The lungs are healing!

December has started out on a good note. Let's hope we can keep it going! Since Charlotte has been home from the hospital, she has been doing really well! She is 14 lbs now and gaining weight really well. We had a follow up with her GI doctor, and she is really impressed about how well Charlotte is doing with the tube placed back in her stomach. Because Charlotte is continuously fed for 24 hours, she has an overgrowth of bacteria in her belly due to the high PH level, and so the GI doctor has placed her on a medication called Flagyl to help her stomach get rid of all the bacteria until she can tolerate normal, large volume meals every three hours. It is not erythromycin, and it seems to be working well! We will start compressing her meals down slowly until she tolerates a large meal every three hours. We also had a follow up with her lung doctor, and they have decided to allow Charlotte to take her oxygen off for 4 hours at a time during the day! This is a huge burden lifted because now we don't have to lug the oxygen tank with us to doctors appointments and on walks. Less tubes equals more freedom. Charlotte looks so much healthier and older without the breathing tube on her face. Also, the lung doctor decided that Charlotte does not need to take her diuretic medicine anymore. This medicine was helping Charlotte get rid of all the fluid in her body that was staying in her heart and lungs. Now that her heart and lungs have healed, they don't see any fluid at all. Diuretics can cause stomach cramping, abdominal pain, and vomiting, so this is another step in the right direction for her stomach!  Charlottes red blood cell count is also improving. Anemia (low red blood cell counts) can happen for a long time when a baby is born prematurely. Now that Charlotte is older, she is making red blood cells well, and she hasn't required any red blood cell transfusions. She had her 9 month pediatrician follow up, and the pediatrician was so impressed about how well she's doing. In other exciting news, Charlotte was able to meet Santa for the first time! Santa was visiting the Baltimore Inner Harbor, and she got her picture taken with Santa and her family. 

Erythromycin: the devil's drug

     What a horrible month. November was possibly the most trying month we have had since Charlotte came home from the NICU. We spent most of it, including Thanksgiving, in the hospital. Her vomiting continued to worsen, and we decided to drive all the way to the Children's Hospital of Philadelphia ER to get a second opinion. They performed several tests and could not find a reason for the vomiting either. So, back at square one, we decided to stop one of the medicines that Charlotte takes for motility of her gut: erythromycin. This medicine was supposed to be used to help Charotte's bowels work more efficiently. Premature children often have a diagnosis called "delayed gastric emptying" because their bowels don't work as well as a full term baby. A gastric emptying test is normally performed to confirm whether a baby has this diagnosis or not. There had never been a study done that showed that Charlotte had delayed gastric emptying at all. We had spoken to our GI physician at Maryland about stopping the medicine a few weeks ago because we suspected it was making Charlotte sick to her stomach. We tried to stop the medicine for a day, but Charlotte continued to vomit. After speaking with Laura's mom who is a nurse, she advised that the medicine does often make people sick to their stomach and it needs to be stopped for several days in order for it to truly be expelled from the body completely. So, we stopped the medicine for several days, and the vomiting lessened. Also during this time, Charlotte was found to have severe anemia which means her red blood cell count was very low. A normal level is 10-12. Charlotte was 4.3. The hematologists suspected a GI bleed. During the hospital stays, they scoped Charlotte's entire GI tract to see if they could find a source for bleeding. They did not find an active bleed, but they think that there had been a bleed somewhere that resolved itself because they did find blood in Charlotte's stool. So, Charlotte received several units of blood, and ever since, her red blood cell count has been stable. Because her GI issues have been improving, they moved her GJ tube back to a g-tube which means she is now fed into her stomach. Next Monday we will start to compress her feedings into bolus feeds which means she will get her food in shorter periods of time and she will be able to be off of the g tube!
     During this time period our kidney doctor attempted to fix her kidney reflux with a surgery. They were unable to do the surgery because her urethra was too small to enter with a scope. Therefore, they left a catheter in her in order to stretch her urethra. Well, Charlotte poops all the time because she is fed continuously by a g-tube. Therefore, the catheter was constantly contaminated and caused an infection, and we went back to the hospital for more antibiotics.
     The hematologists also performed a bone marrow aspiration to see how Charlotte's bone marrow is working. They found that her red and white blood cell lines are working normally. She continues to require transfusions for her platelets.
     Charlotte had her sleep study and is able to use her oxygen at 1/4 liter at night which is half of what we have been normally using. We see the pulmonologist next week. Hopefully they will let Charlotte take off the oxygen for good during the day.
      The good news is that for the past two weeks, Charlotte has not vomited at all. Also, we have made another huge change. One of Laura's managers at the nursing homes decided to generously donate her breast milk to us. Laura was unable to produce after 3 months due to worsening depression from Charlotte's illnesses. Since stopping the erythromycin and using breast milk instead of formula, Charlotte has had a huge turn around. She is able to do tummy time more often. She is able to sit better because she can put pressure on her belly. She cries less. It has been amazing. Hopefully her GI problems continue to steadily improve. November has been a long month for the Harris family. Still, Charlotte is 13 lbs 4 ounces and gaining steadily! Here's hoping December is a better month!

