One family's story: our unborn baby's birth defect

by Shane, Meegan, Talon and Tristian Perkins

Perkins jpegA little better than a year ago, Shane and I and our 1-year-old son were excited to find out that we were going to have another child. But when we got to our 20 week ultrasound on a Friday in September of 2008, my husband and I were told the words you never want to hear when you are pregnant: “There is a problem with the baby.” We were shown spots of fluid in the baby’s left lung and were told that it most likely was a CCAM (congenital cystic adenomatoid malformation), an abnormal growth of lung tissue that prevents the normal growth of the lungs.

Our world stood still and all we could think to ask was, “What did I do wrong and why did this happen?” We were assured that it was nothing that we did and that was there anything we could have done to prevent this. We had thousands of questions that needed immediate answers. We were told the soonest we could be seen was on Monday with a specialist in Manchester, New Hampshire. This was the longest weekend of our lives. With support from our family and friends, we knew all we could do until Monday was wait and pray.

Further tests confirmed that it was a CCAM. We had a lot of our questions answered and decided that terminating the pregnancy was totally out of the question. This was our child and we were going to fight for this baby with everything possible. The doctor felt strongly that we could fight this, but made no guarantees and described the long road ahead. We were ready and willing to fight; we would do whatever it took.

Testing showed that the CCAM was very large and it was pushing on the baby’s diaphragm and causing the baby’s heart to be pushed to the right side of its body. So not only did we have to focus on the CCAM growth, but we had to watch for hydrops (life-threatening problem of severe swelling in the fetus and newborn), heart failure and pre-term labor.

It was determined that all care would be done at Children’s Hospital Boston at the AFCC (Advanced Fetal Care Center). The first appointment consisted of an MRI to see the size of the CCAM, then we had to have an echocardiogram of the baby’s heart, then an ultra sound and finally to meet with the surgeon Dr. Russell Jennings. This was a very long day, but we received a lot of information. The diagnosis showed that the CCAM consisted of cysts (fluid-filled sacs) and was very large. There was also a sequestration – abnormal connection to a main blood vessel that was feeding the growth. There were no guarantees that the baby would survive this, but the doctor was willing to do everything he could. We felt very comfortable and confident that we were in the right place and our son was getting the best care possible. None of our questions were left unanswered.

Each visit was a relief to hear that the CCAM had not continued to grow and there were no signs of hydrops or heart failure. Other cases had shown that the CCAM would stop growing at 28 weeks, so each day that we made it through was one day closer to the 28 week mark.

The day that we were told the CCAM was growing again and the baby was just below the point of heart failure was devastating. Again, we pulled it together and with the support of our families we moved forward. We were given the option for me to have a shot of steroids that in the past had stopped the growth of the CCAM for a period of time and would help the baby. At 24 weeks, I got my first shot of steroids. The next two weeks showed that the CCAM had stopped growing, an answer to all the prayers. The steroids did exactly what they were suppose to do.

At 26 weeks, I had one more shot of steroids. They stopped the growth of the CCAM once again and our baby made it to 28 weeks. It was at this point, that they predicted the CCAM would stop growing—and it did.

I continued the trips to Manchester, New Hampshire and Boston until the 34 week mark. Then I had another MRI at Children’s that showed the CCAM was still very large, but the baby was outgrowing it and I could now transfer my visits to Beth Israel Deaconess Medical Center in Boston. Although things had turned around dramatically, we still knew there was a chance the baby would need surgery at birth and we were prepared. Each day and each struggle strengthened not only our hope but our commitment to our child. By now, we knew that he was a boy, and that he was going to make it.

Tristian 9lbs 2oz 1-22-09With the skilled care of our team of doctors and nurses ready in case any problems came up, thousands of prayers, and a child that was determined to take his place in our family, Tristian Shane Perkins came into this world on January 22, 2009 weighing in at 9 lbs., 2 oz.

He was monitored the rest of the night in the NICU and was able to come to our room the following morning. Tritsian was doing absolutely fantastic and we got to take him home to New Hampshire two days later. We just couldn’t believe that he was so healthy and strong and we got to take him home so soon.

