Her Story (Part 3)

We brought our sweet baby girl home and it was scary. Like most parents we listened intently to her breathe but our senses were heightened even more. We really had no idea what was “wrong” with her so we didn’t know what to expect. Of course, we knew some of the more obvious issues but not everything. I can’t tell you how much time I have put into trying to decide exactly how to write this portion. I tried writing it in chronological order and it skipped so much and was overwhelming to try to keep in order so then I decided to just take it one issue at a time. Basically the genetics and chromosome testing came back normal. Normal meaning that they did not see a specific problem with her genes or chromosomes and that her defects did not fit a certain pattern. It was a long wait and I can remember exactly where I was in our house when I got the phone call with the results. I was relieved yet I remember saying, “so what now?” If we don’t have a name for this then how do we know what to expect. I learned a lot about syndromes and disorders. The geneticists look at a set of birth defects and say whether or not they fit a certain pattern (named syndrome or disorder). If they do, you are diagnosed as having that if not then it’s a matter of whether your case is interesting enough to study. They told us that one day there might be a Sallie syndrome. Different doctors have speculated on what they think she might have but no real conclusions have been made. When she was younger we felt after a while that some of them were just enticed by the fact that it was a challenge to find out “what was wrong.” We asked if there was anything that could be done to help her with all the syndromes that were mentioned and we were told no so we said leave her alone. Our ENT who we love dearly and we know has Sallie’s best interest at heart feels that genetics might be able to find something now. He said that sometimes as children get older disorders can be more easily identified and therefore we might know through that how to best help her. We talked with our pediatrician who is really a no nonsense kind of man and we knew he would not want us to do something with her that would not possibly benefit all of us and he agree with our ENT. So, all of that to say we go back to genetics in a couple of weeks. I can’t tell you what this means for me. I will do anything in this world for her but I got so disgusted with the whole genetics deal that I thought I would never go back but we have the appointment now and we are going. We may not learn anything new and that is fine and if we do then that is great as well. Please pray for me. The closer this gets the more scared I get. I get lots of thoughts in my head about what we may find out and I need peace about this. So, since we don’t have a name for what is “wrong” she is labeled as having isolated birth defects. They are all midline (meaning midline of the body) but that still really does not tell us anything. When I was pregnant with Lizzie my OB/GYN sent me to meet with genetics because of Sallie (you can imagine how thrilled I was). We went over everything and she mentioned that the problem could have been that my cord with Sallie was only 2 vessels and it should be 3 and that possibly the defects were caused because there was not enough blood supply to her at the time those particular organs affected were being formed. However, she is the only professional we have talked to with this opinion so… Back to the isolated birth defects.

1) Hypothyroidism: At 7 weeks blood work confirmed that she did have this. She was put on medicine and her levels were monitored closely to adjust dosages for the first 3 years at which point the endocrinologist released us back to our pediatrician for him to monitor. At age 4 her levels tested normal and we took her off the medication. We have lab requests now for them to check her levels. We will always keep a check on them to ensure they remain in the normal range.

2) Unilateral Kidney: Her one kidney functions fine and we try to do everything possible to protect it. In the past couple of years she has had some problems with UTI’s and within the past year we discovered through testing that she does have some urinary reflux but it is not major. Once again, it just has to be monitored.

3) Bi-cuspid Aortic Valve: The aortic valve should be tri-cuspid (meaning 3 branches) and Sallie’s only has 2 branches. This was monitored very closely for a while. She was also born with PDA and a heart murmur both of which corrected within the first year of her life. As for the bi-cuspid aortic valve we have been told that she should not have any problems with it until possibly when she is older and her heart is having to work harder and this issue causes it to have to work even harder. She does have to have antibiotics before she has any procedure done to prevent infection in the heart. Sallie’s first surgery was at 4 months old and because of her heart and other issues we were told that she was in the highest risk category for anesthesia. The day they took her from me was for sure the most scared I have ever been. Anesthesia would not take her in until her cardiologist came to the room, did an ultrasound and said she was good to go. Handing your baby over to strangers is an incredibly tough thing to do but really tough when they are making sure you understand that she is at a great risk. Praise God she has always done remarkably well with anesthesia. The only problems we have had is stabilizing her oxygen levels after surgeries and this is mostly due to the fact that she gets so upset until they get her back to us and then her levels get all messed up and she will generally have to have some breathing treatments to get her back to normal. We are quick to tell them to get us to her as quickly as possible and we will all be better off and we have been very fortunate that they listen to us and get us back there so we are with her when she wakes us. We are currently waiting on an appt. with a cardiologist here just to have her heart checked and make sure things are still the same. They are waiting on her complete history before scheduling the appointment. We are professional “waiters”.

