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:
- surgically removing it (this would also require tendon reconstruction) and given Emily’s heart history, they would like to avoid this.
- 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.
- 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!
