he put on gloves from the box in the wall. I reminded him that he needed sterile gloves so that I would not get an infection. He tapped my shoulder and asked me to lie down. He said he knew what he was doing and was not using sterile gloves. He opened the huber needle and held it up and said he only needed to keep the needle from being touched.

That's not what I was taught. But that night I was just too sick to argue or get up and leave or even ask to speak to a supervisor.

I ended up getting sepsis a few days latter. I had made a mental note of the nurse's name and told the doctors about what happened in the ER. He claimed he did the procedure according to the code when they asked him about what he had done. It was my word against his.

In the end, they had to pull the port out. Unfortunately, when I had gotten the port they told me it would last me a lifetime.

I guess I was lucky that my lifetime was longer than the port but still!

A second port

I had a new port installed on the other side of my chest wall. The scars kind of kelated but really are not too bad.

I volunteer at the University of Michigan hospital to be a lung model for 1^st year medical students. The attending physician always asks them to guess what the scars are from. If they seem like a dull bunch I tell them it is where my breast implants were removed. It is funny to see them nod like they understand. The scars are well above my breasts!

How do you travel with a port?

In 1999 I ended up in the hospital while I was on vacation in Orlando. I had come down with the flu. My friend and I were seeing the sites in Orlando as described by Vegetarian Times magazine. The magazine noted that the largest vegetarian cafeteria in the United States is at Florida hospital in Orlando. We laughed and agreed we would not be going to that destination. Yet, that is where I spent the last 3 days of my vacation.

I always worried about strangers touching my port and messing it up but felt OK at this hospital because they specialized in cancer. Often cancer patients have ports to get chemotherapy. So, I kind of trusted that they knew what they were doing. The only thing was that they did not put as much heparin in the line as I usually did.

I was so glad to go home. My poor friend was stuck worrying about me and alone on vacation. I was worried that I would not make flight and wondered what I would do then. My fever went down just in time. My friend took great care of me on the way home.

When I got home though my port did not work very well. It was kind of sluggish. My regular home nurse kept trying to pump it and other ideas she had to flush it. She even volunteered to go to the clinic with me to get it checked out.

What are the dangers and risks of ports?

I will never forget that day. At the clinic it was a nurse I had never seen before and it was kind of nice to have Linda, my home nurse with me. She came to the clinic for new ideas on what to do. She had been trained never to force flush the port.

Yet the nurse in the clinic kind of whammed on the flush and got the thing to work. She looked over to Linda and said sometimes you just have to force things. When she did it, it did not really hurt-it just felt forceful. Kind of like somebody jabbing you hard on the shoulder with a finger. It does not hurt but you feel the power. That day was April 14, 1999.

The next day I went to work and felt funny. Nothing I could put a finger on exactly. I wear a headband every single day and have for years. That day the head band felt too tight, and I had a vague headache. The headband had never hurt before.

My co-workers asked me if I felt OK. They thought I looked a little off but nothing specific. The next morning, a Saturday I woke up and my face was swollen so much it was hard to move and make a smile in the mirror. I had never had THAT happen before. So, I called my primary care physician and went to the ER.

In the ER the house officer kind of made light of it. He thought I might just be fat. He asked to see my driver's license and said he could not tell anything was wrong. They did the usual blood tests, took a chest x-ray and looked at me and scratched their heads and said I looked fine.

After 6 hours I felt a bit better and decided to go home before I picked up some ER germ. The rest of the day I felt like things were getting better and better.

The next day, Sunday, I went to church. When I got there everybody came over and asked what was wrong with my face since it was all swollen. It was just like the day before when the doctor said I was fine.

My friends all fawned over me that it must be an allergy since it was spring. Whatever! The next morning I was all tomato faced again; it was big and round. I could not smile, and I had no lines in my face.

I went in to see my primary care physician. She looked at my face and thought it was an allergy too. She gave me a prescription for Allegra and sent me home. Again as the day went on the swelling went down but I did not feel quite right.

The next morning my neck was swollen and stiff. It felt like a tree trunk because I could not move it. My face was all swollen and rigid too. So, I called my trusty CF doctor. He listened to me on the phone and had me come in to see him in the afternoon.

The only thing I was worried about was that in the afternoons the swelling would go down. But that day it went down only a little. He had me put on a gown and started examining me from head to toe. At my PCP's office I had kept all my clothes on and she had just looked at my face.

After Dr. Simon examined me he said he wanted to look in the mirror and look at my chest under my chin and tell him what I saw.

