Change in plans

I just wanted to say thank you for praying, especially for wisdom and direction for the doctors...Emily's surgery has been cancelled for now! Things are falling into place - Emily's cardiologist is working to find a doctor so we can get a second opinion on how to best treat her knee arteriovenous malformations (avm). We're feeling frustrated as we'd like to get this taken care of, but we're also feeling relieved as well!
Thanks for your love and prayers!

Time for an update

Sorry I haven't posted in awhile....Emily is doing well! She is enjoying the new homeschool co-op, taking spanish and drama. She is in her element in drama class, using her british accent with purpose now! She's made some new friends there and through the church. Her oxygen sats are the same and she remains on oxygen 24/7 with a few hour 'break' during the day. She had an appointment with the cardiologist - it was nice to have only a 40 min drive to get there :) The echo showed great heart function...Praise God!! We also made a trip to Cincinnati Children's Hospital for 3 days in December; she had a couple of appointments with the team of doctors that are following her there. The hematologist is still investigating the bleeding issues, her tests are coming back "unusual" but he still feels that she has some form of acquired von Willebrand disease - a blood clotting disorder. Thankfully, she has not had any new episodes of bleeding from her gums or her knee. She also had a liver ultrasound and a MRI/MRA for both knees. The plan right now is to have sclerotherapy for the arteriovenous malformation (avm) on her left knee. Sclerotherapy is a procedure to treat vessel malformations by injecting a solution into them to help them shrink. The surgery is scheduled for Feb. 22. They won't be able to do it the traditional way on Emily and this procedure has not been performed extensively this way. We are so grateful that you have been there to lift Emily/us up in prayer and we ask that you please continue to pray for wisdom/skill for the doctors. Pray that this would be the best plan of action, and best place for Emily to be. Pray also that we would have clarity and peace of mind. Thank you all so much for your love and continued support - we love you!!

Seventeen?

Emily recently had a birthday....Wow, I can't believe that our baby is 17 now! She has been feeling pretty good. Her oxygen saturation levels are a bit higher. We laugh because when a friend of ours was encouraging us to move back to Illinois he joked that Illinois air has more oxygen in it than Indiana, well....we've moved back to Illinois and her sats are higher, hmmm? - haha - whatever the case, we'll take it! They are mid to high 80's while on 2L of oxygen and low to mid 80's off oxygen. September 8 she had a cardiology appointment. The echocardiogram looked good with good heart function so Dr. kept everything the same and wants her to return in 3 months. We're still waiting to hear from Cincinnati Children's Hospital about the labs that were done, or what the plan to take care of the AVMs in her knees and wrist will be, but her knee continues to heal everyday :)

Orthopedic appointment

Emily had an appointment in Chicago to check on her scoliosis. Thankfully, it was a very uneventful trip - we like those!! Since the curve had not changed at her last visit 6 months ago, and she is not having any symptoms, they decided not to do an xray this time. In and out in a little over an hour....unheard of :)

Cincinnati appointment

Emily had an appointment in Cincinnati yesterday – it was a long day but everything went well. I really don’t know what to report as I left there a bit more confused myself. Hopefully, while we wait for additional info from the doctors, I'll be able to process it all....? I feel very confident with Emily in their care but as in Dr. Adams’ words yesterday (words we’ve heard over and over) “Emily is very complicated". They have been consulting with Boston Children’s Hospital about things, and need to re-connect with Emily’s home cardiology team as they are considering the prospect of surgically removing the AVMs now. She has an appointment July 1st for more labs and to see another hematologist. Thanks for checking in on us and please pray for the doctor’s wisdom in how to best treat our Miss Em!

P.S. Fun part of the day...we stopped on the way home to see friends that live in Indy. It was great to catch up and helped to break up the drive as well!!

Bringing you up to speed

(beware: long post)

Wow, I can't believe I haven't sent an update since November '08...I've created this blog as a means to do just that! After Emily had the heart catheterization to embolize 6 collateral blood vessels, she's had a few procedures and appointments to further explore the gum and knee joint bleeding, the knee AVMs, and the possibility of the genetic disorder HHT (Hereditary Hemorrhagic Telangiectasia).

**AVMs (Arteriovenous Malformations) are when capillaries are bypassed and the arteries are connected directly to the veins; there are a lot of these abnormal connections so an AVM looks like a tangled up mass of vessels. HHT is a genetic condition in which one can develop AVMs in the nose, lung, brain, liver, etc.

I’ll try to summarize what she’s been up to since November:

Cardiology: 4 trips all good. heart function is good, everything is the same including O2 sats. She remains on oxygen.

Orthopedic: scoliosis hasn't progressed any further but a bone density test shows that her levels are very low so she is now taking a vitamin D3 supplement.

Cincinnati Children's Hospital:
-Vascular clinic: 12/08 brain MRI/MRA negative for AVMs but showed some old activity probably from when she had surgeries and loss of oxygen as a baby. 2/09 ultrasound of the abdomen looking for AVMs in the liver came back negative.
-Genetics: genetic testing for HHT and PTEN came back negative.

Neurologist: confirmed that brain MRI shows old activity but wants to re-do one in the summer to confirm. She was having some episodes of tingling on the left side of her face; he believes that was due to a neck muscle spasm from her scoliosis pressing on the occipital bone and facial nerve. The tingling has has since subsided.

Ophthalmologist: negative for AVMs

Dentist: cleaning done with no gum bleeding.


Praise God - lots of negatives! However, Emily’s knee started acting up again on 5/9/09. This happened before exactly 1 year ago....if you remember, they found that she has suprapatellar AVMs in both knees (looks like a tangled up mass of vessels right above her knee cap) Thankfully, it is only causing a problem in the one knee. These blood vessels tend to be fragile and in this case have leaked blood into the joint space causing a lot of swelling and severe pain. We made a trip to the vascular clinic at Cincinnati Children’s Hospital. Emily had an MRI and then an ultrasound the next day. We also met with an Interventional Radiologist. They then informed us of the ways to treat AVMs:

1. surgically removing it (this would also require tendon reconstruction) and given Emily’s heart history, they would like to avoid this.
2. insert a catheter through the femoral artery in her groin. Since she will need future heart catheterizations, and that access, so they would like to avoid this too.
3. sclerotherapy - under anesthesia they directly inject a small amount of alcohol to the nidus (the center of the AVM) to eliminate the vessels.

Sclerotherapy will probably be the best option but they want to consult colleagues first. As they put it, Emily is a special and unusual case! They want her knee to heal and the swelling to resolve before they do anything. They sent her home with pain meds, but she ended up with a horrible weekend and wasn’t even making it the 4 hours until her next dose! Back to Cincinnati we went….this time to see an orthopedic there who confirmed that the fluid is coagulated and cannot be aspirated and it is just going to take time to resolve – they think that the blood vessels may still be oozing, reiterating the need to take care of the AVM asap. She’ll probably need to have some physical therapy as she cannot straighten her knee and bleeding into the joints can cause cartilage damage. The hematologist there has also done additional blood work to triple check clotting issues with her blood.

She has been much more comfortable the last few days and even made it to church! (thanks to the crutches from a friend) We are praying that she remains comfortable and heals quickly. Thank you so much for your love and continued prayers!