It Is Worth It Lord
23 March 2004
Our little guy's story has many twists and turns, so bare with me as I try to explain the many wonders our son presents us. He is now 4 1/2 and absolutely beautiful. We are thankful for everyday with our children.
During my pregnancy with Daniel, my water ruptured at 19 weeks. This is too early to deliver for survival so we prepared ourselves for a difficult delivery. Somehow, after two weeks of no labor or infection, we realized that he may just make it to a viable stage. I was admitted to our medical center until he decided to show his lovely face. Well, 12 weeks post-rupture our son arrived. We knew he didn't have adequate fluid in utero and life felt suspended as we waited to hear if his lungs developed enough or at all. It turned out that they were smaller than average, but big enough for his tiny body. We rejoiced with his small, weak cry.
Later the next day we found out that he was very sick with a septic infection. Again, we were told to prepare for Daniel not making it through the day. We prayed over him and prayed separately. He had some more scary episodes in the NICU, however, within two weeks he was breathing with C-PAP only and slowly coming off IV's and medications. We went home at 6weeks.
We had lots of doctor appointments and medical equipment to find a place for in our house. With no place to go since preemies should be at home during the winter months, we had a wonderful time coming up with fun family activities. We spent every holiday at home while our families were gathered right down the road. It definitely hurt at the time, but looking back, I treasure the opportunity to have those special holidays with my husband and two boys. It certainly made us realize how much we love our extended families and needed them. We truly enjoy get-togethers with family.
After a period of 9 months with little complications, we noticed that Daniel's head was very large with sunset eyes. An MRI was scheduled to determine hydrocephalus. During the scan they noticed that Daniel not only has hydrocephalus, but that he also has a dural avm in addition to all but one major vein thrombosed. Interestingly, both my husband and I know about AVMs from college and family (there is no connection between family members). We were astounded and a little numb. The loud swishing sound in Daniel's head was his bruit.
Our neurosurgeon sent us to Hospital University of Pennsylvania for embolectomy of the avm. The interventional neuroradiologist was able to shut down about 70% of the fistula. He also noticed some concerning vessels while doing the images. Daniel had only one major vein left to carry the blood back to the heart. It had stenosis and looked threatening. He suggested not doing anything at the moment since clotting has not been an issue since whenever it happened (which no one really knows).
The embolectomy went very well. We waited for a few months to see if shutting down the flow to the fistula would relieve the hydrocephalus, but it wasn't doing the trick. We scheduled VP Shunt placement in 1/01. The valve placed was too strong, which left Daniel over drained and very sick. He had a tonic-clonic seizure that led to a code-blue when they gave him too much Ativan. After they picked me up off the floor and gave my husband his heart back when it jumped out of his skin, Daniel revived quickly and went immediately for a CT scan. Amazingly, he began handling the pressure change (sort of).
After a few days we went home. He began throwing up on a daily basis (signs of problems) but more scans said everything is fine. About one month after surgery, Daniel couldn't wake up completely and wouldn't eat or drink. We rushed to the hospital where he began another seizure. Due to the lack of anti-convulsants on hand, it took them 1 1/2 hours to stop the seizure. Goodness knows, he was wrapped in the love of God. The latest scan revealed over drainage. A new valve was placed and a sub-dural catheter to drain fluid around the brain. The VP shunt wasn't able to drain the outside of Daniel's brain. The surgery went well and we went home a few days later.
During the next month, we noticed that Daniel seemed to be losing skills he had just worked so hard to gain. He could no longer pull himself up and he cried most of the time. Within four weeks, we were back in the ER. Daniel began posturing and probably had a huge headache. It took the staff four days to understand what was happening. They originally thought he was just sick, then they thought he was having seizures and finally a bold physician requested an MRI in the middle of the night. He suspected the brain stem was involved. The neurosurgeon who watched the MRI as it was happening said very loudly, "That is the largest brain stem swelling I have ever seen."
My husband called me at home where I was taking care of our other son. He had already contacted family to come and get him and then my parents for someone to ride along with me to the hospital. We knew that Daniel was once again on the edge of life. The stenosis of the remaining drainage vein did prove to be life-threatening. It clotted off with dehydration forcing the AVM to increase its size and drain all the blood directly into the brain stem. He was slipping into a coma and the posturing only continued to get worse. By early morning we understood that Daniel was very sick and we may lose him.
The doctors consulted with us about our options. We decided to have Daniel flown to Philadelphia for another embolization, hoping this would restrict his blood flow to the brain stem, relieving pressure and reduce the swelling. They finally intubated him and off he went to a hospital two hours away. We were already packed so we jumped in our car and sped off to Philadelphia. Lovingly, our pastor and an elder met up with us at a gas station on the way to pray before leaving town.
Daniel had the embolectomy and survived the surgery. He was unresponsive and still very sick. He also had RSV (a serious respiratory disease for preemies and post-preemies). After a week in Philadelphia, we decided that in any case, we wanted Daniel closer to home and had him flown back to recover. It started with his eyelids opening, but not really looking at us. one week post surgery he tracked our movements just slightly. We rejoiced with every regained skill, no matter how small. After three weeks in rehab he was able to sit in a wheelchair with supports. Eating was still a problem, but it would come or maybe not. We didn't much care at the time, we were thrilled at the opportunity to bring him home again.
His oral motor control was slow at returning so he had a MIC-Button placed so we could feed him through the stomach instead of the NG tube (up the nose and down the throat). He gained so much weight that we had to back off on the formula. I didn't mind seeing a pudgy rosy-cheeked child since it is the complete opposite of where we started. By the end of the summer, Daniel worked his way to sitting in a wheelchair with only a lap belt.
