Meet the McNeil Family

Meet the McNeil Family

Friday, October 30, 2015

Loving Without A Net

As with any journey, there are lots of twists and turns along the way. Yesterday our hospice nurse returned to check on Waverly and she was pleased to see improvement. The fluid in Wavey's lungs had gone away. We were given the all clear to begin feeding her again and things were looking up. Then she starting sleeping and slept almost the entire day, but for an hour before bed. She woke up after a long night's sleep and went right back to sleep this morning. Her little body is slowing down. She is now having difficulty with basic body functioning.

I am going to be much less informative in upcoming posts. Matt and I are beginning to feel a sense of pulling back to maintain some privacy and dignity for Waverly. I will still post updates, but with more emotional detail.

"It is a unique and terrible privilege to witness the entire arc of a life, to see it through from its inception to its end. But it is also an opportunity to love without a net, without the future, without the past, but right now." - Emily Rapp

Wednesday, October 28, 2015

An Update on Wavey Mae Moonshine

I have been meaning to update the blog for a few days, but every day seems to bring a new change or challenge and I wait to see if we will level off. I think change is the word of this season, so I will share the current normal.

Wavey remained fairly stable throughout the end of the last week and over the weekend. Although sleepy at times, she has had periods of bright eyedness and a few glimmers of smiles. 

Wavey has been continuing to have difficulty tolerating her g-tube feeds, so on Friday I took her in to see her Gastroenterologist. We changed her feeding plan to a 24 hour continuous feed and diluted her formula with water to try to alleviate the pain responses we had been seeing.

On Monday we took her to CNMC to see one of our favorite doctors and a valued member of Team Waverly & Oliver. She hadn't seen Waverly in a few months and was copied on a lot of emails. She wanted to *see* Wavey and offer any support she could. She was incredibly helpful with some medication questions I had.

One of the more difficult changes for me has been managing Waverly's care. We have always treated symptoms and been aggressive with her care. With the regression we have seen over the past few weeks in particular, I feel like we have moved from symptom management to pain/comfort management. We have had to make difficult decisions involving keeping Waverly pain free, but sleepy. After seeing Waverly wince when simply touched, the decision to move to this plan was clear. It is just taking me longer than expected to completely adopt it as our new way of life.

Tuesday morning Waverly continued to look puffy in her face in particular. Our hospice nurse visited and was concerned that she is retaining way too much fluid. She advised us to stop her feeds for 24 hours and allow her body to release and absorb as much of the fluid as possible. Waverly's lungs sounded fluid filled and she felt we needed to act quickly to prevent pneumonia.

My anxiety is definitely heightened once again and I feel like the short window of feeling we had leveled off is gone. Our nurse is coming back to check on Waverly tomorrow afternoon. Until then we will restart her feeds at noon today with less volume and at a slower rate.

I have identified very strongly as a mother in the special needs world. As much as the transition was hard fought and filled with sadness, I eventually felt comfortable and understood. I learned the language, how to navigate, and found an identity there. I am realizing that I am now shifting worlds again. Leaving behind the land of IEPs, developmental ages and inclusion. Waverly is now medically fragile and she is on hospice.


That word holds so much power. When I say it, people get it. I no longer have to explain Sanfilippo Syndrome, rare disease, progressive disorder. I simply have to say she is on hospice and I am understood.

Wednesday, October 21, 2015

Settling In

I woke up yesterday morning with a sense of peace. The pressure on my chest subsided and the knot in my stomach loosened a bit. Like most people in the midst of crisis, you begin to adapt to the chaos. And we have been living in this slow moving disaster for 7.5 years. We have always known what the outcome would be and tried to remain realistic with our hopes. We were told when the kids were diagnosed that treatments were not the horizon. We are certainly closer, but not nearly close enough anymore.

Waverly is stable. We haven't seen many changes since Friday. She had a lot more coughing yesterday afternoon and evening. She also seemed more spastic with her movements. We have yet to see a bump in spirit from the antibiotics, but at least we know any pain the infection has caused is slowly subsiding as they take effect.

We are trying to navigate our new normal. Oliver went back to school yesterday. Matt returned to work this morning. We have some visitors lined up over the coming days. I have had some people volunteer to pick up/bring home Oliver from school for me and/or sit with Waverly whilst I drive him. Attempting to keep his routine as regular as possible is going to be one of our goals. Friends have dropped off meals daily since this ordeal began and I have discovered that even ice cream can arrive via Fed-Ex as a lovely gifts from friends who want to share sweetness.

Our hospice nurse and doctor both visited the home yesterday. I think we have a good pain management plan in place. We talked about the nuances of Sanfilippo. I am grateful for the in home support they offer.

There is no time frame. We could settle into and Waverly remains stable for weeks, months, longer. Or things could drastically change. 

After 7.5 years of living in utter stress, we will at times look around and say we're ok. They we glance at Waverly and realize quickly that this is not ok. This is a drastic shift from her most recent normal. When people who know and love her visit, they are shocked by the changes.

One of our pastors visited us yesterday. Matt and I loved the opportunity to talk and pray with her. One of the things I appreciated the most was her prayer of comfort. It wasn't for healing or a miracle, but for Waverly to be comforted and cradled. I think when people are suffering terrible pain or strife, we too often call on God for a miracle without acknowledging the true suffering being experienced. We need to take the time to be present. 

Our job is to relieve her of pain and bring moments of joy into her life. Favorite books, movies and songs. Light up toys and music. Walks in the sunshine, cuddles in her favorite blankets, time with those who love and adore her.

I do not know when, but I do know my daughter is dying. My son is dying. And it is a privilege to hold their hands and provide them with as much dignity and love as we can muster.

