Trapped in a Glass Case of Emotion

Apologies for the delayed entry since delivery. Things have been very hectic in our world and I'm just now getting the opportunity to catch up five months later.

Delivery went well and Presley Leighton was born on 3/28/13 completely asymptomatic. She had a chest x-ray done within the first 24 hours and the CCAM did show up on the x-ray. Two months later, we had another chest x-ray done and the x-ray did not show the CCAM. While the physicians assistant was optimistic that it might have resolved itself, I learned on my CCAM mommy boards that this is fairly common. We scheduled a chest CT scan last month which occurred right before she turned five months of age. A follow up phone call with the PA showed that the radiologist DID see the CCAM on the CT and that it was actually in the center lobe and not the bottom lobe of her right lung as we had originally thought. We did not find out any further information from the PA at this time. We simply set up a follow up appointment with the actual surgeon to go over the radiologist's findings and discuss next steps.

Presley had her appointment with the pediatric surgeon this morning (9/12/13) and I left feeling very confused and conflicted. First of all, the mass is still present and is 3cm x 3cm which means that it comprises about half to three fourths of the center lobe of her right lung. However, the radiologist was unable to actually identify the cysts and instead said that it could possibly be a trapped pocket of air thus indicating that there is a possibility that it might actually be Congenital lobar emphysema instead. Congenital Lobar Emphysema is a respiratory disease that occurs in infants when air enters the lungs but cannot leave easily. The lungs become over-inflated, causing respiratory function to decrease and air to leak out into the space around the lungs. Most of the severe cases of Lobar Emphysema can be identified at birth as the lobe continues to inflate and inflate and inflate. We would’ve noticed it already if that were the case. However, it could be deflating but slower than normal. She does not believe this to be the case though. She still believes that the mass is actually a CCAM. Either way, they would be treated the same way (with surgery) and would probably only be able to rule it out once it had been removed. According to the surgeon, the location of her CCAM makes it a little more difficult. The center lobe is the trickiest to operate on so she would prefer to wait six months and repeat the CT scan again. If the CCAM is still present, we will operate then.

An outsider might view this meeting as good news. No parent ever WANTS there child to require surgery. Why opt to have the surgery now when she hasn’t displayed any symptoms in the first five months of life? Well, according to our surgeon, most chest infections occur between 12 months to 18 months so it’s not quite as likely that she will demonstrate symptoms during this first year. They might show up a little later and by that point, she will have damaged tissue from the infections. From my perspective, I'm terrified that every single cold that Kendall brings home is going to turn into a chest infection and then pneumonia. To me, I keep wondering why wait if you’re just going to take it out in six months anyway? The period of time that we will be waiting will actually occur during cold/flu season which in turn make her more susceptible to chest infections. By the time she has surgery in six months, will she have built up scar tissue that will make the surgery even more complicated? Additionally, how much more difficult will recovery be for Presley be in six months? Right now, she isn’t mobile. In six months, she will be crawling and possibly even walking. Will it be more difficult to keep her still while she is recovering post surgery?

I, myself, explained to our surgeon how conflicted I was. I told her about all of the stories that I’ve read about on my Mums of CCAM Babies and Parents of CCAM babies boards. It’s amazing how different surgeons have such varying opinions on how to handle these cases. Some (mostly abroad) are very conservative and choose to never operate on the CCAM. Others, such as the surgeons at CHOP (Children’s Hospital of Philadelphia, which is the top hospital in the world for dealing with CCAMs) recommend removal of the CCAM as early as possible. One of my friends even had her son’s removed at 5 weeks old. Others have had theirs removed within a few days of birth depending on complications. This is a very heated topic on our boards and of course, every parent wants to believe that they have made the best choice. Everyone is an expert because their doctor knows best, isn’t that how it typically works. ;) The only thing we can do is trust the information we are provided, make a decision and never look back and wonder “what if”. After bringing the varying opinions up to my surgeon, she clarified that she is NOT one of those doctors who recommend NEVER removing the CCAM. She believes that if it is still present in six months, it needs to be removed. There is always the very rare instance that it might have been misdiagnosed and actually be a rare form of cancer which appears the same as CCAMs on CTs. For this reason, it needs to come out so that we can truly rule that out. However, she is also under the belief that if we wait six months, the CCAM may either resolve itself or it may have grown which would make its removal easier. She then continued to say that based upon the size of the CCAM, she does not believe that it will ever resolve itself. Contradictory, right?

