Day 14: Discharged After Seizure

After 2 nights in the hospital, Bethel is back out again. Hopefully, no more issues from here on out because she's been through so much. The pain and nausea was unbearable for her and for me to watch. She's starting to show signs of difficulty forming words and processing thoughts at the moment. This not too uncommon for patients that undergo surgery on the left brain, so she may need speech therapy and is a temporary condition. As far as the seizure, the doctors told us it's most likely a manifestation from the surgery and fluid collection that irritated the brain. Again this is a temporary condition and the doctors do not expect her to have chronic seizures in the future. She's been prescribed anti-seizure medication, Keppra, as a precaution.

Tomorrow we have one last appointment (fingers crossed) with Teresa, Dr. Steinberg's nurse, for evaluation to travel back and to remove her staples.

Day 12: Seizure

Bethel was discharged from the hospital yesterday (11/19/11). She was doing very well and was able to walk over to Lucile Packard Children's Hospital to follow up on 6 yr old boy who underwent the same procedure. His family also brought him to see Dr. Steinberg. He also seems to be doing very well.

Around 12:30 pm today Bethel had a seizure in the apartment. This time I knew it was real because I've witnesses a former college roommate seize in front of me. Her body suddenly stiffened up while sitting on the sofa, her eyes rolled back and her mouth was wide open. Within seconds of me realizing what was going on she began to convulse and foam at the mouth. She was resting her head on the lastest incision side, so I moved her onto the ground and laid her on her right side. She became completely unconscious at this point and I could not get her to respond. I was so scared and felt so helpless. I called 911 and the paramedics arrived within 10 mins of the call. She was beginning to come out of the seizure at this point.

Upon arrival to the Stanford ER, Bethel was conscious and could say her name and age. She was very disoriented and she had difficulty with words. The resident neurosurgeon on duty had participated in her first surgery and recognized her. The doctor ordered a CT scan and it appeared normal. They did see somethings they want to discuss with Dr. Steinberg, but I was told seizures are not uncommon after brain surgery. It may take time for the brain to adjust to the increased blood flow that had been lacking for a long time.

She's spending the night for observation. Thanks to Misono (Bethel's former college roommate) and her husband Dan for visiting and feeding us. We had plans to have lunch with them, but it unfortunately turned into a hospital visit.

Day 10: Post Surgery (Left EC-IC)

Bethel is doing extremely well this time. She left the ICU and was transferred to the G1 for further recovery yesterday afternoon. She is not experiencing the sick to the stomach nausea from the first surgery, so she's been eating well. Her dressing was removed today and only has 1 maintenance IV line hooked up. This time the surgeon stapled her incision rather than with sutures. The right sutures were removed during the operation on Wednesday, so she only have the left staples to be removed. The doctors plan to discharge her tomorrow and we have 1 final appointment with Dr. Steinberg  next Wednesday before we leave for home. Below are pictures of her battle wounds against Moyamoya:

Second surgery in the ICU (11/16/11)

Dressing removed (11/18/11) revealing the closure staples

Day after first surgery on the right (11/10/11)

Dressing removed after the right surgery (11/11/11)

Day 8: Surgery #2

Bethel was nervous and scared this morning. We arrived at the Neurosurgery Check-in at 5:10 am and she was prepped and ready for the OR by 7:15 am. Her fear made it more difficult for me to say "bye" to her. Some how she was still able to make a joke...how this would be a better timed procedure if it had taken place prior to Halloween.

She has heavy bruising from all of the IV lines from last week, so they will try sticking her on the opposite side today. Her anesthesiology is increasing her anti-nausea medications for this procedure to help prevent adverse effects of anesthesia. I'll continue to update throughout the day. Thanks for everyone's prayers and thoughts.

UPDATE:
1:20 pm Dr. Steinberg came out with great news. The second surgery went just as well as the first. Her baseline cerebral flow on the left was 9 ml/min and is now 35 ml/min.

Day 6: Post Op

Bethel was discharged on Saturday (11/12) afternoon and is doing well. She's experiencing some facial swelling, incision pains, and numbness that comes and goes. The numbness was a bit concerning but it is a common symptom post surgery. She tires easily and takes naps throughout the day. Yesterday, her mom and I got her out of the apartment and took a car ride up north just to get out. Each passing day she's been eating much better.

Day 3: Recovery

Bethel is still having a hard time dealing with nausea post op. She's very sensitive to the medications to control her swelling, pain, and nausea. Her central IV in the chest was removed today and she was switched to all oral meds as well as soft foods. Otherwise, she's doing very well. I walked her 3 times today on the first floor of the hospital. Her voice is weak and her speech is a bit slurred at times. Her dressing was removed around 10:00 am which was held on by 3 staples. The surgical team did a great job suturing the incision. It is about 5 inches long.

Dr. Steinberg came by in the late afternoon for a visit. He's very optimistic with the results and believes Bethel may be discharged tomorrow if she eats well. He also suggested alternative nausea medications that may help Bethel deal with the second surgery.

Day 2: Post Op

Bethel is doing very well, but she is having nausea as a result of the anesthesia. She is on a clear fluid diet for today...hospital food is not very appetizing. The ICU monitored her blood pressure very closely. Dr. Steinberg recommends his patients have an average (systolic/diastolic) blood pressure of 90 to 100 mmHg for the for the first night.

