32 month old diagnosed with refractory KD in Atlanta

[youngwk2000]

First let me tell you that in my opinion Egleston would had been better to go first than Scottish Rite. My daughter had a fever on Sunday and complained about an earache. Few days prior she would wake up crying but thought it was just a bad dream. Sunday ran a fever and complained of left ear hurting. Monday took her in for checkup and Pediatrcian said she had an ear infection. Prescribed Amoxicillin. She wouldn't take antibiotics, kept throwing up medication and even liquids. Her fever persisted and Tuesday noticed the rash and red eyes in whites and around skin. Called Pediatrician Wednesday talked to a nurse described rash around eyes, genitals and cracks around body along with fever even with Tylenol and vomiting. Nurse said that was normal with the fever and I asked for zofran so she would stop throwing up and hold antibiotics down. She talked to the Dr and we were given the prescription. That night something felt wrong and my daughter just looked wrong staring at me crying. At this point I decided to take her to the pediatrician again and our Pediatrician recognized all the classic symptons of Kawasaki. She sent us to Scottish Rite Childrens Hospital to be admitted right away. The nurse hands shook and couldn't get the cathoder in right to get a urine sample, my little girl was crying in pain. Infectious disease Dr started us on Ivig and high dose aspirin that night. Her symptons got worse, swelling rash, strawberry tongue, bleeding lips, high fever, swollen lymph gland in neck, swollen extremites, and body joint pain. They did an echocardio and her heart was fine. The first Dr Balcerak told us she will be fine and the most important thing was her heart was fine don't worry. Fever swelling rash really bad left swollen lymph node and second ivig was given. Another echocardio and ultrasound on the lymph node. Heart had some fluid around it but still ok and the lymph node was not infected. She looked better but not completely so 3rd ivig was given along with steroids. Blood work was looking better and rash was going away but swelling and fever was there. After the 3rd Ivig I askd she stop IV fluids because I didn't want her to retain water and she looked bloated. Balcarek agreed but I had to watch the nurses because sometimes they are clueless and kept trying to give her IV and we kept getting an oral laxative that was cancelled for a suppository. I kept her hydrated with water, few little sips all the time. We're still being told don't worry she will get better then we were handed off to a different Infectious disease Dr. Shore which I really didn't understand why except she thought the worst was over but I thought they would consult each other. I never saw the first infectious disease Dr Balcerak again even though my daughter was not close to being better for discharge. Dr.Shore kept claiming he was an expert on Kawasaki and seen 1000's of patients and everyone consults him for advice. I wasn't impressed because he never really took an interest and examined my daughter for more than 10 seconds but I had my daughter's life in his hands. He gave 2 pulse steroid treatments with zantac for upset stomache and fever spiked but went down with Tylenol. I had to watch the nurse because the first dose of steroids was lost in transit and we were waiting for a replacement. I left my wife for a second and came back after a few minutes I asked about the steroids and after shift change nobody could confirm it was given. The first shift nurse never logged it on her way out and wasnt answering her phone. I was furious and when they did ask her on the phone I couldnt believe them since every other medication was logged. I made them search for the iv syringe for proof and they found it in the trash can. After watching her go through such horrible physical pain and changes we were relieved she looked better and actually wanted to try to walk. Dr. Shore still did not make it a point to come by with sincere interest to see her progress. I asked the pediatrician on staff about Dr. Shore and she said he just left. We ran down the hall to call him and yelled for him. Again he took a few seconds to diagnose her and said her blood looked better and if she needed another dose of steroids we'll see. That night she regressed developed a fever and started to swell again with pain. She cried if I would pick her up. Dr Shore tried another steroid treatment but it didn't work and she was still in pain with fevers. Fortunately we were not discharged early because we would have returned but I felt he still did not take a keen interest in this resistant KD case and wasn't confident he was capable of treating our daughter after the standard treatments werent working. He really didnt take the time to discuss next possible treatments except possibly stronger steroids but he was a self proclaimed expert. At this point her left lymph node was gone and I was watching her right lymph node swell up through out the day, she was still swollen in the hands and feet and I was getting real worried. The infectious disease Dr Shore didn't come to see us til 10pm last night and I knew he wasnt going to see us early enough the next day or even at all. A pediatrician Dr Beach at Scottish Rite told me a Rheumotologist was coming to see my daughter but he was coming from Egleston Childrens Hospital around lunch the next day. More pain and worry. Lunch passed and swelling was getting worse and still no Drs. My daughter has been thoroughly traumatized by the KD and her arms and hands have so many track marks from drawing blood and finding new iv spots. . She cries anytime someone comes in. I was getting worried because she was in pain , I was actually going to walk out and fly to San Diego with my daughter to the airport that day. I have been doing online