Finally, I brought up the idea that the radiation from CT scans have been linked to childhood cancer. If we decide not to operate now, and have another CT done in six months, then what next? We can’t continue to do CTs and up the risk. She said that we would move to other methods of imaging such as MRIs. However, for MRIs, they are more difficult for children and take longer than CTs. We would definitely have to put her under and there is the chance that she might have to be intubated. Then we get into the risk of how many times do you want to put your child under anesthesia?

Upon explaining my conflicting feelings to her, the surgeon validated my concerns. As a parent, she understands me not wanting to wait but also being nervous about pushing for surgery. She did say that if we didn’t want to wait six months, she would be willing to push up the CT for three months in order to move things forward a bit. That’s a great compromise but I still feel like we’re trapped in purgatory that we’ve been in since Presley was diagnosed at her 20 week about LAST November. Imagine waiting 17 weeks of your already hormonal pregnancy not knowing whether or not your child was going to make it. Then, when you go into get induced, waiting on edge to hear them cry which signifies they’re breathing. Next, you have to wait for NICU to check them out and make sure that they are okay to be in the room with you while you recover or whether they’ll whisk them away to be cared for in NICU/PICU. Finally, when you get that all clear, the doctor tells you, “Let’s wait six months and see how it goes.” Now I’m at the point where I get to wait on the edge of my seat for an additional three to six months just to get this ball rolling again. Will it ever be over?

"D Day" Has Been Scheduled

Well, I'm a little late on reporting but a lot has happened since my last blog update in February. Within the last few weeks, I have been feeling a little off and due to my previous experience with preeclampsia, I had a feeling that my old friend was about to make an appearance again. At my Ob appointment on 3/6, my blood pressure was high again (143/97, 154/99, and 135/90). I mentioned to my Ob that I was nauseous, had experienced sharp cramping during the night, my head felt foggy (almost as if I was overly medicated), and I had seen sparkles again. She seemed a bit rushed at this appointment and therefore dismissed my concerns. When I asked her if she was going to order another 24 hour protein check, she said that I had just had my protein checked a week and a half before and while it was on the border of preeclampsia, it was still stable. She continued to say, "Do I think you're going to get preeclampsia? Yes, I know you will. We'll just have to wait and see when it shows up." When I continued to ask her if she was concerned with my bp, she dismissed it as, "high but just mildly high." I then continued to ask her about the feeling that I was experiencing which left me feeling as though I was "out of body", foggy, or just heavily medicated. She asked me if I felt like I had lost control of my body or if I would be able to respond if I told my body what to do. This has been described as the initial feeling people who are experiencing strokes have described prior to the stroke. I confirmed to her that I felt in control of my body and knew I could respond if need be. I was just slow to move/think.
I left my appointment very upset and concerned. As a patient, you want to trust in your healthcare provider's expertise and that they have your best interest at heart at all times. However, I knew that she could not personally know how I felt. I was the only person who could advocate for myself and my baby. I was also conflicted because if I did develop preeclampsia, and had to deliver early, I was worried about the safety of baby girl K. I know that the lungs are not considered to be fully developed until 36 weeks and seeing as though her CCAM was already consuming one lobe, I was afraid that she may not have enough good lung tissue to support life at this point. After a personal vent on FB, many of my friends and family were able to convince me that perhaps my doctor was just having a bad day and that it may be best to call her back in the morning requesting that she order a protein test for my own peace of mind. While I'm typically against any kind of conflict, and typically would not question her expertise, I could not dismiss my feelings and risk our safety. I called my doctor and left a message for her. She called me back almost immediately and told me that she respected my concern given my past history and would order a urine test/blood tests for me to pick up that afternoon. I did just that. Unfortunately, I turned in my 24 hour test on Friday and therefore would have to wait until Monday morning to find out my results. Imagine my surprise on Monday morning when my doctor called me back to say, "Well, as you were probably already expecting, you DO, in fact, have preeclampsia. Your protein was 330 and 300 signifies preeclampsia. We have set your induction date for 3/27 which will put you at 37 weeks." While I knew that things didn't feel right internally, it was a shock to hear her confirm my fears. Additionally, there was the extra stress that we are moving houses on 3/25 (two days before my scheduled induction). Things were about to get crazy!

Over the last few weeks, I've been trying to take things easy to keep my blood pressure down. However, anyone who has a toddler knows that this is almost impossible. With the additional stress of packing/moving, I've been an emotional basket case. Luckily, after discovering my preeclampsia, my doctor did order me to have the baby monitored twice a week to ensure that my preeclampsia was not putting her in distress in any way. These appointments have gone really well. Baby girl's heartrate has been in the 150s and seems to be moving okay. Additionally, my fluid levels have been in the acceptable range. These appointments have also confirmed what I've been feeling. I'm experiencing regular contractions which can mean early labor.

