Fall 2012 Update

I seem to be having a very difficult time keeping up with this blog. Each time I sit down to reflect I either struggle with writing coherent paragraphs or end up being distracted by kids/dogs/cat. It is much easier to post some cute pictures from our Summer and Fall adventures.

I really should pick up from my last post which was in April. My mom hosted a fund raiser for William in May to raise money for a wheelchair van and home renovations. It was a great success and we are so thankful for all the family and friends that helped and donated! We put all the money raised in a special bank account and are finally making use of it! This week or next we will begin a small home addition that will include a no threshold entrance, a handicap accessible bathroom for William, and his own bedroom that will fit a hospital bed and other equipment. Such thrilling changes coming our way! We are still making progress with getting the van with a lift for William's wheelchair as well. The addition is taking first priority at the moment and then we will purchase the van. My old car has a little more life in it... but by April we will need a bigger vehicle anyway. If you haven't kept up with our family on facebook our latest big news is that we are expecting Beyers baby 3 in late April :-) We will discover the gender of our baby in the making in just a few weeks. Ella is hoping for a sister of course but I suspect it is a boy! Either way we are praying for a healthy baby.
                      In June William had a mickey button g-tube placed into his stomach for liquids and occasionally meals when he is ill. It was a difficult decision for Wayne and I to make but we have absolutely no regrets now. He recovered from the placement surgery quickly and has really benefited from the tube. While we use the tube daily for his hydration needs we are also grateful that he maintains the ability (and enjoys) eating by mouth!
                      July and August were fun filled summer months for our family. We took our annual trip to Lake placid in the Adirondack mountains.  Grandma also treated the kids and I to a great wolf lodge Canada trip on my birthday. Ella still talks about the lodge and the howling wolves :-) In September both my big kids became kindergartners! William attends a great school for children with more complex medical needs. He started taking the bus in his wheelchair and attends full day. It was a big adjustment for both William and I but has been wonderful.  Ella goes to our local half day k program. I try to get in and volunteer often since Ella has had a slightly difficult time adjusting. Her teacher is wonderful though- very sensitive to the needs of more reserved/quiet children! Ella loves riding the bus and going to the specials- especially gym. She still receives speech therapy after school; so I pick her up on those days. We get some quality mother/daughter time for lunch and early afternoon activities while Will is still in school. We recently joined a girl scout troop; Ella joined and I am co-leading. I love the girl-power values that the girl scout program promotes and hope it will boost Ella's self-confidence. So far she has been super excited that mommy is a leader ;)

                  For the most part William has been doing well health wise this Fall. He has been exposed to many "back to school" colds and fought most of them off at home with rest, snuggles, and neb treatments. Although a handful of times since last winter his body has really had to battle to get rid of illness.  Just this week we experienced one of those times; his body temp fell to 91.5 and his heart rate and respirations were low as well. His home care nurse and pediatrician were alarmed and sent us to the emergency department. Luckily he did not need oxygen- just some warm IV fluids and warming via Bair hugger at the hospital. After a day in the ED his temp climbed back up to his more normal 95/96. His blood work and chest x-ray came back clear.... no pnuemonia, RSV, or flu.  It is scary to think that his body started to shut down to some degree due to just a common cold but we are thankful for the doctors and nurses at our local children's hospital and that he made such a quick recovery! He may have recovered at home and ideally we would like to avoid the hospital as much as possible this winter especially with me being pregnant but it was scary to watch him become so cold and lethargic!
                       On a final note our family is excited for the upcoming Holidays and special traditions. One of our favorite Thanksgiving traditions is making gingerbread people with Grandma....yum! We also look forward to our annual Rochester Philharmonic Orchestra outing with my father and siblings. This year the RPO pops concert will be on Wayne and my 9th wedding anniversary- December 20th! We love to enjoy good food and family this time of year... there really is so much to be thankful for!! Happy Holidays fellow bloggers :-) Take care and stay warm, xo Erin

A few Easter Pics and Neurology Update

Ella and Wayne kite flying on Easter
A candid shot of my loves and I .... they are getting so big!
William waving Hello

Our very tolerant dog Porter :-) Ella enjoys trying to dress him in pink!