research and treatments and came across this forum and found Dr Jane Burns. She is out of UC San Diego and has the Rady Kawasaki clinic in San Diego. A forum member listed her number and email address. I emailed her and within the hour she emailed me back for my Dr. to call her in regards to her opinion on further treatment. It was getting late again and I wanted a Dr to contact Dr Burns. I had the nurses try to contact the Rheumatologist, I was giving up on Dr Shore. Dr Vogel was on his way w/in 2 hours. I gave Dr Vogel Dr Burns info after he examined my daughter and discussed next level of treatment. I discussed cyclosporine. He said he would contact Dr. Burns and discuss further treatment. He was worried about side effect of cyclosporine. I was really happy he would contact Dr.Jane Burns in San Diego. Dr. Shore showed up later. Dr Vogel spoke to Dr Burns and Dr Shore and a decision was made to try remicade. She got one dose of Remicade with lower doses of aspirin. She seemed a little better. She was still getting intermittent fevers and using tylenol for them. Her crp was going up again. Dr. Vogel decided to start cyclosporine treatment based on Dr. Jane Burns San Diego protocol. A third echo was done and now what we feared most was she had aneurysms in her left and right arteries. Left was 8mm and right 3.5mm. We now had a cardiologist involved and after his review of the another echocardio after the remicade was done, he decided to move us to Egleston Childrens Hospital since they have a cardio dept and Scottish Rite did not in case Emily had a heart attack. We were given a picc line before we were transported, thank God. It will now be less traumatizing for Emily to draw blood or give medication. He was hoping that last echocardio showed a decrease but it did not. Emily could not do any sports or strenuous exercise anymore. Emily was started on blood thinners and had to consult with hematologist.She was started on Lovenox and Plavix. The Cardiologist was now consulting with Dr. Newberger out of Boston. He wanted to give Emily Abciximab for her aneurysm that may help her years down the line. She stopped the lovenox and plavix and was given one dose of iv abciximab. Then later resumed blood thinners, two shots of lovenox and no more plavix. After we were transferred to Egelston cyclosporine was resumed. I believe there were 5 or 6 iv's and same lower doses of steroids then switched to oral cyclosporine and steroids. Emily showed gradual improvement. Fever went away, joint pain went down, swelling in feet, hands, and knees went down. Her appetite is back, she is talking, and moving her legs, arms, fingers, and sitting up. The Dr's all consult each other, review her blood tests and every morning get together and go to each room in the morning, something that seemed lacking at Scottish Rite. We are looking to go home tomorrow. It was a long 3 weeks that felt like an eternity. Emily has been thoroughly traumatized from the daily blood work sometimes 2 or three a day. One nurse at Scottish Rite kept searching for a vein she never found and I watched my child scream in pain. I personally stopped it and asked for the iv team in the morning but they could not wait so a trauma nurse came up to do it. She got it on the first try. It was brutal to watch the first failure, I could not imagine the pain my daughter was in on top of the pain she was already in. Emily is now on oral reduced cyclosporine, steroids, lesser dose aspirin, prevacid for upset stomach (dont want ulcers when you are on blood thinners) and lovenox shots (she screams everytime I give them, my heart sinks but her mom said she can't do it so I'll be the bad guy) Tonight they draw blood right before the cyclosporine and two hours after, they need to check her levels again for a peak and trough. One more echocardio to check her heart. If everything looks good we are looking to be outpatients. Emily cannot do sports, strenuous exercise, cut herself, continue medication that will gradually taper down until her bloodwork and physically looks good. She will stay on the lovenox until the cardiologist says her heart is improving but she will continue to need a cardiologist for years to come. We are hoping she will eventually have a normal life and that the KD does not come back. My advice to a parent is get your child to the right hospital if she has KD and if your gut tells you there is something not right find someone who agrees with you. KD is the leading cause of childhood heart disease and so many pediatricians miss the diagnoses and window of oppurtunity to treat it. If your child does have KD and the standard treatments are not working have your Dr contact Dr Jane Burns in San Diego for consultation. If you are in Atlanta take your child to Egleston and not Scottish Rite. They are willing to consult with Dr Burns, they have a cardiology dept, and the rheumotologist and hematologist are already there.
We just had the last echocardio and the tech calling for the Dr and asking if they needed more pictures. We never saw the Dr and it's making me nervous. It's later in the afternoon and most of the cardiologist have gone home and I will find out tomorrow morning, the day we are suppose to get discharged.
I read something from Dr. Newberger "clinical similarities between Kawasaki disease and acrodynia (mercury hypersensitivity)" I was thinking flu vacines have mercury as a preservative. I never gave our kids flu vacines until all the media hype about vacinations. Most outbreaks occur between winter and spring. Makes me wonder if I should give my kids anymore flu vacinations but Emily will need them after all the immunosuppresants she's been given. ??? The single shot flu shots don't have mercury, I think I will request those for her, you never know. I hope they find the cause of this disease so we can destroy it.