Tristian had an appointment with Dr. Jennings in July of 2009 and was scheduled for surgery September 3, 2009. During this operation, Dr. Jennings would remove the mass from Tristian’s lung. Leaving the mass in place would pose a risk of infection. Also, having surgery at such a young age would allow new lung tissue to grow in the space left by the mass, so there was no question in our minds that this surgery needed to be done.

We were nervous and anxious to get the surgery over with. The idea of him going under anesthesia and having surgery at the age of 7 months was  scary, but deep down we knew he would be okay. If he could make it through all of the obstacles so far, he could make it through this. Having surgery at such a young age would also allow new lung tissue to grow to replace the mass that was being removed, so there was no question in our minds that this surgery needed to be done.

The day of surgery was the longest day of our lives. The hardest part was to give him up to the doctors and watch them walk away knowing we could not be in the room to comfort him. I knew he was in the best hands and they told me as they took him away, “Don’t worry, we will take very good care of him.” I trusted them. Tristian was in surgery for five hours and every hour a nurse updated us on his condition. Two days later we got to take him home.

We hope that our story has helped anyone reading it know that if you’re facing a challenge in your pregnancy or if your ultrasound shows some problems, think of Tristian (and all the other babies and families) and all he went through. Have faith in the doctors and yourself and your families. Most of all, know that babies are a miracle from the beginning to end and miracles happen every day, you just have to have faith.

Tristian 1 year oldTristian just turned 1 on January 22, and is a happy and healthy little boy. How far we have come is nothing short of amazing.

We want to thank the Advanced Fetal Care Center team, Dr. Jennings and the surgery team, Dr. Johnson at Dartmouth Hitchcock in Manchester, New Hampshire, and Dr. Lim and his team at Beth Israel for the time they spent with us to make sure we had the best care we could possibly have during pregnancy and for a safe delivery. Of course we also want to thank our family and friends for their support and prayers. We could never have done it without you and Tristian is here and healthy because of all of you.

Become a fan of the Advanced Fetal Care Center on Facebook.

  • Sandra

    Thank you so very much for sharing this story. Today, my daughter who is due the first of Sept. was told that the regular ultra sound + a 3-D ultra sound showed a black spot appearing to be in the lung area. She goes for another ultra sound next week by someone else, to be for sure.

    We of course are scared, but as you had, we have a heavenly father that we trust in and by faith walk with.

    Sandra

  • Donna

    Thank you so much for sharing this. We were told just a couple of days ago that our baby has a mass on his/her right lung. It has been a scary couple of days to say the least. We will be going in for a full evaluation next week. It gives us hope to hear of Tristan’s story. We hope to one day be a positive inspiration to others as you have been to us. Thank you again.

  • Donna

    Thank you so much for sharing your story. We were told at our 20 week ultrasound (just a couple of days ago) that our baby has a mass on his/her right lung. This has been a scary couple of days to say the least. Hearing stories like Tristan’s gives us hope. We go in for a full evaluation this week. We hope to one day be a positive inspiration to others as you have been to us. Thank you again.

  • Cammie2

    Thanks so much for sharing your story. We received 2 surprises at our 20 week ultrasound. 1- We were having a girl! and 2- She had something called a CCAM. It was heartbreaking because everyone wants to hear that their baby is perfect! We have been working with wonderful doctors in the Philadelphia area and thanks to our family and friends we are hanging in there. We have been going for ultrasounds every couple weeks and have been hoping that the CCAM either stays the same or shrinks. So far we feel lucky to have made it to the 29 week mark with no hydrops and continue to pray that the mass stays small up until the end. Thanks again for sharing yours and Tristian’s story because it gives us hope that our little girl will be okay.

  • Des & Jess

    Thank you so much for sharing your story and calming me down as well as putting my worries to rest.

