one day at a time . . .

Posts tagged ‘My Story’

The Botched Chest Tube

Ok unknowncystic.. Here’s the story…

It’s the summer of 1990, and I’m getting ready for my wedding on September 1st. I had been losing lots of weight – unexplained. I had been visiting a GI dr who diagnosed me with intestinal fermentation??? He put me on several pills and enzymes with no success. By my wedding day I was down to 103 pounds at 5’5″. I was skin and bones. A few days before my wedding I felt back pain. I figured I strained a muscle or something… At this point I was not diagnosed with CF. On my wedding day the pain was still there – but I had the time of my life – it was awesome!

So, we go on our honeymoon to Hawaii and for the first 5 days we scheduled 2 tours a day! My breathing was worsening. I would run out of breath simply walking a few feet. But, even still, went on all these tours! The kicker was one called “Action Hawaii”… You’d think the word ACTION would give us a clue. So part of this was hiking up Diamond Head. It starts of with a nicely paved path. But then, it turns into stone and rocks and an incline. It was so hard! But I pushed through it. Then we reach a point where we had to climb 100 steps! At this point I told my husband – I’ll wait here – you go and take pictures. He said – NO WAY – you’re coming up – so I go on his back and he carries me up ALL 100 STEPS! It was amazing – other tourists were taking pictures of us! By the time we got back down, our group was waiting for us – when they saw us they all clapped. It was so funny – but they must have been like – what’s wrong with this young girl – she can’t even hike a mountain!

The remainder of the vacation was awesome. I wouldn’t let my pains and breathing stop us. We even missed a sunset cruise because I couldn’t make it to the boat in time!

We get back home after two weeks, I go to work and continue experiencing the pain and shortness of breath. Finally, one day at work, my breathing and pain had gotten so bad that I left and went to the emergency room at Westchester Medical Hospital.

And here’s where it happens…

They take an x-ray and discover a collapse in my upper left lung – about 30%. So, the dr tells me he can insert a portable chest tube – it will take a few minutes and I can go home and come back in a few days for removal. He says he can do it in his office, but says to follow protocol he needs to do it in the emergency room setting. I call my husband and tell him that I will have this quick procedure done and meet him at home. He says – NO WAY – YOU WAIT FOR ME. I insist it’s not necessary – but he insisted a bit stronger 🙂

So my hubby gets there, the dr explains what he’s going to do and begins. While he starts to insert this portable chest tube I get this uncontrollable urge to laugh, so I laugh, he continues. Then it happens. As he’s inserting – I seized! Tis is where hubby starts to freak out – and he’s asking what’s going on – they are telling him he has to leave – he’s insisting that he stay – but had no choice. Lots of doctors and nurses were entering my area – all in a rush with concern. My husband was freaking out.

When I came to, I had the normal large chest tube inserted and had to be hospitalized for a few days. I was on morphine for the pain. This was one of the most painful things I’ve ever experienced!

Later, a nurse came to me in confidence and said the the doctor screwed up, let air in,  and that’s why I seized.  No one ever admitted to me that I in fact seized – except for that one nurse. It was placed in the front upper left side of my chest. I have a permanent visible scar – with an amazing story to go with it . . .

My Story Part II – Baby

So, once I reached my baseline, I was able to continue working, and did IV antibiotics roughly every 6 months. Continued my daily therapy, and CF was a part of my life.

The next goal was to try and have a baby.  After 2 and 1/2 years of marriage we began trying. The doctors informed me that it may be a challenge as mucus also clogs the uterus. In addition, at the time, I was still seeing a pediatric CF specialist. It’s not like she had a bunch of pregnant patients!

So after trying for 6 month with no success, it was time to go to fertility doctors. Tests were run, but nothing significant found. We tried several IUIs, with no success. I also had an exploratory laparoscopy done – but again, nothing abnormal. After 2 and 1/2 years of trying, my first IVF was successful! I found out I was pregnant September of 1994! It was so exciting. All the drugs, injections, mood swings to get here was all worth it.

Next, how will I manage this pregnancy? Will I be able to breathe? Will I be able to stay healthy? Will I lose lung function afterwords? Did I make a mistake?

