Sunday, June 17, 2012

Paige's story

Paige Alivia gave us quite a surprise when she was born. First, off when we went in for the birth, birth certificates were all ready with the name Parker on the papers. In fact, we had been told throughout the pregnancy that we were having a precious little boy! So when Paige popped out, the doctor said, “Honey, did you know HE had a cleft lip! He’s beautiful and they can easily fix this!” With all the shock, the doctor didn’t notice until about 30 seconds after this that HE was in fact a SHE. So many surprises arrived with the birth of Paige. Paige was born with a unilateral cleft lip and palate. A unilateral cleft lip and palate means that at around 6 weeks gestation, Paige’s roof of her mouth and lip did not fuse together properly. We had routine ultrasounds, but did not have a scan of the mouth as we already had two precious perfect children at home. In addition to the clefting, Paige also did not pass her infant newborn hearing exam. Children with clefts often have Eustachian tube failure as the tube is closed more and fluid cannot clearly pass through. The fluid builds up and causes numerous ear infections and loss of hearing. At birth, we were struggling with the roller coaster of emotions of having a child born with a cleft. We blamed God, we questioned everything. As a mother, I began to accept my child moments after birth but for my husband, he was angry with God for quite some time. He truly had to go through the entire grief process to come to terms with what our child would have to go through. The feelings of grief were more geared toward the fact that our beautiful, precious newborn would have to endure more in her life than my other children had ever. When we got home, I spent days, evenings and nights researching clefting. Google became my new best friend and I would stare at the screen while I held my beautiful girl and cry that I would have to put her through so much hurt. I questioned my pregnancy and all I had done during those life-growing months. It hurt so much more to think that I could have caused this in any way. The first few days were very hard without the answers we were seeking. Our pediatrician basically told us that we would see a specialist and that would make everything alright. Waiting until Paige was 5 days old to meet the doctors at Pittsburgh Children’s Hospital in Pittsburgh, Pa was the most difficult 5 days of our life. Our visit in our local hospital for the birth of our cleft child was also difficult. There are many misconceptions with cleft children. With the birth of my other two children, soon as they were born, they were immediately breastfeeding. This not only causes a better relationship with breastfeeding but also a strong bond between mother and child. This was not a possibility with Paige. We were later told that only 1% of cleft palate children are successful with breastfeeding. However, I was determined to be that 1%. I tried and tried and tried to get Paige to suckle. When the reality settled in, I was very depressed and saddened by the fact that Paige would not be able to have that bond. She was able to successfully receive breast milk until she was almost 3 months old. At that point, I was back to work full time and emotionally unable to continue with the demands of working, raising three children and her medical needs. At the hospital, we were told to stay three nights in the hospital because the nurses were convinced that Paige would be able to breastfeed if we were persistent about it. This gave me hope only to find out later that it really was just an emotional waste of time. The first visit to Pittsburgh was the first of the many weekly trips, 215 miles away. When we arrived at the hospital, we were scared. We held Paige tight and looked around at all these children visiting the hospital. I remember immediately looking to see if there were any other children with a cleft in the waiting room, something I still do to this day. One child stood out to us. As we sat waiting to meet the doctors and team, a woman came up to us and offered to show us her 6 month old baby boy, who was also born with an unilateral cleft lip. She said she was the grandmother of the little boy but that the mother could not handle the cleft experience so they were taking over care of the little boy. He was the most beautiful little boy I had ever seen. Full of life and with a sweet scar to show for his recent cleft lip surgery. She told us that we had light at the end of this long tunnel and that the team of doctors was caring and supportive through every step of it. I held back my tears as we played with this little boy. That was the end of our worries of having a “normal” child. The first visit to Children’s Hospital was a very long, emotional day for us. The first person we met was Paige’s speech therapist and also coordinator of the cleft center. He explained to us the process of the cleft team, the roles each person played and then began to tell us that Paige also would be fitted this day with a NAM. A NAM is a Nasoalveolar molding that is fitted to sit in the roof of her mouth and would stay there until she had her first surgery, at about 4 months old. He then proceeded to show us the pictures of other children with a NAM and how it made a huge difference in the overall appearance of their surgeries. As he began to show us the pictures of these other children, my heart sank. Not only was my child going to show to the world that she had a cleft lip for 4 months and then the rest of her life, but she also had to wear this device that clearly had 2 steri-straps that were tapped to her cheeks to hold it into place. He gave us the choice to talk about it and stepped out of the room. As he walked out the room, my husband and I both had the same thoughts. We did not like the fact that we had to do this but there was no choice, our child would get the best of everything. She would be fitted for the NAM that day and wear it until her first surgery. We then met her pediatrician, surgical team and doctor, orthodontist and social worker at this visit. When Paige was fitted