Pneumonia

     Well, that about sums it up. For the past month, we have been in the hospital. That is why we have not had any time to update everyone on how Charlotte is doing. After a long stretch of Charlotte's health improving, she began vomiting daily. We assumed she caught a little virus and didn't think much of it at first. However, Charlotte was due for her 6 month vaccines. She did not tolerate her vaccines well. They literally blew her vomiting out of the water. She vomited for days, and eventually swallowed it into her lungs. So, she was hospitalized. Charlotte has a complicated medical history right now due to being born so prematurely, and so when we enter the hospital, it is always a complicated process. They draw blood labs, and Charlotte doesn't produce red blood cells well. So, they sometimes have to transfuse her to maintain her blood counts. She also requires weekly platelet transfusions. Well, one day while in the hospital they gave her red blood cells and platelets on the same day but did not give her a diuretic due to her low potassium from vomiting. This caused Charlotte to swell up with all the fluid, and her lungs partially collapsed due to the pressure. David and I left for a half hour to have dinner across the street and came back to see doctors surrounding our daughter who was unresponsive because she was struggling so hard to breathe. To watch your daughter swollen from head to toe and unresponsive to your touch or sound as she struggles to breathe is something that David and I hope to never see again. The feelings we went through are indescribable. They included rage, sadness, fear, exhaustion, anxiety, etc. (We had a long talk with the doctors about how they screwed up big time, we knew it, and assured them that if anything else happened we will take action. Laura's whole family is in the medical field and are well aware of proper medical practices.) So, she was sent to the pediatric intensive care unit and placed on 8L of oxygen. They considered placing her on a CPAP machine, but she was able to tolerate a regular nasal cannula. Thank you, God. We spent several sleepless nights in the PICU where they have one awful recliner in which to "sleep". So, David and I took turns going back and forth to the hospital. Charlotte was able to wean back down to her normal oxygen requirement. The medications cured the pneumonia, removed the fluid, and healed her lungs. After returning home, in less than 12 hours she was vomiting again and back in the hospital for the same thing. We spent another week in the hospital. When we came home again, we couldn't lay Charlotte flat because when she sat back up, she would vomit. So, we had to resort to changing her while sitting up. Changing a child while sitting is something I wish no parent would ever have to do. It's messy and exhausting. Well, then Charlotte got a UTI. One of Charlotte's many diagnoses is called VUR. Vesicoureteral reflux is the backward flow of urine from the bladder into the kidneys. Normally, urine flows from the kidneys through the ureters to the bladder. The muscles of the bladder and ureters, along with the pressure of urine in the bladder, prevent urine from flowing backward through the ureters. VUR allows bacteria, which may be present in the urine in the bladder, to reach the kidneys. This can lead to kidney infection. Upon arriving home from treatment for the UTI, Charlotte got pneumonia again. During our admission, Charlotte had her G tube changed to a J tube in order to stop the aspiration events. This means that the food Charlotte gets through her tube will go into her intestine and be absorbed and not her stomach. Therefore, if she throws up, it will not be formula because there is nothing going into her stomach. This will prevent her lungs from becoming infected. Our lives have consisted of daily phone calls with endless amounts of doctors trying to figure out why she is vomiting, what medications she does or does not need, which medications we can change to improve her vomiting, setting up follow up doctors appointments, etc. 