I looked and it was all veiny!! Veins that I had never seen before. He agreed with me - I was all veiny. He asked why I had not noticed this. "Well", I replied I have not been trying on any ball gowns lately".

I mean, how often do women need to look at their chest? My dad said men stand bare chested and look in the mirror to shave every morning so they notice their chests. Whatever.

Dr. Simon left the room to make some phone calls and when he came back, he said we were going down to get an ultrasound of my arms right away. He thought I might have a blood clot. He came with me and watched the tech do the procedure and asked her all kinds of questions.

I always loved Dr. Simon but I felt so cared for by him that day. Unfortunately, the tech found nothing. Dr. Simon said we would have to wait and see because he was not sure what was wrong.

By Wednesday morning, I was still swollen in the neck and chest but there wasn't as much of a shift in my condition from morning to evening. Now I sure was looking in the mirror at these gross veins on my chest wondering what they were.

On Thursday morning I called in sick because I could not turn my head to drive the car. I hurt a lot more. My head hurt when I lay down so I sat up. I called Dr. Simon again and he sent me to an allergist.

The allergist was a nice guy. He asked me to tell him what had been going on. I told him about going to the dentist the week before and that my port had been flushed. He studied me and just shook his head.

Since the pain was positional it was defiantly not an allergy. He listened very carefully and said that I had probably told him all that he needed to know to tell me what was wrong but he just did not have the knowledge to know what to do with the info I gave him.

Dr. Simon said to wait some more. OK, but every day things seemed to get worse. The next week he sent me to Dr. Fine. One of the leading primary care physicians in the country. Dr. Fine had no clue either but suggested I get a head and neck CT.

I have had lots of CT's so I had no fear of the tube or the IV with the dye. But when they injected the dye my arm hurt so bad I wanted to jump off the table. Afterward I had quite the headache too. The tech said I was fine, the scan went fine I could go home. I felt too off to drive so I lied down in the hospital lobby for a while until I thought I could sit in the car and make my way home.

At this point my neck wasn't too bad at night so I could drive but after that CT I really should have called a friend or a cab. Both options seemed like they would take too long so I just went home very slowly.

On Monday, Dr. Simon called me back and informed me that I needed to get my CT done over. When they looked at the pictures no dye had gone through. That was odd, something must have been wrong with the IV.

I KNEW it was not supposed to hurt. When they did the redo this time I had a friend drive me. The dye still really, really hurt. And the headache after wards, was even worse.

Meanwhile, I also got an appointment with my rhino-otto-laryngologist. He specialized in neck stuff so maybe he could help. He did not seem to think things looked too bad. He looked in my mouth and said there were no veins showing in my mouth so it could not be superior vena cava syndrome and wished me luck with whatever was wrong.

The second CT looked fine. Rats. What is wrong with me? I kept wondering. Next I got an appointment with a cardiologist. The doctor again was very nice. He said he had no idea what was wrong either. I had all kinds of tests looking for cancer. A clear chest x-ray and a clear CT scan.

He said I was not crazy though or just fat. The vein pattern on my chest was not something you could just conjure up. This felt reassuring but not helpful. This doctor sent me for a chest CT. I dreaded this because the last two felt terrible and the tech said I was over-reacting. (Deep sigh).

I had the chest CT on a Friday night and on Tuesday night at about 7:00 Dr. Simon called me.

He asked if anybody had called me about the CT. No, nobody.

He had just gotten back from his vacation and first thing he did was look up my records. He wanted me to the ER right away as the CT scan showed a blood clot in the superior vena cava in the area of the port.

I wanted to stay home and go to the hospital in the morning figuring they would not do anything overnight but he insisted that I had to go immediately. Off I was to be admitted.

They decided that I had to have my port removed because a blood clot was causing an obstruction in the superior vena cava.

Scary things happened while I was in the hospital.

It would suddenly get very hard to breath and I would feel intense pressure building in my head. I would see strange spectrums too. These episodes would last about 10 minutes and then go away. The doctor came and sat by my bed. He assured me that he believed these were real but he did not know what to do.

The port was removed and supposedly I was all better. I felt a little better when I went home from the hospital but not great.

Warning signs of other complications?

While I was in the hospital my grandmother from Massachusetts flew in with a friend for a vacation we had planned over the Memorial Day weekend.

However, I could hardly stand riding in the car as a passenger because my neck and face were still so swollen it was hard to turn my head still. I could barely walk one block down the street because I would have such intense pressure in my head. All I really wanted to do was sit on the couch as perfectly still as possible.