    In May 2011 at 20 weeks the scans showed we are having a girl, which we named Jessica. The scan also showed that she had a hernia in her diaphragm and her intestines pushed up through the hernia, pushing her heart to the right and squashed it so badly it was difficult to get a clear picture of it and see whether it was healthy. We were faced with the terrible options of termination or having her suffocate at birth. My husband an I, our family and friends went through hell in the days that followed and I even told our Minister that I do have faith but at some point we need to face the facts it was even a let her go now or watch her struggle for life at birth. I was crushed and really needed a miracle straight out of the Bible. We got an appointment at a Fetal Assessment Centre 3 days after the original diagnosis. I was so scared and cried every time I opened my mouth to speak.

    We got great news from the Specialist which changed everything! Jessica has CCAM and had so many cysts it formed a string of cysts that it looked the same as the intestines would that got trapped.

    We were subsequently monitored by our OBGYN and each time the scans looked better. The cysts didn’t grow and Jessica’s heart progressively grew and moved into the correct position.

    Today, we are 6 days away from birth, Jessica has grown very well, her infected lung is almost clear and her heart is strong and almost 100% in place.

    We are thrilled about the progress made by our MIRACLE – the one I initially did not believe possible. We also found out that Jessica means God’s Grace and find it very fitting!

    I personally will never doubt the power of prayer again. Thank you to my Husband who has been and remains my rock, my family and friends and all those people we don’t know but who prayed for Jessica’s health! But my biggest thanks goes to my Heavenly Father, God ALMIGHTY!!!

    Looking forward to meeting our Miracle next Wednesday!! We love you dearly Jessica!!!

    Love
    Desere Stins

  • Elizabeth Norman

    My son had a CCAM that was diagnosed at 22.5 weeks in utero.  Like you, we had to wait all weekend to see a specialist at Georgetown University Hospital and another specialist at Johns Hopkins.  Our CCAM was very large and we were told upon diagnosis at an ultrasound facility to abort our child — who will be celebrating his 16th birthday on August 24th, 2011.  

    This defect is very poorly understood outside the realm of specialists like perinatologists, neonatalogists, and pediatric surgeons.  A diagnosis by a radiologist cannot really tell you anything other than your need to see a specialist. Keep your wits about you and don’t panic.  

    I learned that this is a very dangerous and emotionally taxing defect, but it is also very treatable. 

    Our son’s CCAM resolved at birth. One minute it was there, and after my caesarean delivery, it was gone.  I was very carefully watched during every aspect of my pregnancy and Andrew was very closely monitored.   I recommend prayer and the best doctors you can find.  I will always believe that God’s hand has touched my son’s lung — he has a beautiful singing voice and we talk often of the nature of miracles.  

  • AA

    We live in Canada and my wife is 24 weeks pregnant. 20 Week ultra sound showed that baby’s left lung isn’t growing properly.  Right lung is fine. 

    My wife’s Gynecologist referred her to another Gynecologist who was very calm and told us that  my wife would have to be very closely monitored.  He wants an ultra-sound done every month and wants us to see a neogatologist. We have full confidence in doctors here.  However, we need your prayers.  It is very inspiring to read encouraging stories.

    • AA

      Hello Everyone,
      Just wanted to update everyone on our side since I wrote last. 
      My wife had the baby boy (named Ibrahim) in March 2012 through normal delivery. By the grace of God, our son was doing fine at the time of delivery. The doctors couldn’t believe how normal and active he looked. A quick x-ray of the baby’s chest confirmed the CPAM (previously called CCAM). Two weeks later we went for a CAT Scan which also confirmed the presence of abnormal tissues in his right lung. The pediatric surgeon at the hospital told us that he recommends a surgery when our son is 7-8 months old. The assured us the outcome of the surgery is very positive and our son should be out of the hospital in 2-3 days post surgery. 
      Today our son is just over two months old.  He is normal as any kid with no problems at all.  Sometimes it makes us wonder if we really need the surgery.  I am sure some of you have gone through the same dilemma.  Your thoughts?