for her NAM, we were not allowed to go into the room with her. They informed us that they have to have a team hold her upside down, place the tray of molding in her mouth and hold her there for a few minutes in order to get the impression to make the NAM. For this reason, parents are not allowed to observe. We would then wait about 3 hours to have the NAM made. The time waiting was difficult. He both sat there and just simply took in the atmosphere around us. Children are being wheeled past us in wheel chairs, children with hearing aids walk to the office next to us, and many children required more invasive medical equipment. This was a difficult experience for us, but my heart was breaking for the parents of these children. About twenty minutes later, the coordinator of the program came to get us in the waiting room. He had a very concerned look on his face and he took us directly to go see Paige. When we walked into the room, Paige was laying on a bed and many doctors were standing around her. He told us that during the procedure, Paige’s breathing became very difficult and that he was very concerned. He wanted us to have her examined immediately by our pediatrician at home and to also be seen by the Ear, Nose, Throat doctor (ENT). Final reports indicated that Paige also had Laryngomalacia, a condition that causes immature cartridges in the airway to collapse when breathing. This condition put more limitations on our baby girl. She had to sleep with her crib slanted, had to be seated directly up when she was eating and had to be closely monitored when she was sleeping. Paige began her life with her NAM. Feeding with the NAM in her mouth was difficult at first. This was more so for her parents than for Paige. The NAM had to be removed after each feeding to wash and then reinsert in Paige’s mouth. If the tape got wet, it then had to be removed and replaced. We were completely dedicated to the NAM however. Paige rarely had the NAM out of her mouth and most of those times were simply to take pictures or to get a bath. On top of all this, we had a normal baby girl who required the necessary attention of any newborn. I was unable to breastfeed Paige because she had no suction due to the cleft in the roof of her mouth and therefore, I was spending time out of the day to pump, wash bottles and then to actually spend time feeding her in her specialized bottle. Paige was an absolute doll through it all and remained one of the easiest babies. Her poor little cheeks would get so sore and red from the tape and we would spend more time tending to that. Each day was a joy with Paige as we learned to adjust to her growing needs. Most of the time, Paige would only cry if she did not have her NAM in her mouth because no NAM meant NO food! As we adjusted to Paige’s needs, we also learned to deal with society, family and friends who did not understand clefts. At this point, I was definitely not an expert but every day I spent researching for my daughter. One situation still stands out to me. At a few weeks old, we planned to take our children to the local fair. This was something that the children had been looking forward to for weeks and we did not want to disappointment them. Not only was this an adjustment for us as parents, it was very scary to our young children. My son was very concerned that these procedures were hurting Paige and my daughter didn’t want to hold her very often because she was afraid she would hurt her too. Therefore, a trip to the local fair was something they looked forward to. While at the fair, many people would look in the stroller expecting to see a precious baby. At one point, we had stopped to let our children ride the carousel. They lady running the ride asked to see our little girl. I opened up the stroller cover and she gasped. She asked what had happened. I calmly explained that she was born with a cleft and that this was a device that would help her before surgery. She then stated, “oh oh oh… well my son just had a kid a little while back and that’s not something they did with their kid.” Clearly she did not understand. Clearly, this was not something that people advertised. At that point, I realized I needed to help people to understand clefting and how it affects other people. It was at that point that I really did put a God-willed purpose behind receiving Paige as my daughter. The next few weeks consisted of pediatrician appointments and weekly trips to Pittsburgh to adjust the NAM and to visit the ENT. Paige was then scheduled for her first surgery at 2 months of age. This surgery was to help improve hearing by placing tubes in her ears and then also to do a scope down her throat to make sure that she did not have any other conditions outside the laryngomalacia. This surgery was very scary as it was the first of any of our children. One of the best things I had done as a mother of a child with a cleft was to connect with other moms with cleft children. I was sought out by a grandmother who was seeking support online for her daughter who just had her very first child, who was born with a severe cleft lip and palate. I instantly became friends with her and we connected through our children who was only one month different in age and who also attended the same hospital. Therefore, on the night of our first surgery, our family got a hotel room close to the hospital to meet up with Erica and her beautiful daughter Lily. The girls became the best of friends. We did not direct them to hold hands but was overjoyed to see them want to hold hands. One person mentioned that they were saying, “we will get through this together.” I know one thing is sure, I wouldn’t have been as solid and stable through the surgeries if I didn’t have Erica who would say, “You know Lily does that too!” We had to be at the hospital at 6:30am on a Thursday for surgery for her ear tubes and scope. We arrived a few minutes early and everything moved quickly. Paige was not allowed to eat after 2 am so she started to get fussy but was really great all morning. We talk to the doctor and discussed the procedure, length of time and expected outcome