4) Cleft palate: We went to what is called cleft clinic a lot. We would go over feeding and the plastic surgeon would monitor the palate to decide the exact time it needed to be repaired. Let me add here the difference between cleft palate and cleft lip. Cleft palate is a whole in the top of the mouth. It can be of the hard palate or the soft palate or both. Sallie’s was of both. Cleft lip is the slit or whole that is seen on the lip or outside of the mouth. Sallie’s was only palate, not lip. You could not look at her unless you were looking inside her mouth and know she had a cleft palate. Feeding was a major issue for her. Like I mentioned before we literally forced every drop of formula into her. It was awful! Some cleft babies do not have tons of feeding issues but Sallie also had major reflux and the combination was not a good one. Most do not do well with baby food but she did much better with baby food than liquids. Her palate surgery was at 9 months. She had already had several surgeries but this was one of the longest and worst. Her tongue was stitched to her mouth for a while which was completely frustrating for her and us. She healed nicely and fought well though (in true Sallie fashion!) I feel I am saying this about each issue but we are also waiting to see a plastic surgeon here because there is a possibility she will have to have more palate surgeries. Our ENT wants us to see who he thinks is the best around so he is trying to get her in sooner as opposed to later. We should hear back in the next day or so. We have to ensure that her palate is in the best possible condition for speech. We are unsure how much they can do but we want to do all we can. Once again, please pray.

