More on CLE and Future Pregnancies

Follow my blog with Bloglovin Back in February, I shared with you about our game plan for handling CLE in our current pregnancy.  Our third child is due in about 4 weeks, and our second child, Andrew, was born with CLE.

Please remember that CLE is not typically genetic.  Most of the time, it happens spontaneously.  I hope this brings you as much comfort as it did me. 

My Ob/Gyn referred me to a genetic counselor and we also had a level 2 ultrasound to check the health of baby #3.  I never updated you on the status of that meeting because we ended up nowhere. 

On the ultrasound, our third child's lungs looked perfectly healthy, but since CLE is often not able to be diagnosed in the womb, this wasn't very reassuring to us.

The meeting with the genetic counselor was even less reassuring.  CLE is rare, and our counselor wasn't as informed as we'd hoped she'd be.  She said that it's never genetic.  I knew better than that.  On our CLE parents Facebook page, there is a woman who needed a lobectomy for CLE as a baby, and her daughter required one as well, due to CLE.  Additionally, my pediatrician showed me one of his medical textbooks that stated that rarely, CLE is genetic, and that fathers and sons have been known to have CLE.

I left our meeting feeling discouraged at the lack of answers as well as the lack of information.  However, when we ended our discussion, the genetic counselor said that she would look into CLE further and call me back. 

Fifteen minutes later, my cell phone was ringing.   

The genetic counselor said that a pediatric geneticist wanted to meet with us, and she scheduled an appointment for our one year old at Arkansas Children's Hospital.

We had our appointment last week.

In the months that have lapsed, I have given up on anyone being able to completely reassure me that all future babies will be healthy and free of CLE.  Statistics, however, are on our side.  Additionally, my lungs are fine, as are my husbands.  Most likely, our son's CLE was completely sporadic.  So going into this appointment, I expected to gain very little information. 

The genetic counselor told me that at the very least, they would take blood from Andrew with the goal of creating a DNA microarray.  One day, should more information on CLE be available, they will have his DNA to study and compare.  Every year, exponentially more information is available in the area of DNA and genetics.  I decided to approach our appointment with the interests of science in mind, knowing that we would probably get few reassurances.

Here's what our pediatric geneticist had to say. 

There are three explanations for CLE:

1.  It's sporadic and simply happens.  This is, by far, the most common cause.

2.  Genetic:  A mother or father passes it on to their child.  This happens very rarely, and only a few cases of parents & children both having CLE exist.

3.  A Genetic Disorder causes it.  This was completely new information for me.  The doctor said that there are a few very rare genetic disorders that are connected with CLE.  The reason he wanted to see us was so that he could do a thorough examination of Andrew and see if my son has any of these disorders.  The doctor said "I wanted to see this little guy in person."  Since our appointment has been scheduled for months, I'm really glad that I didn't know he would be looking for a disorder, or I would've had one more thing to worry about.

The doctor was wonderful with Andrew.  He had an old fashioned leather doctor's bag that was full of toys perfect for my 17 month old.  My toddler, who was stripped down to his diaper, didn't want to be poked and prodded by the doctor, but the toys provided a great distraction, and the doctor was one that had been around kids enough to know how to poke and prod without eliciting too many screeches from a touchy toddler. 

The conclusion?   My son is too healthy and too perfect to have any of those genetic disorders. 
Any parent would love to hear this about their child, but one whose child had half a lung removed as a newborn really loves hearing such a positive statement about their child.

I wish I could tell you which genetic disorders he was looking for, but when he rattled off the name of one of them, I had no frame of reference to be able to remember it.  I have no medical background, so it's all Greek to me. 

The doctor said that the specific genetic disorders he was looking for have physical manifestations that would've been visible upon examination.  Blood work is routine at a genetics appointment, but there was no need to take Andrew's blood for a DNA microarray or any other purpose, because the doctor was completely certain that Andrew is fine. 

As for my future children and even grandchildren, the doctor said that I would have a better chance of winning the lottery than having another child born with CLE.  Our pediatrician wants to do an x-ray of our baby's lungs a few weeks after birth, for precautionary measures, but the genetic doctor says that it's really not necessary. 

Ultimately, I still have no reassurances that this isn't genetic, except that statistics are on our side.  You can trust statistics if you like; I feel like God has given me a peace on this issue.  Either our child's lungs are fine or they're not, and if they're not, He'll carry us through that journey, just like he did with Andrew.

If you want more information on the different causes of CLE, here's a website that my son's surgeon emailed to me.  It doesn't have the information about the genetic disorders.  I think those causes are so rare that there's little reason to be concerned about them.

Isabelle and Andrew enjoying the summer.

Maggie - Lobectomy at 8 months, Philadelphia, PA

by Beth, mom

October 1, 2009 - Birth day

I had a normal pregnancy, ending with a scheduled Cesarean Delivery due to my baby’s breach presentation. Maggie was born on Thursday, October 1, 2009, weighing in at 9 lbs, 3 oz – a big baby who looked amazing and healthy, until she turned blue.

Immediately, she was given blow-by oxygen, stabilized and transferred to the NICU in the local hospital where I delivered her. Three days later, when I went home, she came home. What followed for the next 9 months was nothing short of a parent’s worst nightmare.

I took Maggie to her pediatrician for her routine 5-day well-check. The pediatrician did not like the way Maggie was breathing. She had very strong retractions (pulling in of the chest & stomach) and very shallow breathing. The pediatrician's first action was to rule out Mitochondrial Disease, have x-rays completed, and blood work drawn. 

I drove Maggie over to the local hospital for the x-rays and blood work. I was told to wait at the hospital for the doctor to call me with the results. I waited & the blood work came back relatively normal, so I was sent home with Maggie to wait for the x-ray results. The doctor called me later that evening and said “it appears that her right lung has collapsed”. Well, of course, I flipped out! “But,” she continued, “the NICU doctor looked at her original x-rays and thought there was improvement, so we’ll keep an eye on that.”  In the meantime, 2 days later, I met with the local pediatric cardiologist. They did an EKG, ultrasound, and non-stress test. Her heart appeared to be fine.

