Even when the surgery to correct CDH is successful, the defect still has lasting effects and leaves behind challenges to overcome. These challenges are different for every "cherub," but here are the challenges we are facing with Makenna.
Oxygen Therapy:
One of Makenna's largest challenges to overcome was coming home on oxygen. When it was finally time to leave the hospital, her lungs were still a little to immature for breathing 100% room air. Being at home with the oxygen never really became easy, but the challenges associated with it quickly became normal. At the house we had a 55 pound oxygen concentrator with a 25 foot line. Makenna was tethered to the concentrator which meant she could only travel a 25 foot radius around it. We placed it next to our bedroom door and that allowed her to reach most of the living room, the kitchen sink, and half of our bedroom. She couldn't go to her nursery or to the bathroom for baths unless we moved the concentrator, which didn't happen often. We also had to be very careful because as we maneuvered around, her line often got caught or wrapped around the furniture and that pulled on
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her face or made it easy for us to trip. Oxygen also meant that Makenna had to have her nasal cannulas on all the time. She was definitely use to having them on and most of the time we could get away with minimal tape; however, she learned that she could grab them and rip them off if she wanted to. We had many days that were spent constantly replacing and re-taping, which got really old. Oxygen also dried out her sinuses, made her congested, and the tape left irritated welts on her cheeks. But it gave her the added support to breathe with little to no struggle and it definitely gave us a piece of mind. I liked knowing that she has a little extra support while her lungs were getting stronger. Thankfully after 110 days of assisted breathing the oxygen was removed on 1/27!
Physical Therapy:
Not all CDH babies need physical therapy, but those on ECMO or with extended NICU stays might due to motor delays that require assistance. Makenna receives an hour of physical therapy a week and practices her therapy exercises with us several times a day. When she was on ECMO her head was turned to the side for a couple weeks. This caused her to develop something called torticollis in her neck. Torticollis basically means the muscles in her neck aren't equally developed, so her head tilts to the side and cannot be held straight at midline. As a result she still doesn't have good head control at almost 4 months old. She also has uneven tone in her arms and legs because she consistently favors one side over the other. This is because in the hospital 1 arm had her arterial line and a splint and one leg had a picc line, so she couldn't move them very easily.
Behavioral / Occupational Therapy:
Having been on ECMO puts her at risk for delays in cognitive ability. As a result she receives services through "early steps" for an hour a week to work on various age appropriate cognitive skills. This includes tracking objects with her eyes, reaching for things, and identifying which direction noises come from.
We spend a lot of time working on this stuff each day as well. So far (in my opinion) she isn't showing signs of cognitive delays, but she is definitely desensitized to certain noises and lights. She doesn't follow beeps or lights with her eyes...this could be a sign of delays, but I blame it more on being in the noisy and bright NICU for so long.
Manual Therapy:
Makenna had a tongue tie and lip tie that were causing difficulties nursing effectively and gave her tongue protrusion. It seems as though this is a common problem with CDH babies, possibly because both CDH and tongue ties are both midline defects. Who knows! We had them released with a laser, but for the next couple of weeks we have to massage her mouth a few times a day to keep it from reattaching. She is working with a lactation consultant on how to feed efficiently and visiting a chiropractor for bodywork. I'm not exactly sure what her chiropractor did; however, after her first visit Makenna did 10 minutes of tummy time without fussing once! Prior to her visit we could only get a few seconds before she would start screaming, so whatever she does works wonders.
Reflux:
Makenna was never given a GI test, so we don't know the extent of her reflux. I'm sure she has some reflux; however, from what we can tell it is being well controlled with medicine. There is a chance that her reflux is worse than we think and just isn't causing her to spit up. However, she is gaining weight, so thankfully she doesn't need the Nissen surgery or a gtube.
Medicine:
Makenna receives reglan, pepcid, lasix, and vitamins with iron everyday. Each medicine has it's own requirements (given 1, 2, or 3 times a day) and the vitamins have to be taken separately because they hurt her belly. As a result we have a strict medicine schedule we follow, which includes administering some medicine in the middle of the night. Thankfully we have begun the process of weaning off some of the medicines. It will be nice to have one less thing to worry about each day.
Head circumference:
As a result of ECMO we need to keep close tabs on her head circumference. ECMO can cause "water on the brain" (hydroencephalitis), so it is important to monitor her closely for possible signs. When we first brought her home this was done with weekly pediatrician visits for six weeks, but since her head growth has been consistently normal, we now get to measure her weekly at home.