On 3/13, I had my final appointment with my perinatologist. I was 35 weeks and being induced at 37 weeks so I knew I would not need to see Dr. Richards again. At this appointment we received some fabulous news. Dr. R was unable to detect/measure the CCAM during his ultrasound. While I was ecstatic to hear this news, I was also cautiously optimistic. In my CCAM group, I have come across several women who were provided the same news prior to delivery only to have the CT scan (post delivery) reveal that the CCAM was still there, just undetectable by ultrasound. While I don't believe that the CCAM has disappeared altogether, I'm VERY excited to know that it may have shrunk (or at least remained stable) while baby girl has continued to grow. Her odds are getting better and better with every appointment. Additionally, the peri confirmed that she does have plenty of good lung tissue and that none of her other organs have been displaced or impacted by the CCAM. All good news!

Monday, I had my final Ob appointment before delivery. My doctor did check me and confirmed that I'm 2 cm dilated, 50% effaced and a -1 station. My bp was 159/93 and 146/87. Following my appointment, I had my fetal monitoring. My bp was still high (136/97 and 136/90) and they confirmed that my contractions were exactly 3 minutes apart. My Peri happened to stop by during my monitoring and checked my stats. He said that as long as I wasn't uncomfortable and was not feeling the contractions at the time, he felt okay letting me leave. He did say that he wouldn't be surprised if I just went into labor on my own prior to induction day.

So, that brings me to today. . .3/21. I'm currently 4 days away from moving day and 6 days away from my induction. I've been experiencing frequent and regular contractions (about 3 minutes apart) but no other symptoms of early labor. I do have my monitoring later this afternoon so we'll see how that goes. Otherwise, there won't be much to report until later next week. I'm very anxious but it also feels very surreal that a week from now, we will finally be a family of four. Please keep us in your thoughts and prayers during delivery. I will be so anxious to hear her first cries which will ensure that she is breathing and in the clear. . .at least until her surgery. Thanks for sharing an interest in our journey. Hopefully, I am able to reach someone out there who may have received the same diagnosis that we were given at our 20 week appointment and is feeling overwhelmed/terrified with the lack of information provided to them.

Keep the Good News Coming!

Sorry to keep everyone waiting. Blogger was having issues yesterday so this was my first opportunity to post. Friday was our latest appointment with Dr. Richards (the Perinatologist). I was 29 weeks 2 days which means that the CCAM growth typically tends to taper off or even shrink around the 28 week mark. Going into this appointment, I knew that any news that we received would be a good indicator of what this CCAM was going to do for the remainder of the pregnancy. Luckily, we received fantastic news. The CCAM did not grow (did not shrink either) yet my sweet baby girl did. It even took Dr. Richards a few minutes to locate the CCAM in the first place. He said that if he didn't know it was there, it would've been easy to miss. I was elated! He continued to say that he thinks that maybe, depending on how things go after birth, she may not need surgery. Of course I was thrilled to hear him say those words. It did get me thinking though. In my CCAM support groups, a few of the girls have opted not to do the surgery at all because their babies were born asymptomatic. I can completely understand not wanting to opt for surgery if everything appears to be okay on the outside. However, there are studies that have shown that failure to remove the lesion can lead to a higher risk of infections and malignant degeneration. From what I understand, some of these studies show that symptoms may not show up for up to 18 months and at that point, most of the long term damage may have already been done. It's a lot to think about but at least we have awhile to consider it. Until the baby is born, we won't have a good grasp of how much this CCAM is going to affect her. Once she is born, assuming she is not born in distress, they will most likely order a CT scan to really gain a better understanding of the location and size. This also makes me nervous. There is evidence that CT scans (with contrast), which is what we would most likely need to obtain the best imaging, can possibly lead to cancer since children are more sensitive to radiation. Obviously, I don't think that the doctors would recommend this unless they thought that the benefits outweighed the risks but it's still a concern nonetheless. Being a parent is so difficult when you're having to make decisions for someone who can't advocate for themselves. Obviously we want our children to thrive but I would also hate to be the cause for health concerns later in life.

Other than that, he let us know that baby girl is measuring large. She is currently 3 lb 4 oz. which is fantastic. While that won't help to ensure that her good lung tissue has fully developed should I have to deliver early, Dr. Richards did assure us that her size would be beneficial when inserting an IV and other medical procedures that she may need should she be born early.