The doctors said to just wait and things would get better. After they removed the port they put me on Coumadin. I told my uncle this and he let me know that on his farm coumadin is rat poison. The rats eat it and it causes them to bleed to death. Great.

Grandma also reminded me that Grampa had a severe bleed out from coumadin. He had to stay in the hospital for a week. "Great! I am taking rat poison that made my grandfather really sick and I still feel bad!"

I stopped calling the doctor after that and just went to the lab for the clotting tests required when you are on Coumadin. But each day I kept feeling a little worse and a little worse.

After almost two weeks after my port had been removed I could hardly walk around my very small condo. The pressure was too much. I almost never slept because it was too hard to lie down. I mostly sat on my back deck, the couch or the front steps.

One morning my neighbor Andy took his dog out at 6:00 AM and I was sitting on the front steps. When he came home from medical school at 4:00 PM I was still sitting on the front steps. I had sat there all day because I was out of energy for any more activity. Any activity just caused too much pain.

My neighbor asked if I had been there all day.

"Yep" It was even hard to talk at that point.

He wanted to take me to the ER. He looked at me and not only my head and neck were swollen but my fingers, chest wall and arms were too. At that point I could hardly even fit into my grandfathers old shirts because of the swelling. Plus, he said I was frighteningly blue colored.

"No, no ER was all I could say. The ER just seemed too frightening. It would use all my energy.

Andy brought over another neighbor to look at me. She was a nurse. She agreed with Andy that I looked pretty bad. They both offered to stay with me.

"No, as long as I can breath I am not going to the ER!"

That night though I could not breath without a lot of concentration. I kept as calm as possible or the pressure would be too much to bear.

Coughing? I could not cough. It did not seem like my chest could even move to cough. I just felt like I was suffocating. I meditated and visualized breathing so I would not waste my energy.

In the morning I asked Andy to take me to the doctor.

"Sure" was his relieved reply.

The doctor was my back-up pulmonary doctor instead of Dr. Simon. I had almost forgotten about him. I felt relieved that I would be seeing somebody that I knew because I had no energy for nonsense.

I knew something was up when he asked if the medical student could look in my eyes. That never happens in the pulmonary clinic. So, I asked what they were seeing. Apparently my brain was swelling and pushing my eyes out.

It was actually a relief to go into the hospital this time. Whatever was wrong, I really had no more options for just taking care of things at home.

The hospital bed was really comfortable. Sitting up and feeling the soft mattress was such a relief from just sitting on the front steps.

In the morning I felt a little better. Sitting up and not having to walk more than a few steps had relived some of the intense pressure in my head.

The doctors ordered an MRI which was about the only test I had not had yet.

The MRI was torture. Moving around, getting on a gurney expended energy that I didn't have, and caused the pressure to soar. Being still for the procedure was unbearable if not impossible. It was very hard to breath. They said to breath at certain intervals but I could not hold my breath that long. When they shot the dye through it was just as painful as for the other tests.

While I was still on the MRI table the tech came and said they wanted to do the procedure again- the dye did not go through.

Yeow! It was a relief to be back in the good old hospital room in that comfortable hospital bed. For me, that is a sure sign that I must have been sick!

Very shortly after that, the whole team of doctors came up and the house officer spoke. He said they had made a terrible mistake. I was much sicker than we thought.

The MRI showed no blood flow at all. He said they were not sure how I could be living with a situation like that. He said they had made an aggressive treatment plan.

"Humph, what does that mean?" I could barely get it out.

The plan was for interventional radiology to put a stent in the superior vena cava. This plan was considered aggressive because they had never tried putting a stent into the SVC and the odds were I had a 30% chance of surviving the procedure.

I did not need to ask the odds if I did nothing.

Down in radiology I met the doctor who came up with the plan. He was not going to do the procedure because he had been on duty for too long. He was pretty excited about it though. He took pictures to show medical students the before and after.

One thing though-he had me lying on the radiology table and then tried standing beside me for the picture. No, it wouldn't work - it was not symmetrical! He stood on a stool with wheels for a different angle.

"Yikes doc!"

I am certain risk management would frown on doctors standing on stools with wheels with big heavy cameras, leaning over patients. But the angle was still wrong.

He climbed aboard the table! He nearly smashed his head on the radiology stuff hanging from the ceiling. Then he straddled me on that little narrow table to get just the right picture.

The procedure went great. To put the stent in they made a very small incision in my groin and at the base of my neck. The worst parts were shaving my nether regions and taking the band aids off.

As soon as I woke up I felt like a great weight had been lifted. It was as if I had been wearing the lead apron they put over you at the dentist for months.