Unfortunately Daniel started doing weird things again. This time, he stopped breathing enough to turn blue around the lips and fingers, staring to the left or right and then coughing abruptly only to fall immediately asleep. For those of you familiar with complex-partial seizures, you know how strange they can look. The neurologist suspected epilepsy. He is treated with medication. We still see occasional seizures. About one month later another MRI was performed and again, they suspected re-growth. AAHhhhhhhhh!!!!!!! When does it stop!!!!!
We went back for another embolectomy. To their surprise, the fistula actually thrombosed on its own and they occluded anything remaining. For the following two years we had many appointments but few emergencies. Nurses came to our home regularly to care for Daniel at night while on his g-tube. He still has the feeding tube but only for meds and supplemental nutrition if he is sick.
In January of this year we did another routing MRI. This time, there wasn't anyone at the hospital to review the films since both the neurosurgeon and the neurologist left the same week, right before the scans. The physician in Philadelphia was kind enough to review the films for us just to make sure we didn't miss anything. The first time in 2 1/2 years that they noticed some recruitment of blood flow to the AVM. We have been referred for a second opinion in NY at Beth Israel. We received a call just this week stating that his appointments are scheduled for May 4.
Our pace has slowed and the overall tone in the house is heavy. Daniel was also diagnosed autistic in the beginning of February of this year. This isn't too surprising since he so closely resembled children with autism for the past two years. His medical background has kept everybody from recognizing the behaviors as autistic and not brain injury. As he gains skills the autism is more noticeable. So, with much support, prayers and love from family and friends, we begin a new phase with the diagnosis of autism.
Daniel is truly a beautiful child with a laugh that is contagious. He is different from his peers, but a joy to those who have the opportunity to know him.
I've read many of the narratives and you all know what a difficult road it is. The journey of a thousand miles just turned into a journey of many circles, steep climbs, switchbacks and rocky surface. Every now and then a smooth paved road lies ahead to bring relief to those who are heavy burdened. Thank you for hanging in there for those who took the time to read through the entire narrative. We enjoy reading the narratives as each person has a unique situation.
Update: 18 May 2004
We returned from Beth Israel in New York with good news! After seeing a team of physicians and then Dr. Berenstein (interventional neuroradiologist), it was decided that Daniel will not have an arteriogram just yet. We will go back to NY in Sept. and have another MRI,A done there so they can have the scans on film instead of CD. Apparently, Dr. Berenstein is not fond of digital pictures. After review of the films, he will have a better understanding of how Daniel's avm is functioning, if it is at all. There is definitely arterial growth, but they are not sure if it is progressive.
The trip to NY was smooth, however, the visit with the physicians was otherwise. Total culture shock. I've been to NY plenty of times, but never in the medical community. Little old amish country just does things differently. Fortunately, my husband grew up in South America and is familiar with latin culture, so he was able to understand Dr. Berenstein and his mannerisms better than myself. Well, each adventure brings more lessons and growth. I am thankful for their expertise and also for a somewhat entertaining day. The most interesting part was driving through Harlem and needing to ask for directions out of the city.
This week is Daniel's IEP meeting which we will decide the appropriate educational program for next year. My husband and myself and 7other professionals will develop goals for next year. No pressure there! Our current educational system does not have a classroom for kids like Daniel. Somehow, he seems to fall in between everyone else in development. The autism complicates matters and everyone is scrambling to find a classroom. We've visited every classroom in our county and haven't found the right one for Daniel. We are praying for low anxiety as we tackle another issue.
Thank you for your continued support.
Update: 17 April 2005
Approximatley one year ago I wrote an update regarding our son and his dural av fistula. At that time we were planning an MRI/A/V for Sept '04. Between then and now much has happened medically and educationally. And, we also moved! This was such a blessing in so many ways . My husband has been asking me everyday if I think it's time to cut the grass. This is his very first riding mower and he was very anxious to get out there and ride.
Aside from enjoying our home we keep ourselves very busy with both of our children. Our oldest has some extra needs that require some additional patience and understanding from us and his teachers at school and Daniel, well, it's always interesting. In Oct. the MRI scans showed new arterial growth. An arteriogram was scheduled for the middle of December. The films clearly showed new growth and much larger than expected. The physician called us before embolizing just to let us know what he saw. He was able to shut down about 50% of the fistula so we discussed scheduling another procedure in a few months.
After going back in March the films showed complete thromboses of the posterior dural fistula. This was wonderful to hear. Unfortunately, they discovered that Daniel's arteries decided to grow three more fistulas. It is truely amazing to see the films and how far each artery had to travel to reach its' destination. His physician was able to embolise all of them, but now the discussion leads to the "what if Daniel's body has some triggers that make it grow new vessels."
We are now getting information on anti-angiogenesis therapy. This is typically used for tumors, however, there is always a possibility it could be used in many more ways. If anyone has any information regarding anti-angiogenesis therapy and how it relates to avm's, I would really like to receive that information.
We return to NY for an MRI/A/V in May. The physicians at the INN at Roosevelt Hospital have been wonderful. We are thankful for their expertise and care of Daniel and our family. God is ultimately in control of our lives and so we rest in Him.
By the way, We are again in the process of finding the right classroom for Daniel for next year. We decided he would be fully included in pre-school this year . The teacher is great, the children and the families are supportive and encouraging and his theraputic staff has gone out of their way to accomodate us and even to shed some tears with us. There are many more challenges along the way. We try to take it one step at a time and breathe a little in between.
Thank you all.
Discussion, comments, or questions: Sheri Miller
© Copyright 2004 Sheri Miller
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