A week later, I took her in to the pediatrician for her 2 week routine check-up.  Same practice, different doctor. She was alarmed by her rapid breathing and immediately left the room. She came back and said that she was going to have Maggie admitted to Fairfax Hospital for evaluation, that she did not like the way she was breathing and we need to get to the bottom of it.  

In the pouring rain, I drove to Fairfax Hospital (about 40 minutes away with no traffic, which never happens in Northern Virginia). Once there, I went to the admissions desk & was told that there was a room ready for my daughter on the pediatric floor. I was scared because I knew the flu & H1N1 were running rampant in our area. Maggie and I were met by two nurses who immediately hooked Maggie up to every piece of equipment possible and decided to run an “ng” tube into her nose! I had no clue what was going on & I was just crying & calling my husband frantically. All the while, our 2 older sons were at home with a neighbor. The next day, after a torturous night of sleep, the doctors ran a CT scan on Maggie. There did appear to be collapse in her right lung. From what we understood for the 5-day stay there, upon discharge, all of this should be outgrown in about 2 weeks or so. I left the hospital on a Tuesday with a prescription sheet for an x-ray and a follow-up doctor appointment in 2 weeks.

At home, things seemed normal for Maggie.  She slept a lot and didn’t have much of an appetite because she tired easily. But that Thursday, I noticed – there had been NO change in her & I was concerned. There must be another doctor I could call. So I called the pediatrician (who admitted Maggie to Fairfax Hospital) and asked her for another pulmonologist’s name. She said “If I am that concerned, bring her in.”  So I did. Again, I sent my older 2 sons to the neighbors (thank God for good neighbors!). 

The doctor didn’t like the way Maggie was semi-unresponsive and decided to put a pulse-ox monitor on her. It read 84%, which to me means “Oh crap!” To the doctor and nurse, it meant “Call 911.”  So, again, I was panicking and crying, unsure of what was going on. We were sent off to the local ER to be stabilized (again, rampant with the flu & H1N1, which probably could have killed this baby).  

Since we had already been to the closest “specialist” hospital, we needed to make a choice where to go next. The only place I could think of was Children’s National Medical Center (CNMC) in Washington, DC.

At 2 am, after switching places with my husband, Maggie was transferred to CNMC by ambulance. She immediately had a spinal tap which revealed nothing. She was hooked up again to the NG tube and oxygen, but this time was placed into the NICU’s care. She was the biggest baby they’d ever seen. She was there for the next 2 weeks, for her first Halloween and a few days following. There were tests and more tests and many sleepless nights. My husband was working full-time, so he would usually take the night shift, while I would come in to the city after rush hour & stay all day. We never left her side. 

November, 2009 - One Month Old

On November 5, she was released on ¼ liter of oxygen, nightly NG feeds to raise her calorie intake which would help her (and her lungs) grow, nebulizer medications (Pulmicort and Atrovent), and Prevacid since it was found that she had acid reflux. We were still no closer to a diagnosis and just had a routine to keep her status quo with frequent follow-up appointments.

On December 22, Maggie had a VQ Scan at CNMC, which showed “possible” congenital lobar emphysema. She did not fit the profile for the disease, but it was suggested.

February, 2010 - Four Months Old

On Saturday February 6, 2010, 36” of snow fell in our area. Maggie’s condition was declining. She was coughing a lot and just wasn’t her normal, happy self. My husband and I decided to give her one final nebulizer treatment before making the decision to call 911. Thankfully, our road had been well-plowed earlier that day so the ambulance and life support trucks arrived quickly. 

Maggie was taken to our local hospital & put into a room on the pediatric floor. On Monday, February 8, 2010, the decision was made to transfer Maggie to CNMC. However, our area was expecting another 15-20” of snow that evening. At 1 pm, the decision was made that she would be airlifted to CNMC. Unfortunately, I could not travel with her, so I waited for my husband to pick me up to take me to DC to be with her. As soon as we crossed into DC, I received a call from the Flight Nurse who said “Maggie’s condition worsened and we needed to intubate her.”  While I knew it was a possibility, I still didn’t expect it. 

We rushed to her side in the PICU pod (6 beds in a large room) where she was on a ventilator and had tubes going everywhere. It was the scariest time in my life.

She was found to have “Human Meta-pneumovirus" (hMPV, which is very similar to RSV). On Saturday February 13, 6 days after being intubated, I had a long talk with the doctor about Maggie. I thought this was “the end”. How could I keep my child on a ventilator? What quality of life was this? 

The doctor, probably wanting to slap me, talked to me directly “We are talking about one organ, we can handle this. She can handle this.” 

That being said, I had a big heart-to-heart with Maggie on the eve of her 1^st Valentine’s Day. That next day, after morning rounds, the doctors and Respiratory Therapists (RTs) started extubation readiness testing on Maggie. She failed the testing, but they decided to extubate anyway. I had to leave the room, I was so scared. My husband stayed. He said that she coughed and immediately her oxygen level was up and she was awake and was the most beautiful thing I had seen in my lifetime! She was released 1 week later, but again, we were no closer to a diagnosis.

March 2010 - 5 Months Old

In early March, Maggie contracted rhinovirus (a cold to you & me) and was again, hospitalized on the pulmonary care floor for 1 week. At this point, I was done. I started doing research. Where is the BEST hospital for our child? The answer; clearly was Children’s Hospital of Philadelphia (CHOP). On CHOP’s website, they list their doctors and their areas of specialty. I found one, Dr. Howard Panitch, who specialized in Infant Breathing Disorders. That was the one I was going to try & contact, so I wrote him a letter. He called me about 4 days later and told me that he had set aside a full-day for Maggie on Wednesday March 31, 2010.