Answers: I just will… Yes… With help… NO… NO…

During my pregnancy, my CF dr wanted to see me every month, and then mid way through, every 2 weeks, and then down the stretch, every week. I did require IV antibiotics twice – just after the first trimester, and again around 6 months. The antibiotics were safe and did not harm the baby. The larger I got, the more labored my breathing – but it was manageable. I enjoyed pregnancy so much. The belly – feeling my baby move – it was awesome. I had morning sickness all 9 months – but I didn’t care. Nausea at night – but I didn’t care.

By 7 months, my breathing reached a point where my dr recommended that I stop working – and that’s what I did. I enjoyed my time at home. I gained 36 pounds – my belly was huge!

Now, all 9 months, the OB and CF specialist stressed that they wanted me to have a natural birth – that a c-section would not be good because of the recovery time. They wanted me to be able to move around and cough afterwords, and surgery would delay this.

By 37 weeks, the baby was large enough and so was I, and they decided to plan an induction. They felt the larger I got, the more risk to me. Well, my son had a mind of his own. The induction did not work! I went back home. Then, I went back, 10 days before my due date. Since my cough was getting worse, they pushed it along by breaking my water, along with an induction. Well – this time it worked!

By the evening I was ready to push. My OB and CF dr were there. So after 3 hours of pushing, I was delirious, and my boy wedged his head in such a way that he just wasn’t coming out! Then he was in stress… So they tried the vacuum – that did not work. So in my delirium, my CF dr tells me we need to do a c-section. I was like “WHAT” – “you said I shouldn’t for nine months” – she said – it will be ok.

Let me tell you. The c-section was awesome! Quick – no pain – my boy was born – and the next morning I was in the shower and walking up and down the halls. They gave me the best drugs. I had no pain. Had I known this, I would have skipped the whole labor part and went straight to c-section!

I was hospitalized for 5 days, I was in the hospital for Mother’s Day. I did 3 weeks of IV antibiotics and felt great! And my baby was the best! He was on a schedule immediately – he ate every 4 hours consistently and was sleeping by 5 weeks!

The one thing I do remember, is the morning after he was born, I had him in my arms while I was all alone in my room. I began to cry and thought “what did I do” – how could I have brought a life into the world when I have CF? He needs me – what if I can’t be there?

What I didn’t realize… was… he was my reason for fighting the fight! to push through whatever I have to!

I do it all for him!

Oh – and by the way – after it all – my CF dr admits she was scared the entire 9 months! We laughed about it.

After doing IVs every 6 months – for 2 years after his birth I required NO IVs! And at one point my FEV1 hit 50% – which is a high for me!

That’s the story of how my boy came into my life.


My Story

Ok… So i’ll attempt to share my story the best I can…

I was born in 1967, a healthy baby girl. No medical problems, lived a normal childhood.
When I was in 3rd grade, I had my first pneumonia, missed one month of school, and recovered.
When I was in 6th grade, I had my 2nd pneumonia, and again recovered.
When I was in 9th grade, my 3rd pneumonia, suspicion began.

This is when I went to a pulmonary specialist at Columbia Presbyterian Hospital, Dr Celia Orez. She suspected Cystic Fibrosis, however, at the time the only test available was the sweat test. People with CF secrete an excess amount of salt in their sweat. This is the basic defect – the salt/water exchange in the lungs is off – therefore causing thick sticky mucus to build up and cause infections. Well, my sweat test was negative. The doctor put me in the hospital for 2 weeks – placing me on high salt diets, low salt diets, repeating sweat tests, and could not get a positive result. She then sent me to a world famous CF Specialist in Boston, Dr Schwachman. He met with me and my family, tok tests, reviewed my charts, and concluded that I did not have CF.

This was such a relief to me and my family, especially since at that time, the chances of living past my teenage years were slim.

After this point, I did begin with a persistent cough. I was never free from it, but it did not impact my life. I was diagnosed with bronchiectasis (a sort of chronic bronchitis) and was being followed up with at Westchester Medical Center by a pulmonologist.

So the years go by, and I get married in the Summer of 1990. Prior to the wedding I began to feel a pain in my back with some shortness of breath, but just brushed it away. Once we were in Hawaii, my back pain worsened as well as my breathing. We missed a boat ride because I couldn’t make it to the boat. We went to a hike, and my husband had to physically carry me on his back to make it up the “100 steps”. I could not submerge myself in water because my breathing would worsen. But we had the times of our lives!