[youngwk2000]

We have been home for almost a month. My daughter is on baby aspirin, lovenox, and cyclosporine. She was also on oral steroids that were tapered down and ended a week after we were discharged. The steroids give her mood swings, hair growth, and a voracious appetite. We were glad the symptoms related to the steroids went away when she came off of it, especially her fits of rage she never had before frightened us. She is having joint pain and arthritic symptoms that are slowly getting better. She has a stiff neck that I've been massaging and giving her warm baths. Everyday I believer her physical movement is getting better and there have been no fevers, thank God. She does have stiffness and body aches at night but the mornings she is happy and active. The shots of the medication 2x a day is very stressful. The lovenox shots are bad but are necessary for her aneurysms. We are due for another echocardio in a month and are hoping there is an improvement in her giant aneurysm. Our medical bills from Egleston, Scottish Rite, and all her specialist have gone over 100k, I am so thankful we have insurance but we still have to pay some. Looking back I believe we would have been in a worst position if our our pediatrician didn't recognize KD and send us to a childrens hospital that also recognized the KD and started her on IVIG to help protect her heart. But there needs to be some kind of national standard all pediatricians should be informed I MEAN ALL PEDIATRICIANS not only recognizing kd but starting treatment asap for it. There also needs to be more guidelines for REFRACTORY KD, it seems its a guessing game for these Dr's after the standard treatments do not work, it's better to have them consult someone like Burns or Newberger first. I think if I hadn't contacted Dr Jane Burns, thank God she emailed me back right away, my daughter would not be home getting better. The cyclosporine she was ultimately put on and is currently on at home is what finally subsided the KD and if it wasn't for the great cardiologist who stepped in to administer abxicimab, lovenox, and monitor us and move us to Egleston we would have been worse off. My advice to a parent is inform yourself about KD and all possible treatments, if the treatments your child is on is not working then talk to your Dr. about what is being considered and have them contact a specialist on KD if its refractory. Watch your nurses and make sure they are administering the drugs correctly. I had a few scares and after watching them I started to point out what exactly needed to be done if a nurse wasn't correctly doing her job. One nurse did not really know how to program the IV machine. I asked for another nurse for help. Nurses rotate and you may get an awesome one on the ball but you may get one that is horrible. Medication is so critical so times, lengths, quantity that it is administered is so critical with KD. I am making it my mission to get my daughter back to normal and healthy. Stay on the ball, give her medication at the right times, comfort her, and keep her hydrated/fed with healthy food. At first I tried to give her anything she would eat to get her energy but now we try to feed her heart healthy diet, less fried foods and more vegetables, meats, and fish. We have a rheumotologist and hematologist appt Wed. I am praying if she is off of cyclosporine this damn thing doesn't want to show up again.