She's ready to be transferred out of the ICU, but the hospital rooms are all occupied. Hopefully one will open up this afternoon so she does not have to spend another night in the ICU.

Around 10:30 am, a physical therapist saw her. She was taken out of her bed for the first time and is able to sit up on her own strength. Her recovery is going better than I would ever have imagined. She will have her chest IV and bladder drain cath removed today. A maintenance IV will remain through the duration of her hospital stay to keep her hydrated to help blood flow through the graft. She is expected to be discharged Saturday (11/12) if things continues to go well.

Day 1: Go for surgery

Yesterday afternoon we got a call from Dr. Steinberg's nurse, Teresa, with additional information. He had gone back to the board to review Bethel's case and got a second opinion from the radiologists on scan from the left side. They found some "gaps" where the left brain lacked blood supply, so he's now recommending both surgeries.

We checked Bethel in at 5:15 am PST this morning and she was prepped and ready for the operating room by 7:15 am. There's a patient status board in the waiting area that provides waiting families with information from the room.

8:20 am the anesthesiologist handed her off to the surgeon
9:05 am start of surgery
1:20 pm Dr. Steinberg came out of the OR and told us the surgery was sucessful and went well. He was able to measure significant improvements in blood flow/perfusion compared to the baseline.
2:15 pm transfer from the OR the E2 ICU. We got to see her briefly. She's awake and is able to speak and move in bed.

Visit with Dr. Steinberg

We met Dr. Steinberg and his nurse Teresa yesterday (11/7). They believe Bethel's involuntary movements are hemiballistic and linked to Moyamoya. In fact, some of their patients have similar spells and were resolved after surgery. After reviewing Bethel's angiogram, MRI's and CT's performed at Stanford, Dr. Steinberg is recommending surgery on the right side for now because it is the most severe. He will perform a combination of a direct and indirect bypass for the surgical procedure. For the direct bypass an arterial branch from the temporal artery is sutured to a brain surface artery, and for the indirect bypass he will overlay a scalp artery over the surface of the brain which overtime should grow.

STA-MCA Surgical Procedure and Diagrams

He plans to continue to observe the left side in 6 months to evaluated the progression of Moyamoya. There are signs of narrowing on the left, but otherwise she has good blood flow on that side. The Xenon CT test uses diamox to dilate the arteries during the scans, and she showed good perfusion and augmentation on the left. Approximately 75% of Dr. Steinberg's patients that receive unilateral treatment show progression of the disease on the other side.

Pre-op testing

Bethel and I traveled from Washington, DC to Palo Alto, CA on the first of November for her first pre-op test scheduled early morning of November 2. Some of the testing that Dr. Steinberg ordered have already been done previously back in Maryland.

Her Pre-op schedule is as follow for Week 1:

Day 1: Cerebral Angiogram

Day 2: MRI Brain with and without contrast and Nova Sequencing

Day 3: Xenon CT Scan

Week 2:

Day 4: TCD-Trans Cranial Dopler

           Meet Dr. Steinberg

           Surgery Anesthesia Visit

Day 5: Neuropsychological Testing

Currently (Nov. 5), Bethel had completed the Xenon CT Scan which is part of Stanford's study to quantify collateral perfusion in Moyamoya patients. So far the testings have gone well. Bethel did experience her spells these past 3 days, but it was particularly worse during the Xenon CT which takes approx. 2 hours to complete. She only got two thirds of the way through when the involuntary movements began. The test requires the patient to remain perfectly still for the duration of the test, so they had to stop and admit her in to the ER. Good thing the test took place in the hospital. She's been stuck and poke with IV's and needles so many times I feel bad for her. Otherwise, she's been in good spirits.

The symptoms of Moyamoya

In late July 2011 my wife, Bethel, was diagnosed with Moyamoya disease. The discovery was coincidental to recent weakness, numbness and tingleness she would experience after strenuous activities. Bethel had plans to run the Parks Half Marathon in October, so she was training with a running group in Montgomery County. She averaged 24 miles a week with the group.

She's been dealing with intense migraines that have been worsening in the past 3 years. At times she would retreat into our dark closet until the episodes subsided. The painful headaches would last half a day to a whole day. Her PCP referred her to Dr. Debbie Lin, a Neurologist at the Neurology Center in Rockville, MD for consultation due to her stroke like symptoms. Dr. Lin performed an EEG and a brain MRI/MRA. The results of the MRI were quite shocking. It appeared her right coratid artery was almost completely occluded (blocked) and she had developed a network collateral arteries. These are signs of a very rare progressive disease called Moyamoya.
Reference: http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm.

I was 7 months into my field assignment in Wisconsin when Bethel broke the news to me. We were referred to Johns Hopkins Hospital for a cerebral angiogram to better diagnose the disease and its severity. The angiogram was a painful experience, but it provided clear images of her brain arteries and confirmed she had Moyamoya. JH hospital's neurology team felt that she had developed sufficient collateral arteries to bypass the Moyamoya diseases artery. They recommended she continue a regiment of aspirin and verapamil to reduce the risks of blood clots and strokes. Moyamoya patients are at a very high risk of strokes due to occluded and/or restricted cerebral arteries. No treatment was offered...