Progress Report on the Princess
It's been a few months since I've updated the blog. If you're on Facebook, I give frequent updates about Elizabeth on her page, which is called Team Elizabeth Jeanne. Be sure to follow us on Facebook, and to share the page, and this blog, with your friends. We always welcome new members to our team.
Elizabeth has been relatively health over the past few months EXCEPT for a nasty set of recurrent ear infections. In fact, just yesterday I took her to the pediatrician. She has had a cold since Saturday, and it landed us in the ER with her at Children's on Sunday. She was having a lot of difficulty breathing, and her pulse oxygen kept dipping into the 80's and low 90's. I have oxygen here at home. I also have a cough assist machine, nebulizers, and inhalers, but she was scaring me, so we went to the ER. Fortunately, her lungs were clear. She just picked up another upper respiratory virus. Yesterday, she started with this tight cough, and I felt the doctor should listen to her lungs, etc. Well, her lungs sound clear, but her right ear in infected. This marks the fourth ear infection that she has had since mid-April. The oral antibiotics are proving ineffective, and they are wrecking her already poorly functioning GI tract. So, the doctor and I agreed yesterday that our next step were a dose of antibiotic via a shot. Elizabeth received a shot in each leg yesterday of an antibiotic, and she goes back in on Friday (tomorrow) for a follow-up and a second dose of the shots. Hopefully this will do the trick. I certainly was being given the princess stink eye while I was helping to hold her down for the shots, and for a while after while they watched her for a reaction. Thankfully, she was very cuddly last night and today, so I can say that she has forgiven me.
As for the ear infections, Elizabeth has struggled with ear infections all of her life, from the time she was an infant. Part of this could be just her immune system, and the fact that her brother also had frequent ear infections. They both had surgery to have ear tubes placed right around their first birthdays. However, Elizabeth's major contributing factors to the frequent ear infections lie in the anatomical issues caused by her sub-mucous cleft palate, and also due to her aspiration, weak and unproductive cough due to pulmonary issues, and the fact that she frequently gets upper respiratory infections that she cannot fight easily like you or I could do. She has been on three different oral regimens of antibiotics since mid-April, which caused rampant diarrhea, which caused terrible diaper rashes. She has been battling fevers with these ear infections, and she truly has just felt miserable with them. She has already had ear tubes placed in her ears on two separate occasions, but with the havoc the antibiotics wreak on her digestive system, we have to consider other options. When her ear tubes are in place, and are functioning properly, then she has little to no ear infections. The current set of tubes have been in quite some time. Actually, they have been in her ears longer than they are typically expected to last, so a switch for new tubes is long overdue. So, her ENT (Dr. Jabbour) is going to surgically place a new set of ear tubes on June 24th. We are hoping this puts an end to the ear infections for some time.
Elizabeth has been taking Reglan since December in an attempt to kickstart her gut into tolerating feeds again. The medicine is continuing to work. We know it can cause nasty side effects. It works in the brain as opposed to working in the gut. We are constantly watching her for the neurological side effects caused by the drug. She has been on the medication way longer than you're supposed to be on the drug. However, it has been working for her. I have my own pharmacological theory why it is working, and the doctor's believe my theory is plausible. I won't bore you with the details. Anyway, we have agreed to keep her on it, at least for a while longer. She currently is tolerating bolus G-Tube feeds. That means that she get 3 feeds per day delivered via a pump to her g-tube. These feeds are a special type of formula, and each feed lasts about 2.5 hours. Then, she gets a break between feeds. I have always felt that the formula that she is on is very harsh, and that she does not tolerate it super well. So, I spoke with the Intestinal Care (i-Care) team at CHP about this. I asked if I could move her to a blenderized diet now that she is tolerating bolus feeds. They agreed, and we have an upcoming appointment with Nutrition to enroll her in a clinical trial for a new type of pre-made blenderized formula. I can also prepare blenderized meals for her that will be administered via her g tube to help feed her. It is a much more natural way to feed and get food into her system than formula in a can. We are hoping the transition from blenderized to formula goes smoothly. She has been doing so much better from an intestinal standpoint, that we are only going to be seeing I-care every two months instead of monthly. Things have been looking up from a GI perspective, except the rampant diarrhea caused by the ear infections and oral antibiotics.
We have had over 35 appointments in the past two months. It's really quite crazy. All of the driving back and forth, the traffic, the diarrhea explosions in the car, the pump beeping when I'm rolling down the highway, all make the trips entertaining to say the least. Last week, we had appointments every day at Children's in Pittsburgh. Some weeks are like that. Some weeks we are lucky, and we have no appointments. Some weeks we think we won't have any appointments, and then something comes up and we have to get in to see this doctor or that doctor. It is very unpredictable, and VERY costly with gas. I drive a minivan. Those things are not fuel efficient. We are fortunate to have an excellent team of doctors and departments caring for our Elizabeth.