afterward. The plan was to go home the same day as long as all went well. We also met a nurse who had a cleft lip and palate. He told us his story and how his surgeries went. Paige went into surgery at 8:00am. She was there until 9:45 when we were called back to see her. She didn't even look like herself. She didn't open her eyes and her face was really white. She was screaming! She stopped for a minute when I picked her up but continued to scream then after. I got her to eat 1 ounce of sugar water and then waited for the doctor to give us the results. She was still very upset and they gave her a really strong medication to relieve the pain. She calmed then after but I was unable to put her down. I held her from 9:45 until 4:30 with only putting her down a few minutes to go to the bathroom and to change her diaper. She wanted to be held and by all means, I was holding her! The doctor came to talk to us and told us that everything went as planned. (Although they told us it would only be a 45 min surgery but ended up being 1 hour and 45 mins). He said that her scope indicated a very mild case of reflux with a little swelling around her larynx with also mild laryngomalacia. We were instructed to continue giving her Zantac for reflux and to thicken her bottles. He said that her ears were pretty bad. He said both sides were completely full of fluid and it was thick, therefore he put an incision in her ear and inserted the tubes. He reported also that the hearing loss was more than likely conductive hearing loss and not permanent hearing loss. However, hearing loss is still hearing loss in my book. At this point, I began to teach Paige sign language to help her communication skills. We were then informed that due to her age, they would be keeping us over night. Our first of many overnight stays at Children’s Hospital. Medication for Zantac, NAM adjustments in Pittsburgh, teaching sign language, and all the other joys of being a parent to an infant consumed our life for the next couple of months. We also struggled with persistent ear drainage that required numerous ENT visits to have her ears suctioned and check for infections. Through all of this, Paige did not have ear infections but simply continued hearing loss and drainage. She was tested at audiology in Pittsburgh, where she continued to fail her hearing exams. This emotional roller coaster of determining when she would be ready for that first ever cleft surgery was always in the back of my mind and never ending. We met with her orthodontist every single week to adjust her NAM and to determine when would be the best time for surgery. Her first surgery would be to close her cleft lip and to repair to nose. The NAM was already doing a fantastic job of repairing the fallen appearance of her nose. The NAM was also pulling together her cleft in her gum line, a serious concern of ours that could require additional surgery when she is older and into her teenage years. The time was arriving for that first cleft lip surgery. Our prayers were that this would be the only surgery required to close the lip and repair her nose. Support of our new cleft friends, family and friends helped us through the entire process and surgery. Last pictures of Paige prior to cleft lip surgery yielded an amazing result from where we started. The day arrived. I have never felt so at peace with a surgery. I was incredibly happy that my daughter will have the perfect little smile that she deserved. The doctors who we had grown to trust were going to change our lives forever. We were warned prior to surgery that when we walked into the PACU after the surgery, that we may not recognize our own child. I really thought this was ridiculous but after the fact, very much true. We were also told the entire time that after surgery, you would miss the cleft smile. This I did not find true. I loved my daughter and I loved her smile for what it was but she deserved so much more. She deserved to look as beautiful on the outside as we saw her as her parents. Our doctor took pictures to place on Paige’s bed so we would know to look for the pictures instead of our child. This seemed only ridiculous but ended up being helpful and a treasure to us. Walking back to surgery was hard. We were able to walk to the doors with Paige but then the nurse took her the rest of the way back. I remember looking at her while she blankly stared at me, not knowing what was about to happen. Waiting in the parent’s waiting room was even more difficult. You were able to view your child’s location in surgery by a number assigned to your child. I checked this TV screen at least a million times waiting for it to say “post op.” Paige’s surgery lasted 6 and ½ hours long. Finally, our team of doctors came to tell us the good news. Paige’s lip, nose and gum line was repaired. Her gum line was able to be slightly fused together to hopefully help a tooth to come through. A small gap still remained and may require a bone graph when she gets to be about 8 years old. However, we were able to go back and see our beautiful baby girl. I did not cry, I did not flinch, I grabbed my baby girl and held her consistently for the course of the next few days. Thankfully, when my arms started to hurt, my husband would hold her too. She was very much in pain but I was able to get her to drink a little bit of sugar water and we asked for more medication. One of the best advices I got from other parents was to never ever let them go without medication for even a minute. We looked at our beautiful, swollen little girl with a bluish lip and were simply in awe. As the days went on, Paige started to get back to herself and her little personality began to show back through. Her scar became less noticeable and she was healing perfectly. Her nose looked amazing and absolutely perfect as well. A few months after this surgery, we were told that Paige had an infected stitch in her nose that was removed and later caused her nose to fall a little on that side. Otherwise, her nose is still perfect! As a parent of a cleft child, I began to help the same way that I received, at this point. I was asked