Moving forward we will have one more fetal ultrasound with Dr. Richards where I will be close to 35 weeks gestation. He is confident that we will continue to receive good news.

Additionally, I have an appointment coming up with my regular OB on the 19th. They will continue to monitor my blood pressure and may order another 24 hour urine test to keep an eye on my preeclampsia. Both doctors are pretty positive that I will have it again. Please just continue to pray that I can at least make it to 36 weeks when we will have a better reassurance that baby girl's lungs will be more developed which will of course be favorable.

1/4/13 2nd Perinatologist Appointment

You can't imagine how difficult it is waiting five weeks for an update on your baby's condition. There is absolutely nothing you can do but wait and pray. Today was my second appointment with the perinatologist so that he could measure the CCAM again and provide me with an updated measurement. Going in to the appointment, I knew that the CCAM would've grown. I was only praying that it would've grown proportionately with the baby and that my CVR (CCAM volume ratio) would be relatively low. To reiterate, anything less than 1.6 is good and means that there is a low risk of developing hydrops.

Unfortunately, Tim found out on Wed. that a 3 Star Admiral would be visiting his command today. Therefore, they wanted everyone there to run a set for him to observe. Seeing as though it was so last minute, and several of my friends were out of town or occupied, I decided to take Kendall with me to my appointment. I was very nervous. Nervous that she would run amuck and that my attention would be divided between the information that the doctor was presenting me with (I mean, it had been five weeks since my last measurement) and monitoring her behavior. Additionally, I was nervous that if I did receive bad news, I wouldn't be in any condition to be by myself, or to drive home from the hospital. Luckily, I came prepared and things ran pretty smoothly overall. Kendall was a little restless but did pretty good at listening to my cues during the exam. And, the appointment proved to be very successful. Although it looked large on the screen (consider that the baby is only about 13 inches long at this point), it appears as though the CCAM is measuring about 2.7 cm long. At my first perinatologist appointment during the first week of Dec., it measured about 2.5 cm. At my MRI, on Dec. 10th, it had remained about the same. Now, almost 4 weeks later, it has only grown .2 cm. On top of that, he finally calculated my CVR for me. It is calculated by taking length x height x width x .52/head circumfrance. Seeing as though I had a calculator on my phone, he had me calculate the CVR.  2.7 x 1.86 x 2.9/ 23.8 = .61 CVR. However, looking back at the formula, it appears as though I forgot to times the CAM volume x .52 before dividing by the head circumfrance. If this is correct, it actually looks like my CVR is much lower measuring at .32. I'll have to double check with the perinatologist to make sure that is correct. However, either way, we were both very pleased with the appointment and the fact that the lesion has not grown much over the past five weeks. Don't get me wrong, I'm not in the clear yet. CCAMs typically plateau in growth around the 28-29 week mark and I'm only at 25 weeks 2 days. However, it is very promising that in 5 weeks, my CCAM has not grown much in comparison with the rest of the baby. Please continue to keep my sweet baby girl in your prayers as I just know that they are working.

Additionally, at my appointment, the perinatologist did confirm again that the CCAM appears to be on the lower lobe of the right lung and does not appear to be affecting the growth of the heart at all. All four chambers of the heart looked excellent. He did see some healthy lung tissue on the right lung which is good. Additionally, he said that based upon the MRI, he was pretty positive that it was a CCAM and not a pulmonary sequestration.

Next week is my monthly OB/GYN appointment and my next perinatologist appointment is not until Feb. 1st. Until then. . .

The results of my MRI are in . . ..

Just got off of the phone with my perinatologist. He said that the MRI looked just as he thought it would. The CCAM is measuring about 2.7 cm x 1.5 cm which is good. He has seen and dealt with much larger CCAMs in his experience. Plus, it hasn't really grown at all since my last ultrasound with him about two weeks ago. Apparently, according to him, the growth of the CCAM typically stops or severely slows down around the 28 week mark. Only 6 weeks to go! He said that there was good lung tissue there in the right lung and that the CCAM was pushing up against it slightly but that was to be expected. The CCAM also appears to be microcystic in nature (Type III). At this point in time, he does not think that I am a candidate for surgery in utero. The risks definitely outweigh the rewards. He said he'd have to consult with the pediatric surgeon again on Friday (to be sure) but I have already met with her and I'm pretty positive based upon her reaction that she will not be an advocate for that surgery. This is excellent news! It is something that I've been worrying about since it was first mentioned.