When I got to my room though some crazy stuff happened.

My friend was there to keep guard over me and she was reading me short stories from a book I like. In the middle of every story I would get a wash of pain and shortness of breath that lasted about as long as it takes to shift a car into gear. Then it would subside.

I had to lie perfectly still for 6 hours so I would not bleed out of the puncture wounds. The doctor could not explain what the pain was other than maybe it was the blood flowing for the first time in 8 weeks. Things were unsnarling in there.

The next morning I had no pain from the procedure and the swelling in my neck was down enough to want to try and move it. It was all stiff though from not moving for so long.

On the second morning after the procedure I had lunch and all of a sudden my fingers turned pink again and I felt nearly like my old self. Tired but happy that I was coming too again. Hurray!!! The doctors let me go home that afternoon.

It was father's day and boy was I glad to call my father and celebrate that he still had a daughter.

The rest of the day was spent calling friends and family telling them I had come back to life. It was amazing how much better things were so fast. The good old UofM had done wonders for me again.

That night I woke up at about 1:00 AM with a bad dream that I was swelling again. Oh, what a terrible dream I thought.

But in the morning, I looked in the mirror and I was really swollen. Not a lot, but I did not look as good as the day before.

It was the only time I cried. This really had me baffled. I was supposed to be better now.

Now what? I looked at the clock and gave myself half an hour to feel bad and then resolved to move on to whatever the next challenge was.

I called the doctor and he had me come in. Yup, he agreed I was swelling again. Who knows why. I would have to be admitted again.

This was depressing. I asked to go home and get my stuff. He said fine as long as I was back by 7:00 PM.

That gave me about 2 hours to be home. I went home feeling down. But the tide started to change. A friend from work stopped by and she had a big pile or Wall Street Journals to read. Well, with new stuff to read being in the hospital would not be so bad. My friend also vacuumed for me. I always feel a little better with the house vacuumed. Then another friend stopped in on her way home from work. She said she would take me in to the hospital after we went out to eat. Well, things were really looking up now! With her coming with me I could bring a 6-pack of coke because she could carry my stuff for me.

OK, going in the hospital is not the greatest but I knew I could endure it. Just being there does not bother me too much.

The next day the doctor's decided to do what I call a rotor router on the stent. It was an angioplasty to see if it was blocked. The angioplasty procedure was very similar to the installation.

Afterward I had that same pain that lasted as long as the car shifting.

The doctors said the stent was as open as could be and the pressure was good while lying down perfectly still.

They had done the best they could. They thought I should wait a few weeks to let the blood flow reroute into the superior vena cava again.

As time went on the swelling was less and less but different activities could bring it on. Hand sewing, crocheting, walking more than a half a mile all brought on swelling.

I saw some more specialists. But they all thought the stent was the best solution compared to the risk of trying anything else.

I have come to live with superior vena cava syndrome (SVC) as my new normal. I went on disability after this because my new normal required a lot more rest. I still swell up with activity but I now know it will go away with what my dad calls the couch-cure. I sit on the couch and watch TV perfectly still for hours. No lying down but sitting down.

SVC and CF don't work well together

It is kind of mean to have SVC syndrome and CF. The CF needs activity to clear the lungs. To make the SVC happy as little movement as possible is optimal. Now, I just plan a rest period into my activity. If I travel I get really swollen from all the activity just getting to the airport. So, I plan that the first day I will need some couch time.

Unfortunately riding in the car does not count. Particularly riding with my dad. Moving my head to look out the window at different things is enough activity to make me swell and get a headache.

How do you manage to stay active?

If I want to do an activity known to make me swell like walking my neighbors new dog I just take the penalty of a headache and chalk it up to having fun and exercise. But I plan on skipping something else later on so I can recover. If I totally ignore things then I just get bigger and bigger; the headaches say STOP.

Nowadays I hardly ever get to that point. I know how far to push so I do not have to pay that much of a penalty. To compensate I am less fussy about my garden and raking the leaves. I ask for more help carrying things. I allow lots more time to get crafts like sewing to get done. I travel less.

Recumbent Bike riding

On the plus side I bought a recumbent bike. A regular bike has the wrong posture. The recumbent still makes me swell but it is fun and good exercise. It is really comfortable to just sit on so I can rest some and let things settle down. I don't go very far but tooling around to see the neighbors is a blast.

What are you doing now for medical care?

To date, I have had several roto-routers to keep the stent open. For about a week they help but then things go back to my new baseline.

Source:
Personal experiences of "Eleanor"