[youngwk2000]

My 2 year old daughter is still doing well as can be hoped for, her energy is back and she can't sit still anymore. You would never think she was ever sick with KD. No fevers, aches, or swelling so we put her back in school slowly 2x a week only. She was so excited to go back to school. She has not had any regression of her 8mm aneurysm on her left coronary artery but it has not gotten any larger, her heart function continues to be normal. We continue to hope the next echocardio will show regression. Her last two blood labs were normal and that is a very positive sign. Crp levels are all normal. She is still on childrens aspirin 1x daily, lowered cyclosporine oral dose to .12ml 2x daily, and 2x daily lovenox shots. Looks like we will be getting off the cyclosporine very soon if her bloodwork continues to look normal. The lovenox shots are hard to deal with for a child and I hope we can discontinue them soon. Her cardiologist Dr. Sutherland is great but he is elderly and we know he will not be around for her longterm treatment, makes us sad since he is so great. Her Rheumatologist Dr. Prahalad, Dr. Vogels associate, is great and thinks she is doing great too. Her Hematologist Dr. Patel is also very good and thoroughly explains everything to us including the fact the lovenox shots are the best in terms of side effects and keeping proper levels in her blood. I am very happy with the team of Drs at Children's at Emory. It's been very tiring and trying on us but we know we have to continue to keep positive and don't give up physically or emotionally on her. She never gave up so we shouldn't either. I always give her a hug or hold her when she asks. It was never her fault this happened and I could never imagine the pain she went through. I try to make her shots as painless as I can but she still cries and the bruises are so upsetting. Sometimes I sneak them in when she just falls asleep but they still bruise sometimes. I also crush her aspirin into a powder, mix it with just a tiny bit of strawberry danimals and suck it into a measuring syringe then put it in her mouth. It was so hard to get her to take medicine at first but now she is ok with it and takes her orals with no problems. We pray almost every night together and use holy water our family sends us. We keep the faith together. I just thank God she continues to be healed.

[mom2novboys]

I'm sorry you had a bad experience with Balcarek and Shore. My son was treated in 09 for KD at Scottish Rite by them and I swear by them! They saved his life. Your daughter also has the same cardiologist that my son has and we love him too!

My son is experiencing lots of other problems now that we are trying to get addressed. In the meantime, I run to Scottish Rite anytime anything is wrong with him. I swear by the staff at Scottish Rite. Again, sorry you had such a poor experience there. They are the only people my son trusts!

[youngwk2000]

I really did appreciate Dr. Balcarek recognizing what my pediatrician sent us to Scottish Rite for, she was friendly and assuring. I didn't get the run around at Scottish Rite and denials that it wasn't Kawasaki like some people have been writing about at their hospitals. Initially a female Indian ER Dr., I forgot her name, admitted us and thought it was Kawasaki too, then we met Dr. Balcarek who started us on IVIG right away. I was just disappointed when my daughter's Kawasaki started to become Refractory. I felt the Dr's should of taken more of an interest in her condition at that point and I lost confidence the infectious disease dr at Scottish Rite could handle it. After the standard IVIG treatments don't work then it's really a guessing game for these Dr's and I didn't want my daughter to be experimented on like a lab rate. That is why I did my own research and emailed Dr. Burns. She has been studying treatments on refractory Kawasaki and I was so happy Dr. Vogel from rheumatology at Egleston consulted Dr. Burns and took over treatment of my daughter. I really dont' think if Dr. Balcarek didn't start her on IVIG right away and Dr Vogel didn't take over treatment things would have turned out so well to this point. My daughter was at Scottish Rite and Egleston for 4 weeks before being discharged. I was able to compare both facilities and Dr's, I side towards Egleston. There were so so nurses and great nurses at both hospitals but the Specialist (Cardiologist, Rheumatologist, Hematologist) we needed were all centrally located at Egleston or next door at Emory Childrens. Sottish Rite may be better for certain things than Egleston but Egleston has a whole cardiac unit and floor. Ultimately after you can get through the horrible changes your child goes through it is about mitigating any heart problems and long term care. There were teams of Specialist that came to see us like clockwork at Egleston and that wasn't the case at Scottish Rite. I hope and pray your child gets better too.