     Some good things did happen after our hospital stays. They decided they are going to do a simple outpatient procedure to fix Charlotte's kidney reflux which will cure her VUR. (They hadn't intervened before the UTI because they thought her VUR was mild enough to not cause any problems. After we left the NICU, they had recommended that we use an antibiotic for 1 year to prevent UTIs.) After the surgery, Charlotte will no longer need to be on an antibiotic to prevent UTIs. Antibiotics can cause stomach problems, and so we are happy that she will most likely not need them if the surgery is successful. Also, Charlotte no longer has hypoaldosteronism. This is a long word which means her adrenal glands didn't produce a certain hormone. She was on a steroid to help with this diagnosis. Well, as she has grown her adrenal glands have healed and work just fine now. So, we have stopped her steroid!! One more medication is gone! Steroids can cause stomach problems also, and so this means we are heading in another good direction for her stomach!

     Charlotte is 12 and a half pounds now! She is almost 9 months old, 6 adjusted to her actual due date. She has a jumperoo that she loves to play in. At first her feet didn't touch the floor, but she has grown into it, and now she loves to jump up and down in it! Also, Charlotte loves standing up and is able to sit on her own by propping herself with her arms. Developmentally she is right on track. We have decided to let her enter her first Spartan kids race soon.

     The experiences that David and I have been through have caused us to truly cherish every day with our daughter in a way that many parents can not understand. We have had so many days where we feel like we have almost lost her. Each time she recovers and gives us that big Charlotte grin for the first time, we take a huge sigh of relief and hold on to her a little tighter. We love her a little harder. The big life lesson we have leaned is: Tomorrow is never guaranteed. We must cherish every day and live it to the fullest!

Children's Hospital of Philadelphia

     We have finally chosen the hospital where Charlotte will receive her transplant. After visiting and speaking with multiple hospitals about their transplant programs, we have decided to go to the Children's Hospital of Philadelphia (CHOP). This hospital is top rated in the country, and they see people from all over the world. You can see this displayed as soon as you enter the hospital. It is a gigantic mecca of children's health care filled with people of all colors and races. The doctor who will be handling Charlotte's case is both a bone marrow failure specialist as well as a transplant doctor. It is very rare to find a doctor who knows both the genetic and the treatment side of these diseases. He is the only doctor we have spoken with who has seen cases similar to Charlotte. He has treated several cases of CAMT and he stated, "only 50% had the MPL gene mutation that has been known to cause the disease." Charlotte does not have the typical MPL gene mutation that causes CAMT. This is why we are continuing to search for the cause of Charlotte's disease with genetic testing in order to help others in the future that may have the same genetic link. Research is the key! (God bless the people who area behind the scenes of our journey trying to help us find the genetic link. This information will help David and I in future pregnancies.) CHOP completes 100 bone marrow transplants a year which is far more than any other hospital. They have treated several cases of CAMT (Charlotte's diagnosis.) Dr. Olson, Charlotte's transplant doctor, was unbelievable regarding the amount of knowledge he has in the area of bone marrow failure syndromes. He is very proactive and began the HLA typing process as soon as we met with him. He explained that securing a bone marrow donor can take months, and it is imperative to begin the process, so that when it is time to complete the transplant, the donor is ready to go. He also drew preliminary blood work to check the function of Charlotte's liver and kidneys. The doctors want Charlotte to be bigger and healthier in order to give her organs the best chance of tolerating the transplant process. So, we will do many tests prior to transplant in order to ensure that she is healthy and ready to go.
     In other exciting news, Charlotte had her first follow up appointment with the pulmonologist. She passed with flying colors!!! This means that she had a monitor on her foot that looked at how she was breathing on room air for 15 minutes. She was 99% the entire time. So, 1) See ya later albuterol inhaler! We are down to only 1! 2) She can take the oxygen tube off for 30 minutes several times a day. For example, we can bathe her and take pictures without the oxygen 3) she will now have a repeat sleep study to see exactly how she is breathing for a long period of time. This will tell us how long she can be of of oxygen. (Hopefully its for good!) This should be in the next 2 weeks.
     In the weight gain department, Charlotte is steadily climbing the charts. She is 11 lbs now!  The nutritionist  had to increase Charlotte's calories because she is getting so big! All in all, Charlotte's health is rapidly improving these days, and we are starting to get rid of medications. Big changes are coming our way. It's a stronger and healthier Charlotte Harris!