My husband and I set out for Philadelphia on Tuesday March 30for the night. Our first appointment was at 9am for an infant breathing test where Maggie would be sedated and studied. Following that was a CT scan with contrast, cardiology work-up, and so on. By 1pm, all of the testing was complete. By 3pm, we had a DIAGNOSIS and a PLAN!!!! She did, in fact, have Congenital Lobar Emphysema, affecting the right middle & upper left lobes. We would meet with the Cardio-thoracic plastic surgeon in the morning and discuss our options.

The next morning with the pulmonologist and the surgeon, we decided that we would wait for surgery, unless Maggie was to get sick again, in which case we would schedule the surgery as soon as she was well-enough to do so.

A few days later, Dr. Panitch called and said that the emphysema was evident in the very first CT scans that Maggie had, 1 week after her birth. That comparison with the more recent CT scan showed that the emphysema was mainly in the right middle lobe, so that was what we would target should she need surgery.

In April, Maggie was diagnosed with Parainfluenza and was intubated again for 6 days.

She was released 1 week later from CNMC. During this stay, we discussed the situation with Dr. Panitch and surgery was scheduled for June 23, 2010 in Philadelphia. The right middle section of Maggie’s lung would be removed and her recovery was expected to be similar to her stays at CNMC: ventilator for about a week & then rest for a week before going home.

June 2010 - Lobectomy at Last!

My mom and I arrived in Philadelphia on Monday June 21 for Maggie’s pre-surgery testing. My husband arrived on Tuesday June 22 for pre-surgery doctor’s visits. We awoke (after a restless night) and headed to the hospital for the surgeon’s second surgery of the day at 10am. 

Pre-op took forever! Maggie didn’t get back to the OR area until about 12:30pm. We were then told that the surgery would take about 4 hours. At 1:30pm, the nurse came out and told us that they just took her into the OR at 1:15pm. So, we grabbed lunch and came back, waiting as patiently as we could for news. 

At 2:30 pm, I headed towards the restroom, but saw Maggie’s surgeon heading towards me. I was a little freaked out. She said to come back with her to the waiting room. I followed & she proceeded to tell us (me, my husband and both sets of grandparents) that Maggie was doing great, surgery was complete.  They extubated her immediately and she was breathing fine on her own and her pulse-ox was between 97-100%. We would be able to meet her in the NICU where she would stay for the rest of her stay in about 1 hour. The relief was pouring out of me! I was overjoyed!

Five days later, I took Maggie home and today, she is 2 1/3 years old & amazing. Except for a small incision scar under her right arm, she is no worse for wear.

Today, at 28 Months Old

Maggie was able to discontinue all of her reflux medications in July 2010. Her nebulizer medications were stopped in November 2011. She runs, jumps, swims, and tries to catch up with her big brothers who just adore her. We are blessed every day to have met all of the people we did, that we learned what we learned about “infant breathing disorders,” and how you need to be your child’s advocate and not accept anything but the best care, and most importantly, how our daughter beat the odds!

Maggie at 2, precious and perfect.

CLE and Future Pregnancies

I'm moving into my second trimester of pregnancy with baby #3, and baby #2, Andrew, is our little CLE baby.  Like many families, we've never had a birth defect in our family, so this is unchartered territory for us.

I had a checkup yesterday and talked to my OB/Gyn yesterday about how we need to handle the pregnancy.  Here are the factors that affect future pregnancies: 

• Congenital Lobar Emphysema typically happens spontaneously.  However, there are some cases that are inherited genetically, with even fathers and sons having it as well as mothers and daughters. 
• When CLE is genetic, it is through autosomal dominant inheritance, which means that if you're a carrier, your children have a 50/50 chance of inheriting it. 
• CLE is more common in males, so that can skew the above numbers a bit.
• CLE can be detected and monitored throughout pregnancy by using ultrasounds, but it is not always detectable in the womb.
• There are very clear benefits to knowing your child has CLE ahead of time.  I would want to deliver at a hospital where the doctors are somewhat familiar with CLE and near a children's hospital, so that my child can be transported there quickly if he or she needs an emergency lobectomy.  I live 2 hours away from one children's hospital and 3 hours away from the one where Andrew had his lobectomy.  The hospitals in my smallish town are nice, but they're in no way equipped to deal with such a serious situation, which is why Andrew and I were flown to Dallas, TX for his lobectomy.

My OB/Gyn for this pregnancy is the same one who delivered Andrew and he's also a family friend, so he's a little familiar with the situation because of that.  But like most folks, he's really not incredibly familiar with CLE.  He's going to research and do his homework on CLE and come up with a plan, but at this point, he believes the best thing to do is to refer us to a genetic counselor. 

I'm completely unfamiliar with genetic counseling, but from what my doctor explained, the counselor will talk with us about the genetic side of CLE.  If needed, we'll get testing to find out if my husband and I are carriers of CLE.  If one of us is a carrier, then we'll have to figure things out from there.  The plan will likely include a Level 2 Ultrasound to check for the birth defect. 

Have any of you met with a genetic counselor regarding CLE or any other birth defect?  I'm really curious about the process.  How did your doctors handle future pregnancies after having a baby with a birth defect?  I do think this is one of many situations in which it's nice to have an OB/Gyn who you can trust to make the best decisions for your family.

Kasen - Lobectomy at 5 months in Edmonton, Canada

by Jessica, mom

March 14th 2011- Birthday

The moment you finally get to meet the baby who has been growing inside of you for 9 months is so magical. It has to be one of the best feelings in the world, when after so much anticipation you get to hold your baby in your arms. I don't think you truly know how much you can love another human being until you hold your baby for the first time, and from that moment you know that there is not a single thing you wouldn't do for that child. So far I have been lucky enough to get to experience this amazing feeling twice.  One of those days was on March 14th 2011, when I met my second born, Kasen.