I am writing from Lake placid near a cozy fire next to a sound asleep and snoring William. My mom and I decided to get away for the week that the twins had off of school. Even though its very cold in the Adirondack mountains it feels wonderful to be away from the stresses of home and everyday life. Last week William and I met with Dr. Mink (his neurologist) to discuss the past few challenging months. Will has been so much more alert and happy yet still struggles more than usual with his eating skills and body temperature regulation. He is still dipping down to 92 and rarely reaching above 95 degrees F. During our appointment we discussed shunt placement (to relieve fluid pressure in this brain ventricles), g-tube placement, among other concerns. Dr. Mink does not feel that William would benefit from a shunt at this time since he does not know how quickly the fluid in his brain has been collecting. Placing a shunt would also be extremely risky for Will given how much fluid fills his ventricles and how little grey matter is present. The risk of a life threatening hemorrhage during the operation would be high. We are going to monitor pressure more closely now and re-check all his hormone levels on a regular basis to make sure his hypothalamus is not losing function. He also does not feel that a shunt would help Will maintain body temperature. Although there is concern that with consistently low temps William is going to have a much harder time fighting off cold/infections in the future. I asked Dr. M what I should do and was not given much of a informative direction or advice. Of course I am afraid... especially after seeing William struggle with this last illness. Meanwhile I am keeping him cozy and using a log to chart his temp 3 to 4 times daily.... just to be sure that he is warming up at some points during the day. For this Adirondack trip I packed his heating pad, thermal sleeping bag, smart wool socks and other super cozy gear.

Next week we finally have an appointment with GI to discuss a tube placement into his stomach for some of his feeds (not all). I know many children online that have thrived with g-tubes.... yet I am really struggling with this decision. It seems so unnatural for my son to be fed through a hole in his stomach yet he is still struggling to get enough liquids by mouth. Equally concerning are the results from the swallow study showing that he is aspirating often. Aspirating is not only uncomfortable but increases risk of recurrent respiratory infections and lung damage over time. I am just hoping to be strong for this appointment and to know what the best decision for my son is. The tube placement will require a surgery and a few nights hospital stay... but may be the best choice for a healthy future. Especially during the winters when he is already struggling with respiratory infections.

That being said I am now willing my mind to be at rest this week and take a break from the decision making process! Both Ella and William are having a wonderful time in the Adirondacks so far. It always amazes me how simple trips can be magical adventures for young kids. I am grateful that William can enjoy this time with his sister....so many memories in the making :-)

P.S ... I am not sure how I failed to mention this but we seem to have gained amazing seizure control since starting Clobazam (generic is Onfi). This medication has been used for years in Europe and Canada with success but just recently approved by the FDA in the U.S. Since starting this medication in late January we went from having multiple seizures a day to only 4 (!) in the past three months. During this time we have been able to wean him off one of his other seizure meds that caused drowsiness.

Update

Hi friends and family,

I plan to be in bed in a few minutes so this is going to be a super quick update on our Will man!! This has actually been the best week we have had in months!! He started back at school on Monday after missing a month. He seemed alert and happy to be back. We love all his teachers and therapists and honestly I was excited to see them all again too :-) It is so wonderful to see William alert and smiling again!! He has really enjoyed this beautiful weather we are having in Rochester.

On Monday we had a swallow study which evaluates how safely he is eating. Unfortunately the study showed that he is aspirating ( going into his lungs) on liquids and purees. He lost some muscle control which is affecting his eating abilities. So, the g-tube is now the best choice for him to get his nutrition in a safe manner. We are

in the process of setting up that surgery. He will still be able to eat smaller amounts by mouth for pleasure though :-)

As for his body temperature... he has been staying at higher ranges but still dipping down to 92.7 or so. At our upcoming consult with neurology we will discuss that further. Fortunately his heart rate and oxygen levels are staying within normal limits. A good sign...

Now, I am off to bed! Goodnight and thank you again for all your thoughts and prayers!

Hoping for Time to Heal

Shortly after William's birth during a consult visit with neurology we were told that he would not live a normal life span. My husband asked the questions... "how long... how many years do children with William's condition live?" His severe lack of brain development was discovered during my pregnancy at 24 weeks; the doctor was not sure if he would survive to birth. None of the doctors were willing to commit to a time frame or a number of years and why should they? While I respect our doctors I know they are not some kind of God, they do not hold crystal balls... they can look at their charts and speculate but in the end they just don't know. I am thankful for that. Yesterday on the phone the neurologist proceeded to tell me that William's brain could be shutting down. That William should be perking up after all the antibiotics he was given nearly two weeks ago. That sometimes children like William outgrow their brains. Their bodies are getting bigger and the under developed brain can't keep up. A doctor in the hospital told me the exact same thing. Upon hearing it twice I had to grip the counter to stay steady and summon extreme will power to finish the phone conversation.