5) EARS! I take a deep breath because this could possibly take the rest of my life! At 2 weeks old Sallie developed her first ear infection and except for a few days in between had one non-stop until she was about 4 and then off and on for another year or so. It would be bacterial and then we would do antibiotics trying to combat the bacteria until we had yeast (in the ear) then we would be fighting that. We did lots of injections for the infections. We went into the hospital on several occasions for irrigation a few times a day and to do IV antibiotics. At one point they put in a central line and we did meds at home 2 or 3 times a day. I look back at that and it really seems like a dream. She was 2 and had to sit still for hours for us to run the drugs several times a day. We started out at 3 times a day meaning we had to get her up in the middle of the night to run them. The good Lord, that is all I can say. It was not us, because we don’t have that kind of stamina. While on the first antibiotic through the central line she had an allergic reaction. So scary! Our Dr was 2.5 hours away and we drove over 100 mph the whole way to the ER. We stayed on the phone with them most of the way there. Thankfully her breathing remained OK. We changed meds and continued through the line. Her first set of tubes was at 4 months. I can’t tell you how old she was when she got the next 7 sets of tubes. The day they did the first set of tubes (4 months) they also did what is called an ABR (Auditory Brainstem Response). It is a more specialized hearing test which is done while they are under anesthesia. It measures brain wave activity in the auditory centers of the brain. Even though we had already been through things I never dreamed I would I just had convinced myself that she was not hearing impaired. Why do we do that? Why do we tell ourselves those things happen to other people? I admit that because I noticed that she did not respond I would do things when we were at home alone to try and get her to respond. I remember dropping cookie sheets on the tile floor in our kitchen to try and get a response. However, I still thought it was the amount of fluid on her ears and refused to believe she was hearing impaired. The audiologist (John Wright-love that man) returned to our room that day after doing the test to tell us that she was indeed hearing impaired and everything after that was pretty much a blur. Dr. Hill (our ENT who we also think so much of) came in there with him to answer any questions and for comfort. I really did not absorb anything they said that day. He told us to let it all sink in and to call him the following week. I called him and we went back over everything and he said you do know that insurance doesn’t cover any of this. What? My child was born hearing impaired and insurance is not going to cover it. I could write a book on this topic alone but I am going to move on now. Really, you want me to move on! We made our first appointment with John and went in to be fitted for aids. Hearing aids on a 4 month old-a teething 4 month old. They stayed in her mouth much more than her ears. Talk about getting people’s attention. That’s another topic in itself as well! Because of the horrendous infections described earlier she could not wear her aids most of the time. The aids made the infection worse. We knew we had to get the aids on her consistently. She is not a candidate for cochlear implants because of the type hearing loss she has. She is a candidate for a device known as a BAHA but insurance will not cover the cost of the device or the 2 surgeries that are required to install it. However, we know we serve a God that will somehow make it possible for her to have this if we at some point determine that is what is needed. She is doing well with her aids now so we are continuing with them. At some point in battling the infections, the Dr found through a CT scan that the infection had actually gotten into her mastoid area. This is the area behind your ear and next to your skull. The only way to get to this area is to open up the back of the ear and go in. So, we went in for surgery for him to pack this area with antibiotic saturated gauze to try and get rid of the infection. Once Dr. Hill got in there he realized that not only was there infection in there but there was also something called cholesteatoma which is benign skin growth. However, left untreated it would affect the facial nerves and eventually the brain. He removed everything he could that day. He told us that if even one cell of it was left he would have to go back in. He went back in 7 more times. These were very long surgeries. Anywhere from 4-6 hours. He had to be very careful to not cause any further damage and it is in a very risky location because of all the nerves. He was trying to prevent her from having what is called a radical mastoidectomy. One of my biggest concerns in moving here was the relationship that we had with Sallie’s doctors. They loved her and us and they knew where we had been and the thought of starting over scared me a lot. We first went to an ENT here who specialized more in sinus issues. Sallie did have sinus surgery at age 2. Her sinus cavity was completely impacted. She still has some sinus/allergy issues but that surgery helped her so much. So, we really did not need him. However, he knew just who we did need. The Dr to see-Dr. Bauer. He is wonderful! After seeing him for a while he told us it was time to go back in and see what was there. He agreed that it was best to avoid the mastoidectomy if possible. He went in and cleaned out as we had in the past. The next time we went in to have this done, she was not in for long and they called us to tell us to meet Dr. Bauer in the consultation room. Once again, scared to death. We have no family there with us. He told us that he could try removing the growths again but that it was much worse than he thought and we needed to make a decision. We trusted him and we asked him what he would do if it were his child and he said as hard as it would be I would do the mastoidectomy and with that we told him to take everything out of her ears. The open up the canal really wide and take it all out. Wow, not an easy thing to do. I am not sure that Aaron and I were able to even talk about it for several days. When we took her back for post-ops we set a date 6 months later to go into the other ear and if that ear was the same that he would perform the mastoidectomy on that ear as well. I have to stop now and say I am so thankful to be a child of God. I am writing all this and I am re-living all this and we made it through all this not because of our strength because I can assure you we are not that strong but because of HIM and one day HE will make her whole and for now she is the way He intended her to be and we accept that and praise Him for choosing us to be parents of such a special little girl. As much as I wish I could, I can’t but I know who can and I am so thankful for that. There are lots of things in this world that we can handle and we can make right and I am one of those who tends to think I can do it and God has taught me that I can’t fix it all. I have to trust in Him! I have to rely on Him! So, she goes back in 6 months later and he does the mastoidectomy on that ear as well. We really felt that all our years of battling that were in vain but we do not feel that way anymore. She has not had one infection since that last surgery! Praise God! Now we go in every 6 months to a year to have her ears suctioned while she is under anesthesia because the canal is open and there is nothing in there and everything gets in there. All of this is why she has never been able to wear her aids consistently and therefore develop speech. It takes a child hearing at a normal level 9 months to a year to speak their first word (of course we know this varies with all children) and then you factor in the amount of effort it takes to hear with hearing aids and the fact that it is not as clear as what we hear and it takes a while to develop speech. Also, as I mentioned she has the cleft issue which also hinders speech. Where are we now? We are trudging along at a very slow pace and hoping for progress. We are anxious to see what the plastic surgeon says about her cleft because it is so critical in speech. Also, along the way she had some reconstructive surgeries done to the outside of her ears. This was in order for her aids to fit properly. Because of the amount of surgeries she had following that one of the ears is was messed up again so we are going back to another plastic surgeon to take care of that. This will not be the same one as the one who treats her cleft because they all have different specialties.

So, we wait and see what the future holds. Sallie is so strong. She may be little but she is a fighter and she is mine and I love her just the way she is! She has taught me so much!

Still to come-“Things we have learned” and “Things we wish we could change”