"It's a boy!" yelled out the nurse, but it wasn't a surprise, since 19 weeks ago we had found out it was a boy on our 20 week ultrasound. But it was nice to finally know for sure. They placed him on my chest almost immediately and he let out a tiny cry. The doctor joked around, saying what a big boy he was and he must be at least 9lbs, so they took him to be weighed and cleaned off. Kasen was 8 lbs 4oz, exactly 2lbs more than his big sister did when she was born. He seemed to be a perfectly healthy baby.

June 20th 2011- A Trip to the hospital

Every once and a while Kasen had a slight wheeze while breathing but I never thought anything of it 'til the first time he caught a cold. This cold seemed to have hit him hard, causing him to have trouble breathing every once and a while. He was so congested that every time he took a breath it sounded like a cat was purring. I joked with him that he was my little kitty. Even though he had this terrible cold he was still his normal happy self.

On June 20^th 2011, his cold caused him to stop breathing a few times, so I decided to take him to the hospital. After being seen by a nurse, we were sent into a room they call “Fast Track”. It’s a room where they get people in and out fairly quickly because it’s nothing too serious. This made me very relieved. After hours of waiting in this so called “Fast Track” room we finally got to see a doctor. He suggested we try Kasen on an inhaler and keep him for an hour and see how he does and also for him to get an x-ray just to be safe.

Kasen was given an inhaler and it seemed to have helped him breathe normally. Then we went for the x-ray, which was painful to watch. If you have ever seen a baby get an x-ray you will know why it was so hard for me to watch. The sit them on what looks like a bicycle seat, raise their arms over their heads, and put a tube around them and strap them in so they can’t move. Then everyone has to leave the room and watch through a glass window. The x-ray took about 2 minutes but it seemed like he was stuck in there forever. After the x-ray we headed back to the room to see the doctor and find out what he had to say. He said that he was not sure about the x-ray so he was going to have it looked over by another doctor who would know better.

Later on that night the ER doctor told us that he thought Kasen had Congenital Lobar Emphysema and would need a CT scan to be sure. I was devastated that my poor baby would need to get an IV and be sedated in order for them to do the CT scan. I was such a mess that a nurse had to take Kasen for a few minutes so I could pull myself together. He and I cried as they tried to get an IV in his arm, but he was so small it was hard for them to find a spot to put it. Finally they got it in and now we would wait for the call to go down to get the CT scan.

When we got to the CT scan they put the medicine through his IV to put him to sleep but he fought it. He didn’t want to go to sleep; he whined and tried his hardest to fight it. They ended up having to give him more to get him to sleep. We were told in about 30 minutes he would wake up and someone would look over his CT scan and come let us know.

Kasen fast asleep after his CT Scan.

An hour went by and Kasen was still fast asleep.  I just wanted him to wake up so I would know he was ok. Then a lady came into the hospital with what I think was a broken leg and she was hysterical and screaming at the top of her lungs. It startled Kasen and he finally woke up.  Never in my life had I been so thankful that my baby woke up! But they had him hooked up to so many wires that I couldn’t figure out how to pick him up. So I unhooked him from some of the monitors so I could pick him up.

The ER doctor came back and told us that yes Kasen did have Congenital Lobar Emphysema and we would need to go to the children’s hospital 4 hours away as soon as possible to see a specialist. Since Kasen was stable, we were able to go home for the night and were told to be back at the hospital at 10am to meet with the pediatrician.

We left the hospital only knowing one thing and that was that our son had something called Congenital Lobar Emphysema. We had no idea what it was or what it meant so I went online to do some research. This only led me to be even more confused by this diagnosis. How did my 3 month old get a disease that smokers get? How did this happen and what was going to happen to my baby? I had so many questions that I just could not find online. That night I didn’t sleep more than 5 minutes at a time. I would wake up crying and shaking from a terrible nightmare of losing Kasen. That day was one of the worst days of my life.

June 21^st 2011- Some relief and answers

After a rough night with no sleep we were all up and ready to meet with the pediatrician. We met with Dr. Odaibo at the hospital for him to see Kasen and look over his x-ray and CT scan. Dr. Odaibo was great with Kasen while he was looking him over and even better with my daughter Keira who was bugging him. He explained to us that Congenital Lobar Emphysema was something Kasen was born and was very rare. To explain it in a simple way Kasen gets air into his lungs but air gets trapped and not all of it can get back out. This causes his lung to expand and push on his heart and other lung.

We were able to look at the x-ray and CT scan and see what they were talking about. His upper left lobe of his lung was much bigger than the rest. You could see on the CT scan that his left upper lobe was so big it was on the right side of his body as well. Dr. Odaibo answered all the questions we had to ask and then referred us to Dr. MacLean in Edmonton. Because Kasen was in good condition we didn’t need to rush to Edmonton that moment. We were very relieved and went home and waited for the call from Dr. MacLean to find out when we would be meeting her.

June 28^th 2011 – Our first trip to Stollery Children’s Hospital

We made the 4 hour drive to Edmonton so we could meet with a specialist to get more answers and see what the next steps would be. We had an appointment at Stollery Children’s Hospital to meet with Dr. MacLean. She was a very nice doctor and explained to us more about what Congenital Lobar Emphysema was and different ways to treat it. One of the options was to have surgery to remove the part of the lung that was affected. The other option was to leave it and try and control it. Both options had risks and we really had no idea what we would do.

I really didn’t want my baby to have to have surgery, so I thought it was probably best to keep an eye on Kasen and try and keep it under control. That was until we met with the surgeon, Dr. Dicken. Again he gave us more information and let us ask him all the questions we had. If we didn’t do the surgery Kasen could live a life of having a hard time breathing and there would be a chance that his lung would grow so big it would completely block off his airway and cause him to stop breathing. And if that ever happened we were 4 hours away from the surgeon who was able to do the surgery to save his life.