Other than that, I have a follow up ultrasound appointment scheduled with him on January 4th. He said that he would provide me with my CVR (CCAM volume ratio) at my next appointment. Basically, A CVR less than or equal to 1.6 suggests that the risk of hydrops developing is low. A CVR greater than 1.6 suggests an increased risk of developing hydrops. This number is calculated by taking the length of the CCAM x Height X Width x 0.52/head circumference. Based upon the size of my CCAM, he expects my number to be small. He even said that based upon the size, he expects that there is no reason to think that everything will turn out favorably with this baby. Even though most of this information was already known, it is always nice to have confirmation and it was definitely a relief to hear!

Tomorrow I have my regularly scheduled OB appointment and then we will be ready to enjoy a few weeks off with NO appointments. Hopefully I can enjoy the holidays with my family and take a moment to step away from the concerns that have been plaguing me these last few weeks. When I get back, I will be well rested and ready to take on whatever is thrown our way.

Beautiful people. . .

 

 

Saw this today on a friend's blog and found it to be so appropriate. When I start to doubt myself throughout this whole process, I just need to reflect and realize that this process will only make our family stronger. We are definitely changed as a result of our peanut's diagnosis but hopefully our our struggle will only promote positive growth for our family. 

 

Fetal MRI

This morning I finally had my fetal MRI. Seeing as though I'm claustrophic, I've been dreading this day. I've experienced an MRI before (for a pinched nerve/bulging disk) and felt like the walls of the machine were closing in on me. Fortunately, it only lasted about 20 minutes that time around so it wasn't so terrible.

Upon arriving at my appointment this morning, the doctor went over the all of the necessary instructions and had me fill out the appropriate paperwork because I'm pregnant and opting to have this MRI. She then explained that this experience would probably be much more pleasant than my last one because the tubes have gotten slightly larger. Yet, on the other hand, the magnet has also doubled in strength. Additionally, they now have the ability to project images (of mountains or other serene landscapes) on the walls of the MRI room (not the inside of the tube itself) which would promote a peaceful environment/mindset. It would last slightly longer than my last MRI (about 30-45 minutes). Additionally, instead of going in head first, this time they had me go in feet first so that my head was at the end of the tube and within eye sight of the opening. What she failed to mention was that after laying on the table, they would strap a belt around my chest and cover me with a heavy mat/shield on my chest. Then, seeing as though they wanted a good look at my uterus, they would have me do the entire MRI with my arms above my head instead of at my side. When they first began the exam, everything was fine. They had covered my legs/feet with a light sheet to ensure that I was warm and comfortable. That's when he began asking me to hold my breath. "Deep breath in. Exhale. Hold your breath. . ." I began counting the clicks and pounds of the machine. By the fifteenth click, I was struggling to hold my breath any longer. He barely let me catch my breath before he began again. After a few of these repetitions, I could hear him say though my earphones, "Are you having trouble holding your breath that long?" Ummm, yeah. Ya think? You aren't letting me take another breath after my exhale before holding my breath. The MRI went on for what seemed like forever. They moved the table back and forth throughout the tube. The further in they put me, the tighter I felt my chest get. After about 45 minutes, my shoulder started to feel a sharp pain. I was very uncomfortable but terrified to move. I just kept telling myself to suck it up. The MRI would be over soon. It had to be over soon. I kept waiting and waiting for him to tell me, "Almost there" yet I wasn't getting any sign that the end was near. I started getting more and more anxious. My whole body started to break out in a sweat and I had to tell myself to take more deep breaths. The baby had started getting restless at this point and I was much more in tune with her little jabs and kicks. I could feel the palm of my hand starting to draw tighter and tighter around the little panic button that he had given me in case I needed to get out of the machine. Finally, I couldn't hold it anymore. My shoulder was throbbing. I squeezed the button and heard the man's voice through my headphones. "You okay?" I told him that my shoulder was aching and I needed to come out to adjust it for a minute. It took him a minute but he finally moved me out of the machine and told me that my doctor said that they had everything they needed. Removing the chest pad and belt felt like a huge weight had been lifted off of me. When I finally looked at the clock, I realized that I had been in there for over an hour. No wonder time had seemed to be moving so slow.

After exiting the MRI room, the doctor was there to meet me outside. She didn't have much new information to tell me. She wasn't in the room to witness the entire MRI but she said that from her initial scan, she believed that it was a type III CCAM in the right lung. She did not see any signs of hydrops at this time. However, she confirmed that she would need to look further at my MRI to perform a more in depth analysis and write up her formal report. It would take about two days to get those results back. Hoping that this report provides us with more information as her breakdown today didn't provide us with any new information. I guess I will know more in the next day or two when I stalk my perinatologist for her report. : )