[youngwk2000]

Little Emily is doing so much better now. Still no fever and is very active today. We had a rheumatology appointment today and it went very well. I stopped her cyclosporine treatment a month ago on my own. The last time we met the Dr. we discussed stopping but decided to lower the dose. I felt confident enough to stop her dose before the next Dr. visit since she was only taking .12ml then I stepped it down to .11 to eventually just stopped. I was worried continued use of the cyclosporine would be bad for her. The rheumatologist said it looked like the kawasaki was gone and he felt confident since she has gone so many months without any fevers or any symptoms that she will not relapse. Dr. Prahalad discharged us and her original Rheumatologist, Dr. Vogel, came by to say bye. We were so happy to be discharged but sad to let two great Dr's go. Now all we are dealing with is the giant aneurysm so we continue to see the Cardiologist and Hematologist. She is only taking one baby aspirin and twice daily lovenox shots. She has so much energy were afraid she will hurt herself since she is on blood thinners. The lovenox still leaves bruises and she still cries when she gets them. Her aneurysm isn't getting worse and is slowly shrinking. Right after leaving the hospital when she initially got sick, I was worried about the arthritic symptoms, joint pain, tiredness and night sweats, fortunately it all went away in time. She has such an appetite and so much energy now. We have her back in school 5 days a week. I am so grateful things are getter back to normal. It has been tiring on us all but I think faith, diligence, educating myelf on the disease and keeping positive has helped us all. I think the positive aspect of what has happened is that I have grown closer to my daughter and cherish everyday we spend together. So many children are just content children and don't realize how much their parents care about them but I can see in my daughter how she realizes how much we do love her after helping her get better from this disease. She can't get any live vaccines for 11 -12 months after ivig treatment. We also had a 3 year wellness checkup at her pediatricians and luckily her next round of vaccines will be at 4 years. I will go back to her pediatrician to get her flu vaccine when they receive their shipment. I asked if they were single dose shots and they were. The single dose do not have thimerosal/mercury preservatives.

[youngwk2000]

My little Emily is still doing fine and recovering great. She gave us a scare when she got a cold and had a fever for 4 days but it broke on the 5th day and she got better. I got pretty worried. Her blood work still looks normal. She only takes 1 baby apirin and two shots of lovenox a day. We only see the hematologist and cardiologist now. She still cries when she has to get a shot. We have another echo in December which is 4 months after her last. I am hoping and praying her one 8mm aneurysm has gone down. I want to take her off the lovenox because I worry about her being so active now and getting hurt not to mention she doesn't like it. I still worry about her and I think I always will.

[youngwk2000]

Little Emily is still doing fine. Her aneurysm has shrunk a 1mm and will be checked again in 8-9 months from her last one in September. I am hoping more shrinkage or a miracle it is almost completely gone. in reality giant aneuryms usually needs long term trips to the cardiologist. She is still on baby aspirin and twice daily lovenox shots but her Hematologist is putting her on warfarin/coumadin. We had talked about this from the beginning of transferring her to pills after a year of lovenox. Well it's been a year and I am so happy she will stop taking shots. Even though the lovenox shots are better for her in terms of side effects and maintaining therapeutic levels they are really upsetting for a young child. You can give her shots under her arms(biceps), top of her legs(quads), or her stomach about a few inches away from the sides of her belly button. The nurses were telling me wrong giving it the her on the sides of her legs, consult your hematologist for the correct place and way to give the injections. She will only let me give her shots in her stomach though. You have to pinch then inject but she still can't get used to it. Sometimes its hard to do when she cries and wont stay still. Ive had to give myself injections so she didnt feel so bad. I've found that using the 31 gauge 8mm length needle in the 0.3ml size are the best. They are the thinnest and just the right length and size for one handed injections. it's hit or miss because sometimes you will get really bad bruising and hardness or hardly anything. You have to rotate but since she only likes the stomach i've had to be careful of bruising and hematomas. anyway we started warfarin and until they get the right levels she is on both the lovenox and warfarin they call it bridging. We draw blood tomorrow and if levels look right we stop the shots, she is so happy about that. After a year on lovenox your child should also get a bone density test. We are scheduled for one in April. The lovenox affects bone density.