How could I put his life at risk like that? I would worry every day that something terrible would happen and Kasen would stop breathing. So I agreed to do the surgery even though Kasen seemed to be fine at that moment. The surgery was scheduled for August 29^th 2011. We signed some papers and then started discussing any questions we had about surgery. Dr. Dicken had performed this surgery before and had great outcomes every time.

August 29^th 2001- Surgery

 Waiting for surgery.

Every single day I worried that I was making the wrong decision, and even more now that in just a few hours my baby would be having major surgery to remove his upper left lobe of his lung. I was as calm as I could be because I didn’t want to worry Kasen. I had to be strong for him. We met with the nurses, anesthesiologist and the surgeon one last time before Kasen would go for surgery. I was strong and kept it all together until the nurse came to take Kasen away. I couldn’t keep it in any longer I was so scared for Kasen. I was scared that I made the wrong decision. I was scared something horrible would happen during surgery and I would never get to see my baby again. I didn’t want to let him go but I had to. I gave him one last hug and kiss and told him how much I loved him and handed him to the nurse.

The surgery would take about 3 hours and he would be in recovery for about an hour then he would be brought upstairs where I would be waiting for him. Dr.Dicken told me he would come and let me know how surgery went as soon as he was done. It was the longest 3 hours of my life, waiting to hear if everything went ok. Then 3 hours turned into 4 and 4 hours turned into 5 hours. I was getting worried that something went wrong when finally, Dr. Dicken came in to let me know that the surgery went great and that Kasen was in recovery. He had been called to do another surgery after Kasen's surgery and was unable to let me know right away how things went.

We waited another half an hour for Kasen to come out of recovery. We were later told he had a hard time waking up and then was throwing up a bit so they kept him a bit longer to keep an eye on him. They wheeled him down the hall and I could see them coming so I ran up to them to see my baby. I was so happy he was ok. He looked so peaceful lying in the bed, much better than I expected him to look after just having major surgery. He had so many tubes and cords coming out of him. He had a ton of monitors hooked up to him so they could keep an eye on him.  He'd had an epidural to manage the pain, an IV in his foot, a catheter, and a chest tube to drain fluid.

After surgery and time in recovery room

With all the tubes and cords it made it very hard to pick him up so I needed a nurse to help manage all the cords so I could pick him up to nurse him. We were in what they called the ICE room. This is a room with 4 beds and 2 nurses so they could keep a close eye on patients. We had very little room, just a crib a little cot and a rocking chair all squeezed in the corner. That night, I sat in the rocking chair all night with Kasen in my arms because he just wanted to nurse and be held. Once he was asleep, it was too hard to move him because of all the stuff he was attached too. I was happy to sit and hold him all night if that is what it took to make him comfortable. But one of the nurses thought Kasen should sleep in the crib and every time he finally fell asleep she would move him to the crib. Of course, that woke him up and made him scream and scream until someone could help me pick him back up and sit in the rocking chair.

August 30^th – September 1^st- Recovery

The next morning, we were able to be moved to a room because Kasen was doing great. He was still in a lot of pain because of his chest tube. They managed his pain with his epidural, morphine, Advil, Tylenol, Gravol and occasionally some Benadryl because the morphine made him itchy. Once we were in the new room, I asked if we could have a bed instead of a crib because Kasen was far more comfortable if I was holding him.

I spent almost every minute in the bed with Kasen. I only put him down maybe 2 times a day so I could get up and go to the bathroom and stretch my legs. Other than that I held him in my arms the entire time. It was the only way he was calm and the only way he would sleep. Later on that night, I was moving him so he could nurse on the other side and I accidentally ripped his epidural line. So they took that out and just put his dose of morphine up to manage his pain. It was so hard to keep all his lines straight and keep him from pullling on them. Whenever he was awake he would play with the cords on his chest to monitor him. He would pull them off and the nurse would run in thinking something was wrong.

Kasen and Mommy in the hospital bed watching some TV.

The next day, August 31^st Kasen had an x-ray to check out the fluid in his chest to see if he could have his chest tube removed. Thankfully, he was able to lay in bed and have the x-ray instead of being strapped into that thing again. The x-ray showed that Kasen was able to get his chest tube removed and would have it taken out later on that day. That was great news because the surgeon had told us that the chest tube causes a lot of pain.  Every little movement causes the chest tube to move around inside of Kasen's chest, causing him a lot of pain. He also told us that once the chest tube was taken out we would only need to spend another day at the hospital.

Once the chest tube was taken out, I was able to pick up Kasen and take him out of the bed. I could stand and rock him when he got upset. It was nice for both of us to be able to get out of the bed once and a while. They even started to lower his morphine to see how he managed the pain without it. Every few hours, they would turn it down and wean him off of it. He seemed to be getting back to his normal self more and more every time they lowered it. It was nice for him to be awake more and even smile and laugh.

No more chest tube means we can get up and walk around a bit.

I am much happier without that chest tube.

September 1^st Kasen had another x-ray to make sure everything still looked good. But this time we needed to go downstairs and get the x-ray for a better picture. I sent Kasen into the room with his dad because I didn’t want to see him like that again. A few minutes later, they came out and told me how well Kasen did in there and we all went back upstairs. A few hours later we got the results from the x-ray and found out that Kasen would get to go home today. I was so happy that Kasen was well enough to go home after only 3 days in the hospital. He was such a strong boy for going through all he had to at only 5 months.

Once we were home from the hospital, Kasen was on just Advil and Tylenol for about a week and it managed his pain just fine. We had to be very careful around his incision spot and keep the tape on and not get it wet for 10 days. After the 10 days he was almost 100% back to his normal self. A month after surgery we went back and saw Dr. Dicken for a follow up appointment. The plan was to have another x-ray but because Kasen was doing so great no x-ray was needed.

 Kasen at home just days after major surgery.