for Paige to be a March of Dimes sponsor child for an event at Germany Air Force Base. I graciously accepted. As a family, we started a donation page for Operation Smile and Smile Train every year. I began to connect with other moms online with different organizations. And we also supported Lily when it was her turn for surgery. Our visits to Pittsburgh were not as frequent as prior to surgery for the lip. She no longer had her NAM and had to relearn to eat without it again. The next big hurdle Paige would have to overcome would be to be completely off the bottle by her next surgery for her palate. She was able to get off the bottle by 9 months and used various different Sippy cups because at this point, she still did not have any suction. Surgeries and the NAM put restraints of Paige’s ability to learn to crawl. She was not allowed tummy time because she could push the NAM into the back of her mouth and cause it to become sore or bleed. Also, after surgery, Paige had “no-no bands” on her arms so she could not bend her elbows to touch her mouth. During this time, she was restricted in her movement and was unable to lie on her stomach for any amount of time. Therefore, Paige had a slight delay in crawling but when she was allowed to start crawling and lying on her stomach, she picked it up very quickly. Paige also was signing five to ten words at this point, regularly. She still had not passed a hearing exam and the doctors were very optimistic that after palate surgery, her ears would do better. When it was time for Palate surgery at 10 months old, we had already undergone surgeries and were starting to feel like we could handle anything that came our way. Palate surgery was a bit scary, however. Her surgery lasted 4 ½ hours long and it would determine if our child would learn to hear and speak. This was a lot of contingencies for us to think about during the long wait for surgery. Post op was a lot different for palate surgery for Paige. She was in a great deal more pain and had to be managed with stronger medications this time around. She also had a tube inserted into her nose and down her throat just in case she would have difficulty with swelling in her throat and needed Oxygen. As with lip surgery, Paige required consistent holding and often would sleep on my lap during the day and night in order to get a good night’s sleep. Recovery was much faster with the palate surgery than with the lip surgery for Paige. She was back to herself in a matter of days but still had to wear the “no-no bands” for a couple of weeks. She learned a fun trick of snapping her arms hard enough that the bands would fly off but she was still a good girl and didn’t put her hands in her mouth. Weeks to follow yielded a few more doctors’ appointments for Paige. We were told that she healed perfectly and that her palate was intact after surgery. We were also told that her speech would continue to be evaluated over the course of the next couple of years and if there is a continued problem, an adjustment to the palate may need to be made. Paige was evaluated for speech therapy at the age of 9 months old incase there was a problem with eating after the palate surgery and later for the speech component. However, Paige never had a problem eating after the first few days after surgery. Initially she was not interested in a Sippy cup and required her IV fluids while in the hospital. By the time we were allowed to leave the hospital, Paige was able to eat 4-6 ounces of fluid consistently. Paige was not allowed any solid food for about a month after surgery. This was difficult for Paige who loved her food! After surgery, word began to fly out of Paige’s mouth! She was learning at a rapid pace all the sounds that she originally couldn’t say. Also, we had her tested one month after palate surgery and were excited to share that Paige passed her hearing exam. We had a hearing, fully healed cleft child. This was a remarkable day in our life. Paige learned to walk by the time she was one year old. We were incredibly excited to be able to share our precious, perfect little Paige with the world. In the time spent since that year of growing as a family and changes to our Paige, Paige is thriving as a typical 2 year old. She will turn 3 years old August 2012 and will then be discharged from speech therapy services. She talks very well, has great communication skills and the attitude of any little princess. She is full of energy and has a wonderful personality. Paige has undergone two more surgeries since this palate surgery to replace tubes in her ears. She now has permanent t-tubes and hopefully will not need those replaced until she is an adult. Paige has all of her teeth, with only one of those sideways. We are still unsure if she will need a bone graph when she’s older to fill in the small gap in her gum line. However, at this point I know for a fact that she will have braces and will need to realign her tooth in that gap. The rest of her teeth are wonderful, straight and adorable. Paige visits our family at the Pittsburgh cleft center once a year for a full evaluation. She visits her ENT if there is a problem with her tubes. We are incredibly thankful to the cleft team, doctors, families and friends who have supported us through this entire process. We were saddened to hear that Paige’s orthodontist, the wonderful Dr. Lisa Vecchione passed away in a car accident almost a year ago. She is sadly missed for the love, support and talent she put into my daughter’s NAM and personal care over the course of her first year of life. Without her talents as a doctor, my daughter may not have as beautiful of a smile as she has. We are grateful to the entire team. We carry on with life as though this is in the past but I know that in my heart, God gave us Paige because he wanted us to help others. He wanted us to be humbled by the fact that anyone could be touched by a child with a birth defect. Paige is very excited to be welcoming her baby sister this fall as well. We cried at the ultrasound when it reviled that God granted us with another beautiful little princess, without a cleft.