[Joana_09]

Nice post

[firemedicsapp]

We will be at Emory University Monday 09082014. My husband is a Kawasaki disease patient was not treated as a child. He has coronary aneurysms and has a collapsed aneurysm. My husband has been in cardiac arrest in 2009 and had anther heart attack two months ago. My husband served in the Navy and worked as a Paramedic/firefighter until he went into cardiac arrest. He now takes three blood thinners Plavix, aspirin, and xarelto (was on Plavix). Steve was misdiagnosed as a child, when he was little they believed that kawasaki's disease only affected certain nationalities, this has been proven wrong. I wish you the best of luck with your daughter. I understand that the disease is a scary one with little information on what the cause is or how to prevent it. My husband has a pacemaker/defibrillator now and luckily it as t had to shock him r regulate his heart rate. I do know that with this last heart attack he spiked a fever of 101.1 for three days before e woke up with severe chest pain. We rushed him to the hospital and she showed no cardiac changes on the ekg which baffles the doctors because he did have a heart attack. We use a cardiologist in Auburn, Alabama which is close to home. We are hoping to go to San Diego next year if his health permits so that the Kawaski Foundation can get as much information to us and us to them. Steve is still fairly active (we have a little one ourselves), and we ride horses often. I know that you get scared with every fever every time that she tells you that she doesn't feel good. I would love to tell you that the feeling of panic goes away. I have learned a lot with the Kawasaki disease throughout the years. I know that Steve gets a sunburn easily and his cardiologist says he believes that this has something to do with the disease. If we can give you any information or if w can attempt to answer a question for you shoot me a message.
Good Luck and Thanks
Naomi
firemedicsapp@gmail.com

[youngwk2000]

Thank you.
My daughter is still doing fine now and still on one baby aspirin and coumadin because of her giant aneurysm. She is doing fine now and we are due for another echo.. My daughter and I have grown so close she is stuck to me like bubble gum and I'm fine with that. I keep a hepa/uv filter in her room just in case, I read kd could be fungal carried on winds.
She had 4 cavities filled and our hematologist put us back on lovenox shots during the procedure (she has a little fat around her tummy so no bruising just a small dot where the needle went in) and we'll be gradually bridging back to coumadin and 1 baby aspirin. We have an echo scheduled in a few months and hopefully it has shrunk even more or maybe it will be gone and we can come off of blood thinners, that is ideal.
I was worried about putting her on warfarin/coumadin after researching the side effects but our hematologist told us those were problems associated with not getting levels right and not bridging medications. As of today the only side effects are bruising sometimes bad but they go away. Just a fyi we never did a bone density test as stated previously, our hematologist said she consulted and didn't think she needed any unnecessary radiation.

[youngwk2000]

Things have been quiet on the kd front. My daughter just started kinder but every little fever, runny nose, bruise, and anything to worry about we do, just in case. She still has routine monthly bloodwork with a hematologist and yearly echos for her shrinking aneurysm. Learned something our pediatrician didn't catch, if your child is doing baby aspirin therapy do not give her live nose flu vaccinations because of Reyes Syndrome, just shots. They will be checking her vitamin k levels and check her bone density, the blood thinners have something to do with it.

[glitter22]

I was reading through your post today and we too had the same experience in Phoenix Children Hospital.
It happened exactly one year ago, my daughter was 27 months. She had her first dose of IVIG and the doctor said she was fine and ready to go home. We insisted to stay for two more days till she was little better. Still in hospital her condition was deteriorating. After 72 hours she was started on 2nd dose of IVIG. The next day they were ready to let us go. We refused to. Within 48 hours my daughters condition was worse. We wanted to take her to San Diego. The doctor didn't allow us. We then contacted Boston Children Hospital and seek help from them.
After that they didn't treat her with IVIG and started steroid. She received three days of steroid after which her symptoms vanished.
Hope your daughter is doing fine now.

[Lyndahloo]

God bless! I hope your daughter is doing well. What a harrowing experience you've been through. How very strong you are to be her "rock." This disease is very scary. My son gets his first follow up echo day after tomorrow. Thank you for sharing your story.