January 25th 2012- Today

Today Kasen is a happy and healthy 10 months old. He still has a small scar from his surgery but other than that, you would never be able to tell that he had major surgery less than 5 months ago. He doesn’t wheeze anymore and he doesn’t have any trouble breathing. He gets colds and we don’t have to worry about him not being able to breathe. He crawls and can stand all by himself and is almost always smiling or laughing. He will grow up to live a normal and healthy life, thanks to everyone who took such great care of him at Stollery Children’s Hospital. I am forever grateful to them.

 Kasen, 10 months old.

Scheduled Surgery Versus Emergency Surgery

Want to hear about a scheduled lobectomy?  It's quite different than the story we told last week about Andrew's emergency lobectomy.  When CLE is caught prior to sending the child into severe respiratory distress, the family can schedule surgery for a time in the future, when the child is healthy and doing well.  The result?  Kasen spent 4 days in the hospital instead of nearly two weeks.   The infant was also able to nurse almost immediately after surgery, rather than having to be put on a feeding tube.  You can read all about Kasen's story tomorrow. 

Andrew - Lobectomy at nearly 6 weeks old in Dallas, TX.

by Gabrielle, mom
Most of this text was originally posted on my personal blog, Mamagab.

When It's Not Just a Cold

March of 2011
One Saturday night, my husband and I both woke up hearing our 5 week old baby boy sneezing and coughing. We both groaned. He had picked up a cold from our 2 year old, Isabelle! But babies get colds all the time. I reminded Damian that Isabelle was this age when she got her first cold, so we already knew what remedies to try.

The following Wednesday evening, while we were eating at the Olive Garden, Andrew was steadily coughing, but at the end of each series of coughs, he would breathe in really strangely. I'd never heard anything like that. I still wasn't too concerned because it was just a cold, and I knew how to handle a cold: steam him up in the shower, run a cool mist humidifier, use saline solution in his nose, suck out his nose frequently, and elevate the head of his bassinet to help with drainage. I'd been doing these things for several days, so I should see an improvement soon.

But my husband was incredibly concerned and insisted we take the baby to the ER. And I had to admit, his cough was pretty funky. I could also see some indrawing at his ribcage. That was definitely not a good sign. After dinner, we passed by the pediatric after hours clinic, but they had stopped seeing patients 15 minutes before our arrival. So around 9 pm that Wednesday night, I brought Andrew to the ER.

The nurses who initially checked him out were not concerned at all, and they asked if we'd spoken to our pediatrician's on-call doctor. No, I hadn't thought to do that. Nonetheless, we were sent back to a room, and eventually X-rays were ordered, just in case he had pneumonia.

Around midnight, the ER doc walked in with the X-ray results. He said Andrew's lungs looked abnormal and he wanted to get a CT scan to be sure. He thought he saw cysts, but it was hard to tell. It appeared that Andrew had something congenitally wrong with his lungs. My heart sank at his words. Those were life-changing words, and I felt quite a bit of fear. I was trying to remain calm, but evidently I don't have a poker face, because the doctor repeatedly told me not to freak out. The doctor told me it could be something minor, but we'd need to get a CT scan to see.

I called Damian, who was home with Isabelle, and I also texted a couple of friends to pray (it was midnight, so I notified few people). One friend, Melissa, offered to stay at our house so that Damian could join me at the hospital. We went back for the CT scan and awaited the results. While we waited, Andrew had a couple of really scary coughing fits where he seemed completely unable to breathe at times. The nurses put him on a bit of oxygen. We were also informed that Andrew had tested postive for RSV, so it was definitely more than your typical cold, but it was the lung defect that was really causing the breathing problems. Eventually, around 2:30 in the morning, the ER doctor told us the results.

He informed us that Andrew has Congenital Lobar Emphysema.  His lungs could take air in, but they could not remove it easily. The top lobe on his left lung was ballooning up with air and was so large that his trachea and heart were displaced into the right portion of his chest. Treatment was available, but not at our hospital. We would be flown to a children's hospital in either Little Rock, AR or Dallas, TX. I would fly with the baby and Damian would go in the car so we'd have a way home. Oddly enough, I found out later that he made the diagnosis using WebMD. Seriously. It's such a rare condition that many doctors have not heard of it.

We chose to go to Dallas for treatment, since my oldest sister lives there, and I then left to pack a bag for myself and one for Isabelle. We had no idea what treatment options were available or how long we'd be gone, so I was trying to prepare for everything. I was quite scared, knowing my son had a lung defect, but I honestly had no idea just how dire the situation was. Since we first walked into the hospital, we never felt like anything about our case was urgent. 

I made arrangements for Isabelle to stay with Melissa in the morning, but they were leaving town on Friday. I never really worried about where she would stay, because I knew friends would offer to keep her. I got in the car and headed back to the hospital, where Damian and Andrew were waiting for the plane and EMTs to arrive from Dallas.

Flight to Dallas 
When I arrived at the hospital, we were expecting the plane and EMTs from Dallas to arrive within 45 minutes. I stayed with Andrew while Damian went home to pack and try to nap before making the drive to Dallas.

When the EMTs arrived, I finally felt the urgency that the situation warranted. The team consisted of three paramedics, with one registered nurse (RN), one respiratory therapist (RT), and one who was simply a paramedic. They walked in and began assessing him immediately. The RT said Andrew was not moving air at all in his lungs, and also observed that the oxygen given by the nurse was not doing a thing, since his canula (oxygen tube) was in upside down. They immediately deemed him unfit for air travel and began trying to improve his breathing so that we could make the flight.

I could tell my baby boy was in a very precarious situation. It's frightening to see your tiny baby propped up on a table with a medical team surrounding him, but I could also tell that they were well-equipped to care for him, which was comforting. His heartbeat was in the 220s and they worked to bring it down. The EMTs did a breathing treatment, a deep suction of his nasal cavities, and another breathing treatment to help stabilize his breathing.