During Will's recent week long hospitalization for pneumonia (among other concerns) a CT scan was done of his head. His brain has always had enlarged ventricles that are filled with fluid... now there appears to be more fluid and even less brain tissue. During the week we consulted with several neurologists and a neuro surgeon about the possibility of placing a shunt to drain excess fluid. All opinions were "on the fence" as they are not sure that the fluid is actually causing more pressure. Could it be? yes... the pressure could be affecting the hypothalamus that controls body temperature, sleep/alert cycles, hunger and more. Yet, William's several month battle with one chest cold after another could also be causing these struggles with body temp, alertness, head control, and eating abilities. The doctors were more concerned after seeing the CT scan and considering that these temperature dips have been occurring and documented by William's school since December. In conclusion they decided to wait and observe how he does over the next month. Placing a shunt is brain surgery and has it's risks... so it would have to be done when he is on good health.

After a week in the hospital William and I were so happy to be home. We had quite a scare in the emergency room so Wayne decided to take the week off and help with Ella. While it's comforting to be home it's also been nerve wrecking watching William's temp and heart rate drop. His oxygen stays at good levels and we continue nebulizer treatments as well as "warming sessions" when he hits as low as 93 down to 91. A normal heart rate for a child is 70-120 beats per minute; Ella usually runs up towards 100. William's is always a bit low- 60's but in the hospital dropped to 30's as well as blood pressure/ o2 dropping. I am not a nurse (and now seriously wishing I had that training!!!!) so I am not sure what the danger level is but when I see his beats per minute drop to 40's with a temp drop it is concerning. The hospital doctors were most concerned when his heart rate was in the 30's combined with low blood pressure.

Despite the many doctors we consulted with in the hospital no one is sure whether William just needs more time to recover from illness or if the changes could indicate brain failure. I am trying to remain optimistic and help my son with all my abilities to recover his will to eat, his strength, and happiness. Occasionally I get a huge smile in between napping sessions. I am more scared than I have ever been in my life. Will's neurologist said he has worked with a few kids whose brain's began to shut down; they lost control of regulating temperature which if it persists overtime can slow body functions and ultimately lead to death. I was told this over the phone. I could feel the fear creeping up in my belly and for the whole day felt this odd sensation of floating. A surreal feeling of floating through the day in a shocked haze. My husband came home and asked if doctor Mink gave a timeline for how long these children had to live after their brains starting shutting down. I didn't ask..... I don't want to know. I want to be optimistic and hope that William is just taking a long time to recover from pneumonia and return to his normal with the coming of Spring. One day at a time.... each day he will be loved and encouraged to heal. If it turns out he will need a shunt to drain some fluid from his brain we will do it and give him the best chance possible.

We are going to meet with his pediatrician about hormone levels- thyroid and cortisol- results should be back by tomorrow. If anyone has any experience with extreme body temp changes or a child that has struggles similar to William feel free to message me with advice etc :-)

A Season in Pictures

Philadelphia

First, thank you all for your thoughts, prayers, and encouragements concerning William and this trip ! Many of you know how apprehensive I was and how much planning it took to make it happen. So thank you so much for your support !!

Wayne, William, and I arrived home last night after our three day trip to Children's Hospital of Philadelphia. Honestly, I am having a difficult time writing any kind of reflection on the trip. Our main goal in meeting with a top Neurologist at CHOP was to get a second opinion on how to achieve seizure control. We also had Genetics at Strong send all William's records. It was a full day for William at CHOP. He had an EEG to monitor brain activity and seizures followed by a four hour appointment with Dr. Bergqvist! Overall the visit was definitely worthwhile and productive but also emotionally exhausting. The doctor was visibly surprised by the lack of white matter in the MRI of William's brain. (It was her first time viewing his MRI with us) She admitted that it was rare but she had seen an MRI just like his recently. Unfortunately the child had just passed away. This was incredibly hard to hear and didn't start the appointment off on the best note. I refrained from asking how old the child had been as Dr. B proceeded to tell us that children with William's condition usually do not live a long life and asked if we had a palliative care team set up. This was NOT a complete shock to us but still very difficult to hear from another neurologist. She explained that given William's lack of almost all white matter in the brain it is unlikely that he will progress past an infant like stage. My understanding is that the white matter in the brain transmits signals to the the rest of the body on how to move, see, etc. The doctor also feels that seizure control is not likely to be completely achieved yet gave us several ideas for new medications/ combinations to try. She encouraged us to consider the Ketogenic diet again with better reflux management and a g-tube. We expressed that so far William loves to eat and still has the ability to; so we are not ready to proceed with a g-tube . If/ when he does require a g-tube to feed we may revisit the Keto diet with her assistance. She also recommended a repeat MRI as he is now older and that we continue to pursue genetic testing. In the upcoming months she offered to share his MRI and records with both Genetics and Radiology at CHOP to get more opinions on causes for the malformation and future treatments. She may also send his information to Dr. Dobyns in Chicago; who I believe specializes in lissencephaly and other brain conditions. Meanwhile we will have another appointment with Dr. Mink (Strong Neurology) to discuss the meds she recommended and talk about a repeat MRI. I am very grateful that she is willing to help us gather more information/opinions and try to figure out what the best choices should be for our sweet William. At home we will continue to challenge him to achieve new things- regardless. It is very difficult for me to make choices about quality of life for my Will Man. Some things are obvious; he is part of a loving family that cherishes him as he is. He loves all kinds of music and being with Ella. He smiles when he hears other children and loves a bright sunny warm day. Yet there are difficult decisions. Is it better quality of life for him to be seizing multiple times daily or to be over medicated and sleepy? To be able to enjoy food by mouth while he is able or should a diet to control seizures be the first priority? It is hard to know what the best choice is... and what worked best for Will a year ago is failing now. It good to know that we have excellent doctors working with us; top minds from different Hospitals working to give William the best life possible.

