Dr. Linda Rozell-Shannon, PhD President and Founder
07/02/2007 Ava was 4 weeks early today born at 5 lbs 1 oz, 17 and ¼ inches long. She has a large bruise on the crown of her head, probably from sitting on my pelvic bone so long. She also has a bruise on her right arm. Extends from shoulder to inside elbow. Drs said these would go away in a few days. She looks just like her daddy and she’s so small.
08/02/2007 Ava’s right arm has now turned a reddish color. Dr said it is a birthmark or stork bite. No need to worry. Should go away as she gets older.
09/02/2007 Ava’s arm is now bright red and raised slightly. We found a small red spot on the back of her neck. Thought it was a mark from a button on clothing. Dr looked over and doesn’t seem to be worried.
10/02/2007 The birthmark is considerably larger now, and at bath time one night, noticed that inside the crease of the birthmark has turned raw and filled with puss. Dr has referred us to hematologist. Dr Bayliff gave us antibiotic ointment and a topical cream and we are supposed to wrap it after applying cream. It’s very tender to Ava. Shirts no longer fit for her age and we have to put 2t shirts on her. Large veins in her right chest run to this arm and seem to be feeding it.
11/02/2007 Getting even larger now, so large that every time one ulceration heals, another comes up. Still doing the cream and wrapping. Very frustrating getting her dressed. It’s very tender to touch. The one on the back of her neck is getting larger too. About the size of a nickel. Dr Bayliff just wants to watch it to see what it will do. The color is not as red; there are a few grey spots, which indicate involution. Doesn’t want to do any surgery or laser. Not until closer to school age.
12/02/2007 The hemangioma has grown too much. Now starting to creep onto the inside of her arm and so large that it’s tight and looks as though it’s going to burst. Dr Bayliff has started her on 1 and ½ lm of prednizoloan twice a day to see if we can reduce or stop the growth. Buying clothing is frustrating, nothing seems to fit unless its 3 sizes too big. She wears her brother’s shirts a lot. 2t! Cant handle the meds twice a day, vomits a lot and makes her gag, bringing it to just once a day. I jumped on a website called the vascular birthmark foundation and have talked to several mothers about Ava’s arm. Was contacted by Sherri Foster who wants to send her pictures to a surgeon in NY. Dr Levi tin has emailed me to let me know that he feels that she needs to have surgery. The first one will be laser to close the ulcerations and then two more to de bulk.
01/02/2008 She’s even more moody now than before, which is hard to believe. Her appetite has actually decreased instead of increasing. The steroids are making us all crazy! Her ulcerations have healed!!!!! Knock on wood that they don’t come back. Dr Bayliff doesn’t think that surgery would be good for her right now. Not enough good skin to close the arm back up if it’s removed now. We are decreasing the amount of steroids to 1ml a day now and the color closer to the elbow has almost turned all fleshy. The hemangioma is a lot squishier now. And doesn’t seem to bother her as much when you touch it.
01/07/08 Dr Levitin sent me an email today to let me know that he had talked Ava’s case over with Dr Waner and that they both feel that since her arm has healed that surgery will start around 1 year of age. A little disappointed that this is not happening sooner, but he knows best. Seems like an emotional rollercoaster. Sherri foster advised me that his nurse would be calling me soon with more details of why they want to wait.
01/09/08 Pediatrician appointment today. Dr Dearinger is pleased with Ava’s weight and development. No live vaccines today since she’s on the steroids.
01/12/08 Dr Levitins nurse called today. Jana. She explained to me in detail why they wanted to wait until she was older to do the surgery. Now that her ulcerations have healed, she is in no pain, and there is not enough good skin to close up the incision right now if one was made. Waiting in hopes that the involution process will take place and there will be more good skin to use at that time. She has suggested and EKG since her heart may be working harder to produce the blood to supply the h. we will talk to Dr Bayliff.
01/16/08 Meeting with Dr Bayliff today went well. She feels that her arm has not grown in girth, but that she could tell that the color has started to turn more red, and that the puffiness has gotten bigger. The h (hemangioma) is not as flat as it was when we were on full steroids. She has decided to put her back on 1 and a half ml once a day until we go back on the 31st. we have agreed that I will keep pictures each week and email them to her to let her know if its going back down. If it is then we can more slowly than the last time bring her down. She thinks that maybe we cut the dosage too much each time that regressing more slowly will keep it from rebounding again. She has also suggested First Steps to evaluate her to see if she may need occupational therapy to strengthen her arm. Can’t wait for this to happen. She has suggested to only put lotion on the arm and in the crack to keep it moist to prevent ulcerations. Also ordered an EKG. Results were normal. Yeah!