He was still breathing poorly and needed to be intubated for the flight, but there was quite a bit of discussion on whether they could and should intubate him. The RN from Dallas had been in constant communication with the doctors at Children's Medical Center in Dallas, and after much discussion, the decision was finally made that while he needed intubation, it was a risk they couldn't take. His lung anatomy was so strange that it could cause more problems and perhaps even make the situation worse. Instead, they used a CPAP type of device to provide oxygen for him in flight. After about two hours of prepping him for air travel, we were ready to go around 6:30 am.

We took an ambulance to the Texarkana Airport, where we connected with a private jet belonging to Children's Medical Center. I was told beforehand that the flight would take a little longer than usual. The EMTs asked the pilot to fly at a lower altitude. Air expands as altitude increases, and if the air in Andrew's lungs expanded too much, it would make a dangerous situation worse. Instead, we flew at a lower altitude and took a little longer to get to Dallas. The ride was also bumpier because of the lower altitude, but it would be worth it. I had the feeling that if my baby could just make it through the flight, he would probably be OK. I called Damian just prior to boarding to tell him what a precarious situation Andrew was in, and to pray for Andrew's stability during the flight.

I boarded the flight first and headed to the back of the plane, which would probably seat about 10 people, if it were full of seats. Andrew's gurney was in front of me, and the EMTs sat in seats on the left side of the plane. During the flight, I mostly prayed and tried to find ways of peeping around the equipment to see my son's tiny face, just to make sure he was still OK. The EMTs were well-prepared to jump in should his breathing change, but our prayers were answered and he stayed in the same condition throughout the flight.

Surgery

The plane touched down at Love Field in Dallas, and then we took another ambulance to Children's Medical Center. We arrived at the hospital and quickly made our way to ICU, where a spacious room had already been prepared for him. His medical team was awaiting our arrival, and fortunately the RN EMT had briefed me ahead of time that he would be surrounded by doctors and nurses at once, so I wasn't too overwhelmed. Still, it was quite surreal to step back in the ICU room and see tiny Andrew laying in his bed, surrounded by nurses and doctors being briefed on his condition by the EMTs. They were moving fast. X-rays were taken by a portable machine and pictures of his lungs were instantly displayed on equipment in his room so that everyone would know what they were dealing with.

I was asked to sit, and a surgeon, a fellow, and then an anesthesiologist took turns discussing Andrew's surgery with me and asking me to sign papers to grant my permission for surgery. They would go in between his ribs, doing a thoracotomy, and then a lobectomy would be performed on his left lung. The left lung has two lobes or halves, while the right lung has three lobes. His top left lobe was the one with CLE, so it would be removed completely. Then a chest tube would be inserted. The remaining bottom lung would leak air and fluids until it healed, so the chest tube would drain all of this air and fluid.

When the surgeon described all of it, I was shocked. The entire top half of his left lung? Removed? All of it?!? But it was the only option for his survival.  The next day, I asked one doctor about Andrew's situation when we arrived.  He said that when we arrived at Children's hospital, Andrew only had hours or minutes to live, and he couldn't say for sure which one it was.  The doctor reiterated that he really doesn't like to exaggerate things or act like he saved a life when he didn't, but Andrew's was a dire situation.   

When I met with the surgeon to discuss surgery, her primary concern was that surgery would go smoothly and his bottom left lung would not be compromised in anyway, so that it would grow back well. Yep, that's correct. It will grow back. I was floored at this knowledge. I had no idea that lungs could do such things. In fact, if you google "lung grows back" or "lung tissue regerates," most information will say that it does not. Because it does not. For adults. Children are entirely different, and will regenerate lung tissue and alvioli until about the age of 8 (doctors disagree on the exact age). I asked the surgeon if my son would have a "normal" life (whatever normal looks like). She said absolutely. He would have two healthy lungs and complete use of them, without breathing issues. She said "I'm not saying he's going to be an olympic runner, but I wouldn't rule it out either." Her words were a comfort to this mama's heart.

After my permission was given and the medical team had finished assessing him, they were ready to do surgery. I put my hands on my baby boy, prayed aloud for him, kissed his little forehead, and they whisked him away for surgery.

Once he left, I couldn't help but lose it a little in the hallway, which made some of the nurses and hospital employees nervous, and some went on a quest for a counselor or chaplain.  I had to wait by myself during surgery, but it wasn't as hard as I would've thought. I wasn't alone; I had the Lord. Friends and family also called constantly, which was another way God provided comfort. Andrew was on so many prayer lists during the entire ordeal, which made all the difference.

Surgery would take a few hours, during which I prayed, talked with various friends and family, and pumped. Seriously. Protecting my milk supply became a high priority for me, because it seemed like the only thing I could possibly do for my child at that point. I put myself on a pumping schedule and stuck with it until Andrew was able to nurse again.

Every hour, a nurse would call me and give me an update on Andrew's surgery. Each time, all was well. At this point, I was running on pure adrenaline. It was Thursday, and I hadn't slept since Tuesday night, when I'd only gotten about 3 hours of sleep due to caring for sick kids. Every once in a while, the magnitude of the situation would hit me. I remember the anesthesiologist telling me that the huge hospital I was in had essentially shut down to accommodate for my baby's surgery. And like on the plane, I had the feeling that if we could just get through this, the next few hours of surgery, then everything would be OK. It was frightening at times, but overall, I felt peace and comfort.

I also kept thinking that our baby doesn't have cancer or even something like cerebral palsay or a heart defect. While this was a scary and dangeous situation, the Lord had given us a medical crisis that was totally fixable. How amazing! I feel like David: "Who am I, Soveriegn Lord, and what is my family, that you have brought me this far?" (2 Samuel 7:18)

Once surgery was over, I met with the surgeon. She said everything went perfectly, and no surprises came up at all. She described recovery and reiterated again that his left lung would grow back completely. I still can't believe that God made our bodies to do this. We truly are wonderfully made.

After I met with the surgeon, Andrew was in intensive recovery without visitors, so I was instructed to eat lunch, my first meal since Wednesday evening, and this was Thursday afternoon.  My husband finally arrived from Texarkana, along with our associate pastor and his son.  It was such a relief to no longer be alone at the hospital and to have my husband there with me. 