As an end note: we all got some nasty cold germ that seemed to appear last night. The whole family is sick on the couch.... so I hope this post is put together somewhat well and makes sense!! :-)

My New Mantra

Ella has taken to crying in the morning and William has taken to being floppy/resistant. Not sure if this is normal "back to school after Christmas break" behavior but nevertheless it has caused some serious white knuckled driving. Their "twin take down momma" moments have given me a new Mantra... actually my first. Yep, if you saw me today in my green Honda CRV racing down Winton my lips were sure to be moving. Repeating to myself , " I am doing the best I can"... my new Mantra. The key is to straighten up the spine, sit a little taller, grit your teeth, think happy thoughts (two beautiful children who don't always cry whom I love) and repeat, " I am doing the BEST I can". ;-p

Early Monday Wayne, William, and I leave for Children's Hospital of Philadelphia in pursuit of second opinions on seizure control. The plans have been in the making for months; CHOP has had William's neurological records for a good length of time. This morning I ran around like a mad woman filling the holes in my personal records. An hour later I was alone parked at William's school with a THICK manila envelope containing hospital records, three radiology Cd's, a bag of seizure medications, and a grande Jamaican-me-crazy coffee. (And short three dollars; didn't beat the parking fee in the hospital garage. 0-30 minutes no fee... apparently I am not that fast)... ;-)

After picking up William; speaking with his nurse and teacher I was 20 minutes late picking up Ella! Repeat Mantra...

Once home the kids are happy; bellies full of pb&j and milk and content to play or nap. I pulled out the radiology cd's and dug in. It's a strange thing seeing your baby via CT scans and x-ray images. There he was tiny Will man; just one day old. The images show all his tiny and fragile bones; his tiny legs, arms and baby parts. I found myself upset remembering the day of the twins birth. Although I know William struggled at birth and needed oxygen it still hurts to think how they whisked him away. The birth was long. Ella came first and was lodged in such a way that she took many hours to push out. After William's birth I was hemorrhaging and required oxygen so all my memories are slightly hazy. I remember the general panic in the OR room and how I was jabbed in both legs with needles; something to stop the bleeding. Yet despite all the birth drama what upsets me the most when looking back was not being able to hold my son. I was not able to see William until the next day. Doctors preformed a CT scan and abdominal x-ray before reuniting him with me; poor baby.

Four years later pouring over his tiny bones, brain, and tummy on these x-ray images I am overwhelmed with love for my now not so tiny guy. After studying the MRI scans of his brain I am anything but at peace. Of course I have seen these particular images before but time or frequency does not ease the shock. Where brain tissue should be there is black (how fluid reflects on images). Only a thin ridge of tissue surrounds the fluid filled middle; with slight folds in the frontal lobes and smooth on the back. Why? all I can say is how? and why? I feel tremendous love but not peace. I wonder if I will ever find peace amid the anger and questioning? I do feel grateful though for his life and his tenacity. For his beautiful smiles and strength.

The cd's are now packed with all the other records and ready for travel. I am willing myself to put them from my mind and simply hold my son. My flesh and blood super amazing son that the images can not come close to representing. At four he still loves to snuggle and falls asleep in my lap. He is beautiful and for him I will keep searching for answers; to achieve seizure control and give him the best life possible. To know that my Mantra is a true one.

P.S

Happy New Year!! No really... I probably should have started off the new blogging year with something more cheerful. Our family is actually doing well... on good days and after 9 a.m ;-p Picture and more updates to follow soon.