01/31/08 Kevin took Ava to Dr Bayliff today. We are all pleased with the outcome of the H. The steroids seem to be shrinking the underlying structure of it causing it to appear larger, when in fact its actually just sagging more. Dr is happy with the changing in color and seems to think that we can start to decrease the dosage soon. She did find a small raw spot under the hemangioma near the crook of Ava’s arm where its sitting on her forearm so much and rubbing and wants to stay at the current dosage of steroids because of this. We are to put the creams on three times a day to see if we can get it to heal. She has contacted Dr Adams at the Cincinnati children’s hospital and both still agree that waiting a little longer would be better for Ava, but its up to us. I think waiting will be a good idea so that we can minimize scarring as long as Ava is not uncomfortable. And she doesn’t seem to be so far. I sent photos to Dr Bayliff over the last few weeks so that she could keep track of her progress and she sent them to Dr Adams. First steps will be coming on Friday to evaluate.
02/04/08 Over the weekend seems like Ava’s raw spot has gotten worse. Seems to be more puss filled than before and definitely more tender and bothersome to Ava. She’s been grumpy for several days now. Not like her at all. We gave her Tylenol last night to help with the pain. After applying the ointments we wrapped it. This morning when taking the bandage off, she didn’t cry much, and I wrapped it back up after cleaning and applying ointment. I asked the babysitter to take it off today and put more cream on. Got a phone call from her this afternoon saying that Ava was really fussy so she took the bandage off and it had bright red blood with it. She said she got some cream back on it, but that Ava was very upset about it. I have emailed Dr Bayliff about options. This little raw spot concerns me. It’s very similar to the ulceration that started in the crease of her bicep. What I don’t want to happen is that we just wait to see if its going to heal on its own again, and Ava will be in pain for 4 months. In talking with the nurse at the CCHMC she said that laser might be an option to help this heal so that it doesn’t get worse. I will wait to see what Dr Bayliff will say.
02/07/08 This week has been miserable trying to keep her arm comfortable. Putting the cream on three times a day, and sometimes the clothes stick to it and she screams. She screams even when you just touch her arm because she knows what your about to do. It has been so stressful. Cant wait to talk to Dr Bayliff tomorrow. I have made an appointment with her since I have not gotten a reply on the email.
02/08/08 Ear nose and throat appointment today went well, tubes will be put in on March 7th so see if this will help her hearing. Dr Jones thinks this will remedy the small amount of hearing loss that she has. Dr Bayliff was not in today, so we saw her nurse Elizabeth. She looked at Ava’s arm and said that she would call Dr Bayliff to tell her what is going on. Elizabeth called me this afternoon, and looks like we are scheduling a consultation in Cincinnati! Yeah! We should know something early next week. She advised to keep putting the cream on three times a day until we can get up there to keep it from getting infected. Ava also has a geneticist appointment on Tuesday to go over the leir weills syndrome that runs through Kevins side of the family. Also the Shriners will be calling us to set up and appointment to see if Ava will need help walking. First steps will be coming by tonight. Stressful keeping up with 7 drs for one child!
02/11/08 First steps came out Friday to evaluate Ava. They said that she just barley qualified. There are a few areas of concern. No response to mirror or playing with herself in mirror, no or little response to facial expressions, (I don’t agree) concern about playing socially, able to hold own bottle, and consistently reach for someone. There are a few of these that I don’t agree with, but they are going to send a developmental therapist out to evaluate a little more. I talked to Julie at the children’s hospital in Cincinnati and they are going to go over Ava’s case on wed and set the appointment for us for next week. Avas arm has gotten a little worse. The ulceration is now across the bottom of her hemangioma. She doesn’t seem to be in as much pain, but we are keeping it clean and covered.
02/13/08 We had an appointment with the geneticist today about the leir weill syndrome. We learned a wealth of information. They did a complete family background on both my family and Kevin’s. It took us about an hour just to complete that. Dr Bay seems really nice, and also happens to be a pediatrician! She’s very compassionate and listens very well. She doesn’t talk at us, but with us. Apparently with this particular syndrome, it can be passed male to female or female to male, but not male to male. So there is a question if it is leir weill syndrome, why Ryan has it so bad. That maybe there was something different passed through Melissas side, or the two combined made it worse. She is going to pull Ryans x-rays from Shriners and then also has Kevin do a full body x-ray. She said that it would help her determine the right syndrome. She made notations of Kevin’s outer extremities, and asked him all kinds of questions about pain, and difficulty moving his arms or wrist certain ways. She also said that we might be able to link other families to his as far back as great grandparents to help find out where it originated if there are any medical records left. She said that Kevin looks like classic signs of leir weills but since Ryan got it so bad, that there is a small question. Not to mention when she looked at Ava, she noticed that she has a flat nose bridge and no labia (she did a FULL examination of her), which are signs of a different syndrome. But that the nose may have come from me. She did photos of all of us, legs, arms and faces, profiles etc…. She said that Kevin’s bone spurs on his elbow are a classic sign of a different syndrome as well. So there are a lot of factors at play here. X-rays and blood work will tell us exactly what this is. We will be going back in April to go further into it. She said that once we find out exactly what this is, it would help the whole family medically. Future and past. Kevin and I both agree. We signed an agreement that they can use our information for learning and teaching, but not for publication. Dr May understood and was very helpful.