Recovery

Andrew had lung surgery on March 17, but stayed in the hospital until March 29. Recovery was, as the surgeon described, like watching paint dry. Sometimes we saw progress, but often, he appeared the same from day to day.

He was in ICU for several days, but fortunately, his ICU room had a small couch where Damian and I could sleep, so we were still able to be in there with him. Meals had to be eaten in the waiting room or in the cafeteria, and there was no restroom or shower in our room either, but we had access to one down the hall.  Because he had RSV, he was also in isolation.  Anyone who entered his room had to wear a paper gown, latex gloves, and a paper mask.  Upon exiting, even to go to the restroom or greet a guest, we had to discard the old gown.

While in ICU, he had excellent care around the clock. Each nurse was only assigned two children to care for, and their rooms were side-by-side, so we saw the nurse constantly. He had an IV and then later a feeding tube (with my expressed milk in it) and with all the gadgets attached to him, there was very little Damian and I could do for him. We could comfort him if he was upset or just talk, sing, and pray over him. We chose a life verse for him at this point, which we prayed over him, along with other verses.

Sometimes it was very hard to see any progress. We were doing pretty well, emotionally, until the Monday after his surgery. We had seen no progress since Saturday, when he was alert and "talking." On Sunday, he just slept, and by Monday, it seemed to us that he had regressed. We had also had a rough night, with the nurses having a hard time controlling his pain, and he needed frequent suctioning of his nose, so we woke up several times to his screams. Each time I woke up, I would just pray for Andrew until I fell back asleep again. The respiratory therapist had also attempted to reduce his oxygen on Sunday, but had to turn it back up again because he wasn't handling it well. The nurse said that it sounded like his breathing was diminished on his right side, the side that hadn't been operated on, but the doctor disagreed and said he sounded fine. Still, we couldn't help but be concerned, and the x-rays were inconclusive on his progress. The doctor said that since the x-rays were inconclusive, he would just go by Andrew's clinical appearance, which seemed fine. But that Monday was especially difficult, because it seemed like he wasn't progressing and to us, it felt like he would be there in ICU forever.

You can see his large incision under his arm on the left side of the picture.  It continues following the line of his shoulder blade in the back.  You can also see his chest tube, which is taped in place and tucked under the patch of gauze on the same side as his incision.

I prayed a lot and tried not to listen to the negative thoughts that were nagging me. Sometimes I successfully took those thoughts captive by focusing on what was true & right (Philippians 4:8), but other times, I gave into thoughts that I might lose my baby. It was a real battle. An e-mail was sent out to the church for prayer, and it was amazing how much that encouraged me, as well as small things like encouraging Facebook messages and texts from friends, especially scripture. Still, it was the toughest day we'd had yet. But Tuesday morning, I woke up to the good news that Andrew was being moved out of ICU. His progress was slow, but it was progress, and he was stable enough to be able to go to another floor.

We were moved to the wrong floor initially, and while we were waiting to be moved, the surgeon came by to check Andrew out. She told me then that we'd try giving him a bottle on Wednesday. I asked a question about how to burp him, and she looked at me with a shocked look on her face and asked if I'd been allowed to hold him. No, I said, I sure hadn't. She looked at the nurses in the room and said "Please, let this mom hold her baby. She has got to hold him." While we waited to move to the surgical floor, they got me settled into a rocker and configured the wires and cords so that I could hold Andrew. It felt so good to hold my baby boy for the first time in five days, and I was so happy, I cried. When I laid him down again in his bed so he could be transported, he gave me a sweet smile, his first smile since the ordeal began. He loved the physical contact just as much as I did.

The primary delay in Andrew's recovery was the RSV.  His lower left lobe needed to recover from surgery and seal up, essentially.  But the coughing from RSV would blast air through that recovering lung, hindering the healing process. 

Starting Wednesday, he could be fed, so we got to hold him several times a day for feedings, first with a bottle, and then by nursing him. He started smiling all the time and was a very happy little boy.

Andrew, 7 weeks old, on day 8 of our hospital stay.  I love his big smile in this picture, and it's actually the first picture we have of him smiling.

Things progressed very quickly after that. His pain medications were reduced, and the main thing we were waiting on was the removal of his chest tube. At 2:30 a.m. on Sunday morning, the nurse picked Andrew up to give him to me for a feeding, and spotted his chest tube still laying in his bed! She and I both panicked a little bit. It was quite frightening at the time. If air started accumulating in his chest cavity, then he would need another tube put in. They watched his vital signs closely and did repeated x-rays to watch his pneumothorax. Fortunately, it did not increase, so he didn't need a new chest tube. We were released from the hospital on Tuesday morning.  The only medication Andrew needed at that point was Tylenol for pain management. 

I can't pretend that it was easy to come home. It was a little scary, in fact, since we're completely in charge of Andrew's care now and we didn't have immediate access to nurses and doctors. Emotionally, it was also difficult because of the emotions that come from being, as the doctor described, "hours or minutes" away from losing your child.

Andrew had a follow up appointment at 2 weeks after discharge, 2 months later, and then 6 months later.  At each checkup, his breathing seemed excellent and his x-rays appeared as normal as possible for a little guy with CLE. 

Andrew at 3 months, about 6 weeks after being released from the hospital.

We have been officially released from our surgeons care and see no doctors, other than his regular pediatrician.  He will turn 1 in a couple of weeks and developmentally, he has no delays whatsoever.  Other than a couple of bouts with bronchiolitis this winter, he is every bit the normal, happy, healthy baby boy. 

Andrew with big sister Isabelle.

 

Blog Launch!

I'm preparing to launch the blog soon, and I want to start by sharing my son's story.  If you're interested in sharing your child's story with us, please comment and let me know!  I would love to have a lot of stories prepared to post as soon as we're ready to have the blog go public.