Swallowing!

The past few days have brought lots of firsts for Charlotte! David turned 36, and so for his birthday we celebrated by going to the Baltimore Aquarium. Laura pushed the stroller while David carried Charlotte in a baby carrier for the first time. This has been a dream of David's since she was born. He loved every minute of it! Charlotte loved being able to look at all the brightly colored animals. Her eyes were as wide as saucers the entire time. Our favorite part of the day was the dolphin show. It is great to see how much being around other people and environments has brought out Charlotte's speech. She is talking up a storm like she is one of the girls when she is around Laura's friends. We also made our first trip to Nana's house in Delaware. This is the longest trip Charlotte has ever taken. She got to spend the day with her cousins Emmy and Logan and her Uncle Dennis and Aunt Erin. She loved watching Emmy do gymnastics. Logan played music on his tape player for her. They will be great babysitters when Charlotte is older. It has been about 14 weeks since Charlotte had her last swallow study. She had her swallow study at Mount Washington Pediatric Hospital, and she passed like a champion! She will be allowed to have small amounts of thickened food by mouth as tolerated. She has difficulty with using her tongue since she has not been eating for so long. So, an occupational therapist to is going to work on helping her coordinate her oral motor skills. Charlotte loves to sit in her big girl high chair to play at the table now. So, it on't be long until she will be sitting up to the dinner table with us for meals! Charlotte is 10 and a half pounds now. We are almost on the charts for weight according to her adjusted age! We've come a long way from 1.5 pounds. We visited and toured our first bone marrow transplant clinic, DC Children's Hospital, and we loved it. Charlote started talking during our meeting and had lot to add at our conversation. She is already going to be an integral part of her healthcare decisions! We will also be visiting The Children's Hospital of Philadelphia this coming Tuesday and Johns Hopkins on Thursday. Then, we will decide where Charlotte will have her bone marrow transplant. This hospital will do the typing to find her bone marrow donor match. Because she is young and caucasian, Charlotte has a great chance of finding a perfect match. If she was not fully Caucasian, it would be more difficult. That is why it is so important for individuals of all races and backgrounds to register to be a bone marrow donor. This will give others a better chance of having a life saving match. All in all, its been a wonderful few weeks!

Maternity leave

Charlotte is 6 months now! The time is just flying by now that we are busy bees at home. The past few days have been very eventful. After attempting to take Charlotte out to experience the world, she caught a stomach virus. So, we were in the hospital 3 and a half days. Now we are back to square one with being cautious and keeping Charlotte as healthy as possible. Her room has lots to do and play with, so we will just stick to her room for now. Charlotte loves to explore and play on her playmat. She loves to "talk", roll around, and has recently found her own reflection! Laura has finally started her maternity leave, and David has started back to school. Laura is loving being home with Charlotte every day. It's like having your own personal baby doll that you can dress up and play with whenever you want! They have a great time together. Charlotte has a closet full of fun clothes that Laura bought her as a present to start the "Mommy daughter days." Charlotte is sleeping through the night which makes the days much more fun for Charlotte and Laura because they are both well rested. Charlotte has continuous g-tube feeds until she can swallow by mouth, so she doesn't get hungry at night. We will have our swallow study soon because the speech therapist recommended a follow up swallow study in 12 weeks. Well, the 12th week has arrived. Prayers would be much appreciated because we would love Charlotte to take food by mouth soon. Our household is busy with home health PT, home health OT, home health nursing, weekly platelet transfusions, and frequent other speciality appointments every week. All in all, she is a growing, happy little peanut and we love her to the moon and back. Every day is a new adventure!