A month after we got back, I was at work and the pain had gotten so bad, as well as the breathing. So, I went to the emergency room at Westchester Medical. They determined I had a partial lung collapse on my left side. They said they would insert a portable chest tube, I would be able to go home, return in a few days, and have it removed. Well – not so simple. During the procedure, I seized, and they had to insert the ‘real’ chest tube and I was hospitalized for a few days. The chest tube was very painful, and they had me on morphine.

I felt much better after this and all was fine.

Then in February of 1991, I had a severe hemoptysis episode – coughing up cupfuls of blood. They did a bronchoscopy (very painful), but could not find the source of the bleeding. I took antibiotics and recovered fine.

I forgot to mention that prior to my wedding, I lost 25-30 pounds, without trying. I was seeing a GI who diagnosed me with intestinal fermentation???? Gave me a load of pills, but never gained weight. I was 103 on my wedding day – literally skin and bones. After a visit with him some time in ’91, he didn’t like the sound of my lungs and sent me to the emergency room at Mt Sinai Medical Center.

This is where it all begins to unfold……

They run a cat scan, some blood work and decide to hospitalize me.

The next day, I was sitting in my bed, alone, and the chief of pulmonary – Dr. Alan Tierstein, came to me and said…. You’re lungs look like swiss cheese… You need a double lung transplant… And you need it within the next six months, or you may not make it… He then tells me he’s going off to Europe and his colleague (Dr Maria Padilla) will be following up with me tomorrow… I begin crying, and he walks out! My husband walks in the room, sees me crying, I tell him what happened and he runs off to find the doctor. He finds him, tells my husband the same story and that was it.

Talk about a bedside manor!

This was a terrible, stressful time for me and my family. I still remember sitting in my bed – with a large group of family all sitting around me – the mood somber – and not much talking. How painful it was for me to see them all in pain. I know they were all worried about me – but I worried about them…

After this, I proceeded with all the lung transplant work up. Months were passing and the fear of what was going to be was terrible… I thought – how could I have gotten married? If I knew this before, maybe there wouldn’t be a marriage? How could I do this to my husband? He lost hi mom when he was 11… How can I put him through losing his wife too? Oh – the guilt…

Of course we wanted more opinions. My doctors at Westchester medical agreed… My reports were sent to John Hopkins – and they agreed… I then went to a pulmonologist at Columbia Presbyterian who agreed, but thought it was worth an appointment with the Child Cystic Fibrosis Director, Lynne Quittell. Although CF was her specialty, she still handled other pulmonary diseases.

This was the light at the end of that tunnel. I met with her. I handed her a fat binder of all my records. She asked us to wait outside while she went through my reports. When we walked back in to her office she said. You have Cystic Fibrosis – You do not need a transplant – You need to change your medications – start physical therapy.

So – when all the doctors are telling you transplant, and one says no. Who do you believe???? Oh – and remember the GI doctor… He basically said, in front of me to Dr Quittell (he was on the phone with her) “I’m not very optimistic about her prognosis”… IN FRONT OF ME!!!!!

My heart said to trust Dr Q. And my heart was right. 3 months later, 25-30 pounds heavier, improved lung function – from 30% to 48%, AMAZING. She saved my life!

Later, the genetic blood tests came back confirming that I did in fact carry 1 CF mutation. This was 1991/1992.

And, later in 1999 the 2nd mutation was found.



more on my journey to come… including…

  • the birth of my son
  • the scare of 1999
  • my daily life
  • my fears and thoughts

March 22, 2010

I have never blogged before. But I feel that I need a place to express what I’m going through while being anonymous. I can’t share everything I go through with my friends and family because I pride myself in staying positive. Should I show my insecurities it will only worry them and I cannot do that. So, today I start my blog to the word who doesn’t know me. Over time I will share my story. But right now I’m depressed and scared. I’ve had problems since October of 2008 and it’s been such a struggle. As I get better – it’s great – then I relapse. And I just relapsed again…..

Had a discussion with my doctor last night – we will be trying some more things, however she kind of tried to prepare me that I may have lost more lung function permanently and brought up to possibility of placing me back on the double lung transplant list as a safety backup.

This is VERY SCARY to me! I know that the statistics after transplant are not good for lungs and I just don’t want to go through that.. And it’s not just the possibility of transplant that scares me… If my breathing doesn’t improve, I can’t imagine dealing with this every day with no relief in site… I know I’ve made it 20 years without losing lung function. But I’m down to 40% now – and that sucks!