      • Meegan Perkins

        I am just able to get on and respond. I hope if you have gone through the surgery that everything went well. If you read my story my son was 7 months old. This surgery was elective and our choice to have it done. We were told since CCAM is fluid he would be get very sick a lot, new lung tissue replaces anything taken out at that young of an age, and its possible that this bad lung tissue could turn to cancer (even though its not cancer now. Felt very confident have the ccam removed as it seemed like more complications leaving it in then taking it out. My sons CCAM was tested once removed and tested positive for pneumonia. It was the best choice we could have made for him. I wish all the best with your decision and wish a healthy and happy life for your family and your son.

  • Gewrgios

    Stella, is 23 months,  she had ccam, she made the surgery in Onasseio cardiosurgery center in Athens Greece, when she was 8 months old.
    I have to thank GOD and PANAGIA, and all the known and unknown people who prayied for her.
    A lot of thanks to Dr. Vasileiadis (gynaikologist),Dr.Tsanakas(specialist pneumonologist),  and the surgery team of Dr.Azariadis (cardiothoraxsurger).  
    Advices: Be aware of everything! Our ex-gynaikologist saw the mass in the lung and said nothing to us…(luckily we had a second level ultrasound check next week so we found out about ccam).
    When Stella was trhee months old she was checked for the lungs but only by stethoskope, when I asked the doctor for x-rays he told me “there is no reason”…When she was 8th month the x-ray showed us that the mass was growing and pushing the heart, with a big possibility of pneumothorax, so if we had the x- ray on the third month we had know about it earlier( I do n’t want to think how lucky we were and pneumothorax did n’t happen meanwhile).

  • Meegan Perkins

    Thank you to everyone who has posted their story. I am honored to have been able to share our story. Our son Tristian will be 4 on January 22 2013 and I can’t thank everyone enough that he will be celebrating another birthday. I hope that all the families who have posted are doing well and their children are happy and healthy. I enjoy reading these stories and wish you all the best everyday. Thank you again from the bottom of our hearts.

    The Perkins Family

  • http://www.facebook.com/rachel.c.smith.9 Rachel Courtney Smith

    I wanted to come back and thank-you for sharing your story. I read this late one night after I received news our baby had a CCAM after our 20 week ultrasound. Baby’s heart is also being pushed to the side, and hydrops is at the top of my mind. We’re at 21 weeks now, but reading your story has given me so much hope! Thank-you again for sharing, and congratulations on such a wonderful outcome.

  • Rocio

    I am scared as i was told two weeks ago that my baby boy wont make it. The specialist told me he has 4-6 weeks left. They found cystic hygroma and the following week, they detected fluids right next to the left lung. I am scared :( but your posts are giving me hope. We have to be seen every week, and again, pretty much the doctor is just waiting for the heartbeat to go away.

  • Donna

    For some reason, my comment didn’t show up the two times that I tried, so I am going to try replying to this message instead.

    Thank you so much for posting this story. We were told this past week, at our 20 week ultrasound, that our baby has a mass on his/her right lung. It has been a very scary couple of days, with a lot of worrying about all the “what ifs.” We are inspired by Tristan’s story and we are optimistic. Our full evaluation is scheduled for this Thursday. The waiting is the hardest part. We hope to one day be a positive inspiration to others just as you have been for us. It gives us hope…and that’s what we need right now. Thank you again.

  • Jo Nolan

     The details you gave brought back all the memories i had of finding out from my 20 week scan that my son had c.cam and it had already pushed his heart to the right.I had never heard of it until then. I live in the united kingdom and they do things slightly different they dont like to operate until a child is 3 years old so we had a long wait but at 3 and a half he went into hospital and had a lung lobectomy and came home after 3 days.when i was walking to the recovery room i could hear him shouting mummy i want to go home.which I was so happy to hear.he is fine and is discharged from hospital and is now nearly 6 years old and even the scar is nothing to what i always worried it would be. I had all the worry from my 20 week scan until my son was 3 and a half years old but the day they wake up from that operation your worry is over.my son is as fit as his class mates.we didnt get to talk to other people because the only information we get is from your country so i know your story must of helped so many people.