02/14/08 We have an appointment on Tuesday in Cincinnati! Hopefully we can get things going and get it resolved.
02/20/08 The visit with the surgeons and drs at Children’s went great yesterday. They are very pleased with the way that the H is looking with her on steroids. They think that it’s starting to involute quickly. There were 2 surgeons and several drs that looked at it yesterday. They said that they could take it off now, but if they did it would leave a much larger scar and more risk of infection. We have all decided to wait until Ava is about 2 years old to remove. This way there is more good skin to use to close back up and the scar will not be as large. They are however going to do laser treatment on the part that is ulcerated on Tuesday the 26th at 11am. This will help the ulceration heal and prevent Ava from being in anymore pain with it. There is a possibility that it might take more than one treatment to get it to heal depending on how deep it is. But they said that anytime it ulcerates, that we can come back and do it again to keep her from being in so much pain waiting on it to heal on its own. This is the answer to my prayers. They also gave us some lidocain to put over the top of the ulceration to numb the area until the laser treatment. They are wonderful there and I am really excited about working with them. Dr Bayliff has been so wonderful here and I am very thankful that she recommended Cincinnati. First steps came out Friday to evaluate Ava again. This time it was a developmental therapist. She said that Ava is doing very well for her age. That there are no real concerns with her development. She does however need some therapy for her neck muscles to help her raise her arms to hold her own bottle. She will come one to two times a month, and that was all she thought she needed at this time.
02/27/08 Avas laser surgery went well yesterday. Everyone at children’s is so nice. The dr was very pleased to see that Ava’s arm had healed since we last saw him a week ago, and didn’t think that she would be long in surgery. We brought her back to the OR and no sooner did we get to the waiting room, grab a cup of coffee and a snack and sat down, they called us back to talk with the dr. He told us that she was only under for 5 minutes and that her arm looked great. Dr Bayliff in Lexington will be doing the follow ups. He wants to see her back in about 3-4 months to look at possibly doing surgery earlier than 2 years old. He’s very confident that Ava will do well with removal. After her surgery, and had some food in her belly, she took a long well deserved nap on the way home. She was her normal playful happy self when we got her home. Next week is tubes!
03/10/08 Well, no tubes! They went in to place the tubes in Ava’s ears and found no fluid on the eardrums, so decided not to put them in. Did a hearing test and found that she does still have a mild hearing loss to low frequency sounds. This could be normal, hard to tell at this age, they will keep a watch on her. Only complications we had with this one, was she’s allergic to the glue that they use for the monitors and hearing tests. Her poor little ears developed raw places where the glue was. First steps came and decided that they are going to see Ava about 2 times a month to help with a few things like holding her bottle and tipping it upward, reaching with both arms consistently and also walking and standing flat footed instead of on her tippy toes. They were speechless when they watched Ava pick up a small piece of food with her finger and her thumb, something that a baby her age normally can’t do yet. Ava has come so far in the last month! She’s crawling, going from a laying down position to a sitting up, she pulled up on her own in the crib this morning, and she is even getting her first tooth! So amazing! Next thing you know she will be walking! Thursday is a follow up with Dr Bayliff. I am going to see if we can lower the dosage on her steroids. She developed a diaper rash the other day and seems to be gaining a little more weight in her face that normal. She also seems to have an increase in appetite but only at night! She’s getting up more and more to eat. Thursday is also her appointment with Shriners and first steps is coming back to work with her. We will keep you posted.
03/14/08 Ava saw Dr Bayliff yesterday. She was very pleased with the outcome of her laser surgery. She thinks it’s healing very nicely. We were also told that she should be past the phase that any more ulceration would occur. We have dropped her steroid dosage down to 1.2 ml a day instead of 1.5 and next week we will be at 1.1 and then in to see Dr Bayliff again. She also had an appointment with the orthopedic and they did x-rays of her whole body. He said that her bones all looked normal, but that he still wanted Shriners to look more closely at her. They will call me with the appointment. It will be the same dr that took care of Ryan’s arms. She’s in good spirits other than teething. She’s up several time a night crying out because her gums hurt, so we have started giving her Tylenol before bedtime to help her sleep.