Monday, October 19, 2015

1000 words

I snapped this picture of Oliver whilst at the NIH today. He waited so patiently all day and was content with his iPad and snacks. I just love this image. Little brother. 


Matt and I took Waverly to the NIH today to see her primary doctor who specializes in metabolic disorders. She has known Waverly for over 7 years and we consider her a rock star. She is always accessible and willing to consider all options whenever we have had an issue. She was out of the country last week and returned to the States last night to a slew of messages and some panicky texts from me. Her nurse called me last night and we chatted for about an hour regarding the changes. They decided to admit Wavey into the Day Hospital to run a bunch of diagnostic tests.

After a very long day, they discovered one infection which can be treated with antibiotics. It could be the culprit to some of the issues plaguing Waverly, but not all of the changes we have seen over the past two months.

We have a few teams of doctors managing her care: Hospice and they know end of life. NIH team and they know Sanfilippo. Palliative Care and they know pain management. All great doctors. But not one of them is an expert in end of life care for kids with Sanfilippo.

Our NIH doctor was honest to say that although she has diagnosed kids with Sanfilippo and managed Waverly & Oliver's care over the years, she has never walked through the end of a life with an MPS III patient. And our Hospice team has never seen a child with Sanfilippo before.

We left the appointment thankful to have something to treat (i.e. the infection), but feeling at a loss to explain everything else.

Life with Sanfilippo Syndrome has always been bumpy. Changes come and we have to adjust to new normals constantly. There are dips and highs, but lately we have felt like we are free falling.

Seizures. Irritability. Pain. Tremors. Sleeplessness. Crying. Inability to tolerate her feeds. Sleepiness during the day. Urinary retention. No more smiles. Limited eye contact. Drooling. Irregular heart beat. Aspirating.

I want to stop falling and land safely, even if that means navigating a new normal. I am afraid of hitting bottom.

Saturday, October 17, 2015

The Unknown

I wish I could give you all a firm answer as to what is happening with Waverly. I wish we knew for sure. There is still hope that this could simply be seizure medication related and we just need to find the right med. However given so many new symptoms and changes we have seen over the last few days and weeks, we are faced with the sobering reality that this is disease progression. And she is most definitely in pain, which is the absolute worst part of this entire process.

We have known for over 7 years that Sanfilippo Syndrome would one day take her life, as well as Oliver's. We have lived in slow moving disaster mode for years. We have grieved the loss of hope, we have adjusted to new normals along the journey.

I am conflicted as to how to *feel*, as if there is a right way. I need to just allow my emotions to lead me and be honest with the now.

Thank you for all of the love you have shown our family.

Friday, October 16, 2015

Deep Breaths

Little girl is sleeping peacefully in her bed. I'm continuing to sleep in her room with her, unable to leave in case she needs me. Her breathing is rhythmic and quiet. A beautiful sound after the day I have had. I'm not ready to lose her. I'm not sure one is every ready. However today I begged her to stay. It's not time.

Thanks for your messages, phone calls, texts and prayers. They are reminders that we are loved and Waverly has made an impact on so many. 

Our Waverly Mae

Waverly is unwell. At the moment we are unsure if this is a result of some medication changes due to seizure activity or if this is progression from the disease.

In August, Waverly started having daily seizures. They were simple partial seizures which mainly affected her right side. I had hoped they would simply depart, but after two weeks we knew we had to see our neurologists. They agreed that she was having seizures and would benefit from medication. We started Keppra, a standard tried and true entry medication. At first we saw improvement with zero side effects. However as the weeks passed, Waverly became more irritable and her nightly seizures returned with more voracity.

During this time she also started having a lot of issues sleeping. She would wake at 2am and cry the rest of the night. She was exhausted and exhaustion can be a trigger for seizures. We felt caught in a vicious cycle. She also grew more irritable, with lots of crying and wincing in pain when she was touched.

We met with Palliative Care at CNMC last week. The doctor we met with was fantastic and I instantly felt a rapport with her. She increased a pain medication that Wavey has already been tolerating well. She thought a change to the seizure mediation was a possibility, but wanted neurology to make that decision.

We met with neurology Wednesday. He wasn't definitive that Keppra was causing the decline, but he was willing to change the medication to see if some other type would be more effective. We are now in the midst of tapering off of the Keppra whilst titrating up the new medication. He also prescribed a med to help with sleep, which also has an anti-seizure property.

We are about two months into the seizure journey and it is everything I had feared it would be. Our little girl is groggy, vacant, uncomfortable. She is no longer turning when we call her name. I haven't seen her smile for weeks and laugh for even longer. Elmo no longer brings giggles out and reading "Imogene's Antlers" can't elicit a smirk. She cries a lot.

She is also having difficulty tolerating her feeds during the daytime, so we are attempting to slow down the rate and possibly volume of her food. 

I typically write with a lot of emotion, trying to convey how I am feeling in the midst of all of this. However I am emotionally spent. And I feel this all speaks for itself. 

Please keep our little girl in your thoughts and prayers.

Wednesday, October 7, 2015

Let's Buy the Beg Family a Wheelchair Van!

I'm taking a moment to ask for your help. A family we know has three girls with Sanfilippo Syndrome and they are in need of a wheelchair accessible van. This is a costly item for families and there is typically little support offered. For the Beg family, they need to purchase a large conversion style van to accommodate 3 wheelchairs.

Currently this amazing mother of 5, is driving a minivan and lifting each girl into the van. She then folds up their pediatric strollers and places them in the trunk. Once she arrives at her destination, she unfolds and lifts threefold.

Our family has benefited from the generosity of others. I want to shower the Beg family with the funds to purchase a wheelchair van. I have set up a Go Fund Me campaign and I would greatly appreciate your support. Please give if you are able. And share on your Facebook page or blogs. Help us help Abeerah, Khansa and Zahra.

Thank you.