Milk Soy Protein Intolerance:
Makenna has been fussy, gassy, and congested since we brought her home. She will wake up from a sound sleep screaming in obvious pain and she groans/grunts constantly. She also has mucousy stools with streaks of blood and an eczema type rash. After many pediatrician visits where her vitals were great and they said she was fine, we finally have a possible cause! It is most likely a milk soy protein intolerance induced by the quantity of antibiotics/medications she's been on since birth. It is likely that the antibiotics have striped the good bacteria from her system leaving her intestines inflamed. As a result her body is having trouble breaking down the protein in dairy and has begun to see milk protein as something it needs to protect her from, creating an intolerance. Since she is exclusively breastfed, I have eliminated all dairy from my diet to see if it helps. We are also goings to start giving her infant probiotics to help build back her the good bacteria in her gut. I hope this is the answer to helping her tummy troubles.
Meeting with Dr. Kays:
Every 6 months or so (eventually once a year until 18) we will go back to visit one of our favorite men. Dr. Kays and his team will check her vitals, track her weight gain, evaluate her meds, and look for signs of her hernia returning. Her most recent visit on 1/27th resulted in her being taken off of oxygen and starting to wean off of some medicine!
Staying Healthy:
The last, quite possibly worse, part of recovering from CDH is the isolation. It is vital that she does not get sick because her lungs aren't strong enough yet. This means she must stay home and away from people. We go to great lengths (and through a lot of hand sanitizer) to keep her healthy. Only our immediate family and her doctors/therapists are allow to see her until after flu/RSV season. They are all required to have the flu shot and pertussis shot and must be healthy to enter our home. Also all of her therapist comes to our house first each morning so they do not bring germs from other children they work with into our house. Makenna receives an RSV shot every month as well. This will hopefully lessen the severity if she were to somehow, dispite our efforts, contract RSV.
So there you have it... a look into our life after the CDH repair. Some days I feel as though I need a secretary to help me keep track of all her different appointments each week and medicines/exercises each day. It is a lot to keep track of, especially since she still needs to maintain normal "baby" experiences (I.e. playtime, walks, reading, and listening to music) as well. Thankfully I am blessed to be able to stay home with her. I know my continued devotion is what she needs to overcome these challenges.
Until she overcomes her challenges I can't help but celebrate every little thing she does and every milestone she reaches. When you almost lose your child you see them in a completely different light. I'm not saying I love her more than other moms love their babies, but I definitely feel that I love her a little bit differently.
CDH can't keep us down. My baby fought like a cherub and showed CDH that she's not to be messed with. Everyday I am in awe of her strength. Despite everything she always has a sweet smile on her face. I must admit seeing her strength gives me the strength to keep fighting.
Wow! 4 months already! This has been a month of incredible growth and development for Makenna. It seems as though every few days she learns a new skill and leaves Rex and I saying "Woah, did you see that!?!" She has now definitely transitioned from being a "newborn" to a "baby." It is so exciting to witness. For the longest time she was this small 6-9 pound little girl. She felt so fragile and lacked control of her movements. Now she is almost 12 pounds, feels so strong, and is very alert. She smiles a lot and even laughed for the first time this month (2/2). It was so adorable! She literally went from just smiling to belly laughing and giggling for 5 straight minutes! Since that day she will giggle every so often when we play peek-a-boo or if she just finds our words or actions silly. She also loves when we call her "miracle" and when I say "I love you." She gets huge smiles and starts to giggle as if she knows those words are more special than other words.
Typically I do not include medical stuff in my "all about Makenna" entries; however, this month we had a couple noteworthy things I feel compelled to include! For starters, we went to visit Dr. Kays on 1/27 for her first check-up. He said he was tired of seeing pictures of her on Facebook with the nasal cannulas, so he decided it was time to come off oxygen (Pretty sweet that he takes the time to follow his kids on Facebook!). Now that he took her off, it is so fun seeing her without oxygen and being able to have complete mobility for the first time in her life. It was also amazing to walk into the hotel that night and not get stares, pity, or comments of concern from everyone who happened to notice the giant 3 foot oxygen tank. For the first time she was just seen as a regular, healthy baby. What an accomplishment! We also took her to Jacksonville to have her tongue and lip ties released with a laser. She did fantastic during the procedure. We hope this will be the solution to her inefficiencies with nursing.
