Where to Begin? With Pictures!

This last month Wayne and I have made some very difficult decisions for our sweet William. Of course we were not alone in making these decisions. We had guidance and input from several neurologists and a wonderful Pediatric Palliative care team.

It's difficult for me to write about my emotions now. At some point I stopped doing this blog after our hospital experience with Will in 2012 perhaps as a defensive mechanism. Even as I read back through the old entries I feel raw and exposed. Maybe I've become used to bottling up my fear and worries for him rather than expressing them.

William's body temperature has not really been normal since 2012. At times it does reach the more typical 97F but 94-95 is more normal for him. When he is very lethargic it will drop to 92/91 and he requires "warming". Neurologists have termed this lethargic/cold state "hibernation".  William's brain malformation is the cause and as his body gets bigger his brain may struggle to keep up.

So in 2012 Will's neurologist told us that his brain may be shutting down yet here we are 4 years later!! I can't remember exactly when we started working with palliative care. When I was pregnant with Erik we started shifting more to home care.... suction, oxygen, monitors, and nursing in the home. Having oxygen at home has saved us countless hospital stays! I think a common misconception of working with palliative care is that your child is going to die soon. That is not always the case as many families work with palliative care for years like we have. The team works with parents to improve the child's quality of life.

 In December I did bring William to the ED at Strong hospital because his body temp was below 90 and he was not warming... this time his heart rate was going into the 30's and his respirations per minute were very low. He didn't appear to have a cold virus or any other symptoms so I was pretty scared. We spent a night at Strong and had many tests done (again) and eventually Will warmed and woke up. Once again I was told that the part of his brain that controls basic body regulations is under developed.... nothing else was going on. The blood tests, urine culture, etc came back normal.  He has had a handful of good days since but mostly he has been lethargic and his body temp mostly stays below 93 despite warming efforts... and recently he has more seizures when we try to warm him up. (We are currently adjusting seizure medicines). We were also told that having a constantly low body temp eventually is hard on the heart. No one is sure what to think or what to expect so Wayne and I decided to prepare for the worst. Putting a DNR (do not resuscitate) in place is not a simple or emotionally easy task. A MOLST form most be completed and doctors have to present medical evidence to a court. This is to protect of people with disabilities that can't speak for themselves.  That being said... we do not want William to be put through intubation or on a ventilator if he has a breathing event. William is a kid who likes to be comfortable. If his brain can no longer sustain his body we want to let him go as peacefully as possible. Believe me.... I hate typing this... I hate thinking of him dying.... and I pray that there is a heaven. Ella says that there is a heaven with giant trampolines and loved ones are waiting for us there.... please let her be right!! I hope someday to see William smiling (more than for a few fleeting seconds) and jumping his heart out on a heavenly trampoline.

 I have so many wonderful memories and pictures with my beautiful son. It's been amazing to see him interact with his little brother and continue to love his twin. We had the opportunity to go on a wonderful make a wish trip in 2013 when he and Ella were 6 and Erik was just 6 months old. Every summer we do an annual Adirondack trip, which William loves, despite the 6 + hour car trip. Last summer we brought his oxygen tanks and he slept through most of the trip. Yet, he did have some good alert periods.

I hope that I have many more years with my son. I hope that the bookshelf I am filling with books for him can all be read WITH him. I hope that when he leaves I can still be a good mom to my other kids and survive past the grief. But for now I am living in the moment and trying to do my best for William to live a full and good life.

" I wanted you more than you will ever know, so I sent love to follow wherever you go." "You are my angel, my darling, my star..... and my love will find you, wherever you are." - Nancy Tillman Wherever You Are My love will find you" picture book

Beyers Blog Update in Pictures....

                                                     Erik Mason arrives 5-5-13

                                                         Erik Summer 2013

                                           William's Make A Wish Trip November 2013

                                                           Universal Studios Florida

                                                     The castle at Give Kids the World

                                              William's favorite ride "It's a Small World"

                                                William (and Ella's) Star in the Castle Apple constellation

                                                    Ella making wishes

                                   Family Adirondack trip Summer 2014

                                            William Enjoying some sunlight

                                               

                                             William approves of his minion- Christmas 2015

                                                         Erik (2.5 yrs old) snuggling with Daddy

                                                 Wayne and I in the Holiday spirit

                                              Most recent Picture of all my babies together - January 2016!!!

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