We have had follow ups in pulmonology, hematology, urology, ENT (which I already discussed), and added a new department Pediatric Gynecology. Yes, that's a thing. Dr. Chan, the Pediatric Gynecologist, is amazing and was lucky doctor #13 to be added to Elizabeth's team.
As for pulmonology, things are remaining the same. They advised not pulling her off of her inhalers at this time. She's only a few months out from being cleared to eat by mouth again. She has days when she eats better than others, and she still struggles with many issues with eating. So, it is still entirely possible that on those days when she is struggling with eating that she is aspirating. The inhalers help to keep her airway flowing nicely. She has had fewer respiratory infections than in past periods, which is great. At some point, they want to do a bronchoscopy to get a baseline on the health of her lungs. They advised to to do cough assist whenever she is sick, and even after she eats when she is healthy to help her to ensure that she clears her airway. The honest and truth is that she has a neuro-muscular disease that is progressive and it progressively impacts lung function. So, we may be able to some day pull her inhalers for a while, or not have to have oxygen and cough assist on stand by, and we also may never get to that point. We also may need to up our game on respiratory assistance for her at some point. There's no way to predict, so we just watch her, closely. We monitor her pulse oxygen several times a day. We keep track of trends in her pulse oxygen. We check blood work frequently that tells us how healthy her lungs are and how well they are working. Right now, that is all we can do, so we do it.
Hematology feels that her iron deficiency anemia is going to be resolved now that she is tolerating g-tube feeds. Now her body has extra pathways to absorb iron as food enters her body than it did before when she was being fed directly into her intestine. However, she has some other issues with red blood cell size, wavering hemoglobin counts, and has needed blood transfusions in the past. They still suspect she could have a bleed that they have not found. They also suspect that her genetic disorders could be at play with her blood issues. At this time, we are not going to do anything but watch her biweekly lab work, and we will follow with hematology every 4-5 months.
Urology is also keeping status quo with their care of Elizabeth. It has been suspected since January that she has a rectovaginal fistula. Now, a popular theory is that this fistula is the culprit behind her frequent UTIs, and played more of a role in her frequent UTIs than her mild level of bladder reflux. Despite having several imaging and scoping studies done, we have not seen the fistula or found it's exact location. However, since starting the Bactrim that Urology put Elizabeth on for the frequent UTIs, she has not had a UTI. It is also interesting to note that the Bactrim would kill the same bacteria that would cause UTIs because of the fistula. So, we are keeping her on the Bactrim until we find the fistula, or we feel she is reasonably healthy enough that the UTIs won't throw her over the edge in regards to health.
We recently added Dr. Chan from Pediatric Gynecology to our team to help us find the fistula. She is amazing, and I am so glad that she was able to see Elizabeth. She suggested a few tests that we have not done yet. One of the tests she is going to perform while Elizabeth is under for her ear tube surgery on the 24th. We are still waiting to hear when the second test she suggested can be scheduled. These recto-vaginal fistulas can be very hard to find, but we are hopeful that one day we will figure out why this problem is ongoing.
In other news, Elizabeth started school at the Western PA school for Blind Children on her birthday, May 10th. She finished up on June 3rd, and she will attend for two weeks in July as part of an extended school year program. Then, she will start school again full time at the end of August. She really loves it, and the staff and her teacher are amazing. Elizabeth has done amazing things in the few weeks she was there. For example, she stood several times on her own. It is an hour and fifteen minute bus ride from home each way, but she has really done well so far. It broke my heart quite a bit to put her on the bus, but that's the way it goes. I'm letting her spread her wings, show us all what she can do, and we have been more than impressed so far.
Also, Elizabeth was granted a wish through Make a Wish. They are sending our little family to Disney in July, and we are all so excited. I know that she will love it. She and her brother are both HUGE Disney Fans. We even had a few special Disney character guests show up at their birthday party. Elizabeth was totally captivated by Elsa, and Celtan was super excited that Darth Vadar and Batman showed up. It was a wonderful day. I cannot thank my friend, Beth Taylor Ackelson, enough for putting that together, and the students who took time out of their day to dress up to make their birthday party so special also receive our deepest thanks.
Thank you for all of the love, prayers and support. It truly means so very much to us. Thank you for being